Translate This Page To: Spanish German French Italian Portuguese Norwegian Brought to you From FreeTranslation.com Give a gift in honor of this child. Corey Photo Album Current Page Page 8 Page 7 Page 6 Page 5 Page 4 Page 3 Page 2 Page 1 UPDATE: 1-15-09 woke up this morning and my first thought is, it has been a week since I held Corey and it seems like a lifetime until I am able to do it again. I think of all the parents that have had to do the same thing, a club that no one wants to join but so many of us are forced into. A most perfect child no longer with me. I think of the other parents with sick children and ones who's babies are already angels and how does one continue on. Then I think of the people that do not believe in Heaven and wonder how do they deal with it? Without my faith and God's promise of being together again I don't know if I could go on.I think of the profound impact that Corey has had on the world, all the people that I have grown to love that Corey has brought into my life. He, in his short life has taught more than I could ever do if I live a million more years. I miss him terribly, a part of me is forever missing and will never be replaced................ ******************************* Happy Birthday Princess Emmy!!! How much mommy loves you and still finds hard to believe that you are 3 today! Corey gave us the most special birthday present for Emmy. It has been 2 years since Emmy smiled, we were saying I wonder if Corey gets birthday cake in Heaven for Emmy's birthday and how he always had to grab a huge pile of frosting. Emmy looked at us and gave us a huge smile that lit up the entire house. I want to thank everyone that sent flowers, cards or emails and signed his memorial page, wow Corey has touched lives thruout the the world! There is alot of peace in our house, sadness yes but an incredible amount of joy and peace. As Corey's mom the only thing I had ever wanted was Corey to be pain free and that I know he is. At Christmas I told Alyssa that Corey looked tired, not physically but his eyes looked so tired of fighting. On New Years Eve Corey and I were doing his playlist that is playing right now. Corey was very insistent on what songs were on it. He was snuggled tight against me and looked at me and I knew without a doubt that he wasn't going to be here long. I kissed and hugged him and told him I would be okay and all of us will be. Corey taught me to live life for today, tomorrow takes care of itself. On Corey's last day (Monday) he had the best day I had seen him have in what seems forever, no seizures, no pain, just joy and hugs, smiles and mischief! He had a terrific day at school and this is what I will remember. Corey going to bed, snuggled beside Liam on Emmy's princess pillow, smiling from ear to ear with his Christmas lights and guitar. Right before Corey had gone to bed his lights wouldn't work completely, Liam insisted on new lights, I said wait until tomorrow, Liam said no. He brought them up and Corey's entire face lit up at the sight of them. Corey has always been special, he drew so many people and I can't say how many people have told me Corey refused to let them not love them. We were at a strange hospital with Corey, at first I didn't understand why but in the end it was exactly where we needed to be, he has taught an entire hospital about mito and they all loved him, I will never forget the kindness and compasssion they showed us. Margo UPDATE: 1-9-09 It's Alyssa once again...thankfully...I can say I have no bad news this time because there really isn't much news worse than what's already been said. Sorry to everyone I made the text too small for...hope it's better now. My mom wanted to have me get on here and let everyone know kind of the final arrangements for Corey. The funeral will be held Saturday at noon with a visitation beginning at 10AM at Nelson Doran funeral home in Brainerd. (202 Eighth Avenue Northeast Brainerd, Minnesota 56401) followed by a service at the grave site and a balloon release in Corey's honor and lunch. Memorials may be sent to 14665 CTY RD 139 Pierz, MN 56364. And for those of you in the area, the full obituary will be in Brainerd's Sunday paper. Also, I've started an online memorial for Corey. Sadly, it will only run for 14 days because I can't pay for it but I think that's enough time to show how loved he was :) The address is www.Corey-mckenneyfamily.virtual-memorials.com Please sign the guestbook! Thank you all for your love and support during this very difficult time for my family...I couldn't begin to express how much we all truly appreciate it. UPDATE: 1-6-09 Heaven has gained the most precious angel in the world today. Our beloved Corey Edward passed into the arms of Jesus today. Please pray for me and my family! Margo Corey and his family have been a part of our family since 2004. We have prayed with them, rejoiced with them, and now we cry with them. We will all miss Corey, and comfort can be gained from the knowledge that heaven is a little brighter with him there. Please give Margo and the entire family your support, love, and prayers. In Christ’s Love, Elizabeth UPDATE: 12-31-08 Please keep Corey in your thoughts and prayers. When I was hooking up an extension to his g-tube this morning I got a large amount of blood back. So I went to check his j-tube and that too had blood. He is bleeding bright red blood somewhere in his intestinal tract. The current thought is that he has developed an ulcer. He has severe acid reflux and his last scope showed, a red, boggy stomach and intestine, that is swollen, with numerous sores thruout, including in his esophagus. He is on max meds for this. We have now started one more med in hopes of stopping the bleeding. For now we wait and watch, if he is still bleeding in the AM, he will be transported to Minneapolis. Unfortunately, we are in the middle of a snowstorm. Our small town made the MN news for getting 12 inches of snow overnight. Of course my driveway is completely blown in and I haven't had time to clear it out. But then again our road is not plowed nor did the mail come so I guess it doesn't matter, but I worry if I need an ambulance! Thankfully my neighbor went on his snowmobile to get Corey's meds. The doctor told me to keep Corey quiet and in bed, not a problem as he has not moved all day. I am extremely nervous because a friends small daughter died from a similar situation. Emmy is running a high temp and her urine is super thick and junky. Nothing we are trying is working this time. Emmy had a test late last fall for her allergy to the sun. Her blood was sent all over and apparently some other rare genetic condition was found. They also found in addition to Leigh's syndrome, she also has a complex 1 and 4 mito defect. Her skin condition has a super long name and is shortened to a few letters but it is the cause of her not sweating, severe eczema and poor temp control. I didn't pay to much attention, I will wait for the paperwork from the Mayo Clinic but I know there is no treatment, etc. So pray for my babies and all the other little ones. There are so many little ones that I know that are in the hospital or not doing well at this time. UPDATE: 12-27-08 It was confirmed yesterday that Corey has had a small stroke, didn't surprise me in the least for I already knew it. He is also on another antibiotic. He will also be seen by Emmy's urologist as there is concern that he has developed a neurogenic bladder. Emmy is doing the same, very sleepy with large amounts of sediment and blood cells in her urine. We have changed a few things in her plan so hopefully they will help her kick this infection. We are home all together and that is what is important. The past year has brought a ton of changes but also made my kids and I alot closer and made us realize we are alot stronger than we thought we were. We are also alot happier. Liam, as usual, sums it the best " we have alot to celebrate and this year we can", and the one I like the best "mom you are happy so that makes me the happiest of all, I like to see you with happy eyes instead of sad eyes". Merry Christmas to everyone and Happy Birthday Jesus! Margo UPDATE: 12-22-08 Corey hasn't been awake since Friday. He is running a temp but other than that nothing but some abnormal blood cultures can be found. I am thinking it must be viral. His right side is also abnormally limp and his nad and foot is curled in. The thought is maybe he had a small stroke but only time will tell. Emmy has another infection and a new bacteria in her urine along with her usual. She has been very sleepy and unusually floppy. Most of the time she doesn't have the strength to keep her mouth closed and drools or to keep her eyes open. She is so tired,very tired. Please pray for them! Most of all that we will all be together for Christmas. My other kids are extremely worried about not being together. Margo UPDATE: 12-18-08 Corey finally made it to school today. He was totally exhausted when he got home. So we will have to see how he is tomorrow. Corey finally got his hospital bed today, yahoo finally a bed he can't fall out of. As I look back and compare him to last year, I really can tell the difference and how much the mito has progressed. Emmy has been really tired lately, antibiotics don't seem to be working as well this time. Her urine is best described as extra pulp orange juice, just what you didn't want to hear right? I finally got to attend Evan's Christmas program, he is in the 3rd grade and this is the first time I have not been in the hospital for it. As we were going to it, he said remember last year, you were in the hospital with Emmy and then for the Spring one, Corey was in the hospital. He was so excited that I was able to be there. The kids and I have been so blessed this year, there have been and will be alot of hard times ahead but just when I see no light, God always manages to send someone to cross my path to instill more determination and hope in me. My children and I have never been so at peace, yes we are still afraid for our safety. But yet we feel surrounded by God and his Angels of protection. We are planning on a very special Christmas, we are having a very special cake to honor Jesus's birthday. We have been trying to do one activity each day that is related to the meaning of Christmas, that it is not gifts but all about becoming closer in our walk with Jesus. It is amazing to see what each child can come up with. Margo UPDATE: 11-25-08 Things just seem to go from bad to worse around here lately. I had my CT scan and now based on this I have to have a mri :( I have a couple of disks and nerve and soft tissue damage. Then I was told that the hematoma on my stomach isn't reabsorbing and the damage to my kidney was more than initially thought. My liver is healing nicely. Emmy has another infection in her bladder and kidneys and on top of that is getting a nasty cold and cough :( She has been cranky all weekend and very sleepy. I have even seen her cry tears with no sound, it just breaks my heart. Evan asked me today about Santa, just can't understand why some kids in his class will be getting so much from Santa, while he will be getting one small gift. I have tried to explain to my kids that whenever anyone is kind and nice that is being Santa, that there can't possibly be one man, that if we were all nice and kind then it would be more like Christmas each day. But I can not convince him that Santa and sleigh with reindeer really won't be coming on Christmas Eve. It's hard to hear him cry to his sisters and tell them that he was good and why doesn't Santa love him. Liam just took this all in, wrapped up some of his cars and said to me, maybe Evan won't know that they were mine. I try to focus on how blessed we are, we may not have much financially, but we are all together, the kids and I are emotionally and physically happier than we have been for alot of years, we have food, heat and a roof over our heads. While we may not have family nearby, we have friends and friends online that pray for us each day. Please pray for all our online friends, there are many of them that are struggling right now. Every Thanksgiving I am sad, each Thanksgiving day I will remember Eli, the first child and family I met dealing with Leigh's, it seems like so long ago but then yet it seems like it was yesterday. Margo UPDATE: 11-11-08 HAPPY BIRTHDAY EVAN!!! Evan is 9 years old and according to him it's an extra special day cuz it's his golden birthday :) We went to a waterpark and to Dave and Buster's (kind of like ChuckECheese but with all types of food). I am extremely allergic to chlorine water so of course I avoid pools. The kids had alot of fun and I had alot of help along. Emmy is like me and breaks out in hives from the water so she and I had alot of snuggle time. Corey however loves waterslides, it is alot of work carrying him up and down but the look on his face is priceless, he squeals with joy from the top to the bottom. He also loves Dave's and Busters. He loves to watch the games and loves to grabs tickets with his toes. He then tears them up much to the dismay of the other kids that save them for their prizes lol. Thank you to Starlight/Starbright for giving us the tickets for us to be able to do this. Corey and Emmy are doing okay. Just routine for them. Our insurance has decided (Yahoo!) that she needs hospital type of care at home. So I am hoping that we can get some nursing care. We currently have a pca and they are unable to cath, etc. Now, if only we can find a nurse :( Which I understand can take a lonnnnnggggggg time. We are also discussing getting a central line placed, there is alot of hesitant doctors because of the infection issue and her immune system. I am still throwing up nonstop from the bruises on my stomach. Zofran and I are best friends, the ibuprofen is only making it worse. I go back to the doctor this week to get another ultrasound to make things are still healing properly. I am tired from the minute I get up to the minute I go to bed. Cathing Emmy every 2-3 hours is taking it's toll. It's weird I have been getting up every 4 hours for years. To do meds, change diapers, cathing,nebs, suctioning, feedings, repostioning and irrigating Emmy's bladder takes around an hour. It just seems by the time I am done, it starts all over again. Top it off with financial stress and everything else I am drained. I told my kids this will be a very small Christmas. They seem okay with it, they asked if we still get to do our shoebox for kids in Africa. It made me proud that while I am telling my two little boys there won't be toys this Christmas, their first question was what about the other kids we send stuff to. They always have alot of fun trying to see who gets the most items in their boxes. It teaches kids more than words can ever do, in some places in our world, a new pair of socks, toothbrush or a box of crayons is deeply treasured. Yes there are kids that have never tasted a candycane before. Margo UPDATE: 11-8-08 According to the doctor, it will take 5-6 weeks for all my deep bruises to heal. The nerves in my shoulder will take alot longer but they will heal. I am suppose to take it easy and rest alot. Needless to say, this wasn't a doctor who knows me well nor is it one that is familiar with Corey and Emmy. Actually keeping moving is helping, my shoulder is alot less stiff and I have figured out while I can't always feel anything but pins and needles from my shoulder to the tip of my fingers, I won't drop whatever it is that I am holding. Emmy has started gurgling and running a temp over night. Here we go again! Margo UPDATE: 11-5-08 I am feeling much better, except for my neck and shoulder. I am still having a hard time lifting Corey and Emmy. I have to go back to the doctor as I still have blood in my urine and to make sure the laceration on my liver is healing. I am just one big bruise, ouch! Corey had surgery on Monday on his eyes. He can thru it like a trooper and is back to his usual self today. He hates the eyedrops and is receiving numerous types of eyedrops thru out the day. The doctor also told us that the scar tissue on his eyes has gotten alot worse in 2 months and that he is at great risk for developing an ulcer on his eye. So now we have an additional 2 eyedrops that will be done for life. Corey can hold his eyes shut when he is determined to, tighter than anyone I know lol. We have taken down all our Halloween decorations and have decorated for Thanksgiving. It makes me wonder why there are very few decorations for Thanksgiving. Other than that we are all doing well. Gotta quit typing as my arm is completely numb. Margo ============== Please keep our Mom in your thoughts and prayers. She was in a very serious car accident today. Her vehicle was totaled and she was taken by ambulance to the hospital in Minneapolis. She was in a suburb of Minneapolis when the accident occured. She is sore and bruised all over. They did numerous tests and found severe bruising on several internal organs, including her liver, kidney, spleen and her bladder. She also has alot of blood in her urine. Thankfully none of us were with her. Her only concern was her kids at home. Apparently, she was quite a handful at the hospital, first not wanting to go in the ambulance, then wanting to leave the hospital. She will need to be watched closely over the next few days as her rgans start to heal. However several of us have rearranged our work schedules to take care of Emmy and Corey and all the other stuff she can't do right now. UPDATE: 10-30-08 Wow it's been a long time since I have updated. I have been super busy and haven't felt well. I finally went to the clinic and I have a infection. But I can't seem to get rid of it. The doctor told me to get extra rest. Between meds, nebs and the need to irrigate Emmy's bladder, cath her etc every 2-3 hours it seems like there is never a spare minute, let alone to find time to sleep. I am trying to get some nursing care to help with Emmy's cares but so far no such luck. Between all the things that homecare can't do it seems far easier just to do most of it myself. Corey has been doing okay, I have noticed whenever he gets cold, even the couple of minutes between the house and car, he turns blue on his ears, hands, etc. When the neuro asked me the color of his skin all I could think of was, he is turning into a smurf. Turns out it has to do with his neuropathy and circulation. I was told he will develop frost bite very quickly even in temps 40-50 degrees. Let's see he can't regulate his temp, can't stand heat and now no cooler temps. Now for the best news, Emmy received her Make a Wish. For those of you that know what a sleep safer bed is, a cabinet maker made her a similar bed but with a few additions that I wanted. This guy's wife also does stain glass work so the headboard and footboard has what else? A tiara in the headboard and cinderella type slippers for the footboard. I will try to get Alyssa to get a picture posted soon. He is also making a matching cabinet with a princess design for all her medical equipment. She also received a complete bedding set and curtains as well as numerous sensory items/toys. To complete it her bedroom is being totally transformed and murals will be painted etc, along with carpet, etc. A furniture store has also donated a tempurpedic mattress set for her. Margo UPDATE: 10-27-08 Corey is feeling much better and looks and is acting much like himself:) Now if I can keep him that way until his eye surgery in Nov.:Emmy had her appt in Minneapolis yesterday with the urologist. He removed the foley from her Mitrofanoff and other drain tubes-yeah!!!! So I now cath at least every 3 hours, irrigate her new "bladder" and then instill gentamycin. Just another new thing that I am getting used to. I did find out one disgusting thing. Emmy's insurance won't pay for a sterile cath everytime. So anyone that knows of a lowcost source for 12 or 10 french self caths please let me know. Emmy has enough infections and I need to figure out a way to provide a sterile cath each time. Not much else is happening around here. I have made a few decisions for the kids and I and have made peace with myself in what I have chosen. I will let you know in the upcoming weeks as to what they are but for now I can not. Margo UPDATE: 10-16-08 We really need some prayers. The kids and I are going thru a very difficult time right now. It has nothing to do with Corey and Emmy, they only bring joy into our lives. Everything around us is rapidly changing and no matter what I try or do, it seems like the rug is still being pulled out from under us. I got some very unwelcome news yesterday. I just need to believe that somewhere in all of this, that God has even bigger and better plans for us. But it is hard to believe that when your entire world is crumbling before your eyes. But then again, I hate change! It is hard to convince your kids that everything will be ok when you don't believe it yourself. Please pray that we can somehow feel safe and secure, that the promises that were made to me and my children are kept and that somewhere in the midst of all of this we are given another blessing. Margo UPDATE: 10-9-08 This hospital stuff is just seeming to drag on forever! Emmy has a kidney and uti. Her infection around her incision is much better. We are finishing up the gentamyacin today. I think the plan is to instill the gentamyacin directly into the "new urine collection spot". We are on our way to a few more tests! Margo UPDATE: 10-5-08 I had a wonderful birthday, nothing else matters, I received the greatest gift imagineable, Emmy came thru her surgery and is well on the way to recovery :) For those of you that do not know, she had stints put in her kidneys, ureters reimplanted into her kidneys, bladder removed, bowel and her appendix used to create a new bladder, vesicostomy closed and a Mitrofanoff done. She still has several drain tubes but has far exceeded everyone expectations and spent very little time on a ventilator. The worse part so far was the severe allergy she developed to a pain med. She has also been running a temp but can't find a reason for it, her crp is only mildly elevated. She also had a very long seizure yesterday as well as a long period of apnea. I know all of your prayers helped! Corey is doing okay, I think, even thou I haven't been home! His increase in meds is helping his neuropathy. Our goal is to keep him as healthy as possible because he has surgery in November on his eyes. He is such a fighter and I miss him terribly. Our homecare person told me he cries, mom,mom, mom numerous times a day. I talk to him on the phone but it isn't the same! I'll try to update soon! Margo UPDATE: 10-3-08 This past week has been full of appts and more appts. Wednesday we saw the pulmo and neurologist. We made a few changes with nebs and allergy meds. Both Corey and Emmy's lung function isn't great, both are showing more scar tissue. I was warned that this winter will be very hard on both kids. Of course the usual reminders of everyone getting flu shots, etc. Then we saw our neurologist. That appt was very disheartening. Corey's neuropathy has become much more severe, he now has zero reflexes. That explains the increase in pain. He also told me that seeing Corey not sick or not asleep made him realize how much Corey's tremors and seizures have increased. He also saw Emmy not sick and not asleep for the first time in months, told me he was shocked beyond words at her decline in the recent weeks. Also said her seizures are untreatable. Based on his findings, doctors at Mayo, Gillettes and the U of M, I have to accept this. I wanted numerous opinions as I have heard some kids have success with VNS. Apparently Emmy isn't a candidate for this bt wanted/needed to be sure. All our other appts were routine, just baselines and I already know they are declining so I wasn't shocked to hear it. The next couple of weeks will be very difficult ones for me and my kids. Please continue to keep us in your thoughts and prayers. Also so many of our friends little ones seem to be struggling right now, please keep them in your prayers as well. Margo UPDATE: 9-27-08 As usual, I am sitting here unable to sleep. The never ending issue of not being able to sleep becomes worse each day. My emotions are all over the place. I am trying to be patient with God but sometimes wonder... The legal/court/law enforcement system continue to fail me and my kids, I just don't understand it. Financially everything just continues to get worse. Stress over Emmy's upcoming surgery is getting worse, the kids are in turmoil over it, Alyssa is having a hard time working, just wanting to be with Emmy, she has voiced her fears of being scared that this will be her last opportunity to let Emmy know how much she loves her. The constant worry of how the other kids will do while I am away, making sure they all will be safe is taking it's toll on me, knowing that in order for them all to be safe, they will need to be with different people. They do not like to be seperated from one another. Corey has had alot of pain this week. Medically there is nothing, no ear infection, pneumonia has cleared, that type of thing. It is all nerve and muscle pain or that is their best guess. Emmy has decided that she no longer needs to sleep either. She had her isep plan set for the school year. It hasn't been updated for a year. When you see it all in black and white, it is horrifying at what she has lost. She never did have many skills but to hear your child can't score a newborn level on a developmental test is heartwrenching. Her ataxia and dystonia is becoming worse. Our goals for her are: (well these are mine) that she'd smile and/or make some acknowledgement of different people, look or play with a toy. While my dreams for her are to run and play and talk, grow up and have a family, I need to be realistic. If there is one thing we need it is your prayers, pray for peace for me and my children. Margo UPDATE: 9-21-08 Corey is home again. I was told yesterday that Corey will need eye surgery. The eye doctor said he has very "old eyes", he has a cataract, will be having stints put in and has the start of glaucoma. Fortunately his overall vision has remained the same, just the same old issues with his brain. He is still coughing alot and needs a ton of oxygen but overall is doing much better. Emmy will be getting her preop done. She isn't done with her antibiotics and once again her urine is nasty and showing signs of another infection. Her urine is in the process of getting cultured. Emmy also had her eye exam done. She is extremely nearsighted and has an astigmatism in both eyes. The sad part is the opthamologist told me she has no useable vision. Due to her brain she is not processing what she sees so glasses won't help. Her overall vision has dropped from soso to basically nothing. He also said her strange eye movements are here to stay. I know now why she will turn towards a toy if there is noise but turn off the music and just show her the lights and you get nothing. her hearing also is impaired :( I am so used to this, for the most part I am numb to it, I push it to the farthest recesses of my mind and it is only at night that I face it. Sometimes the only thing that gives me the strength to face tomorrow is God and that each moment of being with my kids makes it worth more than anything in the world. Margo UPDATE: 9-15-08 Corey was taken by ambulance to our local hospital Thursday night. He had developed a mucous plug in his lung. After it was removed he was fine. He wasn't airlifted due to the fog. He was admitted overnight for observation and was discharged Friday morning. Friday afternoon he once again became unresponsive and unable to breathe, he was then airlifted to Minneapolis. He had a couple more mucous plugs, once again they were able to dislodge them. He is currently in the hospital and probably will be for some time based onhis chest xrays. How I hate strep throat, he can never handle the secretions, aspirates and ends up in the hospital with pneumonia. While I was at the hospital, Emmy's urologist ran into me on the floor. Guess he figured out why I didn't answer my phone. He told me Emmy needs surgery asap and that he was scheduling it for as soon as he can get the or. Her vesicostomy will be closed, her ureters will be reinplanted in her kidneys, he's not sure what to do with the bladder as he usually uses the appendix for a makeshift bladder, which apparently hers is "abnormal" and is planning on doing a mitroffenoff. She will then be cathed every 3 hours thru her belly button. It is our only option at this point. So I am torn between being needed in 2 places. Corey is very sick and so is Emmy. Between Alyssa and I we have come up with a plan. Right now I am still in shock, lack of sleep, being sick myself and having the reality of how both kids are doing has left me drained. When they both are at home, you carry on with your normal routine, too busy to deal with the what ifs, it's easier to push back the fact that Mito is taking them away. Please keep Emmy and Corey in your thoughts and prayers, Margo UPDATE: 8-30-08 I am very happy to let you know that Emmy has been released from the hospital and is home again. Sadly her blood levels are not great and based on her kidney function isn't a huge surprise. Once again nothing much else matters, we were given a miracle in being able to leave the hospital with our princess, each time I am at the hospital I thank God for leaving with my child as I am faced with the reality that some people aren't so blessed. We also had to head back down to Minneapolis for Emmy's next appt; we will be seeing alot more of the neuro and urologist than we have previously. Emmy has had quite a few episodes of static seizures lately. We have added more meds for them but they haven't improved. Her dystonia and uncontrolled facial movements have been greatly increased over the last month or so. All the doctors can guess is it is a progression of her disease. On a brighter note, the final details of Emmy's Make a Wish is being completed. Fr those of you that have asked, she is being given a medical rush wish, I know some of you have asked because of her age. Once again, I am in awe at this foundation. Once it is finalized I will share the details with all of you, I am so excited! Corey is doing well, I had thought he was looking skinny and now I know why, over the summer he has grown a total of 5, yes 5! inches but has only gained a few ounces. He has an upcoming eye appt, for some reason his vision appears to be worse. He has been classified as legally blind for a long time but could still see some, it is more att least to me cortical blindness (although the doctors don't think so) but even when I am a couple of feet away and talk to him he looks around but shows no sign of seeing you until you within 6 inches of him. Other than that he is doing very well, he will be in 2nd grade this year but we are torn between having him go to school 2 hours per day or having him put on homebound education. His strength this summer has improved and so has his pain issues. Margo UPDATE: 8-24-08 Emmy remains in the the critical care unit. She is looking better today and was awake for a very short time.Her bloodwork is slowly getting better but we have a long road before we are home. There is some discussion rather to do the next surgery or wait. The plan is to reinsert her ureters into the kidney, use the appendix as her new bladder, augmentation and and a mytroppenoff(sp?).They will also be doing some tests on her colon and intestines. Sorry if this is so mixed up. I am extremely exhausted and worried. There are a ton of things that I need to get done next week and tons of appts. I am also extremely worried about my other kids. They are very upset and scared and after the past ffew months we have had I can't blame them. Please continue to keep all of us in your prayers. Margo UPDATE: 8-11-08 The kids and I are doing okay. Our lives are still turned upside down. I still won't/can't go into any details for fear of our safety. I do thank each of you for all your thoughts and prayers sent our way, they are greatly appreciated! Also, we have alot of truly caring and concerned law enforcement officers. Some say it is just there jobs but wow alot of them are going above and beyond. Looking into Corey and Emmy's faces have touched alot of hearts! Margo UPDATE: 8-04-08 The past couple of weeks have been very hard for all of us. There is alot of things going on that I can't say on here. All I can ask is for all of you to hold us in your thoughts and most importantly your prayers. I really have had a hard time letting go and letting God, wondering exactly when all of this is going end. Margo UPDATE: 7-21-08 Corey and Emmy both had appts in Minneapolis this week. On our way Emmy was covered by a light blanket and has a sunshade in her window. By the time we got to our appt. she was covered in hives and had a few blisters. The doctor we saw was very alarmed because 1) she had sunblock on SPF 70 uva/uvb type 2) she had light clothing that completely covered her plus the cotton blanket 3) she was in air conditioning the entire way and her temp was still elevated. Plus the sun shield. She had some testing done, the preliminary results show she either has porphyria or xeroderma pigmentosum. This basically means she is allergic to sunlight. So from now on she will need to be in a room with no sunlight. That is easy they sell curtains made for that. Whenever she goes outside (even from the house to the car) she will need to wear a special sunsuit and socks and hat, also her face needs to be covered. This on top of wearing sunblock. I also have to get special tinting for our windows. All this stuff is very expensive, then of course the insurance company denied it. I can't wait for an appeal either. The results of what this disease can do to her skin is horrifying plus she is at super high risk for developing skin cancers. Please pray that either the insurance will change their minds or I can manage to find a way to pay for this. She needs this stuff immediately. Corey is doing better now, after a few med changes his pain is better controlled. Please keep all of us in your prayers this upcoming week, it will be an especially difficult and trying week for my kids and I. We need all the prayers we can get! Margo UPDATE: 7-17-08 Emmy has had an extremely rough weekend. A constant battle with seizures, she threw up during several of them and aspirated. She is running a high temp today and is sounding awful. She is on clindamycin, prednisone as well as upping her nebs and is on advil/tylenol for her fever. We are dangling that fine line between dealing with this at home and being sent to Minneapolis. The next few hours will decide. I can almost hear the helicoptor hovering overheard. The only thing we don't have at home is the ability to intubate. Corey has been complaining alot about his legs hurting over the weekend, and this morning he has added his hands to that list. After a warm bath and some massage as well as an extra pain med he seems much better. There is a ton of stuff going on this week, none of which is good. Top this off with Corey and Emmy not doing well and my stress level is at it's max. So please keep all of us in your prayers as I try to deal with all that is going on. Margo UPDATE: 7-12-08 the usual appts nothing is happening. I increased Emmy bladder irrigations to every 2-3 hours instead of the every 4 hours and this seems to be helping. Turns out she also has yeast growing everywhere. Thankfully it is clearing up quickly. Corey is continuing to be doing very well. He continues to amaze everyone around him. He is changing so quickly this summer. I am so thankful to God for all his blessings. I received my first back to school flyer today, reminds me of the dreaded upcoming winter. I just need to trust in our Lord that things will continue to go well. It seems (to me at least) that mito kids need to build up their strength in the summer for so many of them get very sick come winter. Margo **************** Emmy seldom is outside, she like most kids with mito just doesn't do well in higher temps. I noticed during the parade she was breaking out in what appeared to be hives. She was in the shade the entire time. So I asked our pharmacist if she was on something that makes her more sensitive to the sun, he said no. On the 4th we were outside again, she had lightweight clothing on as well as spf 70 suntan lotion that protects from both uva and uba rays. Once again she broke out, this time immediately and it appeared more like blisters. Turns out she has a severe allergy to the sun. Our local clinic is recommending based on the severity I bring her back to the genectist to be tested for some sort of disease where one can't tolerate sunlight. For now we will keep her indoors and if she has to go outside or in the car to keep her covered at all times. Corey had alot of fun at the parades and fireworks. He particularly liked the sirens from the firetrucks. He had alot of fun at the fireworks and was sooooooooooo excited by his glow necklace. He is still doing exceptionally well this summer. All in all we had a great 4th. Anyone looking for mosquito control should try a mosquito magnet. We live in the country by a creek and a swamp and there are a thousand mosquitos per cow. We had only a couple stray mosquitos this weekend. Now if they only invented something to keep away woodticks and poison ivy from finding me......... Margo UPDATE: 6-27-08 Emmy has another bladder and kidney infection. This is just one constant battle with her. We will have to wait for the uroligist to make the final decision but her having her ureters reimplanted is looking more and more like our only option. Corey is doing good. He is just busy enjoying life, words really can't describe how well he has been doing. Margo UPDATE: 6-23-08 Both Corey and Emmy are doing well. Corey is getting so tall, he has grown a couple of inches since May. We are just spending alot of time doing fun, summertime things. There are 2 larger zoos in MN and a smaller one in a nearby town, Corey just loves the animals! There are also the usual grad parties, picnics and barbecues to go to. Most of our time is spent in the pool, at a playground or a beach. I am also getting ready for Sean's birthday (26th) and Mikayla's birthday the 30th. We are also planning on having our usual July 4th barbecue. Not too much else is going on here. Please keep us in your prayers as we are still in the midst of court proceedings. Things are still very stressful but we are all trying hard to remember things are in God's hands and regardless of what may happen, God will take care of us. UPDATE: 6-16-08 Corey has been doing very well. He has been having his ups and downs but for the most part is doing fabulous. The boys have been playing frisbee golf and he wants to play so I bought him a small, soft frisbee, he just loves playing with the other kids. He also has been very excited to see Sean and Cassie's new baby, Skylar. He says, my baby, my baby. It is so sweet to see "Uncle Corey" loving his niece so much :) Miss Emmy is doing fairly well. We have been issues with her urine output and ketosis. But meds seem to be working. No matter what amount of therapy, massages etc her muscles seem to be changing from loose and floppy to becoming intermittently very stiff and tight. Her doctors are noticing this as well. It makes her harder to care for when she is stiff, clothing and changing are difficult. We have been trying everything, warm baths etc. We are considering botox but the docs are hesitant because of her many other issues. Her neuro says its from new damage to her brain. We have had alot of appts lately, just the usual routine twice a year checks that I try to get done before Winter! All the kids have been to the dentist and Corey and Emmy's dental appt is soon. Please keep all of us in your prayers, I won't be going into details on here but there are alot of things causing numerous amounts of stress for me and the kids. Margo UPDATE: 6-12-08 Cal's temp started to spike again tonight like it has for the past several nights. Her heart rate hit 132. I geared up for another fever however when her temp got to 37.9, it stalled. I thought I'd take it again in a few minutes as 38.0 is when they'll allow her Tylenol. To my surprise, it actually went down on it's own to 37.2 over time and her heartrate is down to 122, just a few beats higher than her regular waking rate. This encourages me to think that her body is fighting this "thing" off successfully. I felt a particular urge to pray there be no tylenol tonight and God answered it. The nurses have been giving her PICC line cap a good couple of scrubs with the alcohol swabs after my complaints about policy and the like. And really, that's all I wanted was a little more diligence with sanitizing that little cap - it's the gateway to her bloodstream. To be honest, I'm a bit miffed that I am forbidden to see the hospital policy about how my daughter is handled, but overall, with few exceptions this time, the staff have been good to both of us. We have other battles, unfortunately, that demand our attention and this particular one (policy issue) isn't the hill to die on. This applies particularly with Lucile Packard. We've not gotten any return calls from Dr. Hanley's secretary to schedule Cal's cath and surgery to connect ther ileum to her descending colon and elimate the ostomy bag. Many of you remember the issues we have with her (the scheduling secretary) every single time we need to visit again. After 6 messages, we've not been able to get her to contact us. Please pray with us that this is resolved. It's a source of great concern to us. I asked the attending physician if Callie gives us no more surprises, if we can leave when her round of vencomycin (antibiotic) is finished on the 17th. She looked at me and crossed her fingers, I take that as "that's the plan". We have some friends coming over for Cozy's birthday bash on the 21st and carepage friends are always welcome if you're in the area - just let me know. Will update, Karen P.S. Cal's current vitals are: axillary temp-37.6C heartrate-118. UPDATE: 6-4-08 I will be updating very basic info on the kids. There will be nothing concerning Russ or upcoming appts. This is only to protect the kids as none of us feel safe. I am thinking about adding a password to the site but realize that people with bad intentions will still manage to get it. So for now I will trust that God will continue to keep us safely in his arms. Corey has been doing amazingly well over the last couple of weeks. The decision to keep him home from school this summer was the right decision to make. it has been fairly cool outside, around 60 degrees and this is the perfect weather for a child with mito. He has been a bundle of energy and just a joy to watch. He's been playing with sidewalk chalk, loving his walks, playing with a little fishing pole, loves the worms when they wiggle in his hands and has been helping alot with the garden. He also has been playing with a neighbors baby animals, having a ride on a tractor, etc. It's hard for him to understand that if he has too much activity that later his muscles will twitch violently and cause pain. It's a fine line between letting him do what little boys do and making sure he doesn't overdo it. I am sure other mito parents know exactly what I am trying to say. Also issues with his tummy have stopped since he has gotten the j-tube. He is also getting used to having the pump with him since bolus feedings thru his g-tube is no longer an option. To say the change him over the last couple of weeks is tremendous is an understatement, it is truly a miracle! Miss Emmy is also doing very well, we have gone a couple of weeks now without an infection! It is nice to see her so alert! Even thou she can not cry, smile or talk she is very clear about making her needs known. She still blows bubbles when she is happy but is quick to give you a certain look and growl when things aren't the way she wants them. If she wants certain music on and I put something else on, she growls until I change it, then it's back to the happy noises. It's fun to watch her communicate with us. The other kids aren't doing the best. They are still scared and I have been dealing with alot of issues. They are all getting help (from an outside agency) to help them deal with things. I am praying that while I know things will never be the same that they will get better. It's hard to watch someone you love so much, hurt so much and that I can't take it away. Please continue to keep us in your prayers. The stress is huge and I am anxious and scared about upcoming court dates. It is very hard for me to sleep, I am constantly keeping watch to ensure that we all remain safe. Please pray that God continues to send his Angels to surround and protect us. That God will continue to touch the hearts of Judges and Attorneys to ensure our safety. It's hard to describe how many times over the last few weeks I have felt God's arms holding me and the kids, how many times I have heard him say, things will be okay, trust in me for I am stronger than Satan. Margo UPDATE: 5-21-08 PLEASE, PLEASE KEEP ME AND THE KIDS IN YOUR PRAYERS!!!!! There will be no further updates until Friday unless something major happens. This is based on the advice of the police! UPDATE: 5-12-08 I received the worlds best Mother's Day gift that one could imagine. Late yesterday I was able to leave Minneapolis Children's with Corey safely in my arms heading for home. Our surgeon and GI said we were able to leave early as I am experienced with a brand new j tube site. They wished me a Happy Mother's Day and told me to go home and enjoy my kids :) I spent alot of time thanking God for my kids and praying for those mom's that are without their little ones. It is nice to be home. Outside of a couple of appts in town, we have no appts until early June! Yahoo! I will finally have time to plant flowers and get the yardwork done. Please pray for some issues with homecare and with our neighbors. God knows what they are and I don't want to go into detail. Thank you to everyone that called, emailed and prayed for us, it always helps so much! Margo UPDATE: 5-11-08 Corey's surgery went well. It took alot longer than Emmy's did. He is up on a regular floor today and if he continues to do so well can go home on Monday. I am hoping later on this evening they will start to use his new j-tube for feedings. Not much else is happening, Corey came thru better than I could have imagined! Emmy is okay, the worker said she is still coughing alot but other than that is her same sweet self. Evan was bitten last night by a neighbors dog. Their dogs in our yard has been an ongoing problem. They haven't bitten before but rather chewed and tore up everything from swimming pools to four wheeler seats. Margo UPDATE: 5-5-08 On Saturday we had Sean and Cassie's baby shower. For those of you that do not know yet, the end of May I will be a grandma. It is suppose to be a little girl! Here is a Liam funny, he bought them a hammer. When I asked why he said, they need one and they can hang baby pictures with it :) On Sunday I received a HUGE surprise! I was out in my garage. Alyssa and her friend were over doing laundry, Sean and Cassie showed up as well. When I went inside there was a room full of balloons, a cake, lunch and a shirt. Each kid had signed the shirt and it said Happy Mother's Day on it. I was speechless. They wanted to do something special for me as they knew I'd be in the hospital with Corey next Sunday. Makes me still cry when I think about it. How incredible blessed I am to have such caring and loving people in my life! Today Corey has his preop, tomorrow I have my doctor appt. Emmy is getting sick, I'm praying that it's just allergies and not anything else. My allergies have been awful the last couple of days as well. Corey surgery is scheduled for 7:30 on Thursday. I know that he will be getting a j-tube and based on what the scope looks like they might need to do a couple of additional things. So surgery will last from 2 to 5 hours. Please pray for all of us, Corey's surgery to go well with no complications, Emmy to stay well while I'm gone, my homecare workers sanity! She will once again have all my other kids! Peace for the rest of us, it's always stressful for the rest of us. Corey should be in the hospital approximately 5 days if things go as expected. Have a terrific week! Margo UPDATE: 5-2-08 Corey's birthday was filled with many precious memories. It is those memories that get me through the difficult times. I'm having a hard this week. Just alot of stuff happening. Corey and Emmy are both doing okay so not to worry it's not that. Sometimes I just feel completely overwhelmed and no matter how hard I try, the dirt just keeps on sliding back in on top of me. I am still debating moving, in some aspects it would be much nicer to be closer to Alyssa and to the clinics/hospitals but I hate to leave our school district, friends and neighbors behind. Corey and Emmy both had appts in Minneapolis this week. As much I don't like hospital beds, I really need them for them. Emmy is getting big for her crib and it would be much easier on everyones back. Please pray that our insurance will pay for them. Both Corey and Emmy had adjustments to their braces. We also need to bring Emmy to see someone else cuz her spine has a severe curve in it. The appt went okay and I didn't really hear anything that surprised me. Next Thursday Corey will be back in the hospital, please keep him in your prayers. Once again I am faced with being away from my other kids for a lengthy amount of time, which is never easy on any of us. Margo UPDATE: 4-16-08 Once again there isn't much to update. Jenne was here today again. It really makes me happy to have some company as the picu is a quiet and lonely place. Mike (jenne's husband)picked her up today and is heading to my house to watch Emmy overnight. I really appreciate it as my other homecare worker has been there 24/7. I am meeting up with a friend tomorrow for lunch :) It is amazing what an hour away from the hospital can do to brighten ones day! Best of all I know that Corey will be in very good hands when I can't be at his bedside. My kids told me that my car is buried in snow, hopefully by the time I am home it will all be gone! Right now I'm not aware of any significant plan for Corey. Please pray for clear xrays! Margo UPDATE: 4-12-08 Corey has taken a couple of steps backward and we are hanging on by a toenail trying to not to fall off this cliff. Please say a prayer for him as well as the rest of his family. Margo UPDATE: 4-8-08 Hello, it's Margo this time. After a very long ride to Minneapolis Corey was admitted to the picu. The roads were awful, full of ice and snow. After first arriving at Children's Corey went into what is called static seizure activity. He has an aspiration pneumonia and the picu doc believes that he had a stroke. But sometimes Corey has an stroke like episode so I am praying that this is what happened. We are waiting for a head ct. Jenne, Corey's school nurse will be with me today. I am just planning on taking it one day at a time and am fully expecting a 8-10 day stay. I will update when I know more. Please pray for all of us, I am terrified which is unusual for me but am also worried about Emmy and the other kids. UPDATE: 4-2-08 Emmy had an appointment in Minneapolis today with her urologist. They recultured her urine, did bloodwork and an ultrasound of her kidneys. She is in need of your prayers, she has a vancomycin resistant infection. There really isn't much that we can do for it. We will continue to irrigate her bladder with saline to see if that helps. I really like this doctor, he was the one that mentioned that he prayed for God's guidance in caring for Emmy. He seems to be saddened as I am with this lastest news. As we were leaving he asked if he could hold her, gave me a hug, Emmy a hug and stroked her cheek and told her he'd be praying for her for all can offer is his prayers and trust that God will hold Emmy close. Told me to call him any time and if he was off that his answering service would call him at home. We are planning to have a quiet weekend. I am doing okay as I just have an incredible sense of peace. Emmy, outside of a lowgrade temp, seems very much herself, growling when you move her out of her favorite position, sticking out her tongue and making her happy noises the rest of the time. Looks and acts like a little girl without a care or concern in the world. Margo UPDATE: 3-28-08 I don't know if I am more angry or sad this afternoon. Emmy just doesn't seem to be getting over this lastest kidney infection. Today everything seems to irritate her. Touch her face she growls at you, just a sad, grumpy face and a deep sadness in her eyes. So I called the urologist to inform him of this. Seems our local doctor ran the urine culture but failed to notify me that the antibiotics she is on are useless, the bacteria she is growing aren't sensitive to them. So she needs to be on a different couple of antibiotics. They believe she now has a "super bug", resistant to many antibiotics. Her urine grew 3 different things. Just makes me so sad and angry that as the clinic called it "an oversight on their part in failing to call me", is causing her to suffer and to continue to run temps as this infection continues to sweep thru her tiny, defenseless body. No one she is spending most of her time sleeping. Please pray for me/us, I am going to be making some life changing decisions for my kids and I over the next few weeks. Pray that I will make the best decisions for our family. Margo UPDATE: 3-26-08 I hope that everyone had a very happy and blessed Easter. Ours was very low key and we stayed home. Corey went to sleep early Saturday and didn't wake up until Sunday evening. He stayed awake for a short time and then went back to sleep again. His appt last week with the neurologist and pain doc went ok. His neuropathy is increasing and they did a test once again on his nerves called, I believe an EMG. He has zero reflexes from his hips down and none from his shoulders to his hands. They added another med to use with his neurontin as I believe most of his pain is nerve pain. A couple of seizures meds were also tweaked Emmy's appt didn't go as well. I have noticed that her head when you hold it in your hands seems to be smaller in the back. Also the bumps seem to be getting larger and pointier. The neuro was explaining, when in jumped the pain doc and said to make this story simple it is caused by the plates of her head overlapping because her brain is collapsing from atrophy. I guess I knew this but the comparison he made to something I won't even mention made me sick. The neuro has a way of saying the same thing but in a gentler way. They also said again Emmy doesn't have long as everything is shutting down. When I mentioned God is taking care of her, I was deeply upset when one of them said, get in touch with reality, it's not God it's doctors that save children. Needless to say this doc that has never been a favorite of mine is now much lower. Cuz if it's the medical field I depend on and not God then we would be in a whole lot of trouble! I told them we live not on their timetable but we live on God's. Emmy was thru with antibiotics on Friday, this AM she is running a high temp and her urine is back to bloody, stinky infected again. I am bringing her into the clinic and then we will be headed to Minneapolis to be admitted by the urologist. One of her docs mentioned last week, that we could always forgo using antibiotics and letting her get sepsis /blood infection which would be deadly, said it's an idea. NEVER, EVER will I not allow antibiotics to treat an infection, I can't imagine it. So please keep us in your prayers as we travel down this road of uncertainity, we have chosen to walk this road with God. Please pray for us to have doctors that care for us and believe in God to help us choose the right way on this journey. Margo UPDATE: 3-19-08 As we approach this very special week, I would like to thank everyone for their thoughts and prayers. Between appts and church services I'm not sure when I will next update. Holy week brings back alot of very precious memories, most of them weren't fully appreciated until I became an adult. Yes my kids get Easter baskets but we are reminded of the real reason we celebrate Easter by Resurrection eggs and this year I found a "resurrection nativity", similar to a Christmas nativity. As many of you know, the kids and I have been going thru a very difficult time in our lives. I am grateful to have God and his love for us to help us thru this process. Corey and Emmy aren't doing the best medically but we have found an inner peace that can only be found when you realize that while someone you love may be taken from you that someday you will have enternal life together in Heaven. Corey is losing alot of weight no matter what we try, he is so skinny and has lost 3 pounds in a couple of weeks. He is no longer bouncing back after infections. He is so unsteady but yet he continues to fight and is making most of each day. He truly shows me each day what life is all about and why it is way too short to sit and cry and worry about tomorrow. If we are so busy worrying about tomorrow we have no time to enjoy today. Then there is the lil Princess Emmy, her body isn't very strong but her spirit is. Her lungs, kidneys and bladders are overran by massive infections, she is constantly vomiting bile even if her g-tube is vented nonstop, but yet this little girl acts like she doesn't have a care or concern in the world. Is that what comes from knowing that God will always take care of you? It always seems like there is an intense peace surrounding her, is that the peace you have when Jesus is holding you in his arms when if any other human holds you it only causes pain? As you celebrate this very special week, please keep Corey and Emmy in your thoughts and prayers. Miracles do occur each day! Margo UPDATE: 3-15-08 What is the closest that I can get to Heaven on earth? I had a little taste of Heaven last night. I gave Corey and Emmy a bath, dressed them in somewhat silly easter bunny pajamas and spent a couple of hours snuggling them. Just breathing in their sweet smell, listening to their heartbeats and wanting it to last forever. Corey actually opened up his eyes long enough to give me his silly little grin and to pull my hair. I had a neighbor ask me yesterday how I can do what I do each day. All of you with a special child knows that it is the little moments that so many others take for granted are the ones that we cherish the most and make each minute of heartbreak worth it. Corey has been asleep, it seems like he is always asleep, we have started calling him Rip VanWinkle he sleeps so much. You give him a bath, his meds, dress him, whatever and he stays asleep. Outside of a cold there is nothing medically wrong with him, no other infections, etc. All I ever hear from doctors is, it's the disease progressing. I have come to believe the statement "it is the disease" is short for we do not have a clue! Our cookies are done and eaten so tomorrow we are planning on making our bunny cake. It is the same cake that I have had since I was little using the 2 round cake pans. I still decorate it the same too, complete with jelly beans and green coconut for the grass for the bunny to lay in. As Liam would say, don't tell me we gotta do it the same old way cuz it's tradition, yup it's the same reason we can only color eggs after church on Good Friday, cuz it's tradition! Next week we have a ton of appts in Minneapolis, 3 days and numerous doctors. Makes me tired to think about it! Margo UPDATE: 3-12-08 Emmy is still sick with her bladder/kidney infection. After speaking with her urologist we are taking a wait and see if this is an isolated infection following surgery or not. Neither he nor I think it will be just this once. after this round of antibiotics when she gets another infection we are to head straight to Minneapolis. She will be admitted and further testing will be done on her kidneys and then we will decide what surgery to do next. I don't have much hope for he said, I have put her on the schedule for 3 weeks from today and I will see you in 2-3 weeks. While I am saddened by this latest setback it is nice to know that she has a doctor that really cares and seems as upset as I am over this latest infection. While he doesn't know what to do next, he is working on trying to come up with a plan. Emmy also aspirated and is on meds for another pneumonia :( Corey has caught a nasty cold and cough, so many kids at the schools seem to have the same thing. I am just praying that his cold doesn't turn into anything more serious. Margo UPDATE: 3-10-08 I had to bring Emmy into the clinic yesterday. She has another bad bladder and kidney infection. I called the urologist in Minneapolis that did her surgery. I am so sad, this surgery was suppose to help with all of these infections. Once again 3 days after getting off of antibiotics it started up all over again. It doesn't help when this urologist that is one of the best tells you he no longer knows what to do. We could try the more complicated surgery and remove her bladder but he doesn't think she is strong enough. The other surgery involves removing the bladder, etc. He is going to have a phone conference this week with urologists from around the US this week to see if anyone has any ideas. Please pray for her, any healing I feel will be done by God. I am upset but at the same time have had a great deal of peace in knowing that this is in God's hands. Emmy just looks and acts peaceful like she doesn't have a care in the world. Margo UPDATE: 3-8-08 Tonya- and for those who know her, this is for you. Without others praying for us, I wouldn't be where I am today. Prayers said for our behalf are worth more than all the money in the world! Prayers and worshipping God is what will take me thru the journey ahead that God has planned! UPDATE: 3-5-08 Corey went back to school on Monday and seems to be doing very well. We still see alot of tremoring and pain issues but overall I have no complaints. Emmy still appears to be in alot of pain from the vesicostomy. She had Alyssa in tears as well. Alyssa was giving her a bath and got water on it. Emmy did her "tears flow but I can't cry" thing which breaks everyones hearts that see it. Whenever we move her or change her she does this and looks at you with deep sadness in her eyes, makes me cry each time she does this. Her doctor added another pain med which has helped alot. We also have added Zofran because of tummy issues. During her surgery we found out that her stomach no longer reacts at all to touch and her intestines are getting very sluggish, guess that explains alot of issues she has been having. Right now the gi is debating to change her over tpn instead of j-tube feedings. We are slowly getting used to the vesicostomy. Just figuring out the best way to diaper etc. We go thru about 20 diapers per day as well as diaper doublers and abdominal pads. Any urine on her skin and she immediately starts to break down, hard to fix that when the vesicostomy is designed to drain continuously. But for the first time, I guess in a year, her urine is clearing up! This appears to be the answer that we have been praying for. A couple of the other kids have been home from school with the stomach flu, it just seems to be going around in a big circle thruout the schools here. I was informed today by Evan that I only have a few days between St. Patrick's Day and Easter this year so I should make shamrock cookies this weekend. Whenever he gets something stuck in his head, especially when it comes to cookies, he keeps asking over and over. So I guess we will be making cookies on Sat. I didn't realize that Easter was so soon either, time is getting away from me! We have a ton of appts this month and April, as of today in the next 5 weeks I will be in Minneapolis on 11 different occasions. If only all the snow owuld go away! Thank you for all your thoughts and prayers as the kids and I get used to our new lives. Margo UPDATE: 3-3-08 Emmy's surgery went perfect! In fact it went so well we were allowed to leave the hospital 2 days early! PLEASE if anyone has a diapering solution or experience with a vesicostomy please let me know! The doctor wasn't able to put it as low as he wanted due to her "unique" anatomy so it is up close to her belly button. Plus she also has a g-tube and a seperate j-tube on either side of her belly button. It is working beautifully although it doesn't look very pretty right now. No matter how many diapers and clothing changes we need to do if this cuts down on infections and helps us salvage whatever kidney function she has it will be worth each second. As usual our regular nurses took wonderful care of us. It is these things that matter so much, doctors that want only the best for your kids and nurses that care so much and even if they aren't taking care of your child still stop in to say hi. We had so many different docs from the er to the picu stop by just to see how we were doing. I am so thankful that God has put so many wonderful, caring people in our lives. Please remember all of us in your prayers as the kids and I adjust to our new lives. I am gradually getting used to our new normal. Margo UPDATE: 2-25-08 Corey's surgery went okay. I found out something interesting, your adnoids can grow back, which they have in Corey's case. Shortly after being transferred from the recovery area to a regular room Corey started desatting and was transferred to intensive care. He must've aspirated and so we are once again dealing with a pneumonia. After testing yesterday and more to come this morning it has been decided to move Corey over to a different plan, instead of trying to keep him awake, getting stronger and learning new things our only concern for the future will be his total comfort. The amount of brain atrophy over the last six weeks is heart breaking and according to all new tests their is nothing further to try. Our new objective is to try to stop the neverending seizures and keep him pain free as possible. I knew this deep down before any tests were done but to see it in black and white I can't deny it. The day that has come for so many and the day that we as parents dread has come, when I get home instead of rejoicing I will be making funeral arrangements. My hope and goal for Corey is to take him home surrounded my love and family instead of a hospital room. Margo I now know the reason for Corey's desatting and trip to the picu. It was due to a HUGE medication error. A intensivist and someone representing the hospital came to his bedside to inform me of what happened. Corey is suppose to get .3 mls of amitriptlyne, turns out he got 3 mls. WHich was why he desatted so quickly and during this process of struggling to breath he aspirated. So his lungs had alot of fluid on them. He is back to himself today thankfully. He was started on lasix and clindamycin which has helped alot. He is also being given more pain meds, phenobarbital, amitriptlyne, valium, neurontin and we have added ativan. We also have oxycontin and methadone to use as needed. Overall he seems to be doing much better and outside of his normal oxygen needs is happy and smiley today. All the pain and worry about the future is quickly forgotten when he gives you one of his smiles and kisses you. Emmy is doing well, tomorrow is her preop and I am praying that she will have her surgery on Thursday as planned. She is being very well taken care of which is a huge burden taken off of me. I still worry but I know I have wonderful people taking care of her. Margo UPDATE: 2-22-08 Corey's surgery went okay. I found out something interesting, your adnoids can grow back, which they have in Corey's case. Shortly after being transferred from the recovery area to a regular room Corey started desatting and was transferred to intensive care. He must've aspirated and so we are once again dealing with a pneumonia. After testing yesterday and more to come this morning it has been decided to move Corey over to a different plan, instead of trying to keep him awake, getting stronger and learning new things our only concern for the future will be his total comfort. The amount of brain atrophy over the last six weeks is heart breaking and according to all new tests their is nothing further to try. Our new objective is to try to stop the neverending seizures and keep him pain free as possible. I knew this deep down before any tests were done but to see it in black and white I can't deny it. The day that has come for so many and the day that we as parents dread has come, when I get home instead of rejoicing I will be making funeral arrangements. My hope and goal for Corey is to take him home surrounded my love and family instead of a hospital room. Margo UPDATE: 2-20-08 Corey had his preop and except for strep and the neverending thrush in his mouth he is all ready for surgery on Thursday. I am praying to be home by Sunday. Emmy has her preop on Monday and an appt in Minneapolis on Tuesday. Her surgery will be on the 28th. She will be having a vesicostomy done. I'm worried about the surgery but caring for her afterwards sounds pretty simple. I was told to expect 4-5 days in the hospital for her. Of course Corey and Emmy do not like to follow timelines they always seem to have their own little agendas lol. Between the two of them I am more worried about Corey, he has become very frail lately and just doesn't seem to have any fight left in him. He has basically stopped attempting to walk and now will only pull himself around on the floor using his arms. At least that seems safer for if he tries to stand he falls and gets a new bump, he bruises so easily and he has a bump on the head :( We are going to get a helmet for him to wear, we are just waiting for it to arrive. Someone told me, all little boys get bumps and bruises but it breaks my heart that he has one and I just want him to be safe. When we are at the hospital we are going to be having another mri, there are a few things that are worrying all of us, including his docs, so we need to know if these new issues are our new normal or if it can be treated with meds. My gut is telling me it's here to stay and that's what the docs seem to think as well. He reminds me and others of a little old man with Parkinson's disease. I am done decorating for St. Patrick's day inside, outside I only need to put up the green lights. It has been very cold and windy and dangling from deck railings in the freezing cold doesn't sound like fun! I am so tired of hospitals but when I think of parents that stay for months on end I am ashamed of myself for whining. Makes me realize how fortunate I am. Please keep all of us in your prayers, I will update after Corey's surgery. Margo UPDATE: 2-18-08 Sorry for the lack of updates. We are all home from the hospital. I will be returning with Corey on the 21st for his surgery. We will be inpatient for 3-4 days if all goes well. Then I am praying we will be home for a few days because on the 28th I will be heading back with Emmy for her surgery. This will require a longer stay than Corey's will. She will be having either be having a veciostomy or muddendoroff. Things aren't very good around here. When I returned home with Emmy I was forced to confront an issue that has totally taken all strength I had. I have very little hope and faith left and it has left me to question, where God is. I won't/can't go into details but someone close to me has been stealing Corey and Emmy's narcotics. So I am struggling to deal with why would they, how could they and I fear for our safety. Please keep us all in your prayers, healing for Corey and Emmy's surgery and peace and safety for my kids and I once again. I have had to deal with many crying and upset children this past week, they are very frightened and scared. Margo UPDATE: 2-13-08 Emma is doing alot better. It turns out that she had a mucous plug. She also had a vcug and ultrasound of her kidneys. We asked and saw a new urologist. Emmy will be having surgery on her kidneys and bladder the end of February as well as having a vecosectomy (sp?) a surgical opening will be made in her lower abdomen and the bladderwill be connected to the outside, much like her stomach and small intestine is connected to the outside by a small stoma. Her kidneys are not functioning normally and a portion of each have died due to severe reflux in her kidneys. She is getting each day but it has been determined that the posturing or dystonia is here to stay. so her legs and arms have lost alot more function. But none of this matters as we still have our little miracle with us! Corey still is not feeling the best, his surgery scheduled for the 21st abd hopefully once he gets his "rotting tonsils" as the ent so nicely put it he will be feeling much better. I know that we are sadly losing this battle with mito, I feel deep in the core of my soul that things aren't going to get better. I realize how blessed I am, I was speaking with another parent at the hospital, they had a perfectly normal little girl, in an instant they were faced with a little girl that is brain dead due to a near drowning and deciding to leave her on or take her off life support. I felt so bad as there was little I could do to comfort them, all I could do was listen to their grief and pray with them as they have to make a decision no parent should have to make. Made me realize what a blessing I have been given, I get to spend each day knowing it might be my last day with my kids, not waiting until tomorrow doing it today for tomorrow might not come. margo UPDATE: 2-6-08 Corey still isn't doing the greatest.He is maybe awake 5 to 10 minutes per day. I was hoping after the infection was getting treated his nonstop tremors would subside, as of yet they haven't. We are going to be trying a new med to see if it helps. Sometimes I just think Corey is tired, tired of fighting. The plan is for him to have his tonsils out in the later part of Feb. I should have a surgery date tomorrow. Emmy is doing okay. She once again has a nasty bladder infection or maybe it's the same one that refuses to clear up. I have been put in contact with a new urologist so I am praying he will have something new to try. Thank you for all your thoughts and prayers. Margo UPDATE: 2-2-08 Corey was airlifted from our home to Minneapolis Children's last evening. He is here due to, mucous plug, aspiration pneumonia, strep throat and rsv. The ENT was by this morning and after these infections subside he will be having his tonsils out. I believe a swallow study will be done after his tonsils heal and he will have a bronch. Right now I am expecting to be here about a week or so. This is kind of based on previous experience. It was nice to have so many familiar faces and to have a ped. that knew Corey very well and immediately took over. Emmy is at home with a homecare nurse. Please keep all of us in your thoughts and prayers as we once again travel this road of uncertainty. margo UPDATE: 1-30-08 All of the kids are home from school today as it is very cold and windy here in Minnesota. I think I heard fifty below on the radio this morning. So I decided that today would be a freat day to bake cookies since I have so many helpers! Corey has had a ton of seizures the last 2 days and is holding his head alot. Whenever this happen he gets mean to others but especially to himself. He likes to bite, pull hair and bang his head.I feel so bad knowing there isn't much I can do. Hoping that the increase in meds work and prayer is all about I can do to help. Emmy is doing okay, still battling her usual nasty infections but they are just part of our norm now. She has been so much more lately, she just can't handle saliva anymore. She is on meds to dry up some of it but any is too much. The things she has started to do reminds me so much of Corey, she pulls out her hair, bites her arm if she manages to find it, bites her lips. All these behaviors I know are related to pain. We are getting reevaluated for homecare hours, it's an annual process but with the condition of the states budget I pray that we don't get cut a bunch of hours. I am so tired and drained now, I really don't know if I could do it myself. So please pray that we will get adequate hours to help me out. There are alot of things going on here that have me so worried that I can't sleep. Please pray for peace and comfort. I know that I just need to let go and let God take over. Margo UPDATE: 1-28-08 Hello! Sorry for lack of updates but we now have a new computer so I will update more regularly. For those of you that watched the GMA piece on folic acid, thanks for the emails and phone calls. I immediately called our doc but Corey and Emmy had been tested for it a few times already so it is nothing that would help us. Corey has been doing very well lately, seems like he has had more good days than bad ones. I guess med changes worked well this time. Emmy has been sick again. Her arm has been twisting in, first I thought it was a new seizure pattern but after watching her I wasn't convinced. So after calling and having her checked by the neurologist it was determined that she had a small stroke that affected the top portion of her brain stem. I guess it really didn't surprise me, nothing really does anymore. I could sit here and cry about it but instead have chosen to work on getting her as strong as possible again. I had some questions from people regarding her and her development. So here goes: she has no purposeful movement, she flails and kicks. She does look at toys nor does she attempt to play with them. She does respond maybe 30f the time when you call her name but only briefly. She seems to enjoy music, particularly likes classical music and always perks up whenever we play certain songs by Garth Brooks. Unlike Corey, Kenny Chesney agitates her. She hates things in or around her mouth. She loves to be massaged with lotion but lets you know when she doesn't want to be bugged. It's kind of cute, she gets this grumpy face, looks at you then looks away and makes a growly noise. She does this if you try to straighten her out, put in her in the tub or it's time for her to be cathed, it always makes me laugh as without one word she lets the whole world know that she isn't happy. We have a ton of appts coming up in Minneapolis plus it seems there is alot of school things to attend over the next few weeks. Thank you for all your thoughts and prayers! Margo UPDATE: 1-12-08 Sorry for the lack of updates. Our computer decided to go to computer heaven. First of all, the Princess Emmy will be 2 years old tomorrow!!!!!!!! Wow does time fly by quickly! She is of course having a princess party. Not the Disney kind. We have decorations, a tiara that was made for her with a pinata and the usual other stuff all ready. Everything including her outfit and cake is pink and purple. I am hoping that she will have a good as the last week hasn't been the best. She has another nasty kidney/bladder infection. We were blessed with 3 days of clear lungs and urine but no it's back again. She has been super sleepy, unusually crabby and has done alot of whimpering the past few days. Her cns is on overload and she is just extremely agitated. Corey had his surgery yesterday. His mouth and throat is still bleeding. They had to give him shots to help him clot. All of his permanent teeth are deformed. We were told in a short time he will have no teeth at all. The dentist saved some of them, they remind me of shark teeth with porcupine quills attached. During the xrays, they found that he has an enclosed cleft palate, most of the roof of his mouth is only covered by skin. He also has defective jaw bones, bone where there shouldn't be any and no bone where it should be. Trust Corey to have another unusual birth defect, now Emmy needs the same xrays done as they are assuming she has it too. They weren't able to take out his tonsils as he was bleeding to much so that will be done later. There really isn't much else to tell. I will try to update with Emmy's birthday pics. I am so grateful that we are able to celebrate her birthday on earth with her, what an incredible miracle that is! Margo UPDATE: 1-3-08 On New Years Eve we babysat Alyssa's friends baby girl who is 3 months old. What fun she was to take care of! I was holding her, feeding her, just basically loving her. I said to her how nice it is to take care of a baby that responds to me, she smiles, laughs, etc. Alyssa's friend said to me, why did you say that? I am sure parents that have a child like Emmy will know exactly what I meant. I have forgotten what a regular 3 month old can do. I was in awe how fast she drank her bottle, etc. It also made me incredible sad, realizing that Emmy doesn't come close to the skills that she has. Corey is feeling better, he has been awake for a hour or so in the morning and afternoon. When he is awake he seems interested in playing but still tires very quickly, with him I can say he is well on his way to returning to his normal. Emmy is still sleeping alot and is requiring alot of oxygen, more than she has lately. I am praying that is only because her pneumonia is breaking up. She requires tons of suctioning, breathing treatments, etc. Cathing is a little better, still clogging the tube but has gone from dark brown to green so I think we are beating this infection as well. As I was thinking about 2007, reflecting on the past year I decided to skip to 2008! I am praying that next year I will once again be able to say, it doesn't matter what has happened thruout the year, cuz here we are all together again. If I was blessed with a couple of things this coming year, no it wouldn't be to win the lottery! I would choose Corey and Emmy to be pain free, if God gives one skill back to Emmy I would choose to have her smile. Please pray that Corey and Emmy will at least keep the amount of homecare hours they have now. Their eval is in a couple of weeks. I know some people due to budget cuts in the state are losing some hours, I am always tired now I can't imagine what will happen if they were to loose hours. We do not have the option of 24/7 care cuz neither one has a trach. Yes they require alot of work but I treasure each minute I have with them but I honestly do not know how I would manage without help. Margo UPDATE: 1-1-08 Corey went from bad to worse. He has a couple of large abcesses on his tonsils that had to be drained. The smell was awful, it was gross! The doctor that did it referred to his tonsils to "rotting meat". He will be on antibiotics til the infection subsides and hopefully they will removed when he has his other surgery January 10th. They need to be removed asap but can't be until his lungs are clear. He is still asleep for most of the day but after getting some major kick this infection in the butt antibiotics he seemed a little more active today. He had tons of seizures and needed diastat but that is the norm when he is busy fighting an infection. Between visits/calls to Minneapolis and visits to our local clinic it seems I have done little else. Emmy has another aspiration pneumonia inconjuction with a regular one. Didn't know that was possible but now I know it is. So we are on 3 different antibiotics for her. It seems like between her and Corey someone is always needing a neb or an antibiotic on top of regular meds. So far things are running smoothly and I am praying that in a few days we will be winning this current battle. That's it from our end, we are doing nothing on New Years Eve, except I am dreaming of sleep! The house is quiet as Alyssa took Evan with her for a few days to St. Cloud. Sean and his girlfriend took Liam for the weekend. It is amazing how quiet the house is with the two of them gone. They will both be back tomorrow evening. I bought the kids confetti and noisemakers for tomorrow so they can have their own little News Years celebration with as Liam says the "fake champagne aka sparkling apple juice". They are bugging me for fireworks which is our normal tradition but we'll have to see. I have them just don't know if I have the energy to stay awake to shoot them off! I'll update if anything changes or if we get admitted. So far by the help of the team of docs in Minneapolis we have been able to stay home. We have been blessed with wonderful teams of docs in Brainerd and homecare that are doing their best to make sure everything is place to keep them home. If anyone else has been debating to place their kids on hospice, it has been a Godsend to us. Without hospice and a wonderful team we would definetely be in the hospital. Margo UPDATE: 12-28-07 FRIDAY AM HAPPY BIRTHDAY ALYSSA! She is 20 today! Gee does that make me feel old! Jenne watched the kids last night so Alyssa and I were able to go out for dinner and hang out together at the casino. Corey is not doing well and I am heading to the clinic with him. After a week of antibiotics his tonsils are still red, swollen and touching together. He is asleep close to 24/7. I sure we will be heading to Minneapolis today. Please keep him in your prayers! FRIDAY AM HAPPY BIRTHDAY ALYSSA! She is 20 today! Gee does that make me feel old! Jenne watched the kids last night so Alyssa and I were able to go out for dinner and hang out together at the casino. Corey is not doing well and I am heading to the clinic with him. After a week of antibiotics his tonsils are still red, swollen and touching together. He is asleep close to 24/7. I sure we will be heading to Minneapolis today. Please keep him in your prayers! UPDATE: 12-27-07 We had a very nice Christmas. The kids all got a couple of things on their lists so they were happy. I had told them earlier that they weren't going to get much with Russ not working for awhile and mommy needing a new car. But we made sure they got a few "important" things on their lists. Corey slept most of last Thursday and since that time has only been awake 4-5 hrs the entire time. He is just wiped our from strep and antibiotics seem to make him tired as well. The one thing that sticks in my mind from Christmas Eve is the doctor telling me that he is actively dying. All I could think was aren't we all from birth, actively dying? The one and only smile we saw from him was when he saw his drumset, that boy loves anything to do with music! The next time he is awake we will let him play with his new guitar. Emmy had to be brought into the clinic this am. Her cath keeps on getting clogged. We need to flush her bladder out with saline in hopes to help with this problem but yuck! She tested for staph, gram neg and pos this morning. We switched to antibiotics that are injections and a new oral one. Of course the thrush is back in full force again. On a sweet but sad note, Liam and Evan asked me why for Christmas doesn't God let Emmy and Corey have one good day, why do they have to be sick even for Christmas. Liam told me he just wants God to make Corey and Emmy better, his presents didn't matter anymore.Everytime Emmy cries her silent cry but tears are running down her face Liam gets very upset that no one is doing anything to make her better. He kisses and hugs her and tells her that everything will be okay cuz he is there. Corey's hair has always been very brittle and breaks off easily. Emmy is now loosing her hair in the back and under her long baby curls in front, it is very short, brittle and breaks off when her touch it. It never bothered me with Corey, we just shave it close. But we have made the hard decision to cut Emmy's hair as well. It breaks my heart but all of her hair is breaking, twisting into knots and very frizzy. It is like the shaft is twisted and kinking the hair until it breaks off. So I guess we will be investing in a ton of hats and headbands for her. I and her doctors don't seem to think we have much choice in the matter anymore. Corey's teacher's husband died unexpectedly and our dear neighbor's father died yesterday, please keep their families in your prayers. Margo UPDATE: 12-24-07 Oops guess white type on a white background won't work LOL. I spent most of my time sitting in urgent care with 3 sick kids. Dealing with doctors that are okay with Liam but want nothing to do with Corey and Emmy. It was tell what antibiotics you need and the dosage you usually get for them. Liam and Corey have strep. Emmy in addition to her pneumonia and thrush once again has another kidney and bladder infection. Everytime I cath her the other kids complain about the awful smell, it burns and makes my eyes water. So thank God we were planning on staying home for Christmas as no one feels like leaving the house! Liam is very whiney, Corey is either crying or is asleep, Emmy is sleeping and when she isn't she is crying her silent cry but with tears falling. I am praying that after a couple of days of antibiotics we will be back on our way to recovery. Liam is seldom sick so he is the worst one, doesn't want the medicine, hates pills, why can't it taste better and why does he have to be sick. It is snowing quite heavily here, Alyssa should be showing up soon and will be here till Christmas Day in the evening. We are kind of having 3 dinners cuz no one could agree. Today we are having turkey, tomorrow ham and Christmas steak and salmon. I gotta run, the homecare nurse should be here soon! Please pray that we will all be together for Christmas at home! Margo UPDATE: 12-22-07 Corey's picture was chosen! So for any of you that receive newsletters from MAW will soon see Corey's artwork in one of them! Emmy has another aspiration pneumonia :( We are struggling to keep her home with us for Christmas. Please pray that the antibiotics start working soon. She also has a nasty yeast infection again. The doctors also aren't pleased with how her urine looked yesterday. My only wish for Christmas is for her and Corey to be comfortable. It would be nice to have a day with no infections, no pain or seizures. A few days ago I was talking to a group of their doctors thru the hospice program. I said things were going relatively well when one of them said, looking thru your records, I'll see you Christmas Eve in Minneapolis at the hospital. It seems everytime I say things are going okay something happens! Corey had a very sleepy day yesterday and is still sleeping this morning. I think he is getting sick as he was running a low grade temp and coughing alot. But then again maybe he is just overly tired from all the excitement at school this past week. Today is the first day of Christmas vacation. I decided to head off the "can we open just one present please" by allowing them to each open one gift per day. But whoever complains, argues, or asks for another one doesn't get one the next day. I made sure Liam and Evan's were dvd's, ahhhh peace and quiet last evening! Instead of making all our neighbors trays of cookies this year, I will be making pies, pies and more pies. I thought this would be easier. After peeling tons of apples, squeezing lemons, etc I decided cookies are so much easier. I did make several kinds of cookies for Russ's fellow employees today and of course for Russ and a few of our closest neighbors/friends. Please keep Corey and Emmy in your prayers as well as all the little ones that fight so bravely each day. Remember the families that have lost little ones as well. Margo UPDATE: 12-17-07 Emmy is still dealing with a fever and uti. Her urine has been cultured so the antibiotics should be working but so far they don't seem to be working. She also has another aspiration pneumonia. It is strange to have her asleep for so much of the day but between the infections and meds for them it is just wiping her out. We can cath, bathe, give meds, etc and she doesn't respond. We have had her dressed up each day, she has so many Christmas outfits and is so pretty in all of them. Corey is doing well, he lost a few more ounces but has been spunky and into trouble lately. He has started to throw things at anybody that crosses his path. It started out with his syringe but has progressed to whatever he can find. He seems to find this extremely funny but if the person has Emmy he doesn't do it. What a smart little boy! I just want to send videos to his docs that send out the letters that he is unaware of his surroundings. I went to both boys Christmas programs this week, they were very good but Liam's class in particular did a wonderful job. It makes me wonder how the music teacher gets them to behave so nicely. I have had quite a few of them in my home and my Liam included is very hyper and it amazed me to see what little gentlemen they were, what a talented group of kids! I realized today that I should started on Christmas shopping and baking, it seems like I always think, I'll do it tomorrow. Lucky for me Alyssa and her roommate love to shop and wrap presents. They are doing the majority of it for me. I had to get 6 inches of hair cut off. My hair is usually well below my shoulders. Unfortunately for me Russ failed to mention that engine starting fluid can backup and send out flames, when you are bending down and trying to squirt it in the right place. I saw the flames but couldn't figure out what the smell was, hahaha, it was my hair. At least it wasn't my face! I had been thinking of cutting my hair as Corey and Emmy both pull it but just never got around to it. God does work in mysterious ways! Tomorrow we are planning on baking up tons of different cookies. One of my best childhood memories was baking as a family. But I do know now why my mother hated cleaning the kitchen afterwards. Cookies frosting sprinkles 8 kids equal a huge mess and sprinkles in the corners until Valentine's day. Enjoy your weekend! Margo UPDATE: 12-12-07 Sorry for the lack of updates. Things haven't been going well around here. It has nothing to do with the Corey and Emmy thou. I don't want to go into many details but our family definetely needs your prayers. Things went downhill Thursday and just kept getting worse. It is times like this I definetely am unsure where God is, I know that he only gives us burdens he knows we can deal with but sometimes I question why me. Corey is doing okay. We are still having issues with doctors concerning our choices for him. Once again the turn around in him is pretty amazing. He has been awake alot, tolerating food very well and is very busy enjoying life. Yeah we still with seizures, choking, tummy troubles, but that is our norm we know nothing else. Emmy is okay. I am making arrangements for a different urologist to see her, she is still cathed and after antibiotics, including iv ones, instilling them directly into the bladder her urine remains the consistancy of corn syrup and is green with white chunks, I know gross! The new urologist is going to do a slew of different tests. I am relieved as the previous one didn't want to treat her agressively, as he stated to our ped, she is a dying child, fast progressive disease, etc. I just need to feel we get to make the decisions and not the doc. She has been having alot more seizures than usual which usually means a very nasty infection but there are doctors that refuse that a mother just might know more than them LOL. We have some exciting news to share, Corey's picture that he made for MakeaWish was chosen to be in the finals. It will be displayed at the Mall Of America here in MN for all to see. There were different topics to choose from and since Corey can't draw your typical picture we chose "in my wildest dreams", Corey filled the paper with glue that had glitter in it and stuck pompoms all over it. He had fun and to me that was all that mattered. It took close to a week to dry. I wasn't going to send it in but then I thought well they auction them off and maybe we can help another child get their wish granted. Mall visitors voted on the best ones. I am hoping over Christmas to get to see it displayed. So we now refer to Corey as Picasso. This week I will be getting a link to all the pics, I will post it, they are doing voting now for national winners. As you hurry around these next couple of weeks, trying to finish your shopping etc, please remember all the families that will be without their little ones this Christmas. Margo UPDATE:12-6-07 Corey is doing well. He has been busy playing with the Christmas tree, he thinks he needs and wants all ornaments on the same branch. It is nice to see him playing with something other than a syringe so he gets his way. And the other kids even have to question why there are no breakable or none of their baby ornaments on it? He has lost just under 5 pounds and I'm not sure why. So I tried upping his amount of formula and all we end up with are major tummy troubles. I can figure out why just a half an ounce extra and his system freaks out. Emmy has not been doing well since Sunday. Saturday she was at her best, Sunday started the nonstop seizures, gagging, yucky sounding lungs and urine that looks like green syrup. So we are back on meds but so far they have had no effect. We are in the middle of another snowstorm. So I got the chance to head outside today to shovel. At least the snow gives me an excuse to sneak outside for awhile! Sometimes I have been afraid to go online for so many little ones have earned their wings lately. I won't mention names because I might forget someone. Please keep them in your thoughts and prayers as their families struggle without them this holiday season. Margo UPDATE: 12-1-07 The Dance is a song by Garth Brooks, unfortunately I can't find a way to play it. We are so incredibly blessed to have Corey and Emmy a part of our lives. God had plans for them long before we could even imagine what it would be like. I know some of you know this and some don't but to me it's still a miracle they are here. When I had Evan I had to have an emergency c-section. My uterus ruptured front and back and cervix tore. I required 34 units of packed blood cells before they thought I had a chance to live. We were told another baby was impossible. Not quite a year later I was still having problems and was scheduled for surgery. My regular doctor and wasn't there and the gyn that was insisted on a pregnancy test and ultrasound before surgery. On the screen before me was tiny little thing that looked like a grain of rice with a blip that was it's heartbeat, it quickly stole my heart this little one that wasn't suppose to be and soon grew into our Corey. Then along came Emmy, another miracle from God. How many of us are blessed with not one but two angels from God? That is why I love the song The Dance. Even if I'd known before hand the pain, the dance makes it worth every second of pain. We have made our decision, it was actually not that hard. Some of our doctors and family members may not understand but this is what best for our family. In January we have decided to admit both kids to take them off meds and start over. We need this time with Christmas approaching for it to be family. All appts have been cancelled for December. It will be all about making memories. The Christmas programs, cookie making, decorating,etc. We feel really good about this and felt this is what is most important, family and being together and the knowledge that God is taking care of things. Some of the docs aren't too pleased but if this is any of ours last Christmas, I want to make it the best it can be. When questioned by doctors, they keep on focusing on Corey and how he is in the final stages of mito, they are guessing for only God knows what his plans are. I don't think they understood when I asked them are they sure if the rest of my children will still be here as well, then they switched to Emmy and how her disease is rapidly progressing. I just think of how many of us have healthy children one day and never get the opportunity that we have to say goodbye, that in one instant they can be taken from us. I am blessed for God has given me the opportunity to think about this. My heart breaks as I can't imagine what it is like for your child to be in an accident and you have to make these decisions instantly. Russ wrecked my car yesterday, he hit a deer, the hood is wrecked, blinker, headlights, and bumper and windshield smashed from the deer hitting it. Thankfully he is fine and no one else was involved. It's moments like these that make me realize how much one takes life for granted. My car is wrecked and we didn't have full insurance on it so we have to pay for it ourselves. So all we have to drive is Russ's truck. Which I can't drive and carseats don't fit and just when I was wondering what to do as a new car isn't financially a possiblilty right now our dear friend and neighbor offered us the use of his van until we figure out what to do. Thank you for all the kind thoughts, prayers and emails, it is only with all of your support that we are able to thru this rollercoaster we call life! Margo UPDATE: 11-27-07 How does one as a parent deal with being told the worst possible news of all? I was sitting at Minneapolis Children's Hospital yesterday in a midst of a care conference in shock. I was told based on Corey's most recent testing that he is in the final stages of life. I was told he might have a few weeks to couple of months at most. Here I was expecting pain meds and not this. Perhaps deep down, hidden in the recesses of my heart it is something I have known but have managed to keep it hidden. They would like to admit both Corey and Emmy and take them off all meds. Then start over and reintroduce only essential meds. I have often made the comments to the doctors that I feel that they give me one med to help with one thing but then add 3 more to help with the side effects of the first. Seems I might be right. This is a hard decision as if Corey only has a few weeks I hate to spend it in the hospital (we would be inpatient 2-3 weeks). Then a part of me wonders if the doctor is right are some of Corey's pain issues caused by side affects of meds? I'm sure all of you have heard if you take too much tylenol or advil then stop you will have rebound headaches,it is similar to that but to a greater extent. The team also asked me to list my priorities, they were for Corey: Comfort, quality of life,to maintain skills, to live long as possible, and to attend school. I was questioned about the live as long being third. To me that isn't as important as long as he is comfortable and has quality. This morning I was debating with myself about sending Corey to school, wanting to keep him with me to the promise I have made to him. So off to school he went as I can't take something away that brings him so much joy. Then with a few minutes to spare, we raced to St. Paul for Emmy's urology appt; She will now be cathed 5 times a day, an antibiotic distilled into the bladder and hopefully this will help. As the urologist put it, 1) you have a child that is on hospice 2) with the sounds of her lungs and blood cultures no anesthesiologist would touch her for surgery. So my head is swimming from all this info. As I sit here with my own grief and decisions I need to make, I think and pray for the parents that have made these decisions and I cry for the parents I know that will one day be facing these as well. The other kids are upset as if Corey and Emmy are admitted it will be once again missed Christmas programs, family activities, parties, etc. Last year I was in the hospital with Emmy and missed so much. This makes it all the more difficult as I think of how many times I've had to disappoint the other kids because of medical issues. Please pray for all of us as we deal with everything that is going on. Margo UPDATE: 11-20-07 We had a pretty uneventful weekend, which is just the way I like it! Corey and Emmy will both be seen at the pain clinic in Minneapolis next Monday. I am praying that we will get some insight into how to help him with his breakthru pain. Emmy is tagging along because as our pain doctor put it, if she isn't seen then the following week her pain will get out of control and this way you won't need to make a second trip. Please pray for guidance for their team of doctors for kids with mito do not metabolize meds like other children. I'm am so grateful for this doctor and the respect that he has for us. He also has gone the extra mile in intervening for us in so many ways. Then Emmy has her appt with the urologist. So it will be a long couple of days filled with appts. We are staying home for Thanksgiving. I really want to keep the kids as healthy as possible and Emmy in particular is medically fragile as she continues to fight off four different infections. I will be happy to take down Thanksgiving decorations and replace them with Christmas, they seem so dreary with all the muted earthtones. We are of course making our usual sugar cookies in the shapes of turkeys, pilgrims and mayflowers. This year I am once again attempting to bake a pumpkin pie. It always turns into a soggy, yucky mess. I can't figure out why apple is so easy but pumpkin is so hard for me. But to be on the safe side I plan on buying some. I was making a feeble attempt to think of all the things I am thankful for. All my children will be with me this Thanksgiving, Russ survived his heart attack and is making a slow recovery, I have food, a house and all the basics of life, I have God in my life to carry me thru the saddest times, I can't count the number of friends I wouldn't have made if it weren't for Caringbridge or mito disease, doctors that truly care and want only the best for my children. Most of all I know for a fact that miracles aren't only to be read about in the bible and that there really are angels that are sent into our lifes, sometimes the vision we have of angels aren't the angels that we see before us. Have a very Happy Thanksgiving! Margo UPDATE: 11-16-07 Corey slept his way thru pics yesterday, so none of him. I'm not sure what is going on, he went to sleep on Wednesday night early, slept all day Thursday and is still asleep now. I'm hoping it's just a couple of mito days and that he will be back to himself soon. Our gi called, when Corey had his j-tube put in last month, they also did a biopsy. Based on that biopsy and his ever decreasing motility we are changing formulas. Just one more way we all know that the disease has progressed. Emmy is sick again, another pneumonia :( So we have added another antibiotic. She was awake for her pics yesterday and they are so cute! Unfortunately, the slide show site wouldn't allow me to upload them. Alyssa came home, thinking it was just me that didn't know what I was doing, nope still wouldn't work. So I guess she will try again at Thanksgiving. The photographer made quite a few comments on how sad she was to see Emmy. Last year Christmas pics she could almost sit. This years she always appear to be on a weird background. It is one of the photographers trying to hold her up and manage to keep her head up. She can no longer support her head or trunk. As the photographer said, it's like holding on to a slippery eel. So outside of some weird backgrounds and some where you can see hands, they are very cute. I am hoping I can share them all with you soon! If you would like one send me an email and I will mail you one. Margo Corey and Emmy had appts with the neurologist today. It went pretty well. Just a few adjustments to med. Then he sent us to the pain clinic. Corey has been having quite a bit of break through pain. Both kids will be heading back to the pain clinic the end of the month for full evaluations. Finally the place I struggled to be for so many years has arrived. Doctors that have agreed that comfort measures are the most important thing rather than fear they will become addicts. Part of me wants to jump up and cheer but part of me is sad as I realize all their doctors have agreed there is nothing else to do. It's hard to hear a doctor tell you that it's not getting used to the pain meds but rather it's the disease progressing. Emmy has started urinating a lovely green, junky urine again. So we are back on antibiotics :( Our appt the 26th can't be here soon enough! Sorry I have updated for awhile or been around to visit many sites. I have been so tired and just haven't had the time to get on the computer. It seems like all we do is head to different appts; and then all I do is give meds, breathing treatments, more meds, feedings and hop up every few minutes cuz Emmy's machines go off. I had to download her monitors to the doctor, one night (I think it was Sat) went off 193 times for apnea or low oxygen levels. This plus attempting to have some sort of normal life with the other kids is taking it's toll on me. Corey and Emmy have their pics on Thursday. I am hoping they will both cooperate. Frankly, I'm just looking for a pic where they are both looking somewhere near the camera! Have a great week! Margo UPDATE: 11-9-07 Emmy's testing on her kidneys, bladder and urinary tract did not go well. They stopped the testing in the middle and told us to head to Minneapolis as soon as we can get in. Apparently Emmy's urinary tract and kidneys are worse than first believed. She has massive reflux into her kidney's and her right kidney is no longer functioning normally. After waiting forever for her to urinate they then rescanned her bladder and she had a large amount of residual urine. Also they took a urine sample and after weeks of antibiotics, it was still cloudy, full of chunks and bloody. Sometimes I don't understand how she can look and act so normal (for her anyhow) when her bladder is so infected as are her kidneys. The docs think it is because all she knows is pain :( Sometimes I dream of the day when she will no longer need to be in pain and can be healed in Heaven forever. Corey is doing good and for this I am so grateful. He went down to Gillette's Hospital for a wheelchair eval. Please pray that the insurance company will approve his new wheelchair and that we are able to obtain it quickly. Our local school district failed to pass it's referendum which means massive cuts in the budget. Sadly today, I have learned that Jenne our beloved friend and Corey's nurse, will probably no longer have a job with Corey. Please pray that something will change as I know this will negatively impact Corey's life. If I had extra money I'd pay her myself but I don't. Also how do you pay someone for unconditionally loving and caring for your child as if he were her own? All we have over the past few days is negative news. We could really use something good. UPDATE: 11-5-07 Emmy has another bladder and kidney infection. Following numerous testing on her kidneys and bladders (all of which were bad) we are being sent to another pediatric urologist at a different clinic. The plan as of now is to close off her urethra and to place an umbilicalus catheter. From what I understand they would use her belly button to create a stoma and from there we would cath her. She has a few defects in her urinary tract which is causing her infections. She has constant diarreah and due to her defects it is constantly getting into her urinary tract. They are hoping this will help by closing off her urinary tract. Right now it all sounds very scary so if anyone has any experience with this, please let me know! I am sure it's like every other step of this journey, scary but soon becomes normal for us! Her lungs are back to sounding junky again so we are back on meds for that. It is just a never ending battle around here. Corey is doing good, nothing new to report. He is just busy enjoying all that life has to offer! This week starts another steady stream of appts. We have appts the 6, 7, and 8th. Corey has a wheelchair eval on Tues; Wednesday Emmy has appts with urology and Thursday with the pediatrician. The following week both kids have appts with neurology the 13th, pulmo the 15th and dev peds and physiatrist the 16th. Emmy then has appts the 21st and 26th with urology. We should know on the 26th the entire plan. I am close to being done decorating for Thanksgiving, just a couple more shelves to do. I finally got around to buying the kids winter stuff, jackets, hats, mittens, snowpants and boots. This is the first time I bought their stuff all at the same time. I can't believe how expensive it all was. I also finally made appts for the kids to get Christmas photos done. They were very nice and have made arrangements so Emmy will have extra time. The photographer is going to open up a hour early so we won't be rushed and Emmy and Corey won't have the stress of other people being around. This weekend is opening deer hunting in MN. The deer were in our yard eating at 4 this morning. Everyone has instructions that they better not shoot "my deer" a doe and her twins. I realize that deer hunting is basically a necessity as there are so many and each one that is taken is one less to run in front of my car but I still couldn't hunt myself. Margo UPDATE: 10-29-07 Emmy has had it rough the past few days. On Friday I called our pulmo as she just isn't getting better and asked him about getting in touch with the intensivist for his input. Dr. K called me back. This is the doc that told me when Corey was so sick this summer that only God could heal Corey and prayed with me several times in the picu. We discussed pros/cons of having her admitted but he didn't seem to think admitting her would help. He put her on the plan that he would use had she been admitted. All he said was trust me. It included putting her on frequent mega doses of prednisone, 20x her usual dose, mega doses of antiibiotics, plus an extra "loading" dose of antibiotics. She continued to get sicker over the weekend. He called and said he knew she'd get better but that it was all in God's hands, he was only doing what God told him to use. Overnight Emmy has stabilized, her oxygen level is 93 with only 3 liters. Friday, so you can compare, her oxygen levels were 82 with 10 liters, her heartrate was very slow and she was taking 4 breathes per minute. Today her heartrate is 120 and she is taking 14 unassisted breaths per minute! With all the gunk I was able to suction out this weekend, she no longer sounds like she is drowning in jello. When he called this morning, he told me that before he prescribed treatment for Emmy, that he prayed, asking God for help, cuz he believed we had used all our options and was out of ideas. Now we need to start tapering off the prednisone and going back down to normal theraputic doses of antibiotics. He then informed me this combination is not one that he has used before. Thank God for a doctor that listens to the voice of God even when it goes against standard medical treatment! Corey has a nasty cold and cough (which he so nicely shared with me). But he has been coughing well so we are just doing the standard plan with him. He doesn't seem to be too bothered by it,just sleeping extra and not wanting anything but juice. We didn't do much this weekend. Jenne was over so I took Evan to the Zoo Boo trick or treating. Liam was with a friend so he couldn't go and Corey was sleeping. I had planned on taking all four of the little ones. We don't have much else planned this week, just the usual Halloween activities and a ton of pumpkins to carve. I'm already planning on taking down Halloween stuff the first and getting up Thanksgiving stuff. I'm also planning on starting the outside decorating for Christmas, which I will probably wait until it's below zero and snowing out to do! Margo UPDATE: 10-25-07 Emmy is pretty much the same. She isn't any worse but not any better either. All I can do is hold her and tell her how much we all love her. She continues to fight so hard. I have told her that is okay that mommy will miss her but I will always have her in my heart. She was hanging onto my hand last night, letting me know that she isn't ready to stop this fight. Corey is still having alot of issues with his j-tube. The meds seem to work more effectively but his tummy troubles haaven't let up. We have tried everything we can think of. Spreading meds out more, giving gas drops first. He just cries and grabs and pinches his tummy. The gi is surprised he isn't used to it yet and didn't seem to have any answers. I really don't know what we will do at this point. I was showing the new homecare worker last years Christmas pics. Made me very sad. Last year Emmy could sit with support, tore the flower apart and the ducks she kept on trying to grab. The saddest thing is, this year there will be no smile. Corey was way more active, didn't sleep all the time and he now has a very pronounced tremor, much like someone with Parkinson's disease. But then I realize that I still have them. The photographer is trying to figure out how to take Emmy's pics as she has little head control and will need total support. I hope that you enjoy the song that is playing. I remember way back when I had thought the saddest day was sending Alyssa to kindergarten or having my kids grow up and move away. I think it's the little goodbyes that make us sad is God's way of preparing us for the really big ones. One part of me will be sad but a huge part of me will be rejoicing in the goodbye knowing what the goodbye will mean, no more pain and suffering,the things they can't do on earth will be replaced with everything I had only dreamt of them doing. UPDATE: 10-22-07 Emmy is still hanging on. I watched last night as she choked turned red and then a dusky gray color. I watched her monitor slowly drop, her oxygen level was 7 and her heartrate was zero. All I could think was, my baby is dying and please God I'm not ready for this. I watched as a large chunk of amber colored mucous clogged up the suction machine, she threw up a bunch more mucous and watched as she went from grey back to pink, her oxygen levels went back up and in a few minutes she had one eye open. She was watching me and then growled her little baby kitten growl. Then reality set in, for some reason God sent her back to us. Does she have a story that still needs to be told? Am I to let the world know of God's miracles that don't just appear in the bible but that every day ordinary people are given them? She was clinically dead. So many other people I know have lost their little ones to mucous plugs, why are we so blessed? I promised God that for giving me another day with her, that I will let everyone know that yes, miracles do happen. So the first thing I did was send a telphone recording (our pulse ox and apnea record and can be transmitted via phone to different doctors) of last night's episode to a certain doctor at Mayo. The one that told me she will never believe, that God doesn't have a place in medicine. It was sent with the message if this wasn't God then tell me how you doctors saved her. Yes Emmy is still sick, choking all the time, I think the stuff caught behind the plug is working it's way out of her lungs. I have no doubt that God is healing her of this pneumonia. She sounds awful this morning. But instead of nothing things are begininng to break up. That is a very good thing. Corey has been sleeping nonstop. We noticed a bunch of smaller seizures and weird eye movements. I think he has a large electrical storm going on in his brain. This often proceeds a need for an increase in meds or a grand mal seizure. So we just wait, knowing that the monster will be rearing his ugly head soon. It is finally a sunny day in MN, a shortlived burst of sun as tomorrow it is back to rain and gloom. I am hoping to let some fresh air in. I know all of us are feeling much better seeing the sun is out. To me it means that no matter what life throws at you, the sun will shine again. Margo UPDATE: 10-19-07 I had to take Emmy back to the clinic today. We are changing antibiotics once again as her lungs are worse. Her oxygen sats are low and she is only taking a breath 4-6 times per minute. It is evident to everyone involved with her that her episodes of apnea and the number of times her airway is collapsing is becoming more and more frequent. It doesn't even seem like the bipap is helping anymore. Her pressures are set high and she is still collapsing. We could trach her and have her put on a ventilator but that is only going to prolong her time with us. That would be selfish on our part when we all know in our hearts that her brain is dying, her muscles are dying, her other organs are starting to shut down. For a few mintues last night we saw her pretty blue eyes but other than that she has been sleeping comfortably for the last 48 hours. For that we are grateful. Here is a Liam funny for you, I had him with at the clinic this morning. They were going to hook her up to their pulse ox. Liam rolls his eyes when the nurses were unsure how to use it. He proudly tells them I know how to use all the equipment, you can tell by the sound of the alarm if you gotta stop peeing or if you can finish :) He then proceeded to say how he was the machine carrier, it's his job to help but he won't do it when he's older cuz he's gonna be a wrestler like Hulk Hogan cuz hitting someone with a elbow or chair has to be more fun than being a pack mule! Corey has been asleep for more than 24 hours now. I'm not sure what is going on with him, he looks good and sounds perfect. Then again maybe he's going to slep until the sun starts to shine. It's another foggy, rainy, dreary day in MN. Margo UPDATE: 10-17-07 The little Princess has another bladder and kidney infection as well as the start of another pneumonia. We also have switched back to the other med for thrush. It seems she just never seems to get much of a break between antibiotics. Each time she is cultured it comes back the same old stuff. Nothing much is going on around here, just another fall day that looks like it is going to be rainy, foggy and dreary. Margo UPDATE: 10-16-07 Emmy is sick again. Her lungs do not sound good, her mouth and throat is full of another yeast infection and she is sleeping way too much. This morning she is also running a temp. I knew it was coming back and have been expecting it so I am prepared. Her oxygen levels are staying way too low and they have been into the low 40's overnight. Corey is doing okay. I am in the middle of deciding on some major changes for him and trying to figure out what is best for him. Please pray that I will make the best decisions for him and for our family. It seems too often I try to please others and that usually doesn't work out. I am going to a funeral today for a dear neighbor. It has come as a shock to me. This was someone that was close to my kids and was always willing to lend a helping hand to us. Please pray for their family. I had conferences last night, why does it seem I know exactly what they will say? Especially about Liam, :) he is one child that acts the same no matter where he is! That's it for us, it looks like it will be another dreary, rainy, Fall day around here. One that makes me want to sleep it away. I guess it doesn't help that I have been up for the last 48 hours! Margo UPDATE: 10-15-07 Corey is home and all I can say is what a difference the j-tube has made! He is doing so well and seems so comfortable. Guess my instinct and persistance in getting a j-tube paid off. His seizures seem to be better controlled as well. I think the j-tube is allowing his meds to be fully absorbed instead of just sitting in his tummy. Emmy is her usual self. Her apnea is getting worse so her monitors are going off way more frequently. Last night I stopped keeping track after the 50th time. She is requiring much more oxygen too. In the simplest terms to explain what is happening is this: as her brain atrophies or dies it is forgetting more and more often to tell her to breath. One of these days it will forget all together. I had someone ask me how we deal with this. We live for today and try not to think of what might or might not happen tomorrow. If I am so busy worrying about tomorrow I will have no time to enjoy my children today. None of us know when we will be taken to Heaven. Some think because their children are healthy today they are excluded, the "it won't happen to us". Way to often I hear and see on caringbridge how quickly ones life can be changed forever. I have the advantage I know what is happening to Corey and Emmy and take the time to spend with them today. How many times (myself included!) as parents have we put off our little ones cuz we have so much to do. How many people find out to late that it's the time spent with family and friends that is important, not how many things got done, if the laundry was done, etc. I'm blessed as I will never take tomorrow or next week for granted again. Have a great weekend, ours is going to spent making Halloween sugar cookies and cupcakes! Margo UPDATE: 10-11-07 Corey's surgery went smoothly, He is doing very good. Yanking on his iv and letting everyone know that he isn't pleased with it. When we arrived there was a huge issue on anesthesia and what is exactly "mito protocol" seems the docs had differing opinions on what to use. Finally they decided that maybe mom does know what she is talking about :) For awhile I wasn't sure anything was going to happen. Tonight Corey is back to his usual little self. He did look surprised with his meds, for once it appeared he had almost immediate pain relief. Please pray that this will help him stabilize and find the comfort that we are so desperately seeking. Emmy apparently had a good day as well, nothing out of the ordinary and uneventful. I think our homecare worker is more anxious about dealing with Liam than anything else. Too put it nicely, he has alot of energy, too much sometimes. I have been asked alot recently about getting him tested for adhd but to me he's Liam and a normal little boy. Yeah to some he is a handfull but he does excellent at school and is a very caring little boy. But even I have to admit, he needs alot of physical activity, running, jumping, climbing or else he drives me crazy as he bounces off the walls! Thanks for all the emails, calls and prayers, I don't know what I would do without all the support we receive! Margo UPDATE: 10-10-07 Please keep Corey in your prayers as he will be having surgery tomorrow for a j-tube (type of feeding tube). His preop went okay, he was sounding a little bit gurgly but as I told the ped. that is his normal sleeping sound. He forgets to swallow and it just builds up in the back of his throat. Emmy is back on meds again. I really don't know what to do anymore. As soon as she is off antibiotics it all starts over again. I think she is will on antibiotics, they will do another bronch and biopsy and we will just pray for the best. Her monitors go off alot for low oxygen levels (in the 60's) and for when she forgets to breath, seems like they are much more frequent than before. Other than that she has been fairly good, it has been a pretty normal routine with her. It has been cold outside with a few snow flurries. I am trying to remember what I did with all the hats and mittens from last year but they seemed to have disappeared :) I am hoping that this weekend I will get all the kids winter jackets. I will update tomorrow evening if I get the chance. Margo UPDATE: 10-08-07 Alyssa finally added new pics to the slide~enjoy! Emmy has spent most of her weekend sleeping. Her oxygen levels have been low, in the 70's most of the weekend. We have her on 8 liters of 02 but it is only helping a little. Her monitors keep on going off but there is little I can do about it. Corey has had a fairly uneventful weekend. He has been sleeping quite a bit but that seems to be his new normal. He will have one really good day followed by days of sleeping, then the whole thing is repeated. Overall he is doing well. As you might have guessed, Alyssa came to visit today :) Sometimes it seems like I see and hear more from her now than I did when she lived here. Sean was also here so it was nice to have all of us together. Please keep Corey in your prayers. Pray that his surgery for a j tube (feeding tube into the small intestine) will go as smoothly as Emmy's did. Pray that this will help issues with absorbing meds and formula. Our only option is to move to tpn if this doesn't work. Sometimes it is hard to have faith in God. It is easy when things are going smoothly, bills are getting paid, nothing is falling apart in the house or on a vehicle but then we hit some bumps and glitches. I think we are given these times so we appreciate all the good things a whole bunch more. But sometimes I question God on how much more I can handle. Margo UPDATE: 10-5-07 Corey's surgery to place a j-tube will be Wednesday October 10th. There was a huge issue trying to get it done at our usual hospital, Mineapolis Children's. So it will be done with the same docs only at United Hospitals in St.Paul. I'm not sure what the big deal was as they have sedated Corey tons of times but for some reason it is this time. I am nervous about it being at a hospital that I know nothing about, nor have I ever been there but at least it is will familiar medical personnel. Corey has been much more awake yesterday, it is nice to have my lil monkey awake. He has had a good last 24 hours. If he has one good day and spends the next few asleep, well he is enjoying himself the time he is awake and that is what is most important to me. He seems to be particular fond of rolling pumpkins off the deck. The kids painted all sorts of miniature pumpkins and he just thought it was so funny to push them off. It was so nice to see him like that. Emmy has been sleeping alot lately. This morning she woke up with the same old gurgly sounding lungs. We aren't even off the antibiotics yet this time. We are on two different meds for yeast infections and that too isn't getting any better. We are trying to get her to use a switch to activate toys. Not much success yet as she doesn't seem to care much. Hopefully it will soon click with her that she can be i charge of some things and will start to get the hang of it. That's it from here, enjoy your weekend! Margo UPDATE: 10-03-07 We had to take Corey to his dentist today, which is about a 3 hour drive. I was hesitant to take him as he has been sleeping for the last 24 hours. But it is something I had put off as there are always so many more important or so it seems appts to go to. I felt kinda foolish in telling the dentist that he wasn't a high priority on my list! Corey's 6 year molars came in very strange which prompted me into going. Each one was a normal tooth with another single tooth growing in the center. Also some of his permanent teeth are coming in gray. It's the nasty acid reflux that is destroying his teeth. All his permanent will be capped to prevent decay and his molars will have to be removed as the dentist doesn't really know what to make of it. Liam thinks Corey has shark teeth, he'll just keep on growing new ones! Of course this will have to be done in the OR. As I said he has been asleep for more than 24 hours and didn't move when the dentist checked his teeth. I really don't know what is going on but I am expecting some sort of major medical diaster, it happens every time. Yesterday Corey's g-tube fell out. Sadly all his morning meds were still in his tummy, I changed it at 4 pm. Also his pediasure from noon was still pediasure and hasn't broken down at all. I have a call in to the gi doc, I am sure he will need to go to j feedings. This is just one more sign that the hated mito is progressing. As long as we were at the dentist he looked at Emmy's teeth as well. Hers are okay for now. But as he said they haven't been in there long enough for the reflux to damage them. So they are coated in flouride and she was ready to go. We went to Edina, came home, were gone for 7 1/2 hours and neither one woke up. I have had this overwhelming sense that things are changing and not for the better. It's like I keep on peeking around the corner for this monster called mito but knowing full well, no matter how prepared I am, it always sneaks up. I have a feeling I'm not winning the battle but fighting hard but I know I can't win this war no matter how hard I try. We stopped on the way home to buy some pumpkins. I bought alot of smaller ones for the kids to paint. The neighbors dog chewed up some of my halloween lights so now I have to buy more, grrrrrrrr! Margo UPDATE: 10-1-07 Corey is recovering well. With steroids and antibiotics he is well on his way back to normal. Outside of alot of coughing in his sleep, one would have a hard time to see that he was so sick just a couple of days ago. It is wonderful to have the lil monkey back, the one that is constantly checking to make sure things are being just so to keep his sister comfy, the one that is full of mischief. Emmy is still sick, if I had a dollar for each time she choked and had to be suctioned, I could retire to the Carribean. She is still requiring alot of oxygen, suctioning and is frequently forgetting to breathe. Her monitors are going off way more frequently than usual. But on a positive note, she is awake and looking around quite a bit. I managed with Alyssa'a help to decorate most of the outside for Halloween. Of course today it is raining and the wind is blowing like crazy so we have had to chase some of the stuff down. Alyssa came home yesterday. She told me that Emmy scared her. Said she couldn't believe how much weaker she was in a couple of weeks. She is also planning on coming home tomorrow. She wants to be with Emmy. I know she is torn between her need and want for independence but wanting to be with her sister as much as possible. I know the feeling of how does one carry on with a regular life when things are so horribly wrong. Margo UPDATE: 9-29-07 Last night I had a telephone conference with some of the kid's doctors. As one doctor, whom shall remain nameless, put it very bluntly with Emmy, "we could say based on her xrays, labwork and urine, that we are back to square one but frankly even square one would sound good to me right now". The intensivist said it nicer, "it is out of our hands, I know we have discussed this before, so I know you don't mind when I say this, she is in God's hands and only he will decide". That he went on to say that Corey and Emmy are in his families thoughts and prayers. Corey is doing okay, sleeping peacefully, 02 sats are good with very little assistance. He does require suctioning but as soon as that is done, he is breathing well and maintaining his own. He just has a hard time dealing with so many secretions and tires out quickly from coughing so much. I have seen him much sicker than this. But I realize that things can change quickly. Emmy on the other hand is irritable, whimpers non stop and is struggling to maintain her temp, to cough, swallow and breath all at the same time. So she has a hot water bottle by her feet to help keep her warm. Suctioning frequently and on her bipap which is breathing for her at this time. I am picking up a new med today which we are hoping will keep her comfortable but won't slow down her heartrate and breathing too much. That's it from here, I am just planning on hunkering (is that a word?) down for what I imagine will be a very long weekend without homecare. I will update if anything changes. Margo UPDATE: 9-27-07 I wish I had some more good news but I don't. Emmy started running a temp overnight, has low oxygen levels no matter what we do, her legs, feet and hands are ice cold. She has some sort of fungus growing in her lungs as well as yeast. She also out of the blue has a yeast infection on her bottom as well as in her mouth and throat. She is already on meds for this. Sadly, as I expected she needs to be stronger in order to have a bronch so her lungs can be washed out and cultures/biopsy can be done. Corey has caught a cold and just sounds and looks miserable. But he is much stronger than she is and is able to cough, etc. I started getting the same thing yesterday and have lost my voice and feel rotten. Russ, Evan, Mikayla and Liam all woke up with the same thing too. But from what I hear half the kids in school seem to have the same thing. If this is the start of flu and cold season it is going to make for one long winter. The pulmo told me this will be a hard winter for both kids as he has already been seeing so much nasty stuff going around. Please pray for Emmy, we really need a miracle right now so she can have a bronch. Pray for Corey that he is able to fight this on his own without a hospitalization. Pray for the rest of us to get over these colds quickly. I just don't have the time to be sick and it seems like when I do get sick i t takes me forever to get better. I am sure it's because I don't get much sleep and just don't take the time to let myself heal. Thank you for all your thoughts, prayers and emails, they are so appreciated. It helps out tons to know that we aren't in this journey alone. Margo UPDATE: 9-26-07 Emmy had a stable day and night. She was awake for short periods and was able to sit up in her chair for awhile. Corey is still keeping a close eye on things. But the nonstop hovering has stopped. It may sound silly but he is so intuned to her that I often can tell by his reactions how she is doing. Long before any improvements can be seen he seems to know instinctively how she is doing. He was laying by her and when I asked him if she was feeling better he giggled, then proceeded to pull off her blanket and her oxygen. She looked over at him and growled, it was so funny! Corey seems to be letting me know that everything will be okay. Today we will be heading to Minneapolis. I am nervous about taking her but I realize that this needs to be done. Please pray that things will go smoothly. Sometimes I wonder who is more scared, me or the doctor! This is our last hope and option to clear out her lungs. Yes I am beginning to see the start of the rainbow after the storm. Margo Yesterday: Emmy remained stable overnight. I was blessed with a glimpse of her letting me know that she is still here and fighting. I was suctioning her and she grabbed my hand and did her little growling noise to let me know that she wasn't happy with what I was doing. I said to her, that isn't fair to suction the princess is it and she opened one eye and growled some more! Sometimes I think I have a whole lot less faith than she has. We with her doctors have decided to give her a big burst of steroids and to change her antibiotics. Then we will reaccess the situation in a few days. I have emails asking me how I carry on any sort of normalcy. I do it for my children, so little in their lives is normal, I need to retain the little that we have. I have taught them it's ok to play, laugh, sing and cry, if we just sit around waiting for what might happen, we'd never do much. In a blink of an eye anyone of us may be taken to Heaven, our job on earth is to appreciate each moment of life, not to take for granted that it will be there tomorrow. UPDATE: 9-20-07 Emmy is in need of your prayers. This bout of pneumonia is really taking it's toll on her. She is on her bipap machine and isn't attempting to breath, thank God there is a back up rate so it is breathing for her. She also has 8 liters of oxygen being bled into the line. But still her oxygen levels remain in the 80's. I am getting nervous and Russ is getting nervous as well, it is usually him that tells me you know you can handle this. After I get back from the dentist with the kids this morning I will reevaluate her if she is stable or shows improvement we will stay home but if she is any worse we have all ready arranged for her to be airlifted to Minneapolis. Please pray for her and for the rest of us, I am so stressed out and have had no sleep which makes the world already seem a little less friendly. *********************************** We are back from Mayo. Our appt in Minneapolis went okay. Corey and Emmy had to be fitted for new leg braces. The physiatrist seemed kind of shocked at how Emmy was doing and how many skills she has lost. Corey was basically the same as the last time she saw him. We then headed to Rochester. Both kids hate to be out of their normal routine and not at home. Fun time it wasn't. Corey seemed to like the pool for a short time. How does Mayo expect someone to be in two places at the same time? First we had an appt with the genectist. It seems with Corey's previous doc there all his biopsy results and samples dissappeared with the doctor. She wanted to do more testing in search of the neuromuscular disease and didn't seem to pleased when we refused. She even agreed that whatever they have in addition to mito, is terminal, progressive and untreatable. It makes no sense to me to cut into my babies for a name. I have come to a place where I am at peace with this. It is what it is and it can't be changed so we will continue to treat symptoms as they appear. Then it was on to the lab for bloodwork and urine samples, which was set for the same time as Corey's electocardiogram. Corey cried the entire time, I felt so bad. Then we had the eye exams. Which was a complete waste of time, energy and money. Emmy has an astigmatism and is very near sighted. As for her vision he has no answer as to what she is seeing. He said I think she is still seeing but she is unable to focus or track due to either her brain atrophy or lack of muscle control. But there is really no way of knowing for sure. Then when I asked about glasses for her lazy eye and if we need to do something about the astigmatism all he said was does it really matter as she doesn't use her vision anyhow. All I want is to know that we are doing the best for her and yes it does matter to me. Then it was Corey's turn, he barely examined him and said, his vision is just like Emmy's, there is nothing you need to do. So little info was gained so I am making them an appt at the University of MN eye clinic. Then Emmy had her abdominal ultrasound. It was done because of her reoccuring infections and her liver is severely enlarged. Left kidney was okay, right kidney has a definite defect, is banded into a few seperate parts and refluxes, liver is enlarged with an abnormal second lobe and something is wrong with the ducts. Also from her bladder down to the urethra has defects. Also her spleen is located in the right spot and is very small. I haven't heard yet but they let us leave so I am assuming it is nothing that can or needs to be fixed. This morning Emmy choked and vomited bile, of course some got into her lungs. So she is back on antibiotics and steroids :( She is very gurgly sounding and has a temp this afternoon. I still don't know what caused it and why she would vomit so much bile, it was a huge amount. She seems very tired and has very low tone today. She has had alot of small seizures too so I don't know if that caused it. Tomorrow I get to take some of the kids back to the dentist and some molded for braces. Then we get a break until next Wednesday whent he appts start all over again! Please keep Mikayla in your prayers, her eys have been bothering her alot even with new glasses. She has been getting alot of headaches, etc. It seems like a long time until November when I am taking her to a different eye doc just for a second opinion. Have a terrific week! Margo UPDATE: 9-17-07 If one more things go wrong with my house all of you will hear me scream! On top of our stove breaking I had the not so lovely pleasure of finding a basement Friday morning filled with water. Yuck our sewer backed up. Luckily I was doing laundry so that was what it was. The pump was not working. So after having a septic man come out on an emergency basis that got fixed. So I had the joy of cleaning the basement with bleach. I did catch it before it reached the drywall. Tomorrow we leave bright and early for Minneapolis. Following our appts there we will be heading to Mayo. I am very interested in finding out what is happening with Emmy's vision. The doc at the University of Minnesota doesn't know. She appears to look around but can't track or she can't focus on a toy etc. I think maybe she is just turning her head towards the sunlight or following sounds. Her eyes also seem to go in all sorts of weird directions, never seeming to work together. I am not looking forward to any of these appts for then tend to point out the things wrong never focusing on the things that they can do. Doing all these questionaires made me sad, the only things I got to check yes to is the symptoms she has. There wasn't one thing on their developmental test that I could say yes to. In order to check yes she has to do something on a regular and frewuent basis. So I guess the one time she managed to roll over doesn't count. It also hurts not being able to check the yes for smiles and responds to her name. Corey is doing pretty good and is getting over his pneumonia nicely. He has had a lot of pain over the weekend but utilizing everything we are able for the most part kept him comfortable for the most part. I will update later in the week, sooner if anything major happens. Margo UPDATE: 9-12-07 Our clinic called yesterday. Apparently Minneapolis Children's called them to let them know that Emmy's urine was growing 3 different bacteria so we needed a different antibiotic. One of them I have never heard of. After getting the full er report our neuro upped Emmy's valium again and added another seizure med. She just isn't getting better so I have begun to accept that the nonstop suctioning, the 24/7 oxygen, the constant infections are just our new norm. I also discussed with our ped the possibility of keeping her cathed. We will be seeing a urologist to get his opinion as no one really knows what to do at this point. Just one more doc to add to the need to see more frequently list. Corey seems to be feeling a little better, I think the antibiotics are working. Next week we will be heading to Minneapolis early on Monday. Both kids have a couple of appts and I am trying to get the urology appt added as well. Then after we are done there we head to Mayo. Both kids have quite a few appts there but I am still hoping to head home by the 19th. You just never know when you are there, sometimes a few days can turn into several weeks as they keep on adding more appts. I wouldn't be surprised at all if we ended up staying the week. There really isn't much to say, life just keeps on moving forward. Margo UPDATE: 9-10-07 We made it to Minneapolis yesterday for the HopeKids Festival with the MN Gophers. It was nice to see everyone, there were alot of people that we know thru Caringbridge there or ones with little ones that have mito. Corey slept thruout and seemed to get sicker thruout the day. We ended up leaving early and heading straight to Minneapolis Children's ER. We were brought back immediately. Very surprising as we have had to wait for hours on some occasions. I guess it has helped that they have their charts flagged. Both Emmy and Corey were seen. Emmy's aspiration pneumonia isn't better so a bronch will be needed to wash out her lungs with another biopsy to figure out what is growing in their this time. I have a feeling it is the same old fungus back again. They also cultured her urine which looked once again like yellow cottage cheese. Corey has another pneumonia, once again it's caused by aspiration. It is in it's early stages so we should be able to treat him at home. So after a few prescriptions we were ready to head home. Thanks to everyone that helped out yesterday, I had someone drive my kids and car back home and thanks to Jenne and Mike for driving me to the hospital and hanging out in the ER with us. If I can figure out how to post pics I will. Emmy and Corey looked very cute in their MN Gophers outfits. Emmy had her pics with the cheerleaders (she was in her very own Gopher cheerleader outfit) and both kids had pics done with Goldy the mascot. Thats about it from here, I have to run as both kids need alot of extra nebs per day! Margo UPDATE: 9-3-07 Our family is need of your prayers now more than ever. Emmy is not doing well, she choked again this evening and her lungs after suctioning sound worse than ever. I am having a hard time keeping her oxygen level in the high 80's with 8 liters of oxygen. I have increased nebs but nothing seems to be working. She was also bleeding for what seems to be no reason from her j-tube site. I changed the tube but could see no reason for it. The only thing we can do is keep a close eye on her diapers. My mom had another small stroke overnight and was transferred to a larger hospital this afternoon. She is diabetic and her blood sugars are extremely low. She will be undergoing some more testing tomorrow Once again I am torn between wanting and needing to be in two places. I know my other siblings and family is with my mom but I still want to be with her. But I realize that Emmy is too unstable for me to leave. So I sit and hold my princess and we pray. I know that they are both in God's hands but it isn't making this an easier. I am not finding any comfort in this today. I am a nervous, emotional and spiritual wreck. The other kids are really picking up on it and in turn are very upset and worried. Corey had a not very good day and it is just one more thing for me to worry about. Top this off with all the other things going on and it is just making a stressful situation that much more so. Sorry for such a depressing sounding update but I really didn't have one thing to smile about. I guess I'll have to go check out my favorite lil cowboys from Texas, as they never fail to brighten my day! Margo UPDATE: 8-30-07 Russ is home from the hospital and seems to be doing well. A little bit wiser perhaps in the knowledge that even he isn't invincible and that things can change your life in a heart beat. Emmy has another aspiration pneumonia. We have another series of xrays today. The pulmo wants to compare xrays from the 16th with the new ones. She will be on cipro for 14 days followed by 10 days of zythromax along with the usual steroid burst. We are also in the process of adding and increasing other meds. Her irritability just is getting worse each day. Corey is doing good, still is spending most of his day sleeping but really there is nothing new to report with him. I have to take the 4 oldest kids to the dentist today. Then at noon take Emmy for her xrays. Then this afternoon is Liam, Evan's and Corey's open house at their schools. Lexie and Mikayla had theirs last night. That's it from here, thank you for all your emails, thoughts and prayers. They are very much appreciated! Margo UPDATE: 8-24-07 Emmy started on Doxepin. It is suppose to help her sleep. LOL NOT! The sleep doc said make sure youhave her all hooked up to monitors bipap etc as this will snow her big time and will keep her asleep for 8-10 hours, do not use chloral with it and only give the valium after she has the Doxepin on board for at least one hour. 2 hours later and it hasn't phased her, she is still wide eyed and most definetely awake. Last night she never did go to sleep :( When the sleep doc called on Tuesday he said he brought Emmy up at a convention of sleep specialists, not one of them wanted her as a patient and all wished him luck. He was hoping to have us meet with a sleep specialist from John Hopkins. He told me Emmy doesn'thave sleep issues per say, she has an irritable central nervous system that doesn't allow her brain to trigger the need for sleep. He wants her on another antidepressant med hopefully one that will calm her cns enough to sleep. Only thing is he needs FDA approval to give it to anyone under 18 and no one really knows the correct dosing. Like that really makes me comfortable in giving it to her! Emmy has had a hard time with secretions today, choking, gagging etc so we are upping her robinol again. We are also increasing the seizure meds she is getting. Corey has been acting very strange today, lots of pain, eyes making weird movements, etc. Both Jenne and I are thinking he is on the verge of a grand mal seizure or he is going to have another stroke. Just what the neuro wanted to hear! So we are increasing his phenobarbitol and we also gave him a burst of it to boost his levels. The hope is to stop anything that is going on in his little brain. Have a great weekend and I hope that you will stop and take some time with your family to make a few memories. Margo UPDATE: 8-23-07 Emmy has started on her new seizure meds. We are just started in the process of increasing the amounts so I haven't noticed too much of a decrease in seizures yet.Both of these meds take a week to start working well, as you slowly increase the amount given. We are on the beginning of day 6. She has begun to sound gurgly overnight again, I am sure she aspirated on saliva and we will be starting antibiotics for yet another pneumonia. Last week when we were at the neuro's we discussed her sleep issues, or the fact that she just doesn't sleep. This has been documented numerous times during her sleep studies and hospitalizations. She never appears to go into the rem sleep phase either. He wanted the pulmo to try another med. We saw the pulmo, he agreed something needed to be done but wanted the neuro to prescribe it. Ha now they both passed it off to the sleep doc. He called Friday. Said he talked to both other docs and he doesn't know why he was given this to do as he has only seen Emmy a few times. Told me you have a child with mito, severe central and obstructional apnea, frequent pneumonias and has the highest bipap pressures I have ever put a child on. I am afraid to put her on any meds, frankly your child scares me. To put it bluntly, it is better to have a child that never sleeps than one that doesn't breath anymore! Suggested I get 24 hour homecare and deal with it. He seemed shocked when I said I don't/can't get 24/7 care, so I asked him if he would care to live at my house for awhile as I only have so many hours of homecare per week. All he said was if I had to deal with your children not to mention your healthy kids thrown into the mix well let's just say you handle it a whole lot better than I could imagine. So back to square one. When Emmy is awake, she just doesn't lay there, she wiggles which causes her monitors to go off, you can't turn them off and go to sleep as she stops breathing without any noticeable signs other than she turns blue and the monitors alert you. I have kept track I average around 2 hours of sleep per night usually in 15-20 minute intervals and here I wonder why I look like I have aged a hundred years in 6 years. During the day I am too busy to sleep and if I do I feel worse than if I don't sleep. There isn't much to report on Corey, he is doing the same which is great news! Liam turned 10 yesterday, we just had a cake and presents. He is waiting until school starts to have a party with his buddies. Parents are so busy trying to get last minute stuff done before school starts that it is hard to plan a party. We have alot more appts before school starts. I still need to get supplies and Corey clothes. There just doesn't seem to be enough hours in a day! Alyssa is moving this weekend. So I won't have her to rely on anymore when one or the other is in the hospital. She is excited to be getting her own apt and probably away from me! I think I vaguely remember that age, when it seemed anything was possible but then again I already had her at that age so I wasn't quite as carefree. Mikayla's eye appt did not go well at all. She has some serious issues that will need further investigation. I'm not going into details right now, I want to know for sure what is wrong. She will be seen at Mayo as soon as possible. Margo UPDATE: 8-17-07 We are home from Emmy's appt. It was not a good appt and once again I am faced with the reality of this awful disease. Her chest xray was awful. 10 days into a 14 day course of the strongest antibiotics for an aspiration pneumonia and her lungs are still coarse and raspy. Our pulmo immediately noticed Emmy's seizures and her inability to swallow her saliva. She lets the saliva buildup in her throat and then makes kind of a slurping noise trying to swallow but usually ends up choking instead. She needs another bronch but the pulmo told me until her seizures are better under control, she is stronger and has no other infections he won't/can't do one. We also discussed the upcoming winter months and how hard they will be for her. He told me they are already starting to see rsv, a lot of strep etc and that when this happens so early it will be a tough winter. She will be getting her flu shot and also starting the series of shots for rsv. He also started her on a higher dose of robinol to help with secretions. Our neuro decided to start Emmy on new seizure meds. Of course one of them needs to have a prior authorization. Our pharmacy doesn't even carry it and then told me if the insurance won't cover it the cost will be close to $800 per month. Please pray that the insurance covers it, we have had things covered by them lately that I never thought they would, so hopefully this will be the same in this case. Now all I need to do is figure out how to fit 3 extra meds into her schedule and go back to 12 nebs per day for Emmy. I had taken Liam with me yesterday to sit beside Emmy in the car. He was so excited to get to go. When we were in the waiting room, he was holding Emmy while I was filling out the paperwork. A lady came up to him and told him his sister was so beautiful and that she looks like an angel with her curls. His response? Well of course she is. she has mito and will soon go to Heaven. The lady looked shocked but Liam proceeded to tell her all about mito, all about Corey and how when Corey and Emmy get to Heaven they will have many friends. Then he went on to tell her about Eli,Caleb, Bracken, Mearan and Jacob. By this time the lady was in tears and he said don't cry don't you know when she gets to Heaven she will have friends and just be a regular kid and won't need her machines no more. She told him thank you for showing her how lucky she is, she thought having her little boy getting diagnosed with allergies and asthma was the worse thing in the world. She then told him what a great brother he was. Laim said I know I am and I don't even get paid to hold her! Corey spent yesterday and overnight with his beloved Jenne. It is always such a relief to me to know that he is always well taken care of when I can't be with him. Today I have to take Mikayla to her eye exam. She is hoping that the eye doc says she can have contacts. The last few times she said no that contacts aren't an option. I am hoping to just have a quiet weekend before the next series of appts start next week. School starts in just a couple of weeks, summer has just flown by. The start of school always makes me a little sad, I like to have all the kids home with me. However I don't think I will miss the nonstop bickering and fighting! Enjoy your weekend! Margo UPDATE: 8-12-07 Our beautiful princess is 19 months old today! It seems like such a short time ago that she was a newborn. We were looking at pics today of her past 19 months today. Liam was looking and said to me, do you remember when Emmy used to smile? How come she doesn't do that anymore. It's sad but the last time I remembering a true smile from her was shortly before Thanksgiving. To see her pics and to see her now, the sparkle has left her eyes, she no longer scrunches up her face to smile each when she sees us. I am getting closer to finishing up her room. Soon it will be all Strawberry Shortcake. I am just waiting to get some large wall stickers that we ordered from Ebay. Her bedset came the other day and it is so cute! Tomorrow we leave at 5 am for our appt in Minneapolis with the neuro. One kid or another seems to have appts all week, no break whatsoever. We are also trying to figure out how to remodel our house. Sometimes it seems like it would be easier just to start over. We are intending on removing all carpets and going to all hardwood floors. Need to make the bathroom more accessible and bedrooms larger for medical equipment. It seems most days whichever way I turn I bump, crash or knock over something. Emmy is still in her crib but a hospital bed would be way more convenient to care for her but there is no way I can fit one in. Corey is doing about the same, really there isn't much to say about him. He sleeps for most of the day but has a couple of hrs each day where he is up and playing. I think he is getting a little stronger each day. Okay so maybe it is wishful thinking on my part as no one else seems to see it! Take care and have a wonderful week! Margo UPDATE: 8-9-07 Things continue to go downhill regarding Emmy. It was determined this afternoon that she has another aspiration pneumonia, in both lungs again. It is in the same spots as the one she had a couple of weeks ago. She just was done with the antibiotics for it on Saturday. Of course the one she is on for strep won't cover it so she is on two antibiotics now. I was afraid this would happen. Strep caused an increase in secretions, she doesn't handle them well, top it off with sore, swollen tonsils, well I shouldn't be surprised. No matter how often you suction, it is never enough. If you suction too often her membranes get irritated and often bleed which leads to another set of problems. So we added nystatin both oral and ointment to hopefully avoid the never ending yeast infections and she is back on the prednisone as well. Corey is getting over his strep now that he is on the right antibiotics. He is doing pretty well and has been awake for a couple of hours today. I think he felt he needed to be awake to keep his watch over Emmy, he needs to be right there to make sure I am doing things right! If I move the oxygen for instance he gets very agitated and pinches me until it's back in place, hands me the lotion, etc. It is funny how well he knows and understands exactly what should be happening! On Monday Corey and Emmy have appts with the pain/care team as well as the neurologist. We need to tweek a few meds and come up (hopefully!!!!) with a better pain control plan. Then Thursday Emmy has her appt with the pulmo and ent. I don't know yet if we will be coming home from that one or if she will be admitted. It all depends on what her xray shows. I am sure a huge topic will once again be a trach. Even thou her docs admit that nothing will stop her from aspirating saliva unless we tie everything off down and tie everything off from her stomach up. Even then I was told it would buy us a short amount of time. It comes to a point where as a parent you need to decide if you are keeping your child alive for their quality of life or for yourself. I have always promised them I would never do that to them, I will treat them the way I would want to be treated if the roles were reversed. So a bronch may be done, her lungs may be washed out, antibiotics will never be withheld from either of them, pain meds will be insisted upon, a feeding tube will be used until we know they can no longer tolerate food-their little bodies will know and tell us but no more genetic testing, etc. Yes we have found our peace. We will let God decide how to heal our children, it is up to him to decide if it will be an earthly or heavenly healing. We have all our doctors support as we move forward. We will be the ones deciding when our children are hospitalized. Now for some good news, Emmy will be 19 months old on Sunday :) Liam will be 10, yes 10! On August 21st! Also today I interviewed a homecare nurse, one that I liked immediately! One that I feel will fit right in, and she didn't run screaming for her car! She is young, has no kids of her own and is very flexible on hours she can work!Please pray that all goes well and that she agrees to accept the position! Margo UPDATE: 8-7-07 TUESDAY AM----********************** Emmy woke up this morning with a high temp and a very yucky looking throat. She was diagnosed with strep this am :( we are quite sure this is what triggered her seizures last night, the stress of another infection. Her seizures are back to normal this morning. I really don't know how close to her baseline she is as she is very sleepy and low tone. I really don't know what to say in regards to how she is doing I really don't know. I have been in contact with her various docs this am and all we hear is we'll just have to wait and see if she is strong enough to fight off another infection. The bipap will be breathing for her, it will help to take some of the stress off her little body. Please pray for her and for us as God's plan for her begins to unfold. ************************************ MONDAY 5:56 PM--UPDATE!!!!! Emmy is need in every prayer possible that can be sent her way. She has suffered numerous grand mal seizures in the past 1/2 hour. I had her neuro paged, he called back within 2 minutes. We are currently on high doses of diastat and ativan. She has lost all of use of her right side. Please pray that they are soon controlled. I am sure she has suffered a stroke. Her poor little is all purple where she bit it. Pray that once again we will be blessed with a miracle from God. ****************************** Corey isn't recovering from his strep throat like he should. Tomorrow they will reculture it and change to an antibiotic that is more agressive. Emmy woke up this morning with a high temp; very congested and you guessed it-strep throat. Along with this comes an increase in secretions so she aspirated again :( She was off the clindamycin yesterday. So she is being switched to a high dose of cipro which hopefully will take care of both. She looks so sick and is continuing to lose weight. We are also have increased her oxygen to 10 liters. I have been in touch with her care team in Minneapolis. It makes me wonder how much fight she has in her, she has no reserves left. The closer we get to school starting the closer we are to fall. I just wish time could stand still. As most mito prents can tell you our kids usually do better during the summer. I always said Corey built up his reserves during the summer as fall and winter hit these kids hard. This summer Corey and Emmy have had nonstop infections, my fear is that they won't have their strength back for what is the hardest time of year. I usually breath a huge sigh of relief when spring arrives, but not this year things continued to get worse. We have not decided for sure what we will be doing this fall for Corey, if he should attend school or not. The closer it is, the further he seems to be away from our goals of him getting stronger. I have had tons of questions regarding him. Here it is: Corey sleeps 21-22 hrs per day. He is usually awake in the morning for an hour or less. He gets his bath and will maybe play for a short time before he is asleep again. He sleeps until late afternoon/early evening. He might be up for 1-2 hours and then he is usually asleep again by 7. So you might think he would be easy to care for. During this time he has meds every 3 hrs, needs repostioning, his exercises done, oral cares, feedings, nebs, pulmo vest, suctioning, diaper changes/cathing. We also sing, talk to him or we listen to music. During his awake times, on occasion he will play but usually wants to be held while he stares and is usually impossible to get him to interact with us. His seizures are also increasing. He has full body tremors that never seem to go away, reminds me of a person with Parkinson's, his muscles are constantly twitching, changing between being very low tone to him being spastic. I no longer dream of the promise of tomorrow, we only live for today. Trying to make each moment of their lives the best it can be. I redid Corey's room in Thomas the Train. I am now in the process of redoing Emmy's. I am changing it from the lil princess to a more toddler like Strawberry Shortcake theme. Okay so I know she doesn't care but I do! The kids and I decided that is what she should have over winter. I am trying to brighten her room up as her vision decreases. The princess theme is pale pink and the new one will be hot pink. I think it was Liam that wants it to be strawberry shortcake the most. He chose that cuz I wouldn't let him choose Transformers for her! I am also doing Liam and Evan's room over. They are getting new beds and a new theme as well. They just need to decide what it will be. I have a couple of interviews the next couple of days for a homecare nurse. Please pray that I find just the right person! Margo UPDATE: 7-30-07 Emma continues to struggle as she fights off this lastest pneumonia. The antibiotics are seeming to work well. The only problem with clindamycin is the nasty thrush in the mouth, yeast on her bottom and around her tubes that she gets. We started her off immediately with nystatin, lactobacillus and ointment for her bottom and tubes. It seems to be working. I spoke with the pallative care/Karuna care team on Friday. Also with her pulmo. It has been set up so I can have access 24/7 to antibiotics without bringing her in as well as any iv meds that she may need. It is nice to have this support in keeping her home and any time I feel she is too ill she will be transported to Minneapolis. As much as I fought the idea of hospice care it really has brought me a great deal of comfort. We will be heading with her to see the pulmo on August 16th, if her lungs are clear great if not they will go ahead and wash them out again. Emmy has been sleeping most of the time and continues to keep her oxygen levels up. Her bipap has a backup rate so when she fails to breath it will breathe for her. She doesn't even attempt to breathe over it. Without it on she breathes 3-4 times per minute and retracts with it on no retraction and she looks so peaceful. Once again I am so thankful that we are lucky enough to have this machine. When I think of all the little ones that do not have the priveledge of having one it breaks my heart. Here we are lucky enough to have one plus a backup one. How lucky we are to have the quality of care that we do! Corey is doing okay, still sleeps most of the day but when he is awake is having really good hours. It might be only a couple of hours per day but he is back to playing and walking some. The only things he isn't back to baseline are eating, drinking, urinating and his thought process is still slow. He also tires very quickly. Jenne stayed overnight on Thursday and she brought a very small boxfan. Corey immediately claimed it for himself and takes it everywhere with him. Not to worry it is plastic and has no room for him to stick his fingers in. He sleeps with it and gets very upset if you remove it. I can't imagine snuggling with a fan can be comfortable but to him it is. Jenne has been here each day with Corey, Emmy and myself. I have not been able to find a new worker so it has been a Godsend to have her help. She also dropped everything to go to the ER with Emmy and I. I don't know what I will do when she decides to go back to the school in fall! Please pray for Emmy's pneumonia to clear up, Corey to continue to gain strength and for just the right homecare worker to arrive in our lives. Margo UPDATE: 7-27-07 Emmy is need of your prayers. We spent most of the night in our local ER, she is sick once again with a bilateral aspiration pneumonia. We caught it very early. They wanted to admit her but we decided to take her home. If she doesn't seem to be improving over the next 24 hrs then she will be airlifted to Minneapolis. Our local er does not like to treat Corey and Emmy nor do they have the equipment that she needs. I think the doc was very happy to have us leave. WHen he called the ped on call, his only advice was ask mom what to do. The ER was unusually busy and I thought we would be waiting forever, they just bumped us up in front of everyone so we were through in a few hours. So we have her antibiotics, bursted her steroids, increased her nebs, etc. Now we wait to see if she fights this on her own or if it's back to Minneapolis for another 2 weeks. I am planning on calling both our regular ped and the pulmonologist today. It was just a couple of days over a month since her last pneumonia and bronch. The pulmo was hoping for 3-4 months of no pneumonias to give her lungs a chance to heal. I was hoping that with the bronch and lung wash she wouldn't get so many, hoping that the pulmo was right and that the reason she got so many was the fungus that was growing in her lungs was resistant to antibiotics. Please pray for her as she is really struggling to breath, pray that the antibiotics work quickly. I just don't know with Corey needing me every minute how I could handle being away from him. Margo UPDATE: 7-23-07 Corey had his eval last week with the school district. He will be receiving therapy at home a couple times a week. The plan now is for him to return to school this fall but if not he will then be put on homebound education. Overall he is doing well, still sleeping most of the day but when he is awake he is doing great and getting stronger each day! Emmy had her eval on Friday. They told me they couldn't "score" her on the testing they do because of her motor involvement. It wouldn't be fair to her. I don't really know if it's because she has so few purposeful movements or what. Those tests always make me feel bad as they stress the negatives and never the positives so I could care less. We all know that she is severely delayed and outside of that I do not need to know anything else. It is kind of like mri's, I don't want to see them, it's a slap in the face each time. Yes I know there is atrophy and it's progressing but it's far worse seeing it in black and white. To me the kids are just Corey and just Emmy, that is who they are and what they can do. It's far worse not when you see the curious child, but the adult that stares and whispers. Just ask me, I won't be offended but treat my child like less than a human being and I take offense. I see children that accept them for who they are, now if only their parents were that accepting. We have appts 4 days this week, either people coming to the house or we having to leave. After months of waiting Emmy will finally be getting her iv pole and oxygen tank holder for her wheelchair! I had given up hope and have been carrying all this stuff with me. I think the clincher was when I sent a picture to the insurance company and asked if they could provide me with a packmule for doctors appts :) I even offered for one of their decision makers to follow me to appts and I would even let them carry a pulse ox, apnea monitor, suction machine, oxygen tank and refills, feeding pump, nebulizer, pulmonary vest, bipap, cough assist plus all the other supplies of diapers, syringes, meds, formula, extra clothes, etc. I think they got the point. No offers on the coming along to help carry part. I have been sick all weekend with a stomach virus, Russ now has it, and Liam is starting to complain of a tummyache as well. I suppose we will all get it sometime over this next week. At least I am starting to feel a little bit better, I have a feeling I need to feel better rather I do or not! As many of you can relate moms are never allowed just to be sick but have a sick husband on your hands... Let's just say I'd rather have a household of sick kids . UPDATE: 7-18-07 Just a quick update. Things are about the same around here. Corey was fitted for his new leg braces on Friday. They are AFO's and have Thomas the Train on them. They seem to fit him well but once again it is the sensory issues that causing him not wanting them on. The plan right now is for him to where them while he is asleep and hopefully he will let them on while awake. Yesterday I had a conference call between the pain specialist and neuro and the Karuna care team was involved. We decided to up his pain meds, increase frequency and to up his valium. Emmy's pain meds were upped as well and her valium was doubled. We also discussed how to try to keep them pain free as possible while trying for some quality of life. It's hard as there is this fine line between keeping them painfree but still managing to have them awake. I am finding this may be impossible. We also decided where to move to next in regards to pain. Together we decided our next step will be a methadone/oxycontin combination. We can't use morphine due to the kids allergic reactions, codeine isn't working as they don't have the ability to break it down. We discussed Dilaudid and toradol combination but for some reason because of what they are on now it won't work and it is still a synthetic morphine. We are giving the higher doses another week and then we are going on to the next step. As difficult as these final steps are to discuss it isn't nearly as hard as watching them in pain. Both Corey and Emmy have another cough, I'm praying that it is only a cold and won't become a pneumonia as neither one is very strong. Our therapist from school are coming out today and on Friday for reassessments. Corey has another new machine so those people are coming out to teach me how to use it on Thursday. Wednesday we have appts. From now until fall we seem to have a few appts each week. Emmy is doing well, nothing much has changed with her except pain issues. She is back to crying all the time and pulling her hair, biting herself and not wanting to be touched. I am hoping the increase in meds will give her some comfort! UPDATE: 7-16-07 Sorry I haven't updated lately but I just don't seem to have the energy to do so. I have been asked repeatly over the past week or so how is Corey doing? That is a difficult question to answer. He is doing okay, not really any changes to mention. He spends a good portion of the day asleep, we see glimpses of the little boy he was sometimes. It is hard to explain, I try to stay optimistic but I see the little differences, how he is slowly declining. The little boy that tries so hard, fights so much but I know deep down that he is losing this fight. I feel it within my very soul. I am afraid to sleep, just waiting for this monster to spring forth from where we have pushed him. Emmy is about the same, sometimes I think it is easier for me with her. She really doesn't have many skills so there is less for her to lose. But yet I watch her struggle, she tries so hard to reach a toy but can't, if you put it in her hand it doesn't help, she will drop it by the time she gets it to where she wants it to be. Watching her sleep doesn't bring me joy. I remember watching my other kids sleep and how peaceful they look and how innocent and sweet. To watch her is to watch a battle, each breathe she takes is a struggle, she gurgles, squeaks when her airway is collapsing then the bipap takes over. I hear the noises of medical equipment, the turning of her feeding pump, the swoosh of the bipap, the oxygen concentrator, the monitors, the noises that the music I play for her can't hide it. Her bed that is all so girly is hidden for all the medical equipment that has taken over the room. Tomorrow starts what seems to be neverending appts. Corey gets his new braces. We will be seeing the neuro, pulmo, gi, eye doc, immunologist, pain doc, genectists, physiatrist, developmental peds, ent. Plus Emmy needs new leg braces, Corey will be getting a seating eval, equipment eval and one for a new wheelchair. Plus both kids will be having pt and ot evals at Gillettes as well as being reassessed by our school district. Plus I have dental appts for all my kids. This with the worry of finding homecare has left me exhausted. It is times like this I seriously consider moving to the Minneapolis area, both for easier healthcare access and homecare agencies that deal with children instead of me hearing we have no one qualified to care for your children. Please pray that I find someone soon or else I'll just have to find a way to convince Jenne and her family that they really do want to live with me! :) Margo UPDATE: 7-4-07 Corey is pretty much the same. He still isn't eating or drinking, his gross and fine motor skills have not come back, he spends most of the day asleep but on the rare occasion that he is awake we have seen glimpses of his personality. His teasing, flirty little self once in awhile shines thru! I feel that overall he is making very slow progress. Will he ever be himself again, I really doubt if we will ever get him back to his baseline. His appts went well, the one with the pediatrician was just to check on his lungs, which are clear! It was really just so she could see and document how he is doing. He also had an appt with his physiatrist and neurodevelpomental ped. They were saddened to see the changes and agreed that the only thing left is to try to make Corey's life the best it can be and to make him as pain free as possible. He was fitted for new afo's, and he will be getting a seating unit for at home as well as another new wheelchair since his needs have changed so much plus he has grown alot taller. He will also be getting a pulmonary vest, therapy (ot and pt)at home. This will be extra therapy outside of what the school district provides. Emmy is doing well. Her new bipap machines came yesterday. She will now be on one that breathes for her. Often times she is only breathing 2-3 times per minute. She also doesn't appear to be strong enough most of the time to trigger the machine for the next breathe. Her central apnea (in which her brain forgets to tell her to breath) has increased significantly the last few weeks. Her appt with the ped went well also, she received her shots but didn't even recognize that she had gotten them:( Just more proof how bad her neuropathy in her legs has gotten. She had that test in the hospital and it showed very little nerve response in her hips to her toes. Emmy will be 18 months old on July 12th. I can't believe how fast the time is going. She will be at the age where most kiddos with this form of mito have died. So we are incredible blessed to have her with us. Mikayla turned 14 on June 30. We just had a small family celebration but I did manage to make her a cake as she requested and not as she says cheat by having Alyssa bring home one from Walmart. I also decorated the outside of the house for the 4th, red, white and blue lights, flags and other outdoor decorations. On Sunday I finally managed to get the inside decorated as well. We are also making our usual flag sugar cookies today and dessert. I am so far behind in this stuff! We are going to a parade tonight, tomorrow and to the fireworks tomorrow evening. We will be having our usual barbecue and fireworks at home as well. People questions why I am doing it this year, simple I do it cuz it's tradition. Normal, things that are traditions are important to me, so much is taken away from us. I don't remember as a child what gifts I received what I remember most is the traditions, what happened for each holiday. When Liam and Evan were doing Mothers Day cards at school where they fill in the blanks, they didn't write my best memory is when I got this toy or that but it was, making cookies all together, decorating for each season and holiday, always getting a story read. Memories to me that is what is important. Please remember to pray and support our troops. We will once again be sending a care package to a soldier. We don't send it to anyone in particular but rather to a US soldier to let them know that someone is thinking and praying for them. Take care and have a wonderful and safe 4th of July! UPDATE: 6-29-07 Wahoo! We are home!!! It was determined that keeping Corey in the hospital was of no benefit to himself. I thank God that he was able to leave with Emmy and myself. So many times over the last couple of weeks I thought I'd be returning home with out him in my arms. Corey is no longer the vibrant little boy he once was. He can no longer swallow, needs cathing (but it is getting better) and has lost most of his skills. He is showing some signs of improvement and we are praying that with time and therapy most of his skills will return. Jenne will be spending most days with us, helping with Corey and Emmy. Corey's needs have increased alot and I know that I can't do it alone. We are still waiting for the final verdict on a trache or not. There is differing opinions between doctors and between ones that I trust and know they have her best interest at heart. She still aspirates anything that is put into her mouth, including her own saliva but I'm not sure if that will be the deciding factor or not. The past few days have been really good ones for Emmy, I think that the washing out of her lungs has made the difference., for the first time in her life her lungs are clear. She is always watching Corey intently, I know that she realizes that he has changed. Both kids have appts with our pediatrician and Corey has an appt. tomorrow with the physiatrist. I'm trying to think of what else is going on. Emmy's bipap has a new backup rate that will breathe for her since her sleep study on this admit showed severe central apnea as well as obstructional apnea. I would like to thank everyone for their thoughts, prayers, calls and emails. They were deeply appreciated! It is only by God's grace that we are blessed to have Corey in our lives physically. When he was admitted we were told he had less than a 1hance of survival, his intensive care doctor told us they couldn't do anything and that it was in God's hands. Both kids are on several new meds. I am slowly getting used to our new routine. Please pray that I find a new homecare worker soon, it is causing alot of stress. UPDATE: 6-23-07 Emmy's surgery went well. Her lungs were washed out and they did cultures. Her upper airway collapes severely but we won't know until next week if she needs a trache. There was no immediate danger as long as she stays on the bipap so that is great news!!!!!! She will be changing from neocate infant to neocate plus one formula. The pain doc saw her and she is on new pain meds. The pulmo said he believes her aspiration pneumonias are caused by undetectable (to the human eye) seizures. So today she has a 24 hr video eeg hooked up. So she will be getting new seizure meds too. Corey is doing okay. He still can't walk, tremors, leans towards his left side, can't swallow but now recognizes me and kenny chesney music. His processing time is very slow and the mental deficit he has suffered is apparent to everyone even doctors that see him infrequently. He also has to be cathed every few hrs. His pain meds have been increased and he is now on new seizure meds as well. Once again we are so grateful to God for allowing us more time with our precious angels! Corey and Emmy are in the same room. Both have their own team of nurses and rt's but share the same docs. So our room is chaotic but they are together!Things are going well and there is mention of us leaving in a few days. The doctors believe that Corey will stay this way based on tests. They all agree that there is nothing more they can do and this will be our new normal. Emmy will be having a swallow study and a few more tests. Margo UPDATE: 6-19-07 Just when I thought things were improving .... The good news is that Corey is off the ventilator and breathing on his own. He is still on 15 liters of oxygen. Sometime between Monday am and afternoon he had a stroke. His once strongest left side he has lost any use of it. He had an eeg this morning, waiting for an mri to verify if it's stroke like episode or another major stroke. We are caught up in limbo waiting to see if he will recover. He will also be starting on new seizure meds later today. He is also having an xray of his abdomen as he is extremely bloated and it's not air. The gi is thinking he either has a bowel obstruction or impaction. Emmy will still be arriving tomorrow. For those of you that have asked, we live approximately 2 1/2 hrs from the hospital. In speaking with the neuro today he feels that a trach too is our only option at this point. The hospital is helping to ensure that both kids can share a room or depending on their needs at least be on the same floor. The staff is being extremely kind and trying to make it the best it can be considering the circumstances. Please pray for all of us as wait for God's plan to unfold. UPDATE: 6-16-07 Corey is still holding his own, not much improvement but things are no worse. His blood work hasn't improved and the barcarbonate(sp?) in his blood has increased so he is now on a new med for that and he has also started growing yeast in his lungs, mouth,stomach, etc so he is on a med for that. He is still on the vent and willbe thruout the weekend as his xray hasn't improved and he continues to be very bloated even with the extra lasix. They have decreased his feedings and they are now half strength. I think even thou the tests don't seem to show it that he is improving a little. It breaks my heart to see him like this. There are no glimpses of the vibrant little boy that fights so hard each day, he no longer bothers to struggle thru suctioning and will no longer squeeze my hand. Then again maybe he is tired of it only being me there to entertain him! Thank you for the emails and prayers, it helps out so much! UPDATE: 6-15-07 Corey is still in the picu. His condition has been downgraded to very critical/unstable. He is still on the vent and the pressures have been increased. He has gained 4 pounds in the last 36 hours (all fluid) so they have doubled his lasix. His lungs are worse than they were yesterday most likely caused by the fluid build up. His blood has also become very acidic so this too will be treated. His hemoglobin continues to drop as well. Please keep him in your prayers. UPDATE: 6-14-07 Update Thursday AM: Corey is still in the picu and things are pretty much the same. He is still in a coma and is on the ventilator at this time. At this time there is no plan to start weaning him from the vent. His chest xray looks pretty much the same as yesterdays. They are still working on identifying what is growing there. He looks much better this morning, not so gray/blue. He is now responding to my voice and Jenne's. Jenne his school nurse came to visit and whenever we would talk to each other or to him his heartrate would go up and he will now squeeze our hand. It is nice to have him starting to show that he knows that I am there with him. Things at home are going well. Emmy is doing quite well or at least that is what I am told.The plan is for her still to be admitted here next Wednesday. I am hoping that by that time Corey will be out of the picu so they can share a room. It would make things much easier. Thanks for all the emails and messages, it is so much appreciated to know that we aren't on this journey alone. Please continue to pray for all of us! Margo I had to take Corey to the clinic this morning. He was desatting into the 70's and unresponsive. He has another aspiration pneumonia. He is not doing well at this time, please keep him in your prayers. We have decided to keep him home as our ped agreed there is nothing they can do for him in the hospital that we can't do at home. We also discussed how agressive I want to be in treating him as she feels he is rapidly declining. Right now I feel he is still fighting hard so therefore we will be treating him as usual. ************************************* Corey is acting very strange, not exactly sure what is going on with him. He is sleeping around 18-20 hours per day. His seizures are on the increase again. He had a couple of Grand Mal seizures and once again aspirated so he is back on antibiotics. There really isn't thing that the clinic can find medically going on with him but I am still very uneasy. It's like I'm holding my breathe waiting to see what happens. Today he starts his first day of summer school. Jenne will be with him or he wouldn't be going. I am hoping that he will be awake for it. He wil be going twice a week for a few hours each day. Emmy is doing well or well for her. By well I mean tolerating his feedings ok, only a normal amount of seizures, etc. Funny how well used to mean a much different thing to me. She is handling the bipap quite well and likes the nsal pillows much better than the mask. She is still desatting often but overall is breathing with alot less effort. She has an appt on Thursday just in Brainerd. Everything is set for her hospital visit, all paperwork is done, insurance has approved it, etc. Once again she will be admitted on the 20th. Surgery will be the 21st. They will be doing a bronch, lung biopsy, mri and some sort of test on her heart, I can never remember the name of it and I don't have the paperwork in front of me. Following these tests it will then be determined if one a trache is appropriate and two if she is strong enough to handle that surgery. I am getting extremely frustrated. I have had the papers in for a bathtub chair, oxygen holder and iv pole for her wheelchair for months. Found out yesterday the person in charge "forgot" to submit the papers and order them. GRRRRRRRR!!!!! They have a letter from our insurance company saying all necessary medical equipment is automatically approved for one year. Now you just don't get letters like that everyday! I would consider these items to be extremely necessary. I told them yesterday they have 3 days after that I am taking my business elsewhere. I am so tired if it's not a big ticket item it's always a hassle to get it. Emmy is in desperate need of the tubchair. She is too big for her infant tub. She is so floppy it is becoming next to impossible to bathe her and dangerous as well. It is becoming close to impossible to hold her and wash her at the same time. She loves her bath and anyone with a child with low muscle tone knows how well they can move while in water. I have resorted to hopping in with her but then getting out with Miss Floppsy and myself wrapped in a towel is a problem. I am still looking for a fulltime homecare worker! Please pray that we find just the right person! Margo UPDATE: 6-8-07 First the good news! Corey ahd a checkup yesterday and his pneumonia is completely gone! :) He is not yet back to his baseline but is on his way. He is still sleeping alot but for once in his life it's a peaceful sleep without pain for the most part. We are still seeing alot of seizures so my theory of them being caused by pain is wrong but he is doing very well. On to the princess. The sleep doc called Wednesday. He was very blunt and came straight to the point. Emmy's study was the worst he had ever seen in a child. Now he told me he wanted the repeat study not to set pressures on the machine but to verify the awful results he had the first time. This time they were worse. She has the highest setting on a bipap machine that he has ever set for a child. 19 inhale and 9 exhale. He said it was higher than most middle aged men that are severely overweight. The good news is it isn'ther brain forgetting to breathe but rather her entire airway system is collapsing but her brain is still fighting to breathe, not sure if that's good news or not! Also normal carbon dioxide levels for someone her age should be 15-20hers were continually at 70-79So all the 02 isn't doing any good when it can't get past the collapsed airway system. His recommendation is that she get a trach immediately. I am waiting to see what the pulmo says based on the tests she will be getting the 21st. I am starting to get used to the idea of a trach. Just something new to get used to. I think they said for the time being when she is awake it could be capped and overnight she would be hooked up to the bipap. I guess it's not much different as she is hooked up to it one way or another when she is asleep! So for all of you that have experience with a trach please send me any useful info as it still scares me but I'm sure it will be like most other things, once I am used to it, it becomes routine. I also hear that deep suctioning is way easier than thru the nose and with her needing constant suctioning I can use easier. Her lungs are still chunky sounding so it will be interesting to see exactly what is going on. I am still looking for a 40 hour per week pca with experience, if anyone in the Brainerd area would like to apply please let me know asap. I would like to have things figured out before Emmy is admitted and time is slipping away! Margo UPDATE: 6-6-07 I can't believe it's been so long since I updated! We are finally used to our new routine at home. Corey is doing okay. Sleeping way more than usual and requiring frequent oxygen use. His lungs are starting to sound yucky again. He will be going to the clinic tomorrow for a checkup. He is having some really great hours when he is awake and right now is seldom crying in pain. Our pain plan so far seems to be working. Next week he starts summer school. He will go two days a week for a couple of hours. Of course Jenne will be right there with him. It will be a bigger hassle to get him there, alot more meds and equipment but worth every minute of planning it takes! Emmy had her sleep study on Monday. It was only to get the pressure set on her machine. Even with a cpap and or bipap machine she continued to desat. There is also more evidence of a heart problem. She was a lil stinker, pulled wires off, hated the mask, and since it was being videotaped decided to keep on pulling the blanket over her head. This is something she has never done at home or didn't do it overnight last night either. She has become unusually floppy over the last few days. She has always required alot of support but now just flails about with very little control of any of her movements. I'm not sure what is going on. Finally they have things set up for her to be admitted. It took longer than I expected. She will be admitted June 20th with surgery being the 21st. She will be having a bronch along with a lung biopsy. Tests on her heart and an mri. They are also doing some other tests but they are very minor noninvasive ones. Depending on the results we will then have to decide to trach or not to trach. I know full well that a trach is going to buy us time, she will never have it removed, it opens up a whole new set of problems and that it is for short term solution only. I believe that we have decided no trach and hope that we are given a few really good weeks or months with her this summer. But I need to see the final test results before we make our final decision. We are making some changes in homecare. Please pray for everything to turn out well. That's it for here! We are just enjoying the days of summer with all the kids being home! Margo UPDATE: 5-31-07 We are home!! Every doctor that would come into our room I stressed the importance to me that Corey be allowed to attend Kindergarten graduation. We were finally able to convince them that we could handle the antibiotics at home. So after waiting for our new meds and waiting for PHS to deliver equipment we were able to leave! It took quite awhile and what seems to be a ton of oxygen tanks but we made it! Corey is on 10 liters of oxygen and each medium sized tank only lasts 1 hour. He will now be taking his pulse ox and 02 wherever he goes. They sent us an extra concentrator to use at school but he will still need portable tanks for transporting. Corey was very happy to be home! He did wonderfully at kindergarten graduation. Of course he slept thru most of it but the important thing was that he was there. It is hard for some to understand but when you have doctors saying he would never live this long it's the little things that matter the most! Corey is on a new pain regimen that is working well so far! Our neuro was so impressed that he contacted Dr. Shoffner ( a mito doc) in Alanta about it. A pediatric pain specialist from Germany came up with it. It has had a huge success rate with mito kids pain but is seldom used in the US. Emmy will be admitted the week of June 11 to Children's. She will be having a bronch, lung biopsy, mri and a test to check the amount of nerve damage she has in her hands and feet. They will also decide if it is feasible to track her. Our pulmo is hoping that he can get her to have a few great weeks this summer but really seems to think a trach will only buy us a few extra weeks and that she is declining rapidly and doesn't have the strength to tolerate surgery. We have made the decision to place both kids on Karuna care, it kind of like a ped. hospice. We are in the final process of deciding how we want things to happen as they are both rapidly declining at least according to the doctors! Corey is still fighting hard but I see Emmy is just tired, tired of life and that she just isn't fighting anymore. It's hard to tell one of your children that it's okay for them to stop fighting, that if they are tired that it's okay for them to be taken into God's arm, that mommy only wants the best for them. Today is the last day of school. It always makes me happy to have all the kids home but by next week after the constant bickering I'll probably be wishing for the school bus! I have a ton of people coming over today, more medical equipment is arriving. I just don't know where anymore will fit and here I thought we had every piece imagineable. Emmy's stander is also going to be coming. Our next appt isn't until Monday when it is back to Minneapolis. So I am hoping to have Corey's new med schedule figured out and to enjoy a nice quiet weekend! I want to thank everyone that helped out with the other kids while I was gone. All the emails and calls made things much easier. A huge thank you to Mike for putting up with Corey and I and for being there while I made some of the hardest choices possible. Thanks to Jenne for letting me borrow him and for making sure everything else was going okay! It's a huge thing to ask someone to help care for 6 other kids especially when one has special needs and can't just be left with anyone. Enjoy your weekend! I will be thanking God that once again we were chosen to be given another miracle! We met some other parents at Children's that were leaving the hospital without their precious babies, please keep them in your prayers! Margo UPDATE: 5-28-07 Corey is about the same. He will be having another swallow study on Tuesday. I doubt if we will get our pass on Wednesday for Kinderfriend or kindergarten graduation day. He remains on oxygen. Alyssa had to take Emmy to the clinic today. She has another aspiration pneumonia. Thankfully our pulmo from Children's sent up a plan. Emmy will be admitted down here for a couple of days for a bronch and some other tests. It also look like a trach is in her near future. If she isn't responding to the massive antibiotics she is on she too will be coming to join us. Thank you for all your thoughts and prayers! Margo UPDATE: 5-26-07 Corey is not any better. He is still on 100xygen and is currently on 15 liters. He has both a nasal cannula and blow by on him. Today they did another chest xray and confirmed that he has both an aspiration and bacterial pneumonia. He has an infection around his g-tube as well. They are awaiting a blood culture as they are sure it has spread to his bloodstream. Right now all meds to control seizures and pain that we started after being admitted have been put on hold. Right now the current plan is to get the infection gone before we start addressing his other issues. We are expecting an additional 10-14 day stay. So for now we are just hanging out and praying for the best! Thank you for all the thoughts, prayers andd emails we really appreciate them! We are at: Minneapolis Children's Hospital Room 6164 You can call me on my cell. Margo UPDATE: 5-25-07 Corey is not doing well at all! He is on 100xygen and stats continue to drop into the 70's. He is having a chest xray, bloodwork stat. They are planning on ntubating him based on the results of his tests. It was confirmed that he had a stroke but a small one. Corey isn't even moving during these procedures. Please pray for him and for Emmy as she is havingher own issues and I am torn up over not being with both of them!!!!!!!!!!!!! Iam suspecting this is going to be our new normal with corey. UPDATE: 5-24-07 Corey was transported from our home to Minneapolis Children's. It is assumed right now that he had a stroke yesterday morning. Right now we are waiting for a mri to confirm this. He is sleeping comfortably now after a very rough night. Please keep him in your prayers! He was at school when this all happened but thankfully Jenne immediately noticed his unresponsiveness. Emmy is doing okay and is at home with her homecare and sister takeing care of her. I'll update later once I find out what the plan is. If Corey is stable I'm assuming we will be leaving over the weekend. If his mri shows that there is still a possibilty of another stroke we will be here indefinetely. Margo UPDATE: 5-22-07 Corey and Emmy are both pretty much the same. On Friday we headed to Minneapolis for appts with the pulmo. Corey was changed from claritin to zrytec. He was pleased with how Corey was doing and pleased with how well of a winter he had. Emmy was an entirely different thing. He wasn't pleased with her lungs, her nonstop antibiotics, how many times she has been put on steroids and the results of her sleep study. I asked his opinion of how he thought she would do this summer and he basically hedged the question, stating that Emmy has so many varying factors. She won't need to be seen for a year, basically all he said was whatever meds I need to call and he would prescribe whatever I need but from a pulmonolgy standpoint there is nothing further that can be done. Our ped called and said our pulmo called her and hoped he didn't upset me too much. He has always been very upfront and has cared for the kids so much, sometimes I think it's harder to be the doctor than the parent! I saw the look on his face when he examined Emmy, the shock of her appearance etc. I have time to used to the changes but for someone that sees her only a few times a year it is very apparent. He told our ped that he would be truly surprised if Emmy survived this summer and that a trach will only prolong her life and not save it. So we have decided no trach as it won't truly improve the quality of her life. I just want her to go peacefully. I notice her overall decline on a dialy basis and all I want to do is make her comfortable. Emmy is still her sweet little self, yes she struggles each day and is gunky and gurgly and starting another round of antibiotics but she still is spunky as ever. Loves to pull at her tubes, gives you a smile that will melt anyones heart and is the most beautiful little girl imagineable! Today was Corey's IEP for school. It listed his goals for next year. My main concern was to keep Jenne as his nurse. I hope they understood how important this is to me. When we discussed Corey's physical wellbeing that was the hardest. I don't think that some people get the fact that this is a progressive disease. I know that Corey's physical abilities are on a decline. I know he isn't as strong as he once was and no more than likely he won't meet their goals. Other than that we are doing well. Emmy has a bunch of new equipment coming this week. Most of it will make it easier to transport her but some of the items will hopefully make her life a little easier! Corey "graduates" from kindergarten on the 30th. If I can ever convince Alyssa to post his pics you will get to see them! They are sooooooo cute! Have a great week! Margo UPDATE: 5-14-07 Emmy has been unusually floppy and very cranky over the last few days. Overnight she started to spike a temp again. It remains between 102-103 no matter what. I have been alternating tylenol and motrin all night. She is having a hard time keeping her stats up. Her 02 level will be at 92 and just when I can relax it will drop into the 70's and stay there for several miutes. Also her heart rate is very erractic. Having her oxygen turned up isn't helping out. So we will be heading into the clinic this morning. Corey has been sleeping so much lately. I am hoping it is him trying to get used to new and increase in meds. He had a few really good hours when he was awake yesterday so for that I am grateful. Yesterday Mike and Jenne came over for a barbecue. I didn't do a thing as Mike did all the cooking as well as making me a yummy margarita. Russ did his share as well, he ate and watched Mike do all the work LOL! I was going to be cooking as usual so it was a huge treat for me. Mike even cleaned up the kitchen, now what could be better than that? I will update soon about Emmy's appt this morning. We also have a pulmo appt for both kids on Friday. I am still waiting to hear when Emmy's sleep study will be. I have left several voicemails but one thing I have learned about Gillette's is they aren't the best at returning calls! Enjoy your week! Margo UPDATE: 5-11-07 I am sorry for the lack of updates but nothing really has changed. I find myself angry at doctors and angry with God. Sometimes I find it hard when his plan doesn't match what mine is! Corey is on a new higher dose of neurontin along with an adult dose of ibuprofen with t-3 as needed. It isn't helping and his only escape seems to be sleep. Then we are blessed with a couple of hours of him being nothing but our adorable, smiling little monkey and all the hours of worry just fade away. It's those times that keep me going both physically and emotionally. Emmy is doing the same as well. She is continuing to lose weight even with an increase in calories. We have started mixing her formula with pedialyte as her electrolytes are skewed and her other tests are showing she is no longer absorbing things like she should. Miss Emmy has decided that she no longer cares to sleep, that the world is much too exciting to waste it on sleeping. Over the last two nights, she has managed to take off her pulse ox, take apart her tubing to her feeding pump so she could feed the crib instead and has learned one little yank will set off an alarm on the apnea monitor. This coming from a girl that can seldom hold a toy! Then when I find out the trouble she caused she is laying there grinning from ear to ear, kicking her little feet. She has been fitted for her bipap machine. I haven't heard yet when her next sleep study will be but am assuming it will be this coming week. She has also been started on neurontin and another seizure med. I really don't think it is helping any. Today I was adding up all the medical copays from April, it is several thousands of dollars with Mikayla's broken hand to pay for. So I was complaining then realized how lucky I am to have these bills to pay for it means I have my lil angels with me still. Not too much else is happening here and that is the way I like it! Margo UPDATE: 5-7-07 Corey had a terrific time on Friday with Jenne and Mike. He went to their daughter's school carnival. When they brought him him it was only going to be for a short time and then they were going to leave again. When they were getting ready to leave Corey didn't want to go, this is really unusual for him as usually he loves to go bye-bye with them. So he stayed home. Later that night he had a grand mal seizure followed by tons of clusters of smaller seizures. It makes me wonder if he knew something wasn't quite right and wanted to stay home for that reason. He has been in tons of pain this weekend and nothing is giving him comfort except for when he can escape by sleeping. I am so tired and so angry at doctors that don't care about his pain and refuse to treat him. They all say he is terminal but then they "don't want him to become addicted to pain meds". These doctors I think are afraid of giving him anything as mito kids absorb things differently and I think they are afraid of getting sued. I have told them I will sign anything they want me to that wouldn't allow me to sue. I just know if it was their child something would be done. Why is it kids don't get the same care as adults? Yes he can't talk but you can see he is in pain, anyone with eyes can see that. So I am on a mission, Jenne his nurse and I are going to kick some doctor hiney this week. I am taking her with as she has tons of experience with both preop and post op patients and am hoping they will take her opinion more seriously than me. I sometimes think they feel I should be the one taking the valium so I would just leave them alone. Nothing is more heartbreaking than watching your child in pain, knowing that you have given everything you are allowed to and nothing works. I know from the love that Jenne has for him that it is heartbreaking for her to see as well. PLEASE, PLEASE pray that we get some relief for him. Somewhere there is a doctor that will do something, we just need to find him or her. Emmy is still dealing with a nonstop runny nose and awful sounding cough. She finished her antibiotics this morning and is running a temp this afternoon :( Her sleep study doc called on Friday. She has severe apnea and very low oxygen saturation levels even when awake. There is also something severely wrong with her heart. Her trachea is collapsing and they noticed that it is doing it when she is awake as well. Her 02 at home is doing very little. We will be heading back to Minneapolis for another sleep study, not to retest her but to have a bipap machine set for her. He told me this is only going to buy us some time and is only a short term solution. They can't/won't do a trach until they find out how extensive her heart defect and or damage is. Too risky for surgery for right now. Onto another weird note, when the nurse had to cath her for a UA on Friday she had a hard time. Surprise, she has a defect in her urinary tract as well. I suppose the rest of her gi system is abnormal so why should I be surprised? Her ua was normal just as I suspected when I saw the urine. Something came back in her blood abnormal but the local clinic didn't know what it meant. Something about the way she is absorbing protein and her magnesium and copper levels were weird as well. Her neuro is suppose to be calling tomorrow to tell me what this means. Emmy is frighteningly skinny, no matter how much formula is pumped into her she continues to lose weight. I see her regularly so it's harder for me to notice. Alyssa and her friend are back from Indiana and when she picked her up, Alyssa asked me how much more weight has she lost this week. She said she immediately noticed the difference and started to cry. She loves her little sister so much and this is so hard on her. So as I close please pray for Corey's pain issues, Emmy to gain some weight, her heart to be okay and for her to show these doctors that God has her safely in his arms. Also pray for the other kids as they are taking each new thing very hard and are so afraid of losing Emmy. Margo UPDATE: 5-4-07 Corey is doing really good! He is just busy enjoying his life :) He is still complaining about his hands and feet but the change in meds seem to be working well for the most part. Emmy sleep study wasn't the best. She went to sleep at 9 PM and was awake shortly after 11 PM. She did have a low heartrate, her trachea collapsed and her 02 levels dropped into the 70's and stayed there. When they started oxygen they were able to get her 02 level into the mid 80's but her heart continued to flucuate from 170's down into the 40's. I haven't heard what the plan is or if she will have to have this redone. I had told the doctor and the nurse if she sleeps more than 2 hours this is the best you can hope for. They looked at me like they thought I was crazy until they saw her pull her usual nighttime routine. Then she woke up, went back to her crying and whimpering and pulling her hair. The sleep doc was concerned enough to call in the neuro. Our neurologist upped her valium by a mg every 3 hours and her seizure meds. This hasn't helped so today we go in for bloodwork and an UA. When I asked him what for all he said was he wanted to rule out an infection. Hello! She is on antibiotics! A very high dose I might add, one that would take care of a uti too. Whatever! I just love it when doctors for what they can't explain chalk it up to a "brain related" issue which I have come to believe is short for I don't know, can't figure it out, now just go away! We'll see but she isn't getting any better and I doubt if I will see things change as a result of the ua and bloodwork. Enjoy your weekend! Margo UPDATE: 5-1-07 Sorry for the lack of updates. On Sunday we had Corey's birthday party at a nearby park that has a small zoo. This happens to be one of Corey's favorite places. I had decided a long time ago to no longer have parties for him for they stress him out so bad when there are a ton of people and relatives that he doesn't see on a regular basis. So it was only us as well as a few close people to Corey. I had thought about canceling due to Emmy but decided that we can't always let this disease dictat our lives. The hours it took packing up the stuff that Emmy needs was all worth it when I saw the joy on Corey's face. He absolutely loved it, especially the Curious George cake! No matter where Jenne went with it, he was right behind her. Not that he wants to eat it but rather stick the monkey into the frosting and smush the cake : ) You just can't help but love that face when he gets his "I know I shouldn't be doing this but I'm so cute no one will stop me look". He gets this silly little grin on his face and looks out of the corner of his eye and giggles, makes anyones heart melt. I will try to get Alyssa to post new pics as I can't figure out how to but she is leaving on vacation today. Corey is doing well, still has a cough and runny nose but overall is good. Emmy is still the same. She just isn't recovering from this pneumonia like she should. We are constantly battling low heartrates and 02 levels. She also is crying alot, holding her head, pulling her hair so I'm waiting for a call from the neuro as I'm sure meds need to be raised again. Sadly I can feel and see how much weight she continues to loose. We are back into newborn diapers and smaller clothes. I just don't really have much to say about her as I know she is in God's hands and whatever he has planned I can only trust that she will be healed. Rather it is on earth or in Heaven that isn't for me to choose. I am leaving this afternoon for Minneapolis with her. She will be having another sleep study done. Please pray that she sleeps and shows her stuff. Last time she didn't sleep at all and without a clear sense of how many times she destats and how many periods of apnea it's hard to choose what is our next option. We have the recordings from her monitors at all so there is already a plan but we need one in a controlled setting so the insurance will pay for necessary equipment. This will help in deciding if she can just use a bipap machine, if surgery using a rib bone in her trachea will be enough or if she will need to be trached. It will also help to figure out what is going on with her heart causing it to drop her rate all the time. Right now it is assumed that it is due to brain atrophy but the cardiologist is concerned that rather than a brain issue it is due to electrical problems with the heart itself. Tomorrow morning they will be doing another ekg and ultrasound of her heart and then we will see the neuro, sleep specialist and head home hopefully. I'll update when I know anything from her tests. Thank you for all your thoughts and prayers! Margo UPDATE: 4-28-07 SATURDAY AM---------------- Emmy has continued to struggle overnight, struggling to breath, to keep her stats up, etc. I was up all night doing nebs every couple of hours, adjusting 02 levels and most of all just wanting to enjoy every second with her. Thank God we are still together this morning. I'm not sure what it is about this pneumonia that has me worried as she has had tons of them before or if it's because I see her little body getting so tired of her struggles. It is times like this I wonder if I am making the right choices, having made the decision not to allow her or Corey to be kept alive by machines. Then I realize how selfish I am being, wanting to kepp her with me when I know her life in heaven will be so much better than life on earth. I hate mito and I hate all the suffering these little ones go thru! Our Princess is awake this morning and during her neb grabbed the tube and gave me a smile that will forever melt my heart. It was a look and a smile that said, I'm not ready to give up this fight. Ah the sassy and spunky look I get from Corey so often crosses Emmy's face as well. ************UPDATE FRIDAY PM********** I had to take Emmy back to the clinic this morning. She had another mucous plug last night. She is on another antibiotic and mucous thinner. If her stats go low again we will be calling 911 and were told to request a helicopter. She is really struggling right now and can use your prayers. Even the doctors admit that there is nothing more that the hospital can do than we can at home but just want us to be comfortable. ********************************** Yesterday morning Corey had blood draining from his ear. Fortunately I was able to take him into the clinic early. He of course is on antibiotics for an ear infection and also ear drops. He has permanent tubes so I am assuming it became blocked. His ped. couldn't see the tube so I am hoping it is still intact. Corey weighed a big 22 pounds yesterday! But he has also grown taller by a few inches in the last few months. Corey had a good time even if he was sleepy at his party with all his classmates. It is when he is surrounded by regular kindergarteners that I am most aware of how small he is and how delayed he is. But I am also struck by how accepting kids are of him. If only adults were that way. Most of the regular kids are curious and have questions about him. But outside of that they see him as Corey and nothing beyond that. Emmy also had her appt. yesterday with the ped. I was expecting just the weight check and the exam of her incision. She has lost 9.5 ounces since last week but her incision is healing great! What I wasn't expecting was the results of her latest brain scan. So the ped. and I had a very frank talk about Emmy's future. Emmy has had significant atrophy since December particularly in the brain stem. Compared to Corey's scan at the same age, it is obvious that her disease is rapidly progressing. We also talked about how much more intervention that I want to do in regards to keeping her alive. For now we are okay but discussed tpn, the necessity for a trach,etc. Also the lumps on Emmy's head are becoming more and more significant. They are due to her brain not growing and actually decreasing in size and that there really is nothing other than for cosmetic reasons to fix it. Discussed Emmy's oxygen needs, the increase need for deep suctioning, how she no longer swallows her saliva, her bowel and bladder issues (she is losing the muscles needed to pass both urine and stool). Sorry for all the yucky details but I just wanted everyone to be aware that Emmy is not progressing and probably won't. I am surprised that I handled this so well, even thou I know it, reality and to have it spoken aloud is still a slap in the face. Our ped just looked at me when I told her that I worry about today and I let God decide about tomorrow. Last night Emmy struggled with max amounts of oxygen to get into the 80's for her 02 levels. I can make myself crazy after I do everything humanly possible for her by starring at the machine or else I can do what I did. Yes I turned the alarms down, put away the stethoscope and went to bed. Sounds awful doesn't it? Just asked God to watch over her thru the night for there isn't a thing I can do or a doctor can do either. This morning Emmy is awake with a smile on her face, Corey is up wanting his bath, all the kids are getting ready for school, hugging and kissing Corey and Emmy. Another miracle from God, yes my life is so good! UPDATE: 4-26-07 Yesterday morning Corey had blood draining from his ear. Fortunately I was able to take him into the clinic early. He of course is on antibiotics for an ear infection and also ear drops. He has permanent tubes so I am assuming it became blocked. His ped. couldn't see the tube so I am hoping it is still intact. Corey weighed a big 22 pounds yesterday! But he has also grown taller by a few inches in the last few months. Corey had a good time even if he was sleepy at his party with all his classmates. It is when he is surrounded by regular kindergarteners that I am most aware of how small he is and how delayed he is. But I am also struck by how accepting kids are of him. If only adults were that way. Most of the regular kids are curious and have questions about him. But outside of that they see him as Corey and nothing beyond that. Emmy also had her appt. yesterday with the ped. I was expecting just the weight check and the exam of her incision. She has lost 9.5 ounces since last week but her incision is healing great! What I wasn't expecting was the results of her latest brain scan. So the ped. and I had a very frank talk about Emmy's future. Emmy has had significant atrophy since December particularly in the brain stem. Compared to Corey's scan at the same age, it is obvious that her disease is rapidly progressing. We also talked about how much more intervention that I want to do in regards to keeping her alive. For now we are okay but discussed tpn, the necessity for a trach,etc. Also the lumps on Emmy's head are becoming more and more significant. They are due to her brain not growing and actually decreasing in size and that there really is nothing other than for cosmetic reasons to fix it. Discussed Emmy's oxygen needs, the increase need for deep suctioning, how she no longer swallows her saliva, her bowel and bladder issues (she is losing the muscles needed to pass both urine and stool).Sorry for all the yucky details but I just wanted everyone to be aware that Emmy is not progressing and probably won't. I am surprised that I handled this so well, even thou I know it, reality and to have it spoken aloud is still a slap in the face. Our ped just looked at me when I told her that I worry about today and I let God decide about tomorrow. Last night Emmy struggled with max amounts of oxygen to get into the 80's for her 02 levels. I can make myself crazy after I do everything humanly possible for her by starring at the machine or else I can do what I did. Yes I turned the alarms down, put away the stethoscope and went to bed. Sounds awful doesn't it? Just asked God to watch over her thru the night for there isn't a thing I can do or a doctor can do either. This morning Emmy is awake with a smile on her face, Corey is up wanting his bath, all the kids are getting ready for school, hugging and kissing Corey and Emmy. Another miracle from God, yes my life is so good! UPDATE: 4-25-07 HAPPY 6TH BIRTHDAY COREY!!!!! Wow my precious little boy is 6 today!!! A day full of precious memories, I spent all night holding Corey, unable to sleep, just wanting to thank God for this lil miracle on my lap! Reminded how incredibly blessed I am to have him in my arms, to be allowed to have another birthday with him! All the memories of his life come flooding back. The newborn baby that has struggled from the moment he was born to breath and to live. The moment when he was a few weeks old that I knew deep in my heart that something was so wrong to the realization that there really are angels on earth. To the first few months of accusations that we weren't feeding him, the doctors that told me all babies develop differently, until one day a doctor listened. The trip to Mayo expecting to hear what medicine or diet would make everything all right again. To have a doctor at Mayo give you a list of what he thought it was and the one he told us to hope for was awful. To finally be told that he had a disease I had never heard of and one that there is no treatment or cure. To be told that at 9 months they didn't expect him to live until his first birthday and that this was the best it was going to get..... All the struggles he faces each day, the walking that seems to be getting a little more unsteady but then there's Corey. How does one explain him to someone that has never met him? He's alittle boy with the wisdom of one that knows for sure how much God loves him. He has this huge love for life, lives each day to the fullest. Blue eyes that tell you a million stories without him ever saying a word. Loves his family and especially loves his little sister and has this intuition when something isn't right with her, they seem to have a communication of all their own. Some see him as a handicapped or a terminally ill child. All you need to do is to meet him and soon you realize that while he may not talk, he teaches and says a thousand different things each day. Who would've thought that six years later, the child that the doctors said wouldn't live will soon be "graduating" from kindergarten. The doctors that to this day refuse to say anything but with his brain atrophy he shouldn't be alive and won't admit that Corey is here because of God's plan and that it has nothing to do with them. That while to some he doesn't do much is thriving in his own way. I would like to thank his pediatrician that fights so hard for him. All the people that have made it possible for him to attend school. His teacher, Gigi, the people that agreed that school was okay to try when all the doctors that said no. A special thank you to Jenne that with out her Corey would never have continued to go to school. Words can't express how forever grateful I am to have a nurse for him that not only takes terrific care of him but loves him as well. Thanks to Mike too for becoming a huge part of our lives. Corey will be having a party in his regular classroom today. He is usually in a special needs room but he loves to visit with his regular kindergarten class. We will be waiting to have a party at home until I decide what to do. I wanted this update to be about Corey and his birthday so I will update tomorrow about Emmy. Margo UPDATE: 4-19-07 Oh how I hate it when I go so long between updates. There is no excuse except that life just gets in the way. Emmy is continuing to lose weight. Her j tube is functioning well but her little body isn't tolerating much food. She is suppose to be on continuous feedings but I often need to unhook the pump as she becomes miserable and bloated, then she begins to gag and throw up. The gi said that due to motility everything even with a slow drip is backing up. I had thought that the seperate j-tube would be our answer. Also each time she is fed she becomes agitated and her seizures increase. As long as her body isn't processing food she is happy. It's sad to think that your baby would be so much happier if she never had to eat again. She is already on a very broken down formula so even changing that isn't an option. We have tried increasing the calorie content but that leads to only more problems. She is still having alot of pain and is still on pain meds but so far trying to wean them isn't working. She receives all her meds into the j-tube and if you give her more than 10 mls at a time she vomits. So we do 10 mls over a couple of minutes, followed by a 5 minute break. She is on 9 meds several that are every 3 hours so it seems the meds are never done. If she continues to lose weight (she will get recehecked next week)the gi is anticipating another admit and having her put on tpn. We are slowly getting used to her new routine and all the new equipment needed to take care of her. Please pray that she will tolerate feedings, gain weight and will start to have a bowel movement without an enema. Corey is doing okay, he is still having trouble with his hands and feet as well as walking. But for the most part he is doing well. He has really been trying to let us know what he wants and when he wants it. Not by words but by pointing. If you don't move fast enough he pinches hard. He has also started to give me a syringe or pointing to his cupboard for medicine. He knows exactly what bottle is the neurontin or pain med. It is sad to see a child want medicine. With my other kids I chase them thru the house when they need something. I think Corey knows that when he is in pain these are the two things that help. Our little man Corey will be 6 years old next Wednesday! I can't believe how the years have flown by and often think of how the Mayo clinic told us 5 years ago that he would never see his first birthday. Birthday parties usually make him upset so once again we will be doing something that he will enjoy. I haven't decided what yet. I know he will get his pinnata that he will love but am torn between the zoo or a picnic at a park he loves. I am also trying to convince Alyssa to make him a monkey cake. Have a good week and I'll try to update soon! Margo UPDATE: 4-14-07 Our Princess Emmy is safely at home tonight and for that we are forever grateful. I will do my best to explain what has happened over the past few days but for the most part it is pretty much a blur. It was decided that we could finish antibiotics at home. She has been tolerating her feedings thru the new tube well. It was yesterday before the surgeon fully told me to what extent it took to place the j-tube. He explained that starting from her throat into her lungs/trachea, esophagus thru her stomach, small intestine and colon she has a birth defect that is unrelated to mito and the neuro disease (thank God Corey doesn't have this as well!). They ended up removing approximately 4 inches of the small intestines and used stints to reconnect it. They had some special word for it but it's one I'll never remember! The removed area was the same area they saw in March that was enlarged, red and boggy due to a partial impaction, it was also starting to twist inside itself. They had to straighten out a few other areas as well or they would cause problems further down the road. We need to be extra careful for her getting sick the next few weeks in order not to put strain on her incision or intestines, the flu will end her back up in the hospital and the stints failing could cost her life. So for this reason we will be limiting visitors, I hope you understand, okay Mike this won't include you :). We also won't be taking her out of the house except for medical appts and any nonessential appts have been cancelled. We were told by the gi that in the near future it is likely she will need a colostomy. We also learned that her stomach and intestines have very little motility. Pulmo was also involved addressing concerns regarding her airway. So we are now the not so proud owners of several new machines. Sometimes I think if one more piece of medical equipment is needed our house will burst at the seams! I guess we'll all get used this new normal in our lives but for now we are so grateful to be blessed with her in our lives that little else matters. I came home to find Corey not walking well. I'm not sure what is going on with him and his neuro hasn't been much help. His poor little legs keep locking and his foot is turning in like it never has before. I'm hoping it is his arthritis flaring up but his neuro seems to think it is incorrect messages being sent from his brain. Sometimes I don't think people realize the daily struggles these two go thru. I think most people see them as kids that will progress, maybe slowly but will progress. The fact is they won't, they are both losing and not gaining skills. The little things that Corey does we are grateful for but we know one day he won't be able to walk at all. I don't know which is worse having a child that remains a newborn for their lives or having to watch your child lose skills that they once had. Watching Corey struggle to walk or hold something and seeing the increase in hand tremors etc is heartbreaking. He is starting to so frustrated at trying to hold his straw to take a drink, struggles to eat his beloved french fries and wants to be fed when only a few months ago he wanted to do these things by himself. When people expect him to meet new milestones it makes me mad, when in reality we are struggling hard to hold on to the ones he has. Tomorrow we were suppose to attend the Harlem Globetrotters with HopeKids, MN. Knowing that we can't attend Jenne and Mike offered to take the other kids so they wouldn't have to be dissappointed. How cool is that? I will be forever grateful as Liam and Evan were especially looking forward to this. Thank you for all your thoughts and prayers while Emmy was in the hospital. They are more appreciated than words can ever say. Margo UPDATE: 4-11-07 Sorry for no update yesterday. Emmy came thru the surgery just fine. They had to resect her jejenun and put some "connections" and clamps but it went really well overall. Today she has started to run a temp and her psin issues aren't under control. So they have started her on vanco, increased her morphine to once per hour and added toradol (sp?). Hopefully things will take a turn for the better. Right now my hopes of a weekend discharge or on MOnday are rapidly fading as they are now talking about the end of next week. But we all know how quickly things can change when youa re in the hospital! Margo UPDATE: 4-9-07 We are having a very low key Easter, decided to stay home in fear of exposing Emmy to anything. Corey has spent about 95f today sleeping. I hope that he isn't coming down with anything. It is incredible hard for me to leave him and I don't know what I would do if he was sick. Emmy is her usual self. It is making me worried to know that her docs are worried about this surgery but we realize we no longer have a choice. On her preop papers her pediatrician wrote: okay for surgery at a pediatric facility as long as proper precautiona are taken but remains unsure with her current underlying issues regarding breathing and stats. Oh just what a worried parent doesn't need to know! I am very apprehensive and keep on having a reoccuring dream of her coming home on a vent that quickly turns into being at her gravesite. Even thou I can't sleep and won't until she is safely back home it makes me feel better to know that she is being held tightly in Jesus's arms. Please keep her in your prayers especially at 12:45 PM tomorrow until 5 when the surgery should be over. Also pray for the sanity of Jenne, Mike and Schlaine that have taken on the enormous task of the other kids. Corey is always upset whenever I am away but I know that he will be well taken. Also the other kids are affected deeply. Alyssa will be at the hospital in between her work and stuff. Sean just tries to keep himself busy. Lexie will hide in a book, Mikayla will cry alot, Liam is sure to get into trouble at school and Evan will remain unusually quiet and will pretend he doesn't care. So many kids each being affected so differently by things no child should ever have to worry about. I will try to update tomorrow evening if possible or I will call someone to let you know how things are. Holding tightly to the belief that with God all things are possible and that in times of need that is when there is only one set of footprints..... Margo UPDATE: 4-5-07 First the good news! Corey's new meds seem to be working. He seldom complains of his hands and feet hurting and is starting to perk up. His seizures that were increased seemed to have settled back down into the usually normal amount per day. Overall he seems to be doing good. He knows that I am getting ready leave as he points to me packing and cries go-go-go. Second good news is that Mikayla's hand is healing perfectly, a couple more weeks and she will be back to normal. We had 11 inches of snow according to the official report. In our yard you can't tell, some places are blown clear while others have a 4 foot drift. Funny how the drifts always end up in the driveway! So I guess our Easter egg hunt will be indoors this year! Emmy has her preop appt this morning. The anesthesiologist called yesterday. He knows the mito protocol for my kids as he is always the one the sedates them but he is worried about the length of the surgery and her recent history of respitory issues. Yesterday was hard for me, until recently we only had oxygen for sick times, emergencies etc. Due to Emmy's increase need for oxygen they replaced our small tank that ran for 4 hours to a concentrator, gave us a large tank for just in case and we now also have portable oxygen that we need to take with us everywhere :( It is just another slap in the face to show how quickly she is sliding down hill and not making the climb back up. For now she is only requiring oxygen while asleep but if her stats continue like they are we will soon be on it fulltime. We will also be trying her on a bipap machine at the hospital instead of a cpap machine. The pulmo told me this will only buy me time until a trach but don't expect this as the answer. Please keep all of us in our prayers, especially the princess! Margo UPDATE: 4-3-07 Emmy had another episode last night where her stats went way low-heartrate would go from 20 then skyrocket into the 200's and then down again. Her 02 levels would stay in the low 40's. After much suctioning, a neb treatment and oxygen we were able to get her stats to come up-02 in the low 90's and heartrate remained irregular. I spoke with her neuro that said that it was up to me if I wanted her airlifted to Minneapolis but there is really nothing they could do, only if I was uncomfortable with her at home. Her lungs had tons of rubbing noises in them, her trachea was collapsing and she was retracting badly. She didn't sound like it was another mucous plug so we kept her home. I am thinking that when she is tired her muscle tone is so bad and this is what causes it. Later on after I was driving myself crazy cuz nothing really was working I put away the stethoscope, turned her monitors towards the wall so I couldn't stare at them and prayed. I just decided I could sit there all night, making myself crazy or I could trust God to take care of her. I felt so much better after I just decided to let go and let God. Emmy's surgery has been set for Monday April 9th, please pray that all goes well and that she stays healthy until then! Corey is doing okay, he seemed to be greatly affected by Emmy's episode last night and needed just alot of reassurances that things were okay. After awhile everytime I left her bedside he would do something that would trigger the alarm to go off. I would come running back in and she would seem okay but once I caught him purposely pushing the button to make it alarm. He thought it was pretty funny. I guess he thought I should be in there, holding him and keeping watch over his sister. This from the child that doctors say is unaware of what is happening, ha! This is an extremely busy week with spring concerts, Mikayla's ortho appt (if her hand is healing properly she will need surgery, she is still complaining about it), Emmy's preop appt, an appt with the physiatrist, plus a few other appts, etc. I'm not sure when I will update. I will of course if anything changes with either of the kids. UPDATE: 4-2-07 Sorry for the lack of updates but as a family we are trying to cope with how quickly things are changing for Corey and Emmy. As many of you know Corey was recently diagnosed with severe neuropathy. After several med changes I am starting to think we have things beginning to get under control. In order for him not to be in severe pain at all times we have had to medicate him. Unfortunately the side effect of these meds are that for the most part he is either completely out of it or sound asleep. I am hoping that over time he will build up some tolerance to these meds and will be able to have some sort of life without pain but still be able to function. It is very difficult for all of us to see only short periods of the once vibrant little Corey. I have always been very proud of the fact that he can walk, yes very unsteady and has an awkward gait but walking nonetheless. So many times now he can't/won't walk due to pain issues. He also went thru the final test for Neimann Pick Disease on Thursday. There were a couple of things that they needed to reverify. I didn't even ask what this meant but rather will deal with the results when the final ones are in. They already have added Emmy to be tested so I am pretty sure Corey's initial results were positive. On Thursday following Corey and Emmy's appts we went to the circus. We had received free tickets thru HopeKids. Corey didn't pay any attention to the circus itself but he loved to ride the elephant. He had a ton of seizures that day and seemed to be in a great deal of pain but once on the elephants back it seemed to vanish. It was so nice to see him with nothing but joy on his face. He looked like any other child enjoying the circus. He rode the elephant over and over, who knew a free circus could end up costing so much? It was like the commercials for the creditcard, elephant rides $5, the look on Corey's face-priceless! He also enjoyed getting every light up thing they had for sale. On Thursday Emmy had her appt. I had thought it was only to be with the surgeon as we had just seen the gi on Monday but no, much to my surprise all her doctors were there. Neuro, gi, cardiologist, surgeon, pulmo etc. I think I am forgetting a couple. Anyhow she will be having a separate j-tube placed either April 9 or 10th. They had wanted her to have her stomach tied off and her to get a trach but Russ and I aren't ready to do that nor are we convinced that it is in her best interest at this time. The pulmo told me that he knows we will be back in within the next 6 months to have the trach and stuff done. I have a feeling that up until the other surgery is done they will still be trying to convince me. Her j-tube can't be done with a laproscope. She has a defect in the jejenum and stomach so the current plan is to move the jejenum to right side of her body using the liver as an anchor. Currently she has a twist in her jejenum which causes her to be partially impacted frequently. She will have a incision midline from her nipples down and around her belly button. They also want to do a nissen but doubt they can due to the abnormality in her stomach. We were told to expect a minimum of 7 days in the hospital and not to be surprised if we are there for 2 weeks if things go as expected. Of course if she develops an infection or impaction the time will be alot longer. When I asked so long I was told that her wasn't a normal j-tube insertion. They will also try to give her the mickey button right away but told me not to be surprised if she has to have a peg tube tpe first. Well that's it from here! Margo UPDATE: 3-28-07 Hope you enjoy the new pics! Also included is a picture of Jenne and Mike. Jenne is Corey's school nurse and Mike is her husband. I had a rather funny picture of Mike which Alyssa wouldn't put on for me. So today's update is dedicated to this awesome couple that have forever impacted our lives. I was so worried about sending Corey to school and am truly surprised I did! Corey immediately took to Jenne and she to him. I knew instantly that we had our prayers answered in finding the perfect nurse. Each day I send Corey to school I am at ease knowing that he is well cared for and most importantly loved. This awesome couple have also taken care of Corey overnight when Emmy was in the hospital and have volunteered to do so when she has her surgery in the next few weeks. Mike has taken my other kids snow sledding (by himself I might add). Put up with the 3 boys bowling and asking for quarters and has volunteered numerous times to drive me to appts and to tag along to any sports related Hope for Kids events that Russ can't make. He has also taken care of Corey for the little sleepover they had. LOL he found out that a sleepover with Corey is like a 12 year old girl slumber party, not much sleep is happening! Makes him wants to sail away with the Captain, huh Mike? Emmy absolutely adores Mike, even on her crabbiest days she loves to be held by him. I have a sneaking suspicion that she has him wrapped around her little finger! Emmy will be having surgery soon and Corey is need of prayers but I will update on those things another time. I wanted this just to be our way of thanking Mike and Jenne for all they do for us! UPDATE: 3-26-07 Emmy had her appts on Friday which were a complete waste of time and money. I have been anxiously awaiting this appt since OCTOBER. Apparently she doesn't "fit" this doctors current study so he will do nothing to address her pain or sleep issues. He told me 1-all her issues are neurologically linked 2-she is too medically complex for me, I won't even think about treating her pain 3-her seizures are so out of control but you already have the best neuro there is and I won't even begin to try to chase those seizures 4-have you thought about putting her in a group home or sending her to respite 5-stop looking at me like you think I'm a failure. Needless to say I cancelled Corey's appt for this summer. This particular doc is suppose to be the best for pain management in children. PLEASE if anyone knows of a doctor that specializes in pain issues in children let me know. I am willing to travel anywhere to get the help we are desperately searching for. We are heading to Minneapolis shortly, once again Emmy's j tube is out. The vomiting of her meds gave it away this time. She has been super crabby since Friday evening-today when I was putting in a tube to hold the stoma open all her meds were just sitting there not being absorbed. Corey is still in pain with his hands and feet, the new meds along with codeine isn't helping much. The neuro is planning on discussing more options with me tomorrow-at least he returned my call but admitted he needs to contact some other docs and do some research on what to try next. Doesn't that sound like it should reassure me? LOL. He needs to come up with a plan. Corey spent the night with Jenne (nurse from school) and her husband, Mike. He didn't seem to mind but I didn't sleep at all as it was the first time I have been home and he wasn't there for bedtime. Well I better run as I need to leave soon. Margo UPDATE: 3-21-07 Corey's behavior is totally out of control. He is constantly on a biting, pinching. hitting, spitting rampage. He is in constant pain which I think is causing him to act out as he can't tell us what is going on. He is either holding his head, crying about his hands and feet or a combination of the 3. We are trying a new med for pain plus combining it with tylenol and codeine every 4 hours, it doesn't appear to be helping :( His neuro and I were talking about the time is coming when Corey will need to be medicated all the time with morphine. This will leave Corey asleep most of the time. As awful as it may sound to others I told the neuro I hope he dies when it comes to that point. He seemed shocked but to me when Corey has to be so medicated that he is in a coma like state and is only the shell of the vibrant little boy I love I can only pray that God will take him in his arms and into heaven. Corey is a little boy that fights so hard each day and loves life that the thought of him being on a ton of meds and machines breaks my heart. I have told him often the last few days that when his pain is so bad that it is okay for him to take God's hand when he is too tired to fight anymore. Yeah I'm mad,mad at this disease that is stealing my child, angry that instead of signing him up for teeball that I am fighting to get him to see a pain specialist, mad that doctors don't take the pain of children seriously, mad that my family doesn't understand the hell he is going thru, etc. Then one little sassy smile smile or one kiss from Corey and this fight is worth every minute. Emmy is about the same, we have gotten used to her new routine. She alternates between good and bad times. Her seizures are still way out of control, she cries alot but then that beautiful smile or a giggle shows up and once again I am reminded of why this is worth every second. I need to ask for some specific prayers-pray for sleep for Emmy-she doesn't sleep for more than 1/2 hour at a time. Pain relief for Corey and Emmy. Also the transmission on Alyssa's car broke, she still owes a big chunk on it and my van is broken down. Not a good thing when we have a ton of appts to go to and she needs to get to school and work. Pray that something comes our way. Also Emmy has an appt on Friday, pray that this doctor will have something new to try and that it helps with her pain and sleep issues. I take care of the kids overnight by myself and most of the time I am in a fog from lack of sleep. Sometimes I think if I had two hours of sleep my body would die from the shock! Please pray for our friends in Texas, they need help in finding a doctor that can help, pray for God to lead them to the right one! Margo UPDATE: 3-15-07 Emmy is doing okay and we are getting used to our new routine, each day it becomes easier. Our feeding pump broke and the company just doesn't understand why I needed a loaner sent overnight express. Our medical supply company doesn't have a loaner either. We have been using Corey but as far as I know you can't hook up two kids overnight to the same pump. A loaner is suppose to arrive today but that is what they have been saying for the last 2 days. Emmy has a partial impaction in her bowel. We are on a new "bowel cleansing" plan. Not a fun thing and requires numerous clothing changes each day. Overall Emmy is doing well, her lungs are still yucky sounding but each day she seems a little stronger. Corey has been sleeping alot, very unusual for him. I'm not sure if it's because he is catching a cold or if it means there is impending danger around the Corey. 9 out of 10 times him sleeping alot means a series of grand mal seizures followed by a sle is going to happen. So we are keeping an extra close eye on him. Corey received his Spring picture package from school, they are sooooo cute! I will try to get Alyssa to post one but with her one never knows! Margo UPDATE: 3-12-07 Here is the latest on Emmy. She has been discharged from the hospital but there are still many unanswered questions and concerns. She is on 3 new meds, and our new neb count even if we double up on them is 11. The pulmo said even thou she tested neg for cystic fibrosis we need to treat her as if she has it. She has always had very thick, stringy mucous.She will be heading back shortly for a short stay as soon as they get some testing set up and once they come up with a "plan". Her voice box is abnormal as well as her trachea. That is why she always sounds hoarse and never really has a full blown cry but rather whimpers. They are also planning a biopsy of her lung. It will then be determined if a bipap machine will work or if she will be getting trach. The pulmo told us he believes in addition to the mito and neuro disease she also has another genetic disease affecting her airway. I can't remember what he called it as it really didn't matter, it's just one more thing to deal with. Then on to neuro-her seizure meds have been doubled and her valium has been upped by half. This was after the nurses kept on mentioning her nonstop seizures, which we have mentioned almost on a daily basis to the neuro. To us it is her norm but to them it was a shock. Now to gi-her bowels and intestines are boggy and distended. So we will be heading back for a barium study. Usually this means and impaction but she has been having normal bowel movement for her and the only concern is the bile that is backing up. The gi thinks it's her formula and an allergy to it rather than a blockage, she is on neocate a very broken down formula but she will still need further testing. For now all I need to do is keep an eye on her bowel movements. We will also be going ahead with a permanent j tube and getting her stomach tied off as all of us have agreed this is our only option. Right now there is mention of her going on tpn to allow for gut rest but the gi said he wanted it down inpatient as every little change affects her so much. So our new norm is oxygen, always having a pulse ox and monitors on (before they were used for sleep only), more nebs and chest therapy, more meds, daily bowel cleansing, feedings going slower and a few other things. Corey is doing good, hard to unwrap him from around my neck when we arrived home! He gave me the biggest smile and wouldn't let ago. He also insisted on sleeping on my chest the entire night. Nice to know that he missed me! He has been having increasing problems with neuropathy, his hands and feet swelling, getting very hot and red. It seems to bother him alot. Please pray for all of us. Especially for Emmy to get some peaceful sleep and for the docs to find something that will work for Corey's neuropathy. Margo UPDATE: 3-7-07 Emmy aspirated again overnight. Her lungs were sounding alot better but now she is gurgly again. There is something going on with her but I can't put on finger on it so I'm just hoping it's a few "mito" days. She still isn't tolerating her feedings and has lost another half pound. She is continually "dumping" all her feedings. Corey isn't acting like his usual self either, I'm hoping he just needs some time to adjust to the new meds. We are still trying to find that fine line between him not having the amount of behavioral issues to still being able to function and live his life. I spent quite awhile at the clinic yesterday and it wasn't with Corey and Emmy! One of Mikayla's friends hit her in the hand accidently with a rubber ball of Corey's. Her fingers bent back like we have all done a hundred times. She complained it hurt and it did swell slightly. So I iced and since she could move fingers and wrist didn't think too much of it. Monday she complained all day but it looked the same. By Tuesday morning it was black and blue and swelled about 3 times it's usual side. Turns out she fractured her hand in three places, her middle and fourth finger are also fractured with 2 bones chips. We were then sent to the orthopedic surgeon that thankfully was able to position the bones back into place, they had thought she would need surgery. She is now sporting a pink cast from her elbow to the tips of her fingers, with her fingers pointing downward to the floor. Next week she will have an xray to make sure the bones are healing properly or else she will need surgery. Needless to say I cried when they told me how badly it was broken, I felt so bad as I told her I was sure it was only sprained. I guess no mother of the year award for this one! Today we have an appt. that will determine how many nursing hours both kids will get for the next year, please pray that the temporary increase in hours for Emmy will become permanent and that Corey's hours will stay the same. It is hard for me to remember all the little things that we do each day as it is just part of routine. They give you so many units so each thing counts. There are so many days now that Emmy requires so much extra care, not sure if they will consider that or not. Please remember to keep Corey and Emmy in your prayers. UPDATE: 3-5-07 Emmy has another aspiration pneumonia. We spent part of Saturday at the urgent care clinic from hell. Where do you suppose uc docs get their medical license from? I always dread going there and this time was no exception. The doc we saw didn't check anything but her lungs and just assumed I was right when I said it was caused by aspiration. Asked me what she is usually prescribed and what I wanted him to give her. When I told him what she usually gets, he then asked me to spell it, didn't know what a gj tube and told me to take her home, do whatever it is I usually do and if she gets worse I should arrange for her to get transported to Minneapolis. Then his prescription read give mom whatever it is she wanted. So they looked up her last prescription amount, and he just signed the order. Emmy is still struggling, not tolerating feedings and sounds awful which of course increases seizures, it's a nasty cycle! Coreyhas been acting strange all weekend, goes from laughing hysterically to sobbing uncontrollably. I am pretty sure it's a reaction to the new meds but so far the meds hasn't stopped his behaviors in which he is hurting himself and others. Corey's nurses husband Mike brought Liam and Evan sledding yesterday. They were very tired last night but apparently had a very good time, according to Liam it "rocked". They miss out on a lot of stuff because of Corey and Emmy. It was nice to have them have such a good time.Thank you Mike! Well that's about it from here! Margo UPDATE: 2-27-07 Over the last few days Corey's behaviors have gotten very bad. He bites, kicks, spits, pinches, pulls hair and slaps both others and himself. This weekend they were awful, he has left bruises on most of us. We have increased his valium substantially with little or no affect. Medically nothing wrong can be found, no infections etc. We are giving the increase valium a couple more days to work before we need to try something else. In talking with his neurologist he will most likely need to be put on another antipsychotic drug. We have been down this route before with ativan, zoloft and risperdal, most of them left him drugged and unresponsive for the most part. Please pray for us to make the right decisions regarding treatment and guide his docs in picking the right meds. I received a call from Mayo today, they need to retest Corey's blood as somehow the blood for Niemann pick test was contaminated. Also he and Emmy are showing a ton of metabolic errors so they are retesting those results as well. Emmy remains the same and was fairly alert this morning for about a 1/2 hour. Then it was back to the nonstop crying etc. We have one appt. in Minneapolis this week on Friday, please pray for no more snow and for us to be given some answers! UPDATE: 2-26-07 It is really hard to explain how Emmy is doing. For those of you that have met her it will be easier. Even thou she lacked alot of her physical skills she was a little girl that loved people. Smiled often, eyes always looking around and they were very bright blue and she loved to babble and would wiggle at her favorite toys. The little princess I have now has changed drastically, her eyes are no longer eagerly looking around, rather they are clouded and seemed to have faded to a dull gray. She has lost the twinkle in her eyes. She doesn't pay any attention to music or toys and rarely does she smile. She spends most of her day crying inconsolably and for the most part isn't aware unless I try to lay her down. She lays in my arms, she will hold her head up for a few seconds but exhausted she puts it down after a couple of tries. She seems to have her eyes closed and when I open them they make very strange movements, much like a typewriter key hitting the paper, from left to right and then back again. She no longer has a true cry but rather a whimpering, moaning heart wrenching little sob. With her left hand she tries to hit her head and pull out her hair. Her right side is much like a rag doll. On occasion today I saw her leg kick or a tiny finger move. For a few brief seconds we saw the familiar smile flash across her face and then it was like someone pulled the curtains as she went back into her own little world. During her bath and the exercises we do with her, she smiled, kicked her tiny leg and melted my heart. How is she doing? I honestly don't know, her heartrate and 02 levels are stable but for improvement there has been no signs of her returning to herself. Corey on the other hand is okay. For the last couple of days he hasn't been himself, Sometimes I think he is so intuned to Emmy that whatever is going on with her affects him as well. Today he is back to "talking", not words of course, but his constant little noises and giggles and going about his usual little quirks, turning off her mobile, turning on Kenny Chesney music, pulling the cats tail, wanting to play with glue, etc. He has lost more weight so we are trying to sneak in a few extra calories overnight but everytime I try to increase his feedings he wakes up gagging and choking. We are in the middle of a big snowstorm, well the biggest one we have gotten all year. We have had very little snow this year so the kids are excited. Here I am looking forward to spring and not wet muddy boots and clothes Please say a prayer for Emmy, she is in God's hands. Each time I look at her I am sad but that sadness is quickly replaced by joy as I realize how incredibly lucky I am to have her with me when there are so many of our friends that have lost their little ones lately. UPDATE: 2-22-07 Emmy did have a stroke, right now it's hard to know what long lasting affects it will have on her. There is a huge difference between her now and a couple of weeks ago. Her eyes are glazed over, she doesn't seem to be aware of what is going on around her, doesn't smile and spends much of her time struggling to get her hands behind her head. It has also affected her right side. Corey is doing okay and has been having mainly good days. We are in the process of increasing meds as he has become alot more agressive the past week towards others and himself. Please say a prayer for Emmy as I am extremely worried about her. Margo UPDATE: 2-17-07 Sorry for the lack of updates. It is just one of those weeks that I have no energy and am just trying to deal with the reality of life. Corey was sick thruout the weekend. It turned out he had strep and pinkeye. He still doesn't seem to be back to himself. He has lost 3 1/2 pounds since October. Both kids had their neurology appts on Tuesday. Overall the doctor is pleased with Corey and how he is doing. He had quite a few small seizures during the appt so his meds have been increased again. He wasn't so pleased with Emmy but was happy that she wasn't screaming nonstop and will smile on occasion. Her seizure meds were doubled and her valium was raised as well. Corey and Emmy both had bloodwork done. It was a white blood cell enzyme check. It is used to check for Neimann Pick and some other metabolic defects. The preliminary tests are showing "metabolic defects of energy". We will need to wait quite awhile for actual results of specific diseases. It really doesn't matter as none of them have any real cure or treatment. There is some suspicion that Emmy has had a stroke, probably sometime Tuesday night following a major seizure. She can no longer hold her head up well, falls forward and her one leg seems very weak. Once again nothing can be done but pray that her strength returns. She has been really "out of it" and is really floppy with tons of seizures. I am having a hard time just dealing with things and trying my best not to worry about the 3 oldest kids testing. There odds of developing muscular dystrophy or being a carrier are very high. It has been confirmed that my ex husband does indeed have myotonic muscular dystrophy and he has a large number of repeats. Oh Emma's surgery had to be canceled indefinetely, she needs to be much stronger. So far the gj tube has stayed in place. Next week we have a couple of appts in Minneapolis, just routine stuff plus a quick ct of Emmy's brain. Take care and have a wonderful weekend! Margo UPDATE: 2-12-07 Corey is very sick. He has been running a temp, has a very runny and stuffy nose, is not tolerating any feedings and has been basically unresponsive for the past 36 hours. Even when he appears to be awake he just stares and is drooling like crazy. I am pretty sure he has the flu but if he's not better by morning I will be taking him to the clinic. I hate to expose him to all the germs at the clinic just to be told he has the flu or some other virus. We spent hours and hours in the ER at Minneapolis Children's on Friday. Emmy's consult for surgery will not be until next Thursday. I just can't seem to get anyone to understand that any food in her tummy makes her miserable and triggers seizures. I had the er nurse give her 5 mls, which is 1 teaspoon of formula into her g-tube. She was very surprised at how Emmy reacted to it. She called the gi that decided to give the go ahead to put in another gj tube. They were hesitant to do it at the er since she still had a g-tube in place. One try and it was in. Last time it took an hour and half, Emmy was bleedily heavily and very upset. This time she didn't have time to cry it went so fast, less than 5 minutes. Right now the plan is for her to have a permanent seperate j tube placed with a possible nissen done. They aren't sure about the nissen because of a defect in her stomach. She is continuing to aspirate on bile which is causing further lung damage. She also got a very expensive seating unit, which she hates and screams whenever she is put in it. Hopefully she like the other equipment that she is getting better. On Tuesday both kids have very early Neurology appts in Minneapolis followed by the bloodwork and skin stuff that is needed to check them for Niemann Pick disease and pyruvate something or another disease. On Thursday we are heading back to Minneapolis for the surgery consult. Please pray for Corey to get well soon, for me to make the best decisions in regard to Emmy's surgery and for us to travel safely. That's about it for us! I have cookie dough to make as I promised my kids I'd make them valentine cookies. Lot's of fun but messy! Frosting, cookie cutters and sugar sprinkles everywhere! Margo UPDATE: 2-9-07 Emmy J tube has migrated back into her stomach again. We have had this gj tube for 7 weeks and this is the 3rd time we have had problems with it. After discussing it with our pediatrician and our GI doc it has been decided she will have surgery asap to have a seperate j tube surgically placed. This surgery is riskier than a g tube with alot more long term affects but it is our only option. We have tried longer gj tubes but it still only gets us a few extra days. Last time they stitched in place but with all her retching it has still moved. I know that we will never be rid of her dependency on her feeding tube so I am feeling okay about it. Last night she had to be fed thru her g-tube. Even small amounts of food in her tummy causes her such pain, she screams and arches at a tsp. This in turn causes her seizures to increase. Sadly her meds which were given 12 hours earlier were still in her tummy. This morning the dye they used yesterday is still there, the dye was used early Thursday morning. Her stomach is absorbing or moving the food along it is sitting in her tummy fermenting. THe downside of having a seperate j tube are they take a long time to heal, problems often arise following surgery,she will have another tube button on her tummy, she is at a huge risk for anesthesia. The positive side is once it heals it is there for the long term, I can change the tube myself at home, it will always be in the right spot. She has another aspiration pneumonia from the tube being out of place, the motility in her stomach isn't there so this right now is our only option. I am waiting for all the docs to cooridnate schedules. The person doing the scheduling for the surgeon thought March would work. I was like ummmm what are we suppose to do until then, let her starve? If things aren't scheduled today for early next week, Emmy will be at Children's ER to have another gj tube placed today. I hate to have her suffer from the pain that food causes her. I call that ER the ER from hell, last time we waited 16 hours to have her tube replaced but we do whatever we need to do. Corey on the other hand is doing very well. He will be staying with Jenne today so I don't have to bring him with me. Hospitals just terrify him so much! I can be assured that he is having the best of care. From a little boy that didn't want to be out of my sight he has come a long way. He is always willing to go with her. Please pray that surgery is scheduled soon, things go well and that our appt today goes well too. It is very cold out and I am always nervous about taking them out in this type of weather. Margo UPDATE: 2-8-07 Emmy has been approved for additional hours of homecare! Now for the best news of all! Corey's nurse at school, Jenne, has taken the job! She will be helping out after school. I am so relieved. As many of you know I have a very hard time leaving my kids with anyone I do not know and trust. I also do not want Emmy and Corey to have a constant change in caregivers. I know have the best, someone I know and trust plus someone that has experience with all of Emmy's needs. She is so familiar with all of Corey's needs and Emmy's are indentical. Emmy already knows and loves to be held by her so it will be a very easy transition for all of us. Corey is having a good day. He is very tired this evening but he had one very busy and full day! I love to see him doing so well and having time to enjoy life! Emmy has been tired and having alot of little seizures today but for the most part is doing. She is maintaining her stats so all in all it has been a good day. I am continuing to get more info concerning testing for muscular dystrophy for the 3 oldest kids. I am waiting for the rest of the paperwork Once we know the number of repeats and which chromosome of my exhusbands is affected the testing will be fairly simple. I have explained this to them as best as I can but they are still scared as I am. Most of the docs I have been in contact with assume it's about Corey and Emmy and are saddened to hear of another disease impacting my kids. I keep telling myself to trust in God and everything will be okay. Simple words to say but so much harder to have faith. Right now it is hard for all of us to see what God's plan is! Thank you for all the prayers and emails, they mean the world to us! We will be leaving early on Friday with Miss Emmy. We will picking up her new seating thing and looking at some other adaptive equipment for her. Margo UPDATE: 2-6-07 I really don't know quite how to say this but some of my kids have been dealt another blow. Some of you know this and some of you don't, my 3 oldest children aren't Russ's bio kids. Today my ex-husband called and told me that the 3 kids need to be seen asap by his neurologist. He has been positively diagnosed as having adult onset myotonic muscular dystrophy. I remember Corey being tested for this, so how strange it is to be a part of my life again. His neuro is particularly worried about Mikayla. She has severe vision issues and some other minor issues which might mean that she may have teen onset of this disease. My other 2 children need to be tested to see if they are carriers. Fathers with this disease will only pass on teen or adult onset but if a mother passes it on it will be infantile myotonic dystrophy. It just seems crazy that my kids could have another disease. Of course this doesn't affect Emmy and Corey as they are not his children. Corey and Emmy's neuro will be checking my other kids for this. All he could say was I'm so sorry but the incidence of them developing this or being a carrier is high, 75hance they will get it, already have it or be a carrier of it. Right now it just seems like a really bad nightmare. It hasn't really sank in that my "healthy" kids could be far sicker than I ever imagined. Lexie, Liam, Mikayla, Evan and Emma are all sick. Alyssa is getting sick this evening. They all have the flu, Liam has been running a temp since Thursday and looks the sickest I have ever seen him. Corey is just being himself, he is getting more and more silly each day! Outside of some seizures this weekend he has been doing well. Emmy is back to holding her head, vomiting, running a temp and is having a ton of seizures. I am running on very little sleep. Please pray that I stay healthy. Pray for all of us as we deal with all too familiar testing. Also Corey is being tested for a metabolic disease brought to my attention by another parent. The preliminary testing of skin shows he has this inconjunction with the mito. I will not mention the name as I won't know for sure until his wbc enzyme testing comes back. Not that it really matters as this isn't treatable either. When and if Corey's results are proven positive by the other testing to verify the skin results, Emmy will then be tested as well. Right now all I want to do is pull a blanket over my head and sleep but with all the new worries I am unable to sleep. Margo UPDATE: 2-3-07 My day is getting worse and worse. I have no homecare today. Liam and Lexie are both home from school with strep throat. I just got a call from Evan's school-he needs to be picked up as he is running a high temp. and is complaining of a sore throat. I have a bad cold and am starting with a sore throat and temp. Emmy aspirated last night,her lungs are yucky sounding, she is now running a temp and her throat is all swollen. I am taking her to the clinic. So I will hoping the doc will just call in a prescription for Evan and I. I have a feeling this weekend will be a very long one! I have had basically no sleep for the past week which is not helping matters. Please pray that we all kick this bug and are feeling well by Monday. Margo UPDATE: 2-1-07 I'll start with the good news first. Corey is doing well. His nurse at school and her husband "borrowed" Corey for a few hours yesterday. He came home very happy but very tired. I always ask him how his day was but all he said was ne-ne and giggle. I think he was saying her name as it is Jenne. He also brought me home a beautiful rose and card that he picked out with her husband. It is hard for me to let anyone else take care of Corey but he is in good hands as she knows Corey so well and I think he enjoyed the break from me as well :) Our neuro called with an apology. Told me he should listen to my gut instinct as I know my kids so much better than anyone else. I guess we can all have bad days. We will still be seeing a new neuro at Mayo. Corey is being tested for a disease that was brought to my attention by another mother. Even thou our doc hadn't heard of it when he read about it he said Corey and Emmy both have every symptom. Mayo was surprised with the amount of testing Corey had done he hadn't been tested for this. They told me that Corey had been tested for more than a thousand different diseases/syndromes. Corey will be having his blooddraw for this soon. He also has viable skin and muscle tissue at Mayo yet that will make his test quite simple. Emmy will be tested later on. Miss Emmy had her valium and seizure meds raised again. For the first hour she seems okay but then goes back to crying and holding her head. Her seizures are getting worse as is her constant refluxing on saliva. We are getting Emmy's homecare hours as well as Corey's reevaluated. Please pray that we can get some additonal hours of care. Also pray that if we get extra help that we can find just the right person to take the job. We live out in the country and it has been hard to find someone to take care of them as it doesn't pay that well. Emmy is on her 4th day with only a couple of hours of sleep per day. She just can't find peace and relax enough to sleep. We have tried all sort of meds and combination of meds. We are now trying her on aromatherapy. If anyone has any other ideas please let me know, I am willing to try almost anything to give her some rest! Margo UPDATE: 1-30-07 Corey had a nasty seizure this weekend followed by a sle. Thankfully he is better today. We can only assume that his atrophy is resuming. Hopefully we won't see another one. I am very upset with our neuro-he has basically dropped Corey and Emmy as patients. I had asked about further testing, I wanted to try different meds, etc as their seizures are so out of control. I asked for a referrel for a second opinion and his entire attitude changed. We have been with this person since Corey was 9 months old. I have heard him doing this to others but it is heartbreaking to us. I had thought he cared deeply what happened to my kids. Emmy is doing okay. Outside of the usual seizures, feeding difficulties things are going well. Gillette's called today, last Tuesday we chose a chair for her and already today they called that we can pick it up in early Feb. as the insurance approved it and paid for it. I never dreamt they would cover it, instead I thought they would say they just paid for a wheelchair and other equipment. Approved immediately along with a letter stating if she needs any other adaptive equipment to contact this person and she would approve it immediately. We were told it would take 2-3 months to get it denied and then we'd have to file an appeal. Hmmm this is the same insurance company that refused a 35 cent syringe last week. This week we have no out of town appts, next month starts a whole new onslaught thou. Enjoy your week! Margo UPDATE: 1-26-07 Why does one appt. always lead to 10 more???? I will start with Corey as he had very encouraging appts :) For the first time his vision has stabilized. No change in 6 months!!!!!!!! Yes, he is considered and labeled legally blind but for those of you that know him he still sees and has adapted to using his sense of touch quite well. We still have no answer to his ongoing gunky-mattery eye problems thou. So far his optic nerves, retina's etc have atrophied so far and stopped. Why? No one but God knows. We have the results of Corey's new brain scan. No new atrophy for a few months now, thank GOD!!!!!!!! We discussed how little progress Corey has made, both physically and mentally. I think the neuro was warning me but all I could think was yahoo and thank God, he said barring anymore strokes or new atrophy I think Corey mentally will stay at a 12-18 month range and physically a little below that. I doubt with the amount of atrophy he will ever talk, or communicate, write his name, etc. All I could think was we'll take whatever God hands us. When I mentioned this to the other kids one of them said, Corey is sure lucky-no matter how big or old he gets, he'll never have to grow up. When I got to thinking about this-I had to agree-wouldn't be all like to be that age forever, how many worries or concerns did we have at that age? Then he went on to call Corey the miracle boy. Emmy'a appts didn't go as well:( Her optic nerves are pale (the start of the mito affecting them). She still sees but has been diagnosed with opsoclonus as well. Structually she should see okay but he told me neurologically it is all her brains fault when we see her strange eye movements, failure to reach what she is trying to get, etc. That means there is nothing we can do will help her vision. He noticed the closing of her eye, the mouth movements, hand flapping as well and immediately called our neuro of the significant seizure activity he was observing. Emmy also has a seating eval. They will be casting her body, and forming a gel like insert for her wheelchair, customized to her own little self. She also will be getting a surefit seat for in the house. We have also been referred to an ot/pt to see about other equipment they feel would benefit her. The one piece I will be excited to get it a versa form pillow that will have heat, massage, heartbeat etc imbedded in it. If only I was rich there would be a ton of stuff I'd like. Fortunately with the help of insurance we can afford a few of the pieces. Emmy is going to be started on a new pain med. Also a few other things such as aroma therapy, etc. For once we have seen someone that seemed shocked by Emmy's seizure activity and pain she appears to be in. Her brain scan is not good. She is showing atrophy at a much more rapid pace than Corey and also in places where Corey's hasn't atrophied. We really do not have a prognosis but based on the scan if her brain continues down the same path it is on they said less than 2-3 months. Right now our only concern is for her comfort and making each day the best it can be. Her doctors want all her baselines done again so I think over the next few weeks we will gone at appts more than we are home. Right now appts are be coordinated so I'm not driving back and forth constantly. Right now the increase in valium and the new pain aren't working, the first hour is okay but after that she goes back to crying and holding her head. Please pray that we find the right combo of meds for her! Margo UPDATE: 1-23-07 We will be leaving early this morning. After speaking with Emmy's neuro and him hearing how she is doing we will be seen. He has also increased her valium by alot every 3 hours and has added another med. I won't mention the name as you will jump to the same conclusion that I did, it is a drug that is routinely used to sedate people in nursing homes with a great deal of controversy surrounding it. But for Emmy it allowed her to have 4 hours of sleep that was free of restlessness and I am praying painfree as well. There are a ton of side affects but God has taken care of her stats overnight. Right now our only cocern for her is her comfort. I have people asking, aren't you afraid she is becoming addicted to all those drugs, etc. Yes she probably is but it really doesn't matter what matters is her comfort. I will not be updating until Thursday unless something changes. I will have my cell with me but depending on where we are just leave me a message. Margo UPDATE: 1-22-07 Emmy is in need of your prayers. Early on Saturday she started holding her head and crying nonstop again. It's Monday morning and things are continuing to go downhill. She is unable to tolerate any feedings, each time I start a slow drip feed her pain seems to increase as does her seizures. Her stats are awful, low 02 levels along with erratic heartrate. She is unable to clear any secretions and has basically given up trying to cough or will try but just doesn't have the strength. I have done everything I can possible think of with no improvement. Her doctor has increased her valium along with pain meds but she still can't find any peace to sleep. She and I are on day 3 without more than a couple minutes of sleep at a time. With the amount of pain meds and valium she is on, I can't imagine the amount of pain she must have for it to override this amount. I haven't been praying for her earthly healing for she seems so tired today of life and all it's struggles. Please just pray for peace, sleep and comfort for her. Corey is back to himself, medically speaking but is very subdued and has an anxious look about him. How he senses when Emmy is not doing well! He may not talk but the compassion he has for her, the care and concern shows all over him by his demeanor and actions. I will update when I can or if something changes. Right now I plan on taking her to see the pain and sleep specialist. We will also be planning a plan of action this morning with all her other doctors. I also have a metting today with public health, I am trying to get Emmy additional hours of nursing care. The few that she gets per week aren't enough. I would like her to get at least a few hour stretch each day. Please pray for Baby Donovan's family. He earned his wings this weekend. I am sure all prayers for his family and brother would be greatly appreciated. Margo UPDATE: 1-20-07 Corey has had tons of small seizures today followed by a couple of grand mal seizures this evening. We had to use diastat as they were lasting so long. He seems to be doing better now. I am sure the increase has been due to the cold and cough he has. It seems odd for we have just increased his seizure meds by 50 mg a day. He also hasn't been able to eat anything all day. He is gagging and vomiting this evening. He has been so well lately and I tend to dwell on today and not worry about tomorrow that a few good days can make me forget how quickly things can change. Emmy seems to be doing a little better today. Still struggling to cough but we have seen a couple of smiles which is a huge improvement over the last week. We finally got her stats up after finding the what was causing it-another mucous plug. Once we were able to get it removed her 02 level and heart rate returned to normal for her. She still isn't tolerating her full feedings but she is doing better with that as well. On Tuesday we will be heading to Minneapolis. Gillette's had a cancellation with a doctor we have been waiting to see (our appt was for July!). So since Emmy was a priority it was offered to us :) Corey and Emmy both have eye appts on Wednesday so our plan right now is to leave Tuesday morning and return Wednesday evening. We will be having her seating eval as well as seeing a sleep and pain specialist. We just want to make sure that we are doing for the very best for her in regards to pain management. I have stepped on some of our current docs toes but I really don't care. Emmy is just too important not to explore all our options. Other than that absolutely nothing has been going on around here, just staying home and trying to get everyone healthy! Margo UPDATE: 1-17-07 We were back at the doctors office with Emmy yesterday as she is not doing well at all. She is still sick and no matter what we do her 02 stats will not go above 80-82. She looks blue and has stopped crying, just moans. It is a constant stream of nebs, meds, suctioning and then starting all over again. Please pray for her! Before you send me a nasty email or sign something equally nasty in the guestbook about her being at home, think about it. For those of you with a child like Emmy you understand, a hospital poses risks in itself and we have everything the hospital does. She has 02, a pulse ox, apnea monitor, suction, ambubag, etc all here at home. For those of you that can't relate-be happy you can't! There is nothing we can do further for her in the hospital. We also have nursing care as well as doctor visits. I'm just tired of people always questioning our care of Emmy and we have the complete support of her medical team. Emmy's seizure meds and pain meds were raised yesterday,we are trying to have more of an impact on seizure control other than using diastat and trying to keep her comfortable as she continues to fight so hard. Her heart rate is also more erractic with several times per hour of vtach. I haven't even allowed myself to think of the whatifs this time for our princess, we are just too intent on what needs to be done. Corey has a bad cough and cold as well, other than being very sleepy and out of it for most of the day, he is doing well, stats are good and he is tolerating all his feedings as usual. He has a few really good hours each day where he is up and playing but quickly becomes tired. Our homecare nurse made a comment of how much they have changed since summer-I had warned her that the winter months are hard but as you other mito parents understand, it is something you have to see for yourself! We have lost our third insurance appeal for a handheld pulse oximeter. I am trying to get one just to cut down on the equipment we need to lug around. This is just Emmy-you have one small girl, a pulse ox(size of a small dvd player), an apnea monitor, 02, suction machine, feeding pump, diaper bag and neb machine, it gets very heavy. You can't have the wheelchair hold all the weight as it tips it over top it with Corey and the same equipment, you get the picture! Our insurance company will noy pay as they have already paid for the large ones. The new ones with a pediatric probe (not a finger one) costs approximately $700, it's just not in the budget. PLEASE if anyone has one they are no longer using and would like to sell reasonable please contact me as we would love to buy one. Just make sure it is compatible with an infant probe. Margo UPDATE: 1-15-07 Everything went fine with Emmy on Friday. We are hoping that with this being done her feet,legs and hips will remain as what the docs call "useable". She doesn't seem to mind the leg braces. She also received a pink molded helmet, which we are planning on having airbrushed with princess Emmy on it. She was also evaluated for some really cool equipment. A company is donating the technology to help out as this will be customized just for her. She will be receiving a molded, contoured to fit her board, kind of like a scooter board but it will be heated,massage,will rock like a boat on a lake as well as have a human heartbeat embedded into it. It will also have different switches hopefully to entice her in learning to activate a switch to get what she wants. She is also getting what will resemble a bumbo chair but larger custom molded chair with a tray, a molded sitting chair similar to a beanbag and stander. I was so pleased to hear that a company cared enough to get involved, our dev ped and physiatrist said there is nothing on the market so needed to improvise for Miss Emmy. Also thank God our insurance company approved this stuff and will pay 100f all costs. Emmy has been running a fever since Friday night, turns out she had a virus which led to vomiting which in turn caused her to aspirate, another pneumonia :( We kept her g tube vented but still she managed to aspirate. She doesn't look good today and her 02 stats remain in the low 80's no matter what we do, other than neb treatments, starting steroids and antibiotics as well as oxygen there is nothing else to do. She has kind of a bluish, gray look about her. Corey has caught the same virus and is throwing up too. He is running a temp but doesn't seem to be bothered too much by it. He has a increase in seizures, crankier than usual but is still being silly. I'm sure by the end of the week the rest of us will have it as well! I was expecting it as we have been spending too much time in hospitals and clinics! Emmy has a couple of appts this week, next week are both kids eye appts. Other than that it has been super quiet around here! UPDATE: 1-11-07 Happy Birthday Lil' Princess, My sweet Emmy, I can't believe that you will be one tomorrow. It seems like just a moment ago I was holding you for the first time. I have loved from the minute I knew that you were growing inside of me. When you were born I tried so hard to push the thoughts away that something was terribly wrong. Your doctors all told me that you were perfect and doing so well for being born 5 weeks early. Deep down in my heart I knew that you were another angel sent by God. I looked so deep in your eyes and I saw you look back with what I called Corey's wise old look and just knew what you were going to have to endure. Then when you were only 6 weeks old and everyone else agreed that there was something wrong. From the docs that thought you were just failure to thrive to the ones that thought you probably had your own syndrome and not for us to worry. Then came the day that I really didn't need for I already knew that thru genetics you too would have Leigh's and the undiagnosed neuromuscular disease that I knew would cause you to suffer so much. I love the bond that you share with Corey. Your brothers and sisters love you so much Emmy and it breaks all of our hearts to watch you struggle each day. You are the light of our lives, your smile melts our hearts. I doubt if you know how many other people love you and how much you have touched the world sweetheart. As I watch you struggle each day, I think how unfair it is. You hear Mommy whining, I know. I admire your courage for each day you fight but yet you manage to enjoy each day, you live for today. Is it because you already know what your life will lead to? You have this look as if you know a secret the rest of us don't. Does God let you in on all of Heaven's secrets when your struggles are the hardest? As your Mommy I should be planning your future, dance classes, playdates, etc. But so often I find myself planning the celebration of your life instead. No, I don't do funerals, for I know that while we will miss you so much being with us, we will be celebrating your eternal life and forever grateful to God for having chosen us to be your parents and family. Princess Emmy, I promise to fight for you no matter how many doctors I need to trample across to get the care you need and deserve. I also promise when you let me know that we have done enough and that you tired of fighting that I will if at possible keep you with us at home. When you are too tired to fight anymore princess it is okay for you to stop, for one day I promise that we will be together again. Emma I love you with all my heart and soul, with every breath I take and you will always be my little princess. I'll love you for always, Mommy UPDATE: 1-10-07 How come each time I feel like I am handling things so well, I receive some little bit of news that just comes as a big slap in the face? I have had several people ask why I really don't let things out that say how bad things really are, why I try to keep things upbeat on this website. The reason? No one unless they are in the same place can truly get it and who wants read negatives all the time? I know Emmy sin't doing well but then 2 things happened that really were hard for me to take. They are all things I know but still. The first is a letter from the orthopedic surgeon, the type they send following an appt. It stated-Emmy is a very small for age child with severe neurological and developmental delays. I was overwelmed by the overall decline in health in a few short weeks. Her seizures appear to be severe, nonstop thru out our appt. It then goes on to outline the things he will be doing for her ortho-issues etc. The second was that Emmy had a basic checkup yesterday. It was so hard as there were so many other little ones there all for a one year check. Walking, talking, drinking out of sippy cups, etc. Thankfully our ped is always good about getting us back right away but I hate the stares, etc. I did have to giggle as one woman said to her husband after observing Emmy's feeding tube and said to him, oh look the poor baby needs oxygen. Then our ped noticed Emmy's constant seizure activity and mentioned how many skills she has lost. Noticed that Emma's eyes are getting that vacant look and that her pupils weren't working right, etc. Of course these are things I already knew but it still hurts. Coreyneeds to go in for bloodwork very early. This should be fun as he will be screaming the moment we walk thru the door. His veins are badly scarred and having blood drawn is always a big ordeal. His meds are being tweaked so we need some levels from him. He is just his usual self, and has been doing really well so far this winter. It is nice to have him doing so well. I am constantly telling him that mom can only handle one extra sick one at a time, he just laughs and goes about his business. Everything is set in place for Emmy on Friday, hopefully this will take care of her feet/ankle/knee and hip issues. If all goes well we should be home late afternoon. Please keep Emmy in your prayers, Margo UPDATE: 1-8-07 Corey is doing well, only the regular seizures, etc. He has lost a few pounds so we are working on getting him to gain some weight. We are adding 5 ccs an hour and will increase as he tolerates until we see him gaining. I can't figure out how one teaspoon extra could make him miserable but it does. He told me today-uppity, uppity, uppity. I couldn't figure out what the heck he meant until he started playing with the zipper to his pj's. Apparently I hadn't put the zipper up far enough for when I moved it up the 1/2 inch he was all smiles. He is just so funny sometimes! Emmy was casted on Friday and will be getting her braces on the 12th, along with the rest of her feet/ ankle stuff done. Fortunately it all sounds very simple. The ortho told us that luckily we are treating her this early, for if we waited another 6 months it would be much more complicated. There is also something wrong with the muscle that runs from the inside of her foot to the hip area, this too can be corrected. There is a chance that the simple procedure won't work and if this is the case she will need more extensive surgery later on. Braces in exchange for feet, legs and hips are well worth it. We will also be seeing a devopmental ped and physiatrist on Friday. She will also need to be fitted for a stander. Friday also happens to be the lil' princess's first birthday. Hard to believe that she will be one already, the time has gone by so quickly! We were looking at the outfit that she wore home, hard to imagine her ever fitting into it! While the past year has been a constant struggle, each moment has been so precious to each of us. We will be celebrating her birthday the following weekend as I doubt she (or I) will have the energy this weekend! Margo UPDATE: 1-4-07 Corey is doing okay. The winter months are hard for him. The last couple of days he has been having "mito" days, hard to explain except to another parent of a child with mito. He has lost some weight and each ounce he gains is a huge struggle. We can't pinpoint a cause for his weightloss but have decided to try adding extra feeds. I still can't figure out how he knows if you try to sneak in an extra half ounce but he usually ends up vomiting the entire feeding. Corey's behavorial issues are on the increase again, biting, hitting, spitting and pulling hair to himself and others. We are trying to tweak a few meds to see if that helps. He also managed on Monday to cut a huge chunk out of Mikayla's hair. With a pair of all plastic scissors that came with a coloring book. I couldn't cut a thing with them if I wanted to. So Mikayla ended up getting her hair cut to just above her shoulders. Emmy started having tons more seizures and ones that are lasting 5-10 minutes. Her eyes also seem to be hurting/bothering her. Sadly there is nothing we can do as she is losing her vision rapidly and the doctor thinks that is what is bothering them. Our doc received a report from the craniofacial doc and was none to pleased and wants us to see another one. Basically it said Emmy even if she does regain her strength shouldn't be treated. We have run into other doctors before that feel terminally ill children shouldn't be treated but it's still hard as a parent to realize that there are others that feel our kids aren't worth medical treatment. Tomorrow we have another day of appts with Emmy. Please pray for our safety and for the new doctors that we'll be seeing. Margo UPDATE: 1-1-07 Emma's appts went okay. In regards to her craniosynosis-well nothing will be done at this time. We were told it has nothing to do with her issues or the the increase of seizures. The docs are concerned about her soft spot in the front, it is still quite large and not closing but for the time being all this can just be watched. We also received a referrel to a new neurologist from John Hopkins, we just want a second opinion to make sure we are on doing the best we can for Emmy. We have other opinions from other neuros in Minneapolis but frankly none of them want to step on a colleagues toes, all we ever hear is you have the best ped. neuro around. So we will be seeing a neruologist that specializes in central nervous disorders and uncontrolled seizures. Next Friday we head back to Minneapolis to get the final decision on Emma's feet. By then all the scans will have been read and we will know exactly what type and how extensive the surgery will be. Other than that she is recovering nicely from her strep and pneumonia. We have upped her valium and she is now on continuous pain med along with tylenol for break thru pain. She is actually smiling and just seems more comfortable although a large portion of her day is still spent crying and her holding her head and pulling at her hair and face. She also has gained a couple of ounces of weight back. A couple of her meds for her nausea were upped and that along with the g-tube venting is working. We only have a couple of episodes of throwing up each day. I am optimistic once again that she is gradually getting better. Her eeg (the 24 hr one) was not good, tons of seizure activity each hour. She has been doing this thing where she closes her eyes and her mouth starts to quiver and droop, Liam calls it her smushy face, turns out that it is seizure, so are her high pitched squeals that we once thought was her way of showing happiness, seizures too :( All around things are getting better as we are learning to adjust to new meds and have come to face the reality that her crying is our new normal. Corey is over his strep and has been doing well. Today his nurse from school took him for a few hours, he was so excited when I told him she was coming. Alyssa turned 19 on Thursday, I feel so old! Margo ADDED: 1-07-04 We have been told that there is no medical treatment for Corey and that he is very unlikely to live past his 3rd birthday. I have been told at Mayo that it is a miracle that Corey was born alive and that he is still alive. I also want you to know how much joy Corey has brought into our lives, after 6 healthy children he has brought an appreciation for the small things in life that I have never realized. I thank God everyday for the privelidge of being Corey's Mom. Corey is part of Mayo's research on metabolic diseases, there will be no cure for Corey but I am hoping through him other children may someday be cured. I hear often how can such a beautiful child be so ill, unlike many childhood diseases there is no set pattern as there are no other children with the same disease Corey has, he has a rare mutated form of this disease as well as so many other chromosome errors that Mayo has never seen or heard of another child like Corey so they really have no knowledge of the future, we only thank God for each extra day we have with this precious little guy. Condition: Mitochrondrial Myopathy, 21 inborn errors of metabolism, chromosome mosaicism, undiagnosed neuromuscular disease that is causing atrophy throughout his brain, blind, deaf.