Guest guest Posted December 29, 2004 Report Share Posted December 29, 2004 , This is all very scary. My kids have mito and unspecified FODs too. FOD network and UMDF.org are your best resources. When you have specific questions, concerns, or needs please feel free to ask them here. Everyone here is very helpful and knowledgeable.Good luck. Sometimes it just helps to know you are not alone, and believe me, you are not! Dawn Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 29, 2004 Report Share Posted December 29, 2004 Hi , My name is and I have not posted here in a very long time. I was actually planning a reintroduction and an update on my daughter. My little girl, Miranda, just turned 8 on the 27th of this month. She was also on TPN for 14 months. She has complex 4 diagnosed by a muscle biopsy. I am not sure what you are referring to but I do know the Dr's were very urgent about wanting to get Miranda off of TPN for several reasons. One thing, it is very, very hard on the liver since it has to be processed through there or something like that. My daughter also has a serious problem with infections and she had several life threatening bouts with sepsis at her broviac/ mediport site. I am sorry I can not offer you more information and if there are any questions I might be able to help with, please feel free to email me. Also, I am wondering why your daughter is on TPN at this point? Also, where are you located. -- Can anyone help me? I have posted here before. My daughter is Kierra, she is 9 years old. We got her biospies back last week. She has Scadd and Mito. They did not tell me a complex, just that it was in the respitory chain. They want to ween her off of tpn due to something that showed up in her CT scan. I have no idea what all this means. The genetic doctor tried to explain this to me, but I am lost. They made it sound like Kierra could die from this. Because one breaks down her energy and she can't absorb or break down fats and sugars. She has had 2 crisises that landed her in the hospital. I didn't even know that she was in crisis. I would love any help you could offer. I tried to research but I could not understand. They told me to join fod support group, but I have not really found out much there either. I thank you for your help.Kierra 9 years old Tpn through mediport, gp,dysmotility disorder,gerd,scadd, mito, chronic constipation, clotting problems, low body temp, temp. intolerencePlease contact mito-owner with any problems or questions. Quote Link to comment Share on other sites More sharing options...
Guest guest Posted December 29, 2004 Report Share Posted December 29, 2004 I can't help you much but I'd ask the docs if they found a problem in the liver. TPN is hard on the liver and can cause problems. The thing about mito is that it is deadly. That is one of the very difficult things that we are all dealing with-- our mortality and that of our children. Sorry that I could not really help. Have you checked out the umdf site? Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 6, 2005 Report Share Posted January 6, 2005 , I may have this wrong since I've had two kids with evidently two different Complex defects, but the way I'v always understood it is that anything from the respiratory (=damage) chain is usually a complex deficiency. There are five Complex'. At one time, we were gonna try tpn with but the doc's ended up saying that if she had the same thing as her brother , then it could cause all kinds of side effects that would progress the disorder at an even faster rate. Go to NIH or Pubmed's websites and look under Myopathies, then try to find something with the key words Complex Deficiencies and/or Mitochondrial Dysfunctions. NIh used to have alot of studies about the resp. chain defects involoving the Complex's. Hope this might help. Good luck, Angie concernforkierra wrote: I have posted here before. My daughter is Kierra, she is 9 years old. We got her biospies back last week. She has Scadd and Mito. They did not tell me a complex, just that it was in the respitory chain. They want to ween her off of tpn due to something that showed up in her CT scan. I have no idea what all this means. The genetic doctor tried to explain this to me, but I am lost. They made it sound like Kierra could die from this. Because one breaks down her energy and she can't absorb or break down fats and sugars. She has had 2 crisises that landed her in the hospital. I didn't even know that she was in crisis. I would love any help you could offer. I tried to research but I could not understand. They told me to join fod support group, but I have not really found out much there either. I thank you for your help.Kierra 9 years old Tpn through mediport, gp,dysmotility disorder,gerd,scadd, mito, chronic constipation, clotting problems, low body temp, temp. intolerencePlease contact mito-owner with any problems or questions. __________________________________________________ Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 I have posted here before about my daughter Kierra. Last week the last of her biopsies came back and she has all of the complexes of mito. in the resportory chain. And she has SCAD. Is there anyone in the group that has this? I mean I have heard of having more than one, but all of them. And with the fatty oxidation problem things are just a mess. She is one TPN and a feeding tube and is still loosing weight. She has lost 7 lbs in about 5 months. She is 9 and weighs about 51.5 lbs. I am lost and I so need help. My husband and I have a meeting with her genetic dr. on Thursday to discuss her prognosis. They told us that she could go blind, deaf and be in a wheel chair because of loosing muscle strength in her legs. I can't sleep, eat and I feel like I am going to go crazy. I have tried to read up on Mito. but I have not found anything that talks about what to expect with having all of the complexes. She has no mental or physical delays, she is very bright and in the 4th grade. She is now tutored at home. I thank you for your help. Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, tpn, feeding tube Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 I have posted here before about my daughter Kierra. Last week the last of her biopsies came back and she has all of the complexes of mito. in the resportory chain. And she has SCAD. Is there anyone in the group that has this? I mean I have heard of having more than one, but all of them. And with the fatty oxidation problem things are just a mess. She is one TPN and a feeding tube and is still loosing weight. She has lost 7 lbs in about 5 months. She is 9 and weighs about 51.5 lbs. I am lost and I so need help. My husband and I have a meeting with her genetic dr. on Thursday to discuss her prognosis. They told us that she could go blind, deaf and be in a wheel chair because of loosing muscle strength in her legs. I can't sleep, eat and I feel like I am going to go crazy. I have tried to read up on Mito. but I have not found anything that talks about what to expect with having all of the complexes. She has no mental or physical delays, she is very bright and in the 4th grade. She is now tutored at home. I thank you for your help. Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, tpn, feeding tube Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 ...Has Kierra been to see any of the mito specialists...Dr. Bruce Cohen (Cleveland Clinic) or Dr. Shoffner (in Atlanta)? They may be able to help. To be honest, this is the first I have heard of having all of the complexes...my daughter has complex IV. Also, www.umdf.org is a great resource. As for what to expect, no one knows with any of the complexes, which is the hardest part of being mixed up with this awful disease. I will pray for Kierra....this is a great place to come for support. I'm sorry I am really of no help... KK Mommy to Ellie, Leigh's, C-IV and my angel [Norton AntiSpam] Can anyone help me? I have posted here before about my daughter Kierra. Last week the last of her biopsies came back and she has all of the complexes of mito. in the resportory chain. And she has SCAD. Is there anyone in the group that has this? I mean I have heard of having more than one, but all of them. And with the fatty oxidation problem things are just a mess. She is one TPN and a feeding tube and is still loosing weight. She has lost 7 lbs in about 5 months. She is 9 and weighs about 51.5 lbs. I am lost and I so need help. My husband and I have a meeting with her genetic dr. on Thursday to discuss her prognosis. They told us that she could go blind, deaf and be in a wheel chair because of loosing muscle strength in her legs. I can't sleep, eat and I feel like I am going to go crazy. I have tried to read up on Mito. but I have not found anything that talks about what to expect with having all of the complexes. She has no mental or physical delays, she is very bright and in the 4th grade. She is now tutored at home. I thank you for your help.Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, tpn, feeding tubePlease contact mito-owner with any problems or questions. Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 ...Has Kierra been to see any of the mito specialists...Dr. Bruce Cohen (Cleveland Clinic) or Dr. Shoffner (in Atlanta)? They may be able to help. To be honest, this is the first I have heard of having all of the complexes...my daughter has complex IV. Also, www.umdf.org is a great resource. As for what to expect, no one knows with any of the complexes, which is the hardest part of being mixed up with this awful disease. I will pray for Kierra....this is a great place to come for support. I'm sorry I am really of no help... KK Mommy to Ellie, Leigh's, C-IV and my angel [Norton AntiSpam] Can anyone help me? I have posted here before about my daughter Kierra. Last week the last of her biopsies came back and she has all of the complexes of mito. in the resportory chain. And she has SCAD. Is there anyone in the group that has this? I mean I have heard of having more than one, but all of them. And with the fatty oxidation problem things are just a mess. She is one TPN and a feeding tube and is still loosing weight. She has lost 7 lbs in about 5 months. She is 9 and weighs about 51.5 lbs. I am lost and I so need help. My husband and I have a meeting with her genetic dr. on Thursday to discuss her prognosis. They told us that she could go blind, deaf and be in a wheel chair because of loosing muscle strength in her legs. I can't sleep, eat and I feel like I am going to go crazy. I have tried to read up on Mito. but I have not found anything that talks about what to expect with having all of the complexes. She has no mental or physical delays, she is very bright and in the 4th grade. She is now tutored at home. I thank you for your help.Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, tpn, feeding tubePlease contact mito-owner with any problems or questions. Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 I am sorry you are struggling with this new information. I hope we as a group can help support you through all these emotions. It can be scary learning that our child has something " rare " amongst the already rare Mitochondrial Disorder. It makes you really wonder why your child is different and how it happened. If possible, try to find some comfort in the knowledge of what is going on. We have struggled with our docs to find a certain type of Mito in our girls to no avail. One of the reasons our geneticist feels we got a non-specific form of Mito was because we were already treating them both with the cocktail before testing and supposedly it can change the outcome of the test. We wouldn't go back and change when we began the supplements for either of our girls as I believe they wouldn't have lived very long without it, but it really makes me wonder what the biopsy would have revealed if they hadn't been pretreated. Hopefully you will get some good information at your upcoming doctor's visit that will help. As one of the others mentioned, even when it may look bleak or scary, we don't know what tomorrow brings and can only enjoy our time with our kids today. When we learned Asenath had CNS Vasculitis (4 narrow brain arteries at this point) we were told there was only a handful of children in the world that they have found dealing with it as most of the time it is only seen in autopsies. If left untreated, it is usually fatal and even with treatment, the outcome is so unknown, the treatment is not easy or even the correct way to treat is so unknown. We didn't know if Asenath would be with us for only a short time from when we found out or if God would grant her with longer, but her prognosis has been looked upon as very bad. But, here we are a year and a half later still doing relatively well. I know tonight could hold the worst, but I also know we could have years. This really helps me to face the fears and move on. I hope this wasn't too long and hope it helps you at least a little. See www.caringbridge.org/ia/mitomomof9 and www.heartbeatsformito.org to see a photo look into what Mito looks like Darla: mommy to Asenath (4) Mito, CNS Vasculitis, strokes, migraines, seizures, G-tube, hypotonicity, disautonomy, SID, global delays, asthma, cyclic vomiting, bladder issues, wheelchair for distances, eye issues, autistic behaviors, gastric emptying issues... Zipporrah (14 months) Mito, strokes, neuro-motor planning dys., SID, GERD, dysphasia, 100% G-tube fed, speech delays, extreme fatigue, excessive phlegm, asthma, trach issues, aberrant subclavian artery, disautonomy, hypertonicity, migraines, possible seizures, dumping syndrome, iron deficiency, ... Luke (16), Leah (14), Rachael (13), Isaac (10), Tirzah (8), Kezia (4), & Marquis (3), Joey & (12 months) (some with Mito symptoms) Can anyone help me? > > > I have posted here before about my daughter Kierra. Last week the > last of her biopsies came back and she has all of the complexes of > mito. in the resportory chain. And she has SCAD. Is there anyone > in the group that has this? I mean I have heard of having more than > one, but all of them. And with the fatty oxidation problem things > are just a mess. She is one TPN and a feeding tube and is still > loosing weight. She has lost 7 lbs in about 5 months. She is 9 and > weighs about 51.5 lbs. I am lost and I so need help. My husband > and I have a meeting with her genetic dr. on Thursday to discuss her > prognosis. They told us that she could go blind, deaf and be in a > wheel chair because of loosing muscle strength in her legs. I can't > sleep, eat and I feel like I am going to go crazy. I have tried to > read up on Mito. but I have not found anything that talks about what > to expect with having all of the complexes. She has no mental or > physical delays, she is very bright and in the 4th grade. She is > now tutored at home. I thank you for your help. > > Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, > tpn, feeding tube > > > > > > > Please contact mito-owner with any problems or questions. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 8, 2005 Report Share Posted February 8, 2005 I am sorry you are struggling with this new information. I hope we as a group can help support you through all these emotions. It can be scary learning that our child has something " rare " amongst the already rare Mitochondrial Disorder. It makes you really wonder why your child is different and how it happened. If possible, try to find some comfort in the knowledge of what is going on. We have struggled with our docs to find a certain type of Mito in our girls to no avail. One of the reasons our geneticist feels we got a non-specific form of Mito was because we were already treating them both with the cocktail before testing and supposedly it can change the outcome of the test. We wouldn't go back and change when we began the supplements for either of our girls as I believe they wouldn't have lived very long without it, but it really makes me wonder what the biopsy would have revealed if they hadn't been pretreated. Hopefully you will get some good information at your upcoming doctor's visit that will help. As one of the others mentioned, even when it may look bleak or scary, we don't know what tomorrow brings and can only enjoy our time with our kids today. When we learned Asenath had CNS Vasculitis (4 narrow brain arteries at this point) we were told there was only a handful of children in the world that they have found dealing with it as most of the time it is only seen in autopsies. If left untreated, it is usually fatal and even with treatment, the outcome is so unknown, the treatment is not easy or even the correct way to treat is so unknown. We didn't know if Asenath would be with us for only a short time from when we found out or if God would grant her with longer, but her prognosis has been looked upon as very bad. But, here we are a year and a half later still doing relatively well. I know tonight could hold the worst, but I also know we could have years. This really helps me to face the fears and move on. I hope this wasn't too long and hope it helps you at least a little. See www.caringbridge.org/ia/mitomomof9 and www.heartbeatsformito.org to see a photo look into what Mito looks like Darla: mommy to Asenath (4) Mito, CNS Vasculitis, strokes, migraines, seizures, G-tube, hypotonicity, disautonomy, SID, global delays, asthma, cyclic vomiting, bladder issues, wheelchair for distances, eye issues, autistic behaviors, gastric emptying issues... Zipporrah (14 months) Mito, strokes, neuro-motor planning dys., SID, GERD, dysphasia, 100% G-tube fed, speech delays, extreme fatigue, excessive phlegm, asthma, trach issues, aberrant subclavian artery, disautonomy, hypertonicity, migraines, possible seizures, dumping syndrome, iron deficiency, ... Luke (16), Leah (14), Rachael (13), Isaac (10), Tirzah (8), Kezia (4), & Marquis (3), Joey & (12 months) (some with Mito symptoms) Can anyone help me? > > > I have posted here before about my daughter Kierra. Last week the > last of her biopsies came back and she has all of the complexes of > mito. in the resportory chain. And she has SCAD. Is there anyone > in the group that has this? I mean I have heard of having more than > one, but all of them. And with the fatty oxidation problem things > are just a mess. She is one TPN and a feeding tube and is still > loosing weight. She has lost 7 lbs in about 5 months. She is 9 and > weighs about 51.5 lbs. I am lost and I so need help. My husband > and I have a meeting with her genetic dr. on Thursday to discuss her > prognosis. They told us that she could go blind, deaf and be in a > wheel chair because of loosing muscle strength in her legs. I can't > sleep, eat and I feel like I am going to go crazy. I have tried to > read up on Mito. but I have not found anything that talks about what > to expect with having all of the complexes. She has no mental or > physical delays, she is very bright and in the 4th grade. She is > now tutored at home. I thank you for your help. > > Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, > tpn, feeding tube > > > > > > > Please contact mito-owner with any problems or questions. > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 9, 2005 Report Share Posted February 9, 2005 , My name is Ange and I have a daughter who is 5 she has Complex I-III plus FAOD and g-tube and seizures which effect her speech she weighs 33lbs and is happy. Dealing with all the disorders for me and my husband is hard at once if you treat the syptoms as they come it is much easier on everyone. I know that is what everyone says but the doctors can not give us a prognosis we live day by day as I am sure most who post here do. If the doctors are telling you she might be blind, deaf or in a wheel chair then look at her other strengths as we do in she is very development delayed but has such a great out going personality and is all about the fashion she can do a puzzule with no problem but can't recite her ABC's or count to 5. I know it may be hard but I have been dealing with this since was 2 and what I have found out is let them live life as normal as possible deal with one thing at a time and take each day and be happy. I am here if you need to talk or if there is anything help with. Kierra has strengths focus on those not the illiness and it will be easier for everyone I am not saying don't do anything still give her the meds, TPN, feeds and what ever else just everyday life for Kierra shouldn't be all about the illness. Hopefully this gave you some support. Ange -------------- Original message -------------- > > > I have posted here before about my daughter Kierra. Last week the > last of her biopsies came back and she has all of the complexes of > mito. in the resportory chain. And she has SCAD. Is there anyone > in the group that has this? I mean I have heard of having more than > one, but all of them. And with the fatty oxidation problem things > are just a mess. She is one TPN and a feeding tube and is still > loosing weight. She has lost 7 lbs in about 5 months. She is 9 and > weighs about 51.5 lbs. I am lost and I so need help. My husband > and I have a meeting with her genetic dr. on Thursday to discuss her > prognosis. They told us that she could go blind, deaf and be in a > wheel chair because of loosing muscle strength in her legs. I can't > sleep, eat and I feel like I am going to go crazy. I have tried to > read up on Mito. but I have not found anything that talks about what > to expect with having all of the complexes. She has no mental or > physical delays, she is very bright and in the 4th grade. She is > now tutored at home. I thank you for your help. > > Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, > tpn, feeding tube > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 9, 2005 Report Share Posted February 9, 2005 , My name is Ange and I have a daughter who is 5 she has Complex I-III plus FAOD and g-tube and seizures which effect her speech she weighs 33lbs and is happy. Dealing with all the disorders for me and my husband is hard at once if you treat the syptoms as they come it is much easier on everyone. I know that is what everyone says but the doctors can not give us a prognosis we live day by day as I am sure most who post here do. If the doctors are telling you she might be blind, deaf or in a wheel chair then look at her other strengths as we do in she is very development delayed but has such a great out going personality and is all about the fashion she can do a puzzule with no problem but can't recite her ABC's or count to 5. I know it may be hard but I have been dealing with this since was 2 and what I have found out is let them live life as normal as possible deal with one thing at a time and take each day and be happy. I am here if you need to talk or if there is anything help with. Kierra has strengths focus on those not the illiness and it will be easier for everyone I am not saying don't do anything still give her the meds, TPN, feeds and what ever else just everyday life for Kierra shouldn't be all about the illness. Hopefully this gave you some support. Ange -------------- Original message -------------- > > > I have posted here before about my daughter Kierra. Last week the > last of her biopsies came back and she has all of the complexes of > mito. in the resportory chain. And she has SCAD. Is there anyone > in the group that has this? I mean I have heard of having more than > one, but all of them. And with the fatty oxidation problem things > are just a mess. She is one TPN and a feeding tube and is still > loosing weight. She has lost 7 lbs in about 5 months. She is 9 and > weighs about 51.5 lbs. I am lost and I so need help. My husband > and I have a meeting with her genetic dr. on Thursday to discuss her > prognosis. They told us that she could go blind, deaf and be in a > wheel chair because of loosing muscle strength in her legs. I can't > sleep, eat and I feel like I am going to go crazy. I have tried to > read up on Mito. but I have not found anything that talks about what > to expect with having all of the complexes. She has no mental or > physical delays, she is very bright and in the 4th grade. She is > now tutored at home. I thank you for your help. > > Mother to Kierra 9 years old, mito.,SCAD, GP, dysmotility disorder, > tpn, feeding tube > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 9, 2005 Report Share Posted February 9, 2005 Grace is deficient in all but complex IV wich is at the very lowest it can be in the normal range (this from her muscle biopsy.) With her liver biopsy it was different with all the other chains still low but complex IV is actally 10 times the normal range (believed to be a compinsating effect.) Also 100% of her mitochondria are also physically mutated, all very large with very short cristae, all believed not to be functioning normally. In short, her pathology report was horrible. BUT, since starting the cocktail Grace is doing wonderfull. Her labs always to appear pretty much normal. Her liver is no longer killing itself, and is actually working completely normally. She is a relativly active 2 year old. Of course she has issues, and always will but nobody expects her to have a fatal outcome from this disease. We are unable to predict her outcome as far as needing a wheelchair or anything like that, frankly because nobody has ever seen a case like hers before. I guess my best advice is to take the report seriously, but remember some people respond outstanding to the cocktail. Listen to your genetecist, but don't expect them to give you a timeline of when she may go deaf or how old she will be when she will need a wheelchair (if she ever does). Most mito doctors will admit they still only know very little about this disease. Dr. Cohen always starts the appointments off with a smile, because nobody thought she would be doing as well as she is. I hope your child has the same great responce as Grace has. Find just what you're after with the new, more precise MSN Search - try it now! Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 9, 2005 Report Share Posted February 9, 2005 Grace is deficient in all but complex IV wich is at the very lowest it can be in the normal range (this from her muscle biopsy.) With her liver biopsy it was different with all the other chains still low but complex IV is actally 10 times the normal range (believed to be a compinsating effect.) Also 100% of her mitochondria are also physically mutated, all very large with very short cristae, all believed not to be functioning normally. In short, her pathology report was horrible. BUT, since starting the cocktail Grace is doing wonderfull. Her labs always to appear pretty much normal. Her liver is no longer killing itself, and is actually working completely normally. She is a relativly active 2 year old. Of course she has issues, and always will but nobody expects her to have a fatal outcome from this disease. We are unable to predict her outcome as far as needing a wheelchair or anything like that, frankly because nobody has ever seen a case like hers before. I guess my best advice is to take the report seriously, but remember some people respond outstanding to the cocktail. Listen to your genetecist, but don't expect them to give you a timeline of when she may go deaf or how old she will be when she will need a wheelchair (if she ever does). Most mito doctors will admit they still only know very little about this disease. Dr. Cohen always starts the appointments off with a smile, because nobody thought she would be doing as well as she is. I hope your child has the same great responce as Grace has. Find just what you're after with the new, more precise MSN Search - try it now! Quote Link to comment Share on other sites More sharing options...
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