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New diagnosis and so many questions

Posted: February 1st 2009

khawlah

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My son (now 22 months) was found to have mitochondrial disease (muscle biopsy) in late October. He was born with severe pulmonary hypertension and suffered very severe hypoxia during the first few days. Thankfully he lived and has resolved the hypoxia damage. Originally they felt my son had CP but the CP symptoms ebbed and flowed so mitochondrial disease was susspected and confirmed.

My son is bright and happy and he is thriving. I am so grateful and appreciative for this. He has a g-tube for supplemental feeding but does eat by mouth. He recieves PT, OT and SLP for oral motor strengthening. Most of the practical information I have found has been from lisiting to the podcasts of past mitoaction conference calls.

What is the best advice as to how to handle my son day to day? He looks 'normal' but as a mother I certainly know that he is not. He becomes so sensitive and inconsolable and refuses to sleep. This is a great concern to me b/c I often feel he would do much better if he just sleep. When he refuses to sleep he gets very restless and often kicks and hits. I have started keeping a appointment journal with all his fluid/food/med intake but I am unsure how best to use the information. I have noticed a big difference when he is well hydrated vs. 'normally' hydrated.

Any advice would be appreciated.
MotherWhimsey's picture

A lot of kids with Mito

Submitted by MotherWhimsey on Sun, 02/01/2009 - 1:42pm.

A lot of kids with Mito require a lot of hydration. My son is a little older than yours. We have to get at least 41 oz of fluid into him minimum or his autonomic dysfucntion gets worse and he has behavioral problems.
__________________

Courey mom to Aniyah, 4.5 and Linden, 20 mos. Linden has Mito complex III deficiency with slight deficiencies in complexes I and II. He is o2 and tube dependant.

www.lindenberriesandrosebuds.blogspot.com

It helps to know that the

Submitted by khawlah on Sat, 02/07/2009 - 12:13pm.

It helps to know that the hydration may really be one of the issues. I have not had a medical person explain this to me. Do you know why they require so much hydration? I am always interested to know the why. Thanks for your comment.

For me, I was told that I

Submitted by janice on Sat, 02/07/2009 - 10:55pm.

For me, I was told that I don't turn oxygen into water in sick cells. The Physicians manual for mito prior to the new one (which i'm sure the new one has it too) explains exactly what it is.  Which is on this site.  Lack of hydration has triggered a  metabolic crisis' in myself more than once.  We drink more water to meet our bodies needs, as we can't provide it naturally because of the mito error in converting it.  I'm not sure if this pertains to you, but you never know.  This site is full of great information. All the best.

Anthonys mom's picture

Manual? What Manual?

Submitted by Anthonys mom on Sun, 02/08/2009 - 12:11am.

Manual? What Manual?

Hi, sorry...it is on the top

Submitted by janice on Sun, 02/08/2009 - 1:36am.

Hi, sorry...it is on the top of the page under publications, then underneath it click on "symptom guide for clinicians"the previous one that i was given, also has a "sick formula" for when children get an infection. I calculated it for myself(has dosing instructions)and take it for 2-3 days when i feel a cold or flu starting. I haven't been sick in a couple of years since starting it.I'm sure the book came from U.M.D.F. I would gladly look for my copy if you can't find the info.
cellolover's picture

Thanks for writing about why

Submitted by cellolover on Sun, 02/08/2009 - 2:19pm.

Thanks for writing about why we need more water. I knew that I require lots of water, all the time, but did not know why. Now I do! Or at least one reason why people with mito need so much water! I carry a big bottle of water with me everywhere. Much much happier that way.
__________________

Rachel- I have abnormalities in Complex I,II, and V. I also married a wonderful man on June 5, 2010.

Your comments are very

Submitted by khawlah on Sun, 02/08/2009 - 5:03pm.

Your comments are very helpful. I have not been able to find the section that you are refering to in the UMDF Mito 101 (I assummed that was the UMDF book you were thinking of). I found the section on illness discussed taking fluids levels to 150% above baseline during illness. How do you know what baseline is? My son seems to need well above what the GI clinic has recommended but how do I know what would be optimal for him? Is there any way to calculate or is it just trial and error. I like your process of increasing your fluids to prevent illness-i am trying to get to a good system for my son. I have been playing with using water vs. pedilyte via the tube as well as trying to add some pedilyte to his overnight feed but I would love to feel I was working more with facts rather then just hit and miss.

lorimar's picture

A very good source of water

Submitted by lorimar on Sun, 02/08/2009 - 8:14pm.

A very good source of water is Smartwater; calorie free & has the electrolytes, that your body needs.I don't know exactly why we need so much hydration, but what Janice said about oxygen for sick cells is right on. I'll try to read more.Smartwater also taste great, (no advertisement here)take care all.....Lori:)

I don't recall that being

Submitted by janice on Mon, 02/09/2009 - 3:25am.

I don't recall that being the name of it. but then again the brain doesn'nt work as well as it used to!(it's a robins egg color manual) and it is from U.M.D.F.   I will try to copy it and post tommorrow evening.  Sorry, i don't know what childrens water needs are. As an adult, I believe it is approx. 10 glasses, so I drink 15 or more (if I can!)a day (at least 3 litres every 24 hrs. when a cold or bug comes on.) I can tell you what I was told...drink lots of water,eat smaller amounts every 4 hrs.(with a protien at each meal to extend the fuel process),avoid stress,get lots of rest, and take life slow and steady. The variations of mito disorders are quite surprisingly similar in symptoms I find. 

cellolover's picture

Just to muddy the waters a

Submitted by cellolover on Tue, 02/10/2009 - 11:09am.

Just to muddy the waters a bit further..... I decided I should figure out how many ounces of water I drink during a normal day. It turns out I drink about 100 ounces! That seems excessive, but I drink that much because when I drink less I don't feel as good. My best advice on water intake to khawlah would be when your little one starts feeling less than stellar try having him drink a bit and see if that helps. If it does then you know he probably needs some more water than you were giving him before. That's basically what I did with myself. On a different yet related note, does anyone know if humidifiers at night are a good thing?
__________________

Rachel- I have abnormalities in Complex I,II, and V. I also married a wonderful man on June 5, 2010.

Coury, Did I read you post

Submitted by Lils mom on Tue, 02/10/2009 - 11:14am.

Coury, Did I read you post right? Your Linden takes 41oz of fluid a day!! That seems like a LOT of fluid! My Lil only gets 620ml (about 20oz) a day. Any more than that and she projectile vomits EVERYWHERE! The rate has to be so low for her. It sounds great that hydration helps mito disease but how do you do this with GI issues? Lil is already hooked up to her pump 16 hrs a day. Any more and her blood sugars get out of wack and higher dose makes her vomit so I do not know how I would get her more hydrated. Any ideas anyone?
__________________

Meredith wife to my wonderful Carlos

and mom to

Liliana 4yrs Leighs disease

and Triniti 8yrs Angelman Syndrome

MotherWhimsey's picture

Linden has kidney issues and

Submitted by MotherWhimsey on Tue, 02/10/2009 - 2:22pm.

Linden has kidney issues and if we don't get enough fluids in he'll start having problems and puking. He gets about 40 oz of fluids in his feeds and then a couple of oz from flushes. His blood sugar issues are mainly hypoglycemia so sometimes he needs more feeds than he needs to gain weight just to keep his sugar up. We have his formula diluted so he can handle the larger volume of fluids which also makes the feed last longer (cause we have to run it at a slower rate) so that helps keep his blood sugar up. We start a feed after two hours, run it for an hour, then another two off, etc. through the G tube. Then at 6PM to 7AM we run continuous feeds through the J tube. He doesn't have as much of an issue with stomach motility during the day, but at night it just turns off. We have a 3 vomits in 24 hour limit. If he pukes more than three times we have to go to the ER and get an IV or his blood sugar gets really unstable and the dehydration makes his kidney issues worse. If we wait till he gets bad to start fluids then it can last a whole lot longer. If we just go in when we hit our three puke limit then he does much much better. can you run her on just plain fluids or pedialyte during the time that she's not on her pump for regular feeds? I just mix fluids into ours and extend the feeds but that took a long long time to figure out and get his sugars stable. Do you guys have a GJ tube? We have discovered that this is a wonderful wonderful invention. I don't think he's puked since we got his GJ tube. It has been amazing for him. Maybe you guys could ask if you could try that for hydration. good luck, I hope you guys get it figured out.
__________________

Courey mom to Aniyah, 4.5 and Linden, 20 mos. Linden has Mito complex III deficiency with slight deficiencies in complexes I and II. He is o2 and tube dependant.

www.lindenberriesandrosebuds.blogspot.com

Anthonys mom's picture

Mito 101 is from UMDF we

Submitted by Anthonys mom on Wed, 02/11/2009 - 2:04am.

Mito 101 is from UMDF we went to the conference they had this summer. For anyone wanting a copy they came out with a Cd it is really nice. They will send you copies you just need to write them.
Anthonys mom's picture

Rachel, It is so good to

Submitted by Anthonys mom on Wed, 02/11/2009 - 2:09am.

Rachel, It is so good to hear you drink 100 oz of water my little guy has always wanted alot of water. He is G-tube feed and will drink at least 80 oz extra. We went for all kind of testing. Everyone said I should cut back on it. I tried EVERYTHING and he just seems more tired with out it. It feels good to know that an adult feels the same way. I was beganing to feel like I was crazy. When did you find out you had mito?

Courey, We have tryed just

Submitted by Lils mom on Wed, 02/11/2009 - 5:06pm.

Courey, We have tryed just giving water or pedialite in between feeds or mixed with it but she can not tolerate ANY increase in her ammounts. If the doctor even ups any of her medicines (which are all liquid) she may begin vomiting again. She had the GJ tube but she wretches when she vomits and she vomited it out of place one to many times so I told them to go back to the G. They wanted to put in a J and leave the G for meds but I will not put her through another surgery that will cause her more pain because her disease is progfressing SO fast as it is that her doctors do not give her long. I am going to try to up the rate at night by maybe 5-10mls to see if she can tolerate it and if so maybe the hydration will help.
__________________

Meredith wife to my wonderful Carlos

and mom to

Liliana 4yrs Leighs disease

and Triniti 8yrs Angelman Syndrome

I have been working with the

Submitted by khawlah on Wed, 02/11/2009 - 9:48pm.

I have been working with the fluids with my son this week. He has been having more trouble drinking so I have been adding more and more to the tube. I think he is in the 35-40 range for sure. He has had at least 40 for several days now and he is perking up more. Strangely I just got back from a consult and the docs at Columbia think my son's muscle biopsy was mishandled and also feel he is not developmentally delayed enough to have mito. They will do another muscle biopsy and also a lumbar punch, etc to take a second look. Anybody know why else he might have such high fluid demands in the absence of mito?

I was wondering about the GJ

Submitted by khawlah on Wed, 02/11/2009 - 9:53pm.

I was wondering about the GJ tube. I never saw any signs of reflux with my son until after his G tube placement. He started having very big problems during his night feeds and at times can't even tolerate 20ml/hr. Could I really have missed that he had reflux? I was looking for it b/c it was a common question. The docs told me that I don't want to do a j tube and should just control it with flow rate. I do use the g tube for fluids b/c he just can't and won't drink nearly enough without it.
mitomomx2's picture

Hello everyone, My name is

Submitted by mitomomx2 on Wed, 04/22/2009 - 11:33pm.

Hello everyone, My name is RoBen,My husband and I adopted our 2 children a brother and sister(each as babies)that have an un named mito disease. But are working with a team of Drs.who are trying to name it. they know our son's is in complex IV and our daughter's is in Complex III.The Drs are a little confused by this. Evan(11) is alot sicker than his sister Taylor(8)after finding this web site and read your messages to each other, I see I have so much still learn, about care for these children.They both are carnitine Deficient, maligent hyperthermia , muscle jerking when sleeping. Evan has alot more issuses, seizuries optic never damage his list goes on.Thank you for being out there! RoBen
Tina_EvesMum's picture

Hi Roben and welcome. I have

Submitted by Tina_EvesMum on Thu, 04/23/2009 - 1:31am.

Hi Roben and welcome. I have three adopted children all with different complex needs as well as two home grown kids who also each have health concerns. Our youngest was suspected of having a mitochondrial cytopathy after an MRI last year...the ongoing investigatiions have confirmed this but failed to pinpoint the exact problem. Our youngest is a twin and so far her twin seems unnaffected although there are one or two alarm bells. Her twin is with birth family. nice to meet you anyway
__________________

Tina Mum of 5 children Eve has just been given a suspected diagnosis of Mitochondrial Cytopathy.

I do not know what the future holds but I am glad I know who holds the future!

lorimar's picture

Hi Robin, Welcome to the

Submitted by lorimar on Thu, 04/23/2009 - 1:18pm.

Hi Robin, Welcome to the forum, I don't have children, with Mito,but I do have it myself. I have complex 1 defect. With many symptoms, I get the same muscle jerking in my sleep that your son gets. My neurologist, tried me on Requip, and my husband says it's working. You could ask your doctor about it. I hope this is helpful...........Good-Luck to you....Lori:)
Anthonys mom's picture

Welcome RoBen, I am glad

Submitted by Anthonys mom on Thu, 04/23/2009 - 10:40pm.

Welcome RoBen, I am glad you found the website. I hope you will find hope and comfort here.
LGlasbergen's picture

Hi all, just reading all

Submitted by LGlasbergen on Mon, 05/11/2009 - 11:39am.

Hi all, just reading all this, makes me feel that I'm not alone. :)