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,

I'm not a doctor by any means (I don't even play one on T.V. ;) so I

wouldn't begin to presume to know what might be right for . I will say

that certain mito experts recommend the 10mg per kg for 6 months and if no

improvement, they recommend that it be raised to 20mg per kg. If still no

improvement in 6 months, I believe they recommend that it be discontinued,

but I don't know that for sure.

At 47 pounds weighs a little over 21 kg so the recommended dosage

according to the above formula would be 210-420 mgs, so I'd say you are

pretty close to the above recommendation at least.

I strongly recommend however that you check with 's doctor(s) before

making any changes in his meds. :)

Terri

>From: PFlicek@...

>Reply-To: Mitoonelist

>To: Mitoonelist

>Subject: Re: An introduction...

>Date: Sat, 11 Sep 1999 19:44:41 EDT

>

>From: PFlicek@...

>

>Hi, Terri

>My name is and my son has mito chondria myopathy, and he is

>7years old he weighs 47 pounds and takes 200mg of the cq 10 do you think

>thats a big enough dose? I'm the one who put him on it so I'm not sure if

>that is enough for his weight. I break the capsule and put in his

>applesauce

>along with his b-50.

> has been on it for 5 months with no effects but who knows if it is

>working or not. Let me know what you think.

> Thanks F.

>

>---------------------------

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Hi,

I just wanted to comment on giving these vitamins. Dr. Haas put my

kids on CoQ and he only recommended 50mg twice a day (100mg total) and my

kids weigh about 42 pounds each. I would definately check with the doctor

about giving ANY kind of meds, including vitamins. I have actually cut down

the dose for because he was getting flush and fidgety. I had tried

the stuff too (50mg once a day) and was short of breath and dizzy. What

works for one kid, may be devestating to another. Call me paranoid, but I

wouldn't give any kind of meds without checking with the doctor.

Sue ( & Jack)-worn out parents to the greatest kids on earth--

Chris-9: Leigh's Disease (?), MR (mild), hypotonia, non-walker/talker

-7: Leigh's Disease (?), MR (mild), hypotonia, non-walker/talker, trach

tube (larynotracheomalasia), g-tube/fundo Visit us! http://u2.lvcm.com/jscb

> I'm not a doctor by any means (I don't even play one on T.V. ;) so I

> wouldn't begin to presume to know what might be right for . I will

say

> that certain mito experts recommend the 10mg per kg for 6 months and if no

> improvement, they recommend that it be raised to 20mg per kg. If still no

> improvement in 6 months, I believe they recommend that it be discontinued,

> but I don't know that for sure.

>

> At 47 pounds weighs a little over 21 kg so the recommended dosage

> according to the above formula would be 210-420 mgs, so I'd say you are

> pretty close to the above recommendation at least.

>

> I strongly recommend however that you check with 's doctor(s) before

> making any changes in his meds. :)

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Hi,

I just wanted to comment on giving these vitamins. Dr. Haas put my

kids on CoQ and he only recommended 50mg twice a day (100mg total) and my

kids weigh about 42 pounds each. I would definately check with the doctor

about giving ANY kind of meds, including vitamins. I have actually cut down

the dose for because he was getting flush and fidgety. I had tried

the stuff too (50mg once a day) and was short of breath and dizzy. What

works for one kid, may be devestating to another. Call me paranoid, but I

wouldn't give any kind of meds without checking with the doctor.

Sue ( & Jack)-worn out parents to the greatest kids on earth--

Chris-9: Leigh's Disease (?), MR (mild), hypotonia, non-walker/talker

-7: Leigh's Disease (?), MR (mild), hypotonia, non-walker/talker, trach

tube (larynotracheomalasia), g-tube/fundo Visit us! http://u2.lvcm.com/jscb

> I'm not a doctor by any means (I don't even play one on T.V. ;) so I

> wouldn't begin to presume to know what might be right for . I will

say

> that certain mito experts recommend the 10mg per kg for 6 months and if no

> improvement, they recommend that it be raised to 20mg per kg. If still no

> improvement in 6 months, I believe they recommend that it be discontinued,

> but I don't know that for sure.

>

> At 47 pounds weighs a little over 21 kg so the recommended dosage

> according to the above formula would be 210-420 mgs, so I'd say you are

> pretty close to the above recommendation at least.

>

> I strongly recommend however that you check with 's doctor(s) before

> making any changes in his meds. :)

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>works for one kid, may be devestating to another. Call me paranoid, but I

>wouldn't give any kind of meds without checking with the doctor.

Boy, you can't say that enough times. Because of the nature of mito

defects, it's almost like a needle in a haystack. There are 43 processes in

complex I, 5 in complex II, 11 in complex III and 13 in complex IV - plus

all of the things that feed into those processes!

We've been told that two kids with exactly the same defect can have very

different reactions and symptoms, and when you add in that there are 11

processes in Complex III, with multiple parts in each, the likelyhood of two

families with complex III defects even being in the same area is pretty

remote. Probably the best examples are PKU and the protein processing

deficiencies - diet changes impact them, but similar dietary changes may not

help and even hurt.

For any changes go slowly, and check with the docs! And that includes

stopping things as well.

Kathy

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>works for one kid, may be devestating to another. Call me paranoid, but I

>wouldn't give any kind of meds without checking with the doctor.

Boy, you can't say that enough times. Because of the nature of mito

defects, it's almost like a needle in a haystack. There are 43 processes in

complex I, 5 in complex II, 11 in complex III and 13 in complex IV - plus

all of the things that feed into those processes!

We've been told that two kids with exactly the same defect can have very

different reactions and symptoms, and when you add in that there are 11

processes in Complex III, with multiple parts in each, the likelyhood of two

families with complex III defects even being in the same area is pretty

remote. Probably the best examples are PKU and the protein processing

deficiencies - diet changes impact them, but similar dietary changes may not

help and even hurt.

For any changes go slowly, and check with the docs! And that includes

stopping things as well.

Kathy

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-

We are not an exact match, but some of the characteristics are similar.

Kennedy (2, daughter) is a Complex3 mito kid, and also rubs her eyes and

nose very hard. She is severely hypotonic (unable to hold her head up or

turn over), is ventilated, and has never spoken. As time has gone by, her

neurological reactions to surroundings have gradually narrowed until she

rarely goes beyond " content " as a prevalent emotion. Our children are very

different from each other so please don't read Kennedy's condition as your

son's future - I just wanted to share that the frequent eye-rubbing motions

are symptomatic of mitochondrial problems at least in our case. Good luck

to you and God bless your son -

& Ann Hairston

" Kennedy's Personal PICU "

An introduction...

>

>

> Hello all. I have now joined this list due to the encouragement of Terri

Mason, who has been helping me

> sort out some of my son's health problems over the last year.

>

> My son will be 2 1/2 at the end of this month. He was the result of a

perfect pregnancy and perfect

> birth. He had no health problems until last August, except for mild

laryngeal stridor and a skin rash

> (keratosis pilaris), which he STILL has. He cannot walk independently,

although he walks holding hands

> and takes independent steps between furniture. He is mildly hypotonic

(but extremely strong), mildly

> ataxic, and has scoliosis (20 degrees). Although he has never slept

through the night, he was a perfectly

> normal little boy until last August, when he developed nystagmus (four

weeks after his MMR vaccine). It

> was then that we found out he has pale optic nerves, which appear to be

static in nature for the moment.

> He was the smartest little boy in the world until about 4 weeks ago, when

he seemed to stop understanding

> the spoken word. We used to play games where we would sit down and I

would say, " Okay, tell me what's at

> Grandmommy's house, " then he would go on to list the many things that he

loved to do and see at grandma's.

> Now when I ask him, he gives me a dumb look and says nothing. He is

showing signs of autism (big time).

> He has been irritable in strange settings his whole life. He doesn't play

with other kids and doesn't

> like toys (he prefers books and videos). He loves looking at things that

spin, especially fans. He climbs

> on EVERYTHING and seems to amuse himself quite well. He has developed a

strange facial tic and has been

> grunting and babbling non-stop since December, as well as doing some

strange things with his hands (all

> autistic behaviors). Autism and Tourette's runs in my family, but not the

vision problem or ataxia. In

> addition, snores terribly and has sleep apnea. He has also had

loose stools his entire life,

> sometimes white in color, although he has never had a problem eating or

swallowing and has only vomited

> twice in his life. I don't think he has seizures, but he does " shake "

sometimes when he is asleep after

> he has snored, then rubs his nose really hard.

>

> We have run through most tests for , including an MRI last September

which was negative, blood for

> amino and organic acids, as well as mitochondrial deletions. We are

currently awaiting results for

> bloodwork for things like hereditary ataxias and leukodystrophies.

However, the doctors have hinted

> around that they think may fit into the category of mitochondrial

disease, even though his blood work

> was negative.

>

> Does any of this sound familiar? I know doesn't sound as severe as

some of the posts I have read,

> and I still wonder if this isn't some sort of congenital condition. But I

would love to get feedback from

> anyone as to whether or not 's symptoms are typical of mito. disease.

I am worried he will never

> walk, and more worried that I will never have my little boy back. He just

isn't " there " most of the time

> anymore. He is extremely happy, very healthy and energetic,

embarrassingly affectionate, and smart as a

> whip if you can get him to focus (hard to do these days).

>

> I am looking forward to hearing back from some of you. Thanks so much for

listening.

>

> (mom to , 2 1/2, gross/fine motor delay, nystagmus, optic nerve

atrophy, ataxia, autistic

> tendencies)

>

> p.s. Thanks, Terri :-)

>

>

> ---------------------------

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