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I have been 'laying low' for a while, and not posted much, however,

I am wondering how may PDH affected children are out there on this

list. We live in Australia, and know of only 2 other children. I am

sure there are more out there.

Gaby is stable on the keto diet (g tube fed), but still has

significant permanent disabilities. She is about to face big hip

surgery in the near future in order to try and preserve what little

mobility she has (any surgery is always a worry with mito kids!)

ne (mum to Gaby aged 7 PDH, Australia)

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Yes you have to worry and good luck to you and your family i hope you all do

great through this i will be praying god bless

shannon Mom of

Nov.07 2002- May 15, 2003

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what is PDH?

Shirley

> SEanne,

>

> WE hve just recently got a diagnosis of PDH with Lexi, although even

> then the

> results were strange. The doc said that her levels were low but not

> absent

> when they looked for PDH. So she said it is indicative of PDH....and

> that is

> what we are running on right now. Ayear from now it may be different

> as with

> all things Mito! Mitch (Lexi's big brother) was just diagnosed

> with

> muscular dystrophy that is " probably mito related " ...we haven't yet

> done any

> testing onhim for PDH or anything else. Lexi has shown symptoms since

> she was 4

> hours old. Mitch started showing symptoms at age 6 1/2.

>

> ruth

> mom to 2 beautiful treasures

>

>

> Please contact mito-owner with any problems or

> questions.

>

>

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Hi Lexi,

what sort of symptoms did your daughter show as a new born? Has any

treatment been recommedned at all for her? Always interested to

share and learn about this disorder

Cheers, ne (mum to 7 year old Gaby with PDH)

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when my Lexi was born, she began hving difficulty breathing at about 4 hours

old. The docs took her to the NICU for about 4 days. she had an IV at that

time so i didn't nurse her for the first 2 days and then when i tried,she had a

really hard time. i just figured she was about 3 weeks early and a little

uncoordinated. so at 4 days old we went home. and immediately she began to

have problems. like when i would feed her she would turn blue and gag and

writhe...ii'm a speech thearpist so i KNEW she had dysphagia issues. when i

took

her back to the pediatrician he immeditaely put her back in the hospital (for

the first of now 27 hospitalizations). then we discovered she has sleep apnea,

reflux, bradycardia, and low oxygen saturation levels. her first year she

frequently went into respiratory and cardiac arrest. since then she has

developed atypical seizures (they show up as hiccups...no kidding), kidney

problems,

bladder problems (like she will not pee for 48 hours or more), and cyclical

constipation. also fine motor delays and MAJOR sensory integration issues.

heat

and cold intoleranace is also an issue. sheesh....i haven't sat down and

written it all out in ages! but in spite of it all, she is cognitively intact,

babies and barbies and anything to do with kittens!

what other symptoms have others seen with their PDH kiddos?

ruth

aka " Mitch and Lexi's mom! " lol

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Three of my 4 children stopped breathing off and on after birth. The second

child had to have mouth to mouth, the 3rd continually stopped and I would

push her gently, the 4th stopped and we rushed him to the hospital and he

started just as we pulled in. He kept turning bluish so we finally had an

x-ray of his heart which they said was slightly enlarged (they didn't know

why). We thought it was the nitrates in the Phx, AZ water. It was worse when

he ate. He kind of grew out of it I guess. We never looked at his heart

again. He also developed asthma which needed hospital breathing treatments.

He is VERY thin, but strong.

All 4 of my kids had eating problems, ate odd things, projectile vomiting,

congestion from formulas.

Kids and grandkids with constipation problems and large stools. Sleepwalking

and talking, odd rashes and sensitivities to so much, nosebleeds, anemia.

Oldest daughter had continual UTIs and they finally did an exploratory

surgery to see why, same with me and a second cousin. My oldest son is

sterile, a lot of miscarriages in some females in family. Thinning hair is a

problem, and Riley's hair is turning gray now (she's 7)!

Scoggins

Re: Re: PDH children

> when my Lexi was born, she began hving difficulty breathing at about 4

hours

> old. The docs took her to the NICU for about 4 days. she had an IV at

that

> time so i didn't nurse her for the first 2 days and then when i tried,she

had a

> really hard time. i just figured she was about 3 weeks early and a little

> uncoordinated. so at 4 days old we went home. and immediately she began

to

> have problems. like when i would feed her she would turn blue and gag and

> writhe...ii'm a speech thearpist so i KNEW she had dysphagia issues. when

i took

> her back to the pediatrician he immeditaely put her back in the hospital

(for

> the first of now 27 hospitalizations). then we discovered she has sleep

apnea,

> reflux, bradycardia, and low oxygen saturation levels. her first year she

> frequently went into respiratory and cardiac arrest. since then she has

> developed atypical seizures (they show up as hiccups...no kidding), kidney

problems,

> bladder problems (like she will not pee for 48 hours or more), and

cyclical

> constipation. also fine motor delays and MAJOR sensory integration

issues. heat

> and cold intoleranace is also an issue. sheesh....i haven't sat down and

> written it all out in ages! but in spite of it all, she is cognitively

intact,

> babies and barbies and anything to do with kittens!

>

> what other symptoms have others seen with their PDH kiddos?

>

> ruth

> aka " Mitch and Lexi's mom! " lol

>

>

> Please contact mito-owner with any problems or questions.

>

>

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PDH stands for Pyruvate Dehydrogenase Deficiency.

ne

-----Original Message-----From: Shirley Cabaldon Sent: Saturday, 31 May 2003 4:14 PMTo: Mito Subject: Re: PDH childrenwhat is PDH?ShirleyPlease contact mito-owner with any problems or questions.

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wow Ruth..Lexi has had a busy time!!

Gaby's initial presentation was microcephaly, reflux and at about 12 months Seizures.At that time she would simply put both hands above her head and rub her head back and forward between her arms..almost as if she had a sore ear. None of the Drs felt it was seizures..only me..but mothers have great instincts don't they!!

Gaby has lots of expressive language probs, and despite her microcephaly and dvelopmental delay, I have no doubt she understands most of what is said. She has some awe inspiring automatic speech, and can sing over 20 songs (she has near perfect pitch!0, but will not have anything in her mouth apart from her fingers and her dummy (pacifier?) Gaby can gag at the sight of food!

Gaby also had issues with temp control (big time) and apnoes (prior to her fundo). Her temp is better since starting the keto diet. I feel it is due to the increased metabolism from the ketoacidosis, but I could be wrong. Anaesthetics are always a prob, a she drops her temp to 33 degrees celcius if not actively warmed with the 'Bear hugger' blanket before during and after the anaesthetic.

ne mum to Gaby, PDH, G Tube, keto diet, wheelchair dependant, CP,dislocated hips, and loves to sing!

-----Original Message-----From: hilandgang@... Sent: Sunday, 1 June 2003 12:23 AMTo: Mito Subject: Re: Re: PDH childrenwhen my Lexi was born, she began hving difficulty breathing at about 4 hours old. The docs took her to the NICU for about 4 days. she had an IV at that time so i didn't nurse her for the first 2 days and then when i tried,she had a really hard time. i just figured she was about 3 weeks early and a little uncoordinated. so at 4 days old we went home. and immediately she began to have problems. like when i would feed her she would turn blue and gag and writhe...ii'm a speech thearpist so i KNEW she had dysphagia issues. when i took her back to the pediatrician he immeditaely put her back in the hospital (for the first of now 27 hospitalizations). then we discovered she has sleep apnea, reflux, bradycardia, and low oxygen saturation levels. her first year she frequently went into respiratory and cardiac arrest. since then she has developed atypical seizures (they show up as hiccups...no kidding), kidney problems, bladder problems (like she will not pee for 48 hours or more), and cyclical constipation. also fine motor delays and MAJOR sensory integration issues. heat and cold intoleranace is also an issue. sheesh....i haven't sat down and written it all out in ages! but in spite of it all, she is cognitively intact, babies and barbies and anything to do with kittens!what other symptoms have others seen with their PDH kiddos?ruthaka "Mitch and Lexi's mom!" lolPlease contact mito-owner with any problems or questions.

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My daughter, Abby, has PDH as well. She is 15 mos old. with

microcephaly, seizures (infantile spasms), 100% g-tube fed, starting

keto diet tomorrow, is at about a 3 month old developmental level

(just starting to lift her head when on her belly), sleep apnea,

will be getting her adnoids (maybe tonsils too) removed and have ear

tubes inserted on Friday 13th, major problems moving her bowels

independently, hypotonic, visually impaired (doesn't focus), and a

mama's girl. She loves to to be spun around and gives me the best

smile in return. I am really excited to try her on the keto diet

because of the improvements seen in Gaby after being on the diet.

Abby will also be starting dichloroacetate which should help the

pyruvate dehydrogenase work at its best. We are from Canada and are

having to move to another province (Calgary, Alberta) so that we

will have a children's hospital taking care of her from head to

toe. Right now we are doing too much travelling and decided to move

to the doctors where we will hopefully have more peace of mind. It

is so nice to hear that Gaby is singing songs! How old was she when

she said her first word?

e

> I have been 'laying low' for a while, and not posted much,

however,

> I am wondering how may PDH affected children are out there on this

> list. We live in Australia, and know of only 2 other children. I

am

> sure there are more out there.

> Gaby is stable on the keto diet (g tube fed), but still has

> significant permanent disabilities. She is about to face big hip

> surgery in the near future in order to try and preserve what

little

> mobility she has (any surgery is always a worry with mito kids!)

>

> ne (mum to Gaby aged 7 PDH, Australia)

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e, Gaby vocalised sounds as a small infant, but life for her really started after the diet. She didn't 'focus' either pre diet and was thought to have CVI! She soon started wearing glasses for a squint and short sightedness..which has recently reduced to a mild squint correction. She fixes and follows really well, and focuses on things of interest to her. Gaby's overall tone also improved..and so the story goes on! Where are you living at present?

Good luck with the diet e. Pleas ekeep in touch and let us know how Abby goes. Fingers crossed! Don't expect changes over night..it does take a little while, so be patient)

ne

-----Original Message-----From: jaimeedowd2002 Sent: Tuesday, 3 June 2003 3:18 PMTo: Mito Subject: Re: PDH childrenMy daughter, Abby, has PDH as well. She is 15 mos old. with microcephaly, seizures (infantile spasms), 100% g-tube fed, starting keto diet tomorrow, is at about a 3 month old developmental level (just starting to lift her head when on her belly), sleep apnea, will be getting her adnoids (maybe tonsils too) removed and have ear tubes inserted on Friday 13th, major problems moving her bowels independently, hypotonic, visually impaired (doesn't focus), and a mama's girl. She loves to to be spun around and gives me the best smile in return. I am really excited to try her on the keto diet because of the improvements seen in Gaby after being on the diet. Abby will also be starting dichloroacetate which should help the pyruvate dehydrogenase work at its best. We are from Canada and are having to move to another province (Calgary, Alberta) so that we will have a children's hospital taking care of her from head to toe. Right now we are doing too much travelling and decided to move to the doctors where we will hopefully have more peace of mind. It is so nice to hear that Gaby is singing songs! How old was she when she said her first word?e> I have been 'laying low' for a while, and not posted much, however, > I am wondering how may PDH affected children are out there on this > list. We live in Australia, and know of only 2 other children. I am > sure there are more out there.> Gaby is stable on the keto diet (g tube fed), but still has > significant permanent disabilities. She is about to face big hip > surgery in the near future in order to try and preserve what little > mobility she has (any surgery is always a worry with mito kids!)> > ne (mum to Gaby aged 7 PDH, Australia)Please contact mito-owner with any problems or questions.

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Thank you, shirley

> PDH stands for Pyruvate Dehydrogenase Deficiency.

> ne

>

> Re: PDH children

>

> what is PDH?

>

> Shirley

>

>

>

>

> Please contact mito-owner with any problems or

> questions.

>

>

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