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Hey Nicky,

Were also somewhat new to all this, we only got final confirmation

of s diagnosis yesterday although he got sick for the first

time a year ago yesterday (appropriate timing). turned 2

in December, and like you I knew something was wrong well before

anyone else... last January he got sick and they insisted it was a

viral problem... in May I finally pushed enough to get them to do

more testing, and the tests showed something was up (lactic

acidosis, low carnatine, low blood sugar, and a bunch of other

issues) thye did a biosy in October and it came back this week

showing he has a Complex 1 Deficiency.

All in all is quite healthy considering... hes is severly

speech delayed and doesnt talk, although he has learned over 50

signs now, so is very communicative. His hearing screen came back

ok, but they werent able to do a very thorough one as he didnt want

to cooperate, though at this time we dont have any concerns about

his hearing.

As for your questions, our life has been completely changed by all

this... my hopes for have completely shifted, I dont take

much for granted anymore, Our life has been taken over by medical

bills, doctors appointments, speech and physio therapy, pain

control, avoiding viruses, and all the while trying to make sure he

has a fun time in it all... but in the end we do have alot of

fun... i think we appreciate the smaller things more than most

families do, because we know how fragile it all is.

Feeling out of control? oh yea. the last year has been like a

roller coaster ride. everytime he would get the sniffles I would

wonder 'what will this turn into??' this summer he had a temp of

101 when we got on a plane in minneapolis, an hour later we got off

the plane in Winnipeg and his temp was 107... i mean what do you do

in an airport with no tylenol or motrin, and a baby with a temp of

107??? I freaked out in customs (thank goodness they got us through

fast as the linup was over an hour otherwise)! not only having to

learn about this diagnosis, but taking it upon myself to have to

explain it all to the ER doctor when hes sick... who more often than

not doesnt appreciate being taught something by a parent.

Control is NOT something that goes hand in hand with sick

children...

I dont imagine your the only one with a strained marriage... sick

kids are extremely difficult on couples, especially when both

partners deal with this sort of thing differently.

My husband likes to pretend everythings fine, while I like to hash

out every little detail. this has made it difficult on both of us,

as I want to talk about it and he wants to ignore it. But at the

same time I have found that its really nice to buy into his point of

view sometimes, and pretend life is peachy... likewise he

occasionally likes to have 'crash courses' on whats happening, since

im primarily the one that takes to doc appointment. I make

sure he is there for the pivotal appointments (the ones we get

results at and such), but other than that he usually has to work on

appt days. (this also makes it easier for him to imagine

everythings fine). I thought at first that he didnt care about all

of it that much, but over time i realised its just his way of

dealing with it, even if it is opposite to teh way i deal with

it...

How do I keep up? I dont.

lol!

in all of this im 9 months pregnant right now, so getting through

the day in once piece with a happy, well fed child is the only major

priority of mine... As for his needs, they change daily, one day he

will need 2 naps, the next he needs 1, one night he needs to go to

bed at 6, the next night its 8... one day hes up at 6, the next its

9... some days his blood sugar is low and he needs to snack

constantly, other days his sugar is fine... some days his legs hurt

and he needs to spend lots of time in his high chair, other days his

legs are fine... some days hes shakey, otehrs hes not... you get

the point... ive figured out that the best way to keep myself sane

is to stop trying to predict what kind of a day it will be, and let

him call the shots... I never know what he will need, or how he will

be feeling (and how that will jibe with how im doing, as i have my

own health problems to deal with in all this) until the time comes,

so trying to make solid plans is a frustrating thing.

Anyawys, i wasnt meaning to come of as whiney, just realistic... in

spite of all that we really do have alot of fun... granted lately

were pretty slowed down, that has more to do with this baby on the

way than anything...

Welcome to the group, im sure your going to find everyone here a

huge help, theres nothing better than talking to a group of people

who honestly DO know exactly how you feel... makes it all a little

less scary.

Keely (mom to , 2, Complex 1 deficiency)

>

>

> Hi, I new to post to this group. I have a son who is 2.5 yrs.

> diagnosed with pyruvate dehydrogen. deficiency. He was diagnosed

at

> 7 mos. I knew there was something wrong at 3 mos.. I am an

> audiologist and my son with diagnosed with a progressive hearing

> loss at 6 weeks. As an infant, he screamed all day long. He had

> severe head lag. I knew there was something wrong with him. I

asked

> to see a neurologist at 4 mos. They also thought due to the

> progressive nature of his hearing loss that there was something

> genetically wrong. They did a ton of labs. Found that he had

> severe lactate acidosis. He immediately was put on DCA. Which

> revealed a significant improvement. No increased acidosis. His

is

> now 2, Isaiah is the happiest boy ever. He has a g-tube, deaf

(with

> a cochlear implant), doesn't sit up, still with poor head control.

> He is such a wild man. He rolls all over. We are so glad to be

> blessed with him.

>

> I guess my question is for parents with these sick kids. How has

> your life been affected by this. Do you sometimes feel out of

> control. Am I the only on that has a strained marriage. HOw do

you

> keep up.

>

> Nicky

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Hey Nicky,

Were also somewhat new to all this, we only got final confirmation

of s diagnosis yesterday although he got sick for the first

time a year ago yesterday (appropriate timing). turned 2

in December, and like you I knew something was wrong well before

anyone else... last January he got sick and they insisted it was a

viral problem... in May I finally pushed enough to get them to do

more testing, and the tests showed something was up (lactic

acidosis, low carnatine, low blood sugar, and a bunch of other

issues) thye did a biosy in October and it came back this week

showing he has a Complex 1 Deficiency.

All in all is quite healthy considering... hes is severly

speech delayed and doesnt talk, although he has learned over 50

signs now, so is very communicative. His hearing screen came back

ok, but they werent able to do a very thorough one as he didnt want

to cooperate, though at this time we dont have any concerns about

his hearing.

As for your questions, our life has been completely changed by all

this... my hopes for have completely shifted, I dont take

much for granted anymore, Our life has been taken over by medical

bills, doctors appointments, speech and physio therapy, pain

control, avoiding viruses, and all the while trying to make sure he

has a fun time in it all... but in the end we do have alot of

fun... i think we appreciate the smaller things more than most

families do, because we know how fragile it all is.

Feeling out of control? oh yea. the last year has been like a

roller coaster ride. everytime he would get the sniffles I would

wonder 'what will this turn into??' this summer he had a temp of

101 when we got on a plane in minneapolis, an hour later we got off

the plane in Winnipeg and his temp was 107... i mean what do you do

in an airport with no tylenol or motrin, and a baby with a temp of

107??? I freaked out in customs (thank goodness they got us through

fast as the linup was over an hour otherwise)! not only having to

learn about this diagnosis, but taking it upon myself to have to

explain it all to the ER doctor when hes sick... who more often than

not doesnt appreciate being taught something by a parent.

Control is NOT something that goes hand in hand with sick

children...

I dont imagine your the only one with a strained marriage... sick

kids are extremely difficult on couples, especially when both

partners deal with this sort of thing differently.

My husband likes to pretend everythings fine, while I like to hash

out every little detail. this has made it difficult on both of us,

as I want to talk about it and he wants to ignore it. But at the

same time I have found that its really nice to buy into his point of

view sometimes, and pretend life is peachy... likewise he

occasionally likes to have 'crash courses' on whats happening, since

im primarily the one that takes to doc appointment. I make

sure he is there for the pivotal appointments (the ones we get

results at and such), but other than that he usually has to work on

appt days. (this also makes it easier for him to imagine

everythings fine). I thought at first that he didnt care about all

of it that much, but over time i realised its just his way of

dealing with it, even if it is opposite to teh way i deal with

it...

How do I keep up? I dont.

lol!

in all of this im 9 months pregnant right now, so getting through

the day in once piece with a happy, well fed child is the only major

priority of mine... As for his needs, they change daily, one day he

will need 2 naps, the next he needs 1, one night he needs to go to

bed at 6, the next night its 8... one day hes up at 6, the next its

9... some days his blood sugar is low and he needs to snack

constantly, other days his sugar is fine... some days his legs hurt

and he needs to spend lots of time in his high chair, other days his

legs are fine... some days hes shakey, otehrs hes not... you get

the point... ive figured out that the best way to keep myself sane

is to stop trying to predict what kind of a day it will be, and let

him call the shots... I never know what he will need, or how he will

be feeling (and how that will jibe with how im doing, as i have my

own health problems to deal with in all this) until the time comes,

so trying to make solid plans is a frustrating thing.

Anyawys, i wasnt meaning to come of as whiney, just realistic... in

spite of all that we really do have alot of fun... granted lately

were pretty slowed down, that has more to do with this baby on the

way than anything...

Welcome to the group, im sure your going to find everyone here a

huge help, theres nothing better than talking to a group of people

who honestly DO know exactly how you feel... makes it all a little

less scary.

Keely (mom to , 2, Complex 1 deficiency)

>

>

> Hi, I new to post to this group. I have a son who is 2.5 yrs.

> diagnosed with pyruvate dehydrogen. deficiency. He was diagnosed

at

> 7 mos. I knew there was something wrong at 3 mos.. I am an

> audiologist and my son with diagnosed with a progressive hearing

> loss at 6 weeks. As an infant, he screamed all day long. He had

> severe head lag. I knew there was something wrong with him. I

asked

> to see a neurologist at 4 mos. They also thought due to the

> progressive nature of his hearing loss that there was something

> genetically wrong. They did a ton of labs. Found that he had

> severe lactate acidosis. He immediately was put on DCA. Which

> revealed a significant improvement. No increased acidosis. His

is

> now 2, Isaiah is the happiest boy ever. He has a g-tube, deaf

(with

> a cochlear implant), doesn't sit up, still with poor head control.

> He is such a wild man. He rolls all over. We are so glad to be

> blessed with him.

>

> I guess my question is for parents with these sick kids. How has

> your life been affected by this. Do you sometimes feel out of

> control. Am I the only on that has a strained marriage. HOw do

you

> keep up.

>

> Nicky

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Hi Nicky, and welcome, I knew from day one something was wrong with

my son Matt, the only time he didnt cry and scream is when he was

untouched and in absolute quiet. I left my husband when Matt was

just 2 months old, and thank God he has nothing to do with us at

all. I am remarried to a wonderful man who no one would ever

believe wasnt the biological father of all my children. He is there

through thick and thin expecially with Matt. I think any illness

can be a strain on marriages and families. You just have to work as

a team and remember that everyone handles things differently and you

have to respect that in one another, kinda agree to disagree, but

you must still meet in the middle when it comes to caring for the

child, no matter how different the aproach. And it takes awhile to

get a system going as far as siblings and husband, but with patience

and time things do fall into place, and somehow just to start

running somewhat smoothly again. Although our household is not

normal to most, when we started being organized with all the medical

equip, and doc appts., and such, this has become just as routine a

life as anyone elses. Sure there are days that are hard and

unorganized, i make sure i get a good cry in, and then some rest and

then get on with things again. I also have mito adult onset, and i

would have never believed anyone telling me when i first became ill

that things would get back to " normal " , but to my surprise even on

my worse days, i get through that day, i am blessed with a

supportive husband and my other two children are 19 and 22 years of

age, so they are a big big help, dont think i could do it without

thier love and support. And even when other family members dont

understand or cant handle what goes on, i always have this group to

turn to and that has made a big difference in the way i deal with

things too. Hang in there, and hang out here at this group you will

find the support you need, and answers too. Barb

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Hi Nicky, and welcome, I knew from day one something was wrong with

my son Matt, the only time he didnt cry and scream is when he was

untouched and in absolute quiet. I left my husband when Matt was

just 2 months old, and thank God he has nothing to do with us at

all. I am remarried to a wonderful man who no one would ever

believe wasnt the biological father of all my children. He is there

through thick and thin expecially with Matt. I think any illness

can be a strain on marriages and families. You just have to work as

a team and remember that everyone handles things differently and you

have to respect that in one another, kinda agree to disagree, but

you must still meet in the middle when it comes to caring for the

child, no matter how different the aproach. And it takes awhile to

get a system going as far as siblings and husband, but with patience

and time things do fall into place, and somehow just to start

running somewhat smoothly again. Although our household is not

normal to most, when we started being organized with all the medical

equip, and doc appts., and such, this has become just as routine a

life as anyone elses. Sure there are days that are hard and

unorganized, i make sure i get a good cry in, and then some rest and

then get on with things again. I also have mito adult onset, and i

would have never believed anyone telling me when i first became ill

that things would get back to " normal " , but to my surprise even on

my worse days, i get through that day, i am blessed with a

supportive husband and my other two children are 19 and 22 years of

age, so they are a big big help, dont think i could do it without

thier love and support. And even when other family members dont

understand or cant handle what goes on, i always have this group to

turn to and that has made a big difference in the way i deal with

things too. Hang in there, and hang out here at this group you will

find the support you need, and answers too. Barb

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My son's story is very similar to yours. is now 3.5 and just

started to take his first steps in a walker. Around age 2.5 he started

scooted everywhere and then by age 3 he started to sit up and now can

take a few steps with a walker -- it's hard to keep up with it

sometimes.

was also diagnosed with profound bilateral sensorineural hearing

loss by 3 months of age and his implant was put in at 7 months. Even

with his implant would get tired of hearing and want it off by

lunch time. By the time he was two it was no longer working for us. The

audiologist would reprogram the implant but no matter what they would

try the result was the same -- no response. I convinced the doctor's to

explant him and perform a MRI. We soon discovered that 's PONS is

malformed (almost a molar tooth sign) and that CN VIII was no longer

active. So now we use sign language to communicate with .

My husband was devastated at first. He didn't want anything to do with

our disabled son, but he soon decided to pitch in. Now, he cooks,

cleans, stays home with and gives me a break. He even lets me take

our daughter on vacation without him and ! I don't know what I

would do without him around.

Sue Ann Bube

son with mito

Hi, I new to post to this group. I have a son who is 2.5 yrs.

diagnosed with pyruvate dehydrogen. deficiency. He was diagnosed at

7 mos. I knew there was something wrong at 3 mos.. I am an

audiologist and my son with diagnosed with a progressive hearing

loss at 6 weeks. As an infant, he screamed all day long. He had

severe head lag. I knew there was something wrong with him. I asked

to see a neurologist at 4 mos. They also thought due to the

progressive nature of his hearing loss that there was something

genetically wrong. They did a ton of labs. Found that he had

severe lactate acidosis. He immediately was put on DCA. Which

revealed a significant improvement. No increased acidosis. His is

now 2, Isaiah is the happiest boy ever. He has a g-tube, deaf (with

a cochlear implant), doesn't sit up, still with poor head control.

He is such a wild man. He rolls all over. We are so glad to be

blessed with him.

I guess my question is for parents with these sick kids. How has

your life been affected by this. Do you sometimes feel out of

control. Am I the only on that has a strained marriage. HOw do you

keep up.

Nicky

Please contact mito-owner with any problems or

questions.

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> I guess my question is for parents with these sick kids. How has

> your life been affected by this. Do you sometimes feel out of

> control. Am I the only on that has a strained marriage. HOw do

you

> keep up.

>

My 2year old (lea) was diagnosed at 7mos old with partial complex

one. She still has low muscle tone, but it has increased through use

of the mito cocktail. She still isn't walking, although we are

hopeful that she will one day take her first steps.... She gets

around by scooting on her butt- and she can move fast too! It's the

cutest thing. there's a little girl at church who is the same age as

our Lea- and of course she's been walking now for more then a year.

Anyway, a couple weeks ago during bible study Lea was scooting

around the room on her butt... and this other little girl sat down

next to her and started scooting around beside her. It was so

sweet... brought tears to my eyes.

Anyway... this disease has forever changed our lives. We feel

blessed to have Lea and all 6 of her healthy siblings... yet

sometimes when I look at her I wonder why SHE was chosen to have

this. Our youngest baby is 5 1/2 weeks old and I feel like i cannot

relax and enjoy her baby-hood because I'm always watching and

waiting to find out if she has it too.

le

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Our life changed dramatically after Asenath became affected and then

again after we knew Zipporrah too was affected. We entered into the Mito

realm in a hard way with intense migraines and a major stroke paralyzing

Asenath's left side when she was still a toddler. We had to learn to take

it day by day and just try the best we could to provide for her needs, while

at the same time dealing with the stress of knowing we weren't able to stop

her pain and suffering. We went from having no meds regularly other than

asthmatic ones, to 12 meds for Asenath and 5 meds for Zipporrah given up to

three times daily for some of them. I have had to start going to Mayo on a

regular basis to see tons of specialists, started having therapy (SP/cog,

OT, PT) for three different children, needing leg braces for two, a

wheelchair for one, and tons of other changes.

Like any marriage we have our good days and our bad days, but we have

been able to pull together rather than apart when things get rough so we are

doing good. My husband has never been one to deny something is wrong and

always wants to be informed and a part of the med visits and treatment

plans. In the past half of a year he has had to stop going to doc visits

due to his job and the fact that when I go to Mayo it often ends up being a

4-5 day run at minimum (something I am hoping to lessen).

The way I keep up is to relax my expectations knowing I am not a

super-mom and getting accomplished what needs to be done, but after that not

stressing about what I unnecessary things I accomplish. There are days that

I seem to have to talk to way too many docs, order too many meds, give

asthma treatments and steam shower treatments, etc., but I always know that

each day is different and tomorrow may be better. I have found that a

cheerful attitude goes a long way toward feeling better, more in control,

and less flustered/crazy.

We all have our coping mechanisms and have our good days and our bad

days. Don't feel down when you feel overwhelmed. All of us get that way

and need a break. We are just moms (and a few dads and grandmas) trying to

care for our kids the best way we can, and sometimes it just wears us out.

Hope you are feeling more energized and positive today. :)

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)

son with mito

>

>

>

> Hi, I new to post to this group. I have a son who is 2.5 yrs.

> diagnosed with pyruvate dehydrogen. deficiency. He was diagnosed at

> 7 mos. I knew there was something wrong at 3 mos.. I am an

> audiologist and my son with diagnosed with a progressive hearing

> loss at 6 weeks. As an infant, he screamed all day long. He had

> severe head lag. I knew there was something wrong with him. I asked

> to see a neurologist at 4 mos. They also thought due to the

> progressive nature of his hearing loss that there was something

> genetically wrong. They did a ton of labs. Found that he had

> severe lactate acidosis. He immediately was put on DCA. Which

> revealed a significant improvement. No increased acidosis. His is

> now 2, Isaiah is the happiest boy ever. He has a g-tube, deaf (with

> a cochlear implant), doesn't sit up, still with poor head control.

> He is such a wild man. He rolls all over. We are so glad to be

> blessed with him.

>

> I guess my question is for parents with these sick kids. How has

> your life been affected by this. Do you sometimes feel out of

> control. Am I the only on that has a strained marriage. HOw do you

> keep up.

>

> Nicky

>

>

>

>

>

>

>

>

>

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

>

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It looks like I somehow missed your message to the mito group,

sorry. My son is 3 1/4 and also has PDH deficiency. Where do you live?

We are in upstate NY. Sheldon can walk but cannot talk. He seems to

hear fine but has sensory issues. He can't say any words. I'd love to

talk with someone else with PDH def. Is your son on a special diet?

Sheldon is also very happy as long as he's not sick or in pain.

I definitely feel like we have no control.

Hi, I new to post to this group.

I have a son who is 2.5 yrs.

diagnosed with pyruvate dehydrogen. deficiency. He was diagnosed

at

7 mos. I knew there was something wrong at 3 mos.. I am

an

audiologist and my son with diagnosed with a progressive hearing

loss at 6 weeks. As an infant, he screamed all day long.

He had

severe head lag. I knew there was something wrong with him. I

asked

to see a neurologist at 4 mos. They also thought due to the

progressive nature of his hearing loss that there was something

genetically wrong. They did a ton of labs. Found that he

had

severe lactate acidosis. He immediately was put on DCA.

Which

revealed a significant improvement. No increased acidosis.

His is

now 2, Isaiah is the happiest boy ever. He has a g-tube, deaf

(with

a cochlear implant), doesn't sit up, still with poor head control.

He is such a wild man. He rolls all over. We are so glad

to be

blessed with him.

I guess my question is for parents with these sick kids. How

has

your life been affected by this. Do you sometimes feel out

of

control. Am I the only on that has a strained marriage.

HOw do you

keep up.

Nicky

Please contact mito-owner with any problems or

questions.

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