Re: Question - Lactic Acid

June 18, 1999

Kathy, are you saying the blueness around the mouth is a symptom of the

mito? A number of our kids and grandkids have this ( & other places on the

face like around the eyes and forehead) but we have never been able to

figure out the why other than we finally say obviously they are not getting

enough oxygen but it can't just be asthma as some of them don't have asthma.

S.

Re: Question - Lactic Acid

>

>>From: Cheri77777@...

>>

>>>She becomes blue around the mouth, gasps for air, she says her limbs

>>>

>>>feel like bricks and she gets alot of reflux

>>

>>Becky, not to alarm you, but has a physician seen her with the blue around

>>the lips? You may want to have that evaluated and at the every least

>mention

>>it to her primary doc.

>>

>>Cheri

>>

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

September 16, 1999

Don't know anything about PN401, but was wondering what the symptoms of lactic

acidosis are? My son's LA

was tested as normal, but I wonder if you need to be in a crisis for it to be

high. He has only vomited

twice in his life, once from the stomach flu, the other time I'm not sure why

(overeating I think). He

does not have seizures, etc., but does go through his irritable days where he is

unbearable to be around.

Is this a sign of high lactic acid? Or just a stubborn, opinionated

two-year-old?

" Sasser, Becky " wrote:

>

> Speaking of lactic acid, 's lactic acid is always high, sometimes

> higher then other times. I have often been very worried about her lactic

> acid! However, Dr. Cohen told us 's problems are not from the lactic

> acid. If we could get her complex's 2 & 3 working correctly together this

> would solve her problems! Has any one heard any more about PN401? Becky

> Question - Lactic Acid

>

> >From: KnoxVolNut@...

> >

> >What " red flags " could I watch for that might indicate an elevation in

> 's

> >lactic acid?

> >

> >Thanks!

> >

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

September 16, 1999

In a message dated 9/16/99 4:41:52 AM Pacific Daylight Time,

houllion@... writes:

<< disappointing, but

NOTHING ever works for Grant. >>

THis is how I feel most of the time to Gail.

September 16, 1999

Becky -

I have heard that the PN401 test is coming, but VERY slowly. It seems

that the drug co. is trying to get FDA approval to begin the testing.

We are just starting (today, in fact) to wean my son, Grant (11yrs,

intractable szs) from his trial on Uridine (the precursor to PN401). He

became noticeably worse at the onset, then drifted back into a slightly

better, but no better than his baseline, pattern of seizures. Dr. Cohen

said to discontinue it over the next two weeks - disappointing, but

NOTHING ever works for Grant.

Gail

Sasser, Becky wrote:

>

> Speaking of lactic acid, 's lactic acid is always high, sometimes

> higher then other times. I have often been very worried about her lactic

> acid! However, Dr. Cohen told us 's problems are not from the lactic

> acid. If we could get her complex's 2 & 3 working correctly together this

> would solve her problems! Has any one heard any more about PN401? Becky

> Question - Lactic Acid

>

> >From: KnoxVolNut@...

> >

> >What " red flags " could I watch for that might indicate an elevation in

> 's

> >lactic acid?

> >

> >Thanks!

> >

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

September 16, 1999

Speaking of lactic acid, 's lactic acid is always high, sometimes

higher then other times. I have often been very worried about her lactic

acid! However, Dr. Cohen told us 's problems are not from the lactic

acid. If we could get her complex's 2 & 3 working correctly together this

would solve her problems! Has any one heard any more about PN401? Becky

Question - Lactic Acid

>From: KnoxVolNut@...

>

>What " red flags " could I watch for that might indicate an elevation in

's

>lactic acid?

>

>Thanks!

>

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

September 16, 1999

>She becomes blue around the mouth, gasps for air, she says her limbs

>

>feel like bricks and she gets alot of reflux

Becky, not to alarm you, but has a physician seen her with the blue around

the lips? You may want to have that evaluated and at the every least mention

it to her primary doc.

Cheri

September 17, 1999

Gail we will find something that works for Grant and all our kids!!!!! Becky

Question - Lactic Acid

>>

>> >From: KnoxVolNut@...

>> >

>> >What " red flags " could I watch for that might indicate an elevation in

>> 's

>> >lactic acid?

>> >

>> >Thanks!

>> >

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

September 17, 1999

I can tell as soon as over exerts herself to much her lactic acid is

higher. She becomes blue around the mouth, gasps for air, she says her limbs

feel like bricks and she gets alot of reflux. is going to be 17 so

she is becoming smarter and knows what will set this off. Needless, to say

but she is still a teenager and sometimes she doesn't take her own warning

signs serious. Becky

Question - Lactic Acid

>>

>> >From: KnoxVolNut@...

>> >

>> >What " red flags " could I watch for that might indicate an elevation in

>> 's

>> >lactic acid?

>> >

>> >Thanks!

>> >

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

September 17, 1999

Becky,

I'm going to San Diego this Sunday to hopefully try this new drug on my son.

What I heard is this drug stops the mito chondria from deteriorating any more

than it is already .I have heard it works but is not approved yet.

I feel at least it is something for him to try that might just help. I just

called the UCSD of California and asked for the doctors to look over Jake's

records to see if they thought if would work for him.

They then asked us to come down,we live in Minnesota.

He also has a very high lactic level and thats what they look for.

September 18, 1999

Becky -

My son (complex IV) also has blue tinges around his mouth, on occasion. He

is only three so can't tell me how he feels when this happens, but I can

always tell it is a sign he is not feeling too good. I have never thought

to mention it to his doctor, but after what you and Cheri have written I

think I will. I thought it was just something that happened to .

.

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

September 18, 1999

Becky -

My son (complex IV) also has blue tinges around his mouth, on occasion. He

is only three so can't tell me how he feels when this happens, but I can

always tell it is a sign he is not feeling too good. I have never thought

to mention it to his doctor, but after what you and Cheri have written I

think I will. I thought it was just something that happened to .

.

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

September 18, 1999

Becky -

My son (complex IV) also has blue tinges around his mouth, on occasion. He

is only three so can't tell me how he feels when this happens, but I can

always tell it is a sign he is not feeling too good. I have never thought

to mention it to his doctor, but after what you and Cheri have written I

think I will. I thought it was just something that happened to .

.

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

September 18, 1999

Hi Cheri, 's neuro. knows about the blue around her mouth,she has seen

a cardiologist(said slight murmur return visit in 3 yr.) Dr. Cohen said just

a symptom of the mito. Thanks for asking. Becky

Re: Question - Lactic Acid

>From: Cheri77777@...

>

>>She becomes blue around the mouth, gasps for air, she says her limbs

>>

>>feel like bricks and she gets alot of reflux

>

>Becky, not to alarm you, but has a physician seen her with the blue around

>the lips? You may want to have that evaluated and at the every least

mention

>it to her primary doc.

>

>Cheri

>

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

September 18, 1999

I found the comment from Dr Cohen about 's color changes very

intriguing because has always had a blue cast around his lips, at the

botom of his feet and his palms. We exhausted the pulm. and cardio. routes

long ago and except for his rapid resps that are " normal " for him and the

newly diagnosed asthma he checked out a-ok. Do you think this is a generality

for kids with Mito or kids with Complex II-III like and ?

September 18, 1999

>I found the comment from Dr Cohen about 's color changes very

>intriguing because has always had a blue cast around his lips, at

>the

>botom of his feet and his palms. We exhausted the pulm. and cardio. routes

>

>long ago and except for his rapid resps that are " normal " for him and the

>

>newly diagnosed asthma he checked out a-ok. Do you think this is a generality

>

>for kids with Mito or kids with Complex II-III like and ?

>

,

I can't answer your question, but I can say that blueness IS a sign of

specific difficulty with my and not just a broad symptom in general. He

has however had spells of blue hands and feet for no apparent reason and

perhaps that is what Dr. Cohen is referring to and not the

cardio/pulmonary/autonomic stuff deals with as well.

Cheri

September 18, 1999

>I found the comment from Dr Cohen about 's color changes very

>intriguing because has always had a blue cast around his lips, at

>the

>botom of his feet and his palms. We exhausted the pulm. and cardio. routes

>

>long ago and except for his rapid resps that are " normal " for him and the

>

>newly diagnosed asthma he checked out a-ok. Do you think this is a generality

>

>for kids with Mito or kids with Complex II-III like and ?

>

,

I can't answer your question, but I can say that blueness IS a sign of

specific difficulty with my and not just a broad symptom in general. He

has however had spells of blue hands and feet for no apparent reason and

perhaps that is what Dr. Cohen is referring to and not the

cardio/pulmonary/autonomic stuff deals with as well.

Cheri

September 19, 1999

,

One of the rist symptoms I ever noticed with Lexi was blueness around her

mouth, on her forehead, and under her fingernails. She still gets this when

she is fatigued, or when she is having a seizure. We have a pulse oxymeter

at home to keep track of whether the bluing is an actual oxygen desaturation

or just her doing her weird thing of turning blue. Usually, when she is a

little blue but not seizing her sat levels go down to around 93 (nothing to

really stress out about). But she has gone as low as 60%. So I think the

anoxia is definately one of the mito symptoms.

My $0.02 worth.

ruth

September 19, 1999

,

One of the rist symptoms I ever noticed with Lexi was blueness around her

mouth, on her forehead, and under her fingernails. She still gets this when

she is fatigued, or when she is having a seizure. We have a pulse oxymeter

at home to keep track of whether the bluing is an actual oxygen desaturation

or just her doing her weird thing of turning blue. Usually, when she is a

little blue but not seizing her sat levels go down to around 93 (nothing to

really stress out about). But she has gone as low as 60%. So I think the

anoxia is definately one of the mito symptoms.

My $0.02 worth.

ruth

September 19, 1999

My son always had a chronic blueness around his mouth, as well as a

blue tinge to his extremities . His hands and especially feet are like

ice. We noticed that it would increase during physical therapy many times.

last winter our cardiologist put him on a holter monitor for 48 hours.

The results showed a normal sinus rhythm but the rate was extremely

variable - 40-160 - with many runs of bradycardia, (slow heart rate). Our

cardiologist feels that 's blueness is due to his autonomic nervous

system dysfunction and describes these 2 reasons for the " exaggerated sinus

arrhythmia " .

1. he has chronic gastro-esophogeal reflux which causes vagal nerve

stimulation which in turn causes bradycardia

2. The autonomic nervous system is not " fine tuned " and therefore we see

wide swings in rate.

What is interesting and also supports this description of our cardiologist

is that his blue spells decreased markedly when we took him off tube

feedings and put him on intravenous feeds. Until a month ago we had been

seeing a sharp decrease in his reflux and so too his blue spells. The past

months the reflux has been quite significant again and we and his

therapists and doctors have seen more of the circumoral blueness again.

Anne - mom to Brittany, Zachary, Abby and

(Zachary and Sam have complex 1 and 4 defect in OxPhos )

----------

We exhausted the pulm. and cardio. routes

> long ago and except for his rapid resps that are " normal " for him and the

> newly diagnosed asthma he checked out a-ok. Do you think this is a

generality

> for kids with Mito or kids with Complex II-III like and ?

September 19, 1999