RE: heavy vs light exercise

[Guest guest]

Barbara,

maybe you can help with this maybe not..my daughter when doing sports or

sick, has the problem that her aceto-acetic and ketones ( urine) go through the

roof...and she has metabolic acidosis but also her dicarbolic fatty acids go

very high...is that common with a fatty acid defect or CPT like her sister

has?

thanks

[Guest guest]

Very interested in your observations about heavy vs light exercise. To my

amateur mind, the fact that you tolerate heavy but not light exercise would

imply a dependence on fat metabolism for ATP, which in turn would fit very

well with your observation that you do better on an Atkins type diet. The

other implication would be that perhaps the primary defect could be in the

glycolytic pathways. My reasoning would go something like this: Glucose is

utilized as energy primarily at the beginning of exercise or during " light "

exercise, whereas fatty acids are not utilized at the beginning of exercise

but become increasing important after 20-30 minutes of exercise. After an

hour they represent the major energy supply. Individuals with defects in the

glycolytic pathways sometimes experience a " second wind " once they get past

the " glucose " stage of exercise into the " fatty acid " stage of exercise. I'm

not sure if length or intensity is what you mean by " heavy " and " light "

exercise, but what you seem to describe is sometimes what McArdle's patients

report. And what you describe is the opposite of what is reported by

patients with defects in fat metabolism.

Once again, I am very impressed with just how much patients with metabolic

disorders can sort out on their own through observing their response to diet

and exercise. These are crucial clues.

Barbara

_____

From: ohgminion

Sent: Sunday, January 16, 2005 10:45 PM

To:

Subject: Re: carbs and lactic acid

* I think my mito disease is secondary to another disorder, because I

do present differently than many on this list (for example, I

tolerate heavy exercise well, but can't tolerate light exercise).

But my muscle biopsy did show defects in the activity of different

mito complexes.

Take care,

RH

[Guest guest]

For sure I know the high dicarboxylic acids indicate a problem in fat

metabolism, either primary or secondary. Possibly the aceto-acetic stuff and

ketonuria do too, but I'm not positive on that one.

B

_____

From: MitomomX3@...

Sent: Monday, January 17, 2005 10:30 AM

To:

Subject: Re: heavy vs light exercise

Barbara,

maybe you can help with this maybe not..my daughter when doing sports or

sick, has the problem that her aceto-acetic and ketones ( urine) go through

the

roof...and she has metabolic acidosis but also her dicarbolic fatty acids

go

very high...is that common with a fatty acid defect or CPT like her sister

has?

thanks