Re: Re: Exercise Intolerance ??s

[Guest guest]

In a message dated 6/27/02 5:23:09 AM Pacific Daylight Time,

rebecpeck@... writes:

<< I have noticed that Spencer does much better in the morning since I

started carbo loading him. I checked his blood sugar one day and it

was normal. I have been waiting for a really off day before testing

again but a friend whom has a Diabetic son suggested I test Spencer

every day for a week to get a baseline of " normal " for him.

Otherwise the " off " sick day may not tell me much.

I need to really watch how he does carefully following his exercise

routines to see if he turns off during or after exercise.

Thanks,

Bec

>>

Bec,

Has Spencer had an acylcarnitine profile when sick? Or, have the docs tried

a Carnitor trial on him anyway? was having severe exercise

intolerance, which improved somewhat with the carb loading, Bicitra, but it

didn't improve greatly until he was on Carnitor. While 's exercise

intolerance has not improved greatly on the same regimen, he is much less

likely to spill ketones after exercising (which he was doing previously).

Dena

[Guest guest]

In a message dated 6/27/02 3:46:10 PM Pacific Daylight Time,

rebecpeck@... writes:

<< When Spencer was hospitalized for dehydration and vomiting. I know

they tested his Carnitine levels, normal. His ketones were elevated

but they said that is not uncommon with dehydration and vomiting.

Last check his ketones were normal. >>

Do you have his ketone levels from when he was sick? Did they do urinary

organic acids?

Regarding the carnitine levels, 's were normal too. ( had a

ratio of 1.2, which was reported to me as normal, because the doc thought it

was artificially inflated because of all the ketone bodies attaching to the

short chain acylcarnitines, but others have told me that it is not normal at

1.2, no matter what.) Anyway, regarding normal levels-with 96% of the

carnitine being stored in the muscles/tissue and only 4% in the blood (I

think I have this correct), that blood levels can be normal when

tissue/muscle deficiencies exist. In 's case, there is NO QUESTION

as to Carnitor's effectiveness. will tell you that he never wants

to stop it. He went from a child falling asleep on the bus on the way home

from school, wanting to go to bed at 6:30 (which is not his normal!), muscle

aches/fatigue, moodiness, irritability....to total normal energy, no muscle

pains, moodiness etc. within 72 hours of being on the Carnitor!!!

Dena

[Guest guest]

In a message dated 6/27/02 7:56:05 PM Pacific Daylight Time,

rebecpeck@... writes:

<< Is carnitine a supplement you can get over the counter? If it is, I

would like to play lab rat with myself and hubby. We both have

muscle aches and fatigue. I don't ache as much as I use to before I

ditched the milk proteins.

>>

It is over the counter, but NOT nearly as good as Carnitor. Others will

support this. I don't think it could hurt Spencer and would ask the doc for

a trial.

I'm confused about the March 31/April 2 lab tests. You said Ketones

negative, yet urinary organic acids up. Do you have the specific results?

Thanks, Dena

[Guest guest]

In a message dated 6/28/02 10:40:03 AM Pacific Daylight Time,

sawyerjc@... writes:

<< You can also have normal levels of carnitine and still have the ration

of free carnitine to acylcarnitine free be off. If you have normal

carnitine levels, but they are predominately acylcarnitine, that is

bound to the toxins in the blood, then you that is not normal. It is

the ratio that is equally important.

Jeannine >>

's free carnitine was 29 (normal 21-53), short chain acylcarnitine was

30 (normal 3-11) and long chain acylcarnitine was 5 (normal 2-7). The ratio

was 1.2 (normal <0.4). But, the doc told us it was normal " because it was

the ketone bodies which were falsely causing an increase in the short chain

acylcarnitines. "

I do know that has been more resistent to ketosis since going on the

Carnitor, even though he has not noticed an energy increase or an improvement

in his myopathy yet.

Dena

[Guest guest]

waislandgirl@... wrote:

> Regarding the carnitine levels, 's were normal too. (

> had a ratio of 1.2, which was reported to me as normal, because the

> doc thought it was artificially inflated because of all the ketone

> bodies attaching to the short chain acylcarnitines, but others have

> told me that it is not normal at 1.2, no matter what.) Anyway,

> regarding normal levels-with 96% of the carnitine being stored in the

> muscles/tissue and only 4% in the blood (I think I have this correct),

> that blood levels can be normal when tissue/muscle deficiencies exist.

You can also have normal levels of carnitine and still have the ration

of free carnitine to acylcarnitine free be off. If you have normal

carnitine levels, but they are predominately acylcarnitine, that is

bound to the toxins in the blood, then you that is not normal. It is

the ratio that is equally important.

Jeannine

[Guest guest]

rebecpeck wrote:

> * Plasma, total carnitine was 33.3 mm/l which is in the normal

> range; Free carnitine was 25.8 mm/l which was slightly below the

> normal and acyl-carnitine/carnitine esters was 7/5mm/l which is in

> the normal range. The ester to free ratio was 0.3 which is in the

> normal range.

This is what I was referring to. The esters they are talking about are

the acylcarnitines that I referred to. If the ratio is more than 0.4,

than it is considered (in the realm of metabolic disease) to be

abnormal. Although some labs actually use a higher ratio as their norm.

Jeannine

[Guest guest]

Bec

Some lab values are abnormal if below a certain range, rather than above.

I've not looked this one up to see if that is the case. If so, the statement

would be correct, even if confusing.

laurie

PS - Just a reminder for everyone - Carotene and Carnitine are two different

things.

>

> * Carotene was elevated slightly below normal at 21. (This is

> EXACTLY what the report says... elevated below normal... hmmmm, I

> would have used a different word choice here... LOL)

[Guest guest]

In a message dated 6/28/02 5:53:31 PM Pacific Daylight Time,

sawyerjc@... writes:

<< By the way, have

you ever tried biotin for the ketosis?

Jeannine >>

Nope..had never heard of it prior to the mito conference in Dallas. Our

metabolic doc is trying to get a conference together with Dr. Saneto and our

PCP to determine a course of action. I've told them that I either want to go

to Dr. Whiteman at Mayo or to Dr. Cohen for an eval and then have CHMC in

Seattle f/u. Or, if Saneto feels comfortable that the testing besides muscle

bx can be done in Seattle, then we can go to Cohen or Shoffner for bx only.

It's hard to tell what will happen until the Feldman issue is cleared up.

Right now, I don't feel like we are getting unbiased, maximal care at

Seattle, in spite of the genetics/metabolic doc being very bright and now

evaluating for possible mito. As he wrote in the note received yesterday

" We still have not ruled out many other forms, including mitochondrial

disorders of energy metabolism. The mother is appropriately concerned

because of 's continued weakness and muscle atrophy, primarily in the

shoulder girdle. "

Dena

[Guest guest]

waislandgirl@... wrote:

> 's free carnitine was 29 (normal 21-53), short chain

> acylcarnitine was 30 (normal 3-11) and long chain acylcarnitine was 5

> (normal 2-7). The ratio was 1.2 (normal <0.4). But, the doc told us

> it was normal " because it was the ketone bodies which were falsely

> causing an increase in the short chain acylcarnitines. "

I am no expert, but it is my understanding that the ratio is significant

regardless. The way it was explained to me is that you want to look at

the total amount of carnitine in one respect, to see if they are

deficient. But if the total amount is elevated, and appears normal

because there is a signficant percentage of it that is " bad " carnitine

or carnitine esters, than that is not the same as having " enough " good

carnitine available to do it's job.

carnitine detoxifies the body, among other things, and it attaches to

the toxins and forms these esters. Once they are esters they are

supposed to be wasted from the system, not hang around. It is the Free

carnitine that is the good stuff and that is where the ration of 0.4

comes in. Just my thoughts....

I have to wonder if this isn't why is responsive to the

carnitor. It also improves his ability to metabolize fats, which is

probably why he seems to be more resistant to ketosis. By the way, have

you ever tried biotin for the ketosis?

Jeannine

>

>

> I do know that has been more resistent to ketosis since going

> on the

> Carnitor, even though he has not noticed an energy increase or an

> improvement

> in his myopathy yet.