Re: Carnitine or Vitamin E

[Guest guest]

The EPA would be next as you already know- then the vitamin E. You

can after that " try " adding carnosine or carn-aware http://www.carn-aware.com

as that is benign but you do NOT want to supplement with

the amino acid carnitine unless you get the blood draws first to see

if your child is deficient. Not because it's hurtful but because

there appears to be a higher than normal number of children in this

group that test low in the amino acid carnitine (T instead of S) and

once you start to supplement it would change the blood draw results

and you may not know if your child would test deficient. If a child

does test low in carnotine over time that can be very serious and is

linked to sudden death as carnitine supports the muscles around the

heart. So in other words you may not notice any difference when your

child is on or off the carnitine -but if he or she needs to take it

you want them to be under a medical doctor's supervision and on a

prescription formula and there's a chance that they may need to

supplement with this for life. Unless you know -you wouldn't know

and neither would your child as he grew to his teens and into being

an adult. Most people don't know they are carnitine deficient until

it's too late...So the bad news is if your child tests low in

carnitine but the good news is finding out before it's too late!

There are many archives on this and many parents in this group who do

have their children on prescription carnitine. It is highly

recommended if your child tests low to seek out a geneticist who

specializes in metabolic disorders. Carnitine is the only reason I

put my doing amazing 12 year old through all the blood draws a year

or so ago -and he tested normal (thank God!) Just to let you know

however -I never did " try " adding carnitine for him. Funny enough if

carnitine levels are too high you'll develop a fishy smell!

(and why give something they don't need?!)

" At doses of approximately 3 grams/day, carnitine supplements may cause nausea,

vomiting, abdominal cramps, diarrhea, and a " fishy " body odor [1-2]. More rare

side effects include muscle weakness in uremic patients and seizures in those

with seizure disorders "

http://ods.od.nih.gov/factsheets/carnitine.asp

Here's more from an archive on EFAs and what to add next

/message/77247

From Speechville here's an old post of what I saw after I added each new thing:

http://www.speechville.com/alternative-therapy/efa-faqs.html

archive from here on carnitine

/message/81150

EFA info from CHERAB (new site coming soon thanks to and others!!)

http://www.cherab.org/information/indexinformation.html#diet

PS -Tanner's regular pediatrician ordered all the blood draws -all covered by

our BC BS insurance.

=====

[Guest guest]

Janice I say that carnotine is benign -but a deficiency may have no

symptoms but is linked to sudden death. A deficiency in carnitine is

typically very rare- so to me as a parent of a child that falls into

this group it was alarming how here many are testing low. Here's some

information on the various types of carnitine deficiency from NORD

http://www.bchealthguide.org/kbase/nord/nord60.htm

Whether you supplement with vitamin E or not is not a life and death

decision -carnitine could be. As it says in the link below

" the first clinical manifestation in asymptomatic individuals with

primary carnitine deficiency may be sudden death. This also may occur

in patients with secondary carnitine deficiency as a consequence of

ventricular tachycardia or fibrillation. "

http://www.emedicine.com/ped/byname/carnitine-deficiency.htm

To me it's a no brainer to make sure you get your child tested prior

to supplementation. I know you are in Canada so perhaps it's harder

to get the blood draws covered as I'm not familiar with health

coverage there -but here in the US if you know how to advocate you

can get these blood draws ordered by your child's pediatrician and

covered by insurance. As parents most of us (you too right?) come up

with private out of pocket payment for alternative treatment even if

it's not covered.

If the research ever gets funded one day a diagnosis of apraxia will

probably mean an automatic testing. For now we have the group

results. As always it's up to each of us as parents to take the

information shared here and make the decision you believe best for

your child or children.

It would be pretty clear if you were deficient in vitamin E -in other

words you'd get a warning. Again as it says all over the net -the

way most people learn a loved one is deficient in carnitine is after

they die during autopsy -there may be no signs at all during life.

(which means you have to supplement even with no WOW changes)

Below are cuts about vitamin E and carnitine deficiency from

reputable sources. If after reading this one chooses to just

supplement at least I know I can't feel guilty that I did not share

this.

(BTW I was told you can supplement E and fish oil without testing)

Vitamin E deficiency is rare in humans. There are three specific

situations when a vitamin E deficiency is likely to occur.

persons who cannot absorb dietary fat due to an inability to secrete

bile or with rare disorders of fat metabolism are at risk of vitamin

E deficiency [8];

individuals with rare genetic abnormalities in the alpha-tocopherol

transfer protein are at risk of vitamin E deficiency [9]; and

premature, very low birth weight infants (birth weights less than

1500 grams, or 3 pounds, 4 ounces) are at risk of vitamin E

deficiency [3,10].

Blood levels of vitamin E may also be decreased with zinc deficiency

[11]. Vitamin E deficiency is usually characterized by neurological

problems associated with nerve degeneration in hands and feet [5].

These symptoms are also associated with other medical conditions. A

physician can determine if they are the result of a vitamin E

deficiency or are from another cause.

http://ods.od.nih.gov/FACTSHEETS/VITAMINE.ASP#h4

" Carnitine deficiency

Sudden death: Unfortunately, the first clinical manifestation in

asymptomatic individuals with primary carnitine deficiency may be

sudden death. This also may occur in patients with secondary

carnitine deficiency as a consequence of ventricular tachycardia or

fibrillation.

Heart failure: Patients with primary carnitine deficiency develop a

progressive cardiomyopathy that usually presents at a later age. The

cardiac function does not respond to inotropes or diuretics. If the

condition is not diagnosed correctly and no carnitine is

supplemented, progressive heart failure eventually leads to death.

Heart failure caused by dilated cardiomyopathy may be the presenting

syndrome in patients with secondary carnitine deficiency caused by

defects in beta-oxidation, such as long-chain 3-hydroxyacyl-CoA

dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase

(VLCAD) deficiency.

Hypoglycemic hypoketotic encephalopathy: Acute encephalopathy

accompanied by hypoketotic hypoglycemic episodes usually presents in

younger infants with primary carnitine deficiency. Periods of fasting

in association with viral illness trigger these acute episodes. Some

patients have developmental delay and central nervous system

dysfunction associated with these episodes. If no carnitine

replacement is given, recurrent episodes of encephalopathy may ensue. "

http://www.emedicine.com/ped/byname/carnitine-deficiency.htm

As a parent if you compare testing for a carnitine deficiency to a

vitamin E deficiency - it's possible that you don't really understand

it and thus even if you stay on top of it while he's living in your

home - your child won't know of the extreme importance of staying on

top of this with a medical doctor once he's out of your house. I was

also told by that it's best to have the prescription

carnitine so you are sure of the quality vs. OTC.

My fear is that being the numbers are high here and our group started

around 10 years ago -10 years from now I don't want to hear about any

sudden deaths. Also the advice is not my advice -it's the advice

from medical doctors like Dr. .

=====

[Guest guest]

,

Carnitine is also benign and is definately warranted for cases of global

hypotonia. While it is nice to get levels checked for deficiency, it is not

absolutely necessary. All of our children ideally should get thorough metabolic

testing. It really is a good thing to do but many, many people begin

supplementation prior to testing.

Why do you not advocate that we test for vitamin E deficiency before dosing it

as well? It is lovely to get levels checked, I agree, but not always

expeditious for everyone.

Janice

Mother of Mark, 13

[sPAM][ ] Re: Carnitine or Vitamin E

The EPA would be next as you already know- then the vitamin E. You

can after that " try " adding carnosine or carn-aware http://www.carn-aware.com

as that is benign but you do NOT want to supplement with

the amino acid carnitine unless you get the blood draws first to see

if your child is deficient. Not because it's hurtful but because

there appears to be a higher than normal number of children in this

group that test low in the amino acid carnitine (T instead of S) and

once you start to supplement it would change the blood draw results

and you may not know if your child would test deficient. If a child

does test low in carnotine over time that can be very serious and is

linked to sudden death as carnitine supports the muscles around the

heart. So in other words you may not notice any difference when your

child is on or off the carnitine -but if he or she needs to take it

you want them to be under a medical doctor's supervision and on a

prescription formula and there's a chance that they may need to

supplement with this for life. Unless you know -you wouldn't know

and neither would your child as he grew to his teens and into being

an adult. Most people don't know they are carnitine deficient until

it's too late...So the bad news is if your child tests low in

carnitine but the good news is finding out before it's too late!

There are many archives on this and many parents in this group who do

have their children on prescription carnitine. It is highly

recommended if your child tests low to seek out a geneticist who

specializes in metabolic disorders. Carnitine is the only reason I

put my doing amazing 12 year old through all the blood draws a year

or so ago -and he tested normal (thank God!) Just to let you know

however -I never did " try " adding carnitine for him. Funny enough if

carnitine levels are too high you'll develop a fishy smell!

(and why give something they don't need?!)

" At doses of approximately 3 grams/day, carnitine supplements may cause

nausea, vomiting, abdominal cramps, diarrhea, and a " fishy " body odor [1-2].

More rare side effects include muscle weakness in uremic patients and seizures

in those with seizure disorders "

http://ods.od.nih.gov/factsheets/carnitine.asp

Here's more from an archive on EFAs and what to add next

/message/77247

From Speechville here's an old post of what I saw after I added each new

thing:

http://www.speechville.com/alternative-therapy/efa-faqs.html

archive from here on carnitine

/message/81150

EFA info from CHERAB (new site coming soon thanks to and others!!)

http://www.cherab.org/information/indexinformation.html#diet

PS -Tanner's regular pediatrician ordered all the blood draws -all covered by

our BC BS insurance.

=====

[Guest guest]

As someone who had kids who threw hunger strikes (not picky

eating...full on hunger strikes) I find this very interesting:

Periods of fasting

> in association with viral illness trigger these acute episodes. Some

> patients have developmental delay and central nervous system

> dysfunction associated with these episodes. If no carnitine

> replacement is given, recurrent episodes of encephalopathy may ensue. "

> " Carnitine deficiency

>

> Sudden death: Unfortunately, the first clinical manifestation in

> asymptomatic individuals with primary carnitine deficiency may be

> sudden death. This also may occur in patients with secondary

> carnitine deficiency as a consequence of ventricular tachycardia or

> fibrillation.

>

> Heart failure: Patients with primary carnitine deficiency develop a

> progressive cardiomyopathy that usually presents at a later age. The

> cardiac function does not respond to inotropes or diuretics. If the

> condition is not diagnosed correctly and no carnitine is

> supplemented, progressive heart failure eventually leads to death.

>

> Heart failure caused by dilated cardiomyopathy may be the presenting

> syndrome in patients with secondary carnitine deficiency caused by

> defects in beta-oxidation, such as long-chain 3-hydroxyacyl-CoA

> dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase

> (VLCAD) deficiency.

>

> Hypoglycemic hypoketotic encephalopathy: Acute encephalopathy

> accompanied by hypoketotic hypoglycemic episodes usually presents in

> younger infants with primary carnitine deficiency. Periods of fasting

> in association with viral illness trigger these acute episodes. Some

> patients have developmental delay and central nervous system

> dysfunction associated with these episodes. If no carnitine

> replacement is given, recurrent episodes of encephalopathy may ensue. "

> http://www.emedicine.com/ped/byname/carnitine-deficiency.htm

[Guest guest]

Jancie,

I could not agree more with you on Carnatine. Charlie's was on the low side

of normal. We choose to suppliment at the advice of our nutritionist. Seems

to help here! Also I found interesting, that Caroline's PediaSure,

reccommended by ped. MD does have Carnatine in it. It is also riddled w/

MSG, so I stopped giving it. He advised me to keep her on formula.

Thanks,

Colleen

[Guest guest]

This all depends on whether you have primary carnitine deficiency (extremely

rare) or secondary carnitine deficiency as most of our kids DO have.

BUT.... to NOT supplement because you might be deficient is a true contradiction

of terms. Doesn't make sense to me. If you google it.... carnitine is a

regularly used supplement by physicians to combat chronic fatigue syndrome,

hypotonia, diabetes, as well as many other ailments.

It is completely benign and can only do good for the system. Since having a

child who IS affected by hypotonia I am aware that every month you go without

developing muscle tone, the more milestones that are missed for the child.....

you can't get those back without therapy so.....

I started supplementing with carnitine for over 3 months and still tested out

low so my viewpoint is that you can start this immediately with the children and

await any screenings. Since not having carnitine in the system may result in

SUDDEN DEATH...... I would think that most parents would want to supplement

rather then wait!!! You can ask your physician how many days you should stop

supplementing prior to any blood draws so that results are not overly skewed.

The half life of carnitine in the blood is about 4 hours?.... I believe that I

read that but my understanding of biochemistry is definately weak! Anyhow, not

so certain about that one since I am not a doctor nor a biochemist but this is

certainly a question worth asking..... How many days prior to blood draws do

you stop with supplementation? But..... since the results can be so dramatic

with Acetyl-l-carnitine for kids with hypotonia, it is a good thing to do.

You WILL NOT hurt your child by supplementing with it..... you can only give

them benefits.

That is just my opinion.... coming from a mom whose child does have global

dyspraxia and hypotonia and seeking the best for her child. Hypotonia is aweful

and anything you can do to help the body..... well, you should do it.

From what I understand.... carnosine is often not well tolerated by many of the

children. Mark does just fine with it but it is definately one supplement that

many parents report issues with in their kids.... ie. adverse behaviours so be

careful if you choose it.

Neither of us are doctors and parents must do their own research and decide what

is best for themselves..... Obviously, the more testing you can do, the better

but you don't always need to wait for testing to begin supplementing, imo.

Janice

Mother of Mark, 13

[sPAM]Re:[ ] Re: Carnitine or Vitamin E

Janice I say that carnotine is benign -but a deficiency may have no

symptoms but is linked to sudden death. A deficiency in carnitine is

typically very rare- so to me as a parent of a child that falls into

this group it was alarming how here many are testing low. Here's some

information on the various types of carnitine deficiency from NORD

http://www.bchealthguide.org/kbase/nord/nord60.htm

Whether you supplement with vitamin E or not is not a life and death

decision -carnitine could be. As it says in the link below

" the first clinical manifestation in asymptomatic individuals with

primary carnitine deficiency may be sudden death. This also may occur

in patients with secondary carnitine deficiency as a consequence of

ventricular tachycardia or fibrillation. "

http://www.emedicine.com/ped/byname/carnitine-deficiency.htm

To me it's a no brainer to make sure you get your child tested prior

to supplementation. I know you are in Canada so perhaps it's harder

to get the blood draws covered as I'm not familiar with health

coverage there -but here in the US if you know how to advocate you

can get these blood draws ordered by your child's pediatrician and

covered by insurance. As parents most of us (you too right?) come up

with private out of pocket payment for alternative treatment even if

it's not covered.

If the research ever gets funded one day a diagnosis of apraxia will

probably mean an automatic testing. For now we have the group

results. As always it's up to each of us as parents to take the

information shared here and make the decision you believe best for

your child or children.

It would be pretty clear if you were deficient in vitamin E -in other

words you'd get a warning. Again as it says all over the net -the

way most people learn a loved one is deficient in carnitine is after

they die during autopsy -there may be no signs at all during life.

(which means you have to supplement even with no WOW changes)

Below are cuts about vitamin E and carnitine deficiency from

reputable sources. If after reading this one chooses to just

supplement at least I know I can't feel guilty that I did not share

this.

(BTW I was told you can supplement E and fish oil without testing)

Vitamin E deficiency is rare in humans. There are three specific

situations when a vitamin E deficiency is likely to occur.

persons who cannot absorb dietary fat due to an inability to secrete

bile or with rare disorders of fat metabolism are at risk of vitamin

E deficiency [8];

individuals with rare genetic abnormalities in the alpha-tocopherol

transfer protein are at risk of vitamin E deficiency [9]; and

premature, very low birth weight infants (birth weights less than

1500 grams, or 3 pounds, 4 ounces) are at risk of vitamin E

deficiency [3,10].

Blood levels of vitamin E may also be decreased with zinc deficiency

[11]. Vitamin E deficiency is usually characterized by neurological

problems associated with nerve degeneration in hands and feet [5].

These symptoms are also associated with other medical conditions. A

physician can determine if they are the result of a vitamin E

deficiency or are from another cause.

http://ods.od.nih.gov/FACTSHEETS/VITAMINE.ASP#h4

" Carnitine deficiency

Sudden death: Unfortunately, the first clinical manifestation in

asymptomatic individuals with primary carnitine deficiency may be

sudden death. This also may occur in patients with secondary

carnitine deficiency as a consequence of ventricular tachycardia or

fibrillation.

Heart failure: Patients with primary carnitine deficiency develop a

progressive cardiomyopathy that usually presents at a later age. The

cardiac function does not respond to inotropes or diuretics. If the

condition is not diagnosed correctly and no carnitine is

supplemented, progressive heart failure eventually leads to death.

Heart failure caused by dilated cardiomyopathy may be the presenting

syndrome in patients with secondary carnitine deficiency caused by

defects in beta-oxidation, such as long-chain 3-hydroxyacyl-CoA

dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase

(VLCAD) deficiency.

Hypoglycemic hypoketotic encephalopathy: Acute encephalopathy

accompanied by hypoketotic hypoglycemic episodes usually presents in

younger infants with primary carnitine deficiency. Periods of fasting

in association with viral illness trigger these acute episodes. Some

patients have developmental delay and central nervous system

dysfunction associated with these episodes. If no carnitine

replacement is given, recurrent episodes of encephalopathy may ensue. "

http://www.emedicine.com/ped/byname/carnitine-deficiency.htm

As a parent if you compare testing for a carnitine deficiency to a

vitamin E deficiency - it's possible that you don't really understand

it and thus even if you stay on top of it while he's living in your

home - your child won't know of the extreme importance of staying on

top of this with a medical doctor once he's out of your house. I was

also told by that it's best to have the prescription

carnitine so you are sure of the quality vs. OTC.

My fear is that being the numbers are high here and our group started

around 10 years ago -10 years from now I don't want to hear about any

sudden deaths. Also the advice is not my advice -it's the advice

from medical doctors like Dr. .

=====

[Guest guest]

Thank you for putting it in order for me. I will look into having her

tested for the

carnitine.

>

> The EPA would be next as you already know- then the vitamin E. You

> can after that " try " adding carnosine or carn-aware http://www.carn-aware.com

> as that is benign but you do NOT want to supplement with

> the amino acid carnitine unless you get the blood draws first to see

> if your child is deficient. Not because it's hurtful but because

> there appears to be a higher than normal number of children in this

> group that test low in the amino acid carnitine (T instead of S) and

> once you start to supplement it would change the blood draw results

> and you may not know if your child would test deficient. If a child

> does test low in carnotine over time that can be very serious and is

> linked to sudden death as carnitine supports the muscles around the

> heart. So in other words you may not notice any difference when your

> child is on or off the carnitine -but if he or she needs to take it

> you want them to be under a medical doctor's supervision and on a

> prescription formula and there's a chance that they may need to

> supplement with this for life. Unless you know -you wouldn't know

> and neither would your child as he grew to his teens and into being

> an adult. Most people don't know they are carnitine deficient until

> it's too late...So the bad news is if your child tests low in

> carnitine but the good news is finding out before it's too late!

>

> There are many archives on this and many parents in this group who do

> have their children on prescription carnitine. It is highly

> recommended if your child tests low to seek out a geneticist who

> specializes in metabolic disorders. Carnitine is the only reason I

> put my doing amazing 12 year old through all the blood draws a year

> or so ago -and he tested normal (thank God!) Just to let you know

> however -I never did " try " adding carnitine for him. Funny enough if

> carnitine levels are too high you'll develop a fishy smell!

> (and why give something they don't need?!)

>

> " At doses of approximately 3 grams/day, carnitine supplements may cause

nausea,

vomiting, abdominal cramps, diarrhea, and a " fishy " body odor [1-2]. More rare

side

effects include muscle weakness in uremic patients and seizures in those with

seizure

disorders "

> http://ods.od.nih.gov/factsheets/carnitine.asp

>

> Here's more from an archive on EFAs and what to add next

> /message/77247

>

> From Speechville here's an old post of what I saw after I added each new

thing:

> http://www.speechville.com/alternative-therapy/efa-faqs.html

>

> archive from here on carnitine

> /message/81150

>

> EFA info from CHERAB (new site coming soon thanks to and others!!)

> http://www.cherab.org/information/indexinformation.html#diet

>

> PS -Tanner's regular pediatrician ordered all the blood draws -all covered by

our BC BS

insurance.

>

> =====

>