Got to ask the experts RE Carnitine

[Guest guest]

Sorry, I really do not know how low is too low on the Carnitine. I do know, however, that a carnitine defficiancy is actually pretty serious if it is long term. Long term carnitine defficiancy can lead to muscle weakness, especially in the heart. Even with Carnitor, Grace has to go every year for an echo on her heart just to make sure everything is still O.K. Also for what it is worth, Grace was on Carnitor before she was ever even diagnosed with any type of metabolic disorder (actually before the labs even came back showing her defficancy.) Dr.Cohen said she had so many symptoms of a defficiancy that he thought it was in her best interest just to start it, and if needed then he could stop it. Thankfully he did start it, because she ended up with a pretty serious defficiancy. Before she started her Carnitor she had serious problems with her liver, and had some pretty bad food intolerances. She was very close to liver failure, since starting her on a big dose of Carnitor (1 1/2 years ago), her liver is functioning completely normal (with the exception of some permanant damage from before the Carnitor.) Dr. Cohen and I both agree that the Carnitor is her most important drug in her coctail of endless medications. Does your mito doc know about his defficiancy? I really don't understand why they do not want to give it to him yet. Carnitor is a completely benign drug so it will not hurt him in any way if he did not need it.

Sorry I am no help at all on the lactic acid, that is not a hurdle we have crossed yet.

Best wishes.

[Guest guest]

,

Is your son on carnitine supplementation now? I have severely low carnitine levels and am on IV Levo-Carnitine. Too low of carnitine levels can lead to a cardiomyopathy that can sometimes be fatal. I'm not trying to scare you but I would highly reccomend that you get me him on carnitine if the biopsy is very far off.

[Guest guest]

Hey we just got another set of Will's labs back, and his Carnitine is

dropping. The first time it was run in January his Total was 22 Free

was 13 and Acylcarnitine was 9 Now total is 17 free is 10 and

Acylcarinitine is 4. So a fairly significant drop, but what I was

wondering is how low is too low. Is there a danger point in all of

this, is this why Will has been feeling so cruddy lately? I can find

info on Carnitine deficiencies but not finding anything on what happens

if it gets too low. Just trying to figure out what to do. We know he

needs supplementation, but nobody will let us start until the biopsy is

done. So we are just waiting and watching.

Also can anyone explain the difference between Lactate and Lactic Acid.

We've had both run lately and they were high, but everything I'm

finding uses the two interchangably. Are they the same, are they

different, when do we worry. I know in the one chat Dr Cohen said

Lactate by itself is not dangerous but just a sign of a problem, is

lactic acid dangerous.

I'm watching Will deteriorate in front of me and don't know what to do,

We have a great Dr but I can't get past his staff to relay questions

for me, so I come here.

Thank you

In Colorado