DWQA QuestionsCategory: QuestionsBest time to start carnitine supplementation
Nick S. asked 9 months ago
Our sons free carnitine level was 16 with a lower normal range of 20. His total carnitine level was 25 with a lower normal range of 27. We saw Dr. Vockley had mentioned in a video blog that most of his MCADD of his patients do not get placed on carnitine supplementation unless they have levels of free carnitine under 12. Our specialist has advised us to give our son 2ml twice a day. Our son has been pretty good so far without any hypoglycemic episodes without carnitine. We have not been through any real illnesses yet. We have not started the carnitine supplementation.

We are looking for guidance on if we should be starting this yet, We have seen where carnitine levels will change independent of use of carnitine supplementation.

*Are there any negative effects of giving the supplementation?

* What is the benefit of starting carnitine?

* How often should his carnitine levels be checked if he starts the supplementation?

* How do we know when he is able to come off the supplement?

* What is the recommended starting point for carnitine supplementation

I really appreciate your help!
Nick S.

1 Answers
Jerry Vockley answered 9 months ago
Mr. Stojkovski,

The use of carnitine supplement in MCAD deficiency is a complicated an controversial issue. Patients were initially identified as having low carnitine, sometimes near zero, in the early days following identification of the disease. Amazingly, they did not seem to have the same symptoms as patients with a primary carnitine transporter deficiency and similar levels. Initially, most metabolic physicians treated the number and not the patient by starting supplementation. However, over the past few years, the pendulum has swung the opposite way with most of us not treating the number if the patient had no symptoms. So the bottom line is that you really need to work with your own metabolic physician to consider if it needs to be treated and what criteria will be used for stopping. I no longer routinely check the carnitine level in my patients unless they are showing symptoms of deficiency (specifically, muscle weakness). Supplementation should start at the lower end of the treatment range (25 mg/kg/day) and not exceed 1000 mg daily.

I hope this is helpful. Dr. Vockley