DWQA QuestionsCategory: QuestionsCPT II deficiency – Dojolvi, leucine intake, sugary drinks during sick days
Ben asked 1 year ago
HI, I am a patient with adult-onset CPT II deficiency.1) I read that foods high in the amino acid leucine stimulate muscle protein synthesis and promote anabolism. Foods high in leucine mostly come from animal sources. For patients with CPT II deficiency, would it be more beneficial to have a diet higher in animal-based foods (low-fat) rather than plant-based proteins to increase leucine intake?

2) What are your thoughts on tart cherry juice supplementation during sick days (to reduce exercise-induced muscle damage)?

3) During sick days, if there are no digestive issues, would you say that drinking a smoothie throughout the day would be adequate, or would it be safer to drink a sugary drink that does not contain fiber/protein (along with regular meals)?

4) Do you think it matters what we mix DoJolVi with in terms of efficacy (does the type of macro make a difference – Is it preferable to mix DoJolVi with foods higher in carbs/protein)?
Thank you very much in advance

I hope you find the information below helpful.
Dr. Gillingham

1 Answers
INFORM Scientific Network answered 1 year ago

1) I read that foods high in the amino acid leucine stimulate muscle protein synthesis and promote anabolism. Foods high in leucine mostly come from animal sources. For patients with CPT II deficiency, would it be more beneficial to have a diet higher in animal-based foods (low-fat) rather than plant-based proteins to increase leucine intake?

 

Answer: Leucine does stimulate protein synthesis and yes, animal proteins are typically higher in leucine. Whey protein is high and all the branched chain amino acids and we found supplementation with whey protein improved body composition in LC-FAOD (manuscript attached.) However, pea protein has a very similar amino acid composition as whey so if you are looking for a vegetable based protein supplement, I recommend pea protein. Read more by clicking here.

 

2) What are your thoughts on tart cherry juice supplementation during sick days (to reduce exercise-induced muscle damage)

 

Answer: The research in athletes is for the benefits of tart cherry juice is mixed. I think of it similar to other anti-oxidants. It has not been studied in FAOD but would be safe and might provide some antioxidant effect. Read more by clicking here.

 

3) During sick days, if there are no digestive issues, would you say that drinking a smoothie throughout the day would be adequate, or would it be safer to drink a sugary drink that does not contain fiber/protein (along with regular meals)?

 

Answer: I think a smoothie has lots of advantages – fiber and other nutrients top on the list. Yes, smoothie would be a great idea.

 

4) Do you think it matters what we mix DoJolVi with in terms of efficacy (does the type of macro make a difference – Is it preferable to mix DoJolVi with foods higher in carbs/protein)?

 

Answer: I do not think it matters. We did a study with Ultragenyx mixing Dojolvi with applesauce, baking in muffins and mixing in a milk based beverage. All were ok with same efficacy.