DWQA QuestionsCategory: QuestionsSupporting students with CPT1a deficiency
Lea Palmer MPH, RDN, LD asked 4 years ago
Hello, I am a registered dietitian for several Head Start sites in the YK delta area of Alaska. I have seen the ‘CPT1 diagnosis’ come up on several Head Start applications so I am putting together a staff training to help them better understand this genetic disorder and know how to best support these children during the school year.  Other than offering a low-fat, higher carb snack every 2-3 hours, do you have any suggestions for supporting our kids with CPT1a deficiency in this Head Start setting? Thank you for your time & support

1 Answers
Jerry Vockley answered 4 years ago
HI Lea,
Thanks for your question. Yes. Providing frequent snack options during the day to avoid prolonged periods of fasting is the primary goal for CPT1a deficiency.

  • CPT1a is the rate limiting step in fat oxidation in the
  • Children who have a genetic variant of CPT1a have low levels of fat oxidation in their liver and can’t use fat for energy during fasting.
  • Fasting duration depends on your age; younger children have a shorter period of fasting tolerance than older children.
  • Toddlers between 2 and 4 can fast 2-4 hours between meals during the day and sleep 8-10 hours at night when well.
  • If a child gets sick, has a fever, or other illness, there energy needs increase and their fasting tolerance decreases. Head start staff should recommend the child stay home, rest and eat small meals frequently.

We have not conducted studies on the optimum macronutrient distribution for children with CPT1a variants so I would recommend a healthy balanced diet. For other fatty acid oxidation disorders like MCADD, we typically recommend a “Heart Healthy “ approach with a moderate to low fat intake. There is some thought that including healthy omega-3 fats are beneficial, and certainly part of a traditional YK diet.
The primary goal is to offer frequent meals and avoid prolonged fasting.
Melanie Gillingham PhD, RD