Fundamental to the medical management of LCHAD is the avoidance of fasting, particularly during periods of high metabolic stress, such as illness. Overnight fasts should last no longer that twelve hours and infants should receive late evening feedings to reduce this period. The addition of food-grade uncooked cornstarch mixed in liquid at bedtime has helped some infants decrease the frequency of morning hypoglycemia. A diet high in natural fat should be avoided. Medium-chain triglyceride supplementation bypasses the metabolic block and provides safe calories. Supplementation with oral L-Carnitine has not been shown to be beneficial in avoiding or ameliorating clinical symptoms.
High carbohydrate intake should be encouraged during illness, with initiation of intravenous glucose supplementation if the child is unsuccessful in keeping down fluids, or unable to take adequate oral feedings. For individuals with LCHAD deficiency, it is imperative that the lethargic patient receive parenteral dextrose to avoid hypoglycemia during evaluation.
Because the diagnosis and therapy of LCHAD deficiency is complex, the pediatrician is advised to manage the patient in close collaboration with a consulting pediatric metabolic disease specialist. It is recommended that parents travel with a letter of treatment guidelines from the patient’s physician.