| Ongoing nutrition management |
Patients work closely with their metabolic dietitian and metabolic physician for ongoing nutrition management throughout their lifetime |
| Avoidance of fasting |
Infants need to be fed every 3 hours with no more than 4 hours of fasting age 0–4 months |
| An additional hour of fasting is allowed per month of age thereafter up to 8 hours until 12 months of age |
| After infancy, it is important that children avoid fasting for more than 10–12 hours overnight (1) |
| Aggressive treatment during increased metabolic stress |
Emergency treatment letter |
| Providing oral or enteral carbohydrate-rich fluids every 3–4 hours with mild to moderate illness |
| Intravenous fluids with 10% dextrose at 1.5 times maintenance rate with appropriate electrolytes are provided during times of illness, poor oral intake, or when fasting for surgery (28) |
| Supplementation of carnitine |
Carnitine supplementation is controversial |
| Patients may develop secondary carnitine deficiency because fatty acids conjugate with carnitine and are then excreted as acylcarnitines (1) |
| Carnitine supplementation may only be helpful if secondary carnitine deficiency is found, although some mouse studies have suggested long-chain hydroxyl-acylcarnitines may induce arrhythmias (54) |
