For neonates with suspected VLCAD, initiate fasting precautions while awaiting confirmation of the diagnosis. Asymptomatic neonates can continue to breast feed (or feed expressed breast milk) without MCT supplementation, if appropriate fasting precautions are followed (RECOMMENDATION TABLE #7, Recommendations for Fasting Intervals for Individuals with VLCAD when well).

Consider age, disease severity and clinical history when establishing nutrition prescriptions for dietary fat composition (RECOMMENDATION TABLE #8, Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well).  (For breastfeeding recommendations-see Recommendation 1.6)

• When designing a diet for an individual with VLCAD, aim for, but do not exceed, the TOTAL fat intake recommended by the DRI for age. Total fat includes both LCF and MCT.
• For individuals with a MILD form of VLCAD, it is not necessary to replace some of the LCF in the diet with MCT, as long as the individual remains asymptomatic and follows guidelines for fasting and illness management.
• For individuals with a MODERATE form of VLCAD, restrict LCF to 15 to 30% of estimated energy needs considering age, disease severity and clinical history. 
• For individuals with a SEVERE form of VLCAD, restrict LCF to 10 to 15% of estimated energy needs considering age, disease severity and clinical history.
• Decreasing LCF below 10% of total energy intake may not provide additional clinical benefit, even for those with a SEVERE phenotype.
• After LCF needs are determined, add a source of MCT to meet the individual's total fat needs.

For infants and children with VLCAD who are healthy and gaining appropriate weight for age, use the Institute of Medicine Estimated Energy Requirement (EER) calculation for age and physical activity level (PAL) to estimate energy needs (RECOMMENDATION TABLE #8, Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well).

Provide total dietary protein at or above the age-appropriate DRI for protein intake. (RECOMMENDATION TABLE #8, Recommended Fat (total, long chain and medium chain), Energy and Protein Intakes for Individuals with VLCAD when Well)

Use standard equations to determine fluid requirements for an asymptomatic individual with VLCAD.

Support breastfeeding of infants with VLCAD, taking into consideration the following:

• For an asymptomatic infant with a MILD form of VLCAD, allow breastfeeding (or expressed breast milk) without MCT, as long as breast milk supply remains adequate, age appropriate weight gain is maintained, and fasting recommendations are followed.
• For an asymptomatic infant with a MODERATE form of VLCAD, allow breastfeeding (or expressed breast milk) but consider supplementing breast milk with a low LCF, high MCT medical food. (RECOMMENDATION TABLE #5, Medical Foods for the Nutrition Management of VLCAD).
• For an asymptomatic infant with a SEVERE form of VLCAD, the primary source of nutrition should be a low long-chain fat, high MCT medical food.
• For a symptomatic infant, depending on the severity of symptoms and lab monitoring, consider allowing some breast milk while using a low LCF, high MCT medical food to meet energy needs.
• If breastfeeding was discontinued during metabolic decompensation, consider reintroduction of partial breast feeding (or expressed breast milk) after the infant returns to an asymptomatic clinical state, if the mother's breast milk supply remains adequate to do so.

Advise all individuals with VLCAD to avoid excessive fasting, taking into consideration the severity of disease (RECOMMENDATION TABLE #7, Recommendations for Fasting Intervals for Individuals with VLCAD when well).

Consider an individual's age, dietary LCF restriction and plasma or red blood cell (RBC) fatty acid profiles to determine if additional sources of essential fatty acids are required:

• For infants requiring LCF restriction and MCT supplementation, a medical food providing arachidonic acid (ARA) and docosahexaenoic acid (DHA) to meet AI recommendations is suggested to prevent essential fatty acid deficiency (RECOMMENDATION TABLE #5, Medical Foods for the Nutrition Management of VLCAD).
• For individuals requiring additional sources of essential fatty acids to normalize fatty acid profiles, dietary sources, including oils with higher concentrations of linoleic acid (LA) and α-linolenic acid (ALA), should be added to the diet (RECOMMENDATION TABLE #9, Essential Fatty Acid Content of Selected Oils).
• Adjust intakes of LA within 3 to 6% of total energy and ALA within 0.5 to 1.2% of total energy to normalize plasma or RBC essential fatty acid profiles (RECOMMENDATION TABLE #3, Recommended Intakes of Essential Fatty Acids for Individuals with VLCAD)
• Consider supplementation with preformed DHA if normal plasma or RBC DHA concentrations cannot be achieved by diet modification.
• If DHA supplementation is required, appropriate doses include 60 mg/day for infants and toddlers and 100 mg/day for older age groups.

Supplementation with uncooked cornstarch (UCCS) at bedtime is not indicated in the treatment of VLCAD since hypoglycemia is not likely to develop when an individual is asymptomatic, if he/she avoids excessive periods of fasting and meets energy needs.

• To meet fasting guidelines and prevent catabolism in children and adults, consider adding a bedtime snack emphasizing complex carbohydrates.
• For those with a severe phenotype who do not tolerate extended fasting times, an overnight enteral feeding should be considered when a bedtime snack is not sufficient.