Choose medical foods to meet recommended nutrient intake and achieve optimal adherence. When incomplete medical foods are chosen, ensure that vitamin, mineral, energy, and/or fat intake is supplemented from other sources when necessary.

Consumption of medical food throughout the day, in several well-spaced intervals, to allow optimal blood PHE concentrations and dietary PHE tolerance.

Encourage use of breast milk, when possible, either from direct breast feeding or use of expressed breast milk, as the source of PHE (and intact protein) in infants.

Gradually introduce solids, to replace the equivalent amount of PHE/intact protein in infant formula or breast milk, when the infant is developmentally ready (usually at 4-6 months of age).

Minimize elevation of blood PHE during illness by treating the underlying illness, meeting protein and energy needs, and preventing dehydration and electrolyte imbalance.

Ensure appropriate PHE intake in individuals with PKU by having accurate data regarding PHE content of foods, and effective and convenient methods of planning and monitoring dietary PHE intake.

Encourage all individuals to follow treatment recommendations throughout their lives; including those who have relaxed their diet restrictions and those who have never been treated. Recognize and address individual barriers that may impede success.

Adopt clinic procedures that enhance adherence to the nutritional recommendations of “diet for life” by providing individualized educational strategies, referrals to appropriate social service and mental health professionals, age-appropriate group activities, and a plan for transition from pediatric to adult clinical services.