The nurse is reviewing anticipatory guidance with the parents of a 6-month-old infant with phenylketonuria. Which statements by the nurse are appropriate? Select all that apply.

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Phenylketonuria (PKU) is one of a few genetic inborn errors of metabolism. Individuals with PKU lack the enzyme (phenylalanine hydroxylase) required for converting the amino acid phenylalanine into the amino acid tyrosine. As unconverted phenylalanine accumulates, irreversible neurologic damage can occur. A low-phenylalanine diet is essential in the treatment of PKU (Option A). Phenylalanine cannot be entirely eliminated from the diet as it is an essential amino acid and necessary for normal development. The diet must meet nutritional needs while maintaining phenylalanine levels within a safe range (2-6 mg/dL [120-360 µmol/L] for clients age <12). There is no known age at which the diet can be discontinued safely, and lifetime dietary restrictions are recommended for optimal health (Option C). Management of the client with PKU includes: Monitoring serum levels of phenylalanine Including synthetic proteins and special formulas (eg, Lofenalac, Phenyl-Free) in the diet (Option D) Eliminating high-phenylalanine foods (eg, meats, eggs, milk) from the diet (Option B) Encouraging the consumption of natural foods low in phenylalanine (most fruits and vegetables) (Option E) Restriction of dietary tyrosine is not necessary. Tyrosine levels in clients with PKU may be normal or slightly decreased. Educational objective: Phenylketonuria requires lifetime dietary restrictions. Infants should be given special formulas (eg, Lofenalac). For children and adults, high-phenylalanine foods (eg, meats, eggs, milk) should be restricted and replaced with protein substitutes.

The Nurse Is Reviewing Anticipatory Guidance with the Parents of a 6-Month-Old