Phenylketonuria (PKU): Understanding Treatment & Management

Phenylketonuria (PKU) is a condition that is treatable by diet. The goal is to maintain the blood level of the amino acid phenylalanine between 2 to 10mg/dl. Left untreated, PKU results in IQs under 60, seizures and behavior problems. Treatment at an early age prevents these side effects. Begin having your child follow a proper diet shortly after birth for a good prognosis.

Instructions

• 1

  See the doctor 2 to 3 times a week until the infant stabilizes. The doctor tests the blood to determine the amount of the amino acid phenylalanine. When the child's condition has stabilized, cut back to once a week visits.

• 2

  Begin the infant on phenylalanine-free formula until stabilized.

• 3

  Change to infant formula with some phenylalanine and gradually introduce low-protein foods such as applesauce.

• 4

  Keep your child on a strict lifelong low-protein diet with limited levels of phenylalanine.

• 5

  Consult a dietician or nutritionist. Healthy eating includes cereals, starches, fruits and most vegetables.

• 6

  Invest in low-protein products such as imitation cheeses and egg substitutes.

• 7

  Invest in a special nutritional drink or supplement for people with PKU.