Phenylketonuria

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Phenylketonuria Treatment

Treatment includes a diet that is extremely low in phenylalanine; particularly when the child is growing. Strict compliance to the diet is necessary to prevent or minimize mental retardation. This requires close supervision by a registered dietitian or physician, and cooperation of the parent(s) and child.

Phenylalanine occurs in significant amounts in milk, eggs and other common foods. Nutrasweet (aspartame) also contains phenylalanine, and products containing aspartame should be avoided by children with this disorder. A special infant formula called Lofenalac is made for infants with PKU. It can be used throughout life as a protein source that is extremely low in phenylalanine and balanced for the remaining essential amino acids.

Adult women who have PKU and who plan to become pregnant should also adhere to a strict low-phenylalanine diet both before becoming pregnant and throughout the pregnancy.

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