children s health issues
Phenylketonuria (PKU)
Prevention and Treatment of PKU
Phenylalanine-restricted diet To prevent intellectual disability, people must restrict phenylalanine intake (but not eliminate it altogether because people need some phenylalanine to live) beginning in the first few weeks of life. Because all natural sources of protein contain too much phenylalanine for children with PKU, affected children cannot have meat, milk, or other common foods that contain protein. Instead, they must eat a variety of products that are specially manufactured to be phenylalanine-free. Low-protein natural foods, such as fruits, vegetables, and restricted amounts of certain grain cereals, can be eaten. Special nutritional products, including infant formula without phenylalanine, are also available. Examples of these special products include PKU Anamix®, XPhe Maxamaid®, and XPhe Maxamum®; Phenex®-1 and Phenex®-2; Phenyl-Free®1 and Phenyl-Free®2; pku 1, pku 2, and pku 3; PhenylAde®(varieties); PKU Lophlex®LQ; and Phlexy-10®. Women who have PKU and who are planning on becoming pregnant should follow the recommended diet and keep their PKU under control. Doctors may give supplements of the amino acid tyrosine and a drug calledsapropterin. This drug helps increase a person's tolerance of phenylalanine. 