The most effective treatment for phenylketonuria is a proper diet. The amino acid phenylalanine, which a person with phenylketonuria cannot break down, is commonly found in protein-rich foods, such as milk, cheese, nuts, eggs, chicken, soybeans, beef, beans, fish, peas, and chocolate. A phenylalanine-free formula provides nutrients and protein in a safe format for newborns and infants. A diet rich in vegetables, fruit, as well as pastas, cereals, and breads low in protein should be maintained throughout life. Blood tests may be necessary over time to ensure PKU levels remain under control as one ages and undergoes life changes.
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Honor Society of Nursing (STTI) answered
People with phenylketonuria (PKU) can't eat phenylalanine (phe). Phe is an amino acid that is found in all foods that contain protein, like meat, dairy, eggs, and soy products. Patients are treated with special medical formulas made specifically for PKU. The formulas are important because they provide protein that the body needs, but they have no phe, so they are safe for PKU patients. They are also permitted to eat very small amounts of "natural" protein from fruits and vegetables. Some patients with PKU also respond to a new medication called Kuvan that can help control their phe levels.
The mainstay of therapy in PKU is dietary restriction of phenylalanine. This requires the use of medical foods including phenylalanine-free protein substitutes (amino acid mixtures or protein hydrolysates) that supply approximately 75 percent of protein requirements (except phenylalanine).