Phenylketonuria is a condition in which you are unable to break down an amino acid called phenylalanine. It is a genetic condition present at birth. If both parents carry the gene, the baby is at risk for having PKU. A simple blood test done at birth will detect the presence of PKU. When phenylalanine is not broken down, brain toxicity resulting in mental retardation, seizures and muscle jerking can occur. PKU is treatable with diet and Kuvan, a drug newly approved by the Food and Drug Administration. Diet needs are individualized based on your height, weight and laboratory results. A doctor and registered dietitian will work closely with you throughout life to help you manage your nutrition needs and limit your phenylalanine intake.
High Protein Foods
Protein-rich foods need to be avoided in the PKU diet because of the high amounts of phenylalanine. This includes meats, poultry, fish, beans, peas, nuts, soybeans, milk, cheese, ice cream and yogurt. Adequate protein intake through natural foods is impossible with PKU, so your doctor will have you drink a medical formula with the phenylalanine removed from the protein source at each meal to prevent you from becoming malnourished. This is needed throughout your life.
Grains
Pasta, rice and certain cereals contain proteins and need to be limited. Low-protein pastas, breads and baked goods made with wheat starch are allowed but may need to be purchased at a specialty grocery store. A dietitian will work with you to determine which of these foods can be eaten and in what portions.
Artificial Sweeteners
Foods containing aspartame or the brand Nutra-Sweet should be avoided because aspartame releases phenylalanine, says MayoClinic.com. Diet sodas, sugar-free candies and gum or foods baked with aspartame should not be eaten. Many over-the-counter medications contain aspartame. Among them are Children's Tylenol, Flintstones vitamins, Alka-Seltzer and Zantac. Ask the pharmacist to help you find an aspartame-free substitute, and talk to your doctor before taking any new medications.
Label Reading
Manufacturers must specify whether the product contains aspartame and also must state whether the product contains phenylalanine. Check the ingredients list underneath the nutrition facts label to look for this ingredient or the phenylalanine warning. The food should be avoided if you see either of these ingredients listed.
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