Phenylketonuria is a genetic disorder that affects 1 in 25,000 children in the United States. Children with PKU cannot process the protein phenylalanine properly, and if phenylalanine builds up in a child's bloodstream, it can cause permanent brain damage. If your child has PKU, you can prevent brain damage by altering your child's diet to reduce or eliminate her exposure to phenylalanine.
Diagnosis
Because problems caused by PKU can be avoided with early detection, newborn babies are screened soon after birth. Many babies are screened at 24-hours-old, and your pediatrician may recommend a followup screen at 1 to 2 weeks of age. Newborns diagnosed with PKU are started on a special low-phenylalanine formula. Your pediatrician will closely monitor your baby's blood levels of the protein.
Foods High in Phenylalanine
High levels of phenylalanine are present in protein-rich foods such as milk, eggs, cheese, meat, poultry, fish, beef and nuts. Aspartame, an artificial sweetener added to processed foods and drinks, breaks down in the body and becomes phenylalanine. These foods are dangerous for children with PKU and should be avoided. Other foods, such as chocolate, bread, cereal and bananas, also contain significant amounts of phenylalanine, and because each person with PKU is different, your child may or may not be able to eat these foods.
Low PKU Foods
Your pediatrician will recommend a low-PKU formula to supplement your child's diet with a safe type of protein to support growth. This low-PKU formula will be your child's primary source of protein, as you must limit foods that are naturally high in protein, such as animal products, tofu and high-protein grains, such as quinoa and wheat. Choose low-protein breads and cereals, and rice- and corn-based cereals. Pack a low-phenylalanine lunch with rice cakes, grapes and vegetable soup. Serve a baked potato with broccoli for dinner. Marinate cubed vegetables and ask your child to thread them on a skewer. Grill the vegetables and serve with a baked sweet potato.
Precautions
The diagnosis and treatment of PKU allows children to grow and develop without brain damage; however, PKU is a life-changing diagnosis. You will work closely with pediatrician and nutritionist to keep your child's phenylalanine levels in a safe range. Create a safe zone for your child. Communicate with those who also take care of your children, such as grandparents and teachers, about what foods your child must avoid. As your child grows, explain PKU in ways that she can understand and consider meeting with other families for support.
References
- March of Dimes; Birth Defects; PKU; August 2007
- MayoClinic.com; Phenylketonuria: Treatment and Drugs; October 20, 2009
- PKU.com: Where Phe Lurks
- MayoClinic.com; Phenylketonuria: Lifestyle and Home Remedies; October 20, 2009
- University of Wisconsin Extension; Alternative Field Crops Manual; Quinoa; April 12, 2011


