Treatments for Phenylketonuria (PKU) in Children

The main treatment for phenylketonuriais avoiding foods with high protein and taking special medical formula as prescribed. People with PKU should follow a low-phenylalanine diet for the rest of their lives, even if symptoms do not surface. Foods high in phenylalanine include foods such as:

  • beef
  • fish
  • chicken
  • eggs
  • milk
  • chocolate
  • cheese
  • beans
  • nuts
  • peas
  • soybeans
  • diet sodas
  • the artificial sweeteners, aspartame, contains phenylalanine.

Individuals with PKU often are allowed these foods in their diets in moderation:

  • cereals
  • breads
  • pastas
  • rice
  • fruits
  • vegetables
  • milk substitutes

Those with PKU must also take:

  • Phenylalanine-free baby formula or continuation of breast feeding supplemented with special formula.
  • Special medical formula for children and adults which contains either free amino acids but not phenylalanine or glycomacropeptide which has very little phenylalanine

If your child has also been given a trial of the PAH co-factor medication known as sapropterin (Kuvan) and found to be responsive, he or she may also be on this drug.

Coping & support

PKU is a uniquely challenging disease, which requires your child to stay disciplined and follow the diet every day, for the rest of his life. Our team of nurses and nutritionists can help you come up with strategies to help your child be able to comply with the diet, and our psychologists can help your child cope with the daily stress of managing his diet. The diet is expensive but is usually covered by health insurance or public health programs. Our social workers will help you understand what financial support you're entitled from your private insurer, the Public Health Department or Medicaid. Learn more about the patient and family resources available at Children's.

Getting your child to eat vegetables

Vegetables are a great way to get some of the nutrients your child needs as he follows a low- phenylalanine diet. See how Claire McCarthy, MD helps make vegetables  an attractive alternative for her children. Read more on join the conversation on Boston Children's Thriving blog.