Phenylketonuria is a birth defect that disables your body from properly breaking down the amino acid phenylalanine. If left untreated, PKU can eventually lead to irreversible brain damage, behavioral problems and seizures, according to MayoClinic.com. Although you should get specific dietary guidelines from your doctor, the primary treatment for PKU is usually a strict diet that limits intake of foods containing phenylalanine. Fortunately, many foods don't contain large amounts of this amino acid.
Phenyl-Free Foods
Because high-protein foods can contain large amounts of phenylalanine, you will need to avoid all high-protein foods such as milk, dairy products, eggs, chicken, beans and red meat. Foods, drinks and medications that are made with aspartame are also off-limits. The typical PKU diet usually consists of carefully measured amounts of fruits, vegetables and specialty low-protein flour, rice, bread and pasta, according to the PKU clinic at the University of Washington. An example of a PKU breakfast might be a diluted nondairy creamer poured over a low-phenylalanine cereal such as puffed rice.
Supplements
A PKU diet is rounded out by a special phenylalanine-free formula that contains a healthy dose of vitamins and minerals you wouldn't be able to get from food. Your doctor or dietitian will likely recommend that you drink several glasses of this per day. Other supplements may also be necessary. For instance, a fish oil supplement will help replace long-chain fatty acids and aid in neurologic development, according to MedlinePlus. Supplements such as iron, tyrosine and carnitine are also sometimes used to compensate for dietary deficiencies.
Foods for Baby
Babies with PKU also need to drink a special formula because breast milk and regular formula contain phenylalanine. In most cases, a dietitian will calculate a small amount of breast milk or regular formula to mix in with the specialty formula, according to MayoClinic.com. If you have a baby with PKU, you would introduce her to PKU-friendly solids on the same schedule as other babies start to eat solids.
Considerations
Your diet will be individualized based on your diet records and growth charts, and your blood levels of phenylalanine. Your doctor will also frequently monitor your PKU levels as they fluctuate. The levels are more susceptible to change during times such as growth spurts and pregnancy. Generally, you will only be permitted to consume only the smallest amount of phenylalanine needed for normal body functions. Although the PKU diet was once only considered necessary for babies and children, your health will benefit most if you commit to following the diet for life, according to MedlinePlus.
References
- MayoClinic.com: Phenylketonuria: Treatments and Drugs
- University of Washington PKU Clinic: What Is the Diet for PKU?
- MedlinePlus: Phenylketonuria
- National Institute of Child Health & Human Development: Phenylketonuria
- "The Journal of Neuropsychiatry & Clinical Neurosciences"; Tyrosine Supplements for ADHD Symptoms with Comorbid Phenylketonuria; J. Posner, et al; 2009
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