Phenylketonuria (PKU) is a rare inherited metabolic disorder that affects the body’s ability to metabolize a specific amino acid called phenylalanine (PHE). People with PKU have a deficiency in the enzyme needed to break down phenylalanine, which leads to a buildup of this amino acid in the body.
A buildup of PHE can be harmful, as excess amounts can cause serious health problems if not managed properly. As you get older, the need for dietary management does not go away, except for some individuals managed with the drug pegvaliase.
Here are some reasons why it’s important for adults with phenylketonuria (PKU) to follow the PKU diet and drink PKU formula, also known as PKU medical food:
- Controlled Phenylalanine Intake: PKU formula is specially made to be low in or free of phenylalanine (PHE). By consuming PKU formula, individuals can get the protein they need while limiting their PHE intake, helping to keep blood PHE levels within a safe range.
- Protect the Brain: Elevated levels of PHE in the blood can lead to cognitive impairment, behavioral problems, and other neurological issues. Drinking PKU formula and consuming a low-PHE diet helps to avoid the harmful effects of high phenylalanine levels on the brain.
- Support for Overall Nutrition Needs: PKU formula is designed to provide the necessary nutrients (except phenylalanine) that individuals with PKU need for normal growth and development. Formula helps ensure that you receive essential amino acids, vitamins, and minerals. Note: Nutritional content varies by formula. Not all PKU formulas have vitamins and minerals.
- Individualized Management: PKU formula can be tailored to an individual’s specific dietary needs and preferences, making it a versatile tool in managing PKU. Nutricia offers a wide variety of options to help you find a PKU formula you will enjoy drinking. EXPLORE OPTIONS
- Lifelong Management: PKU is a lifelong condition, and following the PKU diet and management recommendations from your healthcare professional is important for long-term health and well-being.
Your metabolic clinic or healthcare provider is an important partner and can share more about the benefits of staying on diet as an adult. They can help you develop a personalized treatment plan that includes the appropriate use of PKU formula. This plan will take into account factors such as age, activity level, and individual metabolic needs. Always consult your metabolic healthcare provider before making changes to your diet.
Need help finding a clinic? Please e-mail NutritionServices@nutricia.com or call us at 1-800-365-7354, Mo-Fr from 8:30 am-5:00 pm Eastern for help.
All products shown are medical foods for the dietary management of phenylketonuria (PKU) and must be used under medical supervision.
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