Managing Your Diet

Everyone has dietary requirements, whether you have PKU or not.  How many calories you need each day depends on your height, weight, age, and level of activity. In addition to caloric intake, everyone must also consume enough protein, essential amino acids, and other nutrients such as vitamins and minerals.

As someone with PKU, your carefully monitored diet is customized for you by your clinic team. Maintaining a healthy diet is critical for people with PKU as too much or too little of anything can quickly impact the way you function and feel.

Do you feel uncomfortable or judged when the clinic staff reviews your Phe levels? Are you disappointed in yourself or do feel guilty when your Phe levels are high?Don’t go this route! Your doctor and dietitians recognize that you are trying to maintain your Phe levels within the recommended range, and that it is not always easy. They know you are working hard, and want to offer alternatives to help achieve your goals. Rather than feeling badly about a high Phe level, use the information to be more determined about how you will regain control. Remember: emotions do not help you maintain your Phe levels—actions do!

Feel like there’s nothing to eat? Try some trusty favorites: milk substitutes, MixQuick, So Delicious coconut yogurt, frozen hash brown patties, nopalitos (cactus), Cambrooke's butterscotch cookies, LoPro meat substitute (Tweekz), Country Sunrise microwave pasta meals, potatoes, corn, rice, and low-protein pasta, pizza, soup, broccoli burgers, bread, cheese, pancakes, and muffins.

Maintaining your low-Phe diet is the key to staying healthy.
Each person with PKU has a different diet that is developed just for them. This diet is based on: 
  • The person’s Phe levels
  • The amount of Phe the person can consume (called Phe tolerance)
  • Other individual considerations such as age and weight

An important part of your diet will be consuming your prescribed amount of medical formula. Your medical formula includes the vitamins, minerals, and protein you need to stay healthy. For some, Kuvan® (sapropterin dihydrochloride) or large neutral amino acids (LNAAs) may also help in maintaining Phe levels, when they are combined with a low-Phe diet. Other treatments are in development, including enzyme substitution and gene therapy.

Stay on diet!! Drink your formula, battle the insurance companies, do whatever it takes.

Les, 35 years old

Your individualized low-Phe diet will include fruits, vegetables, fats, sugars, and low-protein medical foods, such as low-protein pasta and breads. Foods such as regular pasta, bread, rice, and starchy vegetables (e.g., potatoes, peas, corn) are likely to be limited in your diet. Your dietitian may also provide you with some options for “free foods,” which are foods that contain no Phe and can be consumed as fillers (for example, some sugar candies).

Foods such as red meat, chicken, fish, eggs, milk, yogurt, cheese, nuts, soybeans, and beans are too high in protein to be part of a PKU diet, except when the dietary restriction required is minimal.

Your dietitian will instruct you as to which foods—and how much of them—you can eat. Even low-protein foods cannot be eaten in unlimited quantities. All foods must be measured to make sure that Phe intake stays within the daily limit.

Work with your dietitian to figure out what works best for YOU in managing your diet.

Jen, 38 years old

To ensure that you eat the appropriate amount of food for your prescribed diet, you will need to measure it and use a food reference guide to assess the amount of Phe in the food. The most accurate way to measure your food is to use a scale However, you can also measure portions of food and formula by using measuring cups, counting Phe exchanges, or estimating the Phe using nutritional information on food labels. Keep a record of the amount of Phe you eat. And make sure to bring this record with you to your PKU clinic appointments so you can discuss your diet with the clinic staff.

To maintain your low-Phe diet, expert clinicians recommend that you:

  • Plan ahead! You may stray from your low-Phe diet if do not have the right foods in the house or a strategy for meals outside your home
  • Seek out endless support from your friends and family
  • Take advantage of the support your clinic is there to provide
Your PKU Treatment Plan

Information on your treatment plan and how to get the most out of each clinic visit. MORE >

PKU Treatment Guidelines

New medical and dietary guidelines were recently released by doctors and dieticians.  Learn more about what you need to do ensure the best possible health outcomes. MORE >

Managing The Effects of PKU

Learn more about ADHD, memory problems, processing information, anxiety and depression.  This section includes tips on managing ADHD, time management, tips to stay on task, how to focus your attention and improving your memory. MORE >

Returning To The PKU Diet

Many teens and adults are no longer on diet.  Learn more about the effects of being off-diet, tips on getting back on diet, ideas on getting enough formula and how to make a successful transition. MORE >

Socializing and Your Diet

Information on eating out, socializing with friends and going to college. MORE >