Managing PKU With the 3 Rs

To help maintain consistently low blood Phe levels, management involves a three-pronged approach: Record, Restrict, and Replace. People with PKU must record the amount of Phe consumed, restrict their diet to the right amount of low-Phe foods, and replace those high-Phe foods with low-Phe alternatives, specifically a regimen of Phe-free medical protein supplement. While this type of management is not a cure, it has been effective in helping to prevent many of the symptoms of PKU. Your doctor should be consulted to help you understand PKU management, including Record, Restrict, and Replace.

Record

You should work to keep track of how much Phe you consume every day. A registered dietitian should be consulted and can give you a daily number of exchanges (one exchange = 15 mg of Phe) appropriate for you. This number is your Phe tolerance. Everything you eat in a day should count toward the number of allowable exchanges. A good practice is to record daily intake on a Phe calendar.

Restrict

You’ll soon realize that there are many foods that have too much Phe to be included in your diet. These include meat, eggs, and dairy, and because of their high Phe content, they must be avoided entirely. There are a variety of substitute foods, and on this Website you will find frequently updated recipe ideas. Come back often to check new ideas.

Replace

Because protein is an essential part of any diet, you must consume some protein. Therefore, the centerpiece of a PKU diet is a special low-Phe or Phe-free protein formula, which is available by prescription. This Phe-free formula comes in two forms—powder and mixed drinks—and a variety of flavors. Some formulas also have extra vitamins in them.

Keeping a healthy diet and being careful not to have too much Phe can be a balancing act. Below is an example of how to Record, Restrict, and Replace for a 14-year-old PKU patient who is allowed about 400 mg of Phe per day.