The three Rs: Record, Restrict, and Replace

To help maintain consistently low blood Phe levels, PKU 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 high-Phe foods with low-Phe alternatives, specifically a regimen of Phe-free medical protein supplements. 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. 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.
• 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.

Maintaining a healthy diet and being careful not to have too much Phe can be a balancing act. High blood Phe levels can lead to impaired thinking and behavioral problems. KUVAN has been proven to lower blood Phe levels in combination with your Phe-restricted diet.

Next: About KUVAN

 

Indication

KUVAN^® (sapropterin dihydrochloride) Tablets is approved to reduce blood Phe levels in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). KUVAN is to be used in conjunction with a Phe-restricted diet.

Important Safety Information

High blood Phe levels are toxic to the brain and can lead to lower intelligence and decrease in the ability to focus, remember and organize information. Any change you make to your diet may impact your blood Phe level. Follow your doctor’s instructions carefully. Your doctor and dietitian will continue to monitor and may adjust your diet throughout your treatment with KUVAN.

If you have a fever, or if you are sick, your Phe level may go up. Tell your doctor and dietitian as soon as possible so they can see if they have to adjust your treatment to help keep your blood Phe levels in the desired range.

KUVAN is a prescription medicine and should not be taken by people who are allergic to any of its ingredients. Tell your doctor if you have ever had liver or kidney problems, are nursing or pregnant or may become pregnant, have poor nutrition or are anorexic. Your doctor will decide if KUVAN is right for you. Tell your doctor about all the medicines you take.

The most common side effects reported when using KUVAN are headache, diarrhea, abdominal pain, upper respiratory tract infection (like a cold), throat pain, vomiting, and nausea.

To report SUSPECTED ADVERSE REACTIONS, contact BioMarin Pharmaceutical Inc. at 1-866-906-6100, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please read the full Patient Information by clicking here.