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Information for Patients & Caregivers
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What is Phenylketonuria?

Importance of Phe

Managing PKU


KUVAN

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The Importance of Phenylalanine Control

Video

It's all about Phe

People with PKU cannot process one of the amino acids found in high protein foods. This amino acid, called phenylalanine or Phe for short, builds up in the body.1 Too much Phe is toxic to the brain and can cause many problems.
  • In infants and children, if PKU is not treated, the resulting high Phe can cause severe mental retardation.1 Even if PKU is treated, problems like brain changes, lower intelligence, and behavior problems may still occur2
  • In adults and teens, high Phe can cause lower IQ, poor attention span, mood swings, irritability, depression, slow reaction time, and other problems3,4,5,6

Lower Phe is better*1

To protect the brain, people with PKU need to keep Phe levels low. Low-Phe helps you think better. It helps you control moods. It helps you to be your best. Low-Phe is good, and lower is better.*1,2

There is a lot of Phe in high-protein foods. People with PKU eat a diet low in Phe, avoiding high-Phe foods and limiting lower-Phe foods.1,2
* Lower is better until you get Phe into the normal range of 1-2 mg/dL. Going lower than 1-2 mg/dL can be unhealthy because your body needs a minimum amount of Phe. Talk with your doctor about the right target range for you.1,2

Long-term studies of neurocognitive outcomes with KUVAN treatment have not been conducted. BioMarin is conducting a trial to evaluate long-term neurocognitive outcomes in PKU patients treated with KUVAN.
High Phe & PKU Symptoms

Indication

KUVAN® (sapropterin dihydrochloride) Tablets are 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 low-Phe 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 your diet and blood Phe levels 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.


This site is intended for use by U.S. residents only.

KUVAN® is a registered trademark of BioMarin Pharmaceutical Inc. Developed in collaboration with Asubio Pharma Co., Ltd.
©2010 BioMarin Pharmaceutical Inc. All rights reserved.