Kuvan

Generic name: Sapropterin dihydrochloride

What is Kuvan?

Kuvan is used to treat people with phenylketonuria (PKU). PKU is a genetic condition in which the body cannot break down a certain type of amino acid found in food called phenylalanine. This causes the level of phenylalanine to rise in the blood, which can result in lower intelligence and behavioral problems.

What is the most important information I should know about Kuvan?

It is not possible to know whether or not Kuvan will work for you until you start taking it. Your doctor will monitor your blood phenylalanine levels when you start taking Kuvan to see if the drug is working. When you are taking Kuvan, any change you make to your diet may affect your blood phenylalanine levels.

Who should not take Kuvan?

At this time, there are no known reasons to avoid taking Kuvan.

What should I tell my doctor before I take the first dose of Kuvan?

Tell your doctor about all prescription, over-the-counter, and herbal medications you are taking before beginning treatment with Kuvan. Also talk to your doctor about your complete medical history, especially if you have any of the following: fever, liver problems, poor nutrition or anorexia, or allergies to Kuvan or any of its ingredients.

What is the usual dosage?

The information below is based on the dosage guidelines your doctor uses. Depending on your condition and medical history, your doctor may prescribe a different regimen. Do not change the dosage or stop taking your medication without your doctor's approval.

Adults: The initial recommended dosage is 10 mg/kg (milligrams per kilogram) a day. The usual dosage range is 5-20 mg/kg a day.

How should I take Kuvan?

Take Kuvan exactly as prescribed by your doctor. You should take the tablets at the same time each day with food.

Dissolve Kuvan tablets in 4-8 ounces (one-half to 1 cup) of water or apple juice and drink within 15 minutes of dissolving. It is okay to swallow the liquid even if there are small pieces of the tablet still floating in the mixture.

What should I avoid while taking Kuvan?

Do not make any changes to your dietary phenylalanine intake without talking to your doctor first.

What are possible food and drug interactions associated with Kuvan?

If Kuvan is used with certain other drugs, the effects of either could be increased, decreased, or altered. It is especially important to check with your doctor before combining Kuvan with the following: levodopa, drugs for erectile dysfunction such as sildenafil, and drugs that affect folate metabolism such as methotrexate.

What are the possible side effects of Kuvan?

Side effects cannot be anticipated. If any develop or change in intensity, tell your doctor as soon as possible. Only your doctor can determine if it is safe for you to continue taking this drug.

Side effects may include: headache, diarrhea, abdominal pain, throat pain, upper respiratory tract infection (such as a cold), vomiting

Can I receive Kuvan if I am pregnant or breastfeeding?

It is best to avoid Kuvan during pregnancy and breastfeeding. Women who are exposed to Kuvan during pregnancy are encouraged to enroll in the Kuvan registry (ask your doctor for more information about the registry).

What should I do if I miss a dose of Kuvan?

If you miss a dose, take it when you remember. If you miss a day, wait and take the next dose on schedule. Do not take a double dose to make up for the skipped dose.

How should I store Kuvan?

Store at room temperature.