Kuvan

Generic Name: Sapropterin

  • What is Kuvan?

    Kuvan is used to treat people with phenylketonuria (PKU). PKU is a genetic condition in which an enzyme in the body does not work right and cannot break down phenylalanine, an amino acid found in food. This causes the level of phenylalanine to rise in the blood, which can result in lower intelligence and decreased ability to focus, remember, and organize information.

  • 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.

    You should follow a phenylalanine-restricted diet in addition to treatment with Kuvan. When you are taking Kuvan, any change you make to your diet may affect your blood phenylalanine levels. Your doctor will monitor your dietary phenylalanine intake.

    If you have a fever, or if you are sick, your phenylalanine levels may go up. Tell your doctor as soon as possible. He or she may have to adjust your treatment to help keep your phenylalanine levels in the desired range.

  • Who should not take Kuvan?

    Do not take Kuvan if you have a known severe allergy to any of its ingredients.

  • 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 or kidney 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 and children ≥4 years: The initial recommended dosage is 10 milligrams (mg) a day per 2.2 pounds of body weight. The dosage may be adjusted within the range of 5-20 mg 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. To make the tablets dissolve faster, you can stir or crush them. It is okay to swallow the liquid even if there are small pieces of the tablet still floating in the mixture. If after drinking your medicine you still see small pieces of the tablet, you should add more water or apple juice to make sure that you take all of your medicine.

  • What should I avoid while taking Kuvan?

    Do not adjust 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, nausea

  • Can I receive Kuvan if I am pregnant or breastfeeding?

    The effects of Kuvan during pregnancy and breastfeeding are unknown. It is best to avoid Kuvan during breastfeeding because of the potential for harm in nursing infants. Tell your doctor immediately if you are pregnant, plan to become pregnant, or are breastfeeding.

    Women who are exposed to Kuvan during pregnancy are encouraged to enroll in the Kuvan registry (ask your doctor for more information).

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

    If you miss a dose of Kuvan, take the missed dose as soon as you remember it that day. However, if you miss a day, skip the one you missed and return to your regular dosing schedule. Do not take two doses at once. If you are unsure, ask your doctor or pharmacist for advice.

  • How should I store Kuvan?

    Store at room temperature in a cool, dry place. Protect from humidity and moisture.

Meet the Pharmacists

I'm Beth Isaac, PharmD. Welcome to PDR Health!

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