Sapropterin

Generic Name: sapropterin (SAP-roe-TER-in)
Brand Name: Kuvan

Sapropterin is used for:

Reducing blood phenylalanine levels in certain patients with phenylketonuria. It is used along with a diet that is low in phenylalanine.

Sapropterin is a form of BH4. It helps to activate phenylalanine hydroxylase, the enzyme that breaks down phenylalanine in the body.

Do NOT use sapropterin if:

  • you have had a severe allergic reaction (eg, severe rash, hives, difficulty breathing, dizziness) to any ingredient in sapropterin

Contact your doctor or health care provider right away if any of these apply to you.

Slideshow: View Frightful (But Dead Serious) Drug Side Effects

Before using sapropterin:

Some medical conditions may interact with sapropterin. Tell your doctor or pharmacist if you have any medical conditions, especially if any of the following apply to you:

  • if you are pregnant, planning to become pregnant, or are breast-feeding
  • if you are taking any prescription or nonprescription medicine, herbal preparation, or dietary supplement
  • if you have allergies to medicines, foods, or other substances
  • if you have liver or kidney problems, fever, poor nutrition, or loss of appetite

Some MEDICINES MAY INTERACT with sapropterin. Tell your health care provider if you are taking any other medicines, especially any of the following:

  • Levodopa because seizures, irritability, or overstimulation may occur
  • Methotrexate because it may decrease sapropterin's effectiveness
  • Avanafil, sildenafil, tadalafil, or vardenafil because the risk of their side effects may be increased by sapropterin

This may not be a complete list of all interactions that may occur. Ask your health care provider if sapropterin may interact with other medicines that you take. Check with your health care provider before you start, stop, or change the dose of any medicine.

How to use sapropterin:

Use sapropterin as directed by your doctor. Check the label on the medicine for exact dosing instructions.

  • An extra patient leaflet is available with sapropterin. Talk to your pharmacist if you have questions about this information.
  • Take sapropterin by mouth with a meal.
  • Sapropterin may be swallowed whole or dissolved in 4 to 8 oz (120 to 240 mL) of water or apple juice before swallowing. Allow the tablet to dissolve and drink within 15 minutes.
  • It may take a few minutes for the medicine to dissolve. You may crush or stir the tablet to make it dissolve faster.
  • The tablet may not dissolve completely. There may be small pieces floating on top of the water or apple juice. This is normal and safe for you to swallow.
  • You may still see small pieces of the tablet in the cup after you take your dose. If this occurs, rinse the container with an additional small amount of water or juice and drink the contents to ensure the entire dose is taken.
  • Sapropterin may also be crushed and mixed with a small amount of soft food like applesauce or pudding.
  • Take sapropterin at about the same time each day.
  • Continue to take sapropterin even if you feel well. Do not miss any doses.
  • If you miss a dose of sapropterin, take it as soon as you remember that day. If you remember on the next day, skip the missed dose and go back to your regular dosing schedule. Do not take 2 doses on the same day.

Ask your health care provider any questions you may have about how to use sapropterin.

Important safety information:

  • Follow the diet program given to you by your health care provider. Do not make any changes to your dietary phenylalanine intake without discussing it with your doctor first.
  • If you have a fever or you are sick, your blood phenylalanine level may go up. Contact your doctor as soon as possible if this occurs. Your dose may need to be adjusted.
  • Some people have had low blood phenylalanine levels with sapropterin. The risk is increased in children younger than 7 years old. Blood levels that are too low for a long time may lead to serious health problems. Discuss any questions or concerns with your doctor.
  • Lab tests, including blood phenylalanine levels and liver function, may be performed while you use sapropterin. These tests may be used to monitor your condition or check for side effects. Be sure to keep all doctor and lab appointments.
  • PREGNANCY and BREAST-FEEDING: If you think you may be pregnant, contact your doctor. You will need to discuss the benefits and risks of using sapropterin while you are pregnant. It is not known if sapropterin is found in breast milk. If you are or will be breast-feeding while you use sapropterin, check with your doctor. Discuss any possible risks to your baby.

Possible side effects of sapropterin:

All medicines may cause side effects, but many people have no, or minor, side effects. Check with your doctor if any of these most COMMON side effects persist or become bothersome:

Diarrhea; headache; runny or stuffy nose; sore throat; vomiting.

Seek medical attention right away if any of these SEVERE side effects occur:

Severe allergic reactions (rash; hives; itching; difficulty breathing; tightness in the chest; swelling of the mouth, face, lips, or tongue; wheezing); black, bloody, or tarry stools; chest pain; coughing; dizziness or light-headedness; fainting; fidgeting or moving around too much; flushing; frequent urination; irritability; seizures; severe or persistent stomach pain, nausea, or vomiting; shortness of breath; swelling of the hands, ankles, or feet; talking too much; vomit that looks like coffee grounds.

This is not a complete list of all side effects that may occur. If you have questions about side effects, contact your health care provider. Call your doctor for medical advice about side effects. To report side effects to the appropriate agency, please read the Guide to Reporting Problems to FDA.

If OVERDOSE is suspected:

Contact 1-800-222-1222 (the American Association of Poison Control Centers), your local poison control center, or emergency room immediately.

Proper storage of sapropterin:

Store sapropterin at room temperature, between 68 and 77 degrees F (20 and 25 degrees C). Store in original bottle with the cap closed tightly. Store away from heat, moisture, and light. Do not store in the bathroom. Do not remove the small packet (desiccant) included with your tablets; it absorbs moisture. Keep sapropterin out of the reach of children and away from pets.

General information:

  • If you have any questions about sapropterin, please talk with your doctor, pharmacist, or other health care provider.
  • Sapropterin is to be used only by the patient for whom it is prescribed. Do not share it with other people.
  • If your symptoms do not improve or if they become worse, check with your doctor.
  • Check with your pharmacist about how to dispose of unused medicine.

This information should not be used to decide whether or not to take sapropterin or any other medicine. Only your health care provider has the knowledge and training to decide which medicines are right for you. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a brief summary of general information about sapropterin. It does NOT include all information about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to sapropterin. This information is not specific medical advice and does not replace information you receive from your health care provider. You must talk with your health care provider for complete information about the risks and benefits of using sapropterin.

Issue Date: July 2, 2014
Database Edition 14.3.1.001
Copyright © 2014 Wolters Kluwer Health, Inc.

Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only your health care provider has the knowledge and training to decide which medicines are right for you. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a brief summary of general information about this medicine. It does NOT include all information about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not specific medical advice and does not replace information you receive from your health care provider. You must talk with your healthcare provider for complete information about the risks and benefits of using this medicine.

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