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(sapropterin dihydrochloride) Tablets

(sapropterin dihydrochloride) Powder for Oral Solution

What is Kuvan?

Kuvan is a prescription medicine used to lower blood levels of phenylalanine (Phe), in people with a certain type of Phenylketonuria (PKU). Kuvan is used along with a Phe-restricted diet.

What should I tell my doctor before taking Kuvan?

Before you take Kuvan, tell your doctor if you:

  • have a fever
  • have liver or kidney problems
  • are allergic to sapropterin dihydrochloride or any of the ingredients in Kuvan. See the list of ingredients in Kuvan at the end of this leaflet.
  • have poor nutrition or have loss of appetite
  • are pregnant or plan to become pregnant.
    Pregnancy Registry: There is a pregnancy registry for women who take Kuvan during pregnancy. The purpose of this registry is to collect information about the health of you and your baby. Talk to your healthcare provider about how you can take part in this registry.
  • are breastfeeding or plan to breastfeed. It is not known if Kuvan passes into your breast milk. Talk to your doctor about the best way to feed your baby if you take Kuvan.

Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines, vitamins, herbal, and dietary supplements. Kuvan and other medicines may interact with each other.

Especially tell your doctor if you take:

  • a medicine that contains levodopa
  • an antifolate medicine
  • avanafil (Stendra), sildenafil (Revatio, Viagra), tadalafil (Adcirca, Cialis), vardenafil (Staxyn, Levitra)

Tell your doctor if you are not sure if your medicine is one that is listed above.

Know the medicines you take. Keep a list of them to show your doctor and pharmacist when you get a new medicine.

How should I take Kuvan?

  • Take Kuvan exactly as your doctor tells you. Your doctor should tell you how much Kuvan to take and when to take it.
  • Your doctor may change your dose of Kuvan depending on how you respond to treatment.
  • Take Kuvan 1 time each day with food. It is best to take Kuvan at the same time each day.
  • Kuvan comes as a tablet and powder for oral solution.
    • You can swallow Kuvan tablets whole or dissolve the tablets in water or apple juice before taking.
    • Kuvan powder for oral solution should be dissolved in water or apple juice before taking.
    • See the “Instructions for Use” at the end of this Patient Information leaflet for information about the correct way to dissolve and take a dose of Kuvan tablets or Kuvan powder for oral solution.
  • It is not possible to know if Kuvan will work for you until you start taking Kuvan. Your doctor will check your blood Phe levels when you start taking Kuvan to see if the medicine is working.
  • During treatment with Kuvan:
    • any change you make to your diet may affect your blood Phe level. Follow your doctor's instructions carefully and do not make any changes to your dietary Phe intake without first talking with your doctor. Even if you take Kuvan, if your Phe blood levels are not well controlled, you can develop severe neurologic problems.
    • Your doctor should continue to monitor your blood Phe levels often during your treatment with Kuvan, to make sure that your blood Phe levels are not too high or too low.
    • If you have a fever, or if you are sick, your blood Phe level may go up. Tell your doctor as soon as possible so they can change your dose of Kuvan to help keep your blood Phe levels in the desired range.
  • If you forget to take your dose of Kuvan, take it as soon as you remember that day. Do not take 2 doses in a day.
  • If you take too much Kuvan, call your doctor for advice.

What are the possible side effects of Kuvan?

Kuvan can cause serious side effects, including:

  • Severe allergic reactions. Stop taking Kuvan and get medical help right away if you develop any of these symptoms of a severe allergic reaction:
    • flushing
    • wheezing or trouble breathing
    • nausea
    • rash
    • coughing
    • feeling lightheaded or you faint
  • Inflammation of the lining of the stomach (gastritis). Gastritis can happen with Kuvan and may be severe. Call your doctor right away if you have any of these signs or symptoms:
    • severe upper stomach-area (abdominal) discomfort or pain, nausea and vomiting
    • blood in your vomit or stool
    • black, tarry stools
  • Too much or constant activity (hyperactivity) can happen with Kuvan. Tell your doctor if you have any signs of hyperactivity, including:
    • fidgeting or moving around too much
    • talking too much

The most common side effects of Kuvan are:

Tell your doctor if you have any side effect that bothers you or that does not go away.

These are not all the possible side effects of Kuvan. For more information, ask your doctor or pharmacist. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

How should I store Kuvan?

  • Store Kuvan at room temperature between 68°F to 77°F (20°C to 25°C).
  • Keep Kuvan tablets in the original bottle with the cap closed tightly.
  • Protect from moisture. Do not remove the desiccant (the small packet included with your tablets). The desiccant absorbs moisture.

Keep Kuvan and all medicines out of the reach of children.

General information about the safe and effective use of Kuvan

Medicines are sometimes prescribed for purposes other than those listed in a Patient Information leaflet. Do not use Kuvan for a condition for which it was not prescribed. Do not give Kuvan to other people, even if they have the same symptoms you have. It may harm them.

If you would like more information, talk with your doctor. You can ask your pharmacist or doctor for information about Kuvan that is written for health professionals. For more information, call BioMarin Patient and Physician Support (BPPS) at 1-866-906-6100.

What are the ingredients in Kuvan?

Active ingredient: sapropterin dihydrochloride.

Kuvan tablet inactive ingredients: ascorbic acid, crospovidone, dibasic calcium phosphate, D-mannitol, riboflavin, and sodium stearyl fumarate.

Kuvan powder for oral solution inactive ingredients: ascorbic acid, D-mannitol, potassium citrate, and sucralose.

Instructions for Use

If you are taking Kuvan tablets:

KUVAN tablets can be swallowed whole or dissolved in water or apple juice as follows:

  • To dissolve Kuvan tablets, mix them in 4 to 8 ounces (½ to 1 cup) of water or apple juice.
  • It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, you can stir or crush them.
  • The tablets may not dissolve completely. You may see small pieces floating on top of the water or apple juice. This is normal and safe for you to swallow.
  • Drink within 15 minutes.
  • After drinking your medicine, if you still see small pieces of the tablet, add more water or apple juice and drink, to make sure that you take all of your medicine.

If you are taking Kuvan powder for oral solution:

  • Kuvan powder for oral solution should be dissolved in water or apple juice as follows: To open a packet of Kuvan powder for oral solution, fold and tear or cut at the dotted line in the upper right corner of the packet.
  • Empty the contents of the packet into 4 to 8 ounces (½ to 1 cup) of water or apple juice. The powder should dissolve completely within 15 seconds.
  • Drink within 30 minutes.

This Patient Information has been approved by the U.S. Food and Drug Administration.

Last reviewed on RxList: 1/6/2014
This monograph has been modified to include the generic and brand name in many instances.

Report Problems to the Food and Drug Administration


You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.

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