KUVAN Patient Information

Download the KUVAN Full Prescribing Information in PDF format.

What is Kuvan?
How does Kuvan work?
Who may benefit from taking Kuvan?
What are the risks of taking Kuvan?
What should I tell my doctor before taking Kuvan?
How should I take Kuvan?
What are the possible side effects of Kuvan?
How should I store Kuvan?
General information about Kuvan
What are the ingredients in Kuvan?

For people with PKU
Kuvan (COO-van) (sapropterin dihydrochloride) Tablets
Read this information before you start taking Kuvan and each time you get a refill. There may be new information. This information does not take the place of talking with your doctor about your medical condition or treatment.

What is Kuvan?

Kuvan is a medicine for people with phenylketonuria (PKU). An enzyme in your body PAH (phenylalanine hydroxylase) helps break down phenylalanine (Phe), an amino acid found in food. In patients with PKU this enzyme does not work right. PKU leads to high blood Phe levels. 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.

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How does Kuvan work?

Kuvan acts in your body with the enzyme PAH to reduce your blood Phe levels. Your doctor and health care team will continue to monitor your blood Phe levels and dietary Phe intake.

Kuvan are tablets that you should dissolve in water or apple juice before taking.

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Who may benefit from taking Kuvan?

It is not possible to know whether or not Kuvan will work for you until you start taking Kuvan. Your doctor will monitor your blood Phe levels when you start taking Kuvan to see if the drug is working.

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What are the risks of taking Kuvan?

When you are taking 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 before discussing with your doctor. Your doctor will continue to monitor your blood Phe levels during your treatment with Kuvan.

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 see if they have to adjust your treatment to help keep your blood Phe levels in the desired range.

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What should I tell my doctor before taking Kuvan?

Before you start taking Kuvan, let your doctor know about all of your medical conditions, including if you:

Have a fever
Are pregnant or planning to become pregnant
Are breast feeding
Have liver problems
Are allergic to Kuvan or any other medications
Have poor nutrition or are anorexic
Are taking levodopa
Are taking drugs that inhibit folate metabolism (e.g., methotrexate) because these drugs could affect how Kuvan works in your body
Are taking medicines for erectile dysfunction like Viagra (sildenafil), Levitra (vardenafil), or Cialis (tadalafil)

Tell your doctor about all the medicines you take, including prescription and nonprescription medicines and herbal and dietary supplements. Kuvan and many other medicines may interact with each other. Your doctor needs to know what medicines you take so he or she can decide if Kuvan is right for you.

Know the medicines you take. Keep a list of your medicines with you to show your doctor. Do not take other medicines while taking Kuvan without first talking to your doctor.

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How should I take Kuvan?

Kuvan Tablets are taken at one time each day. Take Kuvan exactly as your doctor has told you.

Take Kuvan once a day with food and preferably at the same time each day.
Kuvan Tablets should be dissolved in 4 to 8 ounces (1/2 to 1 cup) of water or apple juice.
To dissolve the tablets, mix them in water or apple juice, and drink within 15 minutes.
- 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.
- If after drinking your medicine you still see small pieces of the tablets, you should add more water or apple juice to make sure that you take all of your medicine.
If you forget to take your dose of Kuvan, take it as soon as you remember that day. If you miss a day, do not double your dose the next day, just skip the missed dose.
The recommended starting dose of Kuvan is 10 mg/kg taken once a day. Your doctor will tell you the dose you should take and when to take it.
Your doctor can change your dose depending on how you respond to treatment.

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What are the possible side effects of Kuvan?

The most common side effects reported when using Kuvan are:

Headache
Diarrhea
Abdominal pain
Upper respiratory tract infection (like a cold)
Throat pain
Vomiting
Nausea

These are not all the side effects seen with Kuvan. If you are concerned about these or any other side effects you experience while taking Kuvan, ask your doctor or pharmacist for more information.

Be sure to tell your doctor if you have any side effects when you are taking Kuvan.

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How should I store Kuvan?

Store in a cool, dry place between 68°F and 77°F (20°–25°C).
Do not leave Kuvan in hot or humid places, such as your car or bathroom cabinet.
Keep Kuvan in its original bottle with the cap closed tightly.
Protect from moisture. Do not remove the dessicant (the small packet included with your tablets). The dessicant absorbs moisture.
The color of the tablets may change over time to light yellow. This is normal and you can take these tablets.
Do not keep Kuvan that is out of date, or that you no longer need. Be sure that if you throw any medicine away, it is out of the reach of children.
Keep Kuvan and all medicines out of the reach of children.

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General information about Kuvan

Medicines are sometimes prescribed for conditions that are not mentioned in patient information leaflets. Do not use Kuvan for any other condition. Do not give Kuvan to anyone else, even if they have the same condition that you have. It may harm them.

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

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What are the ingredients in Kuvan?

Active Ingredient: sapropterin dihydrochloride.

Inactive Ingredients: ascorbic acid, crospovidone, dibasic calcium phosphate, D-mannitol, riboflavin, and sodium stearyl fumarate.

Kuvan Tablets are mottled, off-white to light yellow, and debossed with “177”.

Kuvan is a registered trademark of BioMarin Pharmaceutical Inc.

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Indication

KUVAN^® (sapropterin dihydrochloride) Tablets is 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 Phe-restricted 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 and may adjust your diet 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.