Kuvan

Generic name: Sapropterin
Drug class: Miscellaneous metabolic agents

Usage of Kuvan

Kuvan (sapropterin) is used along with a restricted diet to control blood phenylalanine levels in adults and children one month of age and older who have phenylketonuria (PKU; an inborn condition in which phenylalanine may build up in the blood and causes decreased intelligence and a decreased ability to focus, remember, and organize information).

Kuvan will only work for some people who have PKU, and the only way to tell if Kuvan will help a particular patient is to give the medication for a period of time and see whether his or her phenylalanine level decreases.

Kuvan belongs to a class of medications called cofactors. It works by helping the body to break down phenylalanine so it will not build up in the blood.

Kuvan side effects

Get emergency medical help if you have signs of an allergic reaction to Kuvan: hives, rash, wheezing, cough, difficult breathing, nausea, feeling light-headed, flushing (warmth, redness, or tingly feeling), swelling of your face, lips, tongue, or throat.

Kuvan may cause serious side effects. Call your doctor at once if you have:

behavior changes, seizures, or problems with speech;

fever, loss of appetite, difficulty swallowing, throat pain, severe stomach discomfort or pain, nausea, vomiting;

bloody or tarry stools, coughing up blood or vomit that looks like coffee grounds; or

hyperactive behavior, such as fidgeting or moving around often, or talking too much.

Common Kuvan side effects may include:

headache;

diarrhea;

vomiting; or

runny or stuffy nose, sore throat, cough.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Before taking Kuvan

You should not use Kuvan if you are allergic to sapropterin.

To make sure Kuvan is safe for you, tell your doctor if you have ever had:

stomach ulcer or bleeding; or

loss of appetite, or if you are malnourished.

It is not known if sapropterin will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

If you are pregnant, your name may be listed on a pregnancy registry to track the effects of sapropterin on the baby.

Ask a doctor if it is safe to breastfeed while using Kuvan.

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How to use Kuvan

Usual Adult Dose of Kuvan for Phenylketonuria:

Initial dose: 10 to 20 mg/kg orally once a day -Blood phenylalanine (Phe) levels should be measured after 1 week, and then periodically; dose should be increased to 20 mg/kg/day if blood Phe levels have not decreased Maintenance dose: Once responsiveness has been established, dose should be adjusted within the range of 5 to 20 mg/kg/day according to biochemical response. Non-Responders: -Patients whose phenylalanine levels do not decrease with treatment at 20 mg/kg/day for 1 month should be considered non-responders and treatment should be discontinued. Comments: -This drug should be used in conjunction with a phenylalanine-restricted diet. -Existing dietary protein and Phe intake should not be modified during the evaluation period -Response to therapy is determined by change in blood phenylalanine levels. Use: To reduce blood phenylalanine levels in patients with hyperphenylalaninemia due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).

Usual Pediatric Dose of Kuvan for Phenylketonuria:

1 month to 6 years: -Initial dose: 10 mg/kg orally once a day 7 years or older: -Initial dose: 10 to 20 mg/kg orally once a day -Measure blood phenylalanine levels after 1 week, and then periodically; dose should be increased to 20 mg/kg/day if blood phenylalanine levels have not decreased Maintenance dose: Once responsiveness has been established, dose should be adjusted within the range of 5 to 20 mg/kg/day according to biochemical response. Non-Responders: -Patients whose phenylalanine levels do not decrease with treatment at 20 mg/kg/day for 1 month should be considered non-responders and treatment should be discontinued. Comments: -This drug should be used in conjunction with a phenylalanine-restricted diet. -Existing dietary protein and Phe intake should not be modified during the evaluation period -Response to therapy is determined by change in blood phenylalanine levels. Use: To reduce blood phenylalanine levels in patients with hyperphenylalaninemia due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).

Warnings

Before using Kuvan, tell your doctor if you are allergic to any drugs, or if you have liver disease, a history of stomach ulcer or bleeding, or a seizure disorder.

Also tell your doctor about any medications you use, especially levodopa (Larodopa, Altamet, Parcopa, Sinemet), methotrexate (Rheumatrex, Trexall), or any medication to treat erectile dysfunction, such as sildenafil (Viagra), tadalafil (Cialis), or vardenafil (Levitra).

If Kuvan is not controlling your condition properly, you may have symptoms such as behavior changes, numbness or tingling, or problems with speech, vision, or balance. Call your doctor if you have any of these symptoms.

What other drugs will affect Kuvan

Tell your doctor about all your other medicines, especially:

levodopa;

methotrexate, valproic acid, phenobarbital, trimethoprim; or

medicine to treat impotence or pulmonary arterial hypertension - sildenafil (Viagra, Revatio), avanafil (Stendra), tadalafil (Adcirca, Cialis) vardenafil (Levitra, Staxyn).

This list is not complete. Other drugs may interact with sapropterin, including prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.

Disclaimer

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