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Kuvan

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Kuvan

Kuvan

INDICATIONS

Kuvan® (sapropterin dihydrochloride) is indicated to reduce blood phenylalanine (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.

DOSAGE AND ADMINISTRATION

Dosage

The recommended starting dose of Kuvan is 10 mg/kg/day taken once daily.

Response to therapy is determined by change in blood Phe following treatment with Kuvan at 10 mg/kg/day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg/day, the dose may be increased to 20 mg/kg/day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg/day are non-responders, and treatment with Kuvan should be discontinued in these patients.

Once responsiveness to Kuvan has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg/day according to response to therapy.

Administration

Kuvan is available as tablets and as powder for oral solution. Kuvan should be taken orally with food to increase absorption, preferably at the same time each day. A missed dose should be taken as soon as possible, but 2 doses should not be taken on the same day.

Instructions For Use

Kuvan Tablets

Kuvan tablets may be swallowed either as whole tablets or dissolved in 4 to 8 oz. (120 to 240 mL) of water or apple juice and taken orally within 15 minutes of dissolution. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, stir or crush them. The tablets may not dissolve completely. Patients may see small pieces floating on top of the water or apple juice. This is normal and safe for patients to swallow. If after drinking the medicine patients still see pieces of the tablet, they can add more water or apple juice to make sure that they take all of the medicine.

Kuvan Powder for Oral Solution

Kuvan powder for oral solution should be dissolved in 4 to 8 oz. (120 to 240 mL) of water or apple juice and taken orally within 30 minutes of dissolution. Empty the contents of the packet(s) in water or apple juice and mix thoroughly. The powder should dissolve rapidly and completely.

HOW SUPPLIED

Dosage Forms And Strengths

Kuvan tablets are for oral use. Each tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base). Tablets are round, off-white to light yellow, mottled, and debossed with “177”.

Kuvan powder for oral solution is available as a unit dose packet containing 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base). The powder is off-white to yellow in color.

Storage And Handling

Kuvan tablets, 100 mg, are round, off-white to light yellow, mottled, and debossed with “177”. The tablets are supplied as follows:

NDC 68135-300-01 Bottle of 30 tablets
NDC 68135-300-02 Bottle of 120 tablets

Kuvan powder for oral solution, 100 mg, is an off-white to yellow powder. Kuvan powder is packaged in unit dose packets as follows:

NDC 68135-301-22 Carton of 30 unit dose packets
NDC 68135-301-11 Single unit dose packet

Storage

Store Kuvan tablets at 20°C to 25°C (68°F –77°F); excursions allowed between 15°C to 30°C (59°F –86°F) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture.

Store Kuvan powder for oral solution at 20°C to 25°C (68°F –77°F); excursions allowed between 15°C to 30°C (59°F –86°F) [see USP Controlled Room Temperature]. Protect from moisture.

BioMarin Pharmaceutical Inc. Novato, CA 94949. Revised: 12/2013

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

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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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