Kuvan 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

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. Doses of Kuvan above 20 mg/kg/day have not been evaluated in clinical trials.

Administration

Kuvan (sapropterin dihydrochloride) Tablets should be administered orally with food to increase absorption, preferably at the same time each day. Kuvan Tablets should be dissolved in 4 to 8 oz. (120 to 240 mL) of water or apple juice and taken 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. A missed dose should be taken as soon as possible, but 2 doses should not be taken on the same day.

Dosage Forms And Strengths

Kuvan (sapropterin dihydrochloride) Tablets are unscored, uncoated, immediate-release tablets 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 (sapropterin dihydrochloride) Tablets are supplied in high-density polyethylene bottles, sealed with aluminized film, and closed with child-resistant caps. Each bottle contains 120 tablets, a silica gel desiccant cartridge, and a pharmaceutical-grade polyester coil. Each Kuvan Tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base).

Bottle of 120 tablets…………………………NDC 68135-300-02

Storage

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

Manufactured for: BioMarin Pharmaceutical Inc. Novato, CA 94949 Manufactured by: Lyne Laboratories, Inc. Brockton, MA 02301. FDA Rev date: 12/13/2007

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