Sapropterin Tablets

Generic name: sapropterin dihydrochloride
Dosage form: tablet
Drug class:Miscellaneous metabolic agents

Medically reviewed by Drugs.com. Last updated on Dec 1, 2021.

INDICATIONS AND USAGE

Sapropterin dihydrochloride tablets are indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Sapropterin dihydrochloride is to be used in conjunction with a Phe-restricted diet.

DOSAGE AND ADMINISTRATION

Dosage

Treatment with sapropterin dihydrochloride should be directed by physicians knowledgeable in the management of PKU.

All patients with PKU who are being treated with sapropterin dihydrochloride should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction.

Starting Dosage

Pediatric Patients 1 month to 6 years: The recommended starting dose of sapropterin dihydrochloride is 10 mg/kg taken once daily.

Patients 7 years and older: The recommended starting dose of sapropterin dihydrochloride is 10 to 20 mg/kg taken once daily.

Dosage Adjustment (Evaluation Period)

Existing dietary protein and Phe intake should not be modified during the evaluation period.

If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with sapropterin dihydrochloride at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of sapropterin dihydrochloride treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day do not show a biochemical response and treatment with sapropterin dihydrochloride should be ...