Sapropterin Dosage

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Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Phenylketonuria

Initial dose: 10 mg/kg orally once a day with food
Maintenance dose: Once responsiveness established, dose may be adjusted within the range of 5 to 20 mg/kg/day according to response to therapy.

Comments:
-Response to therapy is determined by change in blood phenylalanine (Phe) after treatment with sapropterin (10 mg/kg/day) for up to 1 month.
-Blood Phe levels should be checked after 1 week of therapy and periodically for up to a month.
-If blood Phe does not decrease from baseline at 10 mg/kg/day, dose may be increased to 20 mg/kg/day.
-If blood Phe does not decrease after 1 month at 20 mg/kg/day, patient is a non-responder and sapropterin should be discontinued.

Approved indication: In conjunction with a Phe-restricted diet, to reduce blood Phe levels in patients with hyperphenylalaninemia due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria

Usual Pediatric Dose for Phenylketonuria

4 years or older:
Initial dose: 10 mg/kg orally once a day with food
Maintenance dose: Once responsiveness established, dose may be adjusted within the range of 5 to 20 mg/kg/day according to response to therapy.

Comments:
-Response to therapy is determined by change in blood Phe after treatment with sapropterin (10 mg/kg/day) for up to 1 month.
-Blood Phe levels should be checked after 1 week of therapy and periodically for up to a month; frequent blood monitoring recommended to ensure adequate blood Phe level control.
-If blood Phe does not decrease from baseline at 10 mg/kg/day, dose may be increased to 20 mg/kg/day.
-If blood Phe does not decrease after 1 month at 20 mg/kg/day, patient is a non-responder and sapropterin should be discontinued.

Approved indication: In conjunction with a Phe-restricted diet, to reduce blood Phe levels in patients with hyperphenylalaninemia due to BH4-responsive phenylketonuria

Renal Dose Adjustments

Data not available

Comments:
-Patients with renal dysfunction should be carefully monitored when receiving sapropterin.

Liver Dose Adjustments

Data not available

Comments:
-Patients with liver dysfunction should be carefully monitored when receiving sapropterin; hepatic damage associated with impaired Phe metabolism.

Precautions

Consult WARNINGS section for dosing related precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Take with food to increase absorption, preferably same time each day.
-May swallow tablets whole or dissolved in water or apple juice; take dissolved tablets within 15 minutes of dissolution.
-Take dissolved powder for oral solution within 30 minutes of dissolution.
-Take a missed dose as soon as possible, but do not take 2 on the same day.

Storage requirements:
-Protect from moisture.

Reconstitution/preparation techniques:
-Tablets may be dissolved in 4 to 8 ounces (120 to 240 mL) of water or apple juice; the manufacturer's product information should be consulted for further information.
-Powder for oral solution should be dissolved in 4 to 8 ounces (120 to 240 mL) of water or apple juice; should dissolve rapidly and completely.

Monitoring:
-Gastrointestinal: Signs of gastritis
-Hepatic: Liver function tests in patients with liver dysfunction
-Metabolic: Blood Phe levels (during therapy); blood Phe levels in pediatric patients (frequently)
-Nervous system: For hyperactivity

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