Sapropterin Dosage

This dosage information may not include all the information needed to use Sapropterin safely and effectively. See additional information for Sapropterin.

The information at Drugs.com is not a substitute for medical advice. ALWAYS consult your doctor or pharmacist.

Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Phenylketonuria

Initial dose: 10 mg/kg/day taken orally with food once daily, preferably at the same time each day.

Sapropterin tablets should be dissolved in 4 to 8 ounces (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 two doses should not be taken on the same day.

Response to therapy is determined by change in blood phenylalanine (Phe) following treatment with sapropterin at 10 mg/kg/day for a period of up to one month. Blood Phe levels should be checked after one week of sapropterin 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 one month of treatment at 20 mg/kg/day are non-responders, and treatment with sapropterin should be discontinued in these patients.

Once responsiveness to sapropterin 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 sapropterin above 20 mg/kg/day have not been evaluated in clinical trials.

Usual Pediatric Dose for Phenylketonuria

The safety and efficacy of sapropterin in pediatric patients less than 4 years of age have not been assessed in clinical studies.

4 years or older:

Initial dose: 10 mg/kg/day taken orally with food once daily, preferably at the same time each day.

Sapropterin tablets should be dissolved in 4 to 8 ounces (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 two doses should not be taken on the same day.

Response to therapy is determined by change in blood phenylalanine (Phe) following treatment with sapropterin at 10 mg/kg/day for a period of up to one month. Blood Phe levels should be checked after one week of sapropterin treatment and periodically for up to a month. Frequent blood monitoring is recommended in the pediatric population to ensure adequate blood Phe level control. 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 one month of treatment at 20 mg/kg/day are non-responders, and treatment with sapropterin should be discontinued in these patients.

Once responsiveness to sapropterin 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 sapropterin above 20 mg/kg/day have not been evaluated in clinical trials.

Renal Dose Adjustments

Patients with renal impairment have not been evaluated in clinical trials. Patients who have renal impairment should be carefully monitored when receiving sapropterin.

Liver Dose Adjustments

Patients with liver impairment have not been evaluated in clinical trials with sapropterin. Patients who have liver impairment should be carefully monitored when receiving sapropterin because hepatic damage has been associated with impaired Phe metabolism.

Dialysis

Data not available

Hide
(web4)