Dosage & Administration

How to prescribe the appropriate KUVAN® dosage to achieve optimal outcomes for patients with phenylketonuria (PKU) or tetrahydrobiopterin (BH4) deficiency.




Dosage & Administration1-3

  • Each soluble tablet contains 100mg of sapropterin dihydrochloride.
  • Each 500mg sachet contains 500mg of sapropterin dihydrochloride.

KUVAN® should be administered with a meal to increase absorption.

For patients with PKU, KUVAN® should be administered as a single daily dose, and at the same time each day, preferably in the morning.

For patients with BH4 deficiency, divide the total daily dose into 2 or 3 administrations and distribute over the day.1

As HPA due to PKU or BH4 deficiency is a chronic condition, once responsiveness is demonstrated, KUVAN® is intended for long-term use.

Active management of dietary Phe and overall protein intake while taking KUVAN® is required to ensure adequate control of blood Phe levels and nutritional balance. Cessation of treatment must be conducted with close physician observation and monitoring due to the possibility of rebound in blood Phe levels above pre-treatment levels.

Please refer to KUVAN® Product Information for details on dose and administration.

Conducting a response test is the only way to identify KUVAN® responsive patients.1


  1. KUVAN® Product Information.
  2. Lee P, et al. Am J Med Genet A. 2008;146A(22):2851-2859.
  3. Muntau AC, et al. 2017 Mar 9;12(1):47.