Medical Uses
Sephience (sepiapterin) is a phenylalanine hydroxylase (PAH) activator used for the treatment of hyperphenylalaninemia (HPA) in adult and pediatric patients aged 1 month and older with sepiapterin-responsive phenylketonuria (PKU). This therapeutic drug is to be used in conjunction with a phenylalanine (Phe)- restricted diet.
Recommended Dosage:
Patients receiving Sepiapterin should follow a dietary protein- and Phe-restricted diet. The medicine is administered orally once daily with food. The recommended starting dosage of Sepiapterin varies according to age. For infants aged less than 6 months, the dosage is 7.5 mg/kg daily. For children aged 6 months to less than 1 year, the dosage increases to 15 mg/kg daily. For those aged 1 year to less than 2 years, the dosage is 30 mg/kg daily. For patients aged 2 years and older, the recommended dosage is 60 mg/kg daily. For calculated daily doses of less than 1,000 mg, this medicine should be prepared as a liquid mixture with a concentration of 25 mg/mL, and the exact prescribed dose volume (in mL) must be administered.