Medical Uses
Xenpozyme (olipudase alfa-rpcp) is a hydrolytic lysosomal sphingomyelin-specific enzyme indicated for the treatment of non–central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) in adult and pediatric patients.
Recommended Dosage:
The recommended dosage of Olipudase alfa-rpcp should be determined in consultation with physicians experienced in the management of ASMD. To avoid dosing errors, including overdosage, follow all instructions for dosage and administration. Obtain baseline transaminase (alanine aminotransferase [ALT] and aspartate aminotransferase [AST]) levels in all patients within 30 days before treatment initiation. Verify pregnancy status in females of reproductive potential.
The recommended adult and pediatric dosages for the dose escalation and maintenance phases are based on body weight as follows for patients with a body mass index (BMI):
- For less than or equal to 30, the dosage is based on actual body weight (kg)
- For those greater than 30, the dosage is based on adjusted body weight (kg). Calculate an adjusted body weight (kg) based on height in meters.