Fabrazyme

Fabry disease. Reduces globotriaosylceramide (GL-3) deposition in kidney capillary endothelium & certain other cell types.

IV 1 mg/kg infusion every 2 wk. Max initial infusion rate: 0.25 mg/min (15 mg/hr). If tolerated, infusion may be increased in increments of 0.05-0.08 mg/min (3-5 mg/hr) w/ each subsequent infusion. Patient weighing ≥30 kg Min infusion duration: 1.5 hr, <30 kg Max infusion rate: 0.25 mg/min (15 mg/hr). Patient w/ +ve skin test to Fabrazyme or tested +ve for anti-Fabrazyme IgE Initial rechallenge: Low dose at lower infusion rate [eg, ½ the therapeutic dose (0.5 mg/kg) at 1/25^th the initial standard recommended rate (0.01 mg/min or 0.6 mg/hr)]. As tolerated, may be increased to 1 mg/kg & slowly increase infusion by titrating upwards (doubled every 30 min up to max rate of 0.25 mg/min).

Other Agents Affecting Metabolism

A16AB04 - agalsidase beta ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.

POM