Inborn errors of metabolism:
Indications for FABRAZYME:
Adults and Children:
<8yrs: not established. Pretreat with antipyretic and antihistamine. Give by IV infusion. ≥8yrs: 1mg/kg every 2 weeks. Initially infuse at a rate of ≤0.25mg/min; if tolerated, may increase by increments of 0.05–0.08mg/min with each subsequent infusion. <30kg: max rate 0.25mg/min. ≥30kg: minimum duration is 1.5hrs. Slow rate if infusion reactions occur. Rechallenge: initially give a low dose and infusion rate (eg, 0.5mg/kg at 0.01mg/min); once tolerated, may increase dose to 1mg/kg, titrate rate slowly upwards (doubled every 30mins up to max 0.25mg/min); see full labeling.
Have appropriate medical support measures readily available; discontinue if anaphylaxis or severe allergic reactions occur. Compromised cardiac function; monitor closely. Consider testing for IgE antibodies in patients who experienced suspected allergic reactions; if positive, may be rechallenged. Pregnancy. Nursing mothers.
Recombinant human alpha-galactosidase A enzyme.
Upper RTI, chills, pyrexia, headache, cough, paresthesia, fatigue, peripheral edema, dizziness, rash; infusion reactions (discontinue if severe), anaphylaxis, allergic reactions.
Register pregnant patients at www.fabryregistry.com or call (800) 745-4447.