Generic Name and Formulations:
Agalsidase beta 5mg, 35mg; per vial; pwd for IV infusion after reconstitution; preservative-free.
Sanofi Genzyme Company
Indications for FABRAZYME:
Adults and Children:
<8yrs: not recommended. ≥8yrs: Pretreat with antipyretic and antihistamine. Give by IV infusion. 1mg/kg infused every 2 weeks. Initial infusion rate should be ≤0.25mg/min; if tolerated, may increase infusion rate in increments of 0.05–0.08mg/min with each subsequent infusion. <30kg: max rate should remain at 0.25mg/min. ≥30kg: administration duration should not be less than 1.5hrs. Slow rate in the event of infusion reactions. Rechallenge (see literature): initially should be 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 30 minutes up to max 0.25mg/min).
Have appropriate medical support measures available if anaphylaxis or severe allergic reactions develop. Compromised cardiac function; monitor closely. Consider testing for IgE antibodies in patients who experienced suspected allergic reactions; if positive, may be rechallenged. Pregnancy (Cat.B). Nursing mothers.
Recombinant human alpha-galactosidase A enzyme.
Infusion reactions (chills, fever, feeling hot or cold, dyspnea, nausea, flushing, headache, vomiting, paresthesia, fatigue, pruritus, pain in extremity, hypertension, chest pain, throat tightness, abdominal pain, dizziness, tachycardia, nasal congestion, diarrhea, peripheral edema, myalgia, back pain, pallor, bradycardia, urticaria, hypotension, face edema, rash, somnolence; if severe, discontinue immediately), anaphylaxis and allergic reactions (may be fatal; discontinue immediately).
Register pregnant patients at www.fabryregistry.com or call (800) 745-4447.