Fabrazyme

Agalsidase β

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

Recommended dose: 1 mg/kg body wt every 2 wk as IV infusion. Infusion rate: Initially not >0.25 mg/min (15 mg/hr). When patient tolerance is established, rate may be increased in 0.05-0.08 mg/min increments (3-5 mg/hr). Patient ≥30 kg Duration not <1.5 hr. <30 kg Max infusion rate: 0.25 mg/min (15 mg/hr). Rechallenge Initially 0.5 mg/kg at 0.01 mg/min. When patient tolerance is established, dose may be increased to 1 mg/kg, slowly titrating infusion rate. Max infusion rate: 0.25 mg/min.

Discontinue in case of anaphylaxis & severe allergic reactions. Severe infusion reactions. Pre-treatment w/ antipyretics prior to infusion is recommended. Compromised cardiac function. IgE antibodies or skin test reactivity specific to Fabrazyme should be performed prior to initiation of treatment. Pregnancy & lactation. Childn <8 yr. Elderly >65 yr.

Anaphylactic & allergic reactions. Infusion reactions eg, chills, vomiting, hypotension, paresthesia, pyrexia, feeling hot or cold, dyspnea, nausea, flushing, headache, fatigue, pruritus, pain in extremity, HTN, throat tightness, chest, abdominal or back pain, dizziness, tachycardia, nasal congestion, diarrhoea, peripheral oedema, pallor, myalgia, urticaria, bradycardia, somnolence. Stroke, pain, ataxia, bradycardia, cardiac arrhythmia or arrest, decreased cardiac output, vertigo, hypoacousia, nephrotic syndrome.

Other Agents Affecting Metabolism

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

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