Inborn errors of metabolism:
Indications for LUMIZYME:
Pompe disease (GAA deficiency).
Adults and Children:
Give by IV infusion over 4 hours. 20mg/kg every 2 weeks. Initial infusion rate: 1mg/kg/hr; may increase by 2mg/kg/hr every 30 minutes, if tolerated, to max 7mg/kg/hr.
Discontinue immediately if severe anaphylactic or allergic reactions develop; have appropriate medical support measures available and during rechallenge. Increased risk of acute cardiorespiratory failure with acute underlying respiratory illness or compromised cardiac function. Cardiac hypertrophy (increased risk of cardiac arrhythmia when given general anesthesia for central venous catheter placement). Infants with cardiac dysfunction may require prolonged observation times. Monitor for development of systemic immune complex-mediated reactions; consider discontinuing if occurs. Monitor for IgG antibody formation every 3 months for 2 years, then annually. Labor & delivery. Pregnancy. Nursing mothers (consider interrupting breastfeeding during and for 24hrs after therapy).
Hydrolytic lysosomal glycogen-specific enzyme.
Hypersensitivity reactions, anaphylaxis, rash, purexia, flushing/feeling hot, urticaria, headache, hyperhidrosis, nausea, cough, decreased oxygen saturation, tachycardia, tachypnea, chest discomfort, dizziness, muscle twitching, agitation, cyanosis, erythema, hypertension, pallor, rigors, tremor, vomiting, fatigue, myalgia.
To enroll in the Pompe registry call (800) 745-4447.
Single-use vial (50mg)—1, 10