Inborn errors of metabolism:
Indications for ELAPRASE:
Mucopolysaccharidosis II (MPS II; Hunter syndrome).
Adults and Children:
<16mos: not established. Give by IV infusion over 3hrs; may gradually reduce to 1hr if no hypersensitivity reactions occur; max 8hrs. ≥16mos: 0.5mg/kg once weekly. Initial infusion rate: 8mL/hr for first 15mins; if tolerated, may increase by 8mL/hr increments every 15mins; max rate 100mL/hr.
Risk of anaphylaxis.
Monitor for anaphylactic or other acute reactions; discontinue immediately if occur. Have resuscitative measures available. Risk of hypersensitivity, serious adverse reactions, antibody development in those with severe genetic mutations. Acute febrile or respiratory illness; evaluate clinical status and consider delaying infusion. Risk of acute cardiorespiratory failure if susceptible to fluid overload, acute underlying respiratory illness, compromised cardiac and/or respiratory function; may require prolonged observation. Pregnancy. Nursing mothers.
Hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme.
Headache, pruritus, musculoskeletal pain, urticaria, diarrhea, cough, pyrexia, rash, vomiting; hypersensitivity reactions, flushing, hypoxic episodes.
Generic Drug Availability:
Single-use vial (3mL)—1