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.

Boxed Warning:

Risk of anaphylaxis.

ELAPRASE Warnings/Precautions:

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.

ELAPRASE Classification:

Hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme.

Adverse Reactions:

Headache, pruritus, musculoskeletal pain, urticaria, diarrhea, cough, pyrexia, rash, vomiting; hypersensitivity reactions, flushing, hypoxic episodes.

Generic Drug Availability:

NO

How Supplied:

Single-use vial (3mL)—1

Pricing for ELAPRASE

3ml of 6mg/3ml vial (Qty: 1)
Appx. price $3208
GoodRx