Inborn errors of metabolism:
Indications for VIMIZIM:
Mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome).
Adults and Children:
<5yrs: not established. Give by IV infusion over 3.5–4.5 hours based on infusion volume. ≥5yrs: 2mg/kg once weekly. Pre-treat with antihistamines with or without antipyretics 30–60 mins prior to infusion.
Boxed Warning:
Risk of anaphylaxis.
Warnings/Precautions:
Risk of serious anaphylactic reactions; observe patients during and after administration. Have epinephrine inj available. Discontinue immediately if severe hypersensitivity reactions occur. Acute febrile or respiratory illness; evaluate clinical status prior to therapy and consider delaying infusion. Risk of sleep apnea; consider evaluating airway patency prior to initiation. Have supplemental oxygen or continuous positive airway pressure available. Monitor for signs/symptoms of spinal or cervical cord compression. Pregnancy. Nursing mothers.
Pharmacologic Class:
Hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme.
Adverse Reactions:
Pyrexia, vomiting, headache, nausea, abdominal pain, chills, fatigue; sleep apnea, anaphylaxis, possible antibody formation.
Note:
To enroll in the Morquio A Registry for monitoring effects of Vimizim contact MARS@bmrn.com or call (800) 983-4587.
How Supplied:
Single-use vial (5mL)—1
