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 (Cat. C). 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

Pricing for VIMIZIM

5ml of 1mg/ml vial (Qty: 1)
Appx. price $1137
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