Inborn errors of metabolism:

Indications for: NEXVIAZYME

Late-onset Pompe disease (GAA deficiency).

Adults and Children:

<1yr: not established. Consider premedication with antihistamines, antipyretics, and/or corticosteroids prior to initiation. ≥1yr (<30kg): 40mg/kg every 2 weeks; (≥30kg): 20mg/kg every 2 weeks. Give by IV infusion incrementally, based on response. Duration: between 4–7hrs. Initial infusion rate: 1mg/kg/hr; may gradually increase every 30mins as tolerated; see full labeling. Dosage and administration modifications: see full labeling.

Boxed Warning:

Severe hypersensitivity reactions or infusion-associated reactions. Risk of acute cardiorespiratory failure in susceptible patients.

NEXVIAZYME Warnings/Precautions:

Risk of severe hypersensitivity reactions (including anaphylaxis); discontinue immediately if occurs. Have appropriate medical support and resuscitative equipment readily available. Monitor for severe infusion-associated reactions (IARs); consider immediate discontinuation if occurs. Withhold temporarily or slow the infusion rate if mild or moderate hypersensitivity reactions or moderate IARs occur. Increased risk of severe complications from IARs in those with advanced Pompe disease. Fluid volume overload, acute underlying respiratory illness, or compromised cardiac/respiratory function (with fluid restriction): risk of acute cardiorespiratory failure; monitor vitals frequently. Pregnancy. Nursing mothers.

NEXVIAZYME Classification:

Hydrolytic lysosomal glycogen-specific enzyme.

Adverse Reactions:

Headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, urticaria; hypersensitivity reactions, IARs.

Generic Drug Availability:


How Supplied:

Single-dose vial—1