Vestronidase Alfa-vjbk
Class: Enzymes
Chemical Name: β-Glucuronidase (synthetic human), homotetramer
Molecular Formula: C3308H4996N874O940S16
CAS Number: 1638194-78-1
Brands: Mepsevii
Warning
Risk of life-threatening anaphylactic reactions as early as first dose. Appropriate medical support should be readily available.
Observe patients closely during and for ≥60 minutes after infusion. Immediately stop infusion if patient experiences anaphylaxis. (See Hypersensitivity Reactions under Cautions.)
Introduction
Biosynthetic (recombinant DNA origin) form of human β-glucuronidase, a lysosomal enzyme that catabolizes glycosaminoglycans (GAGs).
Uses for Vestronidase Alfa-vjbk
Mucopolysaccharidosis VII
Replacement therapy in patients with mucopolysaccharidosis VII (MPS VII, Sly syndrome); designated an orphan drug by FDA for use in this condition.
May improve motor function (e.g., increase walking distance) in some patients; however, effect on CNS manifestations not determined.
Vestronidase Alfa-vjbk Dosage and Administration
General
Prepare and administer under supervision of a clinician experienced in managing anaphylaxis.
To minimize risk of hypersensitivity reactions, administer a nonsedating antihistamine (with or without an antipyretic) 30–60 minutes prior to each infusion.
Administration
IV Administration
For solution and drug compatibility information, see Compatibility under Stability.
Administer by IV infusion.
Administer using an inline, low-protein-binding 0.2-µm filter.
Do not flush IV line to avoid rapid bolus; due to the slow rate of infusion, may administer additional 0.9% sodium chloride injection through a separate line to maintain sufficient IV flow and prevent clotting or blockage of the IV tubing.
Do not infuse through the same IV line with other drugs.
Vials contain no preservatives and are intended for single use only.
Vestronidase alfa-vjbk injection is a colorless to slightly yellow solution. Because the drug is a protein solution, slight flocculation (thin translucent fibers) may occur. Discard solution if discoloration or particulate matter is present.
Dilution
Must dilute commercially available injection concentrate prior to administration.
Determine number of vials needed based on patient's actual weight and recommended dose of 4 mg/kg. Remove required number of vials from refrigerator and allow them to reach room temperature. Do not heat, microwave, or shake the vials.
Using a sufficiently large needle (e.g., 18 gauge), slowly withdraw calculated volume of vestronidase alfa-vjbk from the appropriate number of vials. Transfer drug to an empty infusion bag. Dilute with an equal volume of 0.9% sodium chloride injection (1:1 ratio). More than a 1:1 dilution ratio may be used if the patient can tolerate additional infusion volume, taking into consideration cardiac function and flui...
