Version May 2024
Anaphylaxis has occurred with vestronidase alfa administration, as early as the first dose, therefore appropriate medical support should be readily available when administered. Closely observe patients during and for 60 minutes after vestronidase alfa infusion. Immediately discontinue the infusion if the patient experiences anaphylaxis.
Mucopolysaccharidosis VII (MPS VII, Sly syndrome): Note: Premedicate with nonsedating antihistamines with or without antipyretics 30 to 60 minutes prior to the start of the infusion.
Infants, Children, and Adolescents: IV: 4 mg/kg/dose every 2 weeks
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); however, adjustment based on renal function is unlikely to be necessary as vestronidase alfa is not expected to be renally cleared.
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); however, adjustment based on hepatic function is unlikely to be necessary as vestronidase alfa is degraded by proteolytic enzymes that are not restricted to hepatic tissue.
(For additional information see "Vestronidase alfa: Drug information")
Note: Premedicate with nonsedating antihistamines with/without antipyretics 30 to 60 minutes prior to the start of the infusion. Administer under supervision of a health care provider with the ability to manage anaphylaxis.
Mucopolysaccharidosis VII (Sly syndrome): IV: 4 mg/kg every 2 weeks.
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); however, adjustment based on renal function is unlikely to be necessary as vestronidase alfa is not expected to be renally cleared.
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied); however, adjustment based on hepatic function is unlikely to be necessary as vestronidase alfa is degraded by proteolytic enzymes that are not restricted to hepatic tissue.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Dermatologic: Skin rash
Gastrointestinal: Diarrhea
Hypersensitivity: Anaphylaxis
Immunologic: Antibody development (may be neutralizing; antibody development did not appear to affect reduction in urinary glycosaminoglycans)
1% to 10%:
Cardiovascular: Peripheral edema
Dermatologic: Pruritus
Local: Swelling at injection site
Frequency not defined: Miscellaneous: Febrile seizures
There are no contraindications listed in the manufacturer's labeling.
Concerns related to adverse effects:
• Extravasation: Infusion site extravasation has been reported; infusion site swelling has also occurred.
• Hypersensitivity/anaphylactoid reactions: Hypersensitivity, including anaphylaxis, has occurred with administration; manifestations included respiratory distress, cyanosis, decreased oxygen saturation, and hypotension. Consider the risks and benefits of re-administering vestronidase alfa following anaphylaxis; in clinical trials, patients experiencing anaphylaxis were able to tolerate subsequent infusions without recurrence of anaphylaxis.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intravenous [preservative free]:
Mepsevii: Vestronidase alfa-vjbk 10 mg/5 mL (5 mL)
No
Solution (Mepsevii Intravenous)
10 mg/5 mL (per mL): $632.88
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
IV: Administer IV over ~4 hours using an infusion set with an in-line low protein-binding 0.2-micron filter. Infuse the first 2.5% of total volume over the first hour (making sure to account for any dead space in the IV line); after the first hour, increase the infusion rate (as tolerated) to complete the infusion over the next 3 hours. Do not flush the line to avoid a rapid bolus of infused enzyme. Due to the low infusion rate in some patients, additional NS may be run through a separate line (piggyback or Y-site) to maintain sufficient IV flow to prevent clotting or line blockage. Do not infuse other products in same IV line. Discontinue the infusion immediately if severe reaction occurs, including anaphylaxis. Observe patient during, and for 60 minutes following, infusion.
May be an irritant; ensure proper needle or catheter placement prior to and during IV infusion. Avoid extravasation.
IV: Infuse IV over ~4 hours; infuse the first 2.5% of total volume over the first hour (accounting for any dead space in IV line); after the first hour, increase infusion rate (as tolerated) to complete infusion over the next 3 hours. Administer using an infusion set with an in-line low protein-binding 0.2 micron filter. Do not flush the line to avoid a rapid bolus of infused enzyme; due to the low infusion rate, additional NS may be added through a separate line (piggyback or Y tube) to maintain sufficient IV flow to prevent clotting or line blockage. Do not infuse other products in same IV line. Discontinue the infusion immediately if severe reaction occurs, including anaphylaxis. Observe patient during and for at least 60 minutes following infusion.
May be an irritant; ensure proper needle or catheter placement prior to and during IV infusion. Avoid extravasation.
Store under refrigeration at 2°C to 8°C (36°F to 46°F). Do not freeze or shake. Protect from light. Following dilution use immediately; if this is not possible, the diluted solution may be stored up to 36 hours under refrigeration followed by up to 6 hours at room temperature ≤25°C (77°F). Infusion should be completed within 42 hours from time of dilution.
Treatment of mucopolysaccharidosis VII (MPS VII, Sly syndrome) (FDA approved in pediatric patients [age not specified] and adults)
None known.
There are no known significant interactions.
Adverse events were not observed in animal reproduction studies.
Monitor for infusion/hypersensitivity reactions (during and for 60 minutes following completion of infusion). Monitor infusion site.
Vestronidase alfa is a recombinant human beta-glucuronidase (GUS), which provides exogenous GUS enzyme for uptake into cellular lysosomes. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow binding of the enzyme to cell surface receptors, leading to cellular uptake of the enzyme, targeting to lysosomes and subsequent catabolism of accumulated glycosaminoglycans (GAGs) in affected tissues.
Distribution: Vd,ss: 251 ± 140 mL/kg (range: 97 to 598 mL/kg).
Metabolism: Proteolytic degradation into small peptides and amino acids.
Half-life elimination: 2.33 ± 0.75 hours (range: 0.86 to 3.03 hours).
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