vestronidase alfa-vjbk (Rx)

Brand and Other Names:Mepsevii

Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injection, IV solution

  • 2mg/mL (10mg/5mL single-dose vial)

Mucopolysaccharidosis VII

Indicated for mucopolysaccharidosis VII (MPS VII, Sly syndrome) in adult and pediatric patients

Premedication 30-60 minutes before IV infusion with a nonsedating antihistamine with or without an antipyretic medication is recommended

See also Administration for precise infusion rate instructions

Dosing Considerations

Observe patients closely during the infusion and following the infusion for a minimum of 60 minutes for the development of anaphylaxis

Limitation of use

  • The effect on CNS manifestations of MPS VII has not been determined

Dosage Forms & Strengths

injection, IV solution

  • 2mg/mL (10mg/5mL single-dose vial)

Mucopolysaccharidosis VII

Indicated for mucopolysaccharidosis VII (MPS VII, Sly syndrome) in adult and pediatric patients

Premedicate 30-60 minutes before IV infusion with a nonsedating antihistamine with or without an antipyretic medication is recommended

See also Administration for precise infusion rate instructions

Dosing Considerations

Observe patients closely during the infusion and following the infusion for a minimum of 60 minutes for the development of anaphylaxis

Limitation of use

  • The effect on CNS manifestations of MPS VII has not been determined
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Interactions

Interaction Checker

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                    Adverse Effects

                    <1%

                    Extravasation (0.5%)

                    Diarrhea (0.4%)

                    Rash (0.4%)

                    Anaphylaxis (0.4%)

                    Infusion site swelling (0.1%)

                    Peripheral swelling (0.1%)

                    Pruritus (0.1%)

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                    Warnings

                    Black Box Warnings

                    Anaphylaxis has occurred as early as the first dose; therefore, appropriate medical support should be readily available during IV infusion

                    Closely observe patient during and for 60 minutes after infusion

                    Immediately discontinue treatment if anaphylaxis occurs

                    Contraindications

                    None

                    Cautions

                    Anaphylaxis was reported in the clinical program; reactions occurred during infusion and were observed as early as the first dose; manifestations included respiratory distress, cyanosis, decreased oxygen saturation, and hypotension; administer under supervision of a healthcare professional with experience managing anaphylaxis (see Dosing Considerations and Black Box Warnings)

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                    Pregnancy & Lactation

                    Pregnancy

                    No data are available regarding use in pregnant women

                    When administered to pregnant rats and rabbits during organogenesis, vestronidase alfa showed no maternal toxicity or adverse developmental outcomes at doses causing maternal serum exposures (AUC) up to 1.6 and 10 times, respectively for rats and rabbits, the exposure at the recommended human dose

                    Lactation

                    Unknown if present in either human or animal milk; the effects on the breastfed infant, or the effects on milk production

                    Pregnancy Categories

                    A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

                    B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

                    C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

                    D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

                    X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

                    NA: Information not available.

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                    Pharmacology

                    Mechanism of Action

                    Mucopolysaccharidosis VII (MPS VII or Sly syndrome) is a lysosomal disorder characterized by the deficiency of beta glucuronidase (GUS) that results in GAG accumulation in cells throughout the body, leading to multisystem tissue and organ damage

                    Recombinant human lysosomal GUS is intended to provide exogenous GUS enzyme for uptake into cellular lysosome; 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 GAGs in affected tissues

                    Absorption

                    Peak plasma concentration: 20 mcg/mL (range: 6.6-34.9 mcg/mL)

                    AUC: 3440 mcg·min/mL (range: 1130- 5820 mcg·min/mL)

                    Distribution

                    Vd: 260 mL/kg (range: 97-598 mL/kg)

                    Metabolism

                    Vestronidase alfa is a recombinant human enzyme and is therefore eliminated by proteolytic degradation into small peptides and amino acids

                    Elimination

                    Clearance: 1.3 mL/min/kg (range: 0.6 to 3.3 mL/min/kg)

                    Half-life: 155 minutes (range: 51-213 minutes)

                    No excretion studies have been conducted in humans; not expected to be eliminated through urine or feces

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                    Administration

                    IV Compatibility

                    0.9% NaCl

                    Compatibility with other products has not been evaluated

                    IV Preparation

                    Determine total dose based on patient’s actual body weight (refer to prescribing information)

                    Remove required number of vials from refrigerator and allow them to reach room temperature; do not heat, microwave, or shake vials

                    Visually inspect solution for particulate matter and discoloration; slight flocculation (thin translucent fibers) may appear; solution should appear colorless to slightly yellow

                    Slowly withdraw the calculated dose using a large needle (eg, 18 gauge) to minimize bubbles in the solution

                    Add 0.9% NaCl equal to the drug volume to infusion bag; final solution should be a 1:1 solution of vestronidase alfa to 0.9% NaCl

                    Diluted solutions may be refrigerated (2-8°C [36-46°F]) for up to 36 hr and may be stored at room temperature (25°C [77°F]) for up to 6 hr

                    IV administration

                    Use an infusion set with an inline, low-protein binding 0.2 micron filter

                    Do not flush the line containing vestronidase alfa to avoid a rapid bolus of infused enzyme

                    Owing to the low infusion rate, additional saline may be added through a separate line (piggyback or Y tube) to maintain sufficient intravenous flow to prevent clotting or line blockage

                    Do not infuse with other products in the infusion tubing

                    Use immediately after dilution and complete the infusion within 42 hr from the time of dilution

                    Discard any unused product

                    Rate of infusion

                    • First hour: Infuse 2.5% of the total volume; account for any dead space in the lines to ensure 2.5% of the total infusion volume is delivered
                    • Subsequent 3 hours: Infuse remaining volume (refer to prescribing information)

                    Storage

                    Unused vials: Store under refrigeration at 2-8°C (36-46°F)

                    Diluted solutions: Store in refrigerator at 2-8°C (36-46°F) for up to 36 hr and may be stored at room temperature at 25°C (77°F) for up to 6 hr

                    Do no freeze or shake

                    Protect from light

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                    Images

                    No images available for this drug.
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                    Patient Handout

                    A Patient Handout is not currently available for this monograph.
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                    Formulary

                    FormularyPatient Discounts

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                    • Compare formulary status to other drugs in the same class.
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                    The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

                    Tier Description
                    1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
                    2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
                    3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
                    4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                    5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                    6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                    NC NOT COVERED – Drugs that are not covered by the plan.
                    Code Definition
                    PA Prior Authorization
                    Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
                    QL Quantity Limits
                    Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
                    ST Step Therapy
                    Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
                    OR Other Restrictions
                    Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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                    Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.