vestronidase alfa-vjbk (Rx)

Brand and Other Names:Mepsevii
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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

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

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

and vestronidase alfa-vjbk

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    Interactions Found

    Contraindicated

      Serious - Use Alternative

        Significant - Monitor Closely

          Minor

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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 to manage 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 GUS that results in GAG accumulation in cells throughout the body leading to multisystem tissue and organ damage

            Recombinant human lysosomal beta glucuronidase (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 in-line, low-protein binding 0.2 micron filter

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

            Due 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 three 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 following storage 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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            Formulary

            FormularyPatient Discounts

            Adding plans allows you to compare formulary status to other drugs in the same class.

            To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.

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            • View the formulary and any restrictions for each plan.
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            • Compare formulary status to other drugs in the same class.
            • Access your plan list on any device – mobile or desktop.

            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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