Velaglucerase alfa
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From MedlinePlus, U.S. National Library of Medicine
Boxed warning
WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement and after extended duration of therapy. Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue VPRIV and immediately initiate appropriate medical treatment, including use of epinephrine.
Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur [see Warnings and Precautions (5.1) ] . WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS See full prescribing information for complete boxed warning. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. ( 5.1 ) Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment.
( 5.1 ) If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue VPRIV and immediately initiate appropriate medical treatment, including use of epinephrine. ( 5.1 )
Hydrolytic Lysosomal Glucocerebroside-specific Enzyme
Dosing in kidney impairment
This label has no separate kidney-impairment dosing section. Check warnings and clinical pharmacology in the full label.
Preparation and administration
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Recommendations Prior to VPRIV treatment Administration of VPRIV should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis [see Warnings and Precautions ] . Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions] .
Reconstitution of the VPRIV Lyophilized Powder VPRIV is a lyophilized powder, which requires reconstitution and dilution, using sterile technique, prior to intravenous infusion. VPRIV should be prepared as follows: (a) Determine the number of vials to be reconstituted based on the individual patient's weight and the prescribed dose. (b) Inject 4.3 mL of Sterile Water for Injection, USP into a vial containing VPRIV lyophilized powder. (c) Mix gently.
DO NOT SHAKE. The reconstituted VPRIV solution will have a 100 Units/mL concentration (400 Units VPRIV in 4 mL of solution). (d) If additional vials are needed, repeat steps (b) and (c). (e) Visually inspect the reconstituted VPRIV solution in the vials. The solution should be clear to slightly opalescent and colorless.
Do not use if the solution is discolored or if foreign particulate matter is present. (f) With a single syringe, withdraw the calculated dose of drug from the appropriate number of vials. Using a separate syringe, withdraw air from a bag of 100 mL of 0.9% Sodium Chloride Injection suitable for intravenous administration.
Then dilute the calculated dose of VPRIV directly into the 0.9% Sodium Chloride Injection. Mix gently. DO NOT SHAKE. Slight flocculation (described as white irregular shaped particles) may occasionally occur. Diluted solution with slight flocculation is acceptable for administration. (g) Because VPRIV contains no preservatives, use the reconstituted VPRIV solution and the diluted VPRIV solution immediately.
If immediate use is not possible, the reconstituted VPRIV solution or the diluted VPRIV solution may be stored for up to 24 hours at 2ºC to 8ºC (36ºF to 46ºF). Do not freeze and protect from light. Complete the infusion within 24 hours of reconstitution of vials. (h) Vials are for one-time use and only for one patient. Discard any unused solution.
Important Administration Instructions Administer the diluted VPRIV solution through an in-line low protein-binding 0.2 or 0.22 µm filter over 60 minutes. Do not infuse VPRIV with other products in the same infusion tubing because the compatibility of a VPRIV solution with other products has not been evaluated.
Contraindications
None. None ( 4 )
Pregnancy and lactation
Pregnancy
8.1 Pregnancy Risk Summary Available data on use of velaglucerase alfa in pregnant women includes more than 300 pregnancies reported from the pharmacovigilance database and published observational cohort studies, including the international Gaucher Disease registry. While available data cannot definitively establish or exclude the absence of a velaglucerase alfa associated risk during pregnancy, these data have not identified an association with use of velaglucerase alfa during pregnancy and major birth defects, miscarriage, or adverse maternal or fetal outcomes.
In animal reproduction studies no fetal harm was observed in rats or rabbits when velaglucerase alfa was administered intravenously during organogenesis at doses with exposures up to 1.8 times and 4.3 times, respectively, the recommended human daily dose (see Data ) . The estimated background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of major birth defects, loss, and other adverse outcomes. In the U.S. general population, the estimated major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively.
Clinical Considerations Disease-Associated Maternal and Embryo/Fetal Risk Women with Type 1 Gaucher disease have an increased risk of spontaneous abortion, especially if disease symptoms are not treated and controlled pre-conception and during a pregnancy. Pregnancy may exacerbate existing Type 1 Gaucher disease symptoms or result in new disease manifestations. Type 1 Gaucher disease manifestations may lead to adverse pregnancy outcomes including hepatosplenomegaly which can interfere with the normal growth of a pregnancy, and thrombocytopenia which can lead to excessive bleeding.
Data Animal Data Embryo-fetal development studies with velaglucerase alfa have been performed during the period of organogenesis in pregnant rats (gestation days 7 through 17) and rabbits (gestation days 6 through 18). In pregnant rats intravenous doses up to 17 mg/kg (102 mg/m 2 , about 1.8 times the recommended human dose of 60 Units/kg or 1.5 mg/kg or 55.5 mg/m 2 based on the body surface area) were administered two to three times weekly. In pregnant rabbits intravenous doses up to 20 mg/kg (240 mg/m 2 , about 4.3 times the recommended human dose of 60 Units/kg based on the body surface area) were administered two to three times weekly.
These studies did not reveal any evidence of impaired fertility or harm to the fetus due to velaglucerase alfa. In a pre- and postnatal development study,…
For breastfeeding, also see LactMed (National Library of Medicine), which summarizes milk levels and infant effects.
Supply and cost
Drug shortage status
Recalls (last 24 months)
Therapeutic equivalence (Orange Book)
Price ladder
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