Dosage & Administration
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Vpriv Prescribing Information
The management of hypersensitivity reactions should be based on the severity of the reaction, e.g., slowing the infusion rate, treatment with medications such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue VPRIV and immediately initiate appropriate medical treatment, including use of epinephrine.
| Boxed Warning | 7/2024 |
Dosage and Administration (2.1 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 (5.1)] .Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions (5.1)] . | 7/2024 |
Warnings and Precautions (Life-threatening hypersensitivity reactions, including anaphylaxis, have occurred in patients treated with enzyme replacement therapies, including VPRIV. VPRIV-treated patients have had these reactions occur in clinical studies and postmarketing experience [see Adverse Reactions (6.1)and Clinical studies (14)] . Hypersensitivity reactions were the most commonly observed adverse reactions in patients treated with VPRIV in clinical studies. Patients were not routinely pre-medicated prior to infusion of VPRIV during clinical studies. The most commonly observed symptoms of hypersensitivity reactions were: headache, dizziness, hypotension, hypertension, nausea, fatigue/asthenia, and pyrexia/body temperature increased. Generally the reactions were mild and, in treatment-naïve patients, onset occurred mostly during the first 6 months of treatment and tended to occur less frequently with time. Additional hypersensitivity reactions of chest discomfort, dyspnea, pruritus and vomiting have been reported in post-marketing experience.Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Administration of VPRIV should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. The management of hypersensitivity reactions should be based on the severity of the reaction, e.g., slowing the infusion rate, treatment with medications such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue VPRIV and immediately initiate appropriate medical treatment, including use of epinephrine. In cases where patients have exhibited symptoms of hypersensitivity to velaglucerase alfa or excipients in the drug product or to other enzyme replacement therapy, pre-treatment with antihistamines and/or corticosteroids may prevent subsequent reactions. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur. | 7/2024 |
VPRIV is indicated for long-term enzyme replacement therapy (ERT) for patients with type 1 Gaucher disease.
- Administration of VPRIV should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis ()2.1 Recommendations Prior to VPRIV treatmentAdministration of VPRIV should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis[see Warnings and Precautions (5.1)].Initiate VPRIV in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment[see Warnings and Precautions (5.1)].
- Recommended Starting Dose in Adults and Pediatric Patients 4 Years of Age or Older:
- Patients Naïve to Enzyme Replacement Therapy: 60 Units/kg ()2.2 Recommended Starting Dosage in Patients Naïve to Enzyme Replacement Therapy
The recommended starting VPRIV dosage in naïve adults and naïve pediatric patients 4 years of age and older is 60 Units/kg administered every other week as a 60-minute intravenous infusion. The dosage can be adjusted based on achievement and maintenance of each patient's therapeutic goals.
- Patients being treated with stable imiglucerase dosages for Gaucher disease: Can switch to VPRIV at previous imiglucerase dose two weeks after last imiglucerase dose ()
2.3 Switching from Imiglucerase to VPRIVAdults and pediatric patients 4 years of age and older currently being treated on a stable dosage of imiglucerase for type 1 Gaucher disease may be switched to VPRIV by starting treatment with VPRIV at the previous imiglucerase dosage two weeks after the last imiglucerase dose. VPRIV should be administered under the supervision of a healthcare professional as a 60-minute intravenous infusion. The dosage can be adjusted based on achievement and maintenance of each patient's therapeutic goals.
- Patients Naïve to Enzyme Replacement Therapy: 60 Units/kg (
- Determine number of vials to be reconstituted based on patient's actual weight and prescribed dose ()
2.4 Reconstitution of the VPRIV Lyophilized PowderVPRIV 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.
- Supplied VPRIV lyophilized powder must be reconstituted with Sterile Water for Injection ()
2.4 Reconstitution of the VPRIV Lyophilized PowderVPRIV 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.
- Reconstituted VPRIV solution must be diluted in 100 mL of 0.9% Sodium Chloride Injection prior to intravenous infusion ()
2.4 Reconstitution of the VPRIV Lyophilized PowderVPRIV 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.
- Administer the diluted VPRIV solution through an in-line low protein-binding 0.2 or 0.22 µm filter ()
2.5 Important Administration InstructionsAdminister 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.
For injection: 400 units of a sterile, white to off-white, lyophilized powder in single-dose vials for reconstitution and dilution.
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
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/m2, about 1.8 times the recommended human dose of 60 Units/kg or 1.5 mg/kg or 55.5 mg/m2based on the body surface area) were administered two to three times weekly. In pregnant rabbits intravenous doses up to 20 mg/kg (240 mg/m2, 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, velaglucerase alfa was administered intravenously to pregnant rats twice weekly from gestation day 6 to lactation day 19. There was no evidence of any adverse effect on pre- and postnatal development at doses up to 17 mg/kg/day (102 mg/m2, about 1.8 times the recommended human dose of 60 Units/kg based on the body surface area).
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.