"The U.S. Food and Drug Administration yesterday approved Ruconest, the first recombinant C1-Esterase Inhibitor product for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).
VPRIV (velaglucerase alfa for injection) is a hydrolytic lysosomal glucocerebroside-specific enzyme indicated for long-term enzyme replacement therapy (ERT) for pediatric and adult patients with type 1 Gaucher disease.
DOSAGE AND ADMINISTRATION
The recommended dose is 60 Units/kg administered every other week as a 60-minute intravenous infusion.
Patients currently being treated with imiglucerase for type 1 Gaucher disease may be switched to VPRIV. Patients previously treated on a stable dose of imiglucerase are recommended to begin treatment with VPRIV at that same dose when they switch from imiglucerase to VPRIV.
Dosage adjustments can be made based on achievement and maintenance of each patient's therapeutic goals. Clinical studies have evaluated doses ranging from 15 Units/kg to 60 Units/kg every other week.
VPRIV should be administered under the supervision of a healthcare professional.
Preparation and Administration Instructions
VPRIV is a lyophilized powder, which requires reconstitution and dilution, and is intended for intravenous infusion using an in-line, low protein-binding 0.2μm filter. VPRIV contains no preservatives. The vials are single-use only. Discard any unused solution.
Preparation and Administration
VPRIV should be prepared using aseptic technique as follows:
Determine the number of vials to be reconstituted based on the individual patient's weight and the prescribed dose. Refer to Table 1 for reconstitution instructions:
Table 1: VPRIV Reconstitution Instructions
|Volume of Sterile Water for Injection, USP, for reconstitution||4.3 mL|
|Concentration after reconstitution||100 Units/mL|
|Withdrawal volume||4 mL|
Upon reconstitution, mix vials gently. DO NOT SHAKE. Prior to further dilution, visually inspect the 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. 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 solution suitable for IV administration. Then dilute the calculated dose of VPRIV Directly into the sodium chloride solution. 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.
VPRIV should be administered over 60 minutes. VPRIV should not be infused with other products in the same infusion tubing as the compatibility in solution with other products has not been evaluated. Administer the diluted solution through an in-line low protein-binding 0.2 μm filter.
As VPRIV contains no preservatives, once reconstituted the product should be used immediately. If immediate use is not possible, the reconstituted or diluted product may be stored for up to 24 hours at 2 to 8°C (36 to 46°F). Do not freeze. Protect from light. The infusion should be completed within 24 hours of reconstitution of vials.
Dosage Forms And Strengths
VPRIV is a sterile, white to off-white, lyophilized powder for reconstitution with Sterile Water for Injection, USP, to yield a final concentration of 100 Units/mL.
VPRIV is available as 400 Units single-use vials.
Storage And Handling
VPRIV is a sterile, preservative free, lyophilized powder requiring reconstitution and further dilution prior to use. It is supplied in individually packaged glass vials, which are closed with a butyl rubber stopper with a fluoro-resin coating and are sealed with an aluminum overseal with a flip-off plastic cap. The vials are intended for single use only. VPRIV is available as: 400 Units/vial NDC 54092-701-04.
VPRIV should be stored in a refrigerator at 2 to 8°C (36 to 46°F). Do not use VPRIV after the expiration date on the vial. Do not freeze.
Protect vial from light.
VPRIV is manufactured by: Shire Human Genetic Therapies, Inc. 300 Shire Way Lexington, MA 02421. Revisd: October 2013.
Last reviewed on RxList: 10/21/2013
This monograph has been modified to include the generic and brand name in many instances.
Additional VPRIV Information
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