"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).
DOSAGE AND ADMINISTRATION
Recommended Starting Dosage In Patients Na´ve To Enzyme Replacement Therapy
VPRIV should be administered under the supervision of a healthcare professional. The recommended starting VPRIV dosage in na´ve adults and na´ve 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.
Switching From Imiglucerase To VPRIV
Adults 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.
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:
- Determine the number of vials to be reconstituted based on the individual patient's weight and the prescribed dose.
- Inject 4.3 mL of Sterile Water for Injection, USP into a vial containing VPRIV lyophilized powder.
- Mix gently. DO NOT SHAKE. The reconstituted VPRIV solution will have a 100 Units/mL concentration (400 Units VPRIV in 4 mL of solution).
- If additional vials are needed, repeat steps (b) and (c)
- 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.
- 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 intravenous 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.
- 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 2 °C to 8°C (36 °F to 46°F). Do not freeze and protect from light. Complete the infusion within 24 hours of reconstitution of vials.
- Vials are for single use only. Discard any unused solution.
Important Administration Instructions
Administer the diluted VPRIV solution through an in-line low protein-binding 0.2μm 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.
Premedication To Reduce Risk Of Subsequent Hypersensitivity Reactions
Consider pre-treatment with antihistamines and/or corticosteroids in patients who exhibited symptoms of hypersensitivity associated with prior VPRIV infusions. Appropriate medical support should be readily available when VPRIV is administered [see WARNINGS AND PRECAUTIONS].
Dosage Forms And Strengths
VPRIV for injection: sterile, white to off-white, lyophilized powder (400 Units in single-use vials) for reconstitution with Sterile Water for Injection, USP, to yield a final concentration of 100 Units/mL.
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.
Store VPRIV at 2 °C to 8°C (36°F 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. Revised: April 2015This monograph has been modified to include the generic and brand name in many instances.
Last reviewed on RxList: 5/21/2015
Additional VPRIV Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
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