Generic drug: velaglucerase alfa
Brand name: VPRIV
What is VPRIV (velaglucerase alfa), and how does it work?
VPRIV (velaglucerase alfa) is an enzyme produced in a fibroblast cell line that catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide in the lysosome of macrophage cell types. VPRIV is indicated for long-term enzyme replacement therapy for pediatric and adult patients with the rare genetic disorder, type 1 Gaucher disease.
What are the side effects of VPRIV?
The most common side effects of VPRIV are allergic reactions. Other side effects of VPRIV include:
- headache,
- dizziness,
- stomach pain,
- back pain,
- joint pain,
- knee pain,
- nausea,
- fatigue,
- weakness,
- dizziness,
- tiredness,
- fever,
- prolonged blood clotting time, and
- cold symptoms such as stuffy nose, sneezing, and sore throat.
What is the dosage for VPRIV?
Recommended Starting Dosage In Patients Naive To Enzyme Replacement Therapy
- VPRIV should be administered under the supervision of a healthcare professional.
- The recommended starting VPRIV dosage in naive adults and naive 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 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.
- 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 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.
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
velaglucerase alfa product infusions. - Appropriate medical support should be readily available when VPRIV is
administered.
What drugs interact with VPRIV?
No information provided
Is VPRIV safe to use while pregnant or breastfeeding?
- 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.
- There are no data available on the presence of velaglucerase alfa in human milk.
- The reported cases from the pharmacovigilance database are insufficient to determine any effects on the breastfed infant or on milk production.
- Endogenous beta-glucocerebrosidase is present in human milk. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for VPRIV and any potential adverse effects on the breastfed child from VPRIV or from the underlying maternal condition.