Dosing and uses of VPRIV (velaglucerase alfa)
Adult dosage forms and strengths
lyophilized powder for injection
- 400 unit/vial
Gaucher Disease Type I
60 Unit/kg IV q2week; infuse over 1 hr
Administration
Adjust dose according to individualize patient requirements
Clinical trials have evaluated dosage range between 15-60 mg q2week
Switching from imiglucerase: Begin treatment with velaglucerase with same dose of imiglucerase the patient was taking
May pretreat with corticosteroids and/or antihistamines to decrease risk of infusion-related reactions
Pediatric dosage forms and strengths
lyophilized powder for injection
- 400 unit/vial
Gaucher Disease Type I
<4 years: Safety/efficacy not established
≥4 years: As adults; 60 Unit/kg IV q2week; infuse over 1 hr
Administration
Adjust dose according to individualize patient requirements
Clinical trials have evaluated dosage range between 15-60 mg q2week
Switching from imiglucerase: Begin treatment with velaglucerase with same dose of imiglucerase the patient was taking
May pretreat with corticosteroids and/or antihistamines to decrease risk of infusion-related reactions
VPRIV (velaglucerase alfa) adverse (side) effects
>10%
Fatigue (13%)
Dizziness (8-22%)
Abdominal pain (15-19%)
Headache (30-35%)
Upper respiratory tract infections (30-32%)
Prolonged aPPT (5-11%)
1-10%
Flushing (>2%)
Tachycardia (>2%)
Hypertension/hypotension (>2%)
Urticaria (>2%)
Rash (>2%)
Postmarketing reports
Chest discomfort
Dyspnea
Pruritus
Warnings
Contraindications
None listed in the manufacturer's labeL
Cautions
The most common adverse effect are infusion-related reactions; body temperature may increase when hypersensitivity reactions occur
Consider slowing infusion rate, treatment with antihistamines, antipyretics and/or corticosteroids, and/or stopping treatment if hypersensitivity reaction occurs during infusion; consider pre-treatment with antihistamines and/or corticosteroids in patients with prior reactions
Most reactions are mild and occur within the first 6 months; pretreatment or symptomatic treatment with corticosteroids, antihistamines, or antipyretics may help manage the reactions
Ensure that personnel administering product are adequately trained in cardiopulmonary resuscitative measures, and have ready access to emergency medical services
Pregnancy and lactation
Pregnancy category: B
Lactation: Unknown whether distributed in breast milk; use caution
Pregnancy categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.
C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.
D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.
X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.
NA: Information not available.
Pharmacology of VPRIV (velaglucerase alfa)
Mechanism of action
Hydrolytic lysosomal glucocerebroside-specific enzyme plays a role in the hydrolysis of glucocerebroside to glucose and ceramide thereby preventing the accumulation of glucocerebroside in macrophages, which is responsible for the signs and symptoms of Gauche'r disease
Pharmacokinetics
Half-Life: 11-12 min
Vd: 82-108 mL/kg
Clearance: 6.72-7.56 mL/min/kg
Administration
IV Compatibilities
Solution: 0.9% NaCL
Y-site: Do no infuse with other drugs in same infusion tubing
IV Preparation
Vial Reconstitution
- Reconstitute vial with sterile water for injection
- 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 above
Upon reconstitution, mix vial gently; DO NOT SHAKe
Inspect reconstituted vial; should be clear to slightly opalescent and colorless
Do not use if solution is discolored or foreign particulate matter present
IV infusion: Withdraw calculated volume and further dilute in 100 mL of 0.9% NaCl; mix gently and DO NOT SHAKe
IV Administration
Diluted solution should be administered using an in-line low protein-binding 0.2 micron filter
Administer IV injection via volumetric infusion pump over 1 hr
Storage
Contains no preservatives
Once reconstituted, use immediately
If immediate use not possible, reconstituted or diluted product may be stored refrigerated for up to 24 hr; do not freeze and protect from light
Store unreconstituted vials refrigerated (2-8 degrees C; 36-46 degrees F), do not freeze and protected from light
