Enzyme replacement therapy for type 1 Gaucher disease
- Vials should be stored in a refrigerator (2 to 8 degrees C)
- Vials should be protected from light
- Reconstitute each 400 unit vial with 4.3 mL of sterile water for injection for a 100 unit/mL solution
- Do not shake vial. Gently swirl each vial to mix solution
- Stability: Reconstituted vials must be further diluted within 6 hours of puncture
- Stability: Further diluted solution of velaglucerase in 0.9% sodium chloride can be stored in the refrigerator for up to 24 hours.
Available formulation: 400 unit vial
Compatible only with 0.9% sodium chloride.
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
|IV intermittent infusion||
Yes DO NOT SHAKE VIAL OR BAG. Slight flocculation (white irregularly shaped particles) may occur
Usual dilution: Further dilute velaglucerase using a 100 mL minibag
Infusion time: 1 hour
|IV continuous infusion||No|
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
- Pediatric: 60 units/kg once every 2 weeks IV
- Dosage adjustments can be made on an individual basis based on achievement and maintenance of therapeutic goals
- Studies have evaluated doses ranging from 15 units to 60 units/kg every 2 weeks IV
- Infusion related reactions: headache, dizziness, hypotension, hypertension, nausea, fatigue and increased body temperature
- Rare infusion-related reactions symptoms: chest discomfort, dyspnea, pruritis
Infusion-related reactions can be managed by slowing the infusion rate or stopping and resuming with an increased infusion time, treatment with medications such as antihistamines, antipyretics etc. Please call the physician if a patient experiences any symptoms of an infusion-related reaction.
Velaglucerase should be administered through a 0.2 micron filter
Slight flocculation (white irregularly shaped particles) may occur
Agitation of the reconstituted solution or diluted bag may increase the chance of precipitation.