Parenteral Manual


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Alternate Name(s): 
Enzyme, Glucocerebrosidase
Original Date: 
August 2007
Revised Date: 
December 2009
  • Long-term enzyme replacement therapy for pediatric and adult patients with confirmed diagnosis of Type1 Gaucher disease resulting in one or more of the following conditions:

1.  Anemia

2.  Thrombocytopenia

3.  Bone disease

4.  Hepatomegaly/splenomegaly

Reconstitution and Stability: 
  • Refrigerate vials. Warm vials to room temperature before reconstitution.
  • Reconstituted product stable 12 hours at room temperature or refrigerated.
  • Diluted in normal saline,  stable 24 hours refrigerated.
  • 200 units Vial:  Reconstitute with 5.1 mL sterile water for injection for a 40 units/mL solution.
  • 400 units Vial:  Reconstitute with 10.2 mL sterile water for injection for a 40 units/mL solution.
  • Do not shake vial. Gently swirl each vial to mix solution.
  • Do not use any vials exhibiting opaque particles or discoloration.

- Solutions compatible:  0.9% NaCl


(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)

IV Direct No
IV Intermittent Infusion


Usual dilution: to a total volume of 100-200 mL with normal saline

Infusion time: 1-2 hours (or 0.5-1 unit/kg/minute)

IV Continuous Infusion No






(For neonatal dosages, refer to Neonatal IV Drug Manual.)

  • Initial dose: Range 2.5 units/kg 3x/week to 60 units/kg once every 2 weeks IV
  • Initial dose and frequency depend on disease severity.
  • Dosage adjustments based on individual patient response/ therapeutic goals.
  • Dosage administered in individual infusions may be adjusted to utilize full vials as long as the monthly administered dosage remains substantially unaltered.  Low toxicity and extended time course of response allows for small dosage adjustments to be made to avoid discarding partially used vial.
  • Experience with doses up to 240 units/kg every 2 weeks reported with no obvious toxicity.
Potential hazards of parenteral administration: 
  • Adverse effects associated with administration and increase in frequency; discomfort, pruritus, burning, swelling or sterile abscess at site of venipuncture
  • Hypersensitivity; pruritus, flushing, urticaria, angioedema, chest discomfort, dyspnea, coughing, cyanosis, hypotension.
  • Anaphylaxis
  • Other; nausea, abdominal pain, vomiting, diarrhea, rash, fatigue, headache, fever, dizziness, chills, backache, tachycardia, transient peripheral edema
  • Slight flocculation (translucent fibers) occurs occasionally after dilution. 
  • Agitation of the reconstituted or diluted bag may increase the chance of precipitation. 
  • Filter diluted solution  through an in-line low protein binding 0.2 or 0.22 micron  filter 
  • Infusion should be discontinued if formation of precipitate is observed.

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