(i-mi-gloo-ser-ase) ,


(trade name)


Therapeutic: replacement enzyme
Pregnancy Category: C


Treatment of symptomatic type 1 Gaucher’s disease.


Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher’s disease.

Therapeutic effects

Improvement in symptoms of Gaucher’s disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly).


Absorption: IV administration results in complete bioavailability.
Distribution: Widely distributed.
Metabolism and Excretion: Excreted mainly by the kidneys.
Half-life: 3.6–10.4 min.

Time/action profile (improvement in symptoms)



Contraindicated in: Hypersensitivity.
Use Cautiously in: Pregnancy or lactation (safety not established).

Adverse Reactions/Side Effects

Central nervous system

  • dizziness
  • headache


  • mild hypotension


  • abdominal discomfort
  • nausea


  • decreased urinary frequency


  • pruritus
  • rash


  • antibody production (most frequent)
  • hypersensitivity reactions
  • fever


Drug-Drug interaction

None significant.


Intravenous (Adults and Children) Range 2.5 units/kg 3 times weekly to 15–60 units/kg q 1–2 wk. Evaluate dosage every 6 mo for possible reduction.


Injection: 200 units/vial

Nursing implications

Nursing assessment

  • Monitor for an improvement in symptoms including hepatomegaly, splenomegaly, anemia, thrombocytopenia, bone demineralization, and increased appetite and energy level periodically throughout therapy. Assess liver and spleen size every 6 mo to determine effectiveness of therapy.
  • Monitor patient for signs of hypersensitivity reactions (pruritus, flushing, urticaria, angioedema, chest pain, dyspnea, hypotension). Pretreatment with antihistamines and decreasing rate of infusion usually allows patient to continue use.
  • Lab Test Considerations: Monitor hemoglobin and platelet count monthly to determine effectiveness of therapy. If hemoglobin is <7 g/dL or platelet count is <50,000, monitor every 2 wk; levels should increase with imiglucerase therapy.
    • Monitor serum acid phosphatase levels every 2 mo; levels should decrease with imiglucerase therapy.
    • Monitor chemistry panel every 6 mo during therapy.

Potential Nursing Diagnoses

Fatigue (Indications)
Risk for injury (Indications)


  • On the day of use, after determining the correct amount of imiglucerase and appropriate number of vials, reconstitute each vial with 5.1 mL of sterile water for injection for a volume of 5.3 mL (40 units/mL). Withdraw 5 mL from each vial and pool with 0.9% NaCl for a final volume of 100–200 mL. Do not use a solution that is discolored or that contains particulate matter.
  • May also be administered undiluted.
  • Small dosage adjustments can be made to avoid discarding partially used bottles, as long as monthly dose remains unaltered.
  • Do not use imiglucerase after the expiration date. Does not contain a preservative. Stable for up to 12 hr at room temperature or if refrigerated.
  • Rate: Administer diluted solution over 1–2 hr or 0.5-1 unit/kg/min.
    • Undiluted solution may be administered at a rate no greater than 1 unit/kg/min.
  • Additive Incompatibility: Information unavailable. Do not admix with other drugs or solutions.

Patient/Family Teaching

  • Inform patient of the purpose of this medication and the importance of treatment at least every 4 wk. Imiglucerase helps control the symptoms but does not cure Gaucher’s disease. Lifelong therapy may be required..
  • Emphasize the importance of follow-up examinations and lab tests.

Evaluation/Desired Outcomes

  • Increasing hemoglobin and platelet counts and decreasing acid phosphatase levels, hepatomegaly, and splenomegaly. In pediatric patients, cachexia and wasting should diminish.


a trademark for an analog of the human enzyme beta-glucocerebrosiderase (imiglucerase) used for long-term therapy of Type 1 Gaucher's disease.
References in periodicals archive ?
biotech's manufacturing problems, which led to shortages of the Cerezyme and Fabrazyme medicines.
31, 2011 /PRNewswire/ -- Shire plc (LSE: SHP, NASDAQ: SHPGY), the global specialty biopharmaceutical company, today announced positive data from several studies designed to evaluate the safety of switching to REPLAGAL (agalsidase alfa) from Fabrazyme (agalsidase beta) and the long-term safety and benefits of switching to VPRIV (velaglucerase alfa) from Cerezyme (imiglucerase).
Genzyme, meanwhile, has been struggling in the wake of manufacturing problems with its key drugs Cerezyme and Fabrazyme, both aimed at genetic conditions.
In the trial, 160 patients with Gaucher disease type 1 who had begun enzyme replacement therapy at least three years prior to randomization and who had reached therapeutic goals were randomized (2:1) to receive either eliglustat tartrate or Cerezyme for one year.
Continuous supply of Cerezyme medicine, according to the client~s needs and according to detailed specifications in the tender documents.
Data from all twenty six adult patients enrolled in the Company's switchover trial of patients switched from Cerezyme to taliglucerase alfa over the nine-month period, were included in the submission.
Genzyme's patent covers novel culture processes that have been critical in enabling the company to produce Cerezyme (imiglucerase for injection) on a large scale, which has ensured that the therapy is available to patients in need throughout the world.
which has a new facility in Northboro, said Wednesday its profit surged in the third quarter on sales of the key revenue driver Cerezyme, marking a recovery from damaging manufacturing problems last year.
Genzyme has long dominated the market for drugs that treat enzyme deficiencies, such as Cerezyme for Gaucher disease and Fabrazyme for Fabry disease.
This affected production of Cerezyme (imiglucerase) for Gaucher disease and Fabrazyme (agalsidase beta) for Fabry disease.
Genzyme General manufactures Cerezyme (imiglucerase for injection), an enzyme replacement therapy for the treatment of Gaucher disease.