(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.
Drug Guide, © 2015 Farlex and Partners
References in periodicals archive ?
Cerezyme for Gaucher disease and Kalydeco for cystic fibrosis costs $300,000 a year (Emanuel, 2015).
That was the case when Shire launched Vpriv in the midst of a shortage of Genzyme's Cerezyme, for Gaucher's disease, a rare disorder of the bones and connective tissue.
The treatment for the patient started with 19-38 units/kg of imiglucerase (Cerezyme; Genzyme, MA, USA) every two weeks.
Weinreb et al., "Improvement of bone disease by imiglucerase (Cerezyme) therapy in patients with skeletal manifestations of type 1 Gaucher disease: results of a 48-month longitudinal cohort study," Clinical Genetics, vol.
The article suggests that, in some cases, doctors and patients may not be willing to switch to an alternative as was the case when Shire launched Vpriv in the midst of a shortage of Genzyme's Cerezyme, for Gaucher's disease, a rare disorder of the bones and connective tissue.
ENCORE is a multi-national, randomized, controlled, open-label, study designed to determine whether eliglustat tartrate is non-inferior to Cerezyme (imiglucerase for injection).
That promises more competition in some established areas, with Pfizer, for example, launching its new Gaucher disease drug Elelyso at a 25% discount to Genzyme's Cerezyme. Large drugmakers are also likely to be under more pressure to curb excessive prices than small biotechs - a fact recognised by GSK, which has said it would like to see more responsible orphan drug pricing.
biotech's manufacturing problems, which led to shortages of the Cerezyme and Fabrazyme medicines.
(7) Product inventories were insufficient to meet projected demand for Cerezyme, resulting in significant patient morbidity and loss of revenue for the company.
Under the definitive agreement, Sanofi will pay Genzyme shareholders one contingent value right (CVR) per share, representing right to additional payments, based on the success of Genzymea[euro](tm)s experimental multiple sclerosis drug Lemtrada over time, or targeted production volumes for 2011 are achieved by its Cerezyme and Fabrazyme drugs.