Cerezyme

imiglucerase

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

Cerezyme

(trade name)

Classification

Therapeutic: replacement enzyme
Pregnancy Category: C

Indications

Treatment of symptomatic type 1 Gaucher’s disease.

Action

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).

Pharmacokinetics

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)

ROUTEONSETPEAKDURATION
IVunknownunknownunknown

Contraindications/Precautions

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

Adverse Reactions/Side Effects

Central nervous system

  • dizziness
  • headache

Cardiovascular

  • mild hypotension

Gastrointestinal

  • abdominal discomfort
  • nausea

Genitourinary

  • decreased urinary frequency

Dermatologic

  • pruritus
  • rash

Miscellaneous

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

Interactions

Drug-Drug interaction

None significant.

Route/Dosage

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.

Availability

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)

Implementation

  • 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.

Cerezyme

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 ?
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.
Successful pregnancy on enzyme replacement therapy with cerezyme.
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.
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.
Genzyme, meanwhile, has been struggling in the wake of manufacturing problems with its key drugs Cerezyme and Fabrazyme, both aimed at genetic conditions.