imiglucerase


imiglucerase

 [im″ĭ-gloo´ser-ās] an analogue of the enzyme lacking in Gaucher's disease, used for treating the adult form of the disease; administered by intravenous infusion.

imiglucerase

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

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
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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.