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an analogue of the enzyme lacking in Gaucher's disease, used for treating the adult form of the disease; administered by intravenous infusion.
Pregnancy Category: C
ClassificationTherapeutic: replacement enzyme
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.
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
- mild hypotension
- abdominal discomfort
- decreased urinary frequency
- antibody production (most frequent)
- hypersensitivity reactions
Drug-Drug interactionNone 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
- 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 DiagnosesFatigue (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.
- 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.
- Increasing hemoglobin and platelet counts and decreasing acid phosphatase levels, hepatomegaly, and splenomegaly. In pediatric patients, cachexia and wasting should diminish.
imiglucerase/im·i·glu·cer·ase/ (im″ĭgloo´ser-ās) an analogue of glucosylceramidase, for which it is used as an enzyme replenisher in type 1 Gaucher's disease.
an analog of a human enzyme produced by recombinant deoxyribonucleic acid technology.
indications It is prescribed as enzyme replacement therapy for patients with type I Gaucher disease.
contraindications There are no known contraindications to the use of imiglucerase by injection.
adverse effects The side effects most often reported include headache, nausea, abdominal discomfort, dizziness, and rash.