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.
a trademark for an analog of the human enzyme beta-glucocerebrosiderase (imiglucerase) used for long-term therapy of Type 1 Gaucher's disease.