imiglucerase


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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) ,

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.

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.

imiglucerase

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.
References in periodicals archive ?
Specific therapy for the nonneuronopathic manifestations of Gaucher disease has been available since 1991 firstly in the form of the macrophage targeted placenta-derived glucocerebrosidase (alglucerase, Ceredase [R], Genzyme Corporation, MA) (7), and subsequently (1994 in USA and 1997 in Europe) by recombinant human enzyme, imiglucerase (Cerezyme[R], Genzyme Corporation, MA) (8).
Drug profiles are provided for afegostat tartrate + ERT, ambroxol, DOS-000011, eliglustat tartrate, Gluco-Cerebosidase, imiglucerase biosimilar, JR-101, LB-201, LB-205, NP-003, PRX-112, Recombinant Enzyme for Gaucher's Disease, Recombinant Enzyme to Replace Beta-Cerebrosidase for Gaucher's Disease, Small Molecule to Activate Glucocerebrosidase for Parkinson's, Gaucher's and Neurodegenerative Diseases and Small Molecules to Activate Glucocerebrosidase for Gaucher and Parkinson Disease in this research available for purchase at http://www.
1 Enzyme replacement therapy (ERT): (1) imiglucerase, an analogue of human intracellular glucocerebrosidase, is the treatment of choice for types 1 and 3 Gaucher disease.
The safety and efficacy of velaglucerase alfa was assessed in adults and children aged 4 years and older via a phase three program, which included Gaucher patients who switched to velaglucerase alfa after being treated with imiglucerase, as well as naE[macron]ve patients, including an active comparison with imiglucerase.
Since then, the Therapeutic Goods Administration has designated two biological drugs as orphans--rabies immunoglobulin and recombinant enzyme imiglucerase for replacement therapy in patients with Gaucher disease.
Saturating patient bases for human growth hormones and recombinant imiglucerase will preclude faster growth.
This therapy uses either imiglucerase or alglucerase, both of which are forms of the enzyme glucocerebrosidase.
VPRIV is approved in 38 countries globally, including the US, the European Union, and Israel and is for patients who are treatment-naive as well as patients who have previously been treated with imiglucerase.
These data add to the growing body of clinical evidence which support the use of VPRIV in patients both transitioning from imiglucerase or who are treatment naE[macron]ve.
The treatment of choice is enzyme replacement therapy with imiglucerase (Cerezyme), and at present there are about 30 patients on this programme.
VPRIV is for patients who are treatment-naE[macron]ve as well as patients who have previously been treated with imiglucerase.
Patients currently being treated with imiglucerase for Type 1 Gaucher disease may be switched to VPRIV.