i-mi-GLOO-ser-ase

Therapeutic Classification: replacement enzyme

• Symptomatic type 1 Gaucher disease.

• Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher disease.

• Improvement in symptoms of Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly).

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Excreted mainly by the kidneys.

Half-life: 3.6–10.4 min.

(improvement in symptoms)

Contraindicated in:

• Hypersensitivity.

Use Cautiously in:

• Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant.

CV: hypotension.

Derm: pruritus, rash.

GI: abdominal discomfort, nausea.

GU: decreased urinary frequency.

Neuro: dizziness, headache.

Misc: fever, HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS), infusion-associated reactions.

Drug-Drug:

• None reported.

• IV (Adults and Children): Initial range of 2.5 units/kg 3 times weekly to 15–60 units/kg every 1–2 wk. Evaluate dosage every 6 mo for possible reduction.

• Lyophilized powder for injection: 400 units/vial;

• Assess for infusion-associated reactions (angioedema, pruritus, rash, urticaria, chest discomfort, chills, fatigue, infusion-site burning, infusion-site discomfort, infusion-site swelling, pyrexia). If a reaction occurs, decreasing the infusion rate, temporarily stopping the infusion and/or administering antihistamines and/or antipyretics may ameliorate the symptoms. Closely monitor patients who have experienced reactions when readministering.
• 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 ↓ rate of infusion usually allows patient to continue use.

• Monitor hemoglobin and platelet count monthly to determine effectiveness of therapy. If hemoglobin is <7 g/dL or platelet count is <50,000 cells/mm³, monitor every 2 wk; levels should ↑ 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.

• Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.
• If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue immediately and initiate appropriate medical treatment (epinephrine). Consider the risks and benefits of readministering following severe hypersensitivity reactions.
• Determine the correct amount of imiglucerase and appropriate number of vials. Reconstitution: Reconstitute each vial with 10.2 mL of sterile water for injection for a volume of 5.3 mL (40 units/mL); inject down the inside wall of each vial. Roll and tilt vial to allow the powder to dissolve completely. Withdraw up to 10 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; thin translucent fibers may occur after dilution and do not affect quality of product. Reconstituted solution is stable for 12 hr at room temperature or refrigerated.
• May also be administered undiluted.
• Small dose 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; may be filtered through an in-line low-protein-binding 0.2-micron filter during administration.
  • Undiluted solution may be administered at a rate no greater than 1 unit/kg/min.
• Additive Incompatibility: Do not admix with other drugs or solutions.

• Explain purpose and the importance of treatment at least every 4 wk to the patient. Imiglucerase helps control the symptoms but does not cure Gaucher disease. Lifelong therapy may be required.
• Advise patient to readPatient Information before starting therapy
• Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
• Teach patient the signs and symptoms of hypersensitivity reactions, and infusion-associated reactions; if any occur, seek immediate medical care.
• Advise patient to notify a health care professional if a dose is missed.
• Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to imiglucerase during pregnancy. Pregnant women exposed to imiglucerase and health care providers are encouraged to contact the Gaucher patient registry at 1-800-745-4447, ext. 15500, or visit .
• 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.

Cerezyme

NDC Code^*

• 58468- Genzyme Corporation
  • 58468-1983- Cerezyme
    • 58468-1983-1- 1 VIAL, GLASS in 1 CARTON (58468-1983-1) > 5 mL in 1 VIAL, GLASS

• 58468- Genzyme Corporation
  • 58468-4663- Cerezyme
    • 58468-4663-1- 1 VIAL, GLASS in 1 CARTON (58468-4663-1) > 10 mL in 1 VIAL, GLASS