Dosing & Uses
Dosage Forms & Strengths
injection, lyophilized powder of reconstitution
- 400units/vial
Gaucher Disease
Indicated for type 1 Gaucher's disease; associated with one or more of the following: anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly
2.5 units/kg IV 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Dosage Forms & Strengths
injection, lyophilized powder of reconstitution
- 400units/vial
Gaucher Disease
<2 years: Safety and efficacy not estabilshed
>2 years: 2.5 units/kg IV 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Interactions
Interaction Checker
No Results
Contraindicated
Serious - Use Alternative
Significant - Monitor Closely
Minor
Contraindicated (0)
Serious - Use Alternative (0)
Monitor Closely (1)
- miglustat
miglustat decreases levels of imiglucerase by increasing renal clearance. Use Caution/Monitor. Effects on imiglucerase levels variable; concomitant use not recommended by mfr of miglustat (Zavesca).
Minor (0)
Adverse Effects
Frequency Not Defined
Dizziness
Headache
Tachycardia
Cyanosis
Flushing
Hypotension
Cough
Dyspnea
Pneumonia
Pulmonary hypertension
Abdominal pain
Diarrhea
Nausea
Vomiting
Anaphylaxis
Hypersensitivity
Angioedema
Pruritus
Rash
Urticaria
Back pain
Chest discomfort
Chills
Fatigue
Infusion-site burning
Infusion-site discomfort
Infusion-site swelling
Pyrexia
Warnings
Contraindications
None listed in the manufacturer's label
Cautions
Antibodies to imiglucerase that may lead to infusion reactions reported in up to 15% of patients within 6 months from the onset of therapy; antibody formation rare after 12 months of therapy
Most patients with infusion reactions are able to continue treatment if pre-treated with antihistamines and/or corticosteroids and infused at a lower rate
Pregnancy & Lactation
Pregnancy
Published observational studies and case reports with use in pregnant females have not identified a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes
No animal reproduction studies have been conducted
Pregnancy exposure registry
- Monitors pregnancy outcomes in females during pregnancy
- Encourage pregnant females exposed to drug to contact the Gaucher patient registry at 1-800-745-4447, extension 15500 or visit www.registrynxt.com
Disease-associated maternal and/or embryofetal risk
- Pregnancy may exacerbate existing Type 1 Gaucher disease symptoms or result in new disease manifestations
- Untreated symptomatic Type 1 Gaucher may lead to complications during pregnancy, including hepatosplenomegaly, which can interfere with the normal growth of a pregnancy and thrombocytopenia, which can lead to excessive bleeding
Lactation
Available published literature suggests a small amount of imiglucerase is present in breast milk immediately following an infusion
Published case reports and postmarketing reports of breastfed infants have not reported adverse effects due to Cerezyme exposure
There are no data available on the effects on milk production
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Analog of beta-glucocerebrosidase, lysosomal enzyme replacement that plays a role in the hydrolysis of glucocerebroside to glucose and ceramide
Absorption
Steady-steady was reached at 30 min
Distribution
Vd: 0.09-0.15 L/kg
Elimination
Half-life: 3.6-10.4 min
Clearance: 9.8-20.3 mL/min/kg
Administration
IV Preparation
Reconstitute with 10.2 mL sterile water for injection for 400 U vial (resulting conc 40 U/mL)
Roll and tilt vial to allow powder to dissolve completely
Visually inspect solution after reconstitution for particulate matter and discoloration; discard if opaque particles or discoloration are observed
Withdraw drug volume from reconstituted vial
Patients weighing <18 kg; Dilute with 0.9% NaCl to a final volume of 100 mL
Patients weighing ≥18 kg: Dilute solution with 0.9% NaCl, to a final volume of 100-200 mL
Gently invert infusion bag to mix the solution, avoiding vigorous shaking and agitation
Visually inspect the solution before administering of the final product for particulate matter and discoloration; slight flocculation of protein particles (described as thin translucent fibers) may occur after dilution and does not affect the quality of the product
IV Administration
Patients who experience hypersensitivity reactions: Premedicate with antihistamines and/or corticosteroids
Monitor patients for the occurrence of new hypersensitivity reactions
Use an in-line low protein-binding 0.2-micron filter during infusion
Patients weighing <18 kg: Infuse over 2 hr
Patients weighing ≥18 kg: Infuse over 1-2 hr
Storage
Unopened vials
- Refrigerate at 2-8°C (36-46°F)
Reconstituted vials
- If not used immediately, store at room temperature at 68-77°F (20-25°C) or refrigerate at 36-46°F (2-8°C) for up to 12 hr
Diluted solution
- After dilution, stable for up to 24 hours when refrigerated at 36-46°F (2-8°C)
Images
Patient Handout
imiglucerase intravenous
IMIGLUCERASE - INJECTION
(im-eh-GLUE-ser-aze)
COMMON BRAND NAME(S): Cerezyme
USES: Imiglucerase is used to treat a certain rare genetic problem (Gaucher disease). Imiglucerase replaces a certain natural substance (an enzyme called glucocerebrosidase) that is missing in people with Gaucher disease.This medication improves blood, bone, liver, and spleen problems caused by Gaucher disease. Imiglucerase does not correct the genetic problem, and treatment must be continued for life.
HOW TO USE: This medication is given slowly into a vein over 1 to 2 hours as directed by your doctor. The dosage and how often it is given depends on your weight, medical condition, and response to treatment. Your doctor may direct you to receive it as often as 3 times per week or as little as once every 2 weeks. Follow your doctor's directions carefully.If you are giving this medication to yourself at home, learn all preparation and usage instructions from your health care professional. Before using, check this product visually for particles or discoloration. If either is present, do not use the liquid. Learn how to store and discard medical supplies safely.Infusion reactions may occur while you are receiving this drug and for a short time after. Tell your doctor right away if you have symptoms such as shortness of breath, cough, flushing, dizziness, or fast heartbeat.Tell your doctor if your condition lasts or gets worse. Blood, liver, and spleen problems usually improve in the first year of treatment. It may take 2 to 3 years of treatment to see the full benefit to your bones.
SIDE EFFECTS: See also How to Use section.Fever, headache, nausea, vomiting, swelling of the hands/feet (edema), or redness/pain/itching/swelling at the injection site may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.
PRECAUTIONS: Before using imiglucerase, tell your doctor or pharmacist if you are allergic to it; or to alglucerase; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history.Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.This medication may pass into breast milk. Consult your doctor before breast-feeding.
DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.
OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.
NOTES: Do not share this medication with others.Lab and/or medical tests (such as IgG antibodies) should be done while you are using this medication. Keep all medical and lab appointments. Consult your doctor for more details.
MISSED DOSE: It is important to get each dose of this medication as scheduled. If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule. Do not double the dose to catch up.
STORAGE: Before mixing, store the vial in the refrigerator. Protect from light and freezing. Once mixed, consult the product instructions and your pharmacist for storage details. Discard any unused portion. Keep all medications away from children and pets.Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.
Information last revised July 2023. Copyright(c) 2023 First Databank, Inc.
IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.
Formulary
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