Dosage & Administration
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Cerezyme Prescribing Information
Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy.
Initiate Cerezyme in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue Cerezyme and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur [see Warnings and Precautions (5.1)].
Cerezyme is indicated for treatment of adults and pediatric patients 2 years of age and older with Type 1 Gaucher disease that results in one or more of the following conditions:
- anemia
- thrombocytopenia
- bone disease
- hepatomegaly or splenomegaly
Recommendations Prior to Cerezyme Treatment
Administration of Cerezyme should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis [see Warnings and Precautions (5.1)].
Initiate Cerezyme in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions (5.1)].
For patients who experience hypersensitivity reactions to Cerezyme, premedicate with antihistamines and/or corticosteroids. Monitor patients for the occurrence of new hypersensitivity reactions [see Warnings and Precautions (5.1)].
Recommended Dosage
Therapy with Cerezyme should be directed by physicians knowledgeable in the management of patients with Gaucher disease.
The recommended dosage of Cerezyme based upon disease severity ranges from 2.5 units/kg three times a week to 60 units/kg once every two weeks. For patients weighing 18 kg and greater, infuse the diluted Cerezyme solution over 1 to 2 hours. For patients weighing less than 18 kg, infuse the diluted Cerezyme solution over 2 hours [see Dosage and Administration (2.3)]. Titrate the dosage based on clinical manifestations of disease and therapeutic goals for the patient.
Preparation and Administration Instructions
Cerezyme does not contain preservatives.
Reconstitution and Dilution Using Aseptic Technique
- Determine the number of Cerezyme vials to be reconstituted based on the individual patient's dosage regimen and remove vial(s) from the refrigerator.
- Reconstitute each 400 unit vial of Cerezyme by slowly injecting 10.2 mL of Sterile Water for Injection, USP, down the inside wall of each vial.
- Roll and tilt the vial to allow the powder to dissolve completely. Each vial will yield a concentration of Cerezyme after reconstitution of 40 units/mL. Visually inspect the solution after reconstitution for particulate matter and discoloration. Discard if opaque particles or discoloration are observed.
- Withdraw up to 10 mL per vial. Discard unused portion.
- Dilute the Cerezyme solution promptly with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 to 200 mL. For patients weighing less than 18 kg, dilute Cerezyme to a final volume of 100 mL. Gently invert infusion bag to mix the solution, avoiding vigorous shaking and agitation.
Visually inspect the solution prior to administration 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. - For patients weighing 18 kg and greater, infuse the diluted Cerezyme solution over 1 to 2 hours. For patients weighing less than 18 kg, infuse the diluted Cerezyme solution over 2 hours.
- The diluted solution may be filtered through an in-line low protein-binding 0.2 µm filter during administration.
Storage and Handling
- If the reconstituted Cerezyme vial is not used immediately, store at room temperature at 68°F to 77°F (20°C to 25°C) or refrigerated at 36°F to 46°F (2°C to 8°C) for up to 12 hours.
- After dilution, Cerezyme is stable for up to 24 hours when stored refrigerated at 36°F to 46°F (2°C to 8°C).
For injection: 400 units of imiglucerase as a white to off-white lyophilized powder in a single-dose vial for reconstitution.
Pregnancy
Pregnancy Exposure Registry
There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to Cerezyme during pregnancy. Pregnant women exposed to Cerezyme and health care providers are encouraged to contact the Gaucher patient registry at 1-800-745-4447, extension 15500 or visit www.registrynxt.com.
Risk Summary
Available data on more than 500 pregnancies from the international Gaucher Disease registry, postmarketing reports, published observational studies and case reports with Cerezyme or non–US-licensed imiglucerase use in pregnant women have not identified a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes. There are risks associated with symptomatic Type I Gaucher disease in pregnancy (see Clinical Considerations). No animal reproduction studies have been conducted with imiglucerase.
The estimated background risk of major birth defects and miscarriage in the indicated population is unknown. All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2% to 4% and 15% to 20%, respectively.
Clinical Considerations
Disease-associated Maternal and/or Embryo/Fetal 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
Risk Summary
Available published literature suggests a small amount of imiglucerase is present in breast milk immediately following an infusion of imiglucerase. 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. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for Cerezyme and any potential adverse effects on the breastfed infant from imiglucerase or from the underlying maternal condition.
Lactating women with Gaucher disease treated with Cerezyme should be encouraged to enroll in the Gaucher patient registry [see Use in Specific Populations (8.1)].
Pediatric Use
The safety and effectiveness of Cerezyme for treatment of Type 1 Gaucher disease that results in one or more of the following conditions: anemia, thrombocytopenia, bone disease, hepatomegaly or splenomegaly have been established in pediatric patients 2 years of age and older. Use of Cerezyme for this indication is supported by evidence from adequate and well-controlled studies of Cerezyme and alglucerase in adults and pediatric patients 12 years of age and older, with additional data obtained from the medical literature and from postmarketing experience in pediatric patients as young as 2 years of age [see Adverse Reactions (6.1), Clinical Studies (14)].
The safety and effectiveness of Cerezyme have not been established in pediatric patients younger than 2 years of age.
None.