Cerezyme

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

• Administration of Cerezyme should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. (
  2.1 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)]

  .
  )
• The recommended dosage ranges from 2.5 units/kg three times a week to 60 units/kg once every two weeks. (
  2.2 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.
  )
• Cerezyme is administered by intravenous infusion over 1 to 2 hours. (
  2.2 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.
  )
• Titrate the dosage based on clinical manifestations of disease and therapeutic goals for the patient. (
  2.2 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.
  )
• See the full prescribing information for preparation and administration instructions. (
  2.3 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.

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.