Dosing Preparation and Administration
Individualized Cerezyme Dosing
Tailoring therapy to meet a patient’s individual therapeutic goals requires individualization of dosage. Responses to Cerezyme may vary. Any change in dosages should be determined by the healthcare provider.
The initial Cerezyme dose should be individualized based on the patient’s age, the results of the comprehensive initial assessment, the patient’s overall severity and burden of disease, and the findings from regular comprehensive monitoring. Initial dosages range from 2.5 units/kg of body weight 3 times a week to 60 units/kg once every 2 weeks. Based on progress related to therapeutic goals, physicians may choose to modify the dose based on individual patient needs.
The objective of maintenance dosing is to enable the patient to maintain therapeutic goals. Maintenance dosing should be based on the results of regular comprehensive monitoring.
Patients must be monitored after initiation of Cerezyme treatment to ensure that therapeutic goals are met. Dosage increases should be considered for patients who do not achieve their therapeutic goals within the expected time frame. Dosage increases should also be considered for patients who do not sustain their therapeutic goals. Go to Therapeutic Goals for more information.
Before dosage is reduced for any patient, there must be careful and thorough evaluation of improvement in disease compartments.
Preparation and Administration
To review the steps required to prepare and administer Cerezyme, you can view the Cerezyme Reconstitution and Administration (R&A) guide and video here, or request copies. Download a PDF copy of the Reconstitution & Administration Guide. Go to Request Materials to order the printed guide and video.
Indication & Usage
Cerezyme® (imiglucerase for injection) is indicated for long-term enzyme replacement therapy for pediatric and adult patients with a confirmed diagnosis of Type 1 Gaucher disease that results in one or more of the following conditions:
- bone disease
- hepatomegaly or splenomegaly
Important Safety Information
Approximately 15% of patients have developed IgG antibodies to Cerezyme during the first year of therapy. Approximately 46% of patients with detectable IgG antibodies experienced symptoms of hypersensitivity, and these patients have a higher risk of hypersensitivity. It is suggested that patients be monitored periodically for IgG antibody formation during the first year of treatment.
Hypersensitivity has also been observed in patients without detectable IgG antibodies. Symptoms suggestive of hypersensitivity have been noted in approximately 6.6% of all patients, and anaphylactoid reactions in less than 1%. Treatment with Cerezyme should be approached with caution in patients who have exhibited hypersensitivity symptoms such as pruritus, flushing, urticarial, angioedema, chest discomfort, dyspnea, coughing, cyanosis, and hypotension. Pre-treatment with antihistamines and/or corticosteroids and a reduced rate of infusion may allow continued treatment in most patients.
In less than 1% of patients, pulmonary hypertension and pneumonia have been observed during treatment with Cerezyme. These are known complications of Gaucher disease regardless of treatment. Patients with respiratory symptoms in the absence of fever should be evaluated for the presence of pulmonary hypertension.
Approximately 13.8% of patients have experienced adverse events related to treatment with Cerezyme. Some of these are injection site reactions such as discomfort, pruritus, burning, swelling or sterile abscess at the site at the site of venipuncture. Additional adverse reactions that have been reported include nausea, abdominal pain, vomiting, diarrhea, rash, fatigue, headache, fever, dizziness, chills, backache, and tachycardia. Transient peripheral edema has also been reported for this therapeutic class of drug.
To report suspected adverse reactions, contact Genzyme at 800-745-4447, option 2 or FDA at 800-FDA-1088 or http://www.fda.gov/Safety/MedWatch
Please see Full Prescribing Information (PDF).