Talking with Patients
Cerezyme treatment may be more likely to succeed when the patient understands the disease and the treatment process. Before treatment begins, patients and family members may want to be counseled about infusion therapy, treatment-associated risks, possible therapeutic outcomes, and the need to keep appointments and maintain compliance. Gaucher disease may raise many emotional questions and concerns, and physicians should be prepared to discuss these issues or refer patients for any appropriate counseling. It is important for patients to be informed of risks associated with Cerezyme therapy.
Infusion therapy concerns
You may wish to inform patients that infusion therapy usually takes place at a doctor's office, infusion center, or through home infusions under careful medical supervision. You can also point out that the treatment follows a routine.
You may consider offering reminders to patients to keep appointments for monitoring and regular check ups. Whether they receive Cerezyme therapy or not, regular monitoring is important for gauging disease progression and tracking therapeutic progress.
Expectations of therapy
Patients and their families may have high hopes for quick results with Cerezyme therapy. Consider offering explanations on how results may vary from patient to patient and depend largely on the severity of symptoms at the beginning of treatment. Please see the Therapeutic Goals section for information on setting and reaching appropriate goals
It is important to encourage patients to stay on therapy. You may want to emphasize that Type 1 Gaucher disease is a lifelong disorder, and that Cerezyme is not a cure. It is important that patients maintain therapeutic compliance in order to continue to benefit.
Therapy and planning ahead
Your patients may require assistance in planning ahead for their therapy when away from home, such as when going to camp, attending college, and enjoying vacations. Genzyme Care Coordination is a service staffed by case managers who can help arrange for continuous Cerezyme therapy. For more information, go to Genzyme Care Coordination.
Talking to Family Members
Patients may have concerns about family planning or talking to family members who may be at risk for carrying the disease. A genetic counselor can help them understand inheritance patterns and can provide advice on family planning and other issues. See Genetic Counseling for more information.
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).