CEREZYME® (IMIGLUCERASE) 400U POWDER FOR SOLUTION FOR INJECTION [SIN13842P]

Product Info

CEREZYME® (IMIGLUCERASE) 400U POWDER FOR SOLUTION FOR INJECTION

[SIN13842P]

Therapeutic Indications
Cerezyme (imiglucerase) is indicated for use as long-term enzyme replacement therapy in patients with a confirmed diagnosis of non-neuronopathic (Type 1) or chronic neuronopathic (Type 3) Gaucher disease who exhibit clinically significant non-neurological manifestations of the disease.

The non-neurological manifestations of Gaucher disease include one or more of the following conditions:

• anemia after exclusion of other causes, such as iron deficiency

• thrombocytopenia

• bone disease after exclusion of other causes such as Vitamin D deficiency

• hepatomegaly or splenomegaly

Dosage and Administration
Disease management should be directed by physicians knowledgeable in the treatment of Gaucher disease.

Posology
Due to the heterogeneity and the multi-systemic nature of Gaucher disease, dosage should be individualised for each patient based on a comprehensive evaluation of all clinical manifestations of the disease. Once individual patient response for all relevant clinical manifestations is well-established, dosages and frequency of administration may be adjusted with the goal to either maintain already reached optimal parameters for all clinical manifestations or further improve those clinical parameters which have not yet been normalised.

A range of dosage regimens has proven effective towards some or all of the non-neurological manifestations of the disease. Initial doses of 60 units/kg of body weight once every 2 weeks have shown improvement in haematological and visceral parameters within 6 months of therapy and continued use has either stopped progression of or improved bone disease. Administration of doses as low as 15 units/kg of body weight once every 2 weeks has been shown to improve haematological parameters and organomegaly, but not bone parameters. The usual frequency of infusion is once every 2 weeks; this is the frequency of infusion for which the most data are available.

Paediatric population
No dose adjustment is necessary for the paediatric population.

Cerezyme has been administered to patients younger than 2 years of age; however, the safety and effectiveness in patients younger than 2 have not been established.

The efficacy of Cerezyme on neurological symptoms of chronic neuronopathic Gaucher patients has not been established and no special dosage regimen can be recommended for these manifestations (see section Pharmacodynamic Properties – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).

Method of Administration
After reconstitution and dilution, the preparation is administered by intravenous infusion. At initial infusions, Cerezyme should be administered at a rate not exceeding 0.5 unit per kg body weight per minute. At subsequent administrations, infusion rate may be increased but should not exceed 1 unit per kg body weight per minute. Infusion rate increases should occur under supervision of a health care professional.

Infusion of Cerezyme at home may be considered for patients who are tolerating their infusions well for several months. Decision to have patient move to home infusion should be made after evaluation and recommendation by the treating physician. Infusion of Cerezyme by the patient or caregiver at home requires training by a health care professional in a clinical setting. The patient or caregiver will be instructed in infusion technique and the keeping of a treatment diary. Patients experiencing adverse events during the infusion need to immediately stop the infusion process and seek the attention of a healthcare professional. Subsequent infusions may need to occur in a clinical setting. Dose and infusion rate should remain constant while at home, and not be changed without supervision of a health care professional.

For instructions on reconstitution and dilution of the medicinal product before administration, see section Special Precautions for Disposal and Other Handling – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information.

Medical or healthcare professionals are encouraged to register Gaucher patients, including those with chronic neuronopathic manifestations of the disease, in the “ICGG Gaucher Registry” (see section Pharmacodynamic Properties – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).

Contraindications
Hypersensitivity to the active substance or to any of the excipients (see section Special Warnings and Precautions for Use – please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information).