Drug Class Description
Alimentary tract and metabolism products – enzymes
Generic Name
Agalsidase Beta
Drug Description
Each vial of Fabrazyme contains a nominal value of 5 mg of agalsidase beta. After reconstitution with 1.1 ml water for injections, each vial of Fabrazyme contains 5 mg/ml of agalsidase beta. The reconstituted solution must be diluted further.Agalsidase beta is a recombinant form of human ?-galactosidase A and is produced by recombinant DNA technology using a mammalian Chinese Hamster Ovary (CHO) cell culture. The amino acid sequence of the recombinant form, as well as the nucleotide sequence which encoded it, are identical to the natural form of ?-galactosidase.
Presentation
Powder for concentrate for solution for infusionWhite to off-white lyophilized cake or powder
Indications
Fabrazyme is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease.
Adult Dosage
Fabrazyme treatment should be supervised by a physician experienced in the management of patients with Fabry Disease or other inherited metabolic diseases.
The recommended dose of Fabrazyme is 1mg/kg body weight administered once every 2 weeks as an intravenous infusion.
Alternative dosing regimens have been used in clinical studies. In one of these studies, after an initial dose of 1.0 mg/kg every 2 weeks for 6 months, 0.3 mg/kg every 2 weeks may maintain clearance of GL-3 in certain cell types in some patients; however, the long term clinical relevance of these findings has not been established.
The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.
No dose adjustment is necessary for patients with renal insufficiency.
Studies in patients with hepatic insufficiency have not been performed.
The safety and efficacy of Fabrazyme in patients older than 65 years have not been established and no dosage regimen can presently be recommended in these patients.
Paediatric patients
