FABRAZYME

What it is

Fabrazyme is agalsidase beta powder for injection . It is supplied as a sterile, non-pyrogenic, white to off-white lyophilised powder in a clear glass vial . Fabrazyme is intended for intravenous infusion .

Approved indications

— Long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease (α-galactosidase deficiency) .

Dosing overview

The recommended dosage is 1 mg/kg of Fabrazyme per dose, infused every 2 weeks . Dosage should be individualised for each patient and small adjustments can be made to avoid discarding partially used vials . The initial infusion rate should be no more than 0.25 mg/min (15 mg/hr) 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 paediatric patients 8–16 years old . The safety and efficacy in patients younger than 8 years of age have not been evaluated; however, patients with Fabry disease younger than 8 years old may be treated with Fabrazyme when clearly needed and after a careful risk/benefit analysis has been conducted by the physician .

Key safety warnings

Patients treated with Fabrazyme may develop infusion-associated reactions, the majority of which are mild to moderate in intensity . If an infusion-associated reaction occurs during a Fabrazyme infusion, decreasing the infusion rate, temporarily stopping the infusion and/or administration of antipyretics, antihistamines, and/or steroids may ameliorate the symptoms . If severe allergic or anaphylactoid reactions occur, immediate discontinuation of the administration of Fabrazyme and current medical standards for emergency treatment are to be observed . Patients may develop IgG antibodies to Fabrazyme; some patients develop IgE or skin reactivity specific to Fabrazyme; and patients with antibodies to Fabrazyme have a higher risk of infusion-associated reactions . Fabrazyme is not recommended to be administered with chloroquine, amiodarone, benoquin or gentamicin due to a theoretical risk of inhibited intracellular α-galactosidase activity .

Contraindications

Treatment with Fabrazyme is contraindicated if there is clinical evidence of anaphylaxis to agalsidase beta or any of the excipients .

Regulatory history

Fabrazyme 35 mg powder for injection vial was first listed on the ARTG on 30 May 2002 . Fabrazyme 5.5 mg powder for injection vial was first listed on the ARTG on 14 April 2003 .