Drug reference
Agalsidase beta, an enzyme produced by recombinant DNA technology are licensed for long-term enzyme replacement therapy in Fabry’s disease (a lysosomal storage disorder caused by deficiency of alpha- galactosidase A).
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Fabry’s disease (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 1 mg/kg every 2 weeks
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→Appendix 1: agalsidase beta Blood and nutrition
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▶Common or very common Angioedema. arrhythmias. arthralgia. asthenia. chest discomfort. chills. cough. diarrhoea. dizziness. drowsiness. dyspnoea. eye disorders . fever. gastrointestinal discomfort. headache. hypertension. hyperthermia. hypotension. increased risk of infection. muscle complaints. musculoskeletal stiffness . nasal complaints. nausea. oedema. oral hypoaesthesia. pain. pallor. palpitations. respiratory disorders. sensation abnormal. skin reactions. syncope. temperature sensation altered. throat complaints. tinnitus. vasodilation. vertigo. vomiting ▶Uncommon Dysphagia. ear discomfort. eye pruritus. influenza like illness. malaise. peripheral coldness. tremor ▶Frequency not known Anaphylactoid reaction. hypersensitivity vasculitis. hypoxia SIDE-EFFECTS, FURTHER INFORMATION Infusion-related reactions; manage by slowing the infusion rate, or minimise by pre-treatment with an antihistamine, antipyretic, or corticosteroid — consult product literature.
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Use with caution.
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Use with caution—no information available.
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For intravenous infusion, given intermittently in Sodium chloride 0.9%, reconstitute initially with Water for Injections (5 mg in 1.1 mL, 35 mg in 7.2 mL) to produce a solution containing 5 mg/mL. Dilute with Sodium Chloride 0.9% (for doses less than 35 mg dilute with at least 50 mL; doses 35–70 mg dilute with at least 100 mL; doses 70–100 mg dilute with at least 250 mL; doses greater than 100 mg dilute with 500 mL) and give through an in-line low protein-binding 0.2 micron filter at an initial rate of no more than 15 mg/hour; for subsequent infusions, infusion rate may be increased gradually once tolerance has been established.
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There can be variation in the licensing of different medicines containing the same drug. Powder for solution for infusion ▶Fabrazyme (Genzyme Therapeutics Ltd) Agalsidase beta 5 mg Fabrazyme 5mg powder for solution for infusion vials | 1 vial P £315.08 Agalsidase beta 35 mg Fabrazyme 35mg powder for solution for infusion vials | 1 vial P £2,196.59 ENZYME STABILISER
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1 mg/kg every 2 weeks
First-line fragment. Read the complete Indications and Dose section above.
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