Drug Action
Elosulfase alfa is an enzyme produced by recombinant DNA technology that provides replacement therapy in conditions caused by N-acetylgalactosamine- 6-sulfatase (GALNS) deficiency.
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Drug reference
Elosulfase alfa is an enzyme produced by recombinant DNA technology that provides replacement therapy in conditions caused by N-acetylgalactosamine- 6-sulfatase (GALNS) deficiency.
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Mucopolysaccharidosis IVA (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 2 mg/kg once weekly
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Elderly—no information available. infusion- related reactions CAUTIONS, FURTHER INFORMATION ▶Infusion-related reactions Infusion-related reactions can occur; manufacturer advises these may be minimised by pre-treatment with an antihistamine and antipyretic, given 30-60 minutes before treatment. If reaction is severe, stop infusion and start appropriate treatment. Caution and close monitoring is advised during re- administration following a severe reaction.
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NICE decisions ▶Elosulfase alfa for treating mucopolysaccharidosis type IVa (December 2015) NICE HST2 Elosulfase alfa, within its marketing authorisation, is recommended for funding for treating mucopolysaccharidosis type IVa (MPS IVa) according to the conditions in the managed access agreement for elosulfase alfa. www.nice.org.uk/guidance/HST2
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▶Common or very common Chills. diarrhoea. dizziness. dyspnoea. fever. gastrointestinal discomfort. headache. hypersensitivity. myalgia. nausea. oropharyngeal pain. vomiting ▶Frequency not known Infusion related reaction
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Manufacturer advises avoid unless essential—limited information available.
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Manufacturer advises use only if potential benefit outweighs risk—present in milk in animal studies.
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For intravenous infusion (Vimizim ®), give intermittently in Sodium chloride 0.9%; body-weight under 25 kg, dilute requisite dose to final volume of 100 mL infusion fluid and mix gently, give over 4 hours through in-line filter (0.2 micron) initially at a rate of 3 mL/hour, then increase to a rate of 6 mL/hour after 15 minutes, then increase gradually if tolerated every 15 minutes by 6 mL/hour to max. 36 mL/hour; body-weight 25 kg or over, dilute requisite dose to final volume of 250 mL and mix gently, give over 4 hours through in-line filter (0.2 micron) initially at a rate of 6 mL/hour, then increase to a rate of 12 mL/hour after 15 minutes, then increase gradually if tolerated every 15 minutes by 12 mL/hour to max. 72 mL/hour. l HANDLING AND STORAGE Manufacturer advises store in a refrigerator at 2–8°C. After dilution use immediately or, if necessary, store at 2-8°C for max. 24 hours, followed by up to 24 hours at 23–27°C.
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There can be variation in the licensing of different medicines containing the same drug. Solution for infusion EXCIPIENTS: May contain Polysorbates, sorbitol ELECTROLYTES: May contain Sodium ▶Vimizim (BioMarin Europe Ltd) A Elosulfase alfa 1 mg per 1 ml Vimizim 5mg/5ml concentrate for solution for infusion vials | 1 vial P £750.00
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Driving and skilled tasks Manufacturer advises patients and carers should be counselled about the effects on driving and performance of skilled tasks—increased risk of dizziness.
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2 mg/kg once weekly
First-line fragment. Read the complete Indications and Dose section above.
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