Imiglucerase

NO UK SPC IN BUNDLE — dose taken from US labelling (CEREZYME, Genzyme); verify against the UK SPC. Ensure physicians knowledgeable in the management of patients with Gaucher disease direct therapy, and administer under the supervision of a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis, in a healthcare setting with cardiopulmonary resuscitation equipment available. INFUSION DURATION: for adults and paediatric patients weighing greater than 20 kg, infuse over 1 to 2 hours; for paediatric patients weighing 20 kg or less, infuse over 2 hours. DILUTION VOLUMES at a dose of 60 units/kg: 1.5 kg to <6 kg — 12 mL in a syringe for infusion; 6 kg to <13 kg — 26 mL in a syringe; 13 kg to <=20 kg — 100 mL infusion bag; >20 kg to <=100 kg — 200 mL infusion bag; >100 kg — 400 mL infusion bag. If prescribed at a dose lower than 60 units/kg, dilute to a final concentration between 6 units/mL and 30 units/mL; if the total volume is 26 mL or less, administer by syringe pump. For patients who have experienced hypersensitivity reactions, premedicate with antihistamines and/or corticosteroids. Discontinue immediately and treat if a severe hypersensitivity or severe infusion-associated reaction occurs; for mild or moderate reactions consider decreasing the infusion rate, temporarily stopping the infusion, and/or giving antihistamines, antipyretics and/or corticosteroids. PAEDIATRIC: safety and effectiveness for non-CNS manifestations of Type 1 and Type 3 Gaucher disease have been established in paediatric patients, but the label gives no separate paediatric units/kg regimen — only the infusion-duration and dilution rules above. paedDose is left null because the dose is expressed in units/kg rather than mg/kg; verify the paediatric regimen against a children's formulary.