Velmanase alfa

Alpha-mannosidosis (LAMZEDE, velmanase alfa-tycv). Prior to administration, consider pre-treating with antihistamines, antipyretics and/or corticosteroids. For females of reproductive potential, verify that the patient is not pregnant before initiating treatment. Administration: the total infusion volume is determined by the patient's actual body weight and should be given over a MINIMUM of 60 minutes for patients weighing up to 49 kg; patients weighing 50 kg and greater should be infused at a MAXIMUM infusion rate of 25 mL/hour to control the protein load. Use an infusion set with a pump and a low-protein-binding 0.2 micron in-line filter. Each reconstituted vial yields 2 mg/mL. Missed doses: if one or more doses are missed, restart treatment as soon as possible provided it is at least 3 days from the next scheduled dose; if within 3 days of the next scheduled dose, give only the next dose per schedule. Hypersensitivity / infusion-associated reactions (IARs): for severe reactions (including anaphylaxis) immediately discontinue and treat; for mild to moderate reactions consider holding the infusion for 15 to 30 minutes, slowing the rate to 25% to 50% of the recommended rate and treating — if symptoms subside, resume at 25% to 50% of the recommended rate and, if tolerated, increase in 25% increments back to the recommended rate; if symptoms persist, stop the infusion and consider re-initiating within 7 to 14 days at 25% to 50% of the recommended rate with appropriate pretreatment. Geriatric: alpha-mannosidosis is largely a disease of paediatric and young adult patients — clinical trials did not include patients 65 years and older. Source: US FDA prescribing information (DailyMed, Chiesi USA, label date 2023-02-21); NO UK SPC was available in the fetched bundle — verify against UK/EMA labelling before use.

The US label states a single weight-based dosage of 1 mg/kg (actual body weight) once every week for all patients; safety and effectiveness for alpha-mannosidosis have been established in paediatric patients, supported by an adequate and well-controlled trial in adult and paediatric patients and an open-label trial in 5 paediatric patients younger than 6 years. Trial 1 included paediatric patients aged 6–17 years who received 1 mg/kg weekly for 52 weeks. Paediatric patients reported a HIGHER incidence of hypersensitivity reactions than adults (14 of the 19 patients with hypersensitivity reactions were paediatric). Infusion duration is weight-based, not age-based (minimum 60 minutes up to 49 kg; maximum 25 mL/hour at 50 kg and above). Verify all paediatric dosing against a children's formulary and against UK labelling — this figure is from US prescribing information only.