Galsulfase

NO UK SPC IN BUNDLE — dose taken from US labelling (NAGLAZYME, BioMarin); verify against the UK SPC. The total volume of the infusion should be delivered over a period of no less than 4 hours. The initial infusion rate should be 6 mL per hour for the first hour; if the infusion is well tolerated, the rate may be increased to 80 mL per hour for the remaining 3 hours. The infusion time can be extended up to 20 hours if infusion reactions occur. For patients 20 kg and under, or those susceptible to fluid volume overload, physicians may consider diluting in a volume of 100 mL, decreasing the infusion rate (mL per hour) so that total infusion duration remains no less than 4 hours. Administration should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis, in a healthcare setting with appropriate medical monitoring and support including access to cardiopulmonary resuscitation equipment. Pretreatment with antihistamines, with or without antipyretics, is recommended 30 to 60 minutes prior to the start of the infusion. Must not be infused with other products in the infusion tubing.

The US label states a single weight-based dosage (1 mg/kg once weekly) and gives no separate paediatric regimen. Clinical studies were conducted in 56 patients aged 5 to 29 years with the majority in the paediatric age group; in addition an open-label study was conducted in four infants (3 months to 12.7 months) treated with 1 mg/kg (n = 2) or 2 mg/kg (n = 2), with safety results consistent with those observed in patients 5 to 29 years old. For patients 20 kg and under, consider diluting in 100 mL with the infusion rate reduced so total duration remains no less than 4 hours. Verify against a children's formulary.