NAGLAZYME CONCENTRATE FOR SOLUTION FOR INFUSION 5MG/5ML

INFUSION, SOLUTION CONCENTRATE

**4.2 Posology and Method of Administration** As for all lysosomal genetic disorders, it is of primary importance, especially in severe forms, to initiate treatment as early as possible, before appearance of non-reversible clinical manifestations of the disease. Naglazyme treatment should be supervised by a physician experienced in the management of patients with MPS VI or other inherited metabolic diseases. Naglazyme should be administered by a healthcare professional in an appropriate clinical setting where resuscitation equipment to manage medical emergencies would be readily available. **4.2.1 Posology** The recommended dosage regimen of NAGLAZYME is 1 mg/kg body weight administered once weekly as an intravenous infusion over a period of no less than 4 hours. **4.2.1.1 Special Populations** _**Renal and Hepatic Impairment**_ The safety and efficacy of NAGLAZYME in patients with renal or hepatic insufficiency have not been evaluated and no alternative dose regimen can be recommended in these patients _**Elderly (Geriatric Use)**_ The safety and efficacy of NAGLAZYME in patients older than 65 years has not been established, and no alternative dosage regimen can be recommended in these patients. It is not known whether older patients respond differently from younger patients. _**Pediatric Use**_ There is no evidence for special considerations when NAGLAZYME is administered to the pediatric population. However, data from patients ≤ 1 year of age are limited _\[see Adverse Reactions (4.8) and Mechanism of Action (5.1)_ – _please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information_ _\]_. **4.2.2 Method of Administration** NAGLAZYME must be diluted with 0.9% Sodium Chloride Injection, USP to a total volume of 100 mL or 250 mL based on the patient’s weight, prior to infusion _\[see Dilution and Administration Instructions (6.2.1)_ – _please refer to the Product Insert/Patient Information Leaflet published on HSA for the full drug information_ _\]_. Patients who are susceptible to fluid overload or with a body weight of 20 kg or less should receive a total volume of 100 mL. Patients with a body weight greater than 20 kg should receive a total volume of 250 mL. Adjust the initial infusion rate to allow approximately 2.5% of the total solution to be infused within the first hour. The remaining solution (approximately 97.5%) should be infused over the next three hours.