Posology : מינונים

4.2    Posology and method of administration
A key issue is to treat children aged <5 years suffering from a severe form of the disease, even though children <5 years were not included in the pivotal phase 3 study. Limited data are available in patients < 1 year of age (see section 5.1).

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. Administration of Naglazyme should be carried out in an appropriate clinical setting where resuscitation equipment to manage medical emergencies would be readily available.

Posology

The recommended dose regimen for galsulfase is 1 mg/kg body weight administered once every week as an intravenous infusion over 4 hours.

Special populations

Elderly
The safety and efficacy of Naglazyme in patients older than 65 years has not been established, and no alternative dose regimen can be recommended in these patients.

Renal and hepatic impairment
The safety and efficacy of Naglazyme in patients with renal or hepatic insufficiency have not been evaluated (see section 5.2) and no alternative dose regimen can be recommended in these patients.

Paediatric population
There is no evidence for special considerations when Naglazyme is administered to the paediatric population. Currently available data are described in section 5.1.

Method of administration

The initial infusion rate is adjusted so that approximately 2.5% of the total solution is infused during the first hour, with infusion of the remaining volume (approximately 97.5%) over the next 3 hours.

100 ml infusion bags should be considered for patients who are susceptible to fluid volume overload and weigh less than 20 kg; in this case the infusion rate (ml/min) should be decreased so that the total duration remains no less than 4 hours.

For information on pre-treatment see section 4.4 and for further instructions see section 6.6.