NAGLAZYME Concentrate for solution for infusion Ref.[9218] Active ingredients: Galsulfase

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.

Several patients have received their total dose of Naglazyme at approximately twice the recommended infusion rate without apparent adverse events.

Shelf life

Unopened vials: 3 years.

Diluted solutions: Chemical and physical in-use stability has been demonstrated for up to 4 days at room temperature (23°C-27°C).

From a microbiological safety point of view, Naglazyme should be used immediately. If not used immediately, in-use storage times and conditions are the responsibility of the user and should normally not be longer than 24 hours at 2°C-8°C followed by up to 24 hours at room temperature (23°C-27°C) during administration.

Store in a refrigerator (2°C-8°C).

Do not freeze.

For storage conditions of the diluted medicinal product, see section 6.3.

Vial (type I glass) with a stopper (siliconized chlorobutyl rubber) and a seal (aluminium) with a flip-off cap (polypropylene).

Pack sizes: 1 and 6 vials.

Not all package sizes may be marketed.

Each vial of Naglazyme is intended for single use only. The concentrate for solution for infusion has to be diluted with sodium chloride 9 mg/ml (0.9%) solution for infusion using aseptic technique. It is recommended that the diluted Naglazyme solution be administered to patients using an infusion set equipped with a 0.2 μm in-line filter.

Any unused product or waste material is to be disposed of in accordance with local requirements.

Preparation of the Naglazyme infusion (aseptic technique is to be used)

The number of vials to be diluted based on the individual patient’s weight must be determined and removed from the refrigerator approximately 20 minutes in advance in order to allow them to reach room temperature.

Before dilution, each vial is to be inspected for particulate matter and discolouration. The clear to slightly opalescent and colourless to pale yellow solution must be free of visible particles.

A volume of the sodium chloride 9 mg/ml (0.9%) solution for infusion is to be withdrawn and discarded from a 250 ml infusion bag equal to the total volume of Naglazyme to be added. 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. When using 100 ml bags, the volume of Naglazyme may be added directly to the infusion bag.

The volume of Naglazyme is to be slowly added to the sodium chloride 9 mg/ml (0.9%) solution for infusion.

The solution is to be mixed gently before infusion.

The solution is to be visually inspected for particulate matter prior to use. Only clear and colourless solutions without visible particles should be used.