Posology : מינונים

4.2    Posology and method of administration

The treatment should be supervised by a physician experienced in the management of patients with alpha-mannosidosis or in the administration of other enzyme replacement therapies (ERT) for lysosomal storage disorder. Administration of Lamzede should be carried out by a healthcare professional with the ability to manage ERT and medical emergencies.

Posology

The recommended dose regimen is 1 mg/kg of body weight administered once every week by intravenous infusion at a controlled speed. For infusion rate see section “Method of administration”.

Special populations

Renal or hepatic impairment
No dose adjustment is necessary for patients with renal or hepatic impairment.
Elderly
No data are available and no relevant use in elderly patients is described.

Paediatric population
No dose adjustment is necessary for the paediatric population.
Method of administration

For intravenous infusion use only.
Instructions on reconstitution of the medicinal product before administration The reconstituted solution should be clear. Do not use if opaque particles are observed or if the solution is discoloured (see section 6.6).

The reconstituted solution of Lamzede should be administered using an infusion set equipped with a pump and an in-line low protein-binding 0.22 µm filter. The infusion duration should be calculated individually considering a maximum infusion rate of 25 mL/hour to control the protein load. The infusion duration should be a minimum of 50 minutes. A slower infusion rate may be prescribed when clinically appropriate according to the physician’s judgment, for example at the beginning of the treatment or in case of previous infusion-related reactions (IRRs).
For the calculation of the infusion rate and the infusion time based on body weight see the table in section 6.6.

The patient should be observed for IRRs for at least one hour after the infusion according to clinical conditions and the physician’s judgment. For further instructions, see section 4.4.