Posology : מינונים

4.2    Posology and method of administration

KANUMA treatment should be supervised by a healthcare professional experienced in the management of patients with LAL deficiency, other metabolic disorders, or chronic liver diseases.
KANUMA should be administered by a trained healthcare professional who can manage medical emergencies.

Posology

It is important to initiate treatment as early as possible after diagnosis of LAL deficiency.
For instructions on the preventive measures and monitoring of hypersensitivity reactions, see section 4.4. Following the occurrence of a hypersensitivity reaction, appropriate pre-treatment should be considered according to the standard of care (see section 4.4).



Infants (< 6 months of age)
The recommended starting dose in infants (< 6 months of age) presenting with rapidly progressive LAL deficiency is 1 mg/kg administered as an intravenous infusion once weekly.
Dose escalation to 3 mg/kg once weekly should be considered based on clinical response.

Children and adults
The recommended dose in children and adults who do not present with rapidly progressive LAL deficiency prior to 6 months of age is 1 mg/kg administered as an intravenous infusion once every other week.

Special populations

Renal or hepatic impairment
No dosing adjustment is recommended in patients with renal or hepatic impairment based on current knowledge of the pharmacokinetics and pharmacodynamics of sebelipase alfa (See section 5.2).

Paediatric population
Administration of KANUMA to infants with confirmed multiple-organ failure should be at the discretion of the treating physician.

Overweight patients
The safety and efficacy of KANUMA in overweight patients have not been thoroughly evaluated and therefore no alternative dose regimens can be recommended for these patients at this time 
Elderly population (≥ 65 years old)
The safety and efficacy of KANUMA in patients older than 65 years have not been evaluated and no alternative dose regimens can be recommended for these patients. See section 5.1.

Method of administration

KANUMA is for intravenous use only.
The total volume of the infusion should be administered over approximately 2 hours. A 1- hour infusion may be considered after patient tolerability is established. The infusion period may be extended in the event of dose escalation.

KANUMA should be administered through a 0.2 μm filter (see section 6.6).

For instructions on dilution of the medicinal product before administration, see section 6.6.