Posology : מינונים

4.2    Posology and method of administration

Treatment should be supervised by a physician experienced in the management of patients with MPS IVA or other inherited metabolic diseases. Administration of Vimizim should be carried out by an appropriately trained healthcare professional with the ability to manage medical emergencies.
Home administration under the supervision of an appropriately trained healthcare professional may be considered for patients who are tolerating their infusions well.

Posology

The recommended dose of elosulfase alfa is 2 mg/kg of body weight administered once a week. The total volume of the infusion should be delivered over approximately 4 hours (see Table 1).

Because of the potential for hypersensitivity reactions with elosulfase alfa, patients should receive antihistamines with or without antipyretics 30 to 60 minutes prior to start of infusion (see section 4.4).

Special populations

Elderly patients (≥ 65 years old)
The safety and efficacy of Vimizim in patients older than 65 years has not been established, and no alternative treatment regimen can be recommended in these patients. It is not known whether elderly patients respond differently from younger patients.

Paediatric population
The posology in the paediatric population is the same as in adults. Currently available data are described in section 4.8 and section 5.1.

Method of administration

For intravenous infusion only.

Patients weighing less than 25 kg should receive a total volume of 100 ml. When diluted in 100 ml, the initial infusion rate should be 3 ml/hr. The infusion rate may be increased as tolerated, every 15 minutes as follows: first increase the rate to 6 ml/hr, then increase the rate every 15 minutes by 6 ml/hr increments until a maximum rate of 36 ml/hr is reached.

Patients weighing 25 kg or more should receive a total volume of 250 ml. When diluted in 250 ml, the initial infusion rate should be 6 ml/hr. The infusion rate may be increased as tolerated, every 15 minutes as follows: first increase the rate to 12 ml/hr, then increase the rate every 15 minutes by 12 ml/hr increments until a maximum rate of 72 ml/hr is reached.

Table 1: Recommended infusion volumes and rates*
Patient          Total          Step 1    Step 2  Step 3     Step 4    Step 5          Step 6       Step 7 weight         infusion         Initial   15-30    30-45     45-60     60-75           75-90         90+ (kg)           volume        infusion   minutes minutes minutes minutes              minutes      minutes (ml)        rate 0-15   (ml/hr) (ml/hr) (ml/hr)      (ml/hr)         (ml/hr)      (ml/hr) minutes
(ml/hr)
< 25         100             3          6      12         18        24              30          36 ≥ 25         250             6         12      24         36        48              60          72 *
Infusion rate may be increased as tolerated by patient.

For instructions for dilution of the medicinal product prior to administration, see section 6.6.