Posology : מינונים

4.2    Posology and method of administration

Treatment should only be initiated by specialist physicians with experience in the management of Wilson’s disease.

Posology

The starting dose would usually correspond to the lowest recommended dose and the dose should subsequently be adapted according to the patient’s clinical response (see section 4.4).

The recommended dose is 800-1,600 mg (4-8 capsules) daily in 2 to 4 divided doses.

The recommended doses of Cufence are expressed as mg of trientine base (i.e. not in mg of the trientine dihydrochloride salt) (see section 4.4).

Special populations
Elderly
There is insufficient clinical information available for Cufence to determine whether there exist differences in responses between the elderly and younger patients. In general, dose selection should be cautious, usually starting at the low end of the dosing range as recommended for adults, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy.

Renal impairment
There is limited information in patients with renal impairment. Therefore, the recommended dose in patients with renal impairment is the same as for adults. For specific precautions see section 4.4.

Hepatic impairment

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There is limited information in patients with hepatic impairment. Therefore the recommended dose in patients with hepatic impairment is the same as for adults. For specific precautions see section 4.4.

Patients primarily presenting hepatic symptoms
The recommended dose in patients primarily presenting hepatic symptoms is the same as the recommended adult dose. It is advised, however, to monitor patients presenting with hepatic symptoms every two to three weeks after initiation of treatment with Cufence.

Patients primarily presenting neurological symptoms
Dose recommendations are the same as for adults. However, up titration should be done with moderation and consideration, and adapted according to the patient’s clinical response such as worsening of tremor as patients could be at risk of neurological deterioration at initiation of treatment (see section 4.4). It is further advised to monitor patients presenting with neurological symptoms every one to two weeks after initiation of treatment with Cufence until target dose is reached.

Paediatric population
The dose is lower than for adults and depends on age and body weight. The dose should be adjusted according to clinical response; 400 – 1,000 mg (2-5 capsules) have been used at initiation of therapy.
(see section 4.4).

Children < 5 years
The safety and efficacy of Cufence in children aged 0 to 5 years have not yet been established. No data are available.

Method of administration
For oral use.

Capsules should be swallowed whole with water.
It is important that Cufence is given on an empty stomach, at least one hour before meals or two hours after meals, and at least one hour apart from any other medicinal product, food or milk (see section 4.5).