Trientine

Wilson's disease. Treatment should only be initiated by specialist physicians experienced in the management of Wilson's disease. 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. Doses are expressed as mg of trientine BASE (not as mg of the trientine dihydrochloride salt) — caution when switching between trientine formulations, as different salts have different base content and bioavailability and dose adjustment may be required. Capsules should be swallowed whole with water 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. Elderly: insufficient clinical information to determine whether responses differ; dose selection should be cautious, usually starting at the low end of the adult dosing range. Patients presenting primarily with hepatic symptoms: same dose as adults, but monitor every two to three weeks after initiation. Patients presenting primarily with neurological symptoms: same dose recommendations as adults, but up-titrate with moderation — treatment can be initiated at the minimum available dose and adapted according to clinical response (e.g. worsening tremor), as patients may be at risk of neurological deterioration at initiation; monitor every one to two weeks after initiation until the target dose is reached. Monitoring: regular medical supervision at least twice a year, more frequently during the initial phase, during disease progression, and when doses are adjusted; overtreatment carries a risk of copper deficiency (especially harmful in children and pregnant women). PAEDIATRIC (non-per-kg, so not held in paedDose): the SPC states the dose is lower than for adults and depends on age and body weight, adjusted according to clinical response — 400–1,000 mg (2–5 capsules) have been used at initiation of therapy. Safety and efficacy in children aged 0 to 5 years have not been established (no data available). Verify all paediatric dosing against a children's formulary. Source: eMC SPC for Trientine 200 mg hard capsules (§4.2). NOTE: the US label (trientine hydrochloride capsules, Par Health USA) gives different figures — initial 750 to 1,250 mg/day for adults, increased to a maximum of 2,000 mg/day — but these are expressed as the hydrochloride salt, not base; do not merge the two.