(FDA approved in bold)

• Wilson’s disease (WD) in patients intolerant of penicillamine

• In WD copper accumulates in body tissues (especially the liver and CNS), causing neurological/psychiatric problems and/or liver failure. Trientine binds to (chelates) copper, allowing it to be excreted in the urine

• 6 months or more

• Continue treatment, if tolerated. Most patients remain on drug for the rest of their life but if serum copper returns to normal (< 10 µg/dL) consider changing to elemental zinc or zinc sulfate. Monitor for recurrence of symptoms or changes in urinary copper excretion

• Increase to as much as 2000 mg daily for poor clinical response or if free serum copper is above 20 mcg/dL. For liver failure or truly refractory patients, liver transplantation is curative

• Change to penicillamine if ineffective. A diet low in copper-containing foods, such as nuts, chocolate, liver, and dried fruit, is recommended

• Adequately treated patients should have free serum copper below 10 mcg/dL. Monitor 24-hour urinary copper excretion every 6–12 months (should be between 0.5–1 mg)