General Information 

Cuvrior (trientine tetrahydrochloride) is a copper chelator. 

Cuvrior is specifically indicated for the treatment of adult patients with stable Wilson’s disease who are de-coppered and tolerant to penicillamine.

Cuvrior is supplied as tablets for oral administration. The recommended starting total daily dosage of Cuvrior in adult patients is 300 mg up to 3,000 mg taken orally in divided doses (two times daily).

Total daily dosage of Cuvrior should not exceed 3,000 mg. If the number of Cuvrior tablets prescribed per day cannot be equally divided among doses, then divide total daily dosage such that the higher number of tablets is taken with the first daily dose.

Take Cuvrior on an empty stomach. Swallow tablets without crushing, chewing, or dissolving tablets.

See full prescribing information for recommended conversion table when switching from penicillamine to Cuvrior.

Cuvrior is not substitutable on a milligram-per-milligram basis with other trientine products. See full prescribing information for additional information on switching from other trientine products.

Mechanism of Action

Cuvrior (trientine tetrahydrochloride), a copper chelator, eliminates absorbed copper from the body by forming a stable complex that is then eliminated through urinary excretion. Trientine also chelates copper in the intestinal tract, reducing copper absorption.

Side Effects

Adverse effects associated with Cuvrior may include, but are not limited to, the following:

• abdominal pain
• change of bowel habits
• rash
• alopecia
• mood swings

Clinical Trial Results

FDA approval of Cuvrior was based on the results of the global phase 3 trial, CHELATE. The CHELATE trial met its primary efficacy endpoint by demonstrating that trientine tetrahydrochloride was non-inferior to d-Penicillamine as measured by copper speciation evaluation of non-ceruloplasmin copper (NCC). The pre-specified composite endpoint of non-ceruloplasmin copper (NCC) and 24-hour urinary copper excretion (UCE), was achieved by 50% of patients treated with trientine tetrahydrochloride versus 24% of patients treated with d-Penicillamine.