Cuvrior (trientine tetrahydrochloride) is now available in the US through the PANTHERx® Rare pharmacy for the treatment of Wilson disease (WD), according to a press release from the pharmacy.

“We are pleased to add Cuvrior to the list of PANTHERx medications. The PANTHERx team works hard to make a positive difference in the lives of our patients who are dealing with devastating and debilitating diseases and Cuvrior is yet another way we can make an impact,” said Rob Snyder, president of PANTHERx Rare.

Read more about Cuvrior for WD

Cuvrior is an oral formulation developed by Orphalan and is US Food and Drug Administration (FDA)-approved for the treatment of WD in adults who are decoppered and able to take penicillamine. It contains the active ingredient trientine which works by chelating copper that has already been absorbed by the body and forming a stable compound that can be excreted in the urine. The treatment also chelates copper in the intestinal tract, preventing it from being absorbed.

PANTHERx Rare is a pharmacy focused on delivering access to treatments for rare diseases. Recently, PANTHERx earned Special Pharmacy of the Year by the National Association of Specialty Pharmacy and Accredited Distinction in Rare Diseases and Orphan Drugs from the Accreditation Commission for Health Care.

Cuvrior was approved last year by the FDA following the results of a phase 3 clinical trial called CHELATE, which showed that it was noninferior to penicillamine as measured by nonceruloplasmin copper (NCC).

The study included 53 adults with WD who were previously taking penicillamine. After a 12-week baseline of penicillamine, some of the patients were randomly allocated to switch to Cuvrior for 24 weeks while the rest continued on penicillamine. The prespecified composite endpoint of NCC and 24-hour urinary copper excretion were met by 50% of patients receiving Cuvrior and 24% of patients receiving penicillamine.

Cuvrior was previously commercialized in Europe under the name Cuprior by Orphalan.


PANTHERx Rare announces release of Cuvrior (trientine tetrahydrochloride) to treat Wilson disease. News release. PANTHERx Rare; April 19, 2023.

Orphalan announces FDA approval of Cuvrior™ for the treatment of adult patients with stable Wilson’s disease who are decoppered and tolerant to penicillamine. News release. Orphalan; May 2, 2022.