Cuvrior

A Makeover for an Old Drug
By Rhonda Rowland

Wilson disease (WD) was always fatal until  Dr. John Walshe, an English physician, discovered penicillamine in 1956.  While WD was no longer a death sentence, with time, complications with the drug started to appear.

Treatment of Wilson disease

Wilson disease is a very treatable condition. With proper therapy, disease progress can be halted and oftentimes symptoms can be improved. Treatment is aimed at removing excess accumulated copper and preventing its reaccumulation. Treatment for Wilson disease is a lifelong process. Patients may become progressively sicker from day to day, so immediate treatment can be critical. Treatment delays may cause irreversible damage.

Chelation therapy drugs approved for treating Wilson disease include penicillamine (Cuprimine® and Depen®), trientine dihydrocholoride (Syprine®) and trientine tetrahydrochoride (Cuvrior ™). These drugs act by chelation or binding of copper, causing its increased urinary excretion.

Zinc acetate is approved for treating Wilson disease under the brand names (Galzin™) in the U.S. and (Wilzin®) in Europe. Zinc acts by inducing metallothionein and blocking the absorption of copper in the intestinal tract. This action both depletes accumulated copper and prevents its reaccumulation. Zinc is used primarily for maintenance treatment and to treat asymptomatic patients and may also be used in conjunction with chelators.  Its effectiveness has been shown by more than 40 years of considerable experience in the USA and Europe. A major advantage of zinc therapy is its lack of side effects.

Patient assistance programs are available for Wilson disease patients taking Cuvrior, Cuprimine, Syprine and Galzin who need more information or help in getting prescriptions for these medications, or who need assistance in affording the medication. Details can be found here: https://wilsondisease.org/living-with-wilson-disease/patient-assistance/

Patients with severe hepatitis or liver failure may require liver transplant. Patients being investigated or treated for Wilson disease should be cared for by specialists in Wilson disease or by specialists in consultation with their primary physicians. Stopping treatment completely will result in death, sometimes as quickly as within three months. Decreasing dosage of medications also can result in unnecessary disease progression.

Taking your medicine as prescribed is extremely critical to the success of Wilson disease treatment. One of the advantages of having WD is that it is VERY treatable with effective, safe medications leading to a normal life expectancy . . . IF . . . you faithfully take your medications, as prescribed, LIFELONG. There are varying reasons for non-adherence in patients with WD and your physician has probably heard them all. Adherence is especially difficult in patients who were asymptomatic as diagnosis. These patients often do not see a cause and effect relationship.

New Version of room temperature trientine – CUVRIOR™

In May 2022 the FDA approved the first new drug for Wilson disease in 30 years – CUVRIOR (trientine tetrahydrochloride), made by a French company Orphalan. The European version of this drug called CUPRIOR has been on the market since 2019 with more than 1,100 patients treated. In a head-to-head study comparing it to penicillamine in stable patients, Cuvrior was shown to be non-inferior when measured by a novel diagnostic test of non-ceruloplasmin-bound (NCC) at 56 weeks. A critical advantage of this drug is that it requires no refrigeration.

Orphalan has a number of patient support resources for US patients prescribed CUVRIOR, such as co-pay assistance and case managers to help patients navigate insurance.

Find information about the CUVRIOR NAVIGATOR patient assistance program here: https://www.cuvrior.com/taking-cuvrior/patient-support-program/

Treatments for Canadians

There is now a generic Trientine available in Canada through the SAP. Here is a link to the form.

Trientine is approved for distribution in Canada. It is on the market and the patient or physician should check with their Pharmacy to see if it is available in their area.

Two companies are approved in Canada that have Trientine. We have a brochure for Waymade that can be downloaded.
Waymade din 02515067 approved for sale July 5, 2021 250 MG
Marcan din 02504855 approved for sale Nov 11, 2021 250 MG.

Low Cost Generic Chelator Options

Cost Plus Drug Company is providing generic Syprine (the generic is called trientine) and generic Cuprimine (the generic is called penicillamine) at a low cost.  Dr. Reddy’s Laboratories will manufacture and supply the medications to Cost Plus Drug Company.

To find out more about this option for buying trientine or penicillamine, please go to https://www.costplusdrugs.com/.  Also see the Cost Plus Drug Company FAQ here   https://costplusdrugs.com/faq/.

To get a prescription filled for either of these medications, please go to https://costplusdrugs.com/medications/categories/wilson-disease/.  The cost of trientine can be found here https://costplusdrugs.com/medications/trientine-hcl-250mg-capsule/ . The cost of penicillamine can be found here https://costplusdrugs.com/medications/penicillamine-250mg-capsule/

Generic Zinc Options

Gluzin™ Pharmaceutical Grade Zinc’s active ingredient is Zinc Gluconate (also called zincum gluconium) which is zinc salt that provides a good source of Zinc mineral. There are many types of zinc salt, but Zinc Gluconate is known to be more soluble than other zinc salts, and friendlier to the stomach.

Gluzin™ has been formulated with minimum fillers to help reduce allergic reactions. For more information on Gluzin™, please go to www.extremeV.com or email us at info@extremeV.com.  For information on how to receive a 15% discount for Wilson Disease Association members go to: www.gluzin.com or to order go here.

Donations

Your support allows the WDA to maintain this website, produce educational materials, support research, and hold meetings for people living with WD, their families, and the healthcare community.

Membership

As a member, you have the opportunity to communicate your concerns, share your experiences, learn about the most recent advances in Wilson disease treatment and research, and contribute to important decisions that need to be made so the WDA can be a strong patient advocacy group

WILSON DISEASE ASSOCIATION

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