About Wilson Disease

WD is a rare genetic disorder where hepatocytes are not able to process and remove excess copper.1,2

It’s estimated more than 10,000 people in the US have WD.1

Actor portrayal

If not treated, or not adequately treated, excess copper accumulates in the liver and other organs causing damage and can even become life-threatening.2

What WD does in the body1,3

In WD, excess copper is released into the bloodstream and deposits into other tissues and organs, such as the brain, kidneys, and cornea.

WD maintenance therapy options can be challenging4,5

All WD treatments are associated with side effects.5
Dosing timing is another factor for patients. Maintenance therapies for WD are often taken 2, 3, or 4 times per day.5,6

More than 50% of WD patients report not adhering to their  prescribed therapy7

Nonadherence leads to the build up of copper, which then could lead to acute hepatitis, hepatic failure, neuropsychiatric deterioration, and ultimately death.7

Ready for an innovative  WD treatment?

Before CUVRIOR, the last FDA-approved therapy in WD was nearly 30 years ago.8

Explore CUVRIOR

REFERENCES

1. Roberts EA, Schilsky ML; American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089-2111. 2. Wilson Disease. NORD website. Accessed July 10, 2023. https://rarediseases.org/rare-diseases/wilson-disease/ 3. Roberts EA, Schilsky ML. Current and emerging issues in Wilson's disease. N Engl J Med. 2023;389(922-938). 4. Jacquelet E, Poujois A, Pheulpin MC, et al. Adherence to treatment, a challenge even in treatable metabolic rare diseases: a cross sectional study of Wilson’s disease. J Inherit Metab Dis. 2021;44(6):1481-1488. 5. Schilsky ML, Roberts EA, Bronstein JM, et al. A multidisciplinary approach to the diagnosis and management of Wilson disease: executive summary of the 2022 practice guidance on Wilson disease from the American Association for the Study of Liver Diseases. Hepatology. 2023;77(4):1428-1455.  6. SYPRINE-trientine hydrochloride capsule. National Library of Medicine website. Accessed September 5, 2023. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=c34f77a7-996b-4470-b5df-d946a7fe5dbe 7. Miloh T, Graper M, Schilsky M. Advances in rare diseases. Evaluating diagnosis and management gaps in wilson disease: results from a qualitative patient survey. Adv Rare Dis. 4:1. 8. CUVRIOR [package insert]. Chicago, IL: Orphalan SA.

INDICATION

CUVRIOR is a copper chelator indicated for the treatment of adult patients with stable Wilson disease who are de-coppered and tolerant to penicillamine.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

CUVRIOR is contraindicated in patients with hypersensitivity to trientine or to any of the excipients in CUVRIOR.

WARNINGS AND PRECAUTIONS

Potential for Worsening of Clinical Symptoms at Initiation of Therapy, including neurological deterioration, may occur at the beginning of CUVRIOR therapy due to mobilization of excess stores of copper. Adjust the dosage or discontinue therapy if clinical condition worsens. Evaluate serum non-ceruloplasmin copper (NCC) levels or 24-hour urinary copper excretion (UCE) when initiating treatment, after 3 months, and approximately every 6 months thereafter.
Copper Deficiency may develop following treatment with CUVRIOR. Periodic monitoring is required.
Iron Deficiency may develop following treatment with CUVRIOR. If iron deficiency develops, a short course of iron supplementation may be given.
Hypersensitivity Reactions, characterized by rash, have been reported with the use of trientine. Rash was reported in 12% (3/26) of CUVRIOR-treated patients, and one patient discontinued treatment due to rash. If rash or other hypersensitivity reaction occurs, consider discontinuing CUVRIOR.

ADVERSE REACTIONS

The most common adverse reactions (>5%) are abdominal pain, change of bowel habits, rash, alopecia, and mood swings.

DRUG INTERACTIONS

Mineral Supplements (e.g. iron, zinc, calcium, magnesium): Avoid concomitant use. If concomitant use is unavoidable, take CUVRIOR at least 2 hours before or 2 hours after iron and take CUVRIOR at least 1 hour before or 2 hours after other mineral supplements.
Other Drugs for Oral Administration: Take CUVRIOR at least 1 hour apart from any other oral drug.

To report SUSPECTED ADVERSE REACTIONS, contact Orphalan at 1-800-961-8320 or FDA at 1-800-FDA-1088 or  www.fda.gov/medwatch.

Please see full Prescribing Information.