What is Wilson Disease?

Wilson Disease is a genetic disorder characterized by gradual accumulation of copper in the body, which affected individuals are unable to remove effectively because of a faulty copper transporter mechanism in the liver. Without appropriate treatment, copper builds up in the tissues and organs, including the liver and brain. 1

Although the body needs copper for it to work correctly, a normal diet contains more copper than it needs. In people without Wilson Disease, the liver processes the necessary amount and excretes the excess copper into the bile, which is then removed from the body in feces. 1
Approximately 1 in every 30,000 of people in the world have a genetic mutation that stops their liver cells from being able to process and remove excess copper. The excess copper accumulates in the liver and in other organs in the body and damages them– this is what causes Wilson Disease. 1

Due to copper deposits in different parts of the body, Wilson Disease leads to a wide range of signs and symptoms meaning that the disease affects each person differently. Some of the main signs and symptoms are outlined below: 1
Hepatic Scarring of the liver (cirrhosis) which prevents normal functioning. Liver failure can occur slowly over years (chronic), or suddenly (acute).
Neurologic Tremors, speech difficulties, migraine headaches, insomnia, and even seizures can occur.
Psychiatric Psychiatric symptoms might include depression and personality changes.
Other The kidneys and bones can also be affected in Wilson Disease, potentially causing kidney stones or arthritis, respectively. Dark rings around the iris (colored part) of the eye or cataracts may occur in some people.
Please note, this is not a comprehensive list of symptoms associated with Wilson Disease.
A low copper diet and regularly taking chelating medicines (such as CUVRIOR) can help to keep copper in the body at acceptable levels1.
If you would like further information on Wilson Disease, please visit this link.


  1. CUVRIORTM (trientine tetrahydrochloride) Prescribing Information.

Indication & Important Safety Information

CUVRIOR is a prescription medicine for the treatment of adult patients with stable Wilson Disease who are de-coppered and able to take penicillamine. CUVRIOR is a chelating medicine which binds copper, helping your organs release copper into your bloodstream which is then removed through your urine.
Important Safety Information
CUVRIOR is a copper chelator indicated for the treatment of adult patients with stable Wilson Disease who are de-coppered and tolerant to penicillamine.
  • Do not use CUVRIOR if you are allergic to trientine or to any of the ingredients in CUVRIOR.
  • Your Wilson’s Disease symptoms could get worse when you start treatment. This could happen because too much copper is removed from the body in a short period of time. Your doctor may need to reduce your dose or stop CUVRIOR treatment.
  • Copper deficiency may develop following treatment with CUVRIOR. Your doctor will do tests to monitor your urine and blood for copper.
  • Iron deficiency may develop while taking CUVRIOR. If this happens, your doctor may tell you to take iron supplements for a limited time.
  • Allergic reactions, such as a rash, have been reported with trientine, the active ingredient in CUVRIOR. If a rash or other allergic reaction occurs, stop taking CUVRIOR and get emergency medical help.
  • The most common side effects are pain in the abdomen, change in bowel habits, rash, hair loss (alopecia), and mood swings.

Take CUVRIOR at least 1 hour apart from any other oral medicine. Tell your doctor about all the medicines you're taking, including over-the-counter medicines, vitamins, and herbal supplements. Avoid taking supplements when taking CUVRIOR. Taking CUVRIOR with mineral supplements (e.g., iron, zinc, calcium, magnesium) can reduce the effectiveness of CUVRIOR. If iron supplementation is necessary, take CUVRIOR at least 2 hours before or 2 hours after taking iron; for other mineral supplements (e.g., zinc, calcium, magnesium), take CUVRIOR at least 1 hour before or 2 hours after. Do not start or stop any medicines while taking CUVRIOR without talking to your doctor.

These are not all the possible side effects of CUVRIOR. You are encouraged to report negative side effects of prescription drugs. Contact Orphalan at 1-800-961-8320 or AdverseEventReporting@orphalan.com or FDA at 1-800-FDA-1088 or www.fda.gov/​medwatch.

Please see full Prescribing Information.