About Wilson's Disease

What Is Wilson's Disease?

Wilson’s disease (WD) is a genetic disease that prevents the body from metabolizing (processing) copper, which then builds up to toxic levels in the liver, brain, and/or other organs.1 Copper enters our bodies through the food we eat and the water we drink. This is normal and necessary because the body needs copper to ensure that our cells work properly and make proteins, nerve cells, and the pigments that color our skin, eyes, and hair. Copper plays a particularly important role in the development and maintenance of the central nervous system.2 However, the average diet contains more copper than the body needs. In most people, the liver processes the necessary amount and excretes the leftover copper into bile, which passes out of the body in feces.1
  • About 1 out of every 30,000 people worldwide has a genetic mutation that causes a breakdown in the body’s normal mechanism for removing excess copper1
  • More than 500 different mutations can cause WD, but all of them affect a gene called ATP7B, which can be found on chromosome 13 in human cells1,3
  • This gene tells the body how to make a protein of the same name that is responsible for removing copper from the body1
  • The mutations that cause WD are autosomal recessive, which means that a person with WD will have inherited 2 copies of a WD mutation, 1 from each parent4
  • Parents and siblings of individuals with WD may or may not have the disease, depending on whether they also carry 2 copies of the mutation
  • Someone with only 1 copy of a WD mutation will metabolize copper normally, but there is a risk of passing the condition onto one’s children4
WD is fatal if not treated, so getting a correct diagnosis is essential.1 Once it’s diagnosed, WD can be effectively treated with a low copper diet and medicines called chelating (pronounced “key-lating”) agents that bind to copper and carry it out of the body.1,5
INDICATIONS AND USAGE
Syprine® (trientine hydrochloride) is used to treat Wilson's disease in patients who cannot take the medication known as penicillamine. Wilson's disease is a condition where the body stores too much copper. Syprine is not recommended to treat cystinuria (a condition where a protein known as cystine is excreted into the urine), rheumatoid arthritis, or a disease affecting the bile ducts in the liver known as biliary cirrhosis.
IMPORTANT SAFETY INFORMATION
  • Do not take Syprine if you are allergic to it or any parts of the formulation.
  • You should remain under regular medical supervision the entire time you are taking Syprine. Your doctor should regularly check to see if you have iron deficiency anemia. This is particularly important for women.
  • Take Syprine on an empty stomach, at least one hour before a meal or two hours after a meal and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed. For the first month of treatment, take your temperature every night, and report any symptom such as fever or skin rash to your doctor.
  • Tell your doctor if you are pregnant, plan to become pregnant, or are nursing.
  • The following adverse reactions have been reported from a clinical study: iron deficiency and a condition affecting the immune system known as systemic lupus erythematosus. In addition, the following adverse reactions have been reported in marketed use: abnormal or uncontrolled muscle contractions, muscle spasm and an immune disease affecting muscles known as myasthenia gravis.
  • Do not take mineral supplements because they may block the absorption of Syprine.
Please click here to see full Prescribing Information for Syprine Capsules.
You are encouraged to report negative side effects of prescription drugs to FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.
References
  1. Roberts EA, Schilsky ML. AASLD Practice Guidelines. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089-2111.
  2. Desai V, Kaler SG. Role of copper in human neurological disorders. Am J Clin Nutr. 2008;88(suppl):855S-858S.
  3. Rodriguez-Castro KI, Hevia-Urrutia FJ, Sturniolo GC. Wilson’s disease: a review of what we have learned. World J Hepatol. 2015;7(29):2859-2870.
  4. National Institute of Diabetes and Digestive and Kidney Diseases. Wilson Disease. http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/wilson-disease/Pages/facts.aspx#sec10. Accessed May 25, 2016.
  5. Wilson Disease Association. Treatment of Wilson Disease. http://www.wilsonsdisease.org/wilson-disease/wilsondisease-treatment.php. Accessed July 1, 2016.

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Indications and Usage

Syprine® (trientine hydrochloride) is used to treat Wilson's disease in patients who cannot take the medication known as penicillamine. Wilson's disease is a condition where the body stores too much copper. Syprine is not recommended to treat cystinuria (a condition where a protein known as cystine is excreted into the urine), rheumatoid arthritis, or a disease affecting the bile ducts in the liver known as biliary cirrhosis.

Important Safety Information

  • Do not take Syprine if you are allergic to it or any parts of the formulation.
  • You should remain under regular medical supervision the entire time you are taking Syprine. Your doctor should regularly check to see if you have iron deficiency anemia. This is particularly important for women.
  • Take Syprine on an empty stomach, at least one hour before a meal or two hours after a meal and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed. For the first month of treatment, take your temperature every night, and report any symptom such as fever or skin rash to your doctor.
  • Tell your doctor if you are pregnant, plan to become pregnant, or are nursing.
  • The following adverse reactions have been reported from a clinical study: iron deficiency and a condition affecting the immune system known as systemic lupus erythematosus. In addition, the following adverse reactions have been reported in marketed use: abnormal or uncontrolled muscle contractions, muscle spasm and an immune disease affecting muscles known as myasthenia gravis.
  • Do not take mineral supplements because they may block the absorption of Syprine.
Please click here to see full Prescribing Information for Syprine Capsules.
 

INDICATIONS AND USAGE

Syprine® (trientine hydrochloride) is used to treat Wilson's disease in patients who cannot take the medication known as penicillamine. Wilson's disease is a condition where the body stores too much copper. SYPRINE® is not recommended to treat cystinuria (a condition where a protein known as cystine is excreted into the urine), rheumatoid arthritis, or a disease affecting the bile ducts in the liver known as biliary cirrhosis.

IMPORTANT SAFETY INFORMATION

  • Do not take Syprine if you are allergic to it or any parts of the formulation.
  • You should remain under regular medical supervision the entire time you are taking Syprine. Your doctor should regularly check to see if you have iron deficiency anemia. This is particularly important for women.
  • Take Syprine on an empty stomach, at least one hour before a meal or two hours after a meal and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed. For the first month of treatment, take your temperature every night, and report any symptom such as fever or skin rash to your doctor.
  • Tell your doctor if you are pregnant, plan to become pregnant, or are nursing.
  • The following adverse reactions have been reported from a clinical study: iron deficiency and a condition affecting the immune system known as systemic lupus erythematosus. In addition, the following adverse reactions have been reported in marketed use: abnormal or uncontrolled muscle contractions, muscle spasm and an immune disease affecting muscles known as myasthenia gravis.
  • Do not take mineral supplements because they may block the absorption of Syprine.
Please click here to see full Prescribing Information for Syprine Capsules.