About Wilson's Disease

What Is Wilson’s Disease?

Wilson’s disease (WD) is a genetic disorder in which there is decreased excretion (elimination of waste matter) of copper into bile; this results in the buildup of copper to toxic levels in the liver, brain, and/or other organs.1 Copper enters our bodies through the food we eat; this is normal and necessary because the body needs copper to ensure our cells work properly and to make proteins, nerve cells, and the pigments that color our skin, eyes, and hair.1,2 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 the 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
  • The 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

INDICATION

Cuprimine®  (Penicillamine) Capsules are used to treat Wilson's disease (a disease where there is too much copper in the body), cystinuria (a disease where an excess amount of certain proteins are in the urine) and in patients with severe, active rheumatoid arthritis who have not had a response to other therapy. Not enough evidence is available to see an effect on treatment of ankylosing spondylitis.

IMPORTANT SAFETY INFORMATION

WARNING: You should be under the close supervision of your doctor when you are taking Cuprimine. Report any side effects promptly to your doctor.
  • Do not take Cuprimine if you are pregnant unless you are taking Cuprimine to treat Wilson’s disease (too much copper in the body) or cystinuria (too much protein in the urine). Mothers on therapy with penicillamine should not nurse their infants.
  • Cuprimine can cause serious blood disorders, and some can be fatal. If you have had aplastic anemia (anemia due to lack of all blood cells) or agranulocytosis (lack of certain white blood cells) and it was related to taking Cuprimine, you should not take it again.
  • Cuprimine can cause kidney damage and should not be used to treat rheumatoid arthritis if you have a history of kidney disease. If you take Cuprimine  to treat cystinuria, routine analysis of your urine may be necessary and you should have an x-ray every year to check for kidney stones.
  • Cuprimine can be associated with fatalities due to other diseases such as Goodpasture’s syndrome (an immune disease that attacks the lungs and kidneys) and myasthenia gravis (an immune disease affecting the muscles). Your doctor may order blood analysis on a regular basis.
  • Cuprimine can affect how your liver works. Tests to determine how your liver is working should be done regularly.
  • Tell your doctor right away if you experience: blood in your urine, unexplained cough or wheezing, coughing up blood, shortness of breath, muscle weakness, drooping eyelids, double vision, watery blisters on the skin or other rash, fever, joint pain, swollen lymph nodes, mouth ulcers, or diminished taste.
  • Cuprimine is a drug that has many side effects, and some can be fatal. Other side effects that can occur include serious lung problems, nervous system symptoms, diseases of the skin and mucous membranes known as pemphigus, allergic reactions (including a condition known as drug fever as well as skin rashes), mouth ulcers, and loss of taste. Talk to your doctor if you experience side effects and also about possible side effects that could occur. Ask your doctor or pharmacist for product labeling written for professionals for a full list of potential adverse reactions.
  • Tell your doctor about all other medicines (prescription and over-the-counter, including vitamins and herbal supplements) that you are taking. Some medicines (such as gold therapy, antimalarial or cancer drugs, oxyphenbutazone or phenylbutazone) should not be used with Cuprimine  because they also may cause serious liver and kidney side effects.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.
Please click here to see full Prescribing Information for Cuprimine  capsules.

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INDICATION

Cuprimine® (Penicillamine) Capsules are used to treat Wilson's disease (a disease where there is too much copper in the body), cystinuria (a disease where an excess amount of certain proteins are in the urine) and in patients with severe, active rheumatoid arthritis who have not had a response to other therapy. Not enough evidence is available to see an effect on treatment of ankylosing spondylitis.

IMPORTANT SAFETY INFORMATION

WARNING: You should be under the close supervision of your doctor when you are taking Cuprimine. Report any side effects promptly to your doctor.

  • Do not take Cuprimine if you are pregnant unless you are taking Cuprimine to treat Wilson’s disease (too much copper in the body) or cystinuria (too much protein in the urine). Mothers on therapy with penicillamine should not nurse their infants.
  • Cuprimine can cause serious blood disorders, and some can be fatal. If you have had aplastic anemia (anemia due to lack of all blood cells) or agranulocytosis (lack of certain white blood cells) and it was related to taking Cuprimine, you should not take it again.
  • Cuprimine can cause kidney damage and should not be used to treat rheumatoid arthritis if you have a history of kidney disease. If you take Cuprimine to treat cystinuria, routine analysis of your urine may be necessary and you should have an x-ray every year to check for kidney stones.
  • Cuprimine can be associated with fatalities due to other diseases such as Goodpasture’s syndrome (an immune disease that attacks the lungs and kidneys) and myasthenia gravis (an immune disease affecting the muscles). Your doctor may order blood analysis on a regular basis.
  • Cuprimine can affect how your liver works. Tests to determine how your liver is working should be done regularly.
  • Tell your doctor right away if you experience: blood in your urine, unexplained cough or wheezing, coughing up blood, shortness of breath, muscle weakness, drooping eyelids, double vision, watery blisters on the skin or other rash, fever, joint pain, swollen lymph nodes, mouth ulcers, or diminished taste.
  • Cuprimine is a drug that has many side effects, and some can be fatal. Other side effects that can occur include serious lung problems, nervous system symptoms, diseases of the skin and mucous membranes known as pemphigus, allergic reactions (including a condition known as drug fever as well as skin rashes), mouth ulcers, and loss of taste. Talk to your doctor if you experience side effects and also about possible side effects that could occur. Ask your doctor or pharmacist for product labeling written for professionals for a full list of potential adverse reactions.
  • Tell your doctor about all other medicines (prescription and over-the-counter, including vitamins and herbal supplements) that you are taking. Some medicines (such as gold therapy, antimalarial or cancer drugs, oxyphenbutazone or phenylbutazone) should not be used with Cuprimine because they also may cause serious liver and kidney side effects.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.
Please click here to see full Prescribing Information for Cuprimine capsules.
 

INDICATION

Cuprimine® (Penicillamine) Capsules are used to treat Wilson's disease (a disease where there is too much copper in the body), cystinuria (a disease where an excess amount of certain proteins are in the urine) and in patients with severe, active rheumatoid arthritis who have not had a response to other therapy. Not enough evidence is available to see an effect on treatment of ankylosing spondylitis.

IMPORTANT SAFETY INFORMATION

WARNING: You should be under the close supervision of your doctor when you are taking Cuprimine. Report any side effects promptly to your doctor.

  • Do not take Cuprimine if you are pregnant unless you are taking Cuprimine to treat Wilson’s disease (too much copper in the body) or cystinuria (too much protein in the urine). Mothers on therapy with penicillamine should not nurse their infants.
  • Cuprimine can cause serious blood disorders, and some can be fatal. If you have had aplastic anemia (anemia due to lack of all blood cells) or agranulocytosis (lack of certain white blood cells) and it was related to taking Cuprimine, you should not take it again.
  • Cuprimine can cause kidney damage and should not be used to treat rheumatoid arthritis if you have a history of kidney disease. If you take Cuprimine to treat cystinuria, routine analysis of your urine may be necessary and you should have an x-ray every year to check for kidney stones.
  • Cuprimine can be associated with fatalities due to other diseases such as Goodpasture’s syndrome (an immune disease that attacks the lungs and kidneys) and myasthenia gravis (an immune disease affecting the muscles). Your doctor may order blood analysis on a regular basis.
  • Cuprimine can affect how your liver works. Tests to determine how your liver is working should be done regularly.
  • Tell your doctor right away if you experience: blood in your urine, unexplained cough or wheezing, coughing up blood, shortness of breath, muscle weakness, drooping eyelids, double vision, watery blisters on the skin or other rash, fever, joint pain, swollen lymph nodes, mouth ulcers, or diminished taste.
  • Cuprimine is a drug that has many side effects, and some can be fatal. Other side effects that can occur include serious lung problems, nervous system symptoms, diseases of the skin and mucous membranes known as pemphigus, allergic reactions (including a condition known as drug fever as well as skin rashes), mouth ulcers, and loss of taste. Talk to your doctor if you experience side effects and also about possible side effects that could occur. Ask your doctor or pharmacist for product labeling written for professionals for a full list of potential adverse reactions.
  • Tell your doctor about all other medicines (prescription and over-the-counter, including vitamins and herbal supplements) that you are taking. Some medicines (such as gold therapy, antimalarial or cancer drugs, oxyphenbutazone or phenylbutazone) should not be used with Cuprimine because they also may cause serious liver and kidney side effects.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.
Please click here to see full Prescribing Information for Cuprimine capsules.