What Are My Treatment Options?

The medications must be taken every day for the rest of your life—in a study of 11 WD patients who stopped taking their copper-removal medication, 8 died of liver disease within 2 and a half years.2 In the same study, another group of 13 patients had to stop taking 1 copper-removal medication, but started another. In this case, all of the patients except 1 who died in a car accident were still alive at the time the study report was published, 2–15 years after they stopped taking the first medicine.2
Medications approved for the treatment of WD include chelating agents and zinc. Chelating agents such as CUPRIMINE® (Penicillamine) Capsules remove copper from the organs where it has built up and pull it into the bloodstream. The kidneys then filter the copper out of the blood and into the urine. Zinc blocks absorption of copper from food in the digestive tract, but it does not help remove excess copper if it has already been absorbed.3
Treatment thus occurs in 2 phases:

Diet and Nutrition

Food choices. Copper enters our bodies through the food we eat and the water we drink, which is a good thing because our bodies need copper to function.4 However, when you have WD and your body can’t rid itself of excess copper, it’s important to limit your copper intake by avoiding foods with a high copper content, especially for the first year after diagnosis. Foods you should avoid (or limit) include5:
  • Shellfish
  • Nuts
  • Chocolate
  • Mushrooms
  • Organ meat (eg, liver paté, tripe, sweetbreads)

Water

  • People with WD should avoid using copper containers for cooking, serving, or storing food3
  • If your tap water runs through copper pipes or comes from a well, be sure to run the water for a while before using any, as this should reduce any copper residues to acceptable levels3

Vitamins and Dietary Supplements

  • Talk to your doctor before taking a multivitamin, and if he or she approves, ask your pharmacist to recommend one that does not contain copper
  • If you are a woman who is pregnant or is planning to become pregnant, you should ask your obstetrician to consult with your WD specialist before prescribing prenatal vitamins
  • Many prenatal vitamins contain a lot of copper and it’s important to find a brand that does not6
You and your doctor should also discuss any other dietary supplements or herbal preparations you are taking, or would like to take, to make sure these will not interact with your medications or worsen problems in your liver.6
It is essential to follow your healthcare professional’s advice when it comes to any potential sources of copper or drug interactions that could be harmful to you.

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.
References
  1. 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.
  2. Scheinberg IH, Jaffe ME, Sternlieb I. The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease. N Engl J Med. 1987;317:209-213.
  3. 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.
  4. Desai V, Kaler SG. Role of copper in human neurological disorders. Am J Clin Nutr. 2008;88(suppl):855S-858S.
  5. Roberts EA, Schilsky ML. AASLD Practice Guidelines. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089-2111.
  6. Wilson Disease Association. Diet and nutrition. http://www.wilsonsdisease.org/wilson-disease/wilsondisease-diet.php. Accessed July 1, 2016.

©2016 Valeant Pharmaceuticals North America LLC. All Rights Reserved. CUP.0032.USA.16

 

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.