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Treatment with CUPRIMINE® (penicillamine) for Wilson’s disease

Wilson’s disease can be effectively treated with a low copper diet and chelating agents that bind copper to facilitate its excretion from the body. CUPRIMINE® is a first-line chelating agent indicated for removal of excess copper in patients with Wilson’s disease.3

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What is
CUPRIMINE®?

CUPRIMINE® is a first-line chelating agent indicated for removal of excess copper in patients with Wilson’s disease.3 CUPRIMINE® was the first oral agent used to remove excess copper in patients with Wilson’s disease.5 The main effect of CUPRIMINE® in Wilson’s disease is to promote the urinary excretion of copper. Although CUPRIMINE® is a general chelator of metals, it also acts by inducing methallothionein in individuals with Wilson’s disease.5

Remind your patient that Wilson’s disease may be managed effectively with medications and diet. It is crucial that he or she does not stop taking his or her medication if he or she begins to feel better because Wilson’s disease is a chronic condition that must be managed throughout life.

CUPRIMINE® may be right for your patient if he or she has been diagnosed with Wilson’s disease, whether or not he or she has symptoms.3 Ask your patient whether he or she has a history of kidney disease or if she is pregnant or nursing or planning to become pregnant. CUPRIMINE® should not be taken by a patient who has had aplastic anemia or agranulocytosis that was related to taking CUPRIMINE®.3

Numerous studies demonstrate the effectiveness of CUPRIMINE® as a treatment for Wilson’s disease. CUPRIMINE® promotes recovery of liver function and improvement in clinical signs during the first 2–6 months of treatment, and continued use prevents disease progression.2

Penicillamine has been shown to improve neurological symptoms, cause Kayser-Fleischer rings to fade and gradually diminish liver symptoms and psychic disturbances. Noticeable improvement may not occur for 1–3 months, but life is prolonged with this therapy. In patients with no Wilson’s disease signs or symptoms, disease effects appear to be prevented indefinitely if daily treatment with CUPRIMINE® is continued.3

Determine the correct dose for your patient, which may be between 0.75 and 1.5 g/d, and prescribe for at least 3 months. After that, the dose may be reduced or the regimen otherwise changed based on how the patient responds. Be sure to ask your patient about any side effects while he or she is taking CUPRIMINE® or any other medicines.2

Safety Considerations

  • Patients with a history of penicillamine-related aplastic anemia or agranulocytosis should not be restarted on penicillamine. Because of its potential for causing renal damage, penicillamine should not be administered to rheumatoid arthritis patients with a history or other evidence of renal insufficiency.
  • The use of penicillamine has been associated with fatalities due to certain diseases such as aplastic anemia, agranulocytosis, thrombocytopenia, Goodpasture's syndrome and myasthenia gravis.

Dosing Considerations

For patients with Wilson’s disease, in the absence of any drug reaction, a dose between 0.75 and 1.5 g that results in an initial 24-hour cupriuresis of over 2 mg should be continued for about 3 months. Adequately treated patients will usually have <10 µg free copper/dL of serum. It is seldom necessary to exceed a dosage of 2 g/d. In patients who cannot tolerate as much as 1 g/d initially, initiating dosage with 250 mg/d and increasing gradually to the requisite amount gives closer control of the effects of the drug and may help to reduce the incidence of adverse reactions.3

Physicians planning to use penicillamine should thoroughly familiarize themselves with its toxicity, special dosage considerations and therapeutic benefits. Penicillamine should never be used casually. Each patient should remain constantly under the close supervision of the physician. Patients should be warned to report promptly any symptoms suggesting toxicity.

WD Rx Access

Providing support for people living with Wilson's disease.

The WD Rx Access program offers $5 monthly co-pays, medication home delivery and one-on-one nurse coaching for eligible commercially insured patients. By signing up patients will also receive the WD Rx Access newsletter, The Word on Wilson’s, with helpful tips and information.

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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 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.

Please click here for full Prescribing Information including Boxed Warning.

Indication
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 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
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.

Please click here for full Prescribing Information including Boxed Warning.