CUPRIMINE® (penicillamine) was the first oral agent used to remove excess copper in patients with Wilson’s disease (WD).1 The main effect of CUPRIMINE® in WD 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 WD1.
Remind your patient that WD 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 WD 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 WD, whether or not he or she has symptoms.2 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®.2
Numerous studies demonstrate the effectiveness of CUPRIMINE® as a treatment for WD. 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.3
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 WD signs or symptoms, disease effects appear to be prevented indefinitely if daily treatment with CUPRIMINE® is continued.2
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.3
- 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.
For patients with WD, 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.2