Depen
Generic Name: Penicillamine
Brand Name: Cuprimine, Depen
Indications
Penicillamine is indicated in the treatment of Wilson's disease, cystinuria, and in patients with severe, active rheumatoid arthritis who have failed to respond to an adequate trial of conventional therapy.
Dosage and Administration
Penicillamine (Cuprimine) is available for oral administration as 125 mg or 250 mg capsules.
Normal Dosage
Wilson’s Disease: Optimal dosage can be determined by measurement of urinary copper excretion and the determination of free copper in the serum. The urine must be collected in copper-free glassware, and should be quantitatively analyzed for copper before and soon after initiation of therapy with Cuprimine.
Determination of 24-hour urinary copper excretion is of greatest value in the first week of therapy with penicillamine. 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 three months, by which time the most reliable method of monitoring maintenance treatment is the determination of free copper in the serum. This equals the difference between quantitatively determined total copper and ceruloplasmin-copper. Adequately treated patients will usually have less than 10 mcg free copper/dL of serum. It is seldom necessary to exceed a dosage of 2 g/day.
Cystinuria: It is recommended that CUPRIMINE be used along with conventional therapy. By reducing urinary cystine, it decreases crystalluria and stone formation. In some instances, it has been reported to decrease the size of, and even to dissolve, stones already formed.
The usual dosage of CUPRIMINE in the treatment of cystinuria is 2 g/day for adults, with a range of 1 to 4 g/day. For pediatric patients, dosage can be based on 30 mg/kg/day. The total daily amount should be divided into four doses. If four equal doses are not feasible, give the larger portion at bedtime. If adverse reactions necessitate a reduction in dosage, it is important to retain the bedtime dose.
Rheumatoid Arthritis: The principal rule of treatment with CUPRIMINE in rheumatoid arthritis is patience. The onset of therapeutic response is typically delayed. Two or three months may be required before the first evidence of a clinical response is noted .When treatment with CUPRIMINE has been interrupted because of adverse reactions or other reasons, the drug should be reintroduced cautiously by starting with a lower dosage and increasing slowly.
The currently recommended dosage regimen in rheumatoid arthritis begins with a single daily dose of 125 mg or 250 mg, which is thereafter increased at one to three month intervals, by 125 mg or 250 mg/day, as patient response and tolerance indicate. If a satisfactory remission of symptoms is achieved, the dose associated with the remission should be continued
Overdose: Seek emergency medical attention. Symptoms of a penicillamine overdose are not known.
Drug Description
Penicillamine is a chelating agent used in the treatment of Wilson's disease. It is also used to reduce cystine excretion in cystinuria and to treat patients with severe, active rheumatoid arthritis unresponsive to conventional therapy. It is 3-mercapto-D-valine. It is a white or practically white, crystalline powder, freely soluble in water, slightly soluble in alcohol, and insoluble in ether, acetone, benzene, and carbon tetrachloride. Although its configuration is D1 it is levorotatory as usually measured:
[a] 25° = -62.5° ± 2° (c = 1, 1N NaOH) calculated on a dried basis.
The empirical formula is C5H11NO2S, giving it a molecular weight of 149.21. The structual formula is:
It reacts readily with formaldehyde or acetone to form a thiazolidine-carboxylic acid.
Side effects
If you experience any of the following serious side effects, seek emergency medical attention or contact your doctor immediately:
- an allergic reaction (shortness of breath; closing of your throat; difficulty breathing; swelling of your lips, face, or tongue; or hives);
- fever or chills;
- a sore throat;
- unusual bleeding or bruising;
- blood in the urine;
- unexplained shortness of breath, coughing, or wheezing;
- abdominal pain;
- yellow skin or eyes;
- muscle weakness; or
- Double vision.
Other, less serious side effects may be more likely to occur. Continue to take penicillamine and notify your doctor if you experience
- itching or a rash;
- nausea, vomiting, diarrhea, or decreased appetite;
- ringing in the ears;
- decreased taste;
- sores in the mouth;
- poor wound healing; or
- Increased wrinkling of the skin.
Side effects other than those listed here may also occur. Talk to your doctor about any side effect that seems unusual or is especially bothersome.
Precautions
- You cannot take penicillamine if you have taken it in the past and it has damaged your blood cells.
- Before taking penicillamine, tell your doctor if you have kidney disease or any other serious illness. You may not be able to take penicillamine, or you may require a lower dose or special monitoring during therapy.
- Penicillamine may cause birth defects in an unborn baby. However, it has also been used during pregnancy with no evidence of defects. Penicillamine should not be used during pregnancy except to treat Wilson's disease and some cases of cystine in the urine. Do not take this medication without first talking to your doctor if you are pregnant.
- It is not known whether penicillamine passes into breast milk. Since penicillamine may harm a nursing infant, breast-feeding is not recommended during treatment with this medication.
Drug Interactions
Do not take penicillamine if you are taking any of the following medicines:
- a gold-therapy product such as auranofin (Ridaura), aurothioglucose (Solganal), or gold sodium thiomalate (Myochrysine, Aurolate);
- an antimalarial medicine such as quinine (Quinamm), mefloquine (Lariam), chloroquine (Aralen), hydroxychloroquine (Plaquenil), primaquine, or pyrimethamine (Daraprim);
- a cancer chemotherapy medicine; or
- phenylbutazone.
Like penicillamine, the medications listed above can affect the blood and the kidneys. Combined with penicillamine, any of these medicines can be very dangerous.
Before taking penicillamine, tell your doctor if you are taking digoxin (Lanoxin, Lanoxicaps). Penicillamine may decrease the effects of digoxin, and your doctor may want to adjust your dosage or monitor your therapy.
Many other drugs, especially antacids and vitamin and mineral supplements, can decrease the effects of penicillamine. Do not take any medicines or over-the-counter drugs or supplements within 1 hour of a penicillamine dose.
Drugs other than those listed here may also interact with penicillamine. Talk to your doctor and pharmacist before taking any prescription or over-the-counter medicines.
