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About
Everything you need to know about Wilson Disease

Treatment of Wilson disease

Wilson disease is a very treatable condition. With proper therapy, disease progress can be halted and oftentimes symptoms can be improved. Treatment is aimed at removing excess accumulated copper and preventing its reaccumulation. Treatment for Wilson disease is a lifelong process. Patients may become progressively sicker from day to day, so immediate treatment can be critical. Treatment delays may cause irreversible damage.

Chelation therapy drugs approved for treating Wilson disease include penicillamine (Cuprimine® and Depen®) and trientine (Syprine® and Trientine Dihydrochloride) Both of these drugs act by chelation or binding of copper, causing its increased urinary excretion.

Mettalothionein inducer drugs approved for treating Wilson disease are (Galzin™) in the U.S. and (Wilzin®) in Europe. Zinc acts by blocking the absorption of copper in the intestinal tract. This action both depletes accumulated copper and prevents its reaccumulation. Zinc's effectiveness has been shown by more than 30 years of considerable experience overseas. A major advantage of zinc therapy is its lack of side effects.

Patients with severe hepatitis or liver failure may require liver transplant. Patients being investigated or treated for Wilson disease should be cared for by specialists in Wilson disease or by specialists in consultation with their primary physicians. Stopping treatment completely will result in death, sometimes as quickly as within three months. Decreasing dosage of medications also can result in unnecessary disease progression.

Taking your medicine as prescribed is extremely critical to the success of Wilson disease treatment. One of the advantages of having WD is that it is VERY treatable with effective, safe medications leading to a normal life expectancy . . . IF . . . you faithfully take your medications, as prescribed, LIFELONG. There are varying reasons for non-adherence in patients with WD and your physician has probably heard them all. Adherence is especially difficult in patients who were asymptomatic as diagnosis. These patients often do not see a cause and effect relationship.

To Purchase

Cuprimine® or Syprine® in the U.S., contact Valeant Pharmaceuticals North America LLC. at 1-877-ATON-549 (1-877-286-6549) . Visit the Valeant site at www.valeant.com. Contact our customer service group to find out about the products, including international distribution, here: http://valeant.com/about/contact.aspx.

Depen® in the U.S., contact Meda Pharmaceuticals at 1-732-564-2200 or send an e-mail inquiry to: This email address is being protected from spambots. You need JavaScript enabled to view it.

Trientine Dihydrochloride: please contact Univar Ltd.

Patient Assistance Programs

New U.S. Patient Assistance Program for Cuprimine and Syprine

Valeant Pharmaceuticals launched its new patient assistance program, Valeant Coverage Plus Program (VCPP), on July 8, 2013. This 

program was newly created to ensure that U.S. patients have better access to their medication with fewer out-of-pocket expenses. Recently, users of Cuprimine and Syprine have been experiencing unaffordable co-pays when refilling their prescriptions.

Valeant recently mailed an informational flyer, Financial Support for Improved Access to Cuprimine® (penicillamine) and Syprine® (trientine hydrochloride) Capsules to all prescribing physicians in the United States. To ensure that your physician is aware of the new program you will need to do your part as well. Before you need a new refill (we suggest at least 2 weeks prior) go to Cuprimine and Syprine to read more about the program and download the Patient Enrollment Form. Complete your portion of the form and contact your physician's office to let them know what needs to be done by them. If you have any questions, please contact a Coverage Plus Representative at 888-607-7267.

Teva Patient Assistance Program for Galzin™

Teva Select Brands sponsors a Patient Assistance Program for Galzin™ (www.galzin.com). If you have any questions regarding the Teva Assistance Program™ please contact them at (877) 254-1039 or visit www.tevacares.org .

 

Patient Access Network Foundation Assistance

Offers co-pay assistance for Wilson disease patients. http://www.panfoundation.org/wilson-disease
International:
The WDA has partnered with MAP International and Aton to support an international medication assistance program for Cuprimine® and Syprine®. Limited amounts of these medications are generously donated by Aton and shipped, at the request of the WDA, by MAP International. Shipping costs for this valuable program are covered by the WDA. To apply for this program please contact Mary Graper at This email address is being protected from spambots. You need JavaScript enabled to view it.

The WDA has also formed a partnership with Univar Ltd. for the compassionate use of Trientine Dihydrochloride. Again, limited amounts of this medication are generously donated and shipped by Univar, at the request of the WDA. This valuable program is completely free to the WDA since Univar also generously donates shipping costs.

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Common Queries and Questions

The course of liver disease in Wilson's disease stands in contrast to other forms of cirrhosis for many people. The chronic liver injury in Wilson's disease is caused by excess free copper, and the liver disease often stabilizes or even improves once the excess copper is treated with zinc acetate maintenance therapy. While some people do progress to need liver transplantation, others may actually see long-term improvement in their liver function over time. It is important to be attentive to issues such as immunizations for viral hepatitis, avoiding excess alcohol consumption, and treating complications of portal hypertension in order to give the liver its best chance to mend. Fred Askari, M.D., Ph.D. Assistant Professor Director, Wilson's Disease Center of Excellence Clinic at the University of Michigan
Generally, the brain is affected symmetrically with excess copper deposition, although symptoms can be worse on one side of the body than another. This may have to do with factors of asymmetric neurologic development, such as being right or left-handed. The copper is often seen most prominently in the basal ganglia, the area deep within the brain that coordinates movements. The face of the giant panda sign refers to a characteristic appearance of the basal ganglia in advanced Wilson's disease. This is a description of the appearance of the basal ganglia wherein one can get an impressionists image of the face of a giant panda. Fred Askari, M.D., Ph.D. Assistant Professor Director, Wilson's Disease Center of Excellence Clinic at the University of Michigan
High serum copper is not an indication of Wilson disease. Since most Wilson patients have a low ceruloplasmin they actually have a lower than normal serum copper. Ceruloplasmin is the protein that binds with copper to remove it from the body. It is the unbound (to ceruloplasmin) copper that is free to roam around the body and accumulate in organs causing Wilson disease damage. An elevated serum copper is more often due to an elevation of the level of serum ceruloplasmin since it contains ~90% of the circulating copper bound to it. Elevations of ceruloplasmin can occur with inflammation, in response to estrogen therapy and in pregnancy. Note: The exception to this is when there is severe liver injury (acute liver failure) caused by Wilson disease. This causes very large amounts of copper to be released into circulation and causes markedly elevated serum copper. When this occurs, patients are very ill and usually have jaundice (yellow eyes and skin color) and very abnormal lab results with respect to liver function and blood coagulation. - Michael Schilsky, M.D., Weill Cornell School of Medicine, WDA Medical Advisor
Hepatic: Asymptomatic hepatomegaly; Isolated splenomegaly; Persistent elevated AST, ALT; Fatty Liver; Acute hepatitis; resembling autoimmune hepatitis; cirrhosis (compensated or decompensated); fulminant hepatic failure Neurological: Movement disorders (tremor, involuntary movements); drooling, dysarthria; rigid dystonia; pseudobulbar palsy; seizures; migraine headaches; insomnia Psychiatric: Depression; neuroses; personality changes; psychosis Other symptoms: Renal abnormalities: amino-aciduria and nephrolithiasis; skeletal abnormalities: premature osteoporosis and arthritis; cardiomyopathy, dysrhythmias; pancreatitis; hypoparathyroidism; menstrual irregularities: infertility, repeated miscarriages From: A Diagnostic Tool for Physicians (3/04)
If the water is over 0.1 ppm (parts per million) (which is 0.1 mg/L), I recommend an alternative source. While 0.1 ppm isn't particularly hazardous, it indicates that significant copper is coming from somewhere, and at certain times or under certain circumstances the level might be quite a bit higher. George J. Brewer, M.D. Department of Human Genetics, University of Michigan Medical School
Yes. Since Wilson's disease often affects the liver, many Wilson’s disease patients cannot afford additional injury to the liver. Hepatitis A or Hepatitis B vaccine is as safe for Wilson's disease patients as it is for others. - H. Ascher Sellner, M.D.

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