Treatments


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.

Patient Assistance Programs

New U.S. Patient Assistance Program for Cuprimine and Syprine: WD Rx Access

Valeant Pharmaceuticals launched a new program, WD Rx Access – an enhanced version of the former Valeant Coverage Plus Program – which reduces the co-payment cost for all eligible patients taking CUPRIMINE® (penicillamine) or SYPRINE® (trientine hydrochloride). The program also includes an offer that makes receiving these medications more convenient.

The co-pay for CUPRIMINE and SYPRINE has been reduced from $25 to $5, for eligible patients. Patients also have the opportunity to have their medicine mailed directly to their home through a specialty pharmacy. Specialty pharmacies offer 24-hour access to pharmacists, auto-fill prescriptions, direct follow-up with physicians and more.

It is important to know that patients need to re-enroll every year for patient assistance. For those currently enrolled, a team member from WD Rx Access will proactively reach out to help re-enrollment at the start of each year. If you are interested in financial assistance, need to re-enroll, or want to take advantage of the specialty pharmacy offer, the WD Rx Access team members can be reached at 888-607-7267, Monday-Friday, 8 AM-6 PM ET.

You can find more information on http://www.tajpharma.com and also learn about new services available for people living with Wilson’s Disease, which include access to a dedicated nurse who can provide support related to treatment, diet, HCP communication and more.

Important Safety Information

“Treatment of patients with Trientine Hydrochloride Capsules may be complicated by severe, sometimes lifethreatening, adverse effects. Trientine Hydrochloride Capsules should be administered under the supervision of a physician experienced in the use of this medication for the treatment of sickle cell anemia.”

“Hydroxyurea is mutagenic and clastogenic, and causes cellular transformation to a tumorigenic phenotype. Hydroxyurea is thus unequivocally genotoxic and a presumed transspecies carcinogen which implies a carcinogenic risk to humans. In patients receiving long-term hydroxyurea for myeloproliferative disorders, such as polycythemia vera and thrombocythemia, secondary leukaemia’s have been reported. It is unknown whether this leukemogenic effect is secondary to hydroxyurea or is associated with the patient's underlying disease. The physician and patient must very carefully consider the potential benefits of Trientine Hydrochloride Capsules relative to the undefined risk of developing secondary malignancies. Trientine Hydrochloride Capsules is used to treat chronic myeloid leukaemia or cervical cancer.”

“Your medical team will discuss with you the options for treating your cancer. They will take into account factors such as the type of cancer, where it is, which stage it is at and whether you have had treatment before. The results of blood tests and other investigations will also be considered. How well you feel and how you are likely to cope with treatment is also important.”

“Your cancer treatment will usually consist of a treatment session with Trientine Hydrochloride Capsules followed by a break of a number of days before the next treatment session with Trientine Hydrochloride Capsules. This cycle may be repeated many times as part of your cancer treatment. Trientine Hydrochloride Capsules works by damaging cancer cells in the body. Trientine Hydrochloride Capsules also affects healthy cells and treatment with Trientine Hydrochloride Capsules may damage your immune system. Your medical team may arrange for you to have some blood tests to check how well your immune system is working. Do not share your medicine with other people. It may not be suitable for them and may harm them. The pharmacy label on your medicine tells you how much medicine you should take. It also tells you how often you should take your medicine. This is the dose that you and your prescriber have agreed you should take. You should not change the dose of your medicine unless you are told to do so by your prescriber. If you feel that the medicine is making you unwell or you do not think it is working, then talk to your prescriber.