Wilson Disease

What is Liver Transplant?

Wilson Disease - Overview and Treatment

Overview

Wilson’s disease is also known as hepatolenticular degeneration and progressive lenticular degeneration. It is a genetically inherited disorder that causes the accumulation of copper in the brain, liver and corneas of the Eyes. Its also a rare disorder that do not discriminate by male, female, race or ethnicity and targets One in 30,000-40,000 people worldwide and is diagnosed among people from 5 to 35 years of age. However it can affect younger as well as older age groups. Its a progressive disease which means if left untreated it may cause Liver Disease and Failure, Dysfunctional Central Nervous System and even death. Though small amount of copper is helpful for the development of bones, nerves, collagen and skin melanin but higher amounts can be fatal. 90% Copper is absorbed by the food through Bile and gets out through feces, rest is excreted through Urine from the system.

The disease got its name from the Britain based Neuro-physician Samuel Alexander Kinnier Wilson, who described the condition in the year of 1912, along with its pathological impact on brain and Liver.

Causes:

  • Wilson’s disease is an autosomal recessive trait, i.e. to develop the disease you must inherit one copy of the defective gene from each parent. If the patient receive an abnormal gene then he or she will not get ill to himself or herself, but he or she will be a carrier to pass that gene to the children.

Symptoms:

  • Kayser-Fleischer rings, a golden brown discoloration of the eye.
  • Sunflower cataracts, a distinctive multi-colored center with spokes radiating outwards.
  • Jaundice or yellowing of eyes.
  • Impairment of memory, speech and vision.
  • Problems with physical coordination.
  • Low blood pressure.
  • Abdominal pain, Fatigue, Lack of Appetite

Diagnosis:

  • Blood test: (Copper testing) Doctors recommend blood test to check the level of a protein that binds copper in the blood, ceruplasmin and the level of copper in blood.
  • Urine test: Doctors recommend urine test to measure the amount of copper in blood.
  • Eye exam: Doctors recommend an eye test to check the level of copper in eyes. This test is recommended for checking for conditions like Kayser-Fleischer rings and sunflower cataract.
  • Genetic testing: A blood test is conducted to identify mutations that can lead to Wilson’s disease.
  • Liver biopsy: Doctors recommend a biopsy, which involves extracting the liver tissue and examining it.

Treatment:

Medications:

Doctors might prescribe medications known as Chelating Agents, which can binds copper and then get it released in the blood stream which will be then filtered by the Kidneys and get released out of the body through Urine. Doctors may prescribe other medicines to treat symptoms as required.

Surgery:

If due to prolonged Copper deposits, the Liver is severely damaged then the Liver Specialist my suggest a Liver Transplant to the patient.  During a Liver Transplant the surgeon removes the diseased Liver and replaces with a Full or Part of healthy Liver from the Donor.

This part of healthy Liver comes from Family/Related Living donor, This is Called Living Donor Liver Transplant(LDLT) and a Full Liver comes from an Unrelated Cadaver Donor this is called Cadaver/Dead Donor Liver Transplant  (DDLT).

Life after Liver Transplant Surgery:

Liver transplants are usually a great success. According to the most recent data(2004) one year survival rate post-transplant is approximately 86 percent for all liver transplant patients. About 78 percent of patients live upto 3 years after liver transplant, 72 percent of patients live upto 5 years after the surgery and 53 percent of patients live upto 20 years after the surgery.

After a liver transplant, a patient is recommended to stay in the I.C.U for a few days and then later shifted to the transplant recovery area for 5-10 days. The patient can then go back to normal day-to-day activities. Regular follow-ups are advised and patients have to take the medicines for lifetime.

Risks involved in a liver transplant include:

  • Organ rejection.
  • Failure of the donated liver.
  • Clots of blood.
  • Bile duct leaks.
  • Shrinking of bile ducts.
  • Long-term complications may also include recurrence of liver disease in the transplanted liver.

Prognosis:

The earlier you find out if you have the gene for Wilson's disease, the better your prognosis is. Wilson's disease can develop into liver failure and brain damage if left untreated. Early treatment can help reverse neurological issues and liver damage.

Cost of Treatment for Wilson Disease:

The annual cost for the average daily adult dose of Syprine (1,000 mg) is $20,000 - 30,000(In India) and substantially Higher in Other Countries, making it the most costly treatment for any liver disease to date.

Medical cost of Liver Transplant in a reputed private Hospital Cost around INR 22-30 lakhs( 2.2 Million to 3 Million Indian Rupees). There will be added Costs for Lifelong immunosuppressant Medicines etc.

Liver Transplant India offers a low-risk and most affordable way to treat Wilson’s disease. Our team of experienced and skilled doctors make life at hospital a painless one and guarantee a speedy recovery.

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