Wilson’s Disease

Minerals, Liver | May 7, 2017 | Author: Naturopath

liver

Wilson’s Disease

Wilson’s disease is a rare genetic disorder in which the body is unable to eliminate copper leading to a dangerous accumulation. This build-up of copper is toxic and damages key organs in the body including the liver, eyes, brain, kidneys and nervous system.

wilsons diseaseUsually excess copper is excreted via bile but in people with Wilson’s disease the copper builds up in the liver and injures liver tissue.

Eventually the liver becomes so damaged causing copper to be released into the bloodstream, where it settles in various organs and structures.

Damaged tissues are replaced with scar tissue which accumulates over time, causing the affected organ to lose its ability to function properly until it eventually fails.

The copper begins to accumulate at birth, with symptoms usually appearing during teenage years.  Roughly one in 30,000 people inherit the faulty gene responsible for Wilson’s disease from both parents. However, sometimes this gene can spontaneously mutate and is not inherited. It is estimated that 1 in 9,000 people are unaffected carriers of this genetic mutation occurring on chromosome 13.

There are treatment options available which can help to manage the condition but without this, copper poisoning is potentially fatal. If left untreated, Wilson’s disease can cause severe brain damage, liver failure and death.

What are the signs and symptoms?

wilson's diseaseA striking feature of Wilson’s disease is that people can present with different clinical features which can include:

  • Kayser-Fleischer corneal rings
  • Abdominal enlargement
  • Anorexia
  • Vomiting of blood
  • Hand tremors
  • Dizziness
  • Skin rash or dermatitis
  • Anxiety, depression and personality changes
  • Convulsions
  • Speech and writing difficulties
  • Headaches
  • Stiffness and reduced movement of the extremities
  • Confusion
  • Dementia
  • Lack of menstruation

Complications

Without appropriate medical treatment, the complications of Wilson’s disease can include the following:

  • Liver disease, such as hepatitis, chronic swelling, cirrhosis or necrosis
  • Kidney and heart problems
  • Low immune system function
  • Enlarged spleen
  • Anaemia
  • Weakened bones
  • Permanent physical or intellectual disability
  • Muscle atrophy
  • Death

wilson's disease genetics

Medical treatment

Medical treatment involves chelation therapy, usually using a medication called penicillamine which binds to the copper and removes it out of the body through urine.

Regular blood and urine tests for copper levels allow treatment to be adjusted if necessary.

It is important for the whole family to undergo genetic testing for Wilson’s disease.

While medical treatment is necessary the following suggestions using supplements and dietary changes have also been shown to be beneficial for those suffering from Wilson’s disease.

Avoiding copper

Copper is usually found in a wide range of foods, a small amount of which is needed for the body. It is advisable for people with Wilson’s disease to eat a diet low in copper which means avoiding;

  • whole-wheat products
  • mushrooms
  • nuts
  • chocolate
  • dried fruit
  • liver 
  • shellfish

Copper can  be present in some cooking utensils and supplements, particularly multivitamins and zinc supplements so be sure to always read the ingredients list.

Zinc therapy

wilson's disease zincA diet high in zinc may be helpful in blocking the absorption of copper in the intestines.

Foods rich in zinc include meats like poultry, fish, lamb and pork. Vegetarian sources are plentiful and include brown rice, rye, potatoes, pepitas, beans, oatmeal and dairy products.

Zinc can be taken as a supplement for a stronger result. It has been found to effective as a first-line treatment in Wilson’s disease related liver disease and as a maintenance treatment in patients with mild liver disease.

 

Vitamin B6

This important nutrient is needed to bolster the nervous system. Unfortunately, the side-effect of penicillamine therapy is vitamin B6 deficiency, which can further disrupt the functioning of muscles, nerves and red blood cell formation among many others. As such, vitamin B6 is added routinely to the treatment regimen at a dose of 25-50mg daily. It can also be supplemented in its activated form called pyridoxal-5-phosphate, so that it is readily available for the body to use.

Antioxidants

wilson's disease antioxidantsWilson’s disease leads to increased oxidative stress and damage caused to many organs and parts of the body. Antioxidants help to reduce this damage. 

Levels of certain antioxidants such as vitamin C and E, selenium and beta-carotene have been found to be low in people with Wilson’s disease.

Other antioxidants that may be helpful include co-enzyme Q10, lipoic acid, grape seed extract and green tea.

Antioxidants are also found in abundance in a range of different coloured fresh fruit and vegetables. Lightly steaming them or eating raw ensures that the high nutrient levels are retained.
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Supporting the liver

As copper accumulates in the liver, taking St Mary’s thistle may help to provide support for copper detoxification and its removal. Silybum, the active component of St Mary’s thistle helps to stimulate phase II liver detoxification which may be impaired in Wilson’s disease.

wilson's disease milk thistleAlthough no current studies confirm the use of this herb in Wilson’s disease, St Mary’s thistle has been extensively studied for its ability to improve liver function, detoxify wastes, reduce inflammation and provide antioxidant support.

It has proven benefit for liver cirrhosis and hepatitis and as the symptoms of Wilson’s disease can present with similar symptoms to these conditions, the use of this herb in Wilson’s disease can then be supported.

St Mary’s thistle can also be combined with other herbs such as turmeric, andrographis or dandelion root for further support.
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Other suggestions

Other general guidelines include avoiding alcohol, smoking and eating refined carbohydrates from sugar, breads, pastries, pasta, noodles, cakes, biscuits and sugary drinks. Other foods to avoid include margarines, deep fried foods and any fatty foods as they put a strain on the liver in the processing of them.

Include instead healthy oils from avocado, fresh fish and good quality cooking oils such as coconut and olive oil to provide health benefits.
 

References

https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/wilsons-disease

http://www.medscape.com/viewarticle/543866_6

Ni W, et al. Zinc monotherapy and a low-copper diet are beneficial in patients with Wilson disease after liver transplantation. CNS Neurosci Ther. 2013 Nov;19(11):905-7

https://www.ncbi.nlm.nih.gov/pubmed/24119323

Ranucci G, et al. Zinc monotherapy is effective in Wilson’s disease patients with mild liver disease diagnosed in childhood: a retrospective study. Orphanet J Rare Dis. 2014 Mar 25;9:41

https://www.ncbi.nlm.nih.gov/pubmed/24661374

Dalgic B, et al. Evaluation of oxidant stress in Wilson’s disease and non-Wilsonian chronic liver disease in childhood. Turk J Gastroenterol. 2005 Mar;16(1):7-11

https://www.ncbi.nlm.nih.gov/pubmed/16252181

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