Rare Disease Day 2017
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The PBC Foundation is the only UK based organisation that specifically deals with PBC.

Rare Disease Day 2017

Rare Disease Day 2017

On Rare Disease Day 2017 we are again reminded of the need to shine a spotlight on disease such as PBC and help the general public gain a better understanding.

Despite it being the third most common cause of premature death in the UK (See ref 1) the public are confused about liver disease, according to research

Ninety four percent of the 2,000 British adults surveyed by YouGov know that alcohol can lead to liver disease, but most (56%) are unaware that immune system disorders can also cause liver disease. Furthermore; 

  • 41% of people are unable to locate the liver despite it being the largest internal organ in the body
  • While 94% of adults know that alcohol causes liver disease, more than half are unaware that immune system disorders can also cause liver disease 
  • A third of Brits incorrectly think that waiting lists for a donor liver are getting shorter

One of the most common immune system disorders that causes liver disease is known as PBC (primary biliary cholangitis), a condition that primarily affects women over 40 and is characterised by symptoms such as chronic fatigue and severe itching.

Collette Thain MBE is Chief Executive of the PBC Foundation, an international charity that provides support and information to those suffering from PBC which is this year celebrating its 20th anniversary.  She said: “People often associate liver disease with lifestyle habits such as alcohol consumption and do not realise that it can also be caused by genetic conditions.

“Over 20,000 people in the UK suffer from PBC, some of whom are not yet diagnosed.  PBC patients, 90% of whom are women, often suffer debilitating symptoms. The fatigue can be likened to walking in tar, the itch can feel like spiders crawling under your skin. There’s only one treatment currently available, but too many people with PBC don't fully respond. This leads to a fearful situation, and many face the distressing prospect of having a liver transplant. Even when on the transplant list, some patients die waiting for a donor organ”.

The research highlights that a third of Brits incorrectly think that waiting lists for a donor liver are getting shorter and only 7% know that most liver diseases recur after receiving a transplant.

Other findings from the survey include:

  • 41% of people are unable to identify the liver when shown a diagram of five key organs, despite it being the largest internal organ in the body; many (30%) mistake it for the pancreas
  • Most people (55%) know that obesity can cause liver disease, but some incorrectly identify smoking (43%) and stress (20%) as potential causes 
  • Less than four in 10 adults (39%)have joined an organ donor register but almost three quarters (72%) would be willing to receive a donor organ if they needed one
  • 20% of people on the organ donor register either have not told their next of kin about their organ donation decision, or have told them but are not confident that their next of kin would support their decision if they die

You can read the UK Strategy for Rare Diseases here: https://www.raredisease.org.uk/uk-strategy-for-rare-diseases

1.) Williams R, Aspinall R, Bellis M, et al. Addressing liver disease in the UK: a blueprint for attaining excellence in healthcare for liver disease and reducing premature mortality from the major lifestyle issue of excess alcohol consumption, obesity, and viral hepatitis.Lancet 2014; published online Nov 27, 2014. http://dx/doi.org/10.1016/S0140-6736(14)61838-9


Notes to editors:

All figures, unless otherwise stated, are from YouGov Plc.  Total sample size was 2014 adults. Fieldwork was undertaken between 31st August - 1st September 2016.  The survey was carried out online. The figures have been weighted and are representative of all GB adults (aged 18+).