What’s in a name? Well, quite a lot if the name happens to be “cirrhosis”. Say the word and there’s a subliminal association with heavy drinkers who have destroyed their own liver as a consequence of their relationship with alcohol.
Hence the word isn’t often imbued with a huge amount of sympathy; indeed, there’s often a “They brought it on themselves” attitude around a person with the condition whenever it’s mentioned.
Now try having cirrhosis in the title of a condition which has nothing at all to do with alcohol abuse. The word cirrhosis simply means scarring and it can be caused by any number of liver diseases. One such condition that can cause scarring is PBC, which until recently was called Primary Biliary Cirrhosis. PBC is an auto-immune condition which affects the liver.
When you have PBC, the body attacks the cells lining the bile ducts, leading to a build-up of bile which over time can cause the liver irreparable damage. As a charity now in its 20th year, the PBC Foundation actively supports people with PBC around the world.
Over a period of two decades we realised that neither service users nor clinicians found the term cirrhosis helpful or accurate. Now that PBC is more commonly diagnosed earlier in the disease journey, the likelihood of it being at the point where cirrhosis has developed is slight.
Even after diagnosis, the chances of those with PBC then going on to develop cirrhosis stands at around 20 per cent. Inevitably, a member of the medical profession was diagnosing a condition containing the word cirrhosis when the reality was quite different. The end-result was patient confusion and anxiety.
Furthermore, we knew of numerous situations where GPs, not familiar with the condition, treated their patient with suspicion once blood and liver tests showed PBC. This had an unnecessarily negative impact on the patient and their relatives.
As the voice of PBC sufferers around the world, we were charged with campaigning for a name change to more accurately reflect the illness and help reduce the stigma surrounding it.
After assessing the appetite for change among our service users and polling them on possible name alternatives in 2014; we found a vast majority were in favour of replacing “cirrhosis” with “cholangitis”. Cholangitis refers to inflammation of the bile ducts and more closely describes the natural progression of PBC. Subsequently, EASL (European Association for Study of the Liver) clinicians conducted their own global surveys about the name change and came to the same view on using the cholangitis term.
Thus, the name, “Primary Biliary Cholangitis” came into usage last year and a position paper outlining the change lead by Professor Ulrich Beuers, a leading international figure in hepatology, was published in eight medical journals in late 2015.
The feedback already tells us that both patients and clinicians are now having different conversations around PBC, that it’s an easier dialogue to have with less opportunity for misinterpretation. It might seem small to many, but for us, this decision brings greater clarity to the illness and much less discrimination, which can only be a good thing.
• Collette Thain, MBE CEO, PBC Foundation