Collette Thain MBE
CEO, PBC Foundation
There are many forms of liver disease, most of which are treatable. But many can coexist with other liver conditions, making the journey even more difficult for the patient, and their family.
Primary biliary cholangitis (PBC) is one of the most rare forms of liver disease. However, it is one of the main causes of liver transplant in adult women in the UK.
An autoimmune disease, PBC has no known cause, no cure and affects 1 in 1000 women over 40.
Symptoms of PBC
The main symptoms of PBC are fatigue and itch. However, that does not begin to do justice to the severity of the symptoms or the effect they have on a patient’s quality of life.
PBC is easy to diagnose if clinicians ask the right questions. The antimitochondrial antibodies (AMA) test is important in identifying PBC, as is an alkaline phosphates level (ALP) test.
When patients refer to itchiness they often liken it to feeling ‘spiders crawling underneath the skin.’ Because it is under the skin, with no visible signs, sufferers have been labelled self-harmers, or told they are experiencing tactile hallucinations.
When patients talk about the fatigue they experience, they sometimes talk of ‘walking through custard,’ or ‘wearing a lead blanket.’
The dangers of these symptoms
These are serious quality of life issues. Some patients with incurable itching have died by suicide. Some have had liver transplants just to cure the itch.
Even if a patient is asymptomatic, there can still be ongoing damage to the liver, making a transplant the only viable solution. Not everyone who needs one is lucky enough to receive a new liver. Those who do know that it is not a cure, but a different set of challenges.
First-line therapy available
But among all the gloom, there is good news. PBC is easy to diagnose if clinicians ask the right questions. The antimitochondrial antibodies (AMA) test is important in identifying PBC, as is an alkaline phosphates level (ALP) test.
There is also a first-line therapy that, when dosed appropriately, can help slow down the progression of disease to give patients an average life expectancy. If first-line therapy fails, there are now options and even a brand new licensed second-line treatment.
So, there is good news. We need patients diagnosed earlier, treated appropriately and referred on to specialists if they do not respond to first-line therapies.
These three actions would make a huge difference, not only to PBC patients, but also to the community and to demand on liver transplant services. Until that happens, our work continues.