Martine Walmsley
Chair of Trustees, PSC Support
Not all liver conditions are preventable. Martine Walmsley, who suffers from the rare liver condition primary sclerosing cholangitis (PSC), highlights the challenges of living with, and managing the condition.
The impact of alcohol misuse and obesity on the liver is a hot topic because, by making simple everyday changes, serious liver damage can be prevented or, in some cases, reversed.
While this is a significant public health focus, it is important to recognise that other causes of liver failure exist too. For example, more than 30,000 people in the UK are thought to have autoimmune liver conditions such as autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis (PSC).
The body ‘attacks’ parts of the liver
PSC affects people of any age, including children. It affects the bile ducts and the liver, and cannot be prevented. In PSC, the body ‘attacks’ parts of the liver, causing infections, scarring and eventually end-stage liver disease and failure, as well as conferring an increased risk of aggressive cancers. Around three-quarters of people with PSC also live with inflammatory bowel disease (IBD) in Northern Europe1.
While most people think that the liver can regenerate itself, there is no medical treatment to stop or slow PSC, and around half of PSC patients need a transplant or develop cancer1. Even though it is rare, PSC is one of the UK’s leading indications2 for liver transplantation.
Diagnosis is difficult
PSC is difficult to diagnose because there is no definitive diagnostic blood test, and it doesn’t always present with specific symptoms until the disease is advanced.
With the current focus on causes of preventable liver disease, our clinicians must not lose sight of other, rarer, possibilities. If someone has abnormal liver blood tests, understanding their context and the history is key. Do they have autoimmune disease in the family? Do they have IBD? PSC and other autoimmune liver diseases should be considered and followed-up with appropriate investigations and tests.
The increased cancer risk, invisible symptoms such as fatigue, itch and pain, and unpredictable, life-threatening complications mean that an effective treatment for PSC patients is urgently required.
Research and patient appetite for clinical trials
PSC is complex and, despite much progress over the last decade, remains poorly understood. It is a devastating and debilitating diagnosis and comes often at a time when people are trying to hold down full-time jobs and/or have young families at home to care for. PSC patients are understandably keen to take part in clinical trials, hoping the interventions being tested will slow or stop progression of the disease.
Interest in PSC research is growing, and there are more clinical trials ongoing for PSC than ever before. Investigators are using the latest technology to find new ways to interfere with underlying disease processes, that, hopefully, will lead to a treatment for PSC.
Gareth, a PSC sufferer who is taking part in a clinical trial to help find a cure, says: “PSC already takes so much away from us. Usually we are having to react to changing symptoms or worsening of the condition but, by signing up for a clinical trial, we are taking back some of that control.”
Improving the big picture
PSC and other autoimmune liver conditions are often overshadowed by the rising tide of preventable liver disease, and care is hampered by already strained health services. By motivating the general public to make healthier lifestyle choices, we can help people stay liver disease-free, reduce that strain on clinical services, and so enable those people with liver disease to access the appropriate care they need at the right time, whatever the cause.
[1] Weismuller TJ*, Trivedi PJ* et al. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis. Gastroenterology. 2017;152(8):1975-84 | [2] NHS Blood and Transplant – Organ Donation and Transplantation Activity Report 2018/19
