Maria was diagnosed with Primary Sclerosing Cholangitis when she was 29 and became symptomatic during her first pregnancy. She welcomes the regular scans and ultrasounds she receives and spoke at a medical conference about the importance of surveillance for liver patients. Thank you for sharing your story with us, Maria
I was 29 years old when I received the diagnosis that changed my life. I had never smoked, nor been overweight or more than an occasional drinker. So, when liver disease was first mentioned to me, it was a real shock. I can vividly recall when the consultant confirmed my diagnosis. He was matter-of-fact when he said “you have the worst type of liver disease because there is no cure, or treatment except liver transplant. It attacks the bile ducts in the liver and affects the liver itself. It also puts you at much higher risk of several aggressive cancers, and if you get bile duct cancer, we won’t give you a liver transplant”.
Primary Sclerosing Cholangitis
The liver disease in question is Primary Sclerosing Cholangitis, helpfully abbreviated as PSC. It is an auto-immune condition, and probably linked to ulcerative colitis, another auto-immune condition I have had since I was a teenager. It was through routine monitoring for my colitis that a member of the gastro team noticed my liver blood tests were abnormal. “Have you had a heavy night out?” she asked. It was a few months later, and after a couple of MRIs, that my PSC diagnosis was confirmed.
Of course, I turned to Google to find out more about the condition. What I found sent me into a spiral of despair. I read that the average life expectancy of a PSC patient is 8-12 years from diagnosis, unless life can be extended with a liver transplant. Suddenly, the life I thought I had ahead of me was turned on its head. This timeline would mean I would be dead or having life-changing surgery by the age of 40.
The question I’m commonly asked by friends is: “Why don’t you have a liver transplant now?” There is a common misconception that: (i) organs are readily available; (ii) transplants are straightforward; and (iii) they are a magic cure. PSC returns post-transplant in approximately one third of cases. Rejection issues are common post-surgery and life expectancy is reduced afterwards. That said, when my time comes for a liver transplant, I know I will be beyond grateful for every minute of extra time it could give me with my family.
While this diagnosis took some time to get my head around, I continued for several years with regular scans, blood tests and appointments. But life continued – I got married, was promoted at work and continued to live as healthily as I could.
Obstetric cholestasis
I became symptomatic with PSC in my first pregnancy. I experienced liver related complications, including Obstetric Cholestasis, which carries a risk of stillbirth. I also started to itch like crazy! I was lucky – although I was induced early – Chloe was a healthy baby. The pregnancy had taken a toll on my liver though, and I suffered liver infections (cholangitis) and had a few surgeries (ERCPs) after she was born to open some blocked bile ducts. At this point, I gave up my career as a solicitor in London. 
With my second pregnancy, I became jaundiced and dangerously unwell. I was admitted to hospital and my daughter was induced seven weeks early. I remember my (amazing) obstetrician saying: ‘Usually all our focus is on delivering the baby, but it has got to the point where we need to get this baby out for your own sake’. Alice spent some time in neonatal intensive care (NICU), but otherwise she’s perfect. Needless to say, we are not having any more children!
I have an annual surveillance colonoscopy for bowel cancer, but the risk of liver cancers are what makes me most anxious, due to the poor prognosis and difficulty in diagnosis.
As I write, I am having my second liver cancer “scare”. My first was in 2021, when I received a phone call out of the blue from a registrar who told me some lesions had been detected in my routine MRI and they could be cancerous. I would need a CT/PET scan and a liver biopsy.
These tests took longer than expected. I hadn’t appreciated the delay between each test while the reports were written up and then waiting for my case to be discussed at the multi-disciplinary meetings. Fortunately, the conclusion after these tests was that there was “no convincing evidence of cancer”, however these lesions have been kept under review with three-six monthly MRI scans and ultrasounds.
This year, one of these monitoring scans showed that one lesion has started growing rapidly again and could be liver cancer (HCC). I am currently waiting for results of further tests. All I can do is attend my appointments and remain positive. Hopefully, the benefit of being monitored regularly means any cancer will be spotted at an early stage where it is treatable.
Anti-itching clinical trial is a positive
My liver is now very scarred, and I have the start of cirrhosis. The itch has been my most debilitating symptom, making it difficult to sleep at night. Not ideal when little ones are waking you up as well! However, since I joined a clinical trial for a new anti-itching drug in January 2024 I’ve been getting four consecutive hours of sleep a night, which may not seem like much, but it is a huge improvement for me!
With the benefit of more sleep, I now have the energy to use my experience with liver disease for good, and help improve liver care for others. I found the British Liver Trust through social media and have used the website to find out about diet and cirrhosis. I then answered their call for patients to speak at a conference entitled Improving surveillance for hepatocellular carcinoma in the UK in April 2024. I spoke as a patient advocate to 150 clinicians and talked about the importance of HCC surveillance and having a clear management plan.
I’m currently on the patient panel for a number of new projects and research groups. I’ve been invited onto the South-East London Cancer Alliance as a patient rep and am working with my medical team to progress a blueprint for excellent care for PSC. I support PSC Support, an amazing charity for those affected by PSC, and through them I keep up to date with accurate information about my condition. I’m also currently on my third clinical trial/study.
PSC has changed my life. But, with so many clinical trials ongoing, there is hope on the horizon.
