When Rachael started experiencing debilitating fatigue her GP put it down to ‘her moods’. However, further blood tests revealed an issue with her liver and over the next few months Rachael had multiple tests as her hepatologist worked to pinpoint the cause. Thank you for sharing your story, Rachael
In the summer of 2024, I started feeling more tired than usual. I didn’t have any other symptoms at first, so I put it down to the stress of buying my first house, but then my mum noticed I was napping as much as my two-year-old niece.
My GP surgery ordered blood tests after which a request appeared on my NHS app for further tests because the results were ‘borderline abnormal’. By now the tiredness had turned into fatigue and I’d be in tears at times because I just couldn’t function properly. I called the surgery and the GP I spoke to said it was just my ‘moods’, but eventually agreed to more blood tests and a liver function test. The LFT was 666, when the normal range should be 30-130 and I was immediately referred to the hospital.
My hepatologist explained what the blood tests meant and said they’d try all the non-invasive tests first to rule out more common conditions. Over the next few months I had countless blood tests, a liver ultrasound, a biopsy, and two MRIs.
The biopsy ruled out autoimmune hepatitis, so then it was potentially PBC or PSC. The MRI showed a narrowing in my bile ducts, but there was also dilation which is very unusual. My consultant put me on ursodeoxycholic acid capsules, but I found them difficult to swallow and dreaded taking them. I’d never been ill before in my life so to suddenly have so many appointments and tests was quite overwhelming. Some days I would be my normal energetic self and then the next day I didn’t even have the energy to get out of bed. I wondered if I’d ever feel normal again!
I found the British Liver Trust as a link from an NHS site because I only go on trusted websites. Along my diagnosis journey when lots of autoimmune conditions were suspected, I basically lived on the British Liver Trust website researching what everything was! When my hepatologist suspected PSC, he gave me a leaflet produced by the British Liver Trust and there’s no other trusted source of information on Caroli disease, so it’s been the only place I refer back to.
Liver resection
I was referred to the surgical team at Leeds at the end of November 2024 and my surgery date was 8th January 2025 where they would remove sections two and three of my liver. They suspected it was Caroli disease, but I wouldn’t get a diagnosis until after surgery.
I was put on a protein diet, had to practice my breathing using a spirometer and keep myself as fit as possible (easier said than done over Christmas). But I stayed positive because I knew there was something I could do now to help myself and feel more in control. 
I was surprisingly calm about the surgery, which ended up being a bigger operation than originally planned. I was warned they may have to take more of my liver away and I had about a third removed. I had robotic keyhole surgery and then a large cut to remove the section of liver. I had tubes coming out of everywhere – a catheter, two or three cannulas, two wound catheters, a drain and then other wires and the tube in my nose. I could press a button for pain relief if I needed it so there wasn’t much pain, but the meds made me feel woozy and it almost felt like it was happening to someone else.
The ICU staff were so supportive and kind. They helped me get out of the bed to sit in a chair, reassured my mum and more besides. I was moved to the ward after a few days and discharged a couple of days after that.
Two months after my surgery the surgeon confirmed it was Caroli disease with no underlying autoimmune condition. I was elated as it meant that surgery should have solved the problem and I didn’t have to be on medication anymore.
Recovering at home felt harder because I didn’t have a nurse there all the time. My mum helped me with the blood thinning injections and I was taking laxatives because of the pain killers I was prescribed. We found ingenious ways of doing things like showering with a bin bag to keep my wound dry and using books as steps to help me into bed. I decided to keep a recovery journal to track all the little wins every day and it helped me recognise how big of an achievement simply having a bowel movement or getting out of bed by myself was at the time. I could see the progress quite quickly in the early days until things started to feel like a new normal or went back to normal completely. Now my scars are certainly looking better but I’m still getting used to them!
Two months after my surgery the surgeon confirmed it was Caroli disease with no underlying autoimmune condition. I was elated as it meant that surgery should have solved the problem and I didn’t have to be on medication anymore.
Unfortunately, a follow-up MRI scan revealed my bile ducts were now dilated at the other side of my liver. It was a rollercoaster summer, waiting for another scan and more appointments. I psyched myself up for more surgery but was instead referred back to hepatology in autumn. There’s a question mark over whether I might have PSC or more Caroli disease – my consultant suspects Caroli and they’ll do regular scans with the possibility of more surgery depending on the results.
It’s crazy to think how much has happened in just over a year and I’m trying to view this next chapter as a new challenge to overcome. But recovery is a journey and everything takes time, you’ve just got to be kind to yourself along the way.
I work full time which has definitely been challenging when dealing with the fatigue. It’s better than before the surgery, but it’s difficult dealing with the bad days and trying to accept a different way of doing things just to get through each week. My employer is very supportive and was fantastic when I was off work for my surgery. However, it’s hard explaining to people that some days I’m perfectly fine and the next I’m too tired to work. I work hybrid and often find myself napping in my lunch break when I’m working from home, or sometimes I fall asleep at my desk! Other days my tiredness levels may be OK, but I struggle with brain fog or being unable to concentrate. I’m trying to find ways of managing it but it’s not easy.
Meeting other Caroli patients
With a rare condition, you can feel like you’re getting passed from place to place and no one quite knows what to do with you. You’ve got to fight for yourself and keep chasing for appointments or scans and go to your appointments armed with as much knowledge as possible so you can have honest conversations about next steps.
I’ve contacted the British Liver Trust’s Nurse-led Helpline a few times to provide some clarity on my situation and they’ve offered great advice about what questions to ask at my appointments. Just having someone you can talk to who understands and who knows about medical environments can be extremely useful. I have attended the online support groups too, including a wellbeing session.
I’m always talking to people about my diagnosis as it helps them understand, and me to process my situation. The Health Unlocked forum can also be great to connect with people going through something similar. Unfortunately, I’ve yet to connect with anyone with Caroli disease but hopefully one day I’ll meet someone I can swap stories with!
