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!

The post Rachael’s story: The British Liver Trust’s Nurse-led Helpline has offered me great advice. Having someone who knows about medical environments is extremely useful” appeared first on British Liver Trust.

My story started when I was just two years old in 1979. My parents were decorating and at some point, they turned to see me with a handful of paints. As a precaution I was taken to the hospital to check in case I had eaten some and thankfully I hadn’t.

However, after examination, the doctor discovered I had an enlarged liver and over the next 10 years I was in and out of various hospitals throughout the North East with no diagnosis. I used to suffer a lot with bloating and my immune system was not good and I regularly got colds etc. My schooling suffered as a result and I couldn’t do regular things with friends like playing football and running about.

My symptoms started on a school camping trip when I was 12 – I felt unwell, then began vomiting what looked like black tar. My parents picked me up and called out the doctor – he prescribed peppermint tablets for indigestion and I still laugh about that to this day. A few days later things took a serious turn. I continued projectile vomiting this black substance – it happened four or five times in the next year and got worse each time. I was rushed to Freeman’s hospital in Newcastle where I was diagnosed and treated for oesophageal varices and put in an induced coma for five or six days to help my recovery.

My mum has since told me how hard the constant worry was for her. I am the oldest of four siblings and when I was ill I used to spend a lot of time at my grandparents so I could have a break from them.

 Finally diagnosed with Caroli Disease

Still undiagnosed, at the age of 13 I was sent to Birmingham Children’s Hospital for a few days for tests. There I was finally diagnosed with Caroli disease, a rare genetic condition which thankfully none of my siblings have been affected by. My consultant said my body was simply not strong enough to take any more bleeds and after being told all the potential grim outcomes of a liver transplant, I was put on the transplant list.

Late on February 24th 1992 we received the call to say a full liver was available. I said my goodbyes to the rest of my family and off we went on the roughly four-hour car trip. I was sat in the back with my mother and so scared and could tell she was also, but we both put on a brave face anyway. About 45 minutes from the hospital we were pulled over by the police for speeding, but upon hearing our news they blue-lighted us the rest of the way. As a 13-year-old boy I thought this was great!

I was in hospital for five weeks after my liver transplant because of a bile duct leak

The transplant took place and I was in hospital for around five weeks due to a bile duct leak. The first few years after the transplant were up and down, I had mild rejection a few times but in the end it all worked out. I had a normal life like other kids and could play again and as I got older I started going to the gym regularly and now love cold dips and saunas. They are all great for my body and mind.

Now 31 years post-liver transplant, I am married and have a 14-year-old daughter. We were lucky enough to live in Australia between 2016 and 2022 and have had plenty of amazing holidays abroad.

Everything that happened in my life is all because a mother chose to donate her 11-year-old child’s organs after they tragically died. Without the transplant I most probably would not have made 15 years old and another family would have lost a young son.

 Email from donor’s mum 

Around seven years ago I was over the moon to have email contact with my donor’s mum. I will never forget how much I cried when I received that first message from her. I got to know a little about my donor and received a photo of them with her – it was amazing.

My message to those who are waiting or have received a transplant is to never give up hope. Keep as strong and healthy as you can and cherish those around who are with you on this journey.

I’d also ask everyone to please have a chat with their family about their wishes regarding organ donation. I’ve shared my story to hopefully inspire others and show the importance of organ donors – they save lives and their families will always be heroes.

The post Jamie’s story: “If you are waiting for a liver transplant, never give up hope” appeared first on British Liver Trust.