James was just five years old when he was diagnosed with congenital hepatic fibrosis, but lived well with the condition until his thirties when his liver began to fail and he became seriously ill. While his initial post transplant recovery was been ‘up and down’, ten months on James feels better than he has done in years. Thank you for sharing your story, James
I was a happy and healthy young boy until I caught chicken pox, aged five. It caused awful stomach pains and the out-of-hours GP told my parents to take me to A&E immediately. My liver and spleen were enlarged and I was transferred from one hospital to another while they tried to work out what was wrong until eventually my parents were told I had liver cancer. I was due to start chemo, but a final liver biopsy showed that it thankfully wasn’t cancer, but congenital hepatic fibrosis.
Things were then pretty much problem free until my thirties. The scarring of the liver caused high portal pressure and my enlarged spleen was swallowing up my platelets, and the risk of oesophageal varices was more of a concern than the liver condition itself.
In the summer of 2022, I started experiencing occasional slight swelling in my feet. I thought nothing of it until it was around my knees and mentioned it at my liver clinic. I was told it was the start of oedema and was prescribed a low dose of spironolactone. I didn’t realise this was the beginning of end-stage liver disease.
In March 2023 I developed gastroenteritis and contracted campylobacter and spent a week in hospital with severe stomach pains. I was administered morphine, but was unaware of how much it bungs you up and wasn’t given any laxatives or other medication when I was discharged.
Prescribed Rifaximin
Two weeks later, I tried to get up in the morning but completely fell over. My pupils were huge and I didn’t know who my fiancée was, or where or who I was. When the paramedics arrived, I was lying down with my eyes rolling in the back of my head, mumbling nonsense and totally unresponsive. I had also become quite aggressive which was completely out of character. I was blue-lighted to A&E, then quickly moved into intensive care where they concluded I’d had a massive episode of hepatic encephalopathy. Due to my body not being able to process the toxins properly the ammonia levels in my brain had reached almost 200. Much more and I would have surely been in a coma or have died. Thankfully, I made a full recovery and was discharged after 11 days. I was prescribed Rifaximin and placed on the transplant list in October 2023 which was amazing. However, that was quashed when my fiancée decided she didn’t want to be with me anymore.
Through Christmas and into the new year my oedema and now ascites became much worse. My condition and losing the love of my life was affecting everything and by mid-March 2024 I had left my job and was being drained every few weeks as the diuretics weren’t really working anymore. I was going downhill and would go into hospital for a week or more at a time, get discharged and a couple of days later I’d be back again. By August I was in full time and was being drained of at least eight litres every week. I also now had a feeding tube which I hated at first. I’d have vivid dreams in the night and would pull it out, it was horrible.
By the end of August 2024 I was taken off the transplant list as I had become too poorly. Due to the shortage of livers they don’t have enough to fix everyone and people become too ill to survive the operation. The palliative care team talked about preparing for end of life, but I don’t remember feeling too upset about it. Prior to this I had been begging to be transferred to the transplant unit in Leeds, but I had now almost given up hope and didn’t want to be poorly anymore.
Ten months after my liver transplant I feel better than I have done in years; my fitness is almost back to my 20s, my meds have stabilised, my bloods are perfect and my post-hospital recovery has been ‘text book’
Amazingly on 16th September I was told: “You’re going to Leeds!” I couldn’t believe it and was so happy. I was rapidly becoming more unwell and was extremely weak, shaky and could only slowly walk a few laps of the ward. The muscle degeneration was also evident. I was assessed by the transplant team, but I don’t really remember much from this point onwards.
On September 29th my bloods had become deranged and I was moved to ICU and put on kidney dialysis with a view to transplant re-listing. Two days later I was placed on the ACLF (Acute on Chronic Liver Failure) list and had multiple blood products overnight due to coagulopathy. The following day I experienced confusion and was put into a coma. I had an endoscopy and CT scan after developing melaena and became top of the transplant list. A potential liver was found but unfortunately it was too fatty, so couldn’t be used.
I was being kept alive by machines, was in a coma and literally had days to live before the miracle happened! Another liver was flown in and at 6.30am on Monday 7th October I was taken to theatre and came out around 3pm after my transplant and umbilical hernia repair, with the liver actually starting to work while I was still on the operating table.
The following day I was extubated and woken up after seven days in a coma – I don’t remember much other than the staff being very kind. Two days later I started experiencing delirium and was put back on dialysis to give my body a chance to recover. The next few days I struggled with breathing and coughing up the grey sputum from being on the ventilator during the coma and was unable to talk. This went on for at least two weeks and one of the physios brought me picture cards and a whiteboard to help me, but I still couldn’t communicate, which was so frustrating.
Posterior Reversible Encephalopathy Syndrome
I was still struggling with other problems when I suddenly had a massive seizure and a drop in consciousness and was put back into a coma while they tried to work out what was wrong. I had developed PRES (Posterior Reversible Encephalopathy Syndrome) due to a reaction to the medication and after an MRI of the head and a couple of days of medication changes, I was woken up for the second time and came off all ICU support.
A few days later I was moved to the post-transplant ward where I spent the next month. My recovery was very up and down, I had lost so much weight and muscle mass (20kg! I only weighed 72kg to begin with) so I was extremely frail. I went from not even being able to lift my head off the pillow, to just about sitting up, then starting to stand, to walking with a Zimmer frame, then finally walking with a crutch. It was a real struggle and I had to learn to do everything again – speak, walk, eat and write. But I was determined – I had Nanna Cheryl’s Boxing Day Party to get to and that became my target. Remarkably I ended up leaving hospital on 14th December and made it to the party!
Through it all I had some of the absolute best doctors and nurses I could have ever wished for. Their patience, kindness, dedication, skill and care was incredible and I am eternally grateful to them! Ten months on and I feel better than I have done in years; my fitness is almost back to my 20s, my meds have stabilised, my bloods are perfect and my post-hospital recovery has been ‘text book’. I’ve joined the gym and try to walk a few times a week and keep fairly active. The only thing I struggle with a little is getting tired at times, but if that’s all it is, then I’m good with that!
For anyone on the list or waiting to maybe go on the list KEEP GOING, it really can and does change your life! Good luck to everyone out there and thank you for reading my story!
