James was diagnosed with PSC in 2006, at the age of 26
The odds seemed good
It was autumn 2005 and I was newly married when I got a dodgy tummy. It didn’t get better, and thus began my voyage into the uncertain world of IBD and liver disease. I was diagnosed with ulcerative colitis in spring 2006 and with PSC shortly thereafter. Fortunately for me, I was 26 and invincible so I didn’t really take on board that this was somewhat more significant than ‘man flu’ or headaches. Sadly for me, I had various family members with access to the internet and it gradually became apparent that things like the prospect of a liver transplant couldn’t just be brushed under the carpet. At the time I was told it was a 25% chance I’d ever need a liver transplant and, while you never know how it will progress, we were probably talking about 20 years’ time. The odds seemed good and so I just put it to the back of mind and got on with life.
In the mix of clinic appointments, blood tests, scans and bowel prep, it’s easy to forget that many people live with PSC with no great problems. With the uncertainty, everyone says to just take a day at a time and there can be lots of good days! For the next 5 years I had occasional UC flare ups but nothing too bad and my liver kept pretty quiet. I worked full time, I played sports, we travelled, got a mortgage and mowed the lawn. I was actually doing a passable impression of a grown up.
In 2011 we were expecting our first child and I needed a liver biopsy (I assume after some suspect blood results but I can’t quite remember). I was still a PSC newbie and so I don’t really know what they were looking for but in my mind it was cancer. It was the first time I had processed the thought of dying and as I lay in recovery I had a really good cry. On reflection, a book where the author describes his wife writing letters to their kids the night before she undergoes surgery for a brain tumour, was a poor choice of post-biopsy reading! Thankfully my wife came and read crossword clues to me.
The biopsy results were fine, my body settled back down and then came 8 more good years. We had 3 amazing kids. We moved house. I learned to cook risotto. I was accumulating adult points at a phenomenal rate! My life was filled with school runs, dance performances in the lounge, swapping shoes to the correct feet and endlessly searching for the vital stuffed toy kangaroo! It’s hard work and tiring but absolutely the best. In April 2019, my son and I enjoy our shared birthday and celebrate as he turns 3 and I turn 40 and, other than a slightly abnormal blood test, life is great and I am really blessed.
The PSC rolllercoaster started
In late June the PSC rollercoaster started on a series of seemingly endless downs that left my wife and I battered and bruised. My liver wasn’t happy. Blood tests led to an ultrasound, an MRI, liver biopsy and finally a three day mini break at the Royal Free spa and transplant assessment centre (for clarity, it’s not a spa!). I turned more and more yellow and fatigue hit me for six. My wife deserved a medal for running the house, helping the kids navigate the emotions of it all and regular visits when I was in hospital. We had a great support network from church and school friends but those months were an even bigger ball ache than the family planning related surgery I had had 2 years earlier!
In hospital I met many great people each with a different story of their journey with PSC. So many faced much tougher journeys than I did and it was really helpful for me to hear their stories. Around my healthy friends it was easy to feel hard done by and that my lot in life was particularly tough but I gained a much more healthy perspective from others with equally sketchy livers. My faith has always meant trusting in God but that became a lot more real as I reflected on what it meant to trust God even if, worst case scenario, this was the end of the road for me.
In about 10 weeks I had gone from my normal life to waiting to hear whether I was able to go on the liver transplant list. The operation that had sat in the back of my mind in the “please, no” category now appeared to be the great lifesaving gift. When the transplant coordinator called a week or so after my assessment, we discovered a new twist in the tale of my temperamental liver. Despite having been given no hope that there was anything that could be done to improve things, it seemed that during my ERCP they had managed to stretch the bile ducts and my bloods were improving. I was now too well to go on the list. Oh look, it’s a whole new bunch of mixed emotions! So pleased not to need the transplant but also disappointed to realise this was probably just delaying the inevitable and the journey was not over.
My family have developed huge resilience
The last few months of 2019 saw my blood tests largely heading back to a good place. My energy returned, the jaundice went and the routine of home life, school and work returned. But we were emotionally exhausted and, as the dust settled, we started to unpick all that had happened. My eldest daughter processed her reflections in a book she titled “The Time of Tears”. We have some scars now that remind us of the journey so far and also point to what seems like the inevitable journey we will have to walk one day. I want to talk of “if it comes” but it feels more like “when it comes” and I hope that this has at least prepared us for that time.
This is such a frustratingly unpredictable disease but I have so much to be thankful for in it and know so many have much harder battles. I have learned to be grateful for all the small things that are easy to take for granted. With the covid-19 shenanigans that have followed hot on the heels of my transplant scare, my family have developed huge resilience and strength and I couldn’t do any of this without them. I’ve also found it so helpful to get to know and hear from other PSC sufferers in their journeys and to be inspired by the determination so many of us have to fight this disease.
In writing this I’ve also discovered how easy it is to accidentally type “lover” instead of “liver” which has real potential to change the meaning of what’s said and bring confusion. For clarity, I have immeasurable fondness for my lover while, quite frankly, my liver can do one!
If you think you might benefit from speaking to other people affected by PSC, like James did, join our private Facebook group. It's a lovely, supportive community where people of all ages share their experiences and tips on living with PSC.