Many of the stories we share are from people born with biliary atresia who underwent the Kasai procedure as babies. But we’ve not heard from anyone who had their Kasai surgery before Sarah – hers took place in January 1983! Here is her story.
I was born in December 1982, and it is my good fortune that the obstetrician who delivered me had delivered my older sister four years earlier. Emma was born with Downs syndrome and it was a shock to my parents as it was unexpected. The obstetrician was particularly vigilant at my birth and promised my mum that if he had any concerns, he would arrange the necessary tests. As it turned out, I was jaundiced and so I was given the split bilirubin blood test which revealed I had biliary atresia. If it wasn’t for my sister, who knows if and when I would have been diagnosed?
Knowledge of liver disease in children then was not what it is now, and the Kasai procedure was very new. In fact, Dr Kasai had recently come over from Japan to Alder Hey Children’s Hospital in Liverpool and trained a number of paediatric surgeons including my surgeon, Mr Cook on this new “Kasai procedure”. I was transferred to Alder Hey for the surgery which I’m told was one of the first to take place in the UK and I think the first in Alder Hey. In fact, I’m told there was a write up about it in the medical journal, The Lancet.
I was given a 60/40 chance of survival. Thankfully, it was a success. I’m incredibly lucky that it was because at that time, liver transplants for babies were not an option in the UK.
Following the surgery, I went from strength to strength and it’s fair to say that my liver condition wasn’t something I really thought about much at all. My parents didn’t talk about it, I didn’t know anyone else with the condition and there was no Children’s Liver Disease Foundation or social media. I was lucky enough to be well, and so my condition didn’t have a real impact on my day-to-day life - I really didn’t have cause to think about it.
I was a sporty child. I played in teams for hockey, netball, rounders, rugby and even competed at county level in the high jump. I attended Brownies, played drums, took swimming lessons, got my Duke of Edinburgh award and was chosen to take part in a trip to Japan with five other students. I’m sure my parents worried about me but, growing up, aside from the regular six-monthly trip to see a friendly doctor, I honestly had no idea that biliary atresia was a serious condition.
The only thing that did bother me as a child was having a scar from my Kasai surgery. I didn’t show it to anyone and wouldn’t get changed in front of people in swimming lessons. I think it’s great that young people are now encouraged to be open about their scars but I certainly wasn’t comfortable with it – I think it was part of the whole ‘not discussing illness’ thing of my generation.
Because I was well throughout my childhood, having biliary atresia didn’t affect my education. I studied chemistry at university and because my natural inclination was to take advice when it came to my health, I didn’t drink. Luckily for me, I didn’t need alcohol to function socially, and I had friends who didn’t drink much so it wasn’t an issue.
Fast forward to 2009 when I’d married my husband, Richard, and we thought about having children. Now, I had been told by my doctors that I would find it difficult to conceive or hold a pregnancy and so I just accepted it, however, we thought we’d just try anyway as in an ideal world, we knew we’d love to have our own children. I had one miscarriage but, we tried again and after an uncomplicated pregnancy, I gave birth to a boy in 2012. We felt very fortunate and assumed this was just a fluke, yet with hope on our side, we tried again, and our second son followed in 2014! Both boys were born a few weeks early and although the first pregnancy was fine, with the second one, I was unwell for the four months prior to the birth. I was told it was an infection and was treated with antibiotics, but the infection continued after the birth.
At this point, the doctor explained that I was suffering from cholangitis and explained that we needed to start thinking about a liver transplant. I was really shocked. This was honestly the first time I’d been told that this was a possibility. With hindsight, I’m glad that it wasn’t at the back of my mind growing up, but it certainly was a huge shock to be given this news just after giving birth!
Two months after having my son, due to low bone density because of a calcium deficiency caused by the pregnancy coupled with the liver issues, one of the blocks in my spine had crushed. I have to admit that the next few months were very tough. I was in and out of Addenbrooke’s hospital with constant infections, I had a baby and a toddler, a husband working away during the day, a mum who cared for my sister who had Down’s syndrome, and I couldn’t walk due to constant spasms in my back! Luckily, I had a great GP, a great consultant, and great friends that enabled me to keep positive during a difficult time.
In February 2017, we got the call we had been waiting for that a suitable donor liver had been found and my liver transplant went ahead at Addenbrooke’s. My mum looked after the boys. I know she found it really hard seeing me in hospital – I’m sure it brought back all the memories of me being so poorly as a baby – but we were lucky. The surgery went well, and I haven’t looked back since.
Sarah at Addenbrooke’s with pioneering British transplant surgeon, Sir Roy Calne
I have check-ups every 6 – 12 months and all is good. Yes, I get tired, but I think that’s largely due to having children, work, general life admin and now, I’m probably also hitting the menopause phase! Who knows if my tiredness is excessive?
I run a Facebook group for transplant patients and I’m enjoying being able to contribute to research in liver transplantation through working groups and committees which is, of course, an area of huge personal interest. I’m currently working on a research project which is exciting as it is research which, if successful, will ultimately lead to more liver transplants being viable, and one day, may make a difference to people like me.
I know how lucky I have been, and I will always be truly grateful beyond measure to the family of my donor who allowed me to have such a wonderful gift. I really hope my story gives hope to others, especially parents of babies who may just have been diagnosed with biliary atresia. Life can be very, very good.
I had the kasai operation in 1981