Many of the young people we support are living full and active lives thanks to their liver transplant. Jane’s daughter, Naomi, celebrates the 27th anniversary of her liver transplant this month. Here is her story.
Naomi was born two weeks late on 12th October 1992 after a normal pregnancy. She was very jaundiced (looking back at photos she is orange!), something which my son Oliver hadn’t suffered with, but I was assured this was something that would clear up quickly. She fed voraciously, taking 4 to 6 ounces of milk at once and never seeming to be satisfied. She cried with hunger all the time. When Naomi was around three weeks old, I had to take Oliver to the doctors and while I was there, he asked if everything was ok with the baby. I said that I was concerned that her eyes were still yellow. He examined her and thought that her liver felt enlarged. It turned out that the previous week, his wife, who was a nurse, had read an article on biliary atresia and alarm bells sounded for him.
Jane was concerned about Naomi’s jaundice
After various blood tests and examinations, Naomi was referred to Leeds General Infirmary where she was diagnosed with biliary atresia at six weeks old following a liver biopsy. At this time, there hadn’t been a case of BA for two and a half years in Leeds. There was no literature available to inform parents – no glossy leaflets or booklets. One of the sisters on the Neonatal Surgical Ward drew diagrams for us so that we could better understand the diagnosis. Naomi had her Kasai on 26th November and we crossed our fingers. Unfortunately, over the next few months it became clear that the Kasai had not worked sufficiently well for Naomi and that a liver transplant would be necessary at some point in the near future. Naomi was put on Pregestimil formula that contained pre-digested fat allowing her body to absorb her food more effectively. The smell was disgusting!
Naomi did start to wean normally and as well as her yukky milk she started to drink baby juice, but she then suffered two extremely serious bouts of cholangitis and stopped eating completely. Her tummy was so swollen with fluids around her liver that I suppose she always felt full. She had to have a naso-gastric tube and she was fed by pump overnight. I had to learn to pass the tube when it needed changing, otherwise she would not have been able to come home. She had a central line fitted and I had to learn to flush this once a week.
When Naomi was around seven or eight months old she began to require blood transfusions. She had a build up of pressure in her blood vessels caused by the advancing liver disease and this was causing them to bleed. As fast as the blood was being put into her she was losing it again. Her condition was so serious that she had to go for a transplant assessment at St James’ Hospital in Leeds. After various scans, blood tests and other examinations, Naomi’s portal vein was found to be narrow which would make attaching a donated liver more difficult. At the time St James’ only did transplants that were straightforward, and this new difficulty resulted in Naomi being sent to Birmingham Children’s Hospital where they were more used to dealing with tricky situations. As we lived in East Yorkshire this was an extremely daunting prospect as Oliver was only three at the time. Anyway, Naomi underwent the transplant assessment at Birmingham and was listed as extremely urgent in December 1993.
On February 23rd 1994 at around 9pm we got the phone call we were desperate for but that we had been dreading and we set off in thick snow arriving in the middle of the night. Naomi went into theatre at the Queen Elizabeth Hospital Birmingham at 9.30am on February 24th 1992 and came out of theatre at 5.30pm. When we went to see her in ICU the first thing I noticed was that she had changed from a horrible grey colour to a lovely pink. Her liver was working well.
Jane with Naomi following her transplant
Naomi received part of the liver of a 26-year-old lady who had suffered a brain haemorrhage.
Immediately post-transplant Naomi started to eat solids and didn’t require her naso-gastric tube anymore. She took full advantage of the increased steroid appetite! On day 16-post transplant, she was allowed to go home!
Naomi went from strength to strength and she and Oliver were able to at last have a normal life. She joined him at playschool and then two years after he started school, she did too.
Unfortunately, after only six weeks at school, Naomi developed autoimmune hepatitis. She was put onto large doses of steroids and had to be home schooled for six weeks as her immune system was non-existent due to the steroids and her other immunosuppressants. Although there is no cure for AIH, with the help of the treatment, Naomi returned to school and never looked back. She progressed through primary and secondary school with relatively few problems and had the normal life that I hoped that her transplant would give her. She passed all her GCSEs with flying colours and went on to achieve an Extended Diploma in Sport at Hull College.
Naomi had started to attend the British Transplant Games in around 2005 and was one of the first children to represent Leeds Little Livers. In 2007 she was selected for the GB team to go the World Transplant Games in Bangkok, Thailand then in 2009 we went to the World Games in Gold Coast, Australia and in 2011 to Gothenburg in Sweden. Naomi achieved a wonderful collection of medals in gold, silver and bronze competing in cycling, 100m, long jump, badminton, table tennis, high jump and the 4 x 100m relay. Around the time of the Sweden games, Naomi began to have problems with her back and eventually was diagnosed with degenerative discs in her lumbar region. This really put paid to her competing, but she has been back to the British Games and the World Games in Newcastle to keep in touch with her friends.
Naomi at the British Transplant Games in Belfast 2011
It was found in 2015 that Naomi had Epstein Barr Virus (the glandular fever virus) and this really played havoc with her medication levels and her general physical condition. Although after a long struggle with it her body has suppressed the virus for now, she suffers with chronic fatigue as a result.
Additionally, because of her AIH Naomi has been on Prednisolone since she was five and is now steroid dependent, her body does not any longer produce any natural steroids. As a result of this she was put on Hydrocortisone and has to carry an emergency injection with her wherever she goes. Her body is at risk of going into shock and this can be fatal.
Despite all of these problems, Naomi has always been and remains the most positive of people. She never dwells on anything and deals with a problem and moves on with her life. It is now 27 years since Naomi received her transplant and her liver, despite the AIH, for now remains healthy and is working well.
Naomi has worked since she was 17, both in retail and supporting children with autism and special educational needs. Unfortunately due to the recent Covid- 19 situation, she lost her job in a local garden centre but she has kept herself busy with growing vegetables and flowers and baking endless amounts of bread and cakes.
All of those years ago I hoped that my little girl would live a healthy, happy life for however long her transplant lasted. I never dreamed that I would be writing about it 27 years later and being able to talk about it with my 28 year old daughter.
All those years ago, CLDF was very new and consequently we didn’t have much contact with them, but I have always supported them and donated to them. I realise the importance not only of supporting families through a horrendous time, but of the research that in the future may mean a better, easier way of helping children like Naomi to lead a normal, fulfilling life.
I hope that other parents can take something from Naomi’s story. Although she has obviously had problems, some quite challenging, without her transplant she would not be here and I would not have had to privilege of watching that gravely ill little girl blossom into the determined, bloody-minded young lady she is now.
I am forever grateful for the decision taken by Naomi’s donor family. When they were losing their daughter, they chose to allow my daughter to live. Every minute of Naomi’s life has been a celebration of organ donation and transplantation. I would urge everybody, whatever your circumstances, to please speak to your family and tell them your wishes.
Because I was so young when I was diagnosed with biliary atresia and had my transplant, I don’t feel that I was greatly affected by it. I never really knew anything different – hospital and clinics were just a part of my normal life. Thankfully I was always out of hospital for birthdays and Christmas so family life always felt as it should have, happy. As I have grown up and become a young adult, I have found that more challenges have arisen both due to my liver and the conditions that I accumulated over time. In school, the only thing I ever missed out on was the swimming lessons and thankfully I am now able to swim because my mum and dad made it a priority. My academic life was thankfully full and successful, I was able to compete my SATS, GCSE’S and also complete a national diploma at Hull college. If anything, I think having a liver condition presented more opportunities and had more of a positive impact when I was young.
My message for all young people is to embrace everything that life throws at you. Positive and negative experiences make us who we are. Even though I have been through a lot and it does affect me more now than ever, I would never change a thing. I have had the most amazing, normal upbringing and in my opinion that is what is the most important thing.
Covid especially, has for the first time in my life, made me realise and have to accept that I am far from normal, but we have all been through worse and there is light at the end of the tunnel. Isolation and shielding is tough but it is there to protect us, make it the best experience you can and be proud that you are living every day in the best way you can!
Naomi and Jane at Jane’s wedding in 2018