What’s it like to be a “proper grown-up” with a childhood liver disease? Here, Giles Hider (aged 48), who has alpha-1 antitrypsin deficiency, shares an insightful perspective.
When I was born in September 1976, there was no Children’s Liver Disease Foundation (CLDF). My mother recalls that I developed a significant pot belly during my early years, and when I was three, tests revealed an enlarged spleen.
This issue only became apparent when I was eight years old. Like most children in 1984, I yearned for a BMX bike. This desire triggered a warning from my mother, who had read about BMX riders sustaining spleen damage from falls. She consulted our GP for advice, and we were referred for further tests. This time, the results confirmed that I still had an enlarged spleen but also revealed the presence of alpha-1 antitrypsin deficiency.
Alpha-1 is a genetic disorder characterised by the need for two faulty genes, one from each parent. Due to the limited understanding of the condition at the time, I was invited to participate in additional tests at King’s College Hospital to support research. I even recall a researcher from King’s College Hospital visiting our family gathering to collect blood samples. During another visit, I was informed that I would likely require a liver transplant in the future. My mother remembers me expressing my determination not to undergo such a procedure.
After that, apart from six monthly hospital visits, life remained largely unchanged for me. However, due to my enlarged spleen, I was advised against participating in contact sports.
Similarly, during my university years, I felt no different from other students. However, following my doctor’s recommendation, I refrained from smoking and alcohol. Whenever people inquired about my reasons, I would simply explain. Honesty with others earned me their respect, and I never felt pressured to engage in drinking.
It was shortly after university that my only significant disappointment in life occurred. I had always aspired to join the Royal Air Force, but I was informed that I was ineligible due to my condition. Consequently, I decided to pursue a career in aircraft design and manufacturing.
In 2002, I relocated to Somerset to work for a helicopter manufacturer. It was here in Somerset that I met my future wife. We tied the knot in 2007, and I must admit, I indulged in a glass of champagne after the ceremony. Before starting our family, we requested that my wife undergo testing for alpha-1 antitrypsin deficiency. Fortunately, the results revealed that both of her associated genes were normal. We now have a son and a daughter, both of them are carriers of the condition with one faulty gene (similar to my parents).
In 2012, I visited Birmingham Hospital and met with a consultant there. The consultant informed me that my liver cirrhosis had progressed to the extent that I would require a liver transplant within the next five to ten years. A few years after this diagnosis, I developed ascites, a common side effect, and commenced medication to manage it. As the years passed, I began to experience further symptoms as my liver struggled, including yellowing of the skin and eyes, leg cramps, and temporary memory loss. After meeting with my consultant at the Bristol Royal Infirmary, I was referred for a transplant suitability assessment at the Royal Free Hospital in London. This assessment resulted in me being placed on the transplant list in July 2023.
We were informed that a call could come at any time, day or night, and that I would have to immediately leave everything behind and travel to London for surgery. After being placed on the list, I received three calls, each requiring me to leave my family and travel to London. However, on each occasion, while waiting to undergo surgery, I was informed that the potential donor was not suitable. This left me with a mix of emotions, not only for myself but also for the families who had lost loved ones. Nevertheless, I had to trust the medical staff’s judgment and believe that they were making the best decisions for my well-being.
On receiving my fourth call from the Royal Free Hospital last October, I embarked on another journey to London. This time, I was given the green light and underwent surgery at 9 pm that very night. I spent two weeks in the hospital, receiving exceptional care from the dedicated staff. I am deeply grateful for their support and excellent care.
During my stay, a research associate kindly collected samples to support their research into alpha-1 antitrypsin deficiency, and potentially find a future cure. Additionally, I had the privilege of meeting a few student doctors, providing them with valuable insights into the condition.
After my discharge, I made weekly visits to the consultant at the Royal Free Hospital for a month. After which I was referred back to my Liver coordinator, Sarah, at the Bristol Royal Infirmary. Currently, I am taking six different types of medication. Although I still have alpha-1 antitrypsin deficiency, the liver transplant has effectively cured me of the effects of the condition. The symptoms that plagued me before the operation have vanished, and I am now on the path to recovery, pushing myself towards being able run 5 km again.
I would like to express my heartfelt gratitude to my donor’s family. I am incredibly thankful for their selfless decision to donate their loved one’s liver, giving me the chance to see my children grow up. I genuinely hope that my story serves as a source of inspiration and support for children with this condition, and their parents. My hope is that the research I have contributed to will eventually lead to a cure.
To anyone living with this condition, I strongly advise that you continue to live your life to the fullest and don’t let it hold you back.
