When childhood liver disease means that a baby has a difficult start in life, it’s natural to feel fearful of the future. So we’re very grateful to Ryan for sharing this update on his daughter, Aria, who is now three and a half.
Aria was born in January 2020 after a normal pregnancy with a very quick labour. She was quickly whisked away for some support for a few hours and she was returned to us for what we believed to be the start of a healthy problem- free life. Over the next few days, however, we had still not been discharged from hospital as Aria was struggling with dangerously high bilirubin levels which causing severe jaundice. Extensive light therapy did not help and further tests indicated that she may have a much more serious underlying problem.
When Aria was 12 days old, my partner, Autumn, and I were taken into a small room with a consultant and advised that Aria was being prepped to fly down from Glasgow to Leeds Children’s Hospital where they had a specialist centre for children’s liver conditions. The team in Glasgow believed that Aria had biliary atresia, a rare childhood liver disease which could be fatal if untreated. The only way to accurately diagnose this was through surgery for a first-hand look at the biliary system. Aria was scheduled in for this to take place at 17 days old but before we even got that far, she developed another unforeseen issue. Her small intestine became blocked and she required major urgent surgery to repair this. Three days after this she underwent her laparotomy and the diagnosis of biliary atresia was confirmed.
A tough first year
Those first few months were very difficult. Aria spent most of her first year in Leeds and underwent numerous surgeries. By September though, she seemed to be doing well, she was allowed home and was enjoying playing with her brother. Out of the blue one evening, however she suffered an ischemic hit to her liver which caused irreversible damage. We were sent straight back to Leeds, tests were rapidly carried out and we were met with the transplant team who explained that Aria was in grave danger and had approximately three weeks to live without a liver transplant.
I stepped up immediately and told them to do what tests they had to do and within minutes they started the assessment. I expected, that as her father, everything would be fine and she would be in safe hands to go forward but early the next morning I received a call saying that even though I was a match in most aspects the section of liver required for her would simply be too big and we had to get Arias mum to travel down to Leeds asap to start her assessment for compatibility.
Autumn arrived that same evening, underwent the same preliminary tests and by the next morning was confirmed as the perfect match. Over the next weeks, she underwent countless tests, scans and assessments to ensure she was not only physically able to provide the organ but mentally too.
On October 15th 2020, Aria received a liver transplant from her mum. It took Aria two weeks to wake up from the surgery and in that time she required a few other surgeries to help maintain her recovery but the signs were all pointing in the right direction; she would spend the next few months in Leeds finally being cleared to come home just a few weeks before Christmas.
Long road to recovery
Aria spent most of the following year in hospital in Glasgow. She was being supported nutritionally via TPN as she could not absorb food with her gut. In addition to building Aria’s strength, we received extensive care training so that we could safely manage her condition outside hospital. At the ned of the year Aria was well enough to spend her first Christmas at home. She managed a few weeks at home before we got a call from Leeds saying they could try a surgery that may or may not work but could prove life changing if it did. We agreed to trying this out.
So in January 2022 Aria once again made the trip down to Leeds with her mum and underwent this surgery to close a stoma, do a little bit of rewiring and a check of her internal repair work. It went much better than expected and in the coming weeks she made it back up to Glasgow again. Here we agreed with doctors to start the process of switching her from TPN to solid feeds through a gastrostomy tube which would take time but would take the stress of the TPN off the liver.
By July of that year, Aria had exceeded every target set and was fit to come home. This time there were no other surgeries planned, no big tests, nothing other than three monthly clinical check-ups. She was finally free to start her life at two and a half years old.
I have to admit for those first few months, despite us knowing she was doing great, we were constantly looking over our shoulder, nervously thinking something bad was going to happen but other than the odd cold or wee bug she never got liver sick again.
Bounce back year
In February 2023 just after Aria’s third birthday, our family celebrated a major milestone when she joined her brother, Edan, in mainstream nursery – something we never thought we would see. We didn’t think she could make it into a “normal” nursery or have a “normal” life but she showed us otherwise. For everything she has been through in previous years, she was determined to show us 2023 was her bounce back year.
In March Aria had her first holiday when she flew to Disneyland Paris and had the most amazing time, meeting all her favourite characters, watching the fireworks and loving the parades. She enjoyed it so much that we went back in June and again in September!
As a parent, when you go from sitting by your child’s bedside in the intensive care unit looking at their motionless body hooked up to countless machines with random beeps and sounds, to a point where your child is in Disneyland laughing, dancing, having tantrums and driving you crazy, that’s something you really don’t take for granted. When you’re sat in that lonely ICU room you don’t ever see a way out. You’re a robot – you go to bed at night hoping for sleep then go back to that seat beside your sick baby over and over for however long it takes.
We had a very difficult situation with Aria. Her condition didn’t come with a “how to fix” book; the surgeons had to make up a lot of it as they went along; so much of her early life was trial and error, but from her they have learned so much that will help other kids.
To any parent who is in the midst of not knowing which way is up and your life consists of hospital, hospital, hospital, there is a way out of it. You just need to have faith in your professionals, ask questions and don’t be shy about challenging decisions or asking why. One thing we learned as parents of a gravely ill baby is that they are stronger than we are and a whole load more resilient too.