Baby girl with rare disease to have liver transplant two weeks after having heart surgery
Suffolk parents Joe and Gemma Kean say a liver transplant will change everything for little Aria
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Your support makes all the difference.Devoted newlyweds whose adorable baby girl was whisked into hospital three days into their honeymoon and had open heart surgery two weeks ago are now on tenterhooks as the “smiley” tot awaits a liver transplant.
When within days of little Aria Kean being born on 21 October 2021 she showed signs of baby jaundice, including yellow skin, her parents, financial advisor Joe Kean, 29, and service advisor Gemma Kean, 27, were not worried as they knew the condition was common.
But when it failed to clear a series of hospital trips followed and, aged seven weeks, she was diagnosed with biliary atresia – a rare disease affecting the bile ducts, gallbladder and other structures involved in producing bile, which is made by the liver to aid digestion.
Aged eight weeks, Aria had a Kasai procedure – surgery that can help re-establish bile flow from the liver to the intestine by joining the two directly – which worked for a few months, but doctors went on to tell her parents she needed both a liver transplant and surgery to close a hole in her heart.
Now, one week after his daughter’s open heart surgery lasting four hours, Joe, of Red Lodge, Suffolk, said: “The liver transplant will change everything for Aria.
“She is so happy and smiley all the time, but she has never been well, she has only ever been poorly.
“Because of her liver, it has affected her hitting her milestones. With the transplant, she will be like a different baby, with all this energy.
“She only had heart surgery on Tuesday July 26 and she is already doing so amazingly.”
He added: “We cannot wait for her to have the transplant now.”
Hoping for a liver transplant in the next few weeks, if no donor is found, Joe will become his daughter’s living liver donor if it becomes an emergency – meaning he will donate part of his organ to Aria.
He said: “When the doctors told us that Aria would need a liver transplant, her mum and I both put ourselves forward, but we realised I would be the best option because Gemma is only nine months post pregnancy and my liver would be in a better condition.
“She’s our baby girl , so of course we would do it in a heartbeat.”
Joe added: “We know now that I am compatible and if she did need it, I could give it to her, which gives us that little bit of reassurance.”
After the health visitor noticed Aria’s yellow skin, a blood test at seven weeks old revealed that her liver was not functioning properly.
Joe said: “They booked her in for an ultrasound and could see that the gallbladder hadn’t formed correctly when she was born, which meant the bile ducts were building up inside her liver.
“They mentioned it might be biliary atresia (BA) but they needed to do a biopsy, which they couldn’t do straight away because Aria had a cold.”
Waiting a week for the biopsy, the day before Aria was due to go in, her parents became worried about the colour of her poo looking grey – a common sign of the disease.
Joe said: “We sent a picture to the hospital and they were certain it was BA and wanted to get her straight into surgery to attach her liver to her bowel while she was eight weeks old, because it is better to do it earlier as it is damaging the liver.
“Hearing that was horrible. She was a newborn baby about to go down for a major operation.”
The anxious parents were also warned by doctors that there was a 33 per cent chance the operation would not successfully repair the liver functions, meaning Aria would very likely still need a liver transplant.
Joe said: “It was really scary, but after the operation she was improving so quickly and all the nurses were saying that she was doing so well.
“For the first few months, it seemed to have worked. She was still behind on milestones, but she was smiling and getting better.
“We were home five days before Christmas, so we were able to spend it together, which we were really grateful for.”
Other than one blip in February, over the next six months, Aria seemed to be improving – but the signs of something being wrong were still there.
He said: “She was smiling, giggling and playing, but she still wasn’t the right colour and she wasn’t feeding anymore without the tube.”
Taking Aria to one of her hospital check-ups in May, Gemma and Joe were told Aria would still need a transplant, setting July 21 as the date for an assessment.
Knowing she would need a big operation imminently, the couple managed to plan their wedding so that Aria could be there on June 16 2022.
Joe said: “We were due to get married in June 2021 in Zante, Greece, but it was postponed due to Covid and we decided to do something closer to home, given that Aria was so poorly.”
He added: “We were married in a hotel in Bury St Edmunds, Suffolk, and it was really lovely.
“We had to be careful with keeping Aria safe from catching anything, but it was really special.”
But just three days after the wedding, during their mini honeymoon in Suffolk, Aria fell ill again and was whisked back into hospital.
After a series of checks, doctors at London’s King’s College Hospital decided to fast-track her liver transplant assessment – only then identifying that Aria had a hole across the two chambers of her heart.
Joe said: “We were absolutely heartbroken.
“The whole time, we had always said at least it was her liver and not her heart, so hearing it was her heart, too, was the worst news.”
Two weeks later, on July 27, Aria was sent for four-hour open heart surgery to fix the hole, which, thankfully, was a great success.
Joe said: “When doctors rang us to say that it had gone really well, we were so relieved.”
It will change everything, as her body has been slower to grow because of this.
He added: “It was hard for the first few days, because she was so upset and we were worried she may have had a stroke – but, thankfully, she hadn’t and really turned a corner.”
Now, a week on from the operation, Gemma says they cannot wait for her to have the liver transplant when she is ready.
She said: “Because she is so little, it’s so hard to show her how proud we are of her, because she has been so strong and brave to get through this.
“Once she has the liver transplant, the doctors have told us that we will be amazed with how quickly she changes.”
Gemma added: “She will instantly be a normal colour and she will start to hit her milestones.
“Even things like growing her teeth – they haven’t quite popped out yet – the doctor said he wouldn’t be surprised if within a few days, we see them.
“It will change everything, as her body has been slower to grow because of this.”
And while Gemma hopes Aria does not need an emergency transplant from her dad, knowing that he can step in and save her life if needed is a huge comfort.
She said: “Imagining Aria growing up and finding out she has a bit of her dad’s liver and knowing he saved her life would be incredible.”
Now friends have launched a GoFundMe page, to raise money for the little family to have some special times, during this difficult journey.
And, above everything, Gemma cannot wait to finally take Aria out for day trips and to treat her when she is well enough.
She said: “I can’t wait to go to baby classes with her or to finally take her swimming. Just to go out and about to playgroups and to take her to new places will be amazing.”
- To donate to the family’s GoFundMe, go to: https://www.gofundme.com/f/GemmajoebabyAria