Sophie

Sophie was born at home in Nov 2022, with a much-anticipated home birth that all went well (and fast!). We noticed she was a very sleepy baby in those first few days, falling asleep after a few minutes of feeds. She was a little jaundice too, but so was her big brother in the first couple weeks so we thought she would just need a bit of time under the lights. It was when she had her first strangely hard and paler poo at 16 days old, and she hadn't put on any weight in 10 days since the first weigh-in post birth, on top of her extra sleepiness, that I knew something wasn't right - and so did my midwife. She sent us to CEC at Christchurch Hospital, told me to pack some snacks because it might be a few hours to get some tests done then we would be sent home. Little did we know, this would be the start of a very long journey ahead. We were asked to stay overnight as there were a few red flags in the liver markers of her blood tests, so more investigation needed to be done. Many, many tests later, Sophie had a liver biopsy just after Christmas with the results coming back after New Years. In hindsight, it was so nice to spend the holidays with our family before our world was really about to change.

At 5 weeks old, our doctor told us the biopsy results were back and that Sophie had a rare and incurable liver disease called Biliary Atresia (BA). Her bile duct was completely blocked, causing bile to back up into her liver, which in time causes irreparable damage and ultimately, liver failure. We had 2 days to process this new information, figure out life logistics with a toddler and a dog at home, before flying up to Auckland to meet the liver team at Starship first thing Monday morning. At 6 weeks 1 days old, S had a major surgery called the Kasai procedure, which isn't a cure but rerouting her bowel to create a path for bile flow to buy her time to grow before needing a transplant - hopefully years.

She recovered well from the Kasai, but the moment we landed back in Christchurch, her pale poos returned and this started our string of stays at Christchurch Hospital, 2 weeks at a time to treat suspected cholangitis (liver infection). One never got better, so Sophie and I were back up in Auckland with a prognosis of "give her some time" - to Sophie's credit, she did improve with time. Her liver function tests were improving each week and we had a good run for two months where life seemed apparently 'normal' for us as with routine hospital tests and reviews, including being well enough for her to have her double inguinal hernia surgery at 4 months old.

However, a month later, back to back viruses and infections started to take their toll and her Kasai was deemed to have failed. Her liver was severely decompensating so much that Sophie and I took a Life Flight up to Starship, due to her deteriorating state. She was really yellow at this point, had an enlarged liver and spleen, which made her tummy so big in comparison to her little body. Thinking back, Sophie was almost always smiling through it all - up until this point, I had always relied on the fact that Sophie never seemed miserable.

We arrived at Starship and Sophie was starting to really struggle with another subsequent infection - which happened to be Covid - and was under close watch, determined to need a transplant within the month. We ran through the transplant evaluation process for Sophie with our team, knowing time was of the essence.

Sophie finally recovered from Covid, and was well enough to officially be put to the top of the paediatric liver transplant list in NZ, just short of 7 months old. Knowing deceased liver donors don't come often - especially for the small size Sophie needed, the live donor process for myself to give part of my liver to Sophie was fast tracked and due to be scheduled ASAP.

Miracles happen - a short 4 days after being listed, we got the call when I had 24 hours left of tests for being a live donor. A deceased donor liver had come available that was a perfect match for Sophie. The relief was huge - Sophie could finally start getting better.

A marathon 11 hr transplant surgery went as well as it possibly could, much to our relief. She's recovered beautifully, with one small infection and one mild episode of rejection in the first 1.5 weeks post-transplant but - touch wood - she's been healthy, happy, enjoying the freedom out of a hospital bed and being able to get into rolling around on the floor properly and doing so well since. Sophie was discharged and we have spent our time keeping Sophie safe in a bubble at a Ronald McDonald House apartment (we are nearly at the 5 week mark). The Kids Foundation have been a huge help both while in and out of hospital, helping our family be together by sponsoring flights for Sophie's brother.

’We are incredibly grateful to the family of the donor; to give S the gift of life is truly heroic amidst their personal devastation. For our family, it was a phenomenal outcome in that Sophie can have life-saving surgery without her own mother being put at risk in a major live donor surgery.’

Vivian Wong,

Mum to Sophie.

Have a listen to Sophie and Vivian's Story on the Listening to Families website https://www.listeningtofamilie...