Ada Rose was born on 30/03/15 after a straight forward pregnancy. After 24 hours we were discharged home, as Ada was perfect and healthy (or so we thought). On day 2 the community midwife mentioned she was slightly jaundice but not to worry as this was normal, again her jaundice was mentioned at about day 10 but without concern. At day 18 the midwife came to see Ada and advised her jaundice had cleared, to be honest I hadn’t worried about is as I thought all babies had jaundice and it was nothing sinister. The week after Ada was still jaundice and the whites of her eyes were really becoming yellow, people were starting to comment on her colour. On day 30 somebody made yet another remark about her colour, this set off alarm bells and made me think maybe this wasn’t normal after all, so I googled ‘prolonged jaundice in a new born’. This was to be my first visit to the CLDF (childrens liver disease foundation) website. At this stage I realised that prolonged jaundice in a new born was in fact not normal and indeed something to worry about. This is where I first saw the words ‘biliary atresia’ A rare and incurable, life threatening liver disease. To my horror the 3 main signs of this disease are prolonged jaundice, pale stools, and yellow urine, of which all 3 Ada had. I was a first time mum and knew no different, I had mentioned the colour of her stools to countless professionals but told ‘not to worry’ ‘all babies poos are different colours’, and Ada had done a wee on the midwife so the colour of her wee hadn’t alarmed me.
When I saw this information on the CLDF website my heart sank. I booked an emergency GP appointment and voiced my concerns. The GP said he wasn’t too worried as she wasn’t ‘that yellow’ and to come back in a week if I wasn’t happy. I knew from the information on CLDF website that early detection of Biliary Atresia was crucial, so refused to leave my GP’s without a same day referral. Thankfully my GP agreed and we were in our local hospital within an hour.
Several bloods tests and ultrasounds later, our nightmare was confirmed. Our precious daughter did have biliary atresia and needed urgent life saving surgery. Less than 48 hours later we handed our 5 weeks old baby girl who weighed only 7 pounds over to the surgeon and his team, in a hope that they could restore bile flow and save Adas liver with a procedure called the Kasai. I have never experienced pain like I did during these days, I would go through labour a million times again given the choice.
Ada was in theatre 7 long hours, and all though the operation had gone well we didn’t know whether or not it had been a success. This procedure is only successful 60% of the time, for the remaining 40% the only hope of survival is a liver transplant, usually in their first year of life. So we just had to wait and hope and pray that Ada would be one of the lucky ones. Unfortunately even when the kasai operation is successful lots of children develop further complications and go on to eventually need a transplant.
Luckily for us Adas operation was confirmed to have been successful 6 weeks after her operation. I believe this is down to several factors but mainly her amazing surgeon Mr Alizai (who will always be our hero) and early detection of the disease. Studies show that the best time to perform the kasai is 4-6 weeks, so Ada was bang in the middle. Late detection of the disease means further damage to the liver, as with Biliary Atresia the bile ducts are blocked or inflamed so the bile builds up and poisons the liver. After 12 weeks the liver is so badly damaged they are unable to perform the Kasai and the only hope would again be a transplant.
One of the things that helped get us through those long 7 hours Ada was in theatre was a visit from a representative from CLDF. He sat with us for several hours, giving us stories of hope, stories of other children living with this disease, and answering my millions of questions.
Biliary Atresia is just 1 of over 100 childhood liver diseases. 2 children each day in the UK are diagnosed with liver disease, yet CLDF are the only charity that supports families like ours. They are a tiny charity with only a handful of paid workers, who rely mainly on donations and families of children they help fundraising.
I honestly do not know where we would be today without CLDF, their online information was the reason Ada was diagnosed early, which I believe contributed massively to the success of her surgery. The support we have been given by them over the last 3 years has been incredible, putting me in touch with other families in similar situations, which has been a huge help to me as I felt so alone, and like I was the only mum in the world to have a child with this disease. They are also constantly there to answer questions, no matter how trivial, and work tirelessly trying to find a cure for Biliary Atresia and other liver diseases and raise awareness of the signs of liver disease in young children. I have no resentment to the professionals that did not pick up on Ada’s disease as it is so rare and there is such a lack of awareness, but hopefully this is something CLDF will start to change.
When Ada was first diagnosed my world fell apart and I really struggled to cope, I decided to throw myself into fundraising and try and turn what I felt was a negative situation into a positive and try and repay CLDF for all they continued to do for us. Even though this helped, I still managed to lose myself during this time, and lost sight of my hopes and dreams. After lots of support from family and friends I finally feel like I have come through this horrible time. With this I decided that along with several of my family and friends  i would attempt to tick one off my bucket list and jump from a plane at 10000ft and raise as much money for CLDF as possible.
Currently Ada is doing very well, she has just started nursery and is enjoying life like most three’nagers’, but one day she will more than likely need a transplant, and I hope that CLDF will still
be about to offer her the support that she is going to need. Without fundraising efforts like ours this charity would not be able to continue, and they would never find that precious cure for our little girl.

I cannot thank you enough for your support. As a family it means so much to us.

Forever grateful

The Butterfields