Ellie’s Story – Biliary Atresia

As told by Ellie’s mum, September 2017.

Ellie (front) and her sister Grace

When did you first know Ellie had Biliary Atresis (BA)?

Things were absolutely fine to begin with. Ellie was our first child, weighing in at just under 9 lbs- yes, she was a bit yellow after a couple of days, but we were assured by our family and midwives, who were all mothers, that this was a touch of neo-natal jaundice which would pass. In the meantime, we just got on with things. I was breast-feeding Ellie and she was absolutely ravenous. For the first few weeks, she could go longer than around 90 mins without feeding. Sleep was scarce, but we all thought that it was normal for a newborn.

At 4 weeks old, we had a home visit from Maria, our nurse. We stripped Ellie naked to be weighed- due to the sheer volume of milk she was consuming, she was doing very well weight-wise. After she was weighed, I put her on a white blanket on the floor. It was at this moment she decided to have a wee- and thank God she did! Maria noticed that Ellie’s wee was yellow. It had not occurred to me that this was unusual in a newborn. Maria said that she just wanted to organise a blood test and she would be back in touch. Brighton Hospital called us in for some bloods- we went, and got on with life as usual. The family were having a get together in Hampshire, which we went to. They all rallied to try and get me to have a sleep, but Ellie was still crying almost constantly. Anyway, we got back home, only to discover that there had been a mix up with the bloods. Could we come in again? We reluctantly did- we really did not suspect that anything was wrong. Whilst waiting for the results, we got on with life again. My husband drove me to Bournemouth where I was performing with the Bournemouth Symphony Orchestra. We made the 3 hour journey and my Mum was going to meet me to help look after Ellie whilst I was working. My husband drove back home. Waiting for him when he got back was an urgent call from the hospital asking him to call the doctors asap. D’Arcy told me that something was wrong so I called the doctor myself to hear what the problem was. Words that meant very little to me were bandied around such as “conjugated bilirubin”,- I couldn’t believe that there was something urgently wrong, but we were told to get Ellie to hospital right away. My husband got back in the car to pick us up.. and we started our Biliary Atresia journey!

We were soon transferred to Kings College Hospital in London. This was the scariest time. On the liver ward with, quite obviously, very poorly children, we were completely dazed. Ellie went through a major round of tests, including a liver biopsy which the doctors advised me not to attend. It was simply awful. However, after a couple of days, we had a diagnosis. Biliary Atresia. I was told to stop breast feeding, as Ellie was unable to digest the milk. That was why she had need so much for all these weeks. Apparently I had been feeding triplets!! They got her onto a particular formula, and she finally got some sleep, as did I.

We were sent home for a week, whilst the preparations for her Kasai operation were made. We went to Centre Parcs with the whole family, knowing that in 2 days, Ellie was having her major operation to create a new bile duct. She had her operation on the Monday, and it was relatively successful. But it was a slow starter.

How did this affect her day to day as a baby, toddler etc?

Ellie was very slow to get mobile- she did not stand by herself, or crawl, until she was 1 year and 10months. How much this was due to her condition, we don’t know. The main impact on her on a daily basis was the medication. There was quite alot of it, including the dreaded Questran- a “wallpaper paste” med that often made a return visit. Essentially, apart from the hospital visits etc, we just got on with it, and made sure that life was as normal as possible for us all. Things did become much more difficult in the latter stages, however.

What support was offered to Ellie and the family?

We had great support from the Children’s Liver Disease Foundation – particularly at diagnosis from their parent packs etc. They work closely with the main liver units, and we were able to get any questions answered through them, if no doctor was available. In subsequent years, we have been fortunate to get a place on their outdoor weekend courses for children with Liver Disease. Ellie really enjoyed this, and she met some lovely children, all in the same, or a very similar boat.

We cannot praise the support of King’s College Hospital enough. Their exemplary care changed all our lives.

What did Ellie know about condition and prognosis?

We always kept Ellie as well informed as her age would allow. We were unsure for many years how long her liver would hold out for – the doctors predicted that she would probably need a new liver around the age of 8. The problem was that Ellie’s Kasai really had taken a bit of time to kick-in and her liver was scarring until it did. Irreversible damage was done, and although it would work well enough for the time being, it would almost certainly not last a lifetime. We asked about living donation, but the doctors felt it was too early to discuss this. As Ellie got older, the word “transplant” was mentioned more frequently. We had to, at this point, talk to Ellie about the potential operation on the horizon. Obviously this threw up tricky questions about the prospective donor, the risks etc but we were honest with her. In an age of Dr Google, I think it is unwise to be otherwise. We were listed for transplant in October 2016.

What attitudes did you experience from people regarding the condition?

As a family, we have been very fortunate to have had nothing but fantastic support from everyone. The sad truth is, however, as Ellie got older, she encountered issues from children her own age. She inevitably became less active as her liver became less able to cope with the demands of a growing body. She was always bleeding from the extreme itching that came with her condition. This meant that she was constantly tired due to lack of sleep. She was called “Simpson”, after our little yellow friends, on the bus. In all honesty, she became a rather sad, tired, poorly little girl. Sleep-deprivation, twinned with horrendous pruritus, was the very worst of it all.

Tell us about the day when you found out the liver was available.
My husband had just climbed the Yorkshire 3 Peaks!! He was a broken man. I had stayed at home with the children- by the phone as usual. We had an early night- and thank goodness we did. We got the call that a liver was available at midnight. The ambulance arrived within 7 minutes, and they blue-lighted us to London. My husband was still suffering from his walk- it was not a comfortable journey. We were on the ward by 5am, waiting expectantly.

Having expected the transplant to be a race against time, we were bewildered to still be on the ward at 3pm, waiting. We truly believed that it was not going to happen. However, it did. We subsequently found out that our donor was on life-support, and they were going to turn it off once everyone was ready and in place for the incredible multiple transplants that had been arranged following the family’s consent to do so.

This was a terrifying day and night. She went to theatre at around 5pm, and we got to see her afterwards on ICU at 4am. A long night. Nothing prepared us for how she looked afterwards. Let’s just say she was utterly dwarfed by the technical equipment around her. She had so many tubes connected to her that we could barely see her amongst it all. The care was absolutely unbelievable.

Amazingly, we were home just 12 days after the operation- testament to our brilliantly strong little girl and to the incredible work done by the NHS.

Since the operation, how have things been?

It has been 6 months now, and things have been remarkably different. We saw the whites of Ellie’s eyes after just 24 hours- that in itself was a special moment. She had never had white eyes before. The itchiness stopped immediately and this had been such a massive issue for us all. Her appetite changed- she is able to eat less frequently now. She actually became nicer! The sleep deprivation had taken a huge toll on her well-being, her daily moods, her capacity to think. She had been sleep-deprived her whole life. She now sleeps like a teenager! She is now more confident in herself because she no longer scratches uncontrollably. Essentially, things are great. The medication is quite extensive, and she will be on this for the rest of her life, but Ellie finds tablets easy to take, so it is not too arduous. The one thing is that we all know how important these meds are. We collectively think about it every day as we mustn’t miss a dose if at all possible.

We are all very aware of the debt we owe to an incredible young woman who tragically lost her life, but was able to save our daughter and others. We continually thank the NHS for their incredible care and support. We owe them our family.