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My future was a bit of the unknown

We’re delighted to bring you the story of Louise who was born with biliary atresia and still has her native liver – 35 years later!  We hear first of all from Louise’s mum Alison.

Louise and her twin Bryan were born in Edinburgh on June 11 1984. We were kept in hospital for a week and I noticed straight away that Louise’s first dirty nappy was white. I immediately expressed my concerns to a nurse but was told that this was normal for the first one. I already had two children and had never seen a nappy this colour, but even though the nappies remained white all week, nursing staff just told me to ‘wait and see’ how it progressed. When I asked to speak to a doctor about it, she told me that Louise was only a little jaundiced and it would pass! I was asked to bring her back when she was six weeks old for the standard new-born check-up and we were sent home.

Louise was a very unsettled baby, she cried a lot and never slept for long. Her nappies continued to be pale and when she was three weeks old, I took her to the doctor to be told again that it was jaundice and it would pass.

When I took Louise for her six-week check, I told the nurse of my concerns and what I had been told. The nurse could see that she was yellow and said that if it had just been baby jaundice, it should have passed by now. So she asked the doctor to take a look. This was when we were taken seriously.  The doctor examined her, took bloods and she was admitted to the Sick Children’s Hospital in Edinburgh for further tests.  We were then told that Louise had biliary atresia. The doctors explained what the condition meant and said that the only treatment available for this condition was a liver transplant which was not yet available in the UK.  We would have to take her to the USA at a cost of thousands of pounds. Otherwise there was nothing which could be done – Louise would not live longer than two years.  It was devastating news.

Our first thought was how we could start fundraising to get Louise to the USA but the next day a surgeon named Mr MacKinlay came to see us and told us about the Kasai procedure.  He explained that it was a relatively new procedure and that only one had been carried out in the UK.  The surgery involved joining a loop of bowel to the under surface of the liver in order to establish bile flow. Mr MacKinlay told us he would be willing to perform a Kasai procedure but that it had to be done before Louise was ten weeks old due to growth of the organs.  He also explained that its success depended on the condition of her liver and that there was no guarantee she would survive the surgery.  This was a very difficult decision for us but we felt we had no option but to proceed.  It was her only chance.  Mr MacKinlay agreed to perform the procedure and we were allowed to take our baby home to spend the weekend with her family.

We brought her back on the Monday and, following a couple of days of preparation, Louise had her Kasai. She was eight weeks old.  The surgery took nine hours and afterward she was in intensive care.  I sat with her all through that first night and prayed and willed for the bile to flow. I can’t recall now if it was the next day or the one after, but I will never forget that first nappy – it had colour! It was greenish and light brown.  When I saw Mr Mackinlay doing his rounds, I approached him to tell him and ask if that meant the operation had been successful. He replied, “Oh my, well if that is the case then yes indeed it is successful”. I never forgot those words.

Louise recovered well from her Kasai and for her first ten years or so had to attend hospital for a check-up every three months. It was then reduced to every six months.  Because she was the first success in Scotland – second in the UK – Mr Mackinlay talked about her and her surgery in his lectures and I believe she was also referred to in some medical journals although I have never seen these articles. Although we were told that Louise had an enlarged spleen, her tests results were generally good.  We knew that the future was unknown as her surgery had been at a pioneering stage, but I don’t recall ever being told that a transplant would be needed in the future.  We raised her no different to her siblings in that she played and did all the things that any well child would do. For us it was important that she was positive and happy and did not feel any less able than her peers.

Louise’s story:

Throughout my childhood I had regular visits to the hospital for ultrasounds and blood tests. They didn’t scare or bother me- it was fun getting out of school- the worst part was having to fast before an ultrasound. After that though I got to choose where we went and what I had for lunch and that made up for it.

When I turned 17 I was transferred from the Sick Kids hospital in Edinburgh to an ‘adult’ hospital, where I was diagnosed with portal hypertension. This had created oesophageal varices and these required banding. Banding is the process in which they use tiny rubber bands to tie around the base of the vein to cut the circulation from them and they shrink. If they grow and burst out then they will drain the blood supply from my liver and I would be in great danger. However they never disappear for good and require regular monitoring. Varices may only be banded once they reach a certain stage so constant monitoring (by way of an endoscopy) is necessary. Until I was checked for them I was completely unaware they existed in general and in me.  They don’t affect me in any way except when they are banded-it’s rather sore afterwards for a few days. I have had banding done now over 30 times.

I still don’t have any regular medication as such- I do suffer from migraines and therefore I have ‘sumatriptan’ and also I have cocodamol for general abdomen pains. I still attend the hospital at least five times a year.  I see the consultant, have bloods done and scans every six months and also endoscopy with or without banding as necessary.

I lead a busy life. I work full time and have a 14 year old daughter. My pregnancy wasn’t as easy as it maybe should be, I had a few extra appointments due to my health-they just wanted to be sure my body was coping. I was taken to hospital by ambulance after a midwife came to visit me at home and my blood pressure was really low (I felt absolutely fine).  I was suffering from pre-eclampsia and subsequently my daughter was born naturally but induced early for both our safety.  Because of my liver health I wasn’t allowed an epidural or any other drugs. I was just given gas and air. My daughter was born perfectly healthy and is now 14 years old and five feet nine inches tall!!

As well as my full-time office job, I also recently trained as a lash artist so I am building up my skills doing semi-permanent eyelash extensions in my spare time. Some days I work 15-16 hours! I sometimes get days or weeks where I am more exhausted than usual but as long as I listen to my body and have an early night or a wee nap when necessary then I am fine, and don’t we all have days like that anyway?

I was always told that my future was a bit of the unknown, that we would just see how I go but, apart from the addition of portal hypertension when I was 17, things have been pretty steady. I do sometimes feel like I’m waiting for it to fail though. And people say the word ‘transplant’ like it’s the answer to my issue when in fact it may not be.  I would have to ‘deteriorate’ first then be assessed and placed on a list.  I am always assured by my consultant that if my liver were to start failing it would be slow and gradual…not sure how assuring that actually is?!  Also I am increasingly aware of my other organs being overstrained and possibly being too worn to cope with a deteriorating liver never mind a new one. So, with so many unknowns, my choice is to live my life and not think about it too much. I’m 35, I’ve done quite well so far, and I’m only ever really reminded of the seriousness of my illness when I visit the hospital.

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