It was all just normal to me

We love to share stories of young adults with childhood liver conditions who are busy getting on with their lives, so it’s great to hear from Beth, who had her liver transplant 29 years ago. The story begins, however, with her mum, Wendy.

 

Beth was born at 8.55am on Sunday 9 October 1994 after a 1 hour 50 minute labour, weighing 6lb 3oz (2.8 kg) – a doddle or so it seemed. My pregnancy had been plain sailing until the last month or so when scans showed that my baby wasn’t growing properly and that I had a low platelet count.

 

Graham, her dad, and I were told that Beth was slightly jaundiced but not bad enough to need to go under the lamp where they usually put jaundiced babies, just to keep her near the window to get some sunlight.  The midwife called her a “greedy baby” in the hospital notes as there seemed no filling her up – which we now know was because she wasn’t able to digest the milk and therefore felt hungry all the time.

 

After two days we brought her home to see if we could settle down into the “routine” everybody tells you about.  No chance! We were feeding her about every two hours, and half an hour after each feed she would want more. A midwife visited every day for the first 10 days and on one of her visits, as I was changing Beth’s nappy, she saw the colour of Beth’s poo, which was almost white – like toothpaste.  Being new parents and having no idea what colour a baby’s poo should be, we just thought it was normal because all she was drinking was white milk.  We also told the midwife about her sick which seemed to spurt out for about three feet in front of her.  We now know that the projectile sick and the poo colour should have been real ‘red flag’ signs that something was wrong but neither symptom triggered any urgent response.

 

I was actually more concerned about Beth’s sticky eye, which I took her to the GP about when she was two weeks old.  He looked into her eyes, which were still yellow with jaundice and he didn’t spot anything wrong either.

 

Referral

After 10 days, the health visitor took over from the midwife and by the time Beth was four weeks old, I went to clinic with her for the first time. The health visitor here put in Beth’s notes that she was still jaundiced and her tummy was slightly distended.  When she saw us the following week she referred us to the GP.   He took a blood test to check Beth’s bilirubin level but this would take a week to come back.  We went back the following week, by this time, Beth was six weeks old and he said her bilirubin was quite high and he repeated the test again, which would take another week to come back.

 

However, he rang us at home that evening and said he had been chatting with the consultant at our local hospital who thought it would be better if we went over there in the morning.  So off we went, and were put in a single room while Beth had more blood tests and prodding and poking from various doctors.  When the results from these came back,  we were told that they suspected she had a disease called biliary atresia and they were trying to find a hospital that could treat her.  We heard them on the phone ringing a few before they told us that they had managed to find a bed for her at Booth Hall Children’s Hospital in Manchester and could we immediately go there.

 

Diagnosis

 

We arrived at the A&E Department at Booth Hall at about 12.30pm and were asked to go up to Ward 5, but when we got to the ward we were put in a tiny kitchen and asked to wait as there wasn’t a bed available yet.  We stayed in this kitchen until about 7.30pm when finally a bed was found.  This ward was bedlam to say the least as there was an epidemic of bronchiolitis and was full of children coughing and spluttering, some worse than others who needed to be in oxygen tents.

 

When we got to see the consultant, he explained to us that they thought that Beth had got biliary atresia and that she should have a biopsy to confirm it.  But they couldn’t get the theatre time and as the Kasai operation that was needed if she did have biliary atresia had an 80% success rate if performed before she was six weeks old, and Beth was almost seven weeks old, it was decided to go ahead with the Kasai.

 

We were then transferred to the surgical ward and met the young surgeon who was to do the operation.  He said that he saw about five babies a year with biliary atresia and as the Kasai operation was quite rare, he was inviting about 35 medical people to come and watch the operation.

 

The surgery took about three hours and we were told that they would know as soon as her bowels started working again if the operation had been successful or not.  Then Beth started coughing – it seems she had caught bronchiolitis when we were first admitted.  At the time she seemed more ill with this than ever she did with biliary atresia. She had to be fed by a tube up her nose and we were kept in isolation from the rest of patients.  We were also told that she wouldn’t be able to go home until she had put some weight on and had got to at least 7lbs.

 

Every morning on the ward round, the surgeon and his team would come and have a look at the contents of Beth’s nappy to see if her poo had gone any darker – but it hadn’t.

 

This is the first time we heard a mention of Dr Kelly from Birmingham.  The surgeon had been in touch with her and she suggested that Beth go on a course of steroids (Prednisolone) to try and kick-start her liver.  We were discharged on 14 December with these plus a bucket load of other medicines, mostly vitamins and special baby milk “Pregestimil” and Maxijul, which is a special sugar to add to baby milk to give more calories.

 

Listed for transplant

 

We then had to wait until our next appointment at Booth Hall on 5 January 1995.  They told us then that the Kasai had failed and Beth would need a liver transplant before she was one-year old and we were being referred to Dr Kelly at Birmingham Children’s Hospital.

 

Once we had been referred to Birmingham, we were contacted by Tessa, the Family Support Officer of the Children’s Liver Disease Foundation (CLDF).  This was a Godsend, she knew exactly what we were going through and understood all about children with liver disease.  She used to ring me at least once a week and listen to my problems and help as much as she could.

 

We went to Birmingham to see Dr Kelly on 25 January 1995.  In the consulting room there were about 12 medical people, Dr Kelly, the clinical nurse specialist, dietician, social worker, physiotherapist, play leader, Uncle Tom Cobley and all.  Dr Kelly told us Beth was a very sick baby and we would need to spend a week in Birmingham while they carried out a transplant assessment, so we went back home and waited for an appointment.

 

I went back to work in February 1995, as I was finding it very difficult to cope at home, so Graham gave up his job to look after Beth.  We were going regularly to see the consultant at Macclesfield who was keeping a close eye on Beth’s condition.  He told us that as there were no nerves in the liver she wasn’t in any pain.  But, as we found out later, she didn’t have any pain from her liver, but the toxins that a healthy liver would normally get rid of were building up in her brain giving her encephalopothy (bad headaches) and she was also itching like mad.  The consultant at Macclesfield wrote to Dr Kelly at Birmingham to see if he could hurry up her transplant assessment “before Beth’s parents go insane”.

 

While we were waiting for an appointment from Birmingham, I saw a magazine article about living related liver transplants.  This meant that a mother or father could donate a segment of their liver to their child.  I wrote to Dr Baker at Kings College Hospital in London to see if we could be considered on their programme.   He sent us a copy of his Report “Living-related transplantation: an essential new service” but when we looked into it, Beth was far too small.  It recommended that the parent giving a segment of liver should only be six times heavier than the child and as Beth only weighed about 10-11lbs it wouldn’t be possible until she was quite a lot bigger. Because of her illness, however, she wasn’t growing at all.

 

We finally got the appointment to go for the transplant assessment in March 1995.  Beth had all the tests done and we were given lots of information to help us decide if it would be the best thing for her.

 

We went to meet the surgeon – Mr Mayer at the Queen Elizabeth Hospital – and we were also shown the intensive care unit where Beth would go after the transplant.  A parent of a child who had just had a liver transplant kindly allowed us to go and see him in intensive care so that we had an idea of what to expect when we went to see Beth after her operation.

 

As Beth wasn’t drinking much of her milk it was decided that she needed to be fed by a tube up her nose and we were to go back the following Thursday to learn how to do it.  Just before we were discharged, Beth was given her 4-month immunisations and they also gave her the 12-18 Month MMR jab as she wouldn’t be able to have this after a transplant as they are “live” vaccines.

 

We went back to Birmingham again on 6 April as Beth still needed to have an angiogram. We also had to learn about tube feeding, but when she was examined, the doctor said she was dehydrated and too poorly to go home.

 

So we stayed in hospital then until after the transplant. Our days were pretty full looking after Beth and learning all about what was happening and how to look after her in future.  We were taught how to make liver feed and how to connect Beth’s feeding tube up to the Kangaroo pump, which would feed her through the night.

 

On Wednesday afternoons, Children’s Liver Disease Foundation (CLDF) held a support group for all the families who were staying on the ward, which helped us to see that the other families were also feeling exactly the same as us.

 

As by now we realised just how ill Beth was, we decided to have her Christened.  The Hospital Chaplain came up to the ward on the morning of 3 May and christened Beth in my arms with me sat on her cot.  All the ward staff and the walking wounded came, including an 18 month-old girl who had had her transplant a few weeks earlier.  The nurses set out all the chairs like it was a mini-theatre and there wasn’t a dry eye in the house.  Beth was oblivious to this and was more concerned with filling her nappy.   As if she hadn’t got enough to cope with, she was also cutting her first tooth.

 

Dr Patrick McKiernan, the consultant looking after Beth at this time, was getting more worried about her condition.  There didn’t seem enough time in the day to give her all the medicines she needed, she was getting more and more irritable being very sick. She was by now too ill to go on the transplant list.

 

Early in May, Dr Patrick McKiernan came to tell us that a liver had been donated and although Beth wasn’t as fit as they would like, and the donor had also had the CMV (cyclo megalo virus), this was Beth’s chance and would we like to take some time to decide what should be done.  There was no decision to make, Beth had to have the transplant or she wouldn’t survive.

 

We had already been told about CMV.  This is a virus that most people don’t notice they have had, it is just like a cold, but in transplant patients it can be fatal.  We had originally been told that Beth would have to have a liver that had not had this virus, but as it turned out we couldn’t afford to be that choosy.  They said that some time after the transplant she would get CMV and would need to go into hospital for about two weeks and she would be very poorly indeed.

 

The ambulance took us to the Queen Elizabeth Hospital at 7.30am next morning and we left Beth just outside the operating theatre in Mr David Mayer’s skilful hands.  It was a beautiful day and Graham and I went walking endlessly round Birmingham until 2pm when we were told that we could go back to find out how the operation was going.

 

Transformation

By this time, Beth was in the intensive care unit with wires and tubes coming out from all over the place, but the operation had been a success. As Beth started waking up from the anaesthetic and opened her eyes, we immediately noticed how white they were and that her skin was starting to go back to pink, having previously been very yellow because of the jaundice.

 

On Sunday afternoon, just two days after her transplant, Beth was discharged back to the Children’s Hospital where she was put in the high dependency unit.

 

On Tuesday Grandma and Nana came to visit us.  They were amazed at how well she was looking, sitting up in a blue chair on the bed, smiling away and looking very pleased with herself.

Before we came out of hospital Graham decided he would learn how to insert a nasogastric tube. Beth continued her excellent progress and was discharged only 12 days after her transplant.

 

The next few weeks were hectic.  We were trying to get to grips with the medicine routine, she had 12 different medicines – 27 doses a day.  Also we had to make the liver feed which looked disgusting but I’m sure it did her good. We were going back to Birmingham every two weeks for a check-up and the consultant at Macclesfield would see us the week in between.

 

Beth seemed to be doing OK but then she turned quite poorly.  We called the doctor out and he just said to leave it for 24 hours and if she was no better then to ring again. She did start picking up the next day but we discovered the next time she had her blood tests at Birmingham that she had actually had CMV, which they had previously told us would necessitate a hospital admission.  But, tough as ever, she fought this one off as well.

 

At Beth’s eight-month review with the health visitor, she said Beth had slight delay in gross and fine motor functions and referred her to the consultant community paediatrician who we saw on 15 August.  He arranged for home visits by a physiotherapist and a speech therapist.  He said that as she had been tube fed, and the muscles used for talking were developed through babies sucking at their bottle, Beth may have a problem with speech.  So the physiotherapist and the speech therapist both visited a few times.  We saw the consultant community paediatrician about four times over the next two years but he was very pleased with all aspects of Beth’s development and discharged us.

 

We had been advised to wait three months before we wrote our thank you letter to the donor family.  This was the hardest letter either of us had ever had to write, but between us we managed and sent the letter to the Transplant Coordinator at the Queen Elizabeth Hospital in Birmingham.  We got a lovely reply from the donor family just before Christmas and they were pleased to hear how Beth had got on.

 

Fundraising

 

By the time the first anniversary of Beth’s transplant was coming up we decided we wanted to raise some money for the Children’s Liver Disease Foundation who had been such a fabulous support and help to us when we needed them.  We decided to have a Grand Raffle and a party and while we were organising this, Beth’s Godparents, Linda and Keith Scowcroft, were also organising an Auction of Promises. This was a brilliant night, people had been so generous giving to and buying from the Auction that this raised over £2400.

 

The party was a great success too, even if Graham did get a bit hot under the collar when he had to make a speech!  Everybody had been so kind to us while Beth was ill and they were so pleased to see her fit and healthy and thoroughly enjoying every minute of the day.    Altogether the Anniversary events had raised the grand sum of £4237.24 for the Children’s Liver Disease Foundation.

 

It’s now 28 years since Beth’s transplant.  She has remained remarkably well and is now under the care of the Adult Liver Unit at Queen Elizabeth’s Hospital in Birmingham.  Like many other young people with liver transplants, she competed several times in the British Transplant Games and was lucky enough to be selected to swim in in the World Transplant Games in Australia in 2009. We still go and spectate at the British Transplant Games every year, to catch up with the many friends we’ve made over the years and support this fabulous event.

We hope that reading Beth’s story helps other families who are facing similar circumstances to ours and also lets donor families know what a tremendous difference their decision to donate their loved ones’ organs makes to other people’s lives.

 

Beth’s story

 

Because I was so young when I had my surgery, I guess I just assumed that everyone had a huge scar. So, it was really only after I started school, that I became aware that not everyone had had a liver transplant. We’d go swimming with school, I’d see other people getting changed and realised that they didn’t have a scar. This made me extremely self-conscious – something which stayed with me for years – I would never want anyone to see my scar and even now I won’t wear certain clothes. As I’ve got older though, I fully appreciate that it is part of my story and I wouldn’t be here without it.

 

Another way I remember my liver condition affecting me is that I was desperate to get my ears pierced but because I was immunosuppressed, I had to get permission from the team at Birmingham Children’s Hospital. I was so pleased to finally get them done when I was 18 after several tetanus injections!

 

Because I was diagnosed and had my transplant so young, everything that went with it was just normal to me. I think I’m lucky that I don’t remember it. I’m sure my mum and dad won’t ever forget it and I think they still worry about me – especially whether I’m remembering to take my tablets.

 

I did have lots of time off for childhood illness, or to prevent me getting them, and also to attend appointments, but despite that, I still did well at school and after my A-levels, I went to Sheffield Hallam University to do a degree in Primary Education.

I don’t feel that having a transplant impacted my university experience. The Uni is quite local to me so I lived at home, travelled in and studied whilst working two jobs. My transplant made me choose not to drink which I think made a bit of difference to begin with as everyone was turning 18 and that’s all they wanted to do, but that calmed down after a while – no-one wants to be surrounded by drunks all the time!

Teaching is something I have wanted to do since I was little and would sit all my toys in a row to take the register! So, getting a First in my degree and then getting a job in a local primary school is something I felt really proud about. I’ve now been teaching for seven years and although I couldn’t pretend it’s an easy job, it’s fair to say that every day is different!  I’ve taught most age groups and am currently teaching the reception class which I am really enjoying.

To any young person who has a similar ambition or goal I would say don’t let your liver condition stop you from going for it. I know I’ve been exceptionally lucky. I’ve met lots of people along the way who have had to have second or third transplants or sadly not made it. I appreciate so much the kind gift that the donor family chose to give me in their most painful moments.