The Longest Post

Mostly Caitlin

Caitlin is settling into nursery, and I am getting to grips with the new routine. She runs in there eagerly every day, with me usually following behind at Hannah’s pace (Hannah will WALK, Mummy will not argue!). The formalities of hanging up coats and bags and putting her name apple on the tree are an irritation to her; she just wants to get in there and play!

I was worried that she might wish she had stayed at pre-school, but nursery has definitely won her over! I have heard about all manner of delights such as the water trough, the cars and roads, the fishing game (she catched boots, and Olivia shared her rod with her, and she catched boots!), chalks, cutting and gluing,……..can I go please? Today she was thrilled to bring home a star shaped “cake biscuit” which was “made from flour and butter, and I had a bit of help from Mrs Linton”.

One of Caitlin’s recent games is ‘Guess What Animal I Am Being’. The other day the clue was “leap, leap, leap”. A rabbit? A kangaroo? A grasshopper? No, none of the above. I gave up. “It’s a leper, mummy! A leper!” ?????? The bible? A leopard? Do lepers, or leopards, leap? Sometimes parenthood makes your head hurt, but more importantly your sides ache.

Caitlin has recently developed the irritating habit of yelling “Nooooooo, Hannaaaaaaaah” every time Hannah so much as looks in her direction. I do have considerable sympathy for Caitlin; Hannah just doesn’t play by the rules. However, it has recently got to the point where Caitlin was doing this constantly, even when Hannah was toddling about minding her own business. I seem to have cured this problem, at least temporarily; this having been achieved by a couple of well-timed “Noooooooo, Caitliiiiiiiiiiin”s from me, which left her somewhat less than impressed. Three cheers for creative parenting! Although I was informed, after she had said sorry to Hannah, that it was my turn to say sorry to her. I can see the logic…

CLDF Conference

Last Saturday saw us at the CLDF conference. I had my customary stress about getting out of the house in the morning, but fortunately a shorter than anticipated journey somewhat alleviated this. This was an excellent event, and I am still trying to take everything in that I heard and learned. First of all, it was great to see Danielle and Ethan again, and Danielle brought some lovely presents for the girls (belated birthday). Caitlin had some Crayola Wonder Paints which are a mess free thing (hurrah) and Hannah had a lovely fluffy pink jumper from Next (buy my girls clothes from Next, be my friend for life). Danielle, if you are reading this, you helped me so much to get through Hannah’s kasai, and you were always there for a chat or a cup of tea or with a stack of magazines to read. Thank you, and I am so glad we have remained friends. And Ethan is a superstar!

It was also lovely to meet both Elly and Jane for the first time, and their beautiful families. After about 7 months corresponding with Elly, and about 5 with Jane, and discovering we have more in common than just babies with liver disease, it was great to meet them both in person. Their friendships are both really important to me, not least because Max and Arthur are a similar age to Hannah, but also because both are intelligent and forthright women. I am just sorry I did not get more time to chat with my friends, as the day proved to be very busy. I would have also liked to have had time to browse around the displays and buy from the shop. A two day conference would have suited me! I think this was mostly due in part to Andy and I having two small and exuberant children to look after at the times when we weren’t in the talks, having to pick them up from and drop them off at the crèche, and lack of organisation and planning on our part. For a start we were silly enough not to bring a buggy for Hannah (she who does NOT like to be carried).

The event as a whole was superb. The talk in the morning, which was the annual report, I foresaw to be fairly tedious, like Andy’s MD’s speech at the annual Christmas party. How wrong I was. Catherine Arkley is an impressive and commanding speaker. She spoke movingly of the early days of the organisation, and the struggles faced by parents in the early 1980’s, and she spoke passionately about the CLDF’s vision for the future. We then heard from two wonderfully inspiring mums who have worked tirelessly to raise money for CLDF – I felt very humble – and saw some incredibly touching video footage of their children playing together.

In the afternoon there was a choice of sessions. Although we had initially thought we would go to the ‘Living with liver disease’ discussion, we decided in the end to go to the main conference, which consisted of two talks, the first about liver biopsy, the second about issues on the transplant list. The liver biopsy talk was very interesting. It firstly dealt with biopsy methods, where the focus was on percutaneous needle biopsy. This was new to me, as Hannah has only had a surgical biopsy at the time of her Kasai. This was followed by information about different stains and what they show up, accompanied with slides. Fascinating stuff!

The second talk, about transplant list issues, was undertaken by a transplant surgeon and a clinical psychologist. In fact, a lot of what was said by the psychologist applied to dealing with liver disease in general so was extremely useful. Such as your feelings towards people who are anti organ donation. I have to admit I already take this somewhat personally. And how the different coping strategies used by different members of the family can cause conflict and misunderstanding. And how friends and family don’t always get it, and think that your child is better or has been cured, but part of you doesn’t really want them to get it, because then they would feel the pain too.

The more practical information was also good. After all, we will be there one day. As Andy described it the other day, “We were woken up in the middle of a routine operation to find out she had some major disease that we knew nothing about. We don’t want to be that badly prepared again.”

The highlight of the day was the Question Time session at the end. It was really interesting to hear the panel’s diverse opinions on topics such as: Whether alcohol is allowed in liver disease/transplant patients (OK apparently, to my surprise, although obviously in strict moderation), what is the prognosis for biliary atresia patients (transplant, though after many years for some), is milk thistle of any benefit (not proven but doesn’t do any harm*), should George Best have received a liver transplant (IMO yes, ethically, although he has done huge harm to organ donation on this country), the list goes on.

The best question was saved for last. If each member of the panel had £150 000 to spend on research, where would it go? I thought the most interesting three were
Treatment for portal hypertension – when is the best time to intervene – before or after a bleed
Study into father’s reactions to their child’s liver disease
Hepatocyte transplant

So it was an excellent day and I can’t believe I have to wait another year for the next one. A big thanks to the CLDF and all the speakers and volunteers. Oh, and the girls enjoyed the crèche (although Hannah was a bit tired of it all by the third session and I left her screaming on the floor, refusing to allow anyone to pick her up). Rounded off the day with takeaway pizza. Splendid.

*I remember reading somewhere that milk thistle could worsen portal hypertension – where the hell was that??? I wish all this sort of information could be collected together in an easily searchable database ;-)


In Memory of Haley

The world has lost another child to liver disease. This time it is Haley. Haley died suddenly on Saturday 1st October aged 11. This amazing little girl, who I would have loved to have had the privilege to meet, fought a long and hard battle against a rare combination of illnesses. She was amazingly talented and wrote and performed songs about her liver disease. I prefer to believe that Haley could not be healed here on earth, so the Lord brought her home to heal her. I didn’t know Haley, but I cried for her and her family. I cannot imagine their pain.


More anniversaries

On Monday it is a year since the beginning of our darkest few days so far, the admission to hospital for surgery, of course at the time we thought this was for choledochal cyst. The day after that was just horrid, starving her for the ultrasound and very distressing blood tests and cannula insertion. The next day was the day of the Kasai, again having to starve her was very upsetting. Remember these babies with no bile flow have to feed every hour or two as they are absorbing very little of their milk. There was a bit of a delay getting her to theatre and she eventually went at 10:45 having been NBM since 4 am. Yuck. The very worst bit was that when the operation was due to be over, Chris came to fetch us. I expected that we would be taken to Hannah in the recovery room, but, no, Prof Millar wanted to speak to us. Andy tells me that Chris made it clear that Hannah was still in surgery, but I didn’t take that in. I thought she was dead, from then until Prof Millar said he would speak to us in HDU rather than in a private room. Instead, of course, it was a change in diagnosis, to biliary atresia. At the time that was a relief. It took several months to take in the consequences. I didn’t really have time to worry, and Hannah did so well from the word go. I have to admit I never worried about a failed Kasai. We were told the surgery might not work, but Hannah showed signs of good bile flow from the very early stages, when I was more worried about when the tubes might come off her and when she might feed again than any long term prognosis. At that time I took a day at a time, and every day Hannah improved and gave me hope. By the time I learned about the true consequences of a failed Kasai, it was obvious that Hannah’s was working. Of course it is also very much a positive anniversary, in that the last year has seen her do so well, and that we have stayed out of the hospital. I very much hope I can say the same thing this time next year.

I have been trying to get down in words my current understanding of and feelings about what the future might hold for us, and the following is the result.

Biliary Atresia

I think that sometimes people wonder why I can’t just get on with things. (Or perhaps I don’t give other people enough credit, and this is in fact a self-criticism.) After all, Hannah has had her surgery, almost a year ago, and has come through with flying colours. She is feisty, growing well and developing normally. She is full of life and energy. What more could I ask for? Why do I keep dwelling on this biliary atresia thing? Why do I have so much fear for the future?

In truth, I don’t dwell on it all the time. Most of the time when I look at Hannah I see a beautiful daughter, not an illness. She is my little ray of sunshine and brings me enormous happiness. I try my hardest to treat her as any other child and to make the absolute most of this relatively normal time which we have been blessed with.

However, I cannot deny the facts and nor do I think it would be healthy for me to do so. Biliary atresia is a very scary disease. You will find this out very quickly if you search for it on Google. I am not worrying about rare complications (although there are plenty of those, most of them pretty nasty), but about very common problems which are life-threatening. Hannah is beating the odds, and I pray and hope every day that she can continue to do so.

To be brutally honest, the miracle that we would hope for is that Hannah has been misdiagnosed. There are some doctors who believe that this is indeed the case for BA patients who don’t need to be transplanted. Unfortunately, this seems unlikely taking into account the experience of the surgical team who made the diagnosis, which was also confirmed by pathology.

What we can also hope for is that alternative treatments for BA become available, before the need for transplantation arises, and that the disease progresses slowly enough in Hannah that she will not reach that stage for many years. She may be one of the lucky few.

I can’t just forget about it and I will not apologise for that. There are a dozen reminders every day, and I cope with this the best I can. To me this means dealing with it, not denying it. We are already among the lucky minority, in that Hannah has had no hospitalisations in almost a year since the Kasai. I thank God for that every day.

I can’t help but be scared about the future, as the likelihood is that things will get worse. I feel frightened about the girls having to grow to understand all this – it gives me shivers to think of it. Best not to go there for now.

There aren’t many people that I can tell how completely shit this whole thing makes me feel sometimes, although I am lucky that there are one or two. I even find it hard to voice concerns on CLASS, BA Network and Liver Families. Almost everyone else’s child seems to have had it worse than Hannah. Sometimes I feel like a bit of a fraud in the world of liver disease. I know that’s not really the case, and that everyone has something valuable to contribute, whether they are going through it now, have in the past, or like us are experiencing an extended period of plain sailing. I know that at the least our story so far can give a little hope to parents of newly diagnosed babies, and that people want to hear good news as well as bad, but a lot of the time I still feel like a bit of an outsider. Not of course that I would want Hannah to get sicker so as to have more common experiences with other parents.

So why is this Biliary Atresia such a scary disease? How can a little girl who looks so well be so poorly inside? Why is this diagnosis so devastating and life changing for us? Why do we feel the need to prepare ourselves for the future?

Biliary atresia causes progressive, irreversible liver damage, called cirrhosis.
No-one knows what causes Biliary Atresia. Until they do, there will be no cure.
There is no cure for cirrhosis. It can be treated by liver transplant.
The liver can continue to function normally until it is 75% damaged. This is known as compensated chronic liver disease. When decompensation occurs, in other words, when symptoms start to appear, transplant is usually both inevitable and imminent.
In the vast majority of biliary atresia patients, decompensation occurs before adulthood. The opinion of the medical profession seems to be that it will eventually occur in every patient.
In one study of 271 BA patients in France, only two individuals had reached the age of 20 or over without showing signs of cirrhosis.
Liver transplant is inevitable in biliary atresia, at some stage, based on current medical knowledge.
It may be that BA patients are better off being transplanted as children, as in the UK 1 in 5 adults on the liver transplant list dies while waiting. The proportion is much smaller for children. Success rates are similar.
My best guess, from all the figures I have read, is that one in five children born with biliary atresia will not live to adulthood.
When liver failure occurs, it is accompanied by a whole host of unpleasant and potentially fatal side effects, including multi organ failure.
Liver disease tries to cover all the bases for you, so there are also a whole lot more unpleasant and potentially fatal side effects that can occur before the liver decompensates, such as portal hypertension which causes internal haemorrhaging.

So it scares the shit out of me. Liver disease is nasty, and unpredictable, and it won’t go away, and Hannah won’t get better. And this is my blog, and my life, and if I want to be bloody miserable and self pitying sometimes I shall be. I am blessed that Hannah has done so well thus far, and I know it. This doesn’t mean I am ever going to like the fact that my beautiful baby girl who I love with all my heart has this shitty disease that could turn everything upside down at any time.

Blogging is such good therapy....