'The Happiest And Very Best Day Of My Life!'

I often use the term 'emotional roller coaster' on this blog. It is a term that often sounds a bit corny but it is how our life seems so so often. Today, Ellie was certainly on an emotional roller coaster. A ride that started up with the excitement of the day ahead then plummeted down when she heard that Wills is probably going to be in hospital on her birthday yet again, and then went up, up, up so that, in the end, she declared today 'the happiest and very best day of my life!" The pictures tell the story!!

We waved William off to CHASE with his nurse, waited for Hope to get home from school and set off to the theatre. We got there a lot earlier than I had planned but I am so glad we hung around! We picked up the tickets first and got our first lovely surprise. With the tickets were three programmes, personally signed by Lee for Hope, Ellie and William. The girls were so happy and, as ever, very pleased to see one for William too.

Ellie so wanted to catch a glimpse of Lee and give him the card and letter she had made for him. It was very early but we decided to go to the Stage Door where I was hoping to have a quick chat with someone and ask if they could ask him to say hello to her as he entered or make sure he got her letter. I explained the situation to a girl on the door who said she would try and talk to someone when they arrived but that it would be a while everyone would turn up. Just as she was saying that she said 'Oh, speak of the devil' and there was Lee!

Ellie got her moment, gave him her card personally and we took some lovely pictures. I gave Lee a very brief version of Ellie's story and why she loves his music and asked him to read her letter. Ellie was beside herself and kept on hugging me and giggling all night. I haven't seen her so happy in such a long time. Hope was very happy for Ellie but she is not so 'star struck'. Over tea, she told me that she thinks everyone is the same, just some people choose to be actors or pop singers - 'People like Lee are very talented and I really love to watch them do what they do but it doesn't mean they are anything special when they are just shopping or out with their mates really does it!' An unusual opinion in someone her age and very healthy too I think. Hope's highlight came later when she got to see backstage. She asked loads of questions about how things work and how practical challenges are met. She was fascinated by the how the back drop and curtain changes work, how actors deal with fast costume changes, the revolving stage and what measures are taken to make sure Joseph is safe when he is elevated.


We were invited backstage by one of the guys we were talking when Lee turned up. After Lee went in, he carried on talking to us and wanted to help make our evening special. Having met Lee so early, we were able to have a long and relaxing meal at TGI Fridays. Hope and I waved at Ellie up on the ceiling and threw her up the odd forkful of food and spoon of ice-cream! Ellie kept on grabbing us and hugging us with excitement.

The show was fantastic. The seats we had were perfect, near the front of the stalls and right in the middle. The whole cast are amazing. Both girls were really happy and excited throughout. I sat between them and it was so nice to be able to cuddle up together and enjoy an evening away from all the stress. None the less, I was relieved to get a text from the hospice to say that all was well with William. Ellie kept on saying over and over again 'I met Lee Mead!' 'I can't believe I met Lee Mead!!' We danced, sung and clapped our way through the 'mega mix' and had loads of fun together. There were a couple next to Ellie who were celebrating their 10th wedding anniversary and had left their own children at home. The poor things had an over excited Ellie chatting to them throughout the interval so they didn't quite get the child-free break they had hoped for. They did say they enjoyed Ellie's company - I hope so!

We then waited in our seats for the guy who had promised to take us back stage. It was fascinating to see everything. We saw how everthing worked, sat on the golden chariot and saw all the props and the animals. We were shown all Lee's costumes and all tried on THE coat. It is really hot and heavy. I am so impressed with how he bounds around the stage so energetically in it. On the way home, Hope and Ellie sung '

"We wore the coat, with golden lining, bright colours shining..."

and

"So Hope, Ellie and Mummy went backstage

No longer feeling cold

And Chris said we could sit in Joseph's chariot of Gold - Of Gold!

We wore your coloured coat, your amazing coloured coat!"

Ellie declared that her dress will never be washed again because Lee hugged her in it and she wore his coat in it.

It was a fantastic evening and we are all so grateful for CHASE for arranging it, the 'Really Useful Company' for donating our fantastic tickets, Lee for giving Ellie a few moments of his precious time and to Chris for showing us around the stage. Yesterday, I said I wanted to give the girls a really special evening to remember forever. All of these people made that happen. I know there are going to be more blogs about surgery and hospitals over the next few weeks. We will all be looking back at this one to cheer us up and I know the girls have a fantastic memory of a very special 'Mummy Day'. That is what was the most important thing about today for all of us. A special time together away from the stress and upset and a time where Hope and Ellie had Mummy all to themselves. This is why Ellie loves Joseph and Lee Mead's music so much. From the moment we decided that watching 'Any Dream Will Do' was our girly time with a rule of no non-urgent medical stuff, Ellie had those special memories of Mummy time. Her CD give her the same feeling when she is away and when William is ill. She now has a very special memory to think of to go with all that. Unfortunately, things have become more unsettled and complicated with William over the last year and the 'ring fenced' girly time got somewhat more ad hoc. We all agreed we need to find it again and have more special days out together too. They enjoyed and appreciated even little things, like going on a tube - impossible with William unless you know in advance there are lifts at all stations. For now though, there are two very tired and very very happy little girls tucked up in bed right now and that makes for a happy Mummy too.

Quick clinic report

We have just been to clinic. Poor Ellie had to come too as she has an inset day. I think she heard a bit too much and is somewhat subdued. William does need a relatively urgent operation to remove his gall bladder as he has 'obstructive jaundice' caused by his gall stones blocking the biliary tract. The good news is that that is why he has jaundice - in general, his liver is OK (ish - for someone on TPN!). Our consultant now has to contact Birmingham to see if they want to bring his transplant assessment forward before he has the operation or want the team here to go ahead. He may go to Kings as they had a specialist liver unit. William often takes a while to stabilise his stoma output after surgery so it could mess up the assessment. Oh well, we are set for another summer holiday in hospitel any which way... Must get Wills packed for CHASE now...

Has William thrown a spanner in the works???

Well, we are all set for a lovely weekend. Ellie is sooooo excited about Joseph tommorow and quite beside herself with any idea that she could possibly bump into Lee Mead at the stage door or the 'scrum' as we have come to know as the autograph signing frenzy after the show. She has made him a card and is busy writing a letter to stick in it - I have never seen her write so neatly! I am ready for the ball, I have my dress, my bling, shoes and even my VPL free undies. I will be able to do my own star spotting as there are rumours of some celeb attendance, and even stronger rumours of others - watch this space!

William is packed and ready to go to the hospice with his lovely CHASE nurse tomorrow. Granny is staying there with him so the girls and I can enjoy tomorrow and the girls are going on after the sibling day on Saturday so Paul and I can enjoy the ball. It is the first time we have had him there so we can all do our own thing a bit and I'm sure it will do us all good. I will miss him heaps on Saturday but will be there Sunday morning.

The only question is - will it all happen???

We had a liver ultrasound today and it showed that William has gall stones, caused by the huge amounts of bile he produces crystallising. William's intestines don't move properly so it can sit around a while. That would be bad enough and perhaps something to sort out in the near future. However, William's bilirubin levels are increasing in each weekly set of bloods. He is now a little yellow and doesn't seem all that well (although not in the way that makes us rush to hospital in fear of a life threatening line infection). He is having intermittent pain and his urine has been very dark for a few weeks. This suggests that one of the stones has blocked a bile duct, a much more urgent situation.

We have clinic tomorrow and I wonder if we will be coming home or staying in! I hope we can go home and come back in next week. If the worse comes to the worse though we will still see Joseph and go to the ball as both are in London and pretty close by the hospital. One thing is for certain, with this and the transplant assessment coming up, we are going to have another hospital dominated summer! All the more reason to do my very best to make sure the girls have a really fab evening tomorrow - even if it means a fruitless wait outside a stage door in the rain!!! Remember a similar situation Mum? Nik Kershaw on a dark, wet and foggy evening!!! I wasn't lucky enough to get a glimpse or an autograph at that stage door vigil but did get a personal one, thanks to a next door neighbour, a few weeks later. I will remember that if we are unlucky tomorrow and tell Ellie that she may get lucky another time.

Yellow (and red and green and brown and scarlet and black...) Oh and a ball!

We had William's blood results today - his bilirubin levels have doubled in the last week He is noticably yellow now, especially in his eyes. I knew this would happen but I didn't want him to go yellow Come on Birmingham!! We have a liver ultrasound on Thursday and clinic on Friday so I will know a bit more about what is going on. He is relatively well and happy - just yellow! The pictures show some of the fun the children have had over the last few weeks, in the sunshine - especially in our lovely new garden.

I have a great weekend coming up so hope to put all this behind me. Via the hospice, The Really Company' have donated tickets for Hope, Ellie and me to see Joseph on Friday. We have stall tickets so will be close to the action. The hospice were hoping to get us in to meet Lee Mead but his management are saying he is too overwhelmed with requests. I am a bit disapointed for them. Ellie loves Lee. We watched 'Any Dream Will Do' together and made sure William's treatment was over in time for us to sit down and have some girlie time together. When William was in hospital, we would phone each other afterwards and discuss how everyone was doing. Ellie associates this with me and listens to Joseph and Lee's album when we are apart when Wills is in hospital. She has them on to sleep and whenever she is missing me. It would have made her little life so far to meet him He does come out of the theatre at the end of the show but there are crowds there then. However, being the determined little thing I am, I have emailed his agent to ask if he could say a special hello to them when he comes out. I have also emailed Denise Van Outen, via Capital Radio. Of course, she will probably not even get the mail as I am sure her producer gets mail addressed to her but you never know, if she gets it, perhaps she can use her girlfriend charm to ask Lee to say hello to Hope and Ellie when he pops his head out of the theatre at the end of the show. Just a simple 'Ah, you must be Hope and Ellie. I heard you were coming, did you enjoy the show' would be enough to make them very very happy! The girl from the hospice said it is easier to arrange 'meet and greets' for the sick children themselves but siblings need and deserve these special treats just as much - if not more! In any case, we will have a special night.

Wills is at the hospice for the weekend with Granny. Hope and Ellie have a sibling day on Saturday and then will be off the hospice too leaving me free to go to a ball It is a Paul for Chelsea and Westminster paediatric surgery. We need an extra theatre. Paul is singing at it. We haven't been out together since I had Wills - nearly 4 years ago!! I am looking forward to that very very much.

Clinic report

Took Wills to clinic this afternoon. It really was one of those afternoons...clinic ran 2 hours late and then we arrived at Fulham Broadway to find the District Line suspended between Putney Bridge and Wimbledon due to fire - that was exactly where we needed to go!! We had to get a bus to Victoria and then I had to pay out for a train ticket. Don't you just hate that!! You just want to get home. I wanted to cry, I really did!!!

Clinic was OKish - nothing more or less that I was expecting. William's liver blood results have gone up a fair bit. They increased the number of days he has fat in his TPN from 3 to 4 as he wasn't growing very well. He has grown back up to his centile which is great but his liver doesn't seem to be coping with the extra. He is having another liver ultrasound in a couple of weeks and we will keep monitoring his liver blood tests. We are currently going to clinic every 2 weeks and will continue to do so for the time being - I have requested that we go back to Friday mornings as at least I can drop the girls to school, rush to clinic and be home to pick them up. I was expecting to be home by 4.30 ish and was home at 6.30! You can't push your luck (not that delayed clinics and travel chaos are my fault) when you rely so heavily on favours from people.

Apparently, our consultant had an email from Birmingham to say we have a slot for William's transplant assessment 'very soon'. She didn't tell me when but said we should be getting a letter very soon. She is still going to tell them about William's liver issues so it may be hurried up even more. Somehow, I don't think we are going to get a huge amount of notice.

A new journey about to begin

am sitting down with a luverly glass of red and listening to the Coldplay gig on radio 1 - What bliss!! Paul has had a rare day off so Wills has been glued to him all day so I have got heaps of work done and now I can enjoy the gig without feeling guilty for what I should be doing. The children are in bed and Paul is watching the footie - ah, luverly (sips wine and enjoys the excitement of 'Life in Technicolour' live...becoming 'Violet Hill')

I wonder if they will play 'Yellow' I always joked with myself that this would be our theme song if Wills got jaundiced. You have to find humour sometimes! Well, William's liver function tests are up a bit at the moment. Compared with some of the children who have severe liver disease, this really is nothing, but for Wills they are up and we are hoping this is just a blip due to all the infections he has had, and not the beginning of a downward spiral. We have clinic tomorrow so I'm sure we will discuss it all. We still haven't heard anything from Birmingham so I am hoping they can chase it up and see if William has a pencilled in slot yet! (Ah, 'In my Place' - one of my total fave songs ever!!!! I think Coldplay are my fave band these days – although The Cure and The Smiths will always be up there)

I am really feeling nostalgic and reflective now. Appropriate for this blog really. Regular followers of my ramblings will know that I have been thinking a lot about all that life has thrown us and how I put all the pieces together. Followers may also know that I am a committed Christian and truly believe that God wants me to use our experiences. Sadly, so many people go through some of the things we are going through. I have been privileged to have the opportunity to share their world and understand, really understand and empathise what they are going through. Chaplains come to the wards and talk to us all and everyone appreciates that (nearly everyone) and draws a lot of strength from what they say. However, they don’t know what it feels like to be standing there in the shoes of that parent who has just been told that their child has to undergo lifesaving surgery that may kill them or that there is a real chance that their child will die before they reach their teenage years. I can give them the hope and comfort that their visitors give but, more than that, when I can say, in all honesty, that I know how it feels for them because I am there with them going through the same. This is central to the Christian Faith as God put Jesus in the same place as the people he was reaching out to so that He could relate to them and empathise with them. I am getting dangerously close to being far to heavy and religious here so I will just take a step back to enjoy ‘Viva La Vida’ – wow, I love that song, I’ve been listening to it over and over the last few days.

Anyway, I have enrolled on a HE Certificate in Theology and Vocation. William starts school in September so I will have a little time, along side my job, to take on a new challenge. I feel I really have to develop some kind of ministry based on what is going on in our lives. I know I could reach out and help people cope with what they are going through and my faith is my rock and what enable me to wake up in the morning with a smile on my face, ready to tackle what the day has in store for us. It is what enables me to go to sleep each night thanking God for something that has happened, even in the most worrying days. So, for me to be able to help others it has to be within a Christian context. For now, I will focus on writing for Christian publications and hope to give some talks as well (so if anyone knows of a group or occasion who use speakers let me know) At the same time, I will carry on talking to parents I meet in hospitals. I will weave more theology into my writing and talking as I learn and develop and who knows where it will lead…

The week ahead

As ever, thanks for your lovely messages of support. It means soooo much! Wills didn't have his notox yesterday. They needed to plan a bit more as he can't have oral sedation so will need IV sedation in a staffed operating theatre. This is planned for Tuesday. At least he can enjoy being on his feet for a few more days. Libra - he is not having walking plasters and is not allowed on his feet during the process, not at first at least anyway. After a while, he will move onto 'barred boots' A pair of boots on a board with a bar between them. He will wear these for a set amount of hours a day, while resting and sleeping so will be able to be on his feet inbetween. There is still a chance he will need surgery after the initial 4 week trial of serial casts following the botox. I am not too chuffed about that but they have explained that he will be unable to walk much longer if we don't fix his feet a bit now.

On Monday, we will discuss mre details about the transplant assessment and we hope to have an idea of when we may go by the end of the week. This has become very urgent as William has virtually run out of veins to but his hickman line in for his feed. He has had 12 lines and most of his main veins are now thrombosed (scarred). This line is really precious and we are no longer allowed to use it to take bloods, so the poor thing will have to be a bit of a pin cushion for a while. The next line will have to go into the hepatic vein that flows into the liver. This is very tricky to use for TPN and the insertion is not straight forward. They have now established that William is completely 'unfeedable'. This, together with tricky line access and recurrent life threatening line infections has made the assessment all the more urgent. Everyone here is expecting Wills to go straight onto the active list following the 2 week assessment at Birmingham. Apart from these discussions, and the botox, next week will be spent stabalising his TPN (intravenous feed) so we can go home on Friday :-) :-) Once again, 'a few days' in hospital has turned into a month!!!

Botox on a Friday (or Tuesday) afternoon

Not for me, although I couldn really do some with the ever deepening worry lines. William was finally seen by an orthopedic doctor about his 'deformed' feet. You can wait ages for such referrals but, all of a sudden, physios appeared and the next day bought along an orthopedic doctor or an 'orthopod'. His verdict -lots of long latin words to describe the abnormalities in William's bones and tendon. The solution - botox injections into the back of his ankes. followed by 'serial castings'. This will be a long process where plaster casts are put on his feet and up to his knees. The first one will be put on immediately after the botox and each week he will go to orthopedic clinic to have his progress reviewed and the angle of his casts gradually altered to correct his feet. This will happen today or Tuesday. After 4 weeks, we will assess if this is working and, if so, continue...for several months If it is not, he will need surgery to lengthen the tendons at the back of his legs.

I didn't really want to do all this before his transplant assessment but have been told it is necessary ASAP or it will be too late to correct. We always knew this was progressing and he may not walk for ever. However, it was thought to be part of a degenerative condition. It seems now that he has a rare, genetic gut problem called 'tricho hepatic enteric syndome', THE for short and, along side this, mild cerebral palsey. Separating these out has made his treatment plans clearer - intestinal failure = TPN and transplant assessment and cerebral palsy has its own tried and tested ways of addressing various problems, such as correcting foot deformities caused by tight tendons or 'spasticity'.

It is all progress but painfully slow and unpleasant for William. He is 3 years old and stuck to a 2 foot drip line 20 hours a day. Now he will have both his legs in plaster and won't be allowed to walk and run around with his walker for as long as it take A toddler confined to a chair for many weeks, perhaps months. I think I will write a toddler entertainment book.

'I think William now meets the criteria for transplant'

We are back in hospital as Wills is still not right following his line infection.

Today, a slightly arrogant (although I often think this when I meet a new doctor for the first time and change my mind when I get used the them) young locum registrar told me William's platelets are up and it doesn't look like his low grade temperature spikes are any concern so we can go home tomorrow. Naturally, I believed him and was very pleased. When I asked his gastro nurse about transport home with the TPN we had with us, he was a tad confused so spoke to William’s consultant. She came in for one of ‘those chats’ and, boy, was it one of those! William can’t go home until Monday at the earliest. Sad though I am, that is sensible. He is not completely right, not too bad, but not right and is now spiking high end of low grade temperatures when we go into his one of the lumens on his line which is a bit ominous. She said we may loose the line but we hope not. She also said that William now meets criteria for small bowel transplant. This has always been something for consideration in the future and, last month, the idea of going for assessment was to see if it was the future the team and we, his parents, thought was best for him. Now, it is felt that this future may have arrived. He has had/has got yet another line infection. He can, and has, become critically ill with such infections and the combination of another failed gut challenge, his increasingly enlarged liver and spleen, uncontrollable ‘massive’ (medical term!) stoma output and the fact that the illeostomy, although making him more comfortable, has not stopped the bugs from his gut translocating into his line, has led to the conclusion the time has come when a transplant can be seen as a life saving procedure and the complications of TPN are an immediate threat to his life. If we loose this line, we will go into his 12th!!! We are running out of venous access. Of course, I was somewhat shell shocked. We will probably be at Birmingham for assessment within weeks, a couple of months at most, and could be on the list at the end of the two week assessment. Of course, they may decide that he is not suitable or that he does not yet need to be on the list but, for the first time, doctors are saying that they think the time has come. They have spoken to the transplant team about him recently so this is not just the Chelsea team thinking this in isolation. A large part of the assessment is for the parents to decide if it is what we want for him so I am trying really hard not to make my mind up before I have all the facts. Paul is certainly more open minded than me (right now, I want it for him because, even though it is a risk, it is a chance!!!). Paul is a bit more stuck on the risk element but I think my little fighter could come through it. Being in and out of hospital so much and with so much uncertainty in life is tough on the whole family and I have to think of the girls as well.

Challenges set and failed

This is another catch-up blog. I really will try harder to keep this up to date. It has been another hectic few weeks with, yet another, stay in hospital. William has been very well since coming home from hospital after his illeosomy and we had all been enjoying time together as a family and doing every day, normal things. Of course, life still revolved around TPN and William's other medical and physical needs but we were home and together.

We needed to try and see if William could cope with the octreotide, the drug that could reduce the huge abount of fluid he loses through his bowel. We also needed to challenge his bowel again to tolerate something going in. Hope and Ellie were staying at their Dad's for a week in the Spring break so I requested that William went to hospital to do all that while they were away so we didn't have to worry about where they would go. So, on the second Monday of the school holidays, William and I went into out Chelsea Pad with strict instructions to the doctors that they could experiment all they liked but we were to be home by Thursday afternoon. We tried a tiny test dose of the octreotide. The first dose gave him a localised skin reaction but the second made him red and mottled all over. So, that option went out ot the window! As we had a couple more days, the doctors decided to challenge his gut with 5 mls per hour of diorylyte. This was swiftly stopped as it increased the output of fluid through his bowel and was making him dehydrated. With this, the doctors realised that we were not going to be able to increase this into a substantial amount of feed to reduce TPN and have him fed, in part, through the gut This feeding pattern would have best helped protect his liver from the TPN but even a tiny bit of something offers some protection for the liver so the next plan was to try 5 mls of neocate (special baby milk with all constitutes in their elemental form, i.e. as if already broken down by the gut) four times a day. This would offer better protection than diorylyte. William's response to this was described by the doctor as 'spectacular!' He lost 3.2 litres from his gut and ended the day we were due to go home dehydrated, unresponsive and with 3 gut bugs living in his line as a result of everything becoming stirred up by the response of the gut to the feed. Far from going home, William instead spent the night keeping the on call doctors busy checking on his responsiveness and prescribing antibiotics and fluids. By the next day, Williams red cell count, neutrophil (a white cell) and platelet counts had dropped and he needed a blood transfusion. We stayed over the weekend and came home to continue IV antibiotics. The girls missed stayed on at their Dad's a few days longer than planned and missed the start of term. So, all in all, a bit of a disaster really!!! We have clinic on May 20th and will discuss where we go next. William's consultant is keen for him to go to Birmingham sooner rather than later for transplant assessment. Although he is not sick enough to go on the list as yet she hopes this will enable us all to see if this is where he is heading and if it is what we want for him. His liver and spleen are now enlarged and, although his liver is still functioning well, his spleen is not doing all it should in fighting infections and producing/regulating blood cells.

We were in the 'Mail on Sunday' 'You' magazine today in an article about 'Wellchild' the charity that organised our garden makeover. I didn't know the article was in today so couldn't tell anyone but it is a good article so check if anyone has it before recycling day.

Referral for transplant assessment

We were at clinic this afternoon. Soon after coming into the hospital, I bumped into one of the play specialists and learned that another of the gastro children died on Easter Sunday He was 18 months old. This child had so many problems it was amazing he lived as long as he did. A real fighter. That is 8 gastro kids that have died since we have been in this world. It is so sad and scary. My thoughts and prayers are with the family. We shared a room with them for several weeks during William's last admission.

I also learned some good news in that one of the other children who was not expected to come off TPN is doing so well that she has weaned right down to IV fluids only and may well be free of lines in the near future.

William had an ultrasound today. The report was not ready in time for clinic and the radiographer was not saying much except that his spleen is enlarged enough to be obviously so by eyeballing the scans. He actually has a bulge from it. All this will be fed back to Birmingham. William's consultant is keen that he goes for his transplant assessment in the near future. He is not sick enough to go on the list yet but it would tell us all if he is suitable and, if so, what the parameters to go live on the list would be for him. It will also tell us if we want to put him through it. The current prognosis is 50% chance of survival. That has actually gone down since Paul and I went to Birmingham for an initial chat about transplant. They have not done many small bowel transplants in children and the stats change according to outcome. The assessment will involve 2 weeks at Birmingham Children's Hospital where Wills will have loads of tests and we will have lots of counselling sessions and discussions so we can make our decision as well. It will be very intense.

Wills is OK but still losing too much fluid through his bowel. So, we are going back to our chelsea pad next week to try a new drug that will be given via a subcutaneous line. He tried this last time but had a reaction to it. However, it was given IV through his central line so we are going to see how it goes subcut. Of course, having had a potentially allergic reaction previously, this has to be done in hospital. If the drug works, we will try to challenge his gut again with a little diarolyte. We will only be in Monday - Thursday so it won't be too bad. The girls are away at their Dad's so it is a perfect time

Where We Are Now and Thoughts for Good Friday

 
 

I recently wrote a piece for our church magazine about Wills and his condition etc. It suddenly occured to me that people here know about Wills and support me so much. So, here is a looong blog entry explaining exactly what is going on with him. There is a religious bit at the end but it is relevant to today...William has a combination of health problems and physical disabilities that baffle every doctor (and there have been quite a few!) who has seen him. His problems affect his intestines, lungs, growth and his mobility and overall control of his body. He is totally unique and this makes things very difficult as the medical professionals have to treat him blind and no-one knows what we can or should expect in terms of prognosis. However, the combination of symptoms and the complications associated with the treatments that keep William alive lead most doctors to conclude that he is ‘life limited’ and unlikely to survive into adulthood.William’s main problem is that he has intestinal failure throughout his small bowel and colon. He is unable to absorb anything through the gut walls and his intestines lack the organised peristalsis that moves food and drink through the gut. This is due to a condition called ‘pseudo obstruction’. William is unable to eat or drink anything and all his nutrition comes from TPN, total parenteral nutrition, a feed that is given intravenously into a catheter, called a hickman line, in his chest. William has his TPN infusing for 20 hours a day so only has 4 hours a day to move around free of the line that attaches him to the feed and the pump that controls its delivery. Without peristalsis, William’s intestines resemble a garden hose with their contents swishing around and pooling in the loops of bowel. Gas also builds up in the gut which can be very painful. The fluid that collects in the bowel becomes a breeding ground for bacteria. As the bowel is designed to absorb its contents into the blood stream, these bugs can easily escape into the blood stream. When this happens, they reach the hickman line and breed there causing line infections. This is very dangerous and can result in septicaemia and the need to remove the line. This can happen at any moment of any day and William can become critically ill incredibly quickly when it does. William struggled with frequent line infections throughout 2007 and had to have several new lines. By the end of the Summer he had infections more often that not and was on intravenous antibiotics most of the time. At the beginning of November, he became very unwell with another infection. His gastroenterology team at Chelsea and Westminster Hospital decided to bring him in for another new line but this became infected within a week. William remained in hospital on a cocktail of strong intravenous antibiotics to keep the infections under control while his team held lengthy discussions as to what to do next. X-rays showed that his pseudo obstruction was worsening and this was causing him a lot of pain due to large areas of distention in his bowel.In January, William had an illeostomy. This is an operation where a section of small bowel is brought through the skin. William has a ‘magic bag’ over his ‘magic holes’ that collects all the contents that drain out. This was done to make the journey through his intestines much shorter in the hope that it would decompress his gut and reduce the distention and resulting pain he was experiencing. The hope was that he would have less pooling in the gut and, therefore, less infection. So far, it seems that the operation has been successful in preventing infection. However, doctors have been shocked to see how much fluid William looses through his gut. He has become clear that he has a ‘leaky gut’ where fluid that should be maintained within cells leaks and pours out through his gut as secretary diarrhoea. Due to William’s pseudo obstruction, this fluid is not reabsorbed so pours out. We were in hospital for longer than expected after the operation as it took time to balance his electrolytes and the amount of fluid he needs in his TPN to prevent dehydration. This time was very difficult for us all, especially Hope and Ellie who were separated from their Mum and Brother. We are now home but William has a demanding programme of medical care and physiotherapy for the spasticity he has in his legs and to clear the mucus that collects in his lungs. This take several hours a day with things that have to be done at set times. Our lives have to be dominated by this and it is often difficult to have a normal family life around it. However, we make the best we can and are prepared to flush the hickman line, deliver medications and empty stoma bags in all sorts of places to ensure Hope, Ellie and William enjoy as full a family life as possible.William’s future is very uncertain. We will try one more time to see if he is able to tolerate anything going through his gut. With the amount of fluid he is losing through his stoma, this is very unlikely but we need to try. At the moment, William is doing OK on TPN. However, TPN is not a long term solution. When a line is removed due to infection it leaves scar tissue behind and that potential line site is damaged. There are a limited set of sites that can be used. William has already had a lot of lines and this is a big concern. TPN puts a lot of pressure on the liver. At the moment, William’s liver is enlarged but functions well. In time, it is likely that it will begin to fail. The only cure for William’s condition is a small bowel and maybe a liver transplant. We are now exploring William’s suitability for this in the future. Transplant’s don’t last forever and survival rates following small bowel transplants in children are not encouraging. At the moment they are only carried out as a last resort in the UK and offered when the child’s underlying condition can be viewed as the end stage of critical illness. However, it is reassuring to know that there is that option. On top of this, William has progressive physical disability (perhaps a form of cerebral palsey) that makes it hard for him to get around and control his body. He has just passed his test for and electric wheelchair that will give him so much more freedom and independence, especially as he can attach his TPN bag to the chair.William’s condition can be heartbreaking and puts a lot of strain on the family. However, it is also a gift from God. Through all this, we are all put into situations where we would not have been otherwise, and where Christians need to be. Sadly, I know many parents of very sick children and several who have already been through the agony of losing a chid. There are others who minister to such families but it is all too easy to brush off their words when there is no way someone talking to you about their faith can have a clue as to how it feels to going through what you are going through. Hope, Ellie and I have all been in situations where we have been able to break down these kinds of barriers because we do know. We are fellow travellers along the same rocky pathway and we understand how painful and difficult a road it is. I have been asked why my God could not save me from the grief we experience. To reach people, Christians have to live their lives. Jesus did not live a life of luxury, parallel to those he reached out to. No, he joined them and lived among them. When we are living in a hospital for several months, we are doing just that. Life can be very difficult and there are times when I wonder how I will cope. I always find guidance in the Bible. I don’t always find solutions that I am looking for. Sometimes, Jesus shares with me that He knows the agony I am experiencing and, although he can’t take that away, He understands because He has been there too. The hardest thing for me is the isolation parenting William brings about. Paul works many evenings and can be on tour for several weeks. William can not be left with anyone other than Paul and I or a nurse trained in his care. There can be times when I am unable to go out and be with people. Worse still are times when I am stuck in the hospital away from Hope and Ellie. I may be being used there but will often question why. When I feel that way, I am drawn to Jesus in Gethsemene. He was left alone at His most difficult hour. Jesus puts us in difficult situations so we can empathise with others and enable them to accept our testimony to Him. When this gets hard to bare we need look no further than to He who has put us there for guidance. He put Himself there first so He knows how it is for us. The most important thing is that God wants to use us where He has us. I am looking for opportunities

A normal Saturday

The Milne family had a normal family Saturday today. There has not been many opportunities to blog such a thing lately . This morning, we went along to 'cycling for all' at our track. This is great. They are an organisation who have loads of adapted bikes for disabled people to ride around the track and, when they get a bit of confidence, around the 'country park' (as country as we get in Croydon/South Norwood) on a Saturday morning. Wills loves it. All three children do. They really enjoy an activity they can do together. The bike I use with Wills has two wheels at the front and he sits in a chair above. It does get a bit of getting used to as the slightest movement with one hand sends it zig zagging. I tried to obey William's demands for speed and push it a bit down one of the straights but this is definately a bike that prefers a steady pace (pictures in the gallery). We had loads of fun and Wills would keep going all morning. However, Paul was watching and pointed out how cold he was getting and so how cold Wills must be. The poor little mite was shivering from head to foot, still requesting we keep going. I guess I was keeping warm with the energy of pushing Wills on that big heavy machine. The children want to make that a regular activity and I am more than happy to oblige, perhaps for a short and sweet session until the weather warms up a bit.

After the cycling, we took the opportunity of having Paul and the car and hit Tesco. I have not been in a supermarket on a Saturday morning for about a year and don't intend to do so again for at least as long! Yikes! I couldn't think! Still, it was a normal family thing to do and I revelled in that. My children were all as bored and hyper as those all around us and William was as good at demanding a Thomas magazine as all the little darlings demanding sweets (including Ellie). They didn't get them.Hope went off to a chocolate party this afternoon and made some scrummy truffles and chocolate coated strawberries. After dropping her off Ellie, William and I went to the library. Wills was disappointed not to find a Thomas book but a story about honey biscuits that included a recipie. I think I know what we will be doing tomorrow afternoon. Wills loves smelling biscuits! They are his favourite food to smell.

Paul is at work and the little darlings are all now in bed. Wills was so tired he was asleep before I finished putting up his TPN. I would now open a bottle of red but I want to be up early in the morning to fit in a few miles before Paul goes off to sing in church. Have to settle for a cuppa instead while I watch casualty and other rubbish Saturday night TV. Long may things stay on this nice even keel

Christmas comes but once a year...

This year, we will be having two. We promised the girls a Christmas when William came home from the hospital. We were separated for the real thing last year and the thought of our special Christmas together really kept us all going through those long weeks after William's illeostomy operation when we were battling to get his fluids and electrolytes balanced.

We had a Christmas party on Friday complete with mulled wine. People were so generous in briging cards and gifts for us so there was an exciting pile under the tree to tease the children. William enjoyed the pary food too - it all smelled good. He carefully arranged two cherry tomatos on the table and announced to all that he had 'made an illeostomy'. He had a fab time playing engines with one of his friends. This was great to see as he is not very good at sharing, especially engines, and can find playing with other children a bit overwhelming.

Today was our 'Christmas Day' and we had the works; a real Christmas tree, bucks fizz and smoked salmon for breakfast, dinner with all the trimmings and lots of chocolate and mince pies. Father Christmas very kindly returned to fill the stockings we left hanging on the fire place over night. There were plenty of Thomas goodies, including a fantastic track table made by Paul. The girls had a mix of pink fluff and glitter and make-up, chemistry sets and pens and pencils. The icing on the cake for them was a laptop...each. They are combined Christmas and Birthday presents (although their birthdays are a way off) but will help them keep up with their school work and friends when they have to stay at Mum and Dad's next time William is in hospital. It is too much for Dad to move his life into our house any more so, in future, Hope and Ellie will go to them in Stoke-on-Trent with educational support to keep up with school. The lap tops are brand new ones, designed for children and a bit different to traditional ones. They are called EEEPC and I'm sure Paul will blog more on the technical info. They are arriving on Tuesday so the girls had to settle with IOU notes today. The laptops have built in web cams so we hope we can use those to chat live when we are separated again.

It was a fab day and felt very authentic. It was strange to go to church for a Lent service rather than carols. There was no Christmas TV either - that was not so missed. The girls made up for both the lack of festive entertainment and religious input with a rendition of the Nativity story complete with songs. Thankfuly for them, my camera was lost under the goegraphical layers of wrapping paper at that point in the day.

Back to normal tomorrow for the first time in 3 1/2 months. The girls are back at school. William has 3 sessions at playschool, a hospital appointment and a driving lesson on the power chair. I have a training day to do with some Brompton Fountain Area Co-ordinators and need to get back on track with work in general. It is going to be a busy week and challenging to get into routines to manage everything along side William's new needs. I'm sure there will be plenty of fun and mishaps along the way to post on here.

A Grand Day Out

Aren't these just lovely pictures! We had a fantastic day out today. We started off watching the guards depart and arrive during the changing of the guard at Buckingham Palace. The Queen was in today so they all saluted her and the retiring life guards paraded to the front of the palace and saluted her with a bugle. I wonder if she actually watched these salutations anymore? We were guided through the action by an old chap who watches every single change - every other day in Winter and every day in the Summer. He was very helpful and spot on with his timings.

From the palace, we walked through Trafalgar Square (stopping at The National Gallery for a nappy change and bag empty), through Leicester Square and China Town to The Rainforest Cafe. We won a £35 meal voucher in a raffle at our community nurse team's family day back in September. The voucher went out of date last week but, when I explained the situation, the manager let us come anyway and in half term, when the orignal voucher wasn't valid. It is an amazing place. We certainly wouldn't have been going without the voucher as it is not at all cheap and the food is pretty standard. The atmosphere and effects were amazing. We had a pair of elephants watching over us as we ate and gorilla's and monkeys were near by. All the animals are animated. A star lit sky was above us and thunder rumbled from time to time. We were promised a good table and we certainly had one. The only problem I had was that there are no disabled facilities and the restaurant is down a steep flight of stairs. However, the staff bent over backwards to help us to get Wills down there. The children had a great time. Willam, as ever, enjoyed pushing engines around the table and smelling chips.

After lunch we revisited Trafalgar Square where the children ran around together. We went back into the gallery for another nappy and bag empty but this time stayed to look around a bit. At the suggestion of visiting it properly, Hope complained that galleries were boring and then complained that we weren's spending long enough at each painting once we were in. I promised her a return visit on our own so we can stop, stare and talk about the paintings together without her younger siblings chomping at the bit.

Hope, Ellie and I decorated our Christmas Tree whilst William played with his engines on the table. A perfect day really. William does struggle with pain while sitting for long periods. We have to build a bit of a jump around to help him drain every couple of hours into days out like this. It is a bit of a military operation really with all that needs to come with us and timing medicines, flushing TPN, bag emptying... All well worth it though, just look at those faces!

Home Sweet Home

 
 

We are home and William is finally enjoying his new garden. He can't wait to get out there as soon as he is off his TPN. I must admit to enjoying simple things like washing his sheets and Hope and Ellie's uniforms and haning them out in out lovely new garden on such a lovely sunny day (although rather cold!).

We are settling down and getting used to the practical challenges, such as William's illeostomy that drains huge amounts every time he moves, coughs, sneezes, laughs... We now know that his bowel really doesn't move at all. We also know that William secretes enormous amounts of fluid and salts though his gut. All this pools in his distended loops and floods out when he moves. This can result in some very messy situations if the bag fails. We have him attached to a urine drainage system over night and when he is in his chair but it is tricky when he is running around. He is very patient though, not least because he hates having his bag changed when it leaks. The illeostomy has certainly helped decompress his gut but he is still very distended and often uncomfortable. His x-rays still show obstruction - in William's case due to "pseudo-obstruction", meaning obstruction due to gut motility failure rather than a mechanical blockage. They are still awaiting some neurology tests to see if he is suitable for transplant assessment. It is highly likely we will find ourselves back at Birmingham for assessment at some stage. We also face some tricky decisions as to whether we opt for painful surgery to correct his feet deformities or decide that we let things be, resulting in increasing disability and potential wheelchair dependence. We will face all this step by step over the coming months but, for now, we are home and enjoying being together. We are getting festive too. For us, it is Christmas Day a week on Sunday!


 
 

 
 
 

Nearly there...

We should be going home the week after next :-) :-) Wills is soooo much better after his op. He is still pouring out way too much from his illeostomy. Some tests suggest he may have a complicated storage disorder - his cells don't store some of the nutrients they should and so they leak into urine and into his bowel. This could be causing his "leaky bowel" and inability to absorb in the gut, as well as neurological problems. He needs a skin and bone biopsy to clarify the diagnosis. We have been trying to get the neuro team to see him for the last 2 months (his consultant and reg left at same time so he slipt though the net a bit) and they finally caught up with us yesterday and said the biopsies were needed. Doh, could have got them 2 weeks ago if they had actually spoken to the gastro team and surgeons. The NHS still struggles with multidisciplinary working. The neuro reg also said Wills needs urgent physio while he is in as he loses function when he is ill...I asked for that on his first day in... Oh well. Things are coming together. Wills is back to 20 hour TPN and fluids for the first time in 2 months and we can take him out during his 4 hour break. He can also toddle free of lines for the first time in as long. I can't wait to get him out of here later this afternoon :-). If all goes well with stabilising him on his TPN we should be home the week after next :-) :-) I can't wait for us all to be home together again.

Tummy Trumps (a long overdue update)

I have been so rubbish at updating this blog lately that readers have switched to Paul's (see blegspot.blogspot) for updates. I am sorry, please do come back and I will try and update more regularly...promise....
In my defence, Paul does go home to our lovely internet access every evening and I have my precious hour a day in Starbucks where I have to juggle priorities and keeping up with work tends to dominate. For some reason, I can't access this on my BlackBerry but can get on the running website, Fetch, hence being able to post brief updates there. That's my excuse, another reason for blog silence has been that the last few weeks have been so confusing with William's illeostomy discussed and discussed and decisions changed and altered acording to how many bugs and yeast he had. It has all been a bit much to keep up with, let alone tell others about.

Even Paul had a bit of a gap in his entries and updated just yesterday. There is no need to repeat what he has said so well so I have shamelessly lifted the following with some different pictures of Wills over the last week. He wanted his beloved Thomas books as soon as he regained consciousnes, although in his bleary post anaesthetic and morphin pumped state he did ask me where the books he was cuddling had gone! I have chosen some pictures that show the stoma. I will take a better picture of that for the blog, not least because it may be of interest to another family who are about to undergo a similar procedure. William has a "double barrel illeostomy" where both ends of the cut bowel are bought through the skin. One drains the stool from the small bowel and the other, the mucus fistula, drains mucus from the colon and can also be used to wash out the lowere bowel. There are also some lovely pictures of Wills with Grandma and Grandad on Monday when he was feeling a lot better.

So, over to Paul...

"William has taken a little while to recover from his operation. He discharged himself early from paediatric HDU simply by constantly declaring to anybody who would listen that he wanted to go back to his room, and generally refusing to co-operate unless his demands were met. I can’t think where he gets his stubborn streak from. However, progress has been slow since then. He took a while to be weaned away from his morphine pump, and confidence in his ‘magic bag’ (the ileostomy bag) has been knocked by the realisation that there was a deal of initial pain involved. The ileostomy itself is none too pretty, and William is keen to ‘clean it off’. In fact, he has two chunks of his small intestine poking through his abdomen wall – ‘stomas’ - and cleaning them off would not be the way forward. But they are doing their job, as they are constantly leaking the unpleasant fluid and gas that were making him so uncomfortable before Christmas. The slight downside is that they are leaking so efficiently that William is now on enormous amounts of IV replacement fluid and still suffering symptoms of dehydration. He has also developed a slightly disconcerting habit of unconsciously farting through these ‘stomas’. As I earlier blogged, there is some quality toilet humour ahead of him.

He is really on the mend now though, and has gingerly returned to his habit of toddling around the ward with his daddy literally in tow. He is re-asserting himself as the ward character, although more through his fantastic eccentricity than any charm offensive. After he had told yet another nurse (S – a wonderfully efficient and caring professional) that he didn’t ‘want that one’, I thought it best to start trying to address his issues with politeness. “Let’s role-play,’ I suggested. ‘I’ll be S. When I say hello, you say hello S.”

‘No,’ suggested William, ‘you be a giraffe.’

A little more work is required. "


Back to me now and today's update...

The doctors were very concerned about William having such a huge output from his stoma. We tried loperamide (immodium) but that just went straight through him. We are now trying an IV drug called octreotide. This is a drug that acts on endocrine system to switch off natural products the body is over sectreting. It can be used to control heavy bleeding, to shrink tumours and to treat over production of insulin. We are hoping it will get control of William's huge over secretion of bile and gastric juices. So far, it looks promising. The draw backs are that it is an artificial version of the hormone that acts as a growth hormone inhibiter and also inhibits insulin production. Wills is on a very low dose so we are hoping it will not have these actions. Even if he does, we have to get on top of the secretions and we hoe to use the drug for just a few months. He is also struggling with an ever dropping red cell count as a result of his losses. He is having another blood transfusion today. We were warned that Wills would take his time to settle down after this op.

We all have to be patient but it is getting harder on us all. Lots of discussions are now taking place on how we can manage long admissions like this in such a way that we are all happy, especially Hope, Ellie and Mum and Dad. Plans are being formulated but can't be blogged until they are discussed further and everyone is happy about them. I just wish we could be given a home in Chelsea so we could always be together and the girls could be schooled without any disruptions. Shame William's hospital is in one of the most exclusive and expensive areas of the country!