Happy Birthday!!!

This weekend was a bumper birthday weekend with Ellie turning 7 on Saturday and William 2 today!! Wills was home from hospital - hooray! He has been home a good long time now, although the elusive trip to GOSH still looms over us but no date as yet. The girls came home with Grandma and Grandad and it was so lovely to see them and see all 3 playing together. Ellie was so upset when she had to go back again. I miss them so much too! Ellie had a lovely day on Saturday and enjoyed her birthday chilli! She loved her presents too, expecially her "Pixel Chix" a cross between polly pocket and a computer game!

William had a great day today and really enjoyed opening his presents and discovering what was inside. He played with everything, with the help of his sisters. His favourites were a Thomas that pops up plastic smoke balls - very amusing to all 3 children (and the adults), duplo diggers, Noahs ark and magic pens. All 3 children had a really exciting day and it was nice to cook Sunday lunch for a family again. William had a visit from his little friends Sophie and Athalia. Unfortunately, William had to make do with a telephone call from Daddy who is still in hospital following his accident.

Daddy did manage a bit more sitting up today though and we are beginning to think of the practicalities of him coming home at the end of this week or beginning of week after (depending on how quick he gets mobile with crutches). Tomorrow, I will order a foot rest and bathroom perch stool and a wheel chair to go out in (Wills will have to sit on Daddy's knee) I am also going to take scissors to some of his horrible draw string trousers to enable them to go over the cage. I was about to experiment this evening but was, apparently, about to cut into expensive ones rather than Paul's cheap and cheerfuls - oops! All in all, things are settling down. We are slowly getting used to William's impairments and things were greatly clarified at clinic on Friday. Wills still needs his surgery for a laparotomy and illeostomy. I am rather dissapointed about this as I thought we may have been able to save him the pain of this but they still need to make sure the Nissen did not cause any adhesions, apparently these are more common in children with underlying neurological conditions. It is highly unlikely he will now have a bowel transplant. He would also be unlikely to have a liver transplant if it started to fail on the TPN. However, so far so good with gret liver function tests and just a fatty swollen and grumpy liver! The main concern is that William will be on TPN for life and so will run out of sites for his Hickman lines. We have to guard his lines with our lives because repeated problems would significantly shorten his! Still, we are coming to terms with things and the fact more disabilities and impairments will probably emerge over time. It is watch and see but, as I always say, he is a happy child! We are also coming to terms with Paul's mechano leg for the next few months. Following a few argumentative days where we took our frustrations out on each otherwe are settling down into thinking practically and looking forward to him coming home, working out solutions to things such as his inability to drop his leg from lying to standing alone due to the weight of the cage and seeing the funny side of things. There are key things I will have to do for him, such as lifting his leg in and out of bed and on and off foot stools. Overall it won't be too much and he will become more independent as time goes on. In the meantime though, I might as well get a uniform with the pair of them!!

One thing that does upset me a bit about the coming year is that William will learn this year that he is different to other children and can't eat, is attached to tubes and drains and can't walk properly. I dread the day he starts asking questions... At least when Paul comes home we will be there for each other again and support each other through the ups and downs with William, even if it will be a while before he can physically care and play with him again - at least he will be able to read stories and sing songs etc...

Escape to the zoo!!!

Well, Paul is still in hospital and making very slow progress with physiotherapy. At least he has started rehab now but it is painfully slow as he is still getting very dizzy when he puts his legs down. We are hoping he will be home by the end of next week once he gets going though. I will have to move his "mechano leg" on and off the bed and sofa though, not to mention clean pins and push skin back off them - lovely! He has told me he will take me to Prague at the end of all this and I am going to well and truely hold him to it!!!

Meanwhile, Wills and I escaped to Whipsnade Zoo on Saturday with our very good friends Helen and Adam. It was a great day and we saw loads of animals and I had a really good laugh with them - something I badly needed! If you read this, thanks guys - it was just what the doctor ordered..."exciting"! We did get the down pour and had to resort to the fashioned plastic bags available in gift shops in zoos etc. I won't put a picture of that on as I was not caught on camera and I won't embarass Adam and Helen but here are some pics of us all having fun...






William loved the elephants, bears, penguins, tigers, giraffes... he also loved watching the sealion show, even though it was raining. He was nice and snug and comfy in his new buggy. This one has padding and supports especially to suit him and so helps him to sit nice and straight. He loves it in there but we are not having much luck with the glasses. He hates having them on, I think the sudden detail to the world is all a bit overwhelming!

We have gastro clinic on Friday and will discuss how the latest developments and diagnosis will impact the next stage in his treatment etc. It has been a hard couple of weeks dealing with all this without Paul around, and having to fit in visiting him and coping with the accident etc. Hopefully, things will pick up soon and we can start to put everything back together again. At least the girls will be home at the weekend and we have Ellie and William's birthdays to look forward to. Ellie has ordered Mummy's chilli con carne for her birthday tea, even though she was offered a take away. It is nice to know they miss Mummy's cooking that much!

At last an update and pics of Wills in specs!

Well, it has been a while before I have felt the emotional and physical energy to post on here. There really wasn't any reserve to cope with Paul's accident and the last week has passed in a bit of a blur. Wills and I headed off the the Chase Hospice for a bit of rest and a battery charge. William finally got to go swimming!



Paul is heaps better now but his leg is badly broken so he has an "external fixater" i.e. a metal frame with screws into his bone. This will stay on for the next 3 months. He had his leg cut open to prevent the swelling damage his nerves. The most frustrating thing has been that the operation to close this cut with skin grafts has been cancelled every day this week since Monday. Physio can not begin until this has been done. It was finally done today and his orthopedic surgeon reckons he will be home in a week or so - I very much doubt this as it is about to be the weekend when hospitals notoriously go slow so real physio won't start until Monday. I should think he will be home by the middle of the week after next - just missing William's birthday! The really annoying thing is that he would probably have been home next week, in time for William's birthday, had they not cancelled his op every day! He has been nil by mouth each day waiting for it so they have been starving him for a week!

Meanwhile, it has been a very busy week for William. Following last week's breakthrough in diagnosis, we have been coming to terms with his current "disabilities" and getting the right equipment and services sorted out. He now has his glasses and looks very cute and studious...







He also has a bigger buggy with strong foot supports and supports added by the occupational therapist at the sides and behind him to prevent him slumping. This will last him until about 10 should he need it. We are borrowing a special little chair with foot supports and a play tray to support him while he plays. These both enable him to interact comfortably without feeling unsupported and needing to keep one hand in "guard position" to steady himself. We are waiting for our own chair and also for some special "Piedro boots" that will offer support, together with an insert moulded to the curve in his feet to help his stability. We have been taught activities to help him to develop in some of the areas he is struggling in at the moment. We will wait and see how things go now. Of course, we are still waiting to go to GOSH, although I am hoping that the general picture will answer enought questions to enable them to leave this admission until Paul is no longer a bionic man and I can focus fully on Wills again. After all, I will have to look after the pair of them for a while as Paul will be unable to do very much with that heavy contraption on! At least he will be able to read stories and play with William. William is lucky he still has a Daddy at all!

Meanwhile, Hope and Ellie are staying with Grandma and Grandad. This is hard as I really miss them. We have had a crisis every summer for the last 3 years and I miss just having a normal summer holiday and taking them places. They are coming back for the weekend next week to celerat Ellie and William's birthdays so at least we will be together then! I know they will be reading this so hello girls - look forward to seeing you in 9 sleeps...

How could it have got any worse??

Well, I don't know what I did in a previous life but this week has been a crazy one. William had his diagnosis of neurological problems and we have been coming to terms with the implications of that. It has been so exhausting, but then, last night things got so much worse. Paul was hit by a car and has mulitple fractures in his leg and breaks and lacerations in his face. He is now in hospital and has had pins put in his leg. I can't believe this has happened. Having to deal with Paul's condition when he is not here to share what is going on with William. He will be out of action for months. I was at the hospital with him all last night and didn't get any sleep then or during today. At the moment this has made everything seem like a big mess but I hope it will all settle after a bit of sleep. I have decided to stay a couple of days at the Chase hospice with Wills next week to get a break and battery recharge and some peace to take everything on board. My wonderful parents (whom I know now read this) are taking Ellie and Hope so I can really catch up with some sleep while Wills is being looked after there. That place really is a lifeline!

Moving in on a diagnosis

Well, today has been quite a day. We had eye clinic in the morning and a general review of all that has been happening with William's local paediatrician in the afternoon. Let's start with the good news - William does not need oxygen over night - hooray!!! His baseline saturation is above 93 (just) so that is OK for him. The desaturations will not be corrected by oxygen as he thinks this is due to reflux again as the Nissens can become loose. If this is so, Wills will need a repeat op. If it is sleep apnea, the oxygen will not help as he won't be breathing it in anyway so he would need a bi-pap machine for that. So, we will be off the The Brompton for another sleep study and Ph study to see what is going on.

William had his eye tests this morning and he is very long sighted so needs glasses. He also has a slight squint that may be due to one eye being worse than the other so may well be corrected by the glasses, otherwise, he will need a patch over the stronger eye. We had a short gap between the eye tests and consultant appointment where I was hoping to have a walk about town and spot of lunch. Instead, we were measuring up for William's glasses. With the eyesight he has, I was fearing "NHS jam jars" but they are actually pretty cute blue circular ones. The optician did comment about his wide nasal bridge and low ears as a slight issue for the frames but these are baby ones and pretty flexible. We get them in about a week so I will post a picture of our little studious looking boy. We really had no idea that he had vision problems and would never have picked it up if they had nbot been looking for more clues for his syndrome. Visual problems do fit the kind of syndrome they are now looking at.

So, after a sprint into town and back and grabbing a quick sandwich, we were back at the hospital to see our consultant. His role in all this is to put everything together and come up with a global descrition if not a definitive diagnosis. He watched William walk and looked at his feet and explained that there is a neurological problem there. His gastroenterologist is thinking more and more his gut problems are also neurological. So, putting it all together, we finally have a working diagnosis of "neural cell (or crest) migration disorder". I had to ask him to repeat his description of this several times as it was all a bit technical. Apparently, when the nervous system develops in the womb, neural cells have to migrate throughout the body to form the full system of nerves in order to make all the connections needed. When Wiliam's nervous system was developing, there was a "traffic jam" somewhere and some of the neural cells never made it to their destination. His gut was one area, his feet another. As a result, messages from the brain for normal functioning can not be sent. It was explained to me that this damage has been done so nothing can be done now to correct it. We will have to watch and wait to see where else may be effected and it is likely he will have some educational limitations. We still need to find out what has caused this, it could be a genetic syndrome but we may never know. His prognosis remains "guarded" and remains "life limited". The big difference for us now is that he does seem to have a condition that effects him medically, phsically and mentally. We will now have to take it as it comes and make sure we do all we can to help him realise his potential. Wills is having some blood tests and an MRI scan just to make sure it is not defecits in his TPN nutrients that are causing lack of growth in the cerebellum but this is just to rule it out as some of his problems, including his gut of course, started before he went onto TPN.

The consultant agreed that William does need piedro boots and, perhaps, splints, so the physio is coming on Thursday. He doesn't think physio will actually help his feet and walking as you can't correct things as no neural path developed. I am not so sure though as I know that the brain can learn to compensate and create new pathways around damage. I know we will not be able to help William to do this in automatic things, such as controllin his intestines and how his feet are growing, but we may be able to help him learn to walk with more control and balance.

Home again!

Well, luckily we didn't get kept in as there is nothing new going on with William and he is OK in himself. It seems that the seep study has highlighted an ongoing problem. William has sleep apnea. His sats drop down to 60% in his sleep so, he is back on oxygen at night! This is so dissapointing as we were so pleased to have him off - it was one good outcome this year! He is still much better than he was when he was before his Nissens as he was on up to 3 litres 24 hours a day and still desaturated overnight! He is on 1 litre when he sleeps for now but it may have to go up and he may need C-PAP or BI-PAP (machines delivering air by mask) overnight. He will have a full sleep study at The Brompton to see if the oxygen is sufficient. The good thing about this is that he is now going to be reviewed by the neurologist locally and will have an MRI scan and ECG to check his brain. It is increasingly felt by eveyone that he has neurological problems with co-ordination. It may be that the apnea is structural if some of the muscles in his upper airway are less toned than they should be. This could be resolved by surgery. His problems may be more neurological in that his brain fails to send a signal to breathe for a few seconds. It is clearly all related to his general condition and "syndrome". We were enjoying Hope and Ellie's school fair (although my stomach was in my boots as I was waiting the verdict on the sleep study and the amount of time I was woken by the alarm told me all was not great!). We then got the call to go to the hospital so came home to pack in case we stayed in. He had a chest x-ray and bloods and the doctor made sure he was well in himself.

When I was packing for the hospital, I didn't notice the TPN fridge seemed warm. When I went to switch the oxygen machine back on after so long, I was horrified to see the TPN fridge plug off!! The children went silent about it all but Hope dissolved into tears in the bathroom and it turned out she had, unwittingly, switched it off when she tried to make a hot chocolate with her frother. She changed her mind and didn't use it or the mess may have alerted me to check things! This was yesterday, so the TPN fridge had been off a day and a half - thus a week and a half of expensive TPN wasted. So began the next panic - no fluid for William who has been off since 12.30 lunch time. Several phone calls later and I was asked to take the TPN prescription to the Mayday so they can fax it to Chelsea and Westminster to confrim the electrolytes while I wait for it to be made. Paul is singing at Glyndeborne tonight so no way could I wake th children up and get there! So, poor Paul will get in at midnight and drive to Chelsea and Westminster to get his fluids. Why does all this happen when he is out late?

I was especially not going to wake Wills up after the performance we had getting his oxygen on. I thought it would be best to wait until he was asleep - wrong! I ended up terrifying the poor thing. Putting the nasal prongs on ended up waking him into a semi-awake state, awake enough to feel like he was being strangled but not enough to be comforted. In the end, he screamed and screamed. The only thing I could do was wake him fully and distract and comfort him with his beloved Thomas and James (engines) and a lovely book about them until he finally fell asleep again.

Off to our local hospital!

This is a very quick post as we are just packing to go to our local hospital for tests. William had a sleep study last night and his oxygen sats dropped several time to about 60%! His baseline was only low 90s so this is a huge dissapointment. We are off to our local now for a heart echo, bloods and chest x-ray to see if there is anything nasty going on - all very worrying (although, apart from the odd blue-tinged moment, and being a bit more tired than usual, Wills looks absolutely fine!

Bad day in the office...

...for our poor respite nurse yesterday. We have recently found a lovely nurse from an agency to come a day a week so I can get out and have work meetings etc. Things had been going really well and William had behaved himself when she was here...until yesterday, on the hottest, closest and most environmentally stressful day of the year! His pump was alarming and occluding. This is all well and good on a ward when you can solve the problem together but she was faced with a misbehaving pump she knew nothing about. She called our community nurses who came and put two heads together and all was well...until William did a super nappy that spilt out all over his play pen mat! An hour or so later, the pump battery broke. This has never happened before and we just change the battery pack once a day so I hadn't shown her how to change them. So, William's TPN had to come to an early stop. Luckily, I was able to put it up early and add the volume missed so he didn't miss out on fluid on such a hot day. I popped back in on the way to pick up the girls from school as the community nurse had called to say she was at the house to flush his lines and the batteries seemed not to be able to plug into the pump. We solved the problem and I rushed for the girls. As soon as I left, William tripped up and cried himself into one of his "blue tinted moments"...something the nurse had not yet seen! All in all, not the best day in the office for the poor girl! I only hope we haven't scared her away because she is so good with him.

So, today there were many calls about salvaging the situation and sorting out some pump training etc for her so I think all will be well. There were also many calls about William's "blue tinted moments". He always recovers from them and we have got used to them but, as in his drunken tippy toe walking, once mentioned it becomes a bit more real. He is having an overnight saturation recorinding on Friday night, for the first time since coming off oxygen, just to make sure all is OK. We will also do some spot checks when he is running around. Hopefully, this is something and nothing. It would be so gutting to have him back on oxygen as that was such a positive result of the Nissens (reflux operation). Of course, it could be that the Nissens is getting loose and he is having silent reflux into his lungs again. Who knows...we'll see what the saturations are like and he will see our local consulant on Tuesday for a general review.

Sweltering in the heat!

Wow, isn't it hot! Poor Wills has been struggling today and went a worrying shade of pale with a blue tinge and very dark rings around his eyes towards bed time! We kept an eye on him and put it down to heat and dehydration. Following discussion with the gastro nurse, he is back on 20 hours of TPN with only a 4 hour break. This will just be for the duration of the heat wave a s6 hours running around with no fluid really is a bit much to ask. The girls came home hot from school and Paul and I have been very tetchy with each other! (this was unfortunate as Paul's Mum was with us this afternoon).

Thanks to vehicle cutters and a fire engine that pumps soft stuff, William will now touch play dough - a major triumph! We played with them and did some sticking. This was great because we really needed to keep him still and calm today.

Our weekend in France...

...Ok, so not really - that would have taken months of organising. Instead we created our own weekend in France...at home! Thanks to the French market that arrived in Croydon this week.







(I do have to admit - I can't take credit for these pics - found them on a website about same market in Woking ;-) )

Following Paul and I having a most chilled couple of hours at Borough Market, the children and I decided to go one better and take a "journey to France". Hope had a phrase book and bought everything in French, much to the delight of the stall holders. We bought bread, sausage, cheese, tomatoes, strawberries, french sweets, spices, olives and chocolate brioche (sp?). We came home for a french buffet (with a glass of wine for Mummy!) in the blazing sun and pretended we were in a cafe the Loire Valley (after a holiday a few years ago). We then pretended we were playing, watching the football and eating dinner in our cottage. This morning, we had chocolate brioche and milk or coffee in the garden for breakfast, again pretending we were in our cottage. After another french buffet for lunch, we made strawberry sorbet. We are still well stocked so we can keep our imaginations going a bit longer if we feel like some escape! Fantastic - a mediteranian weekend with TPN and not straying too far in case of the elusive call from GOSH! I'm sure to many, it would seem that life has dealt us a bad card, and it is certainly a tough one. However, it has made us more creative and adaptable. Where shall we go next weekend?

A catch up post

Well, we finally seem to be recovering from a hectic and stressful week last week. The house is finally clean and tidy again after all the celebrations last week Mum and Dad were here from Stoke-on-Trent Tuesday and Wednesday. One purpose of the visit was to collect the girls if Wills had gone into GOSH but they were also here to see us all following Hope's birthday and to visit CHASE for a Grandparent's session. It has been lovely to have them for a few days and actually relax with them rather than me being in hospital with Wills and them picking up the pieces with the girls. Paul has now begun Glyndeborne performances so will not be around in the evenings much until September, then briefly before going on tour so it was nice to have a busy house for the last few days.

William had his 2 year developmental check today. He had to see a member of the community paediatric team due to his "issues". It turned out to be really useful as she as able to hurry the physio appointment (thanks to Wills walking so badly she commented - "he really is trying..." - he runs at home, I told her, all be it in his unique wobbly style but he does it!). She has also referred him to a communiy paediatrician in the children with disability team whose role will be to co-ordinate with the many people involved in him and pull it all together, refer him to others where needed etc. That is great because we really need that. His feet were a cause of some concern as his arch and instep are now so high they were described as "deformed". If that is the case, they will, hopefully be corrected by boots and/or splints. She was delighted by his wonderful, social personality though - everyon who meets Wills loves him for that. I keep saying it, but I have never met a happier, more socialble little toddler - Hope and Ellie would agree with me too. After that, we had a brief coffee with Grandma and Grandad and said goodbye before popping home to take the TPN down and off to visit the Opportunity Playgroup for children with special needs. This was lovely and I am looking forward to William starting when a place comes up. He will have speah, physio and occupational therapy there. I do have to stay though as the staff are unable to so the medical procedures he needs. Another busy and hectic day!



A sad part of today was saying goodbye to our very best friends round here. The photo above shows us all together for a barbeque a few weeks ago (I, of course am taking the pics as usual). Clare is William's Godmother and has been an amazing support for us, having the girls over and over again when he has been suddenly taken ill. One of her daughters has been Ellie's total best friend from the first day she walked into school. We will all miss them so much but are looking forward to visiting them in Somerset. A great moment though was that one of the community nurses who looked after him when he was on oxygen, for weekly sleep studies etc, and who had left the team, found our number today and got in touch. We were becoming good friends and now there is no professional distance to be maintained I am really looking forward to developing our friendship. We are visiting her Monday and I can't wait! It has been a year since we last saw her, just as William was being diagnosed with "presumed CF" and she was so great through it all. It took me ages to describe how we got from there to where we are with William now!

Ellie's turn for centre stage!

Well, the a "sleep over" was the wrong word for what went on in Hope's room last night. There was very little sleep! The girls had such a great time and it really seemed to go down as a coming of age event! I was mighty pleased to be told I was "a really cool Mum" by one of Hope's friends. I'm not sure I was so cool when I went into their room at 1.30am and about 3am to tell them to be quiet! Most of the time they were and I did turn a blind eye to the fact they were on a pact to stay awake all night - we've all been there! It was only when they woke me that I reminded them of the others in the house - gently, I didn't want to spoile their special night as it was their first sleep over. There was make-up, chocolate, hair stuff and girliness in every inch of the room. You should see it today!

After a continental breakfast and the mundane task of supermarket shop to fill our fridge now we are not off to GOSH, the attention switched to Ellie. I was rather concerned as 2 parents phoned to say their girls had stomach bugs and couldn't come. I imagined them dropping like flies and a low turnout - maybe they have been eating too many Cadbury's chocolate ;-). It gor pretty hot so we opted for team games on paper like pictionary and drawing monsters and telling stories by forlding the page so no-one can see etc. It went down really well.




You may notice the absence of Hope on these pictures - she was totally crashed out on the sofa! It was a great afternoon. Well, the house is now trashed so I am mighty relieved I don't have to finish all my work and pack for GOSH tomorrow!

One of those days big time and no GOSH for now!

As I write, there are 4 very excited and happy girls upstairs telling each other ghost stories and discussing the boys in their class (and this is the 10th birthday - what is to come???)


It has been one of those days! We had clinc at 11 so I decided to go straight from school pick up, rather than pop home for about half an hour, and get a coffee when we got there. The trams decided to come to a complete standstill at Mitcham so we had to get a slow train to Victoria and then a very slow bus. I could have done that much quicker from East Croydon! So, we were late for, what was a very manic clinic. Still, we should have been seen by about 12.30 as they were running an hour late. We finally went in at 1.20 as several nurses who don't usually work there kept putting later appointment's notes on top of Williams and some parents were even switching them. We had a good old chat with the consultant though who is also beginning to feel that William's gut problems are syndrome related and agreed that he is very characteristic of Costello Syndrome. I am not going to go into any more about this syndrome yet. This time last year we were told it likely William had CF and told to read up and get involved etc. I have read a bit about Costello and agree it fits Wills very well but am not getting too involved until he has a clinical diagnosis at least. They are going to talk to the geneticists about it and I will post more if they agree. We also discussed the pros and cons of the illeostomy. It was nice because he shared that it had been a really mad clinic and was pleased to be able to relax with our appointment. It is nice when you get that rapport with your consultant. He stressed the need to get our local paediatrician more involved in overseeing William's increasingly complex and varied issues. Everyone agreed that his feet and walking are becoming an increasing problem and it could be that this, and the gastro problems, are neurological due to the syndrome (whatever it may turn out to be).

Anyway, I flushed Wills off the TPN and was set to rush and get the girls from school as time was now well and truely racing by. I got to the bus stop only to find I had left my handbag with absolutely everything in it! Needless to say, it wasn't there and no-one at the clinic had seen it. I couldn't believe it, I had had it 5 minutes before! Eventually, much to my relief, it turned up at security. By then, I was really getting late for the girls. The tram driver at Wimbledon watched me run passed him and pulled away as I reached the door (something Tramlink drivers are only too good at doing!). I was 30 minutes late for the girls.

Later, Hope went up to the shops for bread. While she was gone, I chased GOSH to be told that the child who was going home is not anymore and William will not be going in next week and may do the week after but then may not for weeks. It was such a comedown as I had been so high on adrenaline getting ready to go. I do feel for the family that thought they were going home though and understand how difficult it is to predict these things but it is hard to have a plan for the girls that is then not needed and just be expected to come up with another one in a few days or few weeks time! It is a rollercoaster, it really is. Anyway, as all this was going on, Hope came home and I even opened the door for her. 20 minutes later, I was getting worried that she hadn't got back so went looking for her, along with a neighbour I met on the way. I couldn't see her and came home thinking she would be there but didn's see her outside. So, getting seriously worried, I came in to think what to do next with the neighbour still with me -only to find she had been home all along and I had let her in! It had been a long day.

All the girls sleeping over called to make sure it was still on in case of a William crisis. One Mum, a good friend of mine, brought round a bottle of wine as I sounded so stressed - bless her. It has now been consumed and I am much calmer. The sleepover has been a huge success. Ellie did not get the predicted strop about being left out and is still having her party tomorrow as life is very much in the air and unpredictable at the moment (GOSH could still ring Monday and say later this week!). Mind you, it always has the potential to be like this. We really do live one day at a time these days.

GOSH on Monday!!!

A couple more photos from Little Chaser's last week - William very grown up on the train and another from the music session.

Well, we got the call. William's bed at GOSH should be available for him on Monday! Much sooner than we expected and I have very mixed thoughts about it. My head is saying it is good that we are going so soon as we can't move forward until we get these last tests done. However, my heart is struggling with the thought of being seperated from the girls once again and feeling so isolated in hospital with William in the evening when he is asleep. Paul will be so busy at Glyndeborne and won't get back until midnight once the shows kick in so we won't see a great deal of him. I hope this stay won't be long but we have been booked into a long stay bed. I am also nervous of what they may come up with as a diagnosis. The community nurses today agreed that his walking is getting worse and his balance and gross motor control is clumsy and unsteady. I just hope that there is no brain involvement. He clearly has "syndrome features" I can see that (although I must say they suit him very well and he is beautiful!) and so many syndromes have brain involvement. I guess, we fear that his walking and balance is worsening so where could that lead? Of course, it may only be that he is deficient in some mineral in the TPN and correcting that could see him walking tightropes! All will soon become clearer and that is great, but scarey too. So far, everytime we have been in hospital for tests new things have been found and the picture has become more complicated. However, there does seem to be a cluster of syndromes that he does fit so, hopefully, things will get clearer soon. Once we know what we are up against we will be able to plan and ensure he reaches his potential in every way and has the best life possible. Then, of course, there is the issue of the transplant assessment that may come soon after this hospital stay.

It's a good job I moved Ellie's party to Saturday. We will, at least, get the girl's birthday parties in before Monday. Hope's birthday is tomorrow so I must get wrapping...

Great Ormond Steet - At last!

Well, we finally heard from GOSH this morning to say that William will be admitted within the next few weeks. Apparently, they plan to have him in "long term" to assess his clinical picture etc before planning their tests. I have heard that GOSH tend to disregard everyone elses tests and start from scratch! Well, there are still a lot of unanswered questions so a new pair of eyes over everything may not be too bad a thing. There is more scope for multi-disciplinary discussion about his lungs, minor heart defect, co-ordination and walking and "syndrome facial features" etc in addition to his main, intestinal problems. I hope that long term does not mean too long. It doesn't look like we will get much warning when the bed comes available so we are now going to have to make sure we are always only a couple of days away from being able to pack up life and send the girls to Grandparents and Wills and me into the hospital. This will mean staying on top of washing etc and making sure I have all the stuff I need to carrry on with work etc. I will also be assembling some treats like a few good books, DVDs etc as the televisions on the patient line will, no doubt, go off at 9! C and W have manged to negotiate all night TV for long term parents which is so great! Paul will be really busy with Glyndeborne so there won't be home until midnight most of the time so it is going to be a bit lonely. In the meantime, ellie's birthday party has now been bought forward to next weekend to make sure we get it in. Hope has hers on Friday anyway so it will be a weekend of it! Poor Ellie, This will be the 3rd time she has had William and hospital overtaking her birthday - he was born the day after her birthday so I was in hospital then, last year he was in the Brompton for it, as well as his first birthday, and this year looks almost set to be another year where he will be in hospital for both Ellie's and his own birthday. Poor Wills has not yet had a birthday or Christmas uninterrupted by hospital! Of course, this isn't too bad for him, in fact he probably enjoys the extra attention, but is tough on the girls - and me. Who knows, we may be out by then but Ellie's party was planned for before the end of term, otherwise, everyone goes away, so we definately need to get that in quickly. Of course, we were told "within weeks" back in March but this time it is in writing. We could still be waiting at Christmas though!!!

Little Chasers

We had a lovely time yesterday at "Little Chaser's" pre-school group at CHASE Christopher's Hospice. It was a music therapy session and William was totally captivated! He couldn't keep his eyes off the leader and smiled and smiled, joining in with the singing and playing instruments as best he could. He was most cross to be dragged out for a while to be assessed by the physio. The verdict was that he does have a tendency to walk on his toes but that could be a habit. However, he seems to have a slightly weaker left side and his tendons are a little tighter. This makes him pick up and bend his leg more on the right, resulting in a bit of a "drunken swagger" leading to lots of falls. His feet are also a bit too pointy with a big instep - more like adult than childrens feet. This would not be too much of a concern apart from the fact it is getting worse rather than better. She is writing a report to our local paediatrician and physis as it needs further investigation. Naturally, we really hope that he isn't going to have a physical disability. He is so determined though and still runs and runs, even though he swaggers into things and falls flat on his face so much at the moment. He reminds us of the song "I get knocked down, but I get up again, you'll never going to keep me down..." He falls and struggles so much more than the girls did yet only cried for a few seconds - such a little fighter!

Ellie is back tomorrow from her school journey to an activity centre. It has been very quiet as Hope has had no-one to argue with but we have missed her loads. William had a respite nurse in on Tuesday so, with Ellie away, Hope had a very rare couple of hours after school alone with Mummy! We spent the time shopping for her "girlie pampering sleep over" she is having next Friday for her birthday. It will be her first sleep over - please stay well Wills!

Walking on Tippitoes

Poor Wills is really struggling with the heat and spiking temperatures very close to our magic 38 degrees at which he has to go into hospital! His nappies are drying up but we are still waiting on the TPN company to see if they can get a stable bag of TPN with even more fluid than the extra he already has to replace his gastrostomy losses. So far, they have not been able to make the solution with extra fluid stable. He is such a sweaty little thing. Still he smiles though!!!

The really worrying thing this morning is his walking. He is walking up on his toes most of the time and wobbles and falls over all the time. This has been getting worse but neither Paul or I have wanted to voice it for fear of making another problem real! However, he now has now been referred for a physio assessment. He seems to have very tight tendons that force him on his toes and his instep is getting so high we struggle to get shoes to fit. He may need to have special little boots to correct it. The question is why though? It may be that this is another feature of a "syndrome". I would be so sad if it turned out that he has some physical disability as well as his medical symptoms. Mind you, William is one little boy who will not let anything stop him. OK, he falls over a lot but he very rarely cries and just picks himself up and gets on with it! He can go very fast on those little toes too!!!

Come on England!

We are just watching "The" match so thought we should share this picture of William with John Terry at Christmas last year, together with "Terry" the cuddly dog he gave William for Christmas. The Chelsea team came in with presents for the children as they are just up the road. it was very exciting for them all (well, the older ones amnyway!). Sadly, our close friends had lost their little Riley in the early hours of the morning, having just got home after nearly a year in hospital so spirits were a bit down among the gastro team and parents. Riley was to be a year old in just 3 days time on Christmas Day! Still, the arrival of the team helped us get back some Christmas spirit and when the day came we all drank a toast to "Smiley Riley!"

Anyway, the purpose of this post was not to think about this but to share with you the picture of Wills with John Terry (although, the memory of Riley will always go with the happy memories of the day). William was lucky to get the captain himself giving him his toy - although the players did seem a little overwhelmed to see a baby on so many drip pumps he needed two drip stands! It was exciting to meet the team and we are cheering them on today with the other England players. I am looking forward to showing William his autographs and pictures in the future - especially if England win this year and the names of some of those he met go down in history - come on England!!!

A wonderful day at Legoland

We have had such a wonderful couple of days it really feels like we have had a weeks holiday. The zoo day was a real treat and then on Sunday we went to Christopher's Hospice for an overnight stay before Legoland on Monday. We didn't get William's swim as the pool was out of action for 24 months, following a "little accident" the day before and then the chemicals were wrong. We will get him in the pool eventually! As you will see later, he did get to use his suit again though. Instead, WIlliam enjoyed the trampoline with his sisters and was really getting confident. He love sitting and being bounced and even ran across it - much to everyone's amusement as he couldn't stop when he got off!
Once again, we were really chilled out in a couple of hours. This time, I got a complete break from all William's care as we have completed the care plans. It was hard to let go but I was determined and by the time we had finished tea William's TPN was up and running and he was even asleep before I could say goodnight! This was a big step towards, eventually, leaving him there for a night to go out and have some time with Paul and some friends.

Legoland was an amazing day. There were two families and we had clearly been very carefully matched for ages of the children and personalities etc. We all got on really well and had a lot of fun, as well as lots of heart-to-hearts between us Mums. We had made plans to meet up at the City Farm that is half way between us before lunch time! Ellie went round holding hands all day with her new friend and Hope and the eldest son were equally mature and serious about everything and engaged in amusing, friendly competitiveness on the rides that had a race element. Even Willliam had some similarities with his new little friend as there was some overlap in their conditions, especially regarding gut motility and absorption etc. She is not on TPN but nearly was at one point. This similarity led to an interesting insight - she is needing the same test we are waiting for at GOSH but, being a GOSH patient, knows the inside info - the machine (the only one in the country) is currently out of action and awaiting a part from Norway which no-one expects to get for months!!! If only we had been told that reason for the delay. William is still in increasing pain everyday so we need to discuss this situation when our consultant is back as it is unfair to keep him suffering indefinately as this test was supposed to be done prior to an illeostomy.

William loved Legoland, they all did. There is a lot for young children. He loved the model London, especially his beloved trains and buses! He couldn't wait to get off his TPN and start climbing in "Duploland" and have a ride on a lego train.

We went on fire engines, hot air ballooons, monorails, trains...the girls took their driving test and were treated to their license. The children had a really great time and were so happy all day. They felt very special going to the front with their exit passes as 3 people could go with each "disabled" person.

We all went on a very exciting water splash ride. Luckily they still let us get to the front although the two little ones with the "red stamps" clearly couldn't go on. They got their chance to get wet later.



William got to use his suit to discover the water jets and activities in the "Kids Wash". He could't have gone in their without the suit as he got dripping wet. He was scared at first as this is all so new to him and I did get some looks as he was screaming. He does for a while and then really enjoys the water. I'm sure some people thought it hugely over the top as he was in a dry suit!



It was an amazing day and we made some new friends which is great for all of us as the children now have some friends who are in the same boat as them. This was one of the main purposes behind the day - to give the children and Mums some fun and happy memories of a great day out and also to help families get together and form supportive friendships. CHASE has a motto "Just because a child's time may be limited it doesn't mean their life should be" The last few days have certainly been so for us, thanks to them! Both of us Mums were saying yesterday what a huge difference top our lives CHASE has made. She is now planning to do the Hydro-active as well for CHASE. Thanks to those who have sponsored me already - these photos today show where that money goes in making time-limited children have full lives that are anything but limited and helping families enjoy the time they have together. There is more about CHASE on my justgiving page which is linked on this blog, under the profile with the links (I can't do flashy links in the text but may get Paul to do it for me later!) We nearly had tp be blue lighted out though as there was an accident on the motorway, resulting in static traffic right into the carpark! William's TPN was due up and was already going to be an hour late once we had waited to see what was going on. Luckily we were shown the staff way out - an interesting tour of the backstage of Legoland.

The children were even giving spending money and, as I am writing, William is busy with his duplo that we added to with his, racing a duplo train Mummy helped him to make with his Bertie Bus. it is keeping him very happy in his pen on his TPN.

Wisley Gardens and Marwell Zoo

We had a lovely day yesterday. I love this picture of all 3 children having such a lovely time together at Wisley Gardens. They were so happy and it is the first time we have had a really long day out together since William was born really. We set off early and got to William's Granny's house in time to get William off his TPN - we had worked it forward so he could be put on before going to the zoo in the evening. His usual time would have been in the middle of the journey from Christopher's Hospice to the zoo. We flushed William off and he toddled and toddled in the garden while we all had an early lunch. We then set of for Wisley Gardens.

William loved feeding the ducks but was much more interested in the huge fish that were swimming so close to his feet...so clost that he wanted to climb in and join them

At least it looks like he isn't going to be scared of the water! (We were hoping to go swimming at the hospice tomorrow but they called earlier to say there was a little accident in the pool this afternoon and it needs to be shut for 24 hours!)

After a few hours at Wisley we were off the Christopher's for some tea and to get William set up on his TPN and then off to the zoo. It was quite a drive and we were in convoy with other families in their cars and loads of taxis. We nearly ran out of petrol as we were depending on a station in the last bit when we couild easily find the zoo - but there just wasn't one! Luckily the taxi drivers found us one just a bit further on with their GPS systems. William was absolutely fascinated with every animal. This picture below shows him wide eyed in the giraffe house - they were being fed and were just feet away. He was amazed! His little face was much the same looking at every animal.

We saw so many animals and William really was interested in all of them. It was a great evening. The zoo held a special, free opening for children from hospices, special schools and residential homes etc and treated them to the animals together with special talks and demonstrations, craft activities, characters wondering around, goodie bags and, apart from the animals, best of all for William miniture trains, tractors and...a visit from the local fire brigade and police. This was William's reaction to the fire engine...

He loved it so much that he just had to climb in and have a go!!!

All in all it was a fantastic day and we were so amazed at how Marwell Zoo did all this for these children and their families. Tomorrow, we are off to Christopher's and may get the chance to swim with William for the first time (if it is not too busy there is a brief slot between the 24 hours "decontamination" and William going back on TPN. Then on Monday, we are off to Legoland with CHASE. The only blot on a perfect time is that poor Wills is in a lot of pain at the moment and really suffering and screaming and crying - still smiling in between though!!!