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!!!

A quiet couple of days

Well, I think I am finally over the trip to Kings. I was so exhausted and achey all day yesterday and ended up in bed by 9! The girls' and I watched Bambi with our big bowl of popcorn yesterday afternoon. I have never actually seen it so it was nice to finally do do (although very sad!) We are all spending a lot of time re-reading Harry Potter at the moment. Hope, Ellie, Paul and I are all at it. I am on the last one and Paul the one before which is going to get very nasty soon as Paul spends about 3 hours a day on the train at the moment!! William enjoyed his Wiggle and Jiggle today but has had a really uncomfortable day with his tummy. His especially thick yukky aspirate is back, complete with the big "coffee granules" and he is "pooling" his output too which means it all hangs about and builds up in his system, resulting eventually in a huge, windy explosion which goes absolutely everywhere. Unfortunately, this causes loads of discomfort and pain. His tummy is hugely distended and his breath even smells so there is a lot building up today. Once he "explodes" we get a few days of constant output. I am really looking forward to him having an ileostomy now as it will make him so much more comfortable. I thought I'd never say that when the suggestion he may need one was first made. He is still smiling though - as always. When he was feeling it a bit today he looked at me and said "oh, Mummy, oh dear" with big eyes in the way children ask you to make it all better with a believe that you can. It was the first time he has done this as his language is improving. It was sweet but heartbreaking as there is noting I can do except massage him a bit and distract him with a favourite book.

Hopefully he will have a good day tomorrow because we are off to the zoo with CHASE. It is going to be a lovely day as we are having an early lunch at William's Granny's before a few hours running around at Wisley Gardens and then to Christopher's Hospice for an early tea at 4 and on to the zoo. William has never been before and, since the trip to the farm on Monday, has been animal mad. He has been looking at a picture book about a lion today and roaring at it so I think he is going to get really excited tomorrow. I only hope he stays awake as it is a special evening opening just for CHASE from 6-9! we should have some lovely pictures to put up here tomorrow night - hopefully of three excited children, not two excited girls and a sleeping toddler!