Sunday, July 30, 2006

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...

Wednesday, July 26, 2006

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!

Thursday, July 20, 2006

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...

Thursday, July 13, 2006

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!

Tuesday, July 11, 2006

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.

Saturday, July 08, 2006

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!

Wednesday, July 05, 2006

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.

Monday, July 03, 2006

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.

Sunday, July 02, 2006

Our weekend in France...

...Ok, so not really - that would have taken months of organising. Instead we created our own weekend in 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?