Rediscovering a social life!

Hooray, due to the fact we were supposed to be spending this week in Great Ormond Street, I actually managed to find some time to get some solid hours of work done, rather than snatching opportunities here and there, and, even better, remembering that it is really nice to talk to friends and meet people!

I have to apologise for being really rubbish at updating the blog this week though so I am going to have to go back to this time last week again. Hope spent last Saturday at a sleep over party so Ellie had some time for "mummy time". This was very well timed as the funfair had arrived just down the road. Ellie and I had a great time on the simulater and twister, eating candifloss and hotdogs and watching a pretty impressive firework display. It is very rare that we have these opportunities for one-to-one time and we both really appreciate it. Hope are planning an evening together soon. That one will probably be a little more cultured as Hope's is currently reading her way through Shakespeare re-written as introductory stories and is very keen on seeing a play for herself. She is also desperate to see a West End musical so we'll have to see what we can do!

Sunday was a big day for William as he started Sunday school. This is the first formal group he has been a part of and it will take some time for everyone to get used to his needs, especially the frustration and agitation that can come when he is overwhelmed through not being given sufficient time to process each individual piece of information he needs to react to. I will be staying with him for the time being but we plan to train enough people in basic Hickman line safety and pump alarms etc so he can have a one to one volunteer and I can spend some weeks in church for the whole service. He really is becoming quite the grown up boy and starts at the "Opportunity Playgroup" this week for 2 afternoons a week. Again, I need to stay as there is no nurse on site but will be in a parents room rather than with him. It will be so good for him to mix and play with other children and he wil get his speech and language therapy and occupational therapy there too.



One thing he will relaly enjoy at nursery is art and craft - he loves painting and sticking. He was making a really funky frog on Monday and ended up giving himself a rather punky look. This did not wash out with baby wipe so William was quite a scene on the school run home. The girls were really disappointed that I washed it all out in the bath!

On Thursday, I met Jo. Jo's little boy, Daniel, sadly died of complications following a liver and bowl transplant. He was a gorgeous little boy and you can read more about him at http://www.makingitbetter.org.uk/ (I still haven't learned those hyperlinks!). Jo and I had chatted on the phone but had not met. We had a lovely lunch together and I quickly felt I was making a new very close friend. After taking Jo to the running shop to get her running shoes, oh yes, she is another who can testify to the addictiveness of beginning with this sport, I picked up the girls and rushed to swimming lessons. There I chatted with Mechelle, another Mum I have met through her daughter and William having the same buggy. it is amazing what a close bond is struck up very quickly when two Mums, who have children with similar medical or special needs, open up and share their experiences, heartaches, hope and fears. Mechelle is moving up to Scotland today but we will stay in touch and saying goodbye was saying goodbye to a friend, even though we only met a couple of weeks ago. Later that evening Barbara, a friend fomr church came round for a chat. It was an amazing day as it has been ages since I have spent so long talking to friends. When things are very hectic and stressful, I am so tired when I finally finish everything that phoning a friend for a chat or to arrange to meet seems just too much. I really must make more effort though as it is so important. Paul also managed to hobble out (and fall over in spectacular fashion!) with his friends on Thursday. I'll let him tell you all about that on his blog (linked on this page). I did remark on the trend that seems to be emerging when he has a night out...



Today, we decided to make the best of the sunshine (between the showers) and met Jo and her son Alex at Crystal Palace Park. Hope had capsized her dingy 5 times today as she has progressed to the "Topper" which is a bit harder to control than the "Opy" she has sailed up to now. The poor thing was drenched and her face was a picture when I told her of the plans that had been made whilst she was on the lake. Even her spare clothes could have been rung out. A quick trip to a charity shop en route soon had her comfy again without breaking the bank! She will be using wet suits from now on... It was really nice to chat with Jo some more and the children had a great time. Last time William played on these swings he was on oxygen so it was lovely to see how far he has come. He had a great time walking around (with me running after him to steer him away from the swings and roundabouts). The girls held his hadn as we walked to the swings and it was so lovely to see them together. Ellie adored Alex and really enjoyed playing with him. I did get a couple of comments from other Mums about Williams unique little walking style. Both were very supportive and encouraging though as we watched William fall down adn pick himself up and wiggle around some more.

Next week is another busy one with the physio and occupational therapist coming round to see how we can brace his pelvis to make it more secure. We also have to sort out his buggy/wheelchair as he continues to slump in the current one but, more worrying, is that he is now tipping it back when he gets agitated, especially when he has his TPN on the back as the bag is pretty heavy. We have a couple of other appointments and William starts the playgroup so we have a new routine to get used to on Wednesday and Thursday.

Well, this has turned out to be a very long entry so thank-you and well done if you are still with me! There is a lot more I could have said too. I will be much better organised next week and will blog more as we go...

Another hectic week!

Well, it has been another crazy, hectic week - hence the first post since Sunday! On Monday, we went back to Chelsea and Westminster to have William's jejunal tube reinserted. This turned out to be particularly tricky as his stomach was well and truely refusing to let anything through (the huge amount of gastric aspirate pouring out of his gastrostomy was testimy to that!). The tube just would not go through the stomach into the jejenum. At one point it ended up folding back on itself right up into his oesophogus - the poor thing was so distressed and poor Grant, out gastro specialist nurse, was really upset at how difficult it was being for William. Eventually, we got it in place. We then rushed home to get Hope to her first secondary school open evening - for once William's TPN and drugs had to be done late when I got back! This was a lovely school and Hope's favourite - the whole issue of school applications and selections will, doubtlesly, form a topic for a blog entry later in the week!

On Tuesday William's CHASE nurse came round for a few hours so I got my first day off TPN since July - hooray!! This was great timing as Hope and I were off to another secondary school.

On Wednesday morning I went to an introductory session for Makaton sign language. This system of learning signs with language is really helping William's development. He only knows a few signs so far but these are helping him to learn to express things such as "please" to ask for something and "more". I am trying to learn enough to sign and speak to him as much as possible to help his language development.



William's physio came round on Thursday to bring William's new Piedro boots. They are absolutely great and look like trendy Timberlands. They help his walking a little bit but he needs a lumber support to help stabilise his pelvis. The physio and OT are coming on a joint visit to fit this in a couple of weeks. We also had our Portage session and began the lengthy task of assessing what he can and can't do in order to tailor his programme. Today, the OT came with his new chair. This is also great as it supports him really well. We are hoping to be able to do some work to get his hands working together more now he is better supported. We may have to try hand splints to stop his fingers bending back too much. He does need a wheelchair with similar trunk as he still has a tendency to fold in half in his buggy. We will sort all this out in the next few weeks but we really are getting there with his equipment and therapeutic needs.

We were all set to go into GOSH on Monday with transport booked, TPN arranged to be delivered there, Paul's Mum ready to look after the mechano leg and children...until 6.00 this evening when the registrar called to cancel his admission again! Apparently, the only doctor who can do the test he needs is not there next week. We don't really understand why he was booked in then, or why it took unitl 6.00 the Friday before to realise the problem as we had the letter a month ago and phoned several times to clarify information. Apparently, the doctor we need has full lists until the new year so Wiliam won't be going in this year! The annoying thing is that we have a lovely new integratged gastrostomy and jejenostomy we were hoping would be fitted while he was having another procedure. This would have been so much more comfortable for him and he wouldn't need to go through the jej tube being reinserted so regularly. It is also very necessary for him to have one, neat tube to fit with his pelvic support. I am hoping Chelsea and Westminster will be able to fit him in to have the tube changed soon. Oh well, at least next week is a calm week though as I have cancelled everything thinking we would be in GOSH!

Meeting of Angels

Well, we got through the week with nothing worse than a bit of extra weariness and tension in the inevitable arguments between Paul and myself during these challenging times. William went to see his gastro consultant on Friday. It was a bit of a hard appointment as I could tell he was noticing William's physical and cognitive limitations more. Mind you, William does have a tendency to retreat into "William land" staring up at the lights and shaking his head around to see the patterns it makes or, worse, get very agitated and bang his head on his pushchair when he is a) somewhere unfamiliar or b) over stimulated. A busy paediatric waiting room is both so they don't really see the best of him! We are fast learning that William can repond very well to things but needs time to process what he is experiencing and then form his repsonse. Hence, he will say goodbye to someone when they are already half way up the street and said goodbye to him several minutes ago. This is fine when he has one task and is given time and space to process it but when he is bombarded by several things such as several things to see, hear, touch...all at once, the time and space he needs to preocess each individual task gets into one huge busy Picadilly Circus style jam and he either retreats or gets very agitated. We have also found he gets very agitated if he his expectations change. We were walking home from taking Hope to the sailing lake on Saturday morning and walked past the tram stop we use to go home from school sometimes. It took him a long time to stop banging and shouting, much to my embarassment in the Co-op! This is, apparently, common in this kind of syndrome and due to problems in the neural connections so everything takes that little bit longer to travel and, in some cases, such as his intestines and part of his pelvis and feet, doesn't happen at all. As I have said before, time will tell what he is able and unable to do.

Unfortunately, we were unable to get his jej tube in as our gastro specialist nurse, the only man it seems able to do it in the entire world, was off sick. I am glad though as I would rather we wait until tomorrow than have a doctor think he/she can do it, only to put him through hours of pain and discomfort and still leave with no tube - this has happened before! The good thing about this was we had some time to pop down the road to the Brompton and introduce my little angel to THE Angel...

William was slightly shy around the tubes and drains that poor Emily is on at the moment but soon settled down into his usual sociable and entertaining self. Clearly Emily's room was much more calming than the clinic. It was lovely to see Emily again and introduce her to William. We tried to get some pictures, taken by Em's lovely friend. William eventually turned round but Em and I were so busy encouraging him to look at the camera that we forgot to do so ourselves! He was in very wriggly mood as he had just come off his TPN drip so wasn't really up for being held down for a photo.

This week is going to be just as mad, if not more so! We are hoping to get William's new buggy/wheelchair assessment in before GOSH next week. We also hope the physio will come with his Piedro boots and to discuss his pelvic bracing. He has portage (pre-school education support) and I have a half day Makaton (sign language) course. Tomorrow, we have to go back to Chelsea and Westminster to have his jej tube in. This would all be hectic but just about managable, however, on top of this Hope and I have 3 secondary schools to see in the evenings. So, there will be much rushing between William's stuff in the day to Hope's evening visits, with TPN and IV drugs to fit inbetween! Tomorrow is the worst as we are at the hospital in the late afternoon, an hour trip home, TPN and drugs to do and a school to get to for 7!!! I think I really do need to morph into Wonder Woman to get all that done on time - maybe I should practise that spin!!!

MRI Madness

Today has been a day off the week of mass medical appointments. However, it was just as busy as I caught up with work and housework. William had his MRI brain scan yesterday and what a palava that was! His jej tube was removed on Tuesday and I had already highlighted the fact that he would be unable to take oral sedation and, even if the tube was there, it is unlikely he would absorb it anyway. His consultant had planned to use sedation that can be absorbed through the mouth. However, no-one else knew of the plan! Everyone was nervous as his notes include a recent sleep study showing desaturations. This, together with the fact no-one had given the form of sedation I was describing, led the nurse on the day ward and the radiographer to conclude that William was not compatable with MRI at our local hospital. I was so frustrated as his tube had been removed specially and putting it back will involve at least an hour, nearer 2, of unpleasantness and discomfort for him. The radiographer said he was going to refuse the procedure and write to the consultant. At this point, I insisted that he speak to him to see if the stuation could be resolved. His consultant said to the nurse he didn't know where the concern about desats came from, I overheard her tell him on the phone this was from a letter he had written to William's gastroenterologist and read the quote to him. This was more than mildly amusing! In the end, they agreed to do it if the consultant gave the sedation and remained with William throughout. So...he came and gave the sedation. William became a little wobbly but no sleep...so he gave more...still awake...and more...yep, you guessed! Eventually we decided that the sedation was not working (surprise surpise!) He probably absorbed some through the mouth but did swallow some so it looks like not enough was absorbed to actually make him sleep, despite 3 doses! He was fighting the sleepiness he did feel. So, I had to get into a gown and go with him into the scanner. This was most uncomfortable and claustrophobic as there was barely space for him, let alone me lying beside him with my head on his chest and my arms cramped up with one stroking his face and the other up against the edge of the machine. We whiled away the time singing to immitate the noises it made. Surfice to say, the images were not great and the whole thing may have to be repeated...under IV sedation at Chelsea and Westminster of GOSH!

Since the scan William has leaked copious (even more than usual) amounts of aspirate from his stomach - so much so he has had to remain on free drainage to cope with it. He was really uncomfortable all afternoon yesterday. His gastro nurse specialist wonders if GOSH will try and give him a little feed into his gastrostomy to see what happens. It seems nothing much has changed and they will soon see what happens...William gives it all back again...with interest! His nurse also suggested that William may yet go to Birmingham for transplant assessment if GOSH agree this is the only solution to his intestinal failure. If so, they may well conclude that he is too complicated for transplant but at least he would get assessed individually and the pros and cons weighted for him, rather than a generalised decision. We will know more about the next stage after we have been to GOSH the week after next.

Wonderful CHASE!

We have just returned from a wonderfully relaxing weekend at CHASE at Christopher's Hospice. It really is the most amazing place, you feel yourself relax as soon as you hit the car park and are never ready to come home at the end of your stay! We left on Friday afternoon and that was far from relaxing. The CHASE minibus came to get us with a volunteer as Paul is still, clearly, unable to drive us anywhere and I am unable. I picked Hope and Ellie up from school early as the bus had to be back by 3.00. When we got back, the bus was already there and I was far from packed and ready! Normally, you could just throw things in a bag and pop into town for anything critical and forgotton. However, Hickman line flushes and IV giving sets don't tend to be available on the highstreet. Hence, such rushed packing is HUGELY stressful. The poor volunteer driver was trying his very best to calm the situation though and we were soon on our way.






Paul and I were able to relax together in the tranquil sensory garden while the children played far enough away, on the trampoline and swings, to maintain the peace. This was the first "quality time" we have had together since Paul's accident on July 12th. William did pop over with his nurse from time to time. There is a lovely iron tree water feature that drips away as if in a light summer shower. William became fasinated with this.
When we were not in relaxing mode we had great fun in the pool together (Paul had to remain dry side but did take a few pictures) and William and Hope had great fun in the music corner. Ellie just loved having lots of baby dolls and pushchairs and fancy dress to hereselt in a big enough space to play lots of make believe and charm the nurses into joining in as only Ellie can. We did find time to make a paper bead necklace together in the art room. It's funny, for all their craving of William free "Mummy time", when they get the chance, both girls would much rather play with the nurses - after all, Mummy is always there isn't she, even if she is a bit busy. However, CHASE nurses have to be enjoyed when you can! I think this was particularly so this time as William has been home (give or take a couple of weeks here and there for line infections) for such a long time now and the girls aren't feeling so Mummy starved. I did offer to take them to Guildford Castle as it was free for British Heritage Day but who wants a castle when you can have baby dolls and a big garden and corridoors to push them around, lots of nurses to charm and a corner full of musical instruments and a magic piano that lights up to show you how to play the classics!





We have never been to Christopher's at a weekend before. We are usually the only family there, with the other children alone while parents take respite on holidays. It was really good to share it with other families. it was lovely to sit and eat meals together and share experiences. It was also interesting to see how each family made the place their own and got what they needed from it in different ways and without getting in the way of each other. Sometimes, for us, this is all about the girls and I having fun and bouncing on the trampoline etc. There was plenty of that this time but the main need was for Paul and I to have time together as there is precious little of that by the time I have done everything William and the girls need, fetched and carried for Paul, cooked meals, cleaned up...etc... The only thing I didn't get was a break from the TPN. This is so specialised that there are not yet enough staff trained to be able to do this when we are there.

Today was a slam back into reality as we kick of the busiest week so far for William. We had an opthamology appointment today which went every well, although we do need to get William in his glasses more (at the moment they are at the optician though as they were a casualty of the weekend!). Tomorrow we have Portage and Grant, out gastro nurse, to take out William's jej tube, Wednesday is the MRI and Friday clinic and a new jej tube. Thank goodness for Thursday!

Ministry of Funny Walks

Well, the school run major juggling act is so far so good! Yesterday, we even managed the mile long walk to school in the morning. The clue has been to leave William in the cot with books, dressed and ready to go, until the last minute and then put him straight into the buggy. The girls are enjoying the "Weetabix week" as in the advert so are having me dream up different toppings each morning. This has been greatly helped by the family organic box we are getting again. We have done plain with milk, apple, banana, yogurt. Tomorrow is strawberries. With William in the cot, the girls get a bit of "Mummy time" to discuss the day ahead too. Quality "Mummy time" is in very short supply at the moment and the girls, especially Hope, are feeling it. At least they will get some at the hospice at the weekend while William is being looked after.

Ellie is very excited about being in the juniors now. The girls are at the same school at the moment and, today, the problems with that began to emerge with each running out of school telling tales of each other's faults in various playground squabbles and fallouts. They were swiftly told to keep their noses out of each other's business. Let's hope the novelty soon wears off. I'm sure it will as Hope, in year 6, will soon tire of year 3 playground politics.

Meanwhile, William had his orthotic appointment today. He had is walking analysed and, as in the sitting posture, it seems the main problem is hyperflexibility and low muscle tone, globally throughout his body but particularly in his lumbar and pelvic regions. They are going to give him Piedro Boots to help him feel more stable and supports for his lumber and pelvic regions. He has a very strange, wobbly gait and walks in his toes a lot, partly because he has a foot deformity (ped cuvus?) but mainly because he has developed this is a strategy to make his legs feel stronger. He is such a little fighter. Many children woud just not walk if they felt wobbly and unsafe but he is developing ways to make himself feel a bit better - all be it ineffective in some ways as he keeps falling but the way he walks is very funny. However, this is causing his achilles tendons to tighten. He falls so much when he is tired we can only let him walk for short periods and may be advised to use a soft crash helmet when he is particularly wobbly. He managed several falls, including a spectacular thump, putting his teeth through his lip, while being assessed.

Still, he gets around as this picture of him setting the world to rights with his special friend, Adam, shows.

While Mummy was away running and making friends...

... the children were at the CHASE family fun day with Paul's Mum and Dad.




They had a fantastic time. The highlight for Ellie was meeting up again with her special friend who came with us to Legoland and has played with Ellie at the sibling day. They really are like two peas in a pod. Hope's highlight was the chocolate fountain! As for William, well, there was a miniture steam train so need I say more. He enjoyed riding on it and got very upset when he was removed from watching it, instead making a beeline back at every opportunity. He also met Piu, a CBEEBIES presenter who used to be Poo from the Teletubbies. I don't think he recognised her but he has a lovely signed photo for his collection.

Following such an exciting day yesterday, I found myself registering for Losely 10K, a run in aid of CHASE so my entry fee will go there which is great. I have absolutely no idea if I can do that but have 2 months to find out, as it is on November 5th. It is far too soon to do another sponsored event but if anyone reading this didn't get round to sponsoring me for the Hydro Active you can still use the Justgiving page (www.justgiving.com/williammilne - I really must learn how to do those hyer links) through October. Any little extra helps make smiley faces like those in the pictures above. I also decided today to join a running club. Well, if I am going to keep at this why do it in the cold and dark alone when I can finish in the clubhouse with a nice glass of wine, making new friends. I am realistic though and know I can only do this on tip top days when my lungs are really behving themselves and not at all tight and twitchy. I am so inspired by what Emily achieved yesterday with her tiny lungs and know I can make more of mine, cautiously though. I also find running such an amazing way to get rid of adrenaline caused by stress. After all, it is there for fight or flight! It also helps me think things through. Whether or not it is at all possible to keep going through the winter coughs and colds I don't know - time will tell.

Another big piece of news today is William is finally going into Great Ormond Street for 5 days on 25th September. This is not for the big tests he was due for but it will get him on their system which is a start.

Well, tomorrow is back to school. I don't know how I will manage William and Paul and school run. That will, no doubt, feature on tomorrow's blog (see Charlie - back to normal service here!). William has speech and language therapy tomorrow and fittings for his boots and foot supports on Wednesday. We are off to CHASE on Friday for a weekend of R and R - can't wait! This is especially good timing as next week is a crazy one with Opthamology on Monday, Grant, the gastro specialty nurse coming on Tuesday to take his jej-tube out (as it contains metal, not good in an MRI scanner) brain MRI scan Wednesday and Chelsea and Westminster for clinic and insertion of a new jej-tube on Friday!

Hydro Active Challenge 2006

The hydroactive challenge day finally arrived today. Last night, I went out for a meal with some of the Emily's Angels team. It was gerat meeting people last night and today, especially people who I have got to know very well through the message boards and blog. It was fantastic to get out and have a nice evening out. I spent the previous 3 days working William's TPN time gradually earlier so I could get ready, put him up and get out on time. So, it can be done!

What was even more amazing is that I managed to get William up and ready to go to the family fun day at Chase and myself up and ready before 8.00 to be on my way for the Hydro Active. It was a lovely fresh morning and I felt full of excitment and anticipation about the challenge I had set myself of running the race without having to walk more than a few seconds to get my breath. One thing I have manged to get nailed during the training is slowing the pace right down rather than walking to take my inhaler and to take it early as soon as the chest tightness begins. The trouble with asthma is that no two days are the same and, while on one, I may find an adrenaline fueled run not too hard at all, another may find me unable to even run for a bus. In the gap between high pollen counts and winter colds and chest infections, today felt good. The second, and most important reason for the anticpation was meeting people who have become such good "e-friends". I was looking forward to seeing those I had met the night before again and I was especially looking forward to meeting Shadow and Emma for the first time. I was also full of excitement for Emily and so hoping she had woken up well and ready to walk over that finish line (I don't know how to do fancy hyperlinks so amd going to have to resort to giving you the address to Emily's blog, together with a huge recommendation that those of you who don't already read her blog look at today's entry to read her inspiring and emotional account of her day today - http://tinypoppet.blogspot.com).)

There were already some charity running vests to be seen at East Croydon station and the concentration of these increased the closer I got to Hyde Park. I walked from the tube station with a lady who has run many Marathons and runs for Chase and raised over £25 000 through her efforts. I felt like a child in a new country in comparison to her experience as we walked into the Hydro Active compound. I really enjoyed seeing all the people milling around in their kaleidescope of running vests and T-shirts, the charity stands, banners and balloons, hydration stations etc. My companion agreed how exciting each event is and how addictive it can be. I must say, I think she could be right.

I was worried that I might not have been able to find the other angels as they would be meeting at F and Chase at C - it turned out that the two charities were placed right next door! I bobbed between the two trying to meet as many people as possible.



The Chase Team (part of them - another picture was taken later when everyone was here)



Emily before the race, conserving energy and being incredibly still and quiet for an excited Em!


Finally, we were off. I was absolutely determined to run to placed myself at the back of the joggers. There was a bottleneck at the start so it took a very long while to get into stride. It was incredibly painful on the knees to run so slowly. The hardest bit was at 3K when the pain was beginning to bite and there was still a good chunk of the race to go. It was still hard work at 4K but, by then, there was no way I was going to walk having got so far and with such a little way to go. There was a hill towards the end - cruel! I still managed to keep going and was running on neat adrenaline by then. It was an amazing feeling to turn the corner and see the finish line. I even managed a sprint (well, sprint for me) and did not need the encouragement from the tannoy to wave and smile as I approached it. I had done it, reached my challenge of running the whole 5K and in, what I was later told was a respectable time, of 30 minutes. I was sad not to have seen Emily cross the line and was hoping to get back and see her. My original plan was to walk with her and the other angels but, somehow, I needed a personal challenge at this point in time and, I must say, the training has really helped with the stress. We have been living on adrenaline lately and "flight" is a much better way to get rid of the buld up than "fight" - Paul will certainly second that!



Emily shortly after walking 550 metres to cross the finish line!



Emily with her trainers (physios) after the race.



Lainy, Suzie and her sister, Livvy, Gill and her daughter after finishing the challenge.


After the challenge was over, we had a lovely picnic in the park where we got to know our "virtual friends" for real and relaxed in their company. The sun was shining and it really was a special time I will certainly never forget.





R-L: Livvy, Lainy, Gill and me




Emily and a very special angel







Emma (Emmie) being a very funky angel!







Andy being a fantastic sport and raising even more money for the CF Trust





Charlie and Abigail






Emily with the 'CF Mums" L-R: Lainy, Livvy, Shadow, Emily, Gill and Suzie


All in all, it was a fantastic day and I am suitably tired but for all the right reasons! Hope, Ellie and William also had a great day at the Chase family fun day with Paul's parents (while Paul had a nice quiet day at home alone) but that is a whole new topic for tomorrow as I'm sure that is enough of my ramblings for today and if I keep writing and add any more pictures I think this Blog will crash!

Sleep Well Little Alex

Well, yesterday we said goodbye to little Alex. Alex struggled for 15 months with heart and lung problems. He beat all the odds to make it home for a few months where he had a wonderful time with his parents and stayed amazingly well. Sadly, he had a heart attack last week and died suddenly at home. Like many of these children, Alex was a happy little boy and enjoyed making the best of his life when he was well. It is so sad when, these days, it is the funerals of such young children that bring new friends and acquaintences together. Alex made a huge impression in his short life and there were a lot of Brompton nurses, as well as well as the parents Alex's parents got to know, some of whom still have their children, others who have already lost them. It is getting harder to cope with these occasions. I was so gutted when Riley died and now find myself heartbroken for the child who we are remembering, as well as mourning again for Riley and then, once the floodgates are open, all our own heart break for William comes pouring out at the end. The stupid thing about this is that, at the wake when everyone is taking a deep breath and trying to enjoy the day, I am still all churned up and well up again and again. I was not the only one, one poor nurse was absolutely shattered and found herself questioning her ability to carry on working in a hospital where so many children have life theratening illnesses.

I had shared many open and frank converations with Alex's parents over the last year and yesterday was no different. It was nice to know that earlier conversations were still remembered and that I may have been able to help them work out some of their feelings. One of the strangest and saddest conversations I had was with Alex's Mum and another Mum whose baby died last year and whose funeral I also attended. We were talking about coffins and choices for readings and how little guidance there is out there for parents who loose young children. We moved on to compare grave plot prices and procedures and how it would be good to have a guide - even joking about the need for a mother and baby type magazine. How tragic that 3 young Mums should be discussing such things rather than the best buggy for toddlers and the price of Clarks shoes! The most poignant topic of this conversation was "Are you still a Mum when your only child has died" Alex was an only child and yes absolutely was the reassurance us others gave! You can never stop being Mummy to your children, not when they grow up and move away and certainly not if they tragically die so young.

You go through such a mixture of emotions on a day like this. The strongest is unbelievable pain and sadness for the family. With that is, of course, relief that your child is still alive but this is coupled somehow with a guilt. You also worry and feel the need to phone home and check all is well at the earliest opportuniy. Later comes the enormous sense of responsibilty as bereaved parents advise and request that you look after your child and keep in touch. I remember Riley's Mum, Helen telling me about such a conversation and later, when he died, how that Mum was the hardest to tell as she felt she had let her down. I know exactly how she felt! As more and more of William's friends pass away it is hard to fight the irrational fear that the net is closing and he will be next. There are only 3 children left of all those we have become closest to in hospital over the last year. Of course, we have met many more children with and without life threatening conditions since. The harsh reality is that we have an increasing circle of friends who have children with life threatening and life limiting conditions and as the years pass we will loose some of these precious children. One lesson I still have not learned is to bring hankies or tissues to funerals! I spent the entire service with tears dripping off my nose. They should hand one out with the order or service!

Rest in peace and breathe easy now little Alex. I know there are some very special little children up there in heaven with you. I hope they are looking after you and you are all happy there doing all those things you could never do in your lives. Be happy!

Family fun!

Today was a most excellent day! William had a heart echo this morning and, for once, we got the all clear! He did have a branch pulmonary stenosis when he was a baby but this appears to have sloved itself, at least for the time being. Heart conditions are prevelent among children with the kind of syndromes William has. For now, all is well but he will be monitored for conditions such as cardiomyopathy throughout his childhood.

The rest of the day was devoted to the family fun day held by our chidren's hospital at home team. This was great! There were loads of games for the children to play, a magician, bubbles, Thomas the Tank Engine ball pond - much loved by William. The best thing of all for Hope was the sailing. She loved it and wants me to take her to the sailing club on Saturday mornings so she can do more. Ellie was too young to go sailing but had a great time having her face painted, playing games and watching the magic, maing new friends as always. There was even massage and aromatherapy for the parents. I have never had a massage before but was being very strongly encouraged to do so. I must say, it was amazingly relaxing, although a little painful at times due to the many knots. Just as all was silent and calm as we were finishing I was lept back into life suddenly by my phone ringing with William's OT - well, the minutes in my own world were great while they lasted! I actually felt dizzy when we were finished. It was a fantastic day, a lot of fun and all the children really enjoyed themselves. We won a raffle prize too - tickets for the Polka Theatre, a children's theatre in Wimbledon.

Even closer to a diagnosis...

We went to see the geneticist today. This was an appointment I have been looking forward to for some time as this is the person whose job it is to put all the bits of pieces that are misbehaving for William together to diagnose an underlying genetic condition. It is a very interesting appointment and the methods are very different to others. Out geneticist is a lovely woman which is important as we really do need to be able to trust her and also because it can be quite upsetting for someone to point out all the aspects of William's appearance that suggest various genetic syndromes. She is lovely about all this and stresses how gorgeous William is. I must say, I totally agree with that. The conclusion today was that William is in the ball park of three related syndromes, Costello, Cardio-facial-cutaneous (CFC) and Noonans. These syndromes overlap quite a lot and testing for them is in its infancy. They are all pretty rare. There are tests, but not all children who have previously been diagnosed clinically are coming up positive on them. This could be because there are other genetic faults that lead to them, or could be that there are other, yet unknown or unnamed, related syndromes. William has a lot of features of CFC and Costello. The geneticist thinks CFC but he would have very extreme gut problems for a child with CFC. He has most of the characteristics of Costello but lacks a couple of key features. It is unlikely to be Noonans as he has too many severe problems. The next stage is to discuss his case with geneticists in Manchester who specialise in these syndromes. They will be asked which of them they think he could be given a clinical diagnosis of and what genetic tests should be done. Of course, it could be that he has a different genetic fault that has features of these two syndromes but we hope to be able to at least have a diagnosis of "syndrome similar to x (Costello or CFC). She agreed that neural cell migration disorder is the likley explanation for William's impairments and that these syndromes would be the causes. So, we can now say that he has neural cell migration disorder caused by one of these syndromes. If you are still with me you are doing very well. This is all highly complicated and could take a long time to sort out for definate. He will now see the geneticist every year to review the diagnosis and map his progress. There will be discussions and letters and, perhaps, genetic tests in the meantime so we hope to have a clearer diagnosis before then.

Always in our hearts and minds...

Today has been one of those unbearably sad days when you hear the news from a friend that their child has died. Today we heard about Alex. We met Alex's parents in The Royal Brompton hospital about a year ago. We have spent many a few weeks in together and his Mum and I would often keep each over going over a glass or two of wine and some food. We had so many heart to hearts about "our boys and the trouble they caused". When we moved down the road to the Chelsea and Westminster, we maintained this support. Alex spent many happy months at home with his parents, despite the fact that, this time last year, there were fears he may not even get out of ITU. Alex would have been 16 months old today. In the last year we have seen 8 children known to us sadly pass away, all of them under 2 years of age. Of these, 2 were especially close. Alex and, of course, our little smiley Riley.

If you have been following this blog, you will have heard of Riley before so let me introduce his gorgeous little face. Last Sunday, 3 of the children who were treated by the gastroenterology team at Chelsea and Westminster, who died in the last year, were remembered by their families and friends and some of the staff who shared their short but beautiful lives. It really was a lovely afternoon. We sat in the park and read out our thoughts and memories and stuck pictures and messages onto collages. After this, we had a picnic and carried on chatting about the children. Riley's Mummy and Daddy, Helen and Adam and I will always chat about Riley and all the things we did together when we shared those 6 weeks living in that room. We miss him so much and William will always be told about his first best mate! Riley was a brave little fighter, as was Alex. We will always remember them. Their time here may have been short but they touched our hearts and our lives. I certainly have some of the best friends I will ever have through sharing the joys and the heartache bought about by their wonderul little boy.

A very busy week

I have just noticed it has been a week since posting on this blog. It has been a very busy week but this gap is still pretty poor as I do like to try and post something more often. We have had lots on for William this week, the busiest day being Thursday when we had our CHASE repite nurse, together with a colleague learning all about William, our speech and language therapist and our health visitor all at the same time! This followed a later morning family play session for "special needs" children at Surestart. The picures show William playing in a car in the soft play room and playing in the multisensory room - something he really enjoys. Anyway, I'm jumping ahead of myself here. Monday started as a relatively quiet day with only weekly bloods and dressing change. William currently has a rather high urea and creatinine level, probably due to his kidneys being slightly miffed by the IV antibiotic cocktail he has just finished. He is also getting anaemic again already which is a bit of a concern as he only had a blood transfusion 2 weeks ago. We ended up spending the afternoon haveing an x-ray as William's jejenal tube had moved a bit. No-one could tell if it was still in the same place but the adult A and E reg was most interested in the image of his intestines as they really looked pretty static with not much activity going on. He could tell that he had intestinal failure!

On Tuesday, William had an OT assessment. This was hugely helpful but a little hard as it is felt that he needs a much more substantial supportive chair than I had thought. His pelvic control is very poor so he needs a lot of support there which means a moulded chair with a lot of straps and padding. The hope is that this will enable him to use both hands together as, in his current chair, he constantly relies on leaning on one hand to stabilise himself. He may need splints on his hands to stop the hyperflexibility that makes it hard for him to pick things up because his fingers can bend back rather than being able to grasp. The OT and physio are going to do a joint visit to look at his walking to see if any bracing may help. We will keep his buggy for now but he may need a more supportive wheelchair to help his posture while he is out. I was quite upset about the level of support he needs but such support now will help him in the long run.

William had a play session on Wednesday morning and thouroughly enjoyed paining. Thursday was a super busy day. We had a great time at Surestart in the morning. William was totally overwhelmed by all the children in the softplay room at first. We had to retreat to the sensory room to calm down. This room is great for William as there is lots to stimulate him and for him to explore and it is also very relaxing for him (and me!). All the children had lunch together and William was totally happy, although he wanted to have a paper plate and a cup to play with. We started learning Makaton sign language with the speech and language therapist in the afternoon. This will help William understand language and enable him to express himself. We are teaching him to sign and say "please" this week - it is very slow progress but he will get there. We are also trying to help him to understand 2 words at a time - another slow process as he really struggles and even a 2 word phrase has to be broken down and demonstrated with toys to help him get the message - he hasn't yet but, again, we'll get there,

We spent Friday at Chelsea and Westminster to check the position of William's jejenostomy with a live scan and see the doctors on the ward round to save having to go to clinic next week. Nothing new to report there except a lot of weight loss from when he was ill over the last few weeks.

Next week is another big week. Hope and Ellie finally come home on Tuesday - hooray!!! William is seing the geneticist on Wednesday. This will be an important appointment as eveyone hopes she will be able to put all the latest bits and pieces together and diagnose a syndrome, perhaps Costello Syndrome or something like it. William also has a heart echo on Thursday as he has a rather unnerving habit of going grey and blue around his mouth and eyes. Somewhere among all that I need to fit in some training for the Hydro active challenge which is coming up very soon. I have nearly raised £300 for CHASE now and Emily's Angels have raised a whopping £6000 for the CF trust. There is a link to the CHASE sponsorship page on the side of this page and a link to Emily's page from the CHASE one. (I will come back and redo this section in proper html to tidy that up and turn the references into hyperlinks)

Paul is still staggering around. He also has clinic next week so we'll see how he is doing.

Well, Paul finally came home on Wednesday. It was all very sudden as he managed to do his flight of stairs and that was it. He called to say he would be home later that day. I was nowhere near ready and, with all William's IVs, had no time to be so at such short notice. The first couple of days were strange and I hardly seemed to have a second to myself with looking after Wills and Paul. Things are settling down as Paul gets more confidence to do things and we are getting used to being together again in these, rather strange, circumstances. We are also getting used to the shock of the accident and how we both feel about William. We haven't really had a chance to talk about our feelings in the last month and that is not healthy for a relationship when you have a child in William's situation. You have to talk about it or you bottle it up and build walls between each other. I know I have been doing this a bit over the last month and it takes time to open up again.

The main battle we are having at the moment is with our GP who, first of all, insisted that Paul presented his temporary registration form (Paul is still registered with the GP near his flat in Tooting) himself. So, I pushed him up in the wheelchair with William on his knee. This was very hard work as Paul weighs much more than me, even without William. When we have to get up and down kerbs, even with the ramps, I struggle to get enough weight on the back of the chair to lift it enough. The result is so comical that several people stopped to offer help and one guy even offered us a lift in the car. So, we got to the GP with the form and Paul's discharge letters and were told by the receptionist he had to come back in the morning. I explained that would be impossible beacuse of William on his TPN. The converstation went something like "Can't he get a cab" "No, he can't get his leg in a car unless it is a large one with plenty or leg room" "So what do you want me to do" (with more than a hint of sarcasm...) William's community nurses were left to sort it all out as they don't want me to get over stressed and tired and don't want me to clean Paul's pins for him because od cross infection risk into William's hickman line. They refused to come and give Paul a home visit but did send the district nurse. Paul will need some prescriptions at some point so we will have to get used to that wheelchair! It would be nice to go out and do something nice in it anyway - but with someone else so William can go in his own chair.

We have another busy week ahead with speech and language therapy and occupational therapy for William.The girls finally come home at the end of the week and I really can't wait to see them again!

New adjustments

Yesterday was a significant day in my journey with William. We spent most of the day sorting out the numerous bags of stuff that accumulated in the hospital. It was a very hazy day for me due to the lack of sleep associated with all the IV drugs, as well as our usual routine of nappy changes and aspirating William's gastric tube. Depsite the fact he is still very tired and under the weather, I decided to take William to the park as he had not had any fresh air for a week. Because of his neutropenia, he is not allowed to mix with other children at the moment. However, we were told that a trip to the park is OK as long as he doesn't get too close. As usual, William enjoyed the swings and toddling around watching the other children. His "William walk" was pretty pronounced, probably due to his tiredness and the fact he had been confined to a cot on 24 hour IV fluids most of the week. For the first time ever, another Mum commented on his "disability". I didn't mind the questions, we have been used to that since the day he left the hospital attached to an oxygen cylinder. This, however, is the first time somone has commented on his physical "impairments" rather than the health related stuff. I watched him walk away and could see that it has become very obvious, especially when he is tired.

The physiotherapist came round today with some Piedro boots to tide him over until we get our appointment for his special insole and own boots to be fitted. She had a few pairs that other children had finished with that hardly looked worn. We found a pair that fitted him (unfortunately, not the really cool blue ones she had!). They do look pretty funky - like "Kickers" or "Timberlands". They will look interesting with shorts though - like he is off for a hike! She had another look at him and his posture isn't perfect. His feet have deteriorated as they are not growing properly with him and are becoming more and more "deformed" as a result. We discussed the potential for using splints and braces etc. For now, we will try the boots and a more specialist chair, perhaps with a harness to keep his back straight. He will need to be referred to an orthopedic team to keep an eye on things. At the moment, there is little scope for surgical intervention but who knows what the future can bring. We will keep an eye on how his walking develops. At the moment, the physio is happy for him to stagger and fall as he has learned to just get up and carry on. It may well be that we have to use a walker later on if he becomes more unsteady. There are no exercises we can do to help at the moment. The things we can do are, luckily, fun stuff like soft play and swimming. William will begin hydrotherapy with the physios in September.

We have come to terms with William's medical issues. Now, we need to come to terms with physical disability and a degree of cognitive impairment. We have been slowly realising this but it really is time to get our heads around the impact this could have for all of us. Through all this though, William is William. A lovely, happy little boy with so much going for him. The hard thing is that we don't know how these things will develop. William is a complicates and unique child (in so many ways!) and there are no clear predictions that can be made. This is very hard for me as I like to know what is ahead. He is having a brain MRI scan in a few weeks time to see if there are any obvious structural problems. The neurological condition the doctors think he has will not be seen in this but we need to make sure there is nothing else going on.

Meanwhile, home IV antibiotics are going well, although very tiring as they only allow 4 1/2 hours sleep between the end of the midnight infusion and preparing the next lot. Our excellent and very sensible community nurse pointed out to me in no uncertain terms today that 10 days of that on my own is totally unrealistic. No no, it's fine, I protested, until I realised that she wanted to arrange help and support not take him back in hospital. The great news is we have respite for night tomorrow, Wednesday and Thursday so I can have a break and some sleep (hooray!). They are also coming in to do the 12.00 lunch time ones so I can have a break.

The other good news is that Paul is coming home "in the next couple of days!". Suddenly, they seem to think he is ready (and they need his bed!).

Back from hospital

We are back from hospital. William managed to grow not one but three bugs in his line! He has been off his TPN since Monday as it is bug food as well as William food. His temperature is down cultures are now clear so we are home but on 3 lots of IV antibiotics. These pictures show how many drugs will be given in the next 10 days and how many he has in one go. These are given every 6 hours - a lot of work but I would so much rather have him home when he is well. He really was feeling very poorly at first, with a temp that kept spinking to 40 degrees. A blood transfusion gave him some energy and made him feel a bit better but he is still very tired and "lack lusture" Not himself at all! He is also neutropenic at the moment, meaning that one of the white cell counts is very low, leaving him susceptable to infections and struggling to fight them when they come. We have to keep him away from busy public places and other children until this improves as certain viruses and infections, such as chicken pox, could be really dangerous for him at the moment. We are all really hoping we get these bugs licked before this line gets colonised. We have been warned that two of them, pseudomonas patatas and acinobacter, can be pretty tenacious. It is a bit of a worrying time, expecially when we were only told on Friday that line infections, losing lines and running out of sites is a key concern for William.

Meanwhile, Paul has been up on crutches today and is pretty confident that home by Friday is not an impossible hope. If not Friday, it will certainly be the start of the following week. His main problem at the moment is that he is a lanky lad with not too much power strength - more your distance runner build. The orthopedic specialist nurse said the "footballers legs" progress quicker at this stage. Still, he has progressed from feeling dizzy sitting up and not manageing to stand for long to 2 laps round the gym on crutches in a week so this week could see a lot more progress. He now has to get stable on crutches, get a bit more stamina and learn the stairs. William has his physiotherapist on Monday coming with some Piedro Boots she has in the hope a pair will fit to keep him going until he gets his own with the special insoles. His neurological and cognitive issues are becoming yet more apparent and were discussed from time to time in the hospital. Everyone seems to think things are becoming clearer. We are all awaiting the results of an MRI scan in September to rule out any structural brain damage and the geneticist in a few weeks time. We have a physio and speech and language therapist now and he is on the waiting list for OT. He will start Portage in September so he is getting a lot of help. The speech and languate therapist came Monday, before he got ill. She was able to tell his understanding is poor for his age and he can only understand one word at a time. She will work with him fortnightly to see what we can do. This is all very helpful as it helps us to tailor our behaviour with him to help him get the best he can from things.

Hope and Ellie are enjoying being on Grandma and Grandad's narrow boat. They are having loads of fun. I really miss them but they are best off being where they can have fun rather than have their summer holiday dominated by IVs and mechano leg. I hope next summer is an easier one and we can all get away and have a good holiday!

A quickie to say...

Unfortunately, William is back in hospital with a line infection caused by bacteria from his gut. His temp rose to 40 degree celsius in mega quick fashion on Monday afternoon so we ended up being blue lighted to hospital. He is getting better now on 3 IV antibiotics, he is also neutropenic (low white cells) at the moment so it isn't helping. Meanwhile, Daddy is getting better and will be home at the beginning of next week. As soon as William has clear cultures in his line we can move to home IV antibiotics and finally be back together again. Then girls will be away another week or so though - not long before the Milne family (including Paul who technically is not a Milne) are back together again!

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...