Unwelcome return visitors

The full report on William's blood cultures came back today. He has the same bug in his line he had a few weeks ago :-( This is not good news as it means the little blighters have been hanging around and building up again when we stopped the IVs just a week or so before he got sick again. The IV of choice now is called imipenin. We were going to start that today but pharmacy felt it a bit too complicated to measure and prepare for home. It would have meant hour long infusions every 6 hours - not much chance to sleep. We can use something else called merepenin. This can be pushed rather than infused and is 8 hourly. He was on this last time. Fingers crossed it does the trick or we will have to go in to have imipenin, or may even loose the line :-( He only had it in at the end of August and has had 2 lots of infection, from the same but, already. I have to admit, I got a bit teary when they told me it was the same bug. He has had 7 line infections since the start of the summer. This year, he has lost 4 lines to infection. This is a bit worrying as there are only so many sites that can be used.

We have had a lovely day today though. William got his new static chair - he had chosen bright orange "like Murdoch" so I bought him a Murdoch engine to go with it. He has spent most of the day pushing this around the tray. When I did prize it off him, we collected leaves and painted them in Autumn colours for leaf printing (just like in CBEEBIES for those of you who have little ones too). Despite still feeling grotty, William had a "driving lesson" on 'bugsy', the special training powered wheelchair yesterday. He was really beginning to get the hang of it. We played games like throwing a ball and going after it. He was beginning to drive a lot more smoothly but still has a lot to learn before he will be deemed safe to drive and allowed his very own powered chair.

Another line infection!

William has been unwell since the weekend. He has bronchiolitis and has had some high fevers. We hoped this was all due to the virus but, yesterday, we got the call to say that one of his blood cultures does have a bug. Once again, it is one of his gut bugs. He had cultures earlier in the week that were clear so it looks like he has ended up with another line infection due to being run down and unwell. He has been neutropenic (low white cells) for the last 2 weeks so that doesn't help either. He is doing OK at home on the IV antibiotics so, hopefully, they will let us stay here.

A very, very long day...

We had our long anticipated appointment with the neuromuscular team at The Hammersmith Hospital yesterday. It was a very long day and left us all absolutely shattered. We left at 8, had a heinious public transport journey with 4 changes and got to the hospital just past 11. We were there until 4.30! No breaks 4 hours of physio, doctors and an ECG. Then we had the long, and much slower in rush hour, journey home. We finally got back at 7.

It was a strange clinic. Very old school. He had a physio assessment and she was very strict with him. I could see what she was trying to do to get him to bend his feet up etc but he didn't really understand her. He had lots of tears yesterday when physios and doctors were trying to straighten his feet and it really hurt him. After physio, I had to find a space to do his IVs. I had a message waiting on my phone to say he was even more neutropenic now so I wasn't overly comfortable that I had dragged him across London on public transport and into a hospital. The clinic is monthly though and we would have waited ages for another slot. The doctors consultation was very strange and frustrating. The reg came and went through all the history - and it is a long and complicated one! That was fine. I am getting bored of going through all this but it is important. Then, the consultant came in and she presented William to him. If he had been in from the start it would have saved about 20 minutes of history repetition! Another consultant came in so there was a neuromuscular guy and a mitochondrial disease specialist. They pretty much talked among themselves and then examined him, still talking among themselves in a language I had no comprehension of. They poked and prodded and stretched, making exclamations such as "oh yes, there is definately some xxxx here and yyyy there" They took loads of pictures of all his features such as his feet, wide set eyes, funny hair etc and added a few more features that they felt to be significant, such as sparse eyebrows and translucent skin. They did comment on how tiny he is too.
After all of that, they pulled their chairs around at the opposite end of the room and had a discussion about him. I was just an observer and hardly understood a word.

They did tell me that they need to do more tests. He doesn't have a muscular disease, in their opinion. He is too strong, all be it in short bursts, but he does have strength. They feel it is not peripheral but a central problem. They have some more wierd and wonderful ideas of very rare syndromes (some to do with glycogen storage, some mitochondrial) to rule out before they just conclude that William has a "unique phenotype and genotype". Like the geneticists, they pointed out how unique he looks and how it is clearly a genetic problem but that it does suit him and he is a lovely looking child. He is very different to the rest of us, as you can see in the picure, and has a very distinctive look. They also commented that Wills now has spacticity, particularly in his legs, this is a new development as they were not too tight until recently. He feet can no longer be manipulated back into 90 degrees.

I will look forward to the letter with the hope I can understand something from that.

Colds

Thanks for your wishes We went to the hospital to put Wills on his TPN. Thankfully, this time there was no repeat of the horrible evening a few weeks ago. We can't afford to take risks though and we will do exactly the same thing another time. All 3 children have rotten colds. Hope and Ellie are fine, just coughing and husky. William is termed a "medically fragile" child so anything extra takes its toll. We will keep an eye on him over the next few days, keep him on his monitors over night and have a low threshold for using oxygen if he struggles a bit over night. Winter is a hard time for these children and we feel like we are constantly on edge. I hope we have a healthy run soon. It is tough for the whole family as the grandparents are always wondering if they will be needed too. As you may have grasped from this blog of late, we can get a bit frazzled! Wills is booked into the hospice for New Year and the girls are at their Dad's. Paul and I are planning a couple of days away together for the first time since William was born. That should do us heaps of good

Love to all those other parent's of medically fragile little ones. I pray they all stay as healthy as possible over the winter months ahead.

The joys of winter!

William's temperature went up to 37.8 at playschool this morning. I called his gastro nurse who requested bloods when he came off TPN at two. Last time we had a day like this, with high temperatures but not quite high enough to hit the panic button, we ended up sending him into septic shock when we restarted his TPN in the evening, pushing all the nasty little bugs into his system. I just can't take that kind of risk again so we had arranged to take him to the local hospital to put his TPN up whenever there is any doubt that he may have a line infection. With a child like Wills, you have to presume a line infection whenever he spikes a fever over 38. So, at 37.8, we are pretty close!

I called the community nurses to arrange everything but they are pretty overworked today with one of them home with their own poorly child. Oh, the joys of winter! Sick children and a struggling NHS. It is so stressful when a bad day for Wills can result in a situation where his life is in danger. Of course, the staff to their best, but it is very disconcerting when your security net falls apart, right when you need it. The ward are full and not overly happy about us coming in tonight. I told her that I am just not prepared to do the TPN at home today. So, we will take him up to the ward and, if he gets very sick, the crash team will be called and he will be stabilised and sent to the nearest bed. He is coughing and wheezing so I hope that is all it is.

So, we wait again, to see what his bloods show, to see if he goes into sepsis or if this is just a winter virus. In the meantime, I will get the girls from school, tidy the house and make some contingency plans. There will be lots more days like this before the Spring.

Spidey Sense

It is really strange, all that Spidey Sense with me on Monday. Along side that, I posted it here and my Mum called me about 2 minutes later as she just happened to look there as I posted. Paul's Mum called on Tuesday morning to ask if Wills was OK as she had a feeling yesterday that things were not right and had just looked at the blog to see if there was anything to let her know if all was well or not. How very strange, Mum and both Grannies (at opposite ends of the country) feeling the Spidey Sense that all was not well - spooky! It must be the time of year

William's bloods show his HB is OKish (OK for him) but he is neutropenic, meaning that he does not have enough white cells to fight infection. This is usually an indication of something going on but, for now, his infection markers are OK. He seems OK in himself, apart from gas, tummy aches and a very very sore bottom. We just need to keep a close eye on things.

Spirals of unease

I have been feeling really uneasy about Wills all day. His play specialist came this morning and remarked on how pale he is. I agreed and she said, with much concern, that he looked a funny, horrible colour - grey really. She was right. He soon picked up but things don't seem right and I can't really put my finger on what it is. He doesn't have a fever. We took his routine bloods and cultures from his line so we will see if there is anything going on. He may just be very anaemic and need a blood transfusion. I hope so really because that will explain things and a blood transfusion will make things a lot better. He is really gassy at the moment and having a lot of pain and lots of bile draining from his stomach. He is clearly feeling yukky and this may have a lot to do with how pale he is. His nappies are pretty spectacular too!

I had a lovely run but started to think too much about Wills and what may happen. This helped to feed my sense of unease and upset. Occasionally, I get into a spiral where I feel nervous about things and then think that is must be due to some maternal intuition so worry even more. Today has been a bit like that. I have William on his monitor tonight. I'm sure all will be well and I will feel much better tomorrow.

The extra hour

So, what did you do with it? I had planned to get up and do William's IV drugs as if there was no extra hour, so at yesterday's 6am/today's 5am. I then planned to go out and run and be back by the time everyone else was busying around getting ready. Did that happen...er...no. Instead, my alarm went off at 5 but I switched it onto doze and ended up giving William his drugs at today's 7am - so an hour late if a normal day, now 2 hours late!

Oh well, it has been a very hectic couple of weeks. Paul has been singing in Birmingham most evenings, getting back in the early hours of the morning. Of course, that has made him very tired. It has also left me doing everything for Wills both late at night and early in the morning, making me tired too. We obviously needed the extra hour of sleep and i can get my run in later.

I have just realised that the hour is going to mess up all William's timings. The biggest impact will be on his TPN. It should stop at 2 but, of course, willl stop at 1 today - just as I am on the field finishing the football session. Not the best place for aseptic procedures! It is the last day of his IV antibiotics today - hooray!!! Now we have to keep everything crossed that the little blighters in his line have well and truley gone. He has been having a few low grade fevers over the last couple of days. Probably all due to winter viruses but we are really hoping the bugs won't come back. Our biggest fear is the yeast and fungal infections he often seems to get in his line after having domestos style IVs to kill the bacteria. A few nervy days of watching for signs and symptoms are ahead of us!

Half Term

The pictures show
1. ALl the medical stuff and nappies that arrived on Monday - what on earth am I supposed to do with all of that? It is a months supply of things we need for William and is totally filling our little "lean to" I did have plans for that to be all arty and bohemian when I bought this house...

2. Ellie arriving home from Honey Pot (Hope would not be photographed!)

3. William attached to his ECG. All smiles but he has been having a very uncomfortable week this week with lots of windy and colic. I am very sorry for the large hole in the ozone layer above Croydon that has appeared this week! We will be without a car from Monday so should be able to do some carbon offsetting.
The last few days have been absolutely crazy.

The girls came back from the "Honey Pot" holiday in the New Forest, on Tuesday, having had a fantastic time. They went to a theme park, a country park, an otter sanctuary, had a halloween party, a cook out...they even had clothes stall where they could choose from donated clothing - all for free. They came home with treats and things they had made. The idea is that this is a respite break for children who need a break from home for various reasons. The timing was great for Hope and Ellie with William having just been very poorly again. He is still on IV antibiotics so we have been unable to have any big days out this half term. It was great they had this instead. They will remain members of the "Honey Pot Club" until they are 12 (not long for Hope) and will have newsetters, a Christmas party, play bus visits and a weekend at the house in the New Forest every year. They so deserve this and I have loved hearing all about it. There is a bit of sadness too as I would much rather we could do more as a family.

We have tried to make the best of half term within our constaints. William had his 24 hour ECG on Wednesday - Thursday so the appointments to put it on and take it off munched up the mornings. We wondered around town and went out for lunch on Wednesday. Yesterday, William's Granny came and we baked and made things out of salt dough. We will be painting them later today.

All of William's activities and visitors stop for half term and he is getting very fed up being stuck in his chair on the TPN until 2 each day. Next week, he begins his "driving lessons" on his electric chair. That will give him a great lease of life as he will, at last, be mobile on his TPN His walking is getting more and more weak and wobbly too so the chair is going to be fantastic.

A good night's sleep then back to normal (I hope)

YAY, we got home today Wills is much better but we will still be doing the IV antibiotics for the next 12 days. I get really nervous doing his TPN after such a nasty line infection. The rate he goes downhill is so so scary and it takes a while to get the scene out of your head. Of course, being mindful of infection risk is important but you really need to stike a balance. Wills tends to get infections from inside his intestines that translocate into his lymphatic system and then settle into his line. It is good to know that our IV technique is not introducing bugs but worrying at the same time as we can do nothing to control William's gut bugs. His intestines don't move properly so bugs settle and breed. His recurrent infections are causing cause for concern, not least because he is developing a nasty habbit of getting septic shock every time!

His heart murmur is, thankfully, nothing to worry about. It was just because he was so ill. However, the irregular rythmn he seems to get from time to time is a bit more concerning. It may be that his neurological condition is affecting his heart. I was really upset to hear this. However, it sees that any impact his condition is, or may have, on his heart, is much more easily fixed than the intestinal failure he already has. He is going to have a 24 hour ECG over the next 2 weeks. If this reveals that his heart rate becomes too irratic, he will be assessed for a pacemaker. Strange isn't it. You can fix neurological failure of the heart to behave normally but there is little that can be done if the intestines fail.

Oh well, a good nights sleep and all will begin to settle back to normal tomorrow.

Here we go again!

Today should have been the start of the cross country season and I was looking forward to our first team league race and hoping to spend yesterday evening sorting my kit and changing my spikes from 12 to 9mm ones, bake some muffins for the girls after the race etc...

Instead, William, once again did his speciality of well to ill in life threatening measures in 0-60 seconds! It was all very stressful. The ambulance service and A and E were incredibly busy and William was not given the right code from the offset. He needed to be foot down and into resus but it took ages to get an ambulance for him (Hope and Ellie had been picked up and gone before it arrived!) and, when it did, Paul beat it to the hospital! We arrived to find no room in rescus and no doctor immediately able to see him. The triage nurse was not a paediatric nurse and thought TPN was a gastrostomy feed (which is not uncommon and forgivable as it is pretty specialised). She kept thinking his infection was on his gastrostomy site - which would not have been so worrying. Maybe the ambulance crew misunderstood too, hence their dithering. When she eventually did his obs, his temp was above 40 degrees and his heart rate 167! He was losing consciousness and his lips were white. It was very scary. The doctor came, A and E doctor, not a paediatrician and, understandably, wanted William's whole history. I just wanted to see someone who knew him and would see how ill he was. When the paeds reg appeared, and was someone who knows him - thank the Lord - she could see at once how ill William was and was furious that she had not been called down before and that the ambulance had not called in to get her down waiting. She had him up on the ward within 15 minutes so we could take bloods and start the antibiotics in a more controlled environment. I have never seen A and E in such a state of carnage. It was frightening when William was so ill.

It was 2 am before I got to bed in the hospital after we being confident William was picking up enough to leave him to get the things we needed for the night , have something to eat (I had just made a yummy chilli for tea when all this happened). Paul had a stint at the hospital this afternoon and I came home to sort out the girls, see Mum and Dad and to tidy and clean the house. The local hospital is close enough to come home for a couple of hours when William has gone to sleep. We usually get into such a mess after some frantic packing and coming and going of stuff each day. It makes such a difference to come home and relax in a clean and tidy house. I now have some yummy smelling apple, spice and walnut muffins in the oven. A huge contrast to yesterday evening!

William has picked up really well today. The tests have confirmed that he does have a line infection and he is responding well to the antibiotics. His medical examinations have revealed a heart murmur though. This is a new thing and we are not sure at the moment if it is anything to worry about or not. He is due to be seeing his local consultant on Thursday and so, while he is in hospital, they will do an ECG and heart echo. One concern is that he had a nasty fungal/yeast infection in his line in August. There is always a risk of spores getting into the heart valves. There is also a chance that line infections have caused inflammation in the heart valves. It may be a feature of his syndrome or a neurological issue and, of course, it could just be an innocent murmur. We will soon find out and, in the meantime, we have learned not to worry too much about things to do with Wills until we know we have to.

Hectic, as ever

I really promise I will keep this blog updated more from now on. Things have been so hectic since we came home from hospital. We have been trying to sort out William's statement of educational need, something that never seems to work out as easily as it should and we are still having meetings and discussions about. In the meantime, I have been settling William into playschool for 3 afternoons and one morning a week. William can not be left without either myself, Paul or a qualified nurse. Thankfully, they have agreed to provide a nurse for William at playschool, despite the fact his statement has yet to be finalised. Luckily for us, one of William's lovely respite nurses works around a school day so this is perfect for her. William adores her and they both seem to have a lot of fun together.

On top of all this, Hope has been setting into her secondary school. She has grown up so much, perhaps too much. It is lovely that she is making lots of friends and likes to incorporate a social life into walking home. We have had to spend the last week or so reigning her in a bit and reminding her that she is still on 11 years old and her whole life can not just yet be organised through her mobile phone. Hope hs been chosen as a school council rep. She is also keen to play basketball and football. These clubs take place at 7.30am so we are set for an early start tomorrow.

We are all getting a bit sporty these days. Paul is currently searching for a gym to join as it has been ordered by the physio as part of his rehab. Hope and Ellie are both about to start a Christian Football Academy, Kick London, that is being organised through church. I am to be a coach at the academy and am in the middle of my FA level 1 coaching certificate. This has taken place on 3 evenings and all day last Sunday. This Sunday is assessment day when I have to deliver a 15 minute session, based on a game given to me last week. My game is a bit of a tricky one involving throws, heads and catches in a 5 v 5 game.

It is all very hectic and, as usual, the appointment letters for William are piling up. He is due to go to the muscular dystrophy clinic at Hammersmith Hospital at the beginnig of November. It now seems that he does have some kind of progressive neuropathy and this is the best team to monitor and help him with this. With his motor deterioration and time spent attached to his TPN in mind, he will soon be given an electric wheelchair. We tried one out on Monday. It needs to be adapted to make it a lot smaller and enable him to reach the controls. This will take a couple of months. This is fine though as William needs a few more lessons with the occupational therapist to learn how to use a joystick to control a chair. Tomorrow, we are being visited to see exactly what we need to do to our house to make it accessible for William. It looks like a major rebuild will be required.

It is all these practical things that dominate our lives so much. I have been juggling two part-time jobs, working from home, with it all. I have just come to the decision that it is all a bit much. One of the jobs - being the director of "The Brompton Fountain" works very well but the other is very stressful. I won't go into too much detail in a public blog. One thing we learn time and time again on our journey with William is that life is just too short. I am also fed up with playing catch up the whole time. Now I can put all my work energy into building The Brompton Fountain. Funds will be a little tight but I am a strong believer that things will work out if it is the right thing to do.

Another Year, another challenge.

It has been a while since I updated this blog with any regularity. So much has been happening and we have been so busy, in and out of hospital, possible diagnoses, starting schools, statement of educational special needs... I promise, I will catch up on all the news over the next few days, and show you some lovely pictures. For today, I want to tell you all about Sunday.

During July 2006, Paul was run over and spent a month in hospital with a horrific leg injury (He has only just been able to put two shoes on again in the last few weeks and finally has a leg that is not held up with mechano see http://www.blegspot.blogspot.com). William's physical disabilities were becoming apparent and, I have to confess, I was struggling. A good friend of mine, Emily, was struggling even more. She was in the end stages of cystic fibrosis and her lungs were barely managing to stay inflated for a few weeks at a time while she waited for a call to say her turn had come to get new ones. One day, I received an email inviting me to join "Emily's Angels" in doing the Hydro Active. Emily was planning to be pushed around in her wheelchair, attached to the oxygen that was keeping her alive 24 hours a day. Not only that, she was going to turn up the oxygen and walk the last 500 metres. To you or I, that would be an ultra! I took up the challenge and decided to raise money for CHASE, who were keeping me sane at the time with support at the hospice and in the community whilst I juggled looking after William and visiting Paul (the girls were at Grandma and Grandads). I planned to walk with Emily and her angels but felt shamed by her 500 metre walk and William's courage as he totters about, falls and gets back up again. If they can do that, I can run 5K, I thought. So, I dusted off a pair of trainers that had not seen a pair of feet for over 10 years and went out for a run in the park. Meanwhile, Emily was in and out of intensive care with her ever deflating lungs and many of us worried that she may not make it to walk those 500 metres. Emily the biggest fighter I know and escaped from hospital to walk over the finish line. I managed to jog around and finished in 32 minutes.

Yesterday, Emily and I were back at the Hydro Active Women's Challenge. A year is a long time and in the last 12 months Emily has had a double lung transplant. I have spent the last 12 months getting ever more addicted to running and more and more serious about my training. So, this year there were 2 major changes. Emily planned to walk the whole 5K, while holding a banner showing herself last year, in her wheelchair on oxygen, and this year, walking and bouncing along. I planned to run a PB and dedicate it to her and William, the inspirations that got me running. It was exciting as well because Hope and Ellie joined in with the Angels. It was also the first time I had put a team together to run for the charity I direct, The Brompton Fountain.

We got t Hyde Park at 9 to get ready to say hello to the team and prepare for a team photo. Granny came to look after William and I left Hope and Ellie with Emily and the other angels before taking my place at the front of the mass start with other club runner capable of a sub 25 minute 5K. We were just behind the elite runners and celebs so got to watch them all be introduced to the crowd. It was really exciting being at the front of such a big event and to set off on the clock. We were away within seconds of the elite runners and could even see the time car becoming more and more distant on the first stretch. I had a clear run and, as usual, set off a bit too fast. However, I managed to keep pretty close to my starting pace and finished the first mile in under 7 minutes I could have kept pushing at that pace if my legs were not a little tired, untapered in the peak of marathon training. I didn't drop too much though and kept my pace below 7:30 most of the time. Just before the final turn, I was counted in by an official letting me know that I was in the top 100 and would receive an official time. As I approached the finish, I could hear the announcer tell the spectators that the runners finishing now are all strong club runners, capable of keeping the pace throughout the course. I finished at 22:18 on my Garmin (which also told me I had run a few metres long). On crossing the line, my number was taken and I was given a bottle of Hydro Active. What a difference from last year when I staggered across the line, red and puffed out in a tutu! It was a strange experience. The area where the goody bags were given out was almost deserted and my photo was taken by 2 official photographers and 2 more asked if I wanted one. Later, when I finished again with Hope and Ellie, we had to queue to get a picture together. I walked past Meseret Defar and Jo Pavey wondering around, waiting for interviews before heading back to find Hope and Ellie. My official time was 22:27 and I came 81st out of more than 15000, many of whom were walking. I am listed, beneath the elite athletes, in the race report on the Hydro Active website (http://www.womenschallenge.co.uk).

I am not known for my great sense of direction so I wandered back to the start and jogged along to find the back of the race. Eventually, I caught sight of Emily's banner and found the girls. We walked the rest of the course together and made a pact that, if the girls can jog it next year, I will do it with them. I did enjoy being up at the front though and would love to make up a bit more ground on the elite runners in front. I WILL go sub 20 in a 5K on tapered legs sometime in the not too distant future. Emily did really well and even managed to run the last 300 metres - amazing! Ellie ran bits of the last mile and ran over the line. (As I walked over the line again, I hoped people would notice that I already had a medal on and was not finishing for the first time).

After the race, we met up with William and Granny again and had a picnic with Emily and some of the Angels. Paul turned up after singing in Church and, by now, it was getting a little cold so we headed off to the Science Museum to see William's beloved engines before setting off for home to catch the highlights. He also had great fun playing with Hope and Ellie with the hands on games in the Launch Pad.

It was a great day that marked for me how far I have come with running this year and that I can feel that there is more to come. Emily was as inspirational as she always is. As a Mum to a child like William, Emily's Mum inspires me at least as much. Her strength and amazing personality has, doubtlessly, contributed so much to what Emily is today and if I can just be part of the Mum she is I will have succeeded so it was great to see her again too. Hope and Ellie had their first taste of a big running event and, I hope, got inspired to work towards running in future. Above all, we had a lovely family day out. Next year, I will develop the charity participation to include a family picnic. It won't be the Hydro Active next year as it becomes the Addidas Women's Challenge. It will remain a special day out for me though. I will always have a soft spot for the race that got me running

Hope's first day

We are finally home from hospital and there is lots to post to catch up. This is a quickie though as I am about to go to bed. Hope started secondary school today. What a milestone day! She had a great day. I marked the day by having a "feel younger and fresher" hair cut. Much needed, not least after the weeks in hospital. The girls also had hair cuts and Hope had her first grown up layer cut. You can see that on the more relaxed, after school picture..

We are still up at the hospital every morning for IV drugs for yeast. William lost his line to a yeast infection that made him very poorly and we have to treat to protect the new one. I will post a longer entry to tell the whole story later in the week, after I have got all urgent work done!!

Life is a rollercoaster...

life is a rollercoaster...
...You've just got to jump on, enjoy the ride and cope your best during the drops.

We started trying William on a teeny tiny bit of diarolyte on sunday. By Monday, it was clear he wasn't tolerating it and tests showed he was passing all the glucose in his stools. There is a new consultant on the team and she relayed this as good news, explaining that he could have a rare inability to absorb glucose and a special feed could get him off TPN. I was so pleased but Paul was a lot more cautious as this would surely have been realised long before now. The dioralyte was changed to water to flush the remains away. On Tuesday, Wills was poorly again with another infection and has still not picked up. This always seems to happen when we try any feed. The gut challenge has been abandoned for now. He will remain on this tiny trickle of water - 12 mls per hour for 16 hours and have a new set of scopes and biopsies next month, followed by a team meeting to see if they can work out a new plan. The same doctor who was so full of hope on Monday had to explain that he will be on TPN for a long time, perhaps forever, and the priority now is sorting all these line infections. It is so gutting to be so full of hope one minute and back down again. The water is a teeny tiny baby step though.

I am so exhausted now, physically through lack of sleep and mentally too. I can't wait to get this infection sorted and get home to normalise. I am keeping up with training but it is a bit treacly at the moment. it is funny how pockets of inspiration appear right where you need them. I came back from a run yesterday and there was a cleaner at work in the bed space next door. She asked if I was an athlete, to which I said I was trying to be a club runner. It turned out that she is a girl called Fatmata Bangura, the women's sprinting champion of Sierra Leone and captain of the women's athletic team. She is a seriois medal hope for 100 and 200 metres in China. She got injured at Crystal Palace so her season is over and her attention switched to next year. She is over here for some training and has to work long hours as her government funds training but not living costs. Unbelievable dedication. I hope to see it all paying off with her up on that podium next year.

Home Sweet Home!

Hooray, we are home. For a while at least anyway as we are scheduled to go back in for a while in August but we get to enjoy a week or so at home first. Wills still has a bit of infection left so is on IV antibiotics and is still not quite himself. We are having to keep a very close eye and not have an oxygen tank set at 15 litres with a non re-breather mask in the corner of the living room in case we ever get a day like that again.

The last couple of days have been crazily busy was we have caught up with our lives as they were before last Wednesday. For Wills, this has meant visits from his physio, bringing the splints we were about to go and collect when he decided, rather dramatically, that he would rather not go to physio on that day and a visit from his OT and a rep to look at a more supportive chair for his ever slumping posture. She also came with the news that we are getting a temporary powered chair, while we wait for them to find the perfect Willsmobile. This may arrive next week so I am just taking a break from a major clutter clear to make some room for him to drive around the house, destroying the paintwork.

Our house is not at all designed for this so the OT is making a case to the next panel for us to have major house alterations. All the doors need to be widened, we need a lift from the dining room into the room above, currently the girls' room that will then become William's room, the bathroom will turn into a wet room (very sad about this as I love my baths!) and we will have an attic room built for the girls. I will make sure there is some kind of partition as they are a nightmare sharing a room at the moment. All that is going to be pretty major. We will get a grant towards the majority of the cost. It will be a about a year before the process reaches the stage of being ready to begin work. Wills will then be around 4 so he will be so happy to have complete independence around the house :-) We are still not completely sure of his disease prognosis but most of those who work with him expect him to be a full time wheelchair user in the foreseeable future. He doesn't do much walking around at the moment, preferring to lie on the floor and play and crawl at times. The trouble is, when he is ill, he deteriorates generally at a quicker rate and never quite picks up.

Home tomorrow!

Wills is coming home tomorrow :-) He will need a further 2 weeks on IV antibiotics but we can do that at home. He is going into Chelsea and Westminster on August 6th for further tests, including his barium follow through and further nerve conduction studies and tests on his mucles. We will also see if we can get his intestines to just tolerate a tiny, tiny amount of something to help stop bacterial overgrowth and more scary line infections. He may well have his illeostomy during this time as well so we could be in for the month (or longer...). Hope and Ellie will be with their Dad and then onto Grandma (where would we be without Grandma!) so I won't have to split myself in two as much as usual. I asked out nurse specialist if we could time his admission then, not least because the girls and I are off to see luverly Lee in Joseph on Thursday next week and Ellie has her birthday the following Sunday and Wills will be 3 on Monday. This will be the first birthday at home with his family (she say's keeping everything crossed!). We plan to make the best of the next 2 weeks as it could well be the full extent of our summer holidays together so will will go out and do loads of exciting things, working it all out around the IV antibiotics. I am not ashamed to wash my hands, roll my sleeves up and do his IVs in a cafe somewhere. You do get some odd looks though!

So, we are trying to settle down and get back into normality. Things feel a bit strange though, I think because we went through a lot this time and very nearly lost our Wills. It is very hard to get back to normal after things like this. It kind of feels a bit surreal at the moment. You have to balance living and enjoying life but being vigilant at the same time. We and our community nursing team are sorting out high flow, portable oxygen tanks ready with re-breathing masks to be at the ready at home and in William's playschool in case of similar emergencies.

Very sadly, I learned today that another of the "gastro kids" on TPN at Chelsea died very suddenly at the end of last week. He was the same age as Wills and may well have died in very similar circumstances that William was in last Wednesday, they have yet to work out what was the cause. I feel very sad that, while we are heaving a big sigh of relief and trying to put the images of Wills so ill out of our minds and get on with life, another family are going through a time that is the worst of our fears. We know that, one day, we may well have a different outcome when William gets so ill. Life for these children really is so fragile. My thoughts and prayers go out to this family at this time.

Quick update while home to do the washing

Wills is still in hospital, fighting a very nasty infection. He is having good days and bad days, still having very high temperatures and is washed out. The infection was caused by bugs in his intestines getting into his blood and causing septacemia, and, of course, settling in his hickman line to cause a line infection. He has now had two such infections that have been classed as "life threatening" as well as numerous others that were pretty serious as well. There is, naturally, some cause for concern about this so we are off to our "Fulham Pad" at Chelsea and Westminser Hospital on Tuesday.

Wills will have a barium follow through study to make sure there are no obstructions and blockages in his gut, and to see just how slow things are at the moment. After that, the team will discuss if an illeostomy will help drain some of the fluid that is growing these bugs (bringing a bit of his bowel out through the skin to drain). They also plan to see if they can get Wills to tolerate a tiny bit of feed into his intestines. At the moment, he is unable to tolerate anything. If we can get him to tolerate a little bit it will help his intestines to normalise a bit and prevent this build up of gut bacteria that is leaking into his blood. We have no idea if he will cope with this. We will just have to see. It will not be enough to feed him and he will still be dependent on TPN for the time being.

If this does not work, there is a chance he will be assessed at Birmingham for intestinal transplant. One near fatal infection could be a one off but two puts him into the bracked of "TPN complications" which coud make him eligable to go on the list. However, we still have th complication that it seems Wills has a degenerative neurological condition and that would be contraindicative of a transplant. There are still so many complicated questions and issues to sort. The infection seems to have bought the issue of transplants to a head again but, in the same conversation, William's local consultant explained how it seems Wills has problems with the junctions between his muscles and nervous system. The impulses are not being sent to the muscles properly and this is likely to be degenerative as the nerves then die. His tests results and clinical picture seems to support this at the moment. So, in the next few weeks or so we need to establish whether his intestinesfunction at all, whether an illeostomy will help, whether he needs to be assessed for transplant now or if he can still safely get by on TPN for a while before this becomes urgent and, of course, whether his neurological condition is a degenerative one. It is strange, we muddle on along and then, all of a sudden things come to a head for a while, then we muddle along again, often just as we were before. We are just about to get into the coming to a head stage for a while. It is a shame, we have been in this stage every single Summer since Wills was born. While everyone else seems to be planning their holidays, our family is once again separated with Wills and me in hospital.

A very scary day

Wills got another line infection yesterday and ended up with septic shock. The speed in which Wills went down yesterday was terrifying. He was playing with his tank engines very happily. He went over to his Dad for a cuddle but was still there for a couple of minutes so we began to worry if he was OK. I went to get the thermometer and, by the time I came back in the room, Wills was blue, semi conscious and shaking violently and Paul was on his mobile for an ambulance. This in under 5 minutes from playing! I ran for the oxygen and blasted it up to the top. Thankfully, the ambulance was there very quickly. They took one look at him and got him straight in and were off with full blues and twos, radioing into the hospital that it was a life threatening situation. The docs were waiting in the ambulance bay and Wills was given resucitation with high flow oygen, drugs, antibiotics and fluids. They were debating calling a CATS team to get him into a paeds intensive care when he began to respond. Good old Wills, a real fighter! It is so so frightening to think that his life can be snuffed out so suddenly. This is not the first time this has happened and he has had a full respiratory arrest. He was on the way to another one this time. We know the signs though and reacted quickly with lots of oxygen. We could have been on a bus, in a park, in a shop - anywhere where we don't have everything to hand though and I don't know what could happen then. Worse still is the thought that this could happen overnight and we may not see. For this reason, Wills is now monitored overnight as his pulse rate speeds towards 200 when this happens and the machine will alarm and let us know. All this because the line that the TPN goes into to feed him is a huge infection risk. Of course, the TPN then feeds the bugs as well as feeding Wills, resulting in instant septacemia. Yesterday, he experienced septic shock. This is a very dangerous position to be in. It is very frightening that we could loose Wills from these infections very suddenly at any time. It is something we try not to think about but have to remain vigilent for situations like yesterday.

There were no beds for Wills in our local or in Chelsea and Westminster so we ended up in St Helliers. They were so lovely there but it is not familiar and it felt pretty lonely when Paul left as I was not at home, or in a place where we are all known.

I am now trying to catch up with everything else that is happening and make sure Hope and Ellie are sorted for the weekend and I have the things I need, as well as the little things and treats I use to make myself feel better in hospital with Wills. They have identified the bug and he should be on the right IV antibiotics tomorrow. Once he has settled, we will go home and continue the treatment ourselves.

The Island Of Sodor

Wills had a fantastic day today, well we all did. As followers of this blog will know, William absolutely adores Thomas the Tank Engine. It is what keeps him going, it really is. When he is unwell, he watches the DVDs and reads his books and pushing engines around on a table calms him down better than anything. Of course, he plays with his Thomas things when he is feeling well too but when he is ill, it is really all he wants (apart from blankie of course!)

So, a day out at The Bluebell Railway Thomas Day Out is William's dream come true! We told him he we were taking him to The Island Of Sodor and he really believes that he has been there today. We saw Thomas and Percy, Diesel and the Troublesome Trucks. Stepney is resident at The Bluebell Railway as, of course, he is the Bluebell Engine so we saw him and had a ride on a train he was pulling. William is now a proud member of the Stepney Club so we will be having a good few trips to the Bluebell Railway for club specials in the years to come. We said hello to Thomas and climbed in with his driver to blow his whistle. We watched Thomas and Percy arrive at the station and then watched Percy shunt the naughty Troublesome Trucks around. We also saw another resident engine, Fenchurch. He is not a Thomas engine but William adopted him as one (he had a face afterall) and was thrilled to ride on carriages he was pulling. Other engines had faces on to look like characters so we also saw Henry, Donald and Murdoch. The Fat Controller was also around to say hello to the children (although he seemed to have lost a bit of weight!) All in all, it was a fantastic day, despite the rain, and made for very excited children, especially Wills.

We were wondering if we would make it at all today as it has been a bit of an eventful week for Wills. He has started draining really nasty liquid from his jejenal tube. Most of it is faecel matter that should be draining elsewhere but is not doing so very effectively at the moment. This may be due to adhesions or partial obstruction caused by infections or may just be because his intestines have slowed down so much. Either way, it is now time for Wills to have surgery to sort things out and make him more comfortable. This is still a tricky balance and William's surgeon is still hopeful he may be a suitable candidate for a transplant at some time, unless it is confirmed that his underlying condition is progressive. In any case, things change and the contraindications for transplant may alter as more research is done and more successful transplants carried out. So, even if he is ruled out now, he may be included again in the future. This is tricky because surgery that could be done to make him more comfortable now may hinder the success of fa uture transplant. It is very hard to decide what is best for him when we really are working in the dark with no clear diagnosis. There are a couple of options for surgery now. One is a staight forward illeostomy, the least risky but may not be the most effective for Wills. Another is to make a higher stoma. This would drain very well but would mean there would be very little remaining small bowel, making him "short gut" and so, writing off any function that may remain in his small bowel. William will have some further x-rays and barium studies over the next few weeks before a discussion with his consultant and surgeon to decide what to do. In the meantime, we are draining all the nasty stuff through two drains, from his jejenum and stomach.

William has been struggling with the heat so is back on 20 hours TPN with only 4 hours free from a 2 ft long drip tube. This is very hard for him as he wants to be active like other children, even if it is in his own wobbly way! We are hoping he will sooon get a powered wheelchair to give him more independence with he is on his line, and when he is too tired and wobbly to walk.

We had just got back from the day at the hospital on Friday when Wills spiked a high temperature, making the trip to see Thomas look very unlikely. Luckily, it seems that was due to dehydration (before going back to 20 hours on the TPN) and, perhaps, a normal virus.

Hope had her birthday last week and enjoyed a very girly sleep over. I really didn't have much to do with it - apart fomr providing the tea, Mum really needs to keep at arms length from her and her friends girly sleep over behaviour now! It is sad really, Hope and Ellie have missed a lot of Mummy time since William was born. Hope has had most of her parties, and was 8 when William was born so growing out of big parties. Wills was born the day after Ellie'e 5th birthday so she missed out that year. We have managed to do something for her each year but it has always been around what has been going on and, usually, with Wills and I in hospital. She has had a couple of birthdays hauling her presents up to open on the ward. This year, she is having an early party next weekend so her friends can all come before the term ends and to make sure she has one before Wills goes in for any surgery that may get scheduled during the summer. We looked at a party catelogue and she really has grown out of the fairy parties etc. That is a bit sad really as I fancied one more pink and pretty party. We are going for Pirates of the Carribean and are really going to town on it with treasure hunts and palm trees and a lovely Elizabeth wedding dress for Ellie to wear. Next year, she wants a sleep over too so it looks like this will be the swan song for home made childrens birthday parties for the girls. Once again, we are keeping everything crossed that Wills stays well and home for another week.