It is a line infection!

Well, we have it confirmed that William has another line infection. These things come on SO suddenly and make him so poorly. We have come very close to loosing him with one so we are pretty nervous about them. He is now on the right IV antibiotics - 3 of them. We will find out in the next couple of days if it will be necessary for him to go into theatre to remove the line and put a new one in. I so hope this isn't the end of a spell of being poorly - it is getting so near to Christmas. He intestines are also playing up with loads of wind, aspirate and explosive nappies. We think this is due to some syrupy sedation he was given at GOSH last week that is sitting around in his intestines and fermenting as well as causing an infection that has now got into the line. Well, have come home to tea, thanks to Granny (Paul's Mum) and am now back to the hospital.

Poorly again!

We are back in hospital again as William woke up from an afternoon sleep with a temperature of 39 degrees, way over our 38 degree cut off for a potential line infection. We had 2 rapid response cars and an ambulance this time - very dramatic. We now wait in hospital for 48 hours to see if it is a line infection or not. If it is, we will be on 2 weeks, if not, we can go home after 48 hours. He does have a cold so I am hoping it is just that. Have just popped home to pack so must get on.

Great Ormond Steet

We finally made it to Great Ormond Street this week for the test that everyone has been waiting for - the duodenal manometry. This is a measure of the peristalsis, or muscular movement through the gut, in response to food. The visit was pretty eventful from the start. I was running late and harassed and stressed as ever in these situations, not helped at all by the fact that Paul still has his leg in a cast and I was travelling on packed commuter trains with William in his "Charlie Chair" (or rather, "Darlie dair") with everything we needed for the next 3 days on my back in a huge mountaineering, and very heavy, rucksack. I eventually decided that I was going to be late and it really wasn't worth rushing to be there for 10 when it was highly unlikely anything would happen before lunch time so rang the ward and apologised and began relaxing a bit. When we arrived near the hospital we saw police everywhere. I eventually asked what the fuss was about and was told that Charles and Camilla were in. Well, a big hospital, I thought and spent the next 20 minutes totally lost in it and wondering if I'd bump into them. I finally found our ward and noticed that there were more and more people in suits the closer I got. When we walked in, we found that our ward was the very ward the Charles and Camilla were in. We got to see them briefly but, had I not have been so late, we would have got to meet them properly. They were there to open the new wing the ward is in. Very exciting. I know this visit has been a long time coming but we weren't quite expecting a royal welcoming party! It is a shame we didn't get to have photos for William's scrap book. I don't think the photo would have knocked the photo with John Terry from pride of place!

William had 2 days of tests and spent 2 nights in. He had an endoscopy and colonoscopy (tubes down and up) to see if there is any inflammation that could be treated. He also had biopsies taken to look at the histology of the gut. The scopes looked OK, as they have done in the past. The following day was the manometry test we have all been waiting for for so long. The hope was that this would show us something that could guide future treatment, such as surgery to remove the worst effected part of the gut. At the same time, he had an EGG, measuring the activity of the stomach via electrodes, similar to an EEG but on the tummy not the brain. The photo shows the complex computers and machinery all around him for these two tests, and Thomas the Tank Engine, the most essential bit of kit of all, keeping him nice and still enough for the measurements to show anything other than wriggling. He was sedated throughout but, having been sedated the previous day, he wasn't about to loose a day to sleep again! The manometry is very clever. A catheter is inserted in the gut with a set of holes all the way down. At regular intervals, water is pushed through the holes and the gut reaction to the water measured via the pressure it exerts on the catheter. The gut should squeeze in repsonse in a rythmic fasion - peristalsis. They decided only to use one measurement for William as the catheter all the way down would have interfered with his own tubes and any information at all would be a useful start. They used his own tube that is inserted just below the stomach to measure the reaction of the top end of the gut to the water. Once he was sedated and lying flat watching Thomas it was clear that his gut didn't really react at all. He seems not to be able to squeeze his food and drink down the gut at all. We are still waiting a deep analysis and formal report but the initial view of the consultant from eye balling the initial data is that William's nervous system is failing to tell the gut to react to the water. This fits with his current neurological picture with the ataxic walk and deformed feet and, as all the gut has also got worse as he has got older (he did actually feed for a while as a baby) it sadly does suggest a degree of degeneration in his condition. We had hoped this test would reveal a solution but it seems that, unless something can be done to fix his neurology, there isn't really anything that could be done to make his guts work again. A transplant would certainly not help as the problem appears to be his nervous system, not the intestines themselves. We still have to wait for the more detailed analysis and, you never know, there may be a glimmer of hope in there, but it looks unlikely he will be able to come off the TPN at the moment. Luckily, TPN is keeping him really well at the moment but it does keep him tied to a drip 18 hours a day and means he lives with the constant threat of septacemia from line infections and the knowledge that this will, eventually, destroy his liver. We also don't yet know what the underlying neurological picture is and what else this will do to him over the coming years. All in all, we are a little closer to understanding what is going on but no closer to a cure or solution. We are seeing our usual gastroenterologist on Friday next week so don't have too long to wait to learn what comes next. We had predicted there would be another long hospitalisation after this one but it looks now as if there wouldn't be much to gain from that and it is more a case of getting on with it, enjoying every moment and hoping that something can one day be done. In the meantime, we have great support and therapy to make sure William gets the best out of life and his smiles and laughters show that he certainly does! The clown doctors certainly got a smile and a laugh from him (eventually, once he stopped being overwhelmed).

A lovely day

Today was a lovely day and William had the time of his life. A few days ago, I was walking form playgroup with William and a guy on a bike stopped us and told me about a cycling club at our local running track where there were loads of adapted bikes so everyone can join in. It is called "cycling for all" and I have never seen so many different types of bike. We went down this morning to investigate and the children had a great time (as did Mum). It was lovely to do something active we could all join in with. William tried a tricycle where he could wind the handle bars around with his hands to power the wheels, rather than use pedals. He really got the idea but, unfortunately, could not reach the handle bars and sit safely at the same time (I'm sure steering would have been interesting too!). So, instead, we tried an adapted side by side bike and a really cool, but terrifying to begin with, T-bike where William sat in a chair in front. The T bike has 2 wheels at the front and one at the back, like a back to front tricycle. It was really hard to steer until I got used to it but we were quickly away. William beamed and beamed and instructed "come on" and "ready steady go" whenever we paused. It was pretty hard work, especially the "side by side" as it was a recumbent and much tougher on the thighs than a usual type of bike.

The fun continued in the afternoon with a "tuneful teatime" tea dance in our new church hall. We all went, even Paul, and had a lovely afternoon tea and danced to the orchestra. Well, William, Ellie and I did. Paul was not quite up to dancing yet and Hope was busy having chatting by the water feature in the courtyard. It is meant to be a remembrance garden but the children are adopting it for their "heart to hearts".

All in all, a fun day with much smiling and laughter.

Grandma and Grandad arrive tomorrow, ready to look after Hope and Ellie while William and I go into Great Ormond Street for 2 nights on Tuesday. Let's hope they don't cancel again!

Planning for the future

Another busy week, although the main reason why I haven't blogged for so long is that I have been totally knocked out by a nasy fluey cold. Since my last entry we have visited the school it it likely William will go to and begun the lengthy process of getting a "statement of educational needs" for him (getting him "statemented" in other words). We have also been to see the neurologist and seen our occupational therapist, who fitted William with the rather fetching support belt shown in the picture. William has found time to play as well. He has a fantastic Link worker who spends 6 hours a week with him so I can get on with doing some work. He loves to play with he books and lego and sticking and paining... with her while I get on.

The visit to St Giles was really good. It is a lovely school, full of happy faces and photos of disabled children achieving things in sports, outdoor pursuits, theatre and art, all sorts of things, on walls all over the school. It was lovely for William to see so many other chldren with wheelchairs (or "Charlie Chairs") and tubes just like his. This has got to be better than being the only child in a school. There is so much going on for the children there and they have a rule that if an activity is offered to them, it must be suitable for all the children or noone goes. The children have plenty of opportunity to reach their goals too and one girl we met is doing 8 GCSEs as well as learning to play the drums. The children get all their physio therapy, occupational therapy, hydro therapy, speech and language therapy, wheelchair, seating support, peidro boots and brace and splint assessment and fittings, medical reviews etc all at school so there is minimal disruption from lessons to attend hospital clinics. It was a really happy and positive school and we felt really lucky we are in the Borough that has such a school. Ofsted have rated it as a Beacon School of its type. As we are in the Borough, if William's statement says this is the right school for him he will automatically be accepted. We are hoping he will have a link into a mainstream school for an afternoon a week so he can also integrate. I would accompany with him for this to avoid the problems of needing a nurse on site. Of course, St Giles has an on site nurse who will be able to deal with everything involved in caring for his line and dealing with the TPN.

The day after visiting the school, we saw the neurologist who does joint clinics at our local hospital. This whole problem with the double referral is still going on and we have another neurology appointment at Chelsea and Westminster next month. The neurologist we saw agreed that William's walking and posture are a concern but has not had the benefit of seeing him over time to watch how it has deteriorated. She said it could be a syndrome or a degenerative condition but could also be a feature of his prematurity - although not one she saw as something he would grow out of, more a damage that was done as a result, like a form of cerebral palsey. She did suggest things could be helped to a degree with surgery. The first thing she wants to do is to get a better MRI brain scan under general anaesthetic and do a lumber puncture to look at the chemistry of his spinal fluid at the same time. She hopes all this will be done before Christmas. With the tests at Great Ormond Street next week, poor William is going to have an eventful end to the year. I also expect next year will see some more hospitalisations while we try things as a result of the tests. This will very likely involve some surgery as there are a lot of procedures that have been on the cards for sometime and awaiting the Great Ormond Street tests, such as the illeostomy and laparotomy and, of course, the small bowel transplant assessment at Birmingham that still hangs around as a big possibility. It is always confusing to see new doctors though as we seem to be moving in on a diagnosis but then someone else has new ideas. This doctor seemed more inclined to look on his problems as potentially a couple separate issues rather than look for some underpinning genetic condition. She did agree he has some interesting features like "sparse hair" and wants to know what the geneticist is thinking at the moment. I think things will be a lot clearer by the end of next year but I always find it disapointing when a new doctor doesn't immediately say "this is a clear example of X!"

Today, we were visited by William's occupational therapist. She fitted his lumber support but also wanted to talk about our house and the adaptations she feels are necessary over the next couple of years. It seems a bit premature to think about this but the grant applications take at least a year and she thinks it unreasonable for us to be carrying him up and down the stairs beyond the age of about 5. By the time we have thought about the solutions, been through the grant applications and completed the building he will be about that age so things have to be thought about so early. The OT thinks we need a wheelchair lift into what is currently the girl's room, in which case we would also need a loft conversion for them. We also need a walk in shower for William to use in a wheelchair if necessary. This is gutting as I love my bath and we don't have space for both. Another option would be to extend out at the back and build William a downstairs bedroom and bathroom and keep upstairs as it is. Of course, this is all very expensive and probably way beyond the grant. The last resort would be for me and Paul to get a sofa bed downstairs and put the girls in our room if William has to go in there. So much to think about, you just don't dream of all the implications when problems emerge in a child so young. The grants are generous though and we are so lucky these things exist. I must say, I am very pleasantly surprised at how much support there is out there for families in our situations. However, hospices still have no Government funding. This leads me onto my next fundraising venture for CHASE but this has been a long enough post already so that will follow another day...

A Happy Boy at Playgroup

Hooray!! William managed a whole afternoon at playgroup today without the need to hold onto my finger! I stayed in there all afternoon by himself and seemed to have a lovely time and even managed to cover himself in orange paint. Unfortunately, I don't have a lovely smiley picture to accompany this as I was in the parents room with a cup of tea!

After playgroup we had Ellie's parents evening. She is doing really well and is a "joy to teach". We have Hope's tomorrow. Tommorow will also find us visiting St Giles, the school it is likely William will go to for nursery in September. We will go with our Portage worker who will then begin the long process of obtaining William's statement of special educational needs. St Giles is a school for children with complex medical conditions and/or physical disabilities. There, he would get his education, together with physio, occupational therapy, speech and language therapy, hydrotherapy and nursing care.

On Friday, we are seeing the neurologist about William's ataxia and involuntary spasms and movements. That will be really interesting.

A quick update

A quick update, after a phone call from Mum saying she has missed the regularity of posts lately! I really must get back into the habit of doing this more regularly as there is always so much to say when I leave it to the end of the week.
It has been another busy week with lots of coming and going for William. This photo is with one of the nursery nurses that come in to play with William and other children who receive pallitative care in the home. He loves this one-to-one attention and we also get it from our fantastic volunteer who acts as a link worker. This is provided, following assessment, from the children with disabilities team from social services and the workers are all volunteers, spending hours in children's homes so parents can get on with other things. For me, this means I can actually gets some work done. William's occupational therapist fitted him for a lumber belt to try and support his posture more. He needs a custom made one to accomodate his tubes, which is pretty cool really as, rather than the standard white corset, his will be made out of blue and orange neoprene (wet suit material). She is also going to talk to the physio about his walking and involuntary spasms, to see if we can start some exercises and hydrotherapy. He had a really bad day earlier in the week when he was so tired be could barely walk at all. It may be that we start looking into a walking frame for such days. We have been encouraged to start using the wheelchair in the house when he has bad days for now (not that we have an awful lot of room for that!). As always with Wills, so much is watch and wait and see how it goes. It will be really interesting to see what the neurologist says next week.



This week, William has had visits from his link worker (twice), nursery nurse, portage worker, occupational therapist, community nurse, respite nurse and CHASE hospice community nurse! We are SO lucky to have so much support. However, the pallitative team and CHASE receive no government funding and rely soley in charitable grants and fundraising. Wills and I are off to the hospice today so I can take part in the Losely Park 10K run tomorrow to raise money for CHASE. It is a lovely sunny and crisp November weekend and it will be an off road run so I am really looking forward to it. The pictures our club took of the cross country last week have now been put on the web. I look SO much better on the second lap so will make sure that I warm up properly tomorrow. Will post how it all went tomorrow but for now, I have less than an hour to finish packing, get WIlliam flushed off his TPN drip and get out of the house!