Catch-up time again

It has been a hectic week, hence the lack of blogging. I have also put very few pictures up lately so here are a few taken over the last few weeks. I still have not had a chance to get my own laptop fixed and am using my work one. I couldn't rotate pictures today (no software on this) so these are just a selection. They were taken at the hospice a few weeks ago and the cold, damp ones are William and Ellie while we were marshalling and watching Hope at the Race for Life. Hope's picture is not up as it needs rotating. Ellie and Wills had a great time playing with cow parsley (making me sneeze). Some of the hospice pictures show one of William's recent discoveries, preparing his meals on a play kitchen and then sitting down with us to "eat". He loves to take part, in the same way a boy likes a steering wheel at the back of a car. If you give him real food to play with, he recoils away from it but really loves playing with pretend food. It is a nice way for him to join in at family meal times.

Well, the last blog followed our flood and William's tube coming out. William's new tube was put in today. We did go in for it on Friday but an emergency took up the staff and the room so we went home without it. It was a very unpleasant and frightening experience for Wills. He had to lie on a table in a room a bit like an operating theatre with a live feed X-ray machine just inches above his tummy. There were about 6 people with gowns and lead aprons around him, passing equipment etc. The procedure itself took about an hour and all the time he had to lie really still while guide wires and tubes were passed through his stomach into the opening of the small bowel below. Grant, the gastro nurse specialist, and I tried to keep him happy with Thomas stories and music on Grant's phone. When it all got a bit much, I promised him a choice of new engine as I felt he deserved a treat for being so good and brave. He asked for Edward and we talked about the games he could play with him when he got home. This procedure should have been done under sedation but oral sedation doesn't work for Wills as he can't absorb it and they would rather only use general anaesthetic where it is really necessary as it causes chest infections for Wills. William is very forgiving though and soon forgets what he has been through (but certainly did not forget the promised Edward and carried it home proudly).

Yesterday, we had a multidisciplinary meeting where all William's therapists and nurses etc, and I, met to talk about what we are all doing with him and what the goals for the next 6 months will be. It was good for everyone to meet each other. So many people are involved in Wills now. Of course, there are lots of things to sort over the next 6 months. Nutrition needs reviewing as William has not grown much and lacks energy. We are going to do 48 hours of fluid balance charts and blood glucose checks in a couple of weeks to see if he needs more calories, fluid or even longer on TPN during the day to maintain his energy and fluid levels. He only gets 6 hours off the drip as it is so I hope he doesn't need to stay on longer. We will see. The other priority for the next few weeks is to stabalise his posture more, so he needs a new, more supportive seating system and wheelchair. He will also get foot and ankle splints. When he starts school and needs finer motor control, he will also get hand splints for fine work as his fingers are hyperflexible and bend back.

Meanwhile, Hope and Ellie are well and happy. Hope got back from Guide camp in time for a week of SATS, which she said she enjoyed! They held auditions for the end of year production, something Hope is very excited about. Now, she is off on school journey for a week and then off to Dads and Granny (Dad's mum) next week, with Ellie as well. A very busy time for her. Ellie is like a fish out of water this week. Honestly, they fight like cat and dog and then she wonders around like a lost puppy when Hope is away, saying all the time how much she misses her. Talk about can't live with and can't live without!

It never rains but it pours...or gushes...

We have had a CHAOTIC day, starting last night. I was washing up and about to go to bed nice and early so I can get up for my run. I heard some water tricking and opened the cupboard below to find quite a steady trickle from the pipe. Next, a river and, within 30 minute an utter torrent. The noise was really quite alarming! I couldn't find the stop cock (still can't find it!) Water was gushing into the house and I had to wait 2 hours for a 24 hour plumber. Paul was out and it was a total disaster. Then, the electrics went, doubtlessly due to a very soggy mains wire under the kitchen. William is dependent on his pump and nebulisers and his TPN has to be kept in the fridge. Paul came home and took the TPN to the hospital to get it in a fridge and the electician arrived and switched the water off at the street. The sound of quiet has never been so welcome, even if it did cost me £140!!!

Luckily, we managed to get the electric back on.The cause of the problem was simply a join in the pipe that had come loose and, under the pressure of the leak, popped off.

I eventually got to bed at 2 Neither of us heard the morning alarms so it was one of those run around chasing your tail, getting later and later mornings. I was relieved William's respite nurse was coming so I could leave some of his care to her but she was also having a shocker (and continued to do so as you will see) so arrived late. Hope walked to school alone but Ellie and I were late.

William, the respite nurse and I arrived at the hospital for his blood transfusion. All progressed fine so I left them to it, explaining to her exactly how to get home. She has been coming to William almost every week for a year now so is well known to us all so I felt confident leaving him. I needed to sort out the insurance (of course, the floor is knackered, and one of the kitchen cupboards) and finish washing and tidying the cupboard contents.

It was all sorted with just over an hour to go before needing to pick up the girls so I set off on my favourite run for the time frame, looking forward to getting off the road and over the Addington Hills (despite the rain). I had only gone about a mile and still in Central Croydon when the phone rang and it was the hospital

William has a gastro-jejenal tube. This has two ports, one that sits in his stomach to drain away the nasty acidic bile that collects there as his stomach empties very slowly, and another port in his jejenum to enable drugs to be delivered there as they irritate his tummy. This is held in place by a balloon on the inside of the stomach. William had been on a bed (he usually sleeps in a cot) and had looked like falling and, in preventing him from doing so, this tube had been torn out, balloon still fully inflated! I stopped my run to make numerous calls to sort this out, as I still had to collect the girls so could not do it myself. Among the bag of emergency kit we carry with Wills, is a normal gastrostomy to insert if the tube should come out as the stoma can close. I explained to the staff how to do this.

After I had sorted things out (or so I thought) I had 30 minues before the girls needed picking up so I decided to do a 5K tempo run. I really needed to burn off some adrenaline as I was pretty cross this had happened as we are so so careful of William's tubes. Without really trying, I ran much faster than usual and smashed my current 5K PB (which was my first ever run so pretty out of date) by 7 minutes at 23:53! I could be a safe bet for the Hydro Active. I hope I can do this time when not so pumped up and stressed as I am hoping for a sub 25 mins.

Things were not so settled at the hospital though and I received a series of calls, on the way home from school, about the tube not draining and X-rays confirming its place etc etc. I was confused as it is a simple thing to insert the basic tube. I suggested I should come up and sort things out but was reassured all was OK and they would be on their way home soon. I stayed home as I didn't want to pass them on route. Unfortunately, all William's gastro specialists are at a conference this week so I can't arrange for things to be properly sorted until next week, when Wills will need a small operation to insert the new gastro-jej tube. I sat at home, desperate to scoop him up, give him a cuddle and make sure all was OK as it had been a rather traumatic pull on the tube.

After a while of wondering why it was taking so long for the 30 minute bus journey, I got a call from his respite nurse to say they were lost! The reception was dreadful and I had no idea where she was and how they could have got there! It was really worrying. I thought about poor Madeline's family as, all I was going through was not knowing where abouts in my own town William was with someone I know but I was still so so worried. It got a bit on top of me I must admit. Wills is so vulnerable and I felt I should have been there with him to prevent these things happening. It could have been so much worse too. The truth is though, I need these respite breaks and a blood transfusion is a routine, regular event for Wills so it wasn't wrong of me to leave him in the care of a nurse he knows well while it took place. Eventually, they got home (2 hours later) wet and cold. The poor girl had a nightmare shift with Wills and was very upset about everything that had happened. I checked his tube and saw the problem immediately. It was WAY to short, the consultant who insterted it had threaded it below the stomach, into the jej. Hence the X-ray. He was in agony. This tube is not designed for this and was not being held in place properly. I reinserted it and, low and behold, loads of gas and aspirate poured out! Once again, I thought that I really should be there 24/7 as it seemed several nurses, a registrar and a consultant did not understand the specialist issues in William's care and treatment.

It took me ages to get the poor thing settled and the girls' moods escalated in reflection! A nice family meal with them and all is now calm. My wine bottle is open and my remaining Easter egg by my side with a "Lost" DVD and a novel. The remaining untidyness can stay remaining until tomorrow!

No clear answers from Birmingham

Home is once again full after a weekend away. Hope has been on guide camp while the rest of us spent the weekend at the hospice. Very welcome following Paul and I travelling to Birmingham for a discussion with the small bowel transplant team on Friday.

I was hoping, as ever, that the trip to Birmingham would bring about instant answers regarding William's condition and treatment. However, as usual with our Wills, the discussion threw up more questions than answers although, overall, we felt the possibility of William receiving a transplant is not too likely. William is not yet critical enough for a transplant. He has at least 2 sites left for his hickman line (although how long these sites last have varied between 10 days and 8 months so this is not good indication of his prognosis) and his liver seems to be behaving itself at the moment. However, William will become critical at some time in his childhood and the idea that a transplant could be his lifeline when this occurs is a great comfort. This situation could arise this year, it could be next, or it could be a few more years down the line but it will happen. As well as intestinal failure, Wills also has physical disabilities that appear to be progressive, recurrent severe lung infections and odd blibs in his immune responses. This is all believed to be due to one underlying condition, probably genetic. We listened to lots of information about small bowel transplants and, towards the end of this, were told that the 2 main situations in which they will not consider transplant is where there is a progressive neurological condition and where there is a disease that is impacting the immune system. This is due to the enormity of the operation and the future impact of anti-rejection drugs. Everyone in the room knew that both of these contra-indications could well rule William out of the option of transplant. So, the next couple of months will see further tests and collation of results to try and decide if William does have the kind of progressive condition that will rule him out of transplant. These results will be shared with the Birmingham team who will, in turn, meet to decide whether he should be assessed for transplant, assessed at alater date when his condition becomes critical or seen as unsuitable for transplant and his care viewed as purely pallitative, enabling any medical or surgical intervention to make him more comfortable to take place. Even if he is seen as suitable for transplant this can not be seen as a magic bullet. It is a horrible operation followed by several months on a medical rollercoaster. Only 55-60% of children who have received a small bowel transplant are alive 5 years after their operation. Lots to think about and some nervous months to come as we wait for the results that will tell us what can be done. Paul and I hope it becomes clear cut it is either ideal for Wills or not possible. It would be a very hard decision to make not.

I was grateful to have the hospice staff to chew all this over with through the weekend. It was an interesting weekend at the hospice. There was a lovely family there who have just lost their son and were receiving bereavement support while he was resting on the "special bedroom" or mistral room. They have a daughter the same age as Ellie and they were inseperable. This did both girls a heap of good and Ellie spoke to me for the first time ever about "when William dies..." This had been something far to frightening for her to think about but seeing another family go through this has shown her that, although very very sad and difficult, she will still be able to play and laugh and make new friends and none of us will change who we are and what we do. We will just be very sad and will always miss Wills. It is strange and sad how my perspective has changed. This was a really important time for Ellie.