An exhausting day!

Our trip to Kings College Hospital today turned out to be a totally exhausting experience!!! It was a horrible place to get to involving train changes with huge staircases and no lifts! I had to carry William, the pushchair, his pump and bags up so many stairs I could have cried by the end of it all. People did help out a couple of times but on one huge long staircase at Denmark Hill there was a girl behind me having to wait (this was a narrower one with no passing space) while I paused and got my breath back and rearranged William and never once offered to help. On the way home I was desperate for a coffee and stood at the top of the stairs with William and my coffee and asked the next person who passed to carry my coffee down. It was such a long day.

William's ultrasound showed that his liver is enlarged and fatty - but we knew that anyway. They took loads of bloods (26mls worth so I dread to think what his Hb is now!!) and will look at those together with his ultrasound to make sure there is nothing else going on other than the effect of TPN. As long as his liver numbers are OKish and he is doing OK on the TPN, they feel it will be better for him not to go for transplant as it will enable advances to be made in bowel transplants (they are still very new and behing other organs) and the bigger he is when he needs it the more chance he will have to get an organ. However, once it is confirmed that he is definately on TPN until a transplant and no hope for any other cure it will be a fine balance to make between leaving it as long as possible and too long. If we wait until liver failure there is always a chance a donor will come too late whereas if we go too soon his life could have been lengthened by waiting... At least we are not at that stage yet. They explained that infections will speed up liver failure - we have already seen this as his liver gets cross and his liver function tests go a bit off when he has infections and he tends to never completely recover back to where he was before. So, it is even more important that we get to the bottom of his current pattern of repeat chest infections - even if it means a repeat Nissens. They are just checking he hasn't got any other form of liver disease but, otherwise, they will be kept informed by Chelsea and Westminster of how things are progressing and will see him again when (and they did say it is a when) his liver function tests go really bad. Today was really about getting a baseline I guess.

We had planned a day out tomorrow but today was so tiring and with such excitment to come later in the week we are going to pace ourselves (well me really) and have a day in. I am thinking a good old girly film Hope, Ellie and I will enjoy when William is asleep and a huge bowl of popcorn!

Godstone Farm

Today we were determined to fit a family day out into our 6 hours off TPN. It was one of those very rare occasions we were all free too! There is a fantastic chidren's farm that we found out was close enough to get there and back and still enjoy a good 3 hours fun. William had his first real close encounter with a rabbit which he was really fascinated in. He was pretty gentle too and the little bunny was definately used to toddlers stroking a little too close to the eyes. He also loved the "cows" and "neighs" and "baas" but best of all was the tractor! Yes, the genetic little boy obsession with all things vehicle seems to have been well and truely switched on at late. On Saturday, his Godmother sent him a little "Bertie Bus" who has been racing "Thomas the Tank Engine" all over the house ever since. William kept running back to his beloved "tata". In fact, he ran everywhere, even up and down some very steep banks on the nature walk and woodland maze. He really does pack his whole day into these 6 precious hours! We also enjoyed the vast stretch of adventure playground where the girls loved the zip wire and William had a fantastic time on the swings and in the sandpit. We even found the time for a cup of coffee and huge slap of cake while all 3 children sat in a very intense pow-wow on the grasss looking very deep in discussion. I was gutted that my camera was out of battery at that point as I forgot to charge it this morning.

On Friday we are off to a special evening opening at the zoo with all the other CHASE families. That will be great and Paul's Glyndebourne rehearsal has been moved from that day so another rare opportunity for a special family time - two in one week! William is just about ready to get a lot from it too as he is really showing an interest in seeing all the animals now. He has come on so far from just a couple of months ago when I first took him to our closest city farm and he really didn't pay a huge amount of attention. Now he just keeps going back to say "bye bye" to everything and watch some more (bye bye being his word for hello and goodbye!).

Tomorrow, we are off to Kings Hospital for William's liver scan and to see his liver consultant. By coincidence, his bilirubin today is the highest it has been in months - still OK at the moment but I don't like upward trends. A good time for his Kings appointment though as they will, hopefully, be able to nip anything in the bud.

Clinic

Another clinic this morning. The first question was about how William is in himself now. Luckily, he is pretty good today or I get the feeling we would have been admitted. We have to bring him in if there is any more blood so he can have an emergency colonoscopy and endoscopy. Following the disastrous result of having oral antibiotics he is now not to have any more oral meds, apart from the Urso and Vitamin K as they are such tiny quantities. We do doubt they are actually being absorbed though. He has to go to Kings for a liver work-up on Tuesday so we may address what to do about them there. They will also look at his fatty liver and the fact his liver enzymes are up a bit at the moment. W discussed his chestiness and recurrent nasty chest infections and pneumonia today. We have to get him reviewed again at the Brompton but, if it continues, he will have another endoscopy to see if he is pooling saliva and stuff in the upper GI tract, ready to aspirate and choke on. It may also be that his Nissens is coming undone and may need repeating. Poor thing, so many operations and procedures. So, all in all, we are muddling along but not quite as well as we were. Hopefully this is just because he has been unwell and it will all pick up again. It is a shame the antibiotic had such a huge impact as it looks more and more like William just can't be fed, at least not for the foreseeable future. Unless there are any emergencies over the next 4 weeks, all this will be re-assessed then as his consultant is on holiday. When he gets back, we will have to see if William needs the endoscopy and if he needs yet more fluid in his TPN to make up for the huge losses he is having in his stools and gastrostomy drainage at the moment.

Wiggle and Jiggle and Splish Splash Splosh!

Well, after a bit of an anxious evening, neither Paul or I wanted to check the morning nappy for fear of what we might find. Paul eventally wondered in (I have a huge fear of finding emergencies and must admit to passing William to Paul and being the one to get help when he was in respiritory arrest with septic shock!). Apart from yellow liquid absolutely everywhere in a most spectacular morning explosions all was normal. We had a few bloody streaks and some discomfort during the morning but not so much as to be more of a worry than something that needs to be discussed at tomorrow's clinic appointment. The nurse specialist called make sure all was OK but we were able to leave it to tomorrow to sort out.

To celebrate maintaining our freedom we had an action packed day. I took William to "Wiggle and Jiggle" a song and action session for toddlers at the library. He absolutely loved it and joined in so much. I was amazed how quickly he picked things up. He knows every action to his favourites, the wheels on the bus, wind the bobbin up and all 5 versus of row your boat. He was so good at staying on the carpet and joining in.

The sun was out at last and when Paul arrived home from a rehearsal in the afternoon, he decided to get out the little paddling pool (filled with buckets) and road test the dry suit at last! William hasn't been in anything more than an inch of water since September and has never yet been swimming so at first he was a little apprehensive. He soon relaxed and really started to enjoy himself. It was lovely to see him in there. He could splash and splash without the fear that it would wet his dressing too much. He had such a great time. Now he will be able to go swimming and paddle in the sea and really enjoy the water rather then have me quickly take him out of the bath the minute he starts to enjoy it. Thanks so much to REACT for buying this for us. I can't wait to take him into the pool at the hospice next weekend.

Busy Day

A very interesting day! This morning I had an interview for another part time job I can do from home..and the good news is...I got it! Will post more about that tomorrow when it is all formalised. As I had William cover for the day I then went to meet the lovely Emily AKA Angel. We chatted for hours (boy can we both talk!) and I couldn't believe that I had never actually met her before. I had such a chilled day and had no idea that poor Wills was having yet another little problem. The poor thing has been really uncomfortable this afternoon and has been passing fresh blood. I wasn't told over the phone as Paul had made all the phone calls and taken him to the hospital for blood tests and there was nothing more I could do...except stress!!! The blood clotting looks OK but we have to contact the Chelsea and Westminster if he passes anymore so we have another night and morning hoping all is OK and wondering if we will be back in hospital again. Both Paul and I are pretty nervous and squemish about what we might find so are both pleading with the other to change him at the moment ;-)

Hooray!!!

Just a brief post today to say that Dad got the all clear at his appointment!!! We were all expecting the worse but it turns out there is just a little thickening at the site of his previous op that the radiologist thought could have been cancer cells. The consultant really doesn't think so but says, even if it is, it will be very easy to treat. They will check it out in a few weeks. I am so relieved!!!

William is a bit better too. He is still very wheezy and has a cough that is frequently turning him purple but is a little more energetic. His bloods today showed an Hb of 13! Fantastic - thanks to his transfusion of packed red cells last week. His liver enzymes are high though, which was not a surprise as his liver is currently huge and bulging. This shows his liver is getting a bit cross with the combination of TPN and a chest infection. All this takes its toll in the long term so we could really do with as little extra illness as possible. He is also breaking out in the most horrible red and angry excema in huge patches. I think he is a little bit run down at the moment - still smiling though as always!

It was all yellow...

OK, so usually you see a lovely cute picture of William here. This time I have put a delightful picture of 6 baby grows heavily stained in the yellow stuff that pours out of William's bottom, gastrostomy and jejenostomy. Last night was particularly spectacular and this is the result - yep, one night! and there are 7 sheets too! I took this picture in case I have to convince our water company that we have a medical need for a large water usage if the shortages get worse. I put it here because one key purpose of this blog is to show people what it is like to have a severe intestinal condition and this is a large part of it!
Poor William's bottom is stained yellow - I often sing the Coldplay song to him. It will become even more relevant when he finally goes yellow with jaundice - well, you have to find the funny side don't you! Not that it is easy to find when you have changed a child and made his bed every couple of hours all night. Luckily for me, our lovely night respite nurse, Margaret, was here last night and usually this only happens about 3 times a night.

It was very moving in church this morning. There had been a funeral on Friday for a young girl who suddenly died due to a huge epileptic fit. Her Mum is a regular member of the church and so a wonderful display of her art work and horse riding rosettes had been left at the front where it had been for her funeral. I was so moved, she was so talented. I thought this was such a lovely idea and certainly something that will go into the notebook I have in case we ever suddenly find ourself in that position. Wills has some lovely art work and photos. I have to capture every moment of his life and have a special scrap book. It will be lovely to have whatever happens as this period of our life will always be worth looking back on as we have gone through, and are still going through, so much. Still, I do have my notebook because if anything does happen I want to be able to remember all the thoughts I had to make it a really special day and I know the grief and shock would stop me being able to do so. Oh and yes, "Yellow" is down there - a bit of a joke to myself to make me smile if I am ever in the situation to need to refer to it!

Unsettling times

First I want to say a huge thank-you to Emily, Emma and Shadow who are alwaus so quick with words of encouragement when things are very good or very bad (we hardly seem to get an inbetween! Maybe because we are so busy making the best of the good times). It makes so much difference knowing people are thinking of us and rooting foir us. Willam says a special hello to Nayan - thank-you for thinking about him.
Well, we made it through the day at home by the skin of our teeth! William is still pretty poorly and is coughing and choking himself purple. He is so congested. It is a very long time since I have seen him like this. All the extra secretions that are swallowed are upsetting his tummy as he can't tolerate anything in it. He doesn't really know what to do with himself but has still spent a lot of the day smiling and laughing as usual! He looks nice and pink though as you can see. This is due to the blood transfusion he had on Thursday and is a rare sight as his Hb slowly drops between transfusions and tends to plumet when he is unwell. I think he is a bit flushed and flustered too but it makes him look well!
Paul is away singing a gig in Somerset today and I hate it when he is away and William is unwell because there is no-one to ask what they think of him and how he is. I have to make the call alone and sometimes wonder if I want him home so much the doctors let him home when he is still a little bit too under the weather.
I was catching up with the CF Trust message boards today and learned that a man with a young family has died and another young lad is very sick and his mother is terrified. I don't know either of them but it really upset me. When you are in similar positions you can physically feel the pain they must be feeling. Of course, this kind of news also brings your own agonising fears that you fight so hard to keep at the back of your to the forefront of your thinking.
This is an axious weekend for our family for another reason as well. My Dad had a huge cancer op 2 years ago. He has recently had a lymph node enlarged and had a full body scan 4 weeks ago. On Friday, he had a call to say his results have been discussed at a multi-disciplinary meeting and he has to come in and see his consultant first thing Monday morning. It sounds very ominous. I don't feel stressed though. I don't know if this is because I have been so stressed over the last year that I am spent or because I have learned now that some things just can't be changed and you just have to be positive and make the best of what you do have. I hope it is the latter. It is probably a combination of the two!

The bubble has finally burst!!!

Well, it had to happen! We were having such a lovely run with William. He has had a cold/hayfever for weeks but woke up on Tuesday morning very very chesty and out of puff. Through the day he sounded dreadful but not too worryingly so. We went to the GP and got some antibiotics. However, an hour or so after falling asleep he was lying looking very pale and breathing at a rate of 60 breaths a minute. His temperature had hit the magic, potential line infection, 38 degrees so off the A and E we went. One of our friends looked after the girls for the night which was fantastic. It turned out that William has pneumonia on the right side. He also had a very low Hb level (anaemic) so needed a blood transfusion. This seems to happen when he is unwell. The initial doctor decided to ignore Chelsea and Westminster's protocol that IV antibiotics have to be started if William has a temperature 38 or above and stopped after 48 hours if blood cultures stay negative. Instead she gave him oral ones for his chest. Unfortunately, these really upset his guts with the result of 400mls of bottle green gastric aspirate over night, continuous windy watery poos, wretching and tummy pain and distention. We didn't know what would happen with the addition of the antibiotics as the volume into his system would be much more than the meds he has at the moment. It is a bit worrying that it was no unpleasant as it doesn't look good for the chance of reintroducing food for the time being. The next morning they started the IVs and he responded well. His blood cultures came back Ok this morning so we are now home but on IV antibiotics for his chest. It is a shame because his chest had been good following his Nissen op for reflux but this is the second lot of IV antibiotics for his chest in 2 months and his X-ray showed "changes" on the right. I hope he stays well enought to be at home but he is very chesty and wheezy and a bit grey and blue around his mouth and face. We are having to keep him on constant drainage at the moment as his poor tummy is really struggling with the coughing. It may be that he aspirated on the "snotty" cold secretions and that caused an aspiration pneumonia. We may have to go back to suction to keep his upper airways clear of normal baby secretions. We'll see!

It was most unpleasant walking into the house as we had to drop everything and take him and the remains of 4 day old chicken thighs and rice were still on plates! Lovely! At least it hadn't been too hot. Paul had been busy with Glyndeborne Opera and the girls staying with friends so none of us had been home. One of us has to be with William all the time when he is in, well you do in any case with such a young child, but when he is in the local hospital we really have to stay on the ball as many staff are not so experienced with TPN etc as the specialist centres. I am really looking forward to a nice bottle of wine, a hot bath and an early night in my own bed!

Clinic

We spent a very long day in clinic yesterday. I had a sneaking suspician that William's jejenal tube had slipped out of place. I asked for an x-ray yesterday and Mummy's intuition was right! His tube had slipt back into his stomach. At the moment, he has a tube threaded through his gastrostomy. He reacts badly to stuff in his tummy so that could well be why he has been draining 100s of mls of bottle green yuk from his gastrostomy and why he has been in pain - I hope this is the explanation for it all anyway. He had his tube reinserted but it isn't a very nice experience for him. We are only giving him a few mls of medicine so it isn't the greatest of signs that such small volumes in his stomach caused such extreme symptoms. They commented that his guts are all pushed over to one side because of the previous surgery and the gastrostomy. I often think that there is no way his guts can be working properly - even if they had been - with such changes and not being used for 7 months, surely they would have atropyed by now!

It is getting more and more likely that William will have to have a laparotomy and illeostomy soon. This will be a huge op but will make him much more comfortable as all the liquid stools are now pooling in his system and he has a big dump of it all (which is spectacular!) but the pooling causes pain and discomfort and can cause backflow into his stomach. None of this can be done until the tests at Great Ormond Street as the information gained will help guide the surgery and what needs to be done. They only want to do one big op and get it all done because of risk in any future transplant.

One glimmer of hope - the consultant told us there are still some diagnostic stones unturned - one is that it may be an inflamatory condition that steroids could help. This is not really expected but any glimmer of hope is well worth holding on to!!! Of course, no-one would say for a second that one course of steroids and he is cured but at least they may help. However, nothing can be done until the tests at GOSH so I really must phone again on Monday and see what is going on.

William is still getting dehydrated in the heat so needs more fluid in his TPN. We may be able to get back to a 6 hour break again then. It is funny how artificial TPN feeding is. William put on a lot of weight due to the TPN but is still very short. He now needs to "grow into his weight" as the consultant put it. So, for the next 3 months he wants him to stay the same weight but grow in height. It is strange how controlled everything can be on TPN!

William stays happy as usual. There will be some tough times ahead but I know he will be smiling again so quickly after the painful surgeries. As soon as he goes into GOSH it will all be kicked off again and there is no knowing how long he will be in hospital for againl. That's what I hate the most - the lack of control and a feeling that his life is amost owned and controlled by someone else and when they say now we have to do what they say and put the rest of life on hold.

Fun (and problems) in the Sun

With Hope and Ellie's school closing on Thursday and Friday for polling and an inset day, we took the opportunity to take our first residential stay at the CHASE hospice - Christopher's. We had a really fab time and were so lucky with the weather (although the poor William was not so lucky in the heat! I'll come back to that later). The biggest hit of was the trampoline. We were the only family staying, the others were children in respite with their parents away, so Hope and Ellie had it all to themselves.

And William enjoyed it too once he plucked up the courage. OK, so the children weren't the only ones and I am a huge kid too!!! I was offered an aromatherapy massage and never really had the space for it as this stay was very busy with all I had to explain and show them about William. I reckon that a good old jump on the trampoline was actually a much better de-stressor!

It was great to jump on there with the girls and enjoy a really happy time playing games with them knowing that someone else was taking care of Wiliam. Even more wonderful were the two undisturbed nights of sleep. The children needing the care sleep downstairs while the families stay in a flat above. All meals are provided and everyone eats together, children, families and all staff. It is a lovely, supportive community. During the day, the children have a one-to-one nurse or carer. They take care of them, play with them, make sure all their clothes are washed, every little thing. They also look after the families. I was so impressed with how the staff could read the situation and join in with the fun one minute and offer a supportive ear another. I was having a moment with a coffee in a peaceful bit of the garden while all the children were playing on the last morning. This bit of the garden is pretty near to the "special bedrooms" for children right at the end. William was playing with Paul and after a while, the nurse came over to turn on the fountain for me and stayed for a chat about the more emotional side of things. I really felt that I could pour it all out. It is good to know that we will be able to come and have fun here, rest and maybe leave William from time to time so Paul and I can enjoy an evening out or a weekend away. It is also good that we will build relationships with people so if the time comes that we do have to say goodbye to William we will be able to do so in a place with lots of happy memories with people who know us and will share some of those memories.

There is so much more I could say about the soft play room, the art room, the pool, the music corner, the sensory room... There really is so much there to help children who may well not live into adulthood, and their families, to live their life to the full. On Tuesday, William and I will go to 'little Chasers' a pre-school group for the youngest children cared for by CHASE. I am looking forward to this and meeting other Mums who go through similar things as me.

So we all enjoyed the lovely taste of Summer, well mainly. William had a great time initially but began to get a high temperature and get very listless while he was running around on his TPN break. We were worried and feared he may have been getting a line infection. As soon as he was back on TPN he settled down. He usually wees for England once back on but didn't wee much for 6 hours, even with 110 mls per hour going in. The next morning, the nurse and I realised that he was dehydrated with all the running around, still leaking his usual yellow and green yuk from his gastrostomy and into his nappy and getting sweaty in the heat at a time when he is not getting any fluid. He has always been a very sweaty little thing in the heat. On the advice of the nurse, I called the team and Chelsea and Westminster who felt that William was getting heat exhaustion. So, now he is back on TPN 20 hours a day with only a 4 hour break to give him a shorter time with no fluid. We will review this at clinic on Friday.

Happy Mayday!!!

We had a wonderful May Holiday weekend. On Friday, a very good friend from the neonatal unit called. Her son was like William, in and out of intensive care and up and down. We spent many a time in the parent's room and agonising over a few mls of milk in the expressing room! Our boys are now nearly 2 and we keep talking of meeting up so this time I said - why not tomorrow! So we met on Saturday and had a lovely time at the farm and at a Mayfair near by. It was so lovely to see the little tiny, sick babies now toddling and lovely to meet again in more relaxed times - we willl keep in touch so much better and have already penciled in plans for a Thomas the Tank Engine show in August. We had a ride on a little steam train at the fair and the boys loved it. William was in his element and didn't want to go away when we had finished the ride, He just wanted to stand for ages and say "bye-bye, bye-bye".

We decided to come back ot the fair today as Ellie wanted to watch more of the May-pole dancing and I knew Wills would love to see more of the train. Hope just wanted more from the chocolate fountain stall! Paul was away at a hen-weekend the previous time (wearing a dress!) so he fancied taking William to see the train again. We had a great afternoon. The sun came out and it was a great atmosphere. The girls had hair braids and Ellie and William joined in the May-pole dancing. You can just about see him dancing with Mummy in the picture. When William had had enough, Mummy and Daddy enjoyed hot cider punch while Ellie carried on! Hard to believe this was all in the middle od South London.

Over the last couple of days I have really been struggling with what could happen to Wills. It is really stupid because he is so well at the moment, maybe that is why I feel able to think such thoughts as they really are only fears at the moment, where at other times could be too close to home. I have found myself really choked up at the most ridiculous of things, seeing William enjoying himself with his sisters, the way he is developing his favourite things. He is so developing as a little person now. I just wish someone could tell us what his future will be. I have made a pact with myself to make sure that every day has a happy memory for all of us. I don't want to waste a single day.