Sleep Well Little Alex

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

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

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

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

Family fun!

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

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

Even closer to a diagnosis...

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

Always in our hearts and minds...

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

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

A very busy week

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

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

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

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

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

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

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

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

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

New adjustments

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

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

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

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

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

Back from hospital

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

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

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

A quickie to say...

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