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.