Hi all

Almost there…..

Luke is very well indeed. He is now practicing to enter ‘Britain’s Got Talent’ as a budding opera star. Our ears have never hurt so much….

At clinic at GOSH two weeks ago it looked like he would be finally coming off the last lot of steroids and then basically onto penicillin only. The GVHD seems to have settled down and amazingly he is now eating normally (although still very selectively). He is off the overnight TPN (hurray!) and the overnight Modulin gastro feed has been significantly reduced; if all goes well he will be of this within a month….

But (sorry there is always a but) we are not quite there…..

A lung function test at GOSH on the same day, suggested that his lungs are only functioning at around 60% of capacity. When he went through the issue of his ruptured bowel he also suffered a deflated left lung; and it now looks like he may have bronciectasis from X-rays and scans. So Luke has just started on some a 2 week course of IV antibiotics to see if this improves things. However at Southampton General Hospital yesterday, they repeated the lung function immediately before the first antibiotics and his lung function is now 71%! (80% is the bottom of normal).

So if all goes well we will be back on course in a couple of weeks time.

Some of you may be aware that Allison’s Mother was admitted for replacement heart valve surgery last week. All went well, and we were expecting her home at some point towards the end of next week, but Allison and Luke brought her home yesterday! Luke described it as ‘a dream’ over dinner last night. I am having much fun now reminding her that the eating of sausages and bacon is in effect cannibalism!

Finally – if you live in the UK would you like a brand new MINI first? The charity we run in our spare time is part of the Big Small Charity Car Draw and for just £2 you have a genuinely good chance of winning a brand new car. Because the car was generously donated, £1.90 of every ticket comes to the charity direct and will be used for medical research into XLP. Please visit www.smallcharitycardraw.co.uk and choose the XLP Research Trust as your charity.

Blessings

David

Very good news – the bug isn’t MRSA….

Also it would appear that Luke doesn’t have a leak in his bowel but the pain was caused by pneumatosis which is treatable by the antibiotics he is on.

He is expected to come home during the day tomorrow or Sunday and then to be home early next week…

Wonderful news

Phew….

David

Hi there

It’s been a while as everything has been progressing positively – until yesterday….

Luke has some constant pain around his bowel so we took him into Southampton General Hospital to be checked out. A scan suggested that there is a small tear where his stoma previously was. This was obviously a huge surprise to us, as he has had no problems down there since Christmas Day. So he is now on antibiotics and nil by mouth, and was fine by the afternoon. So the plan was to observe for a couple of days and then have a home plan.

But we have just heard that Luke may have MRSA. They have detected a ‘Staph’ bug in his lines and only further tests will reveal which it is. So he is now in isolation and we should hear at some point on Friday afternoon.

So our journey through this dark valley seems to be continuing…..

David

Hi there

It’s been a couple of weeks of pretty good news here.

Joshua went to GOSH today for clinic and they were delighted at the progress he has made. Indeed they are now putting a plan together to finally take him off the steroids, they have stopped his 3 weekly immunoglobulin and we are rescheduling his ECP treatment to every 8 weeks – stopping in June. Plus as he is now over 18 he will be seen at an adult hospital (either in London or hopefully at Southampton). Wow. Joshua holds the record of being the longest seen patient by the BMT team post BMT…..but he is just about through!

Luke is also coming down on the steroids and if everything remains well we should reduce them again in a weeks time. His skin GVHD seems to have settled down and his bowels are performing well…. The only downside is that about 10 days ago at school he was pushed and fell over and now has his left hand in plaster. Aside from the initial pain (small chip on a bone) he is very proud of his red caste…

And on Monday I had the pleasure of hosting the world’s first symposium on XLP in London. We had 14 medical researchers from across the globe presenting their findings to date on this rare but life taking condition and many seemed to have eureka moments. Much of the detail was too much for me – but it was a most wonderful day – so thank you all helping make this possible by supporting our key charity.

And if you fancy supporting su then perhaps you would like to become part of a world record and possibly win £10k in the process. We are again involved in the Great Duck race – please visit their web site and support us with a duck or two! http://www.thegreatbritishduckrace.co.uk/

Blessings

David

Hi there

I trust you have had a wonderful time….

My two Christmas ‘wishes’ came true this year.

Firstly, I wanted a day free of hospitals – and as all the boys are so well we definitely got this.

Secondly, I wanted to see Luke’s ‘output’ switch to where it should be coming from – so that we would know that we would soon be ‘stoma’ and bag free, and since early morning on Christmas Day this has also come true.

We had a very relaxing time, watching DVD’s and laughing at how out of tune I am on ‘Band Hero’ on the Wii….

Blessings to you all

David

Hi

Today has been a really positive day for Joshua.

We received back some results from the hospital to say that he can finally tail off the last small dose of steroids he is on. Because Joshua has been on steroids for such a long time, there was real concern that his adrenal gland may not be working at all – but it is normally!

And then Joshua went for a driving assessment. Because of his scleroderma, we were concerned that he may not be able to learn to drive. But the assessment went very well and it looks like he will be able to learn in an automatic car! So good news for Joshua, not so sure about my bank balance nor the other drivers in Hampshire!

Luke remains well and there is no sign of the pains he had before. He is back to his full-on self!

Blessings

David