Seasonal greetings

Well this will be the first time in 4 years that we have spent Christmas at home without having hospital looming over us. Indeed its been an exceptional year – the first time in 8 years we have not spent a night in hospital….. seems almost surreal.

The boys continue to do well and life is coming back to something like normal (what ever that is).

Joshua will be 19 this January and is on course for university next summer to study software engineering. He is going through a delayed growth spurt at this moment and is now about as tall as his Mum.

Nathan is now 17 and loving life at Brockenhurst College. He is studying 4 A levels (Graphic Design, Business Studies, IT and Photography) and has a really positive sunny outlook on life. He has also shot up this year in height.

Daniel will be 15 in March and has taken well to GCSEs with his clear favourite being catering – indeed next summer he has a 2 week work placement at a local restaurant/hotel.

Luke will be 11 in January and really continues to amaze us all. He has made a full recovery from the stoma reversal and has also regrown the height he lost last year when his spine ‘crumbled’ because of the length of time he was on steroids. He does though have alopecia which is not fun but he takes it (as all things) in his stride and with a must do attitude. If all goes to plan then around spring time he should have his central line out.

And both Dad and Mum are doing well – indeed it seems that we almost in an unbelievably good state considering the last 8 years. David’s work is going very well with trips to: Thailand, Israel (including 2 days in Jerusalem!), India (saved by Immodium), Canada (including 3 ‘day trips), USA and much of Europe. We had an amazing holiday down in Devon (on Exmoor) this summer – will never be forgotten.

The XLP Research Trust (www.xlpresearchtrust.org) continues to be a major part of our lives and we have this year expanded the outreach to XLP families (the web site is now available in 14 languages), hosted the worlds first medical conference on the condition and we are about half way through the next research grant round. Thanks for your continued support.

So thank you for standing with us. We feel we are on the edge of coming out of the valley……..

Blessings

David & Allison and the rest of Team Hartley.

Hi there

I guess the fact that it’s been months since the last update you have a clue that things are going well here – at last.

All the boys, and parents, are doing well.

Luke is still waiting for his hair to grow (coming back very slowly) and is still on a holding dose of steroids but otherwise is making great progress. He is full of life and energy and has a genuine deep thirst for life – really wonderful.

Joshua is still battling the scleroderma and in the main winning. He is in his last year at college and we have just been touring universities for next year……

Nathan is loving college and seems to growing daily…

And Daniel is also doing well and taking his GCSE’s in his stride – particularly Food Technology.

We continue to spend most of our spare time fundraising for the XLP Research Trust we set up to help other XLP families and to fund medical research into this awful condition ( we are mid way through making our second lots of medical research awards). The site is now available in 12 different languages as well.

On the 5th December we will all be taking part in a 5K Santa run in Greenwich Park, London. We will probably we walking; with a wheelchair – but it will be the first time we will have been able to do this as a family. It should be a great day. If you would like to join us then please let me know as the charity still has a few places available (you can run or walk!).

If you would like to help sponsor us please visit the link below:-

http://www.justgiving.com/XLP-5KSantaRun

Blessings

David

Hi there

It’s been a while since we last put out an update – which hopefully for those of you who have been following us now for almost 7 years will know that things are going well!

We had a wonderful holiday down at an amazing place in Dartmoor. Indeed as a result of this we now have 4 chickens in a coop in our garden – any suggestions of names would be most appreciated (we have already come up with ‘roast’, ‘fried’, ‘tikka masala’ etc.)

Last Friday we picked Luke and Daniel up from a wonderful camp for children who have been through tough times (‘Over The Wall’). They both had a great time but on the way back we were able to reflect on just how well Luke has recovered from this time last year. You may recall that we had to eventually bring Luke home from this camp last year as he became exhausted. But as he had a stoma bag, was receiving both IV TPN and a milk feed it probably wasn’t too surprising. This year we were told that he was last to bed and first up every morning – and that he volunteered for everything. Indeed he even scaled a climbing wall. And he is finally growing again. In just 6 weeks he has grown an amazing 2.5cm. So he has now put back the height he lost when his spine ‘crumbled’ due to low bone density – and he is again walking much more upright…

Oh and Daniel had a good time to and is exchanging emails with a young lady we met on camp.

Both Joshua and Nathan are well. A week today Nathan will learn the results from his GCSE’s but he does seem confident. He also has a part time summer job at a local opticians (2 and a half days a week) which he is enjoying. If anyone knows of any work for Joshua please do let us know!

And Allison and myself are well – indeed we grabbed two nights away last week which was rather wonderful.

Thanks again for your support – it remains so crucial to us.

Blessings

David

PS – We are just in the process of making our 2nd lot of grant awards for the XLP Research Trust – our 2nd job! If you live in the UK you could support us by making part in the Great Big small Charity Car Draw where you could win a MINI First. Tickets cost £2 – please visit this web site and follow the instructions - www.smallcharitycardraw.co.uk

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