Story written 2004

Nathan and his brothers were diagnosed with X-linked Lymphoproliferative Syndrome (XLP) back in November 2003 after suffering from acute anaemia.

The only cure is a bone marrow transplant which Nathan is due to have in 2005.

Nathan will then be the 3rd of the brothers to go through this – and he potentially could become the piggy back donor for Luke – the youngest of the brothers.

Nathan had lymphoma cancer when he was just 3 and was not expected to pull through but miraculously did and only spent one night in intensive care. It was this episode that first alerted the doctors to the serious problem that we subsequently found out that all 4 boys suffer from – although it took us almost 7 years to get there.

Nathan is the peace maker in the house. Although small for his age he definitely holds his own both at school and home – and he is a real charmer and future lady killer. He has an excellent sense of humour, but is also very determined which we believe is born out of his experiences of life. We are confident that he will end up on the stage one day.

Update 20th February 2008

We sat down last night over tea and talked about the wonderful support we have had from you guys over the last 4 years. There was agreement that as Joshua, Nathan and Daniel are so well now that they should move on to the ‘Moved On’ status. It would be good to keep Luke on for a few more months – he remains well but still a good way to go.

Sincerely thanks for your support.

Update 20th September 2007

Nathan is also very well and importantly, clearly growing again. Shortly we will be having meetings at his school to choose his GCSE options…

Update 4th June 2007

Tomorrow Nathan will travel up to Great Ormond Street Hospital in London with me and on Wednesday morning he will donate some of his own bone marrow to save the life of his brother.

Update 28th April 2007

About 10 days ago we took Nathan and Luke up to GOSH for a standard day of tests as Luke is due his BMT in 2 or 3 weeks time and Nathan is his donor.

This Sunday should be a wonderful day (all of the Hartleys will be there) as we have the first XLP RT Triathlon. We have 27 teams battling it out over a 3.5 mile run, 16 mile cycle ride and a 0.5 mile swim!

Update 17th January 2007

Luke is now booked in for his bone marrow transplant in the middle of March. The big news however is that Nathan will be his donor. The good folks at Great Ormond Street Hospital learnt last week that the donor we were expecting to use is now no longer available – we know no more than this. The consultants at GOSH are ‘not worried’ by this and have already carried out a number of ‘piggy back’ BMT’s. Nathan is delighted that he will have the chance to save the life of his brother! Please continue to pray that the boys stay well as we approach this last hurdle.

Update 17th December 2006

Nathan came home from his BMT literally a year and a day ago. He had a few days in Southampton General in January for a small infection and has now flown through his recovery – wonderful. He has settled back into the hustle and bustle of school and became a teenager yesterday in Lapland – indeed it was Nathan’s wish to go there. He had his throat stretched around summer time and this has made a huge difference to his ability to eat – he has since put weight on and meals are not the battle they used to be. He is still small for his age and so next year we will look for further medical help if we do not see him grow.

We have literally just returned from a remarkable holiday in Lapland courtesy of the ‘Make A Wish’ foundation. The highlight for Allison and Dave was riding a team of 7 husky’s each through a snowy forest with the most amazing pink sky – breath taking and truly amazing. Dave also managed to turn a snow mobile over with Allison on the back…

Indeed with things being relatively ‘normal’ in the Hartley household we have also enjoyed a memorable trip to Disneyland Paris and a week on a farm in beautiful Devon and Dave took Joshua and Nathan camping at ‘Soul Survivor’.

Update 21st September 2006

I took Nathan up to GOSH last Sunday night and had the strange experience of staying on Fox Ward for a night – the ward where three of the boys have successfully gone through their bone marrow transplants. It was strange seeing the nursing staff and seeing the anxiety etched onto the faces of the parents there. Nathan was up to have his oesophagus stretched. For those of you who know Nathan, you will know that he over chews everything and takes an age to swallow. With recent visits to GOSH it became clear that he has a narrowing in his oesophagus and so in he went to get it ‘stretched’. For a young man of his size the size of the opening should be between 16 and 18mm. Nathan’s was only 5mm. The good news is that the ‘stretch’ was successful and it is now up at 16mm – and it was an easy stretch. He already claims to be finding it easier to swallow although his throat is a little sore following the procedure. Otherwise he remains well and we had the good news at the tail end of last week that he can now start his childhood immunizations again – the sure sign that they consider him to be successfully transplanted and engrafted.

Thanks for those of you who have sponsored Nathan and Daniel on their ‘celebration of finishing their bone marrow transplant walk’ they are now 10% of the way to their self imposed target.

Update 23rd June 2006

Nathan is also doing well. He is now off the overnight special milk feed, which is a relief to us all, and his weight is staying stable. Importantly for Nathan he now has grown 2cm since transplant – great news. The most recent bloods taken from him do show that he has a relatively high load of EBV (the glandular fever virus – which for XLP boys pre transplant is often life taking) so the hospital will continue to monitor this.

Update 15th May 2006

Nathan continues to get better and better. Now surviving full days at school and loving it! He is now just on a few medicines although still on the overnight milk feed.

Thanks for your continued support and prayers.

Update 15th April 2006

The news that we wanted – Nathan can return to school on Tuesday! His immune system has recovered sufficiently that the Dr’s from Great Ormond Street Hospital are happy for him to return.

This is really the landmark event that shows that he is now well recovered from his bone marrow transplant. Whilst it will still probably take another 18 months for his immune system to completely catch up it can now successfully defend him against most viruses. Indeed you may recall that Nathan has EBV (the glandular fever virus – this is the deadly virus for boys with XLP) well his ‘load’ has fallen from 17,000 to 6,000.

Nathan started a new school last summer and was only there for 2 weeks before going up to GOSH for his transplant. However he is really looking forward to getting back and into the swing of school life.

Update 18th March 2006

Nathan continues to do well. He is still not eating great but is now the heaviest he has ever been. His immunity is coming back strong and hope is that he will be able to return to school after Easter. This is a much faster return than either Joshua or Daniel, but primarily because he did not need to have his t-cells knocked out as he did not have EBV (the glandular fever virus – XLP boys have no immunity to this disease – for them without a transplant it is a potential life taker). Interestingly he does now have EBV, but his new immune system is holding it at low levels, and no one from the hospital seems concerned.

Update 18th February 2006

Nathan is doing fine. He is now some 130 days post transplant and is gradually eating more. Occasional bouts of sickness but overall doing very well. His blood results are now good so hopefully at the start of March the wonderful GOSH team who look after us will start to wean him off his immune suppressants…

Update 1st February 2006

We’ve just got back with Nathan from a trip to clinic at Great Ormond Street Hospital. Overall they are pleased with Nathan’s progress. Due to kidney damage (which are slowly repairing themselves) Nathan is currently not on a key medicine called Aciclovir – which amongst other things protects from herpes virus. This is a concern at the moment as his mouth has some nasty ulcers and we know the damage this can lead to. But otherwise good – his blood results are now all in the ‘normal range’ (HB, platelets, white cell count) and he remains well in himself.

Last week we feared that he had liver GVHD (Graft vs. Host Disease) coming on – but no – just the kidneys demanding to be rested. Nathan is well in himself and has actually grown 2cm’s since the start of transplant. This is great news for Nathan, we had been told that it would be unlikely that he would start growing again until a year after transplant – we are 8 months ahead of this! As Nathan is very small for his age this is wonderful news!

And for those in the UK please don’t forget to watch Songs of Praise this Sunday (BBC1 – 18:10 GMT) – it should be interesting!

Update 25th January 2006

Nathan continues to do well. His ‘bloods’ are all moving in the right direction. He still has some sickness and is heavily reliant on a milk feed to get the necessary calories in. He is still struggling to eat (Nathan has a narrow oesophagus meaning that he has to chew and chew). But he is getting on fabulously with his home tutor – a great blessing and otherwise remains well.

Update 16th January 2006

Nathan is home after 5 days of stay in hospital. It was a line infection but he is now back to normal temperatures…

This Thursday it will be the first historic milestone – 100 days post transplant.

Update 12th January 2006

Nathan is now in Southampton General with what looks like a line infection. It started on Tuesday but only reached the temperature necessary to have him admitted late last night…

We have ‘been here before’ but obviously not with Nathan. He remains good in himself although not happy with the sickness that seems to accompany the temperature spikes…

Update 1st January 2006

Nathan returned home from his bone marrow transplant on his birthday on the 15th December – it was a long but quite wonderful day. He has now been home for a whole 2 weeks and remains very well. He is on an overnight feed which makes our night times somewhat disturbed and interesting. Aside from his seizure and the damage to his kidneys (!?!) the transplant went well. Nathan remains the great charmer, full of beans and always happy to laugh. His hair is also starting to come back.

Updated 18th December 2005

It has been wonderful having Nathan back home. He has settled right back in and is enjoying good home cooking again – although is on a regular overnight feed. He is doing well and the GVHD rash has now all but disappeared.

Can I take this opportunity, a week before Christmas Day, to wish you all a peaceful Christmas. We are so grateful for the wonderful support over the last few months – it means so much!

Update 15th December 2005

Nathan is home – hallelujah!

Update 9th December 2005

I spoke to Allison at GOSH late afternoon. Nathan is doing so well that they are looking to let him come home next Thursday – 15th. This is wonderful news and well in time for Christmas. Now we do know from previous experiences that in 6 days a lot can change (Joshua was packed and we were told 12 hours before he was expected to come home that he had 2 stay for another 3 weeks!) but Nathan is definitely on the way home!

And next Thursday is of course his 12th birthday – what a fantastic present!

Update 7th December 2005

Nathan is now doing really well. Incredibly both his adenovirus load has dropped significantly and the ‘violent red’ rash has spread no further and is now fading – praise God! He is well in himself and is now spending a lot of waking time making the nurses, doctors and consultants laugh when they come in his room.

He will however definitely be in hospital for his 12th birthday which is next Thursday – 15th December. It would be great of you could inundate him with cards.

Update 30th November 2005

Nathan is in a ‘holding pattern’ at the moment. He remains well in himself but there are still a number of important issues:-

1. The Adenovirus is still on board and the load has increased recently. The +ve signs are that his lymphocyte count has risen into the mid 0.2’s – we need it over 0.3 to see the end of this virus.
2. But the GVHD rash is now back on his lower legs – if it spreads significantly then they will use steroids to dampen it down – which will also dampen down the lymphocytes.
3. And Nathan now has a fungal infection in his lungs. The Dr’s do not seem particularly concerned re this as it has been spotted early and is now receiving treatment – but is it giving him some very high temperatures.

Indeed because of the temperatures Nathan actually went back to bed today – the first time for a long time. He remains in isolation but is allowed off the ward – suitably wrapped up.

So still some way to go – we had hoped that Nathan would have been home for his 12th birthday on the 15th December but this looks unlikely now. And the prospect of a second Christmas with the family divided between GOSH and Romsey is also looming!

Update 23rd November 2005

Nathan is doing very well. The GVHD rash has now all but gone, he continues to absorb his medicines well and the blood gas results suggest that his kidneys have repaired themselves. He still has the adenovirus onboard and this is now what the consultants would like to see go next. He is allowed off the ward for brief times – and so even though we are in the middle of London we have been able to daily visit sheep and goats… Thanks for all your letters, cards and gifts – they are a real boost for him.

We now have to deal with having two sons in two separate hospitals – although we have been told that Joshua should be home again shortly.

Update 11th November 2005

It’s difficult to know where to begin this one.

Firstly Nathan remains well in himself, Mr Smiley and enjoying making stuff and watching CBBC and the new Star Wars movie. He is even baking despite being nil by mouth.

But there are a number of important conflicting medical issues that need to be resolved quickly before things potentially get very serious.

1. Nathan’s adenovirus load has increased significantly this week but is away from being ‘worrying’ as the consultant put it on ward round this morning. Whilst this is a common virus amongst children, for those that are immune suppressed it can be potentially devastating. Unfortunately the first line of defence drug used to suppress this virus has also damaged his kidneys so they have had to withdraw the drug to allow his kidneys to rest. They are now starting a second drug that should help hold the adenovirus back but without impacting his kidneys.
2. The IV drug that caused the fit a week ago was the main drug used to suppress the emerging graft (and help prevent graft vs. host disease). This was immediately withdrawn and an oral replacement given. Unfortunately Nathan is not as yet absorbing it well enough to give good protection – the level yesterday was 45 out of a required 150. This is probably due to the damage done to the kidneys – which are now being rested and should start absorbing soon… (Quickly please).
3. And his Graft Vs Host Disease of the skin is now over most of his body. Fortunately at this time it is not itchy. The plan here is to introduce a short course of steroids to push it back. But the steroids will hold back the graft that should also be tackling the adenovirus…

If you follow all that you will see a potential vicious cycle that we don’t want to head down.

It is important to note that Nathan remains well in himself through all this. Over the last 2 years we have tried to live ‘one day at a time’ but sometimes it seems impossible to do this. Yet we have seen sometimes great and sometimes small but significant miracles happen – I guess we need these now for Nathan.

Update 9th November 2005

On Friday last week Nathan had a fit/seizure. This was very frightening indeed and potentially very serious. The positive news is that he is now fully recovered from it – and indeed remembers nothing of it. There are no signs of any brain damage.

After a CT scan, lumber punch and MRI the team looking after Nathan are convinced that it is down to the toxicity of the main immune suppressant drug they were giving him so this has been stopped. This now leaves him open to potentially acute Graft vs. Host Disease which could be very serious indeed.

The next 48/72 hours are pretty crucial in terms of seeing whether they can replace the IV version with an oral version of the same drug. Nathan is on other immuno suppressant drugs but this is the main one.

Already he has a GVHD rash across most of his torso – which is not in itself serious but a warning of what may come.

Amongst all this we received a great CD that was simply entitled ‘peace’ – the timing could not have been better!

Please continue to pray for Nathan we need to get over and beyond this.

Update 4th November 2005

Nathan’s transplant went without a hitch. We are so unbelievably grateful for the wonderful mystery donor who has saved his life – who ever you are we are forever in your debt. He is now 100% donor engrafted and starting to produce some of his own white cells. And strangely any time now his blood group will change as the donor had a different blood type to Nathan… Up to yesterday afternoon he was doing very well with sickness – but he was very sick yesterday afternoon – a real shame as he had been doing so well.

Update 24th October 2005

I (Ian-family friend) have just had a brief telephone discussion with David and he asked me to post a brief update. Nathan has had his transplant and is doing well and has started the process of recovery. Joshua is improving and it is hoped although still has some way to go before he will be able to return home. Unfortunately Daniel has broken his wrist and now has a lovely red plaster on his arm! Please pray for the family, especially both parents as they are finding it a particularly stressful time.

Update 5th October 2005

Nathan is doing very well. He has settled well into hospital life and is taking it all in his stride. He has amazed us by quickly making the required 1.5 litres of fluid intake, eating reasonably well and managing his teeth (toothpaste and vile mouth wash – 4 times a day). No sickness to date but we know that it is very early days (day 3 of chemo today).

Update 30th September 2005

Nathan is doing OK up at GOSH. He has now had his two pre transplant operations and is doing fine. The first was quite painful as they had to stretch his oesophagus to get the probe down – but both were successful. Allison has been with him this week and I go up for my ‘tour of duty’ starting tomorrow. He is due to start his chemotherapy on Monday.

Update 27th September 2005

Nathan finally went in today to have the two pre operations before he starts his transplant conditioning proper next week. He was very positive and relaxed…

A few folks have asked for a photo – particularly with his short hair (none school regulation) – his hair should fall out in about 3 weeks time.

Update 25th September 2005

It all starts again tomorrow. There was a last minute cancellation for Nathan’s PEG operation last week so he will go up tomorrow (Monday) afternoon to have the PEG put in Tuesday. It is likely that he will then stay in (he may be able to sneak a day at home) before having his central line operation on Friday. In eight days time he starts the chemotherapy before the actual bone marrow transplant which should be on Wednesday 12th October. Nathan is very well going in to transplant and actually looking forward to it! He is having a ‘skin head’ hair cut this afternoon in preparation (I don’t want to go through the shaving of the
head that I had to with Daniel) and is very ‘up for it’.

Update 19th September 2005

The countdown is very much on for Nathan going in for his bone marrow transplant. We had the final green light last Friday and this Thursday (22nd) I will be accompanying Nathan to GOSH for his PEG (tube that goes straight into his stomach for easy administering medicines, food etc) to go in. And on the 29th we go up to start the pre treatment proper and chemotherapy. The expected date of transplant is Wednesday 12th October.

We go into this not complacent. A lot of folks seem to assume that as we have a good match, and that Joshua and Daniel are home cured, that this is ‘just production’. Two weeks ago we were reminded by the consultant of the significant risks that a BMT brings – but as it stands today far better odds than not going through…

Update 3rd September 2005

Nathan had a great day at his new secondary school – winning many new friends.

And for those who can, we will be featured on BBC Breakfast News this Monday morning – it was recorded yesterday so we don’t know what time!

Update 24th August 2005

We now have a confirmed date for Nathan to go up to Great Ormond Street Hospital for his transplant – the end of September, just 4 short weeks away. This may move out slightly due to availability of beds. It was sobering again to go through the procedure with the consultant again today – and to be told the % changes of all being well. Nathan remains well and has no fear of the forthcoming transplant.

Update 29th July 2005

This Sunday both Joshua and Nathan are going ‘Over The Wall’ for a week at a special camp which they are looking forward to.

Update 8th July 2005

Nathan and Luke remain well – Nathan is now due to go to transplant at the earliest in September. So hopefully he should be able to start his new senior/secondary school at the start of September – if only for a few weeks.

Update 11th June 2005

Nathan and Luke remain well. And Mum and Dad actually managed their first evening out on their own for well over a year. It was a little strange (but very nice as well).

Update 24th April 2005

The knock on effect of Daniel being in hospital (see his page) is that it is now unlikely that we will have Daniel well enough for Nathan to go up for his transplant in mid June – so this is likely to be pushed out yet again. Both Nathan and Luke remain well (and EBV free).

Update 18th March 2005

Nathan’s forthcoming transplant has been put back further to the middle of June. This is because there is a strong chance of a second donor being found and this would help us in terms of Luke’s transplant. At the moment we have one wonderful mystery donor who is a good match for both Nathan and Luke (the boys are identical twins apart from being born 6 years apart). When the ‘definite’ donor gives stem cells if there are not enough for both Nathan and Luke (they would freeze some for Luke) then we would have to wait 12 months before going through the process with the same donor again. If we have confirmed that there is a second donor then Nathan can have all the stem cells from the first donor… Plus it gives us another month to ensure that Daniel is good…

So the roller coaster ride continues.

Update 7th March 2005

Nathan is due to go in mid May now for his transplant.

Update 7th January 2005

Nathan remains well. It is planned at the moment that he will go in for his transplant around the end of April – this may be pushed back.

Update 31st December 2004

The elves and secret Santa’s went down very well in our household(s).

Daniel had a great day at GOSH opening presents and now has more than enough activities to keep him set up before returning home – hopefully in around 2 weeks time.

And Joshua, Nathan and Luke were also really blessed by the torrent of gifts…

We do have some photos and will send on to the elf’s ‘soon’ – with one parent at GOSH and the other at home we are not the best co-ordinated at the moment.

But sincerely ‘thank you’.

Update 13th December 2004

Next Friday we take Nathan and Luke up to GOSH for their regular 6 month clinic so they should be able to see Daniel then. We will be talking dates for the next one…

And speaking of Nathan he will be 11 this Wednesday. It seems amazing that it was around 8 years ago that Nathan nearly died of a b cell lymphoma – and that at this time God gave him back to us…

Update 23rd November 2004

Yesterday afternoon Nathan and his parents, had the joy of joining the Anthony Nolan Trust for their 30th Birthday celebration. They were the first donor registry worldwide and have inspired all of the others. It was a truly emotional time and I had to give a small address after watching a video of the boys. We then watched with tears flowing as a young man met with the police officer who was his donor 5 years ago – the kindness of strangers…

Update 29th September 2004

Those of you following our story will be very aware that the 3 most frightening letters for us are E B V… this is the glandular fever virus that the boys have no immunity to – and which can cause death for the boys.

A couple of weeks ago Nathan was diagnosed with it – although it was at the lowest level detectable. Last Thursday new bloods were taken again, the day that he was off school ill (first time in 7 years!). So we assumed the worst that the load would be much higher.

He is now EBV free.

The wonderful medical folks put it down to a ‘blip’ – as Christians we say ‘thank you Jesus’ … (and thank you for those that have prayed for him).

Update 28th September 2004

Nathan is back at school and seems 100%. We are still awaiting his EBV results and what action they intend to take if any.

Update 24th September 2004

Nathan unfortunately now has EBV on board. A routine blood test last week picked it up, as there had been no sign of glandular fever. It is at the very lowest level (50,000 load) detectable – but it is on board. Unfortunately he was quite unwell today so had his first day ill off school for 7 years (since his lymphoma). If the symptoms persist he will also go into our local hospital tomorrow (Friday) although he seems well this evening. New bloods have been taken to see if the EBV has progressed and to decide what action is necessary.

Josh is also in hospital with GHVD and Daniel is undergoing treatment at GOSH

So, it’s a real battle in the Hartley household at the moment – I think we feel that we can fight one battle at a time but not two or three together … welcome your prayers as always.

Story written 2004

Luke and his brothers were diagnosed with X-Linked Lymphoproliferative Syndrome (XLP) back in November 2003 after suffering from acute anaemia.

The only cure is a bone marrow transplant which Luke is due to have in potentially late in 2005 or 2006. He will be the 4th and last of the brothers to go through this and it is possible that his older brother Nathan could become his donor.

Luke is like every other 4 year old – apart from he also wants to do everything that his older brothers also do – at times infuriating them.

Luke is very cute and knows how to play the cute card. He is very sociable and likes being around adults and other children. Daniel is his best friend at home and he has a very close little girl friend called Becky.

He loves everything and everyone and is a pure bundle of joy.

Update 18th August 2010

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.

Update 17th June 2010

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.

However 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 XLP rays and scans. So Luke has just started on 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%! The bottom end of normal is 80% though.

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

Update 16th April 2010

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 and Sunday and then to be home early next week.

Update 15th April 2010

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. 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.

We have just heard though 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.

Update 17th February 2010

Luke is coming down on the steroids and if everything remains well we should reduce them again in a week’s 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.

Update 26th December 2009

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.

Update 22nd December 2009

Luke is now home after his mini ops and as you can imagine is a little sore.

For 48 hours the ‘output’ did dry up out of his wound and naturally we started to get quite excited, but it is back flowing through the wound now so my early Christmas present hasn’t quite arrived.

Update 19th December 2009

Luke came home yesterday afternoon. It has been yet another long haul but it is great to have him home – and with Christmas almost upon us it looks like we may all be under the same roof unlike the last two years! The best Christmas present for me is the change in ‘output’ suggesting the hole is healing on its own. A few days ago for every 10ml out of the wound there was just 1ml from his bottom. Yesterday that ratio changed to 1 to 1 and so far today there has been significantly more out of his bum!

Luke will be going back into Southampton General Hospital on Monday for an operation repositioning his central line and also the ‘cruelest cut of them all’. He will stay in overnight but we would expect him home on Tuesday.

We are all somewhat shattered and intend to have a quite time now.

Update 11th December 2009

It’s been an interesting day – actually potentially wonderful and also a little worrying.

The surgical team met this morning to discuss Luke and there was no one opinion on how to proceed. So the top man (who operated on Luke – saving his life this time last year and to remove the stoma) came to examine the wound. He was very surprised at what he saw. It would appear that the wound has formed itself into a stoma directing output out – and so it was decided that there would be no need for a second stoma! The view is to let this continue and it should naturally heal – so the surgery is off on Tuesday. This could take up to 3 months – but would be better than another stoma for a year and then having to go through this surgery again.

And this afternoon there was a clear slowing down of output but it remains that it is coming both out of the wound and through his bottom.

However Luke will be having surgery probably on Monday 21^st December to reposition his Hickman (central) line and for a circumcision – he has had too many catheters.

Wow.

But we are in a battle – and tonight I left him asleep in hospital with a temperature of 39 degrees – the first temp he has had since he was in. He was crest-fallen as we had planned to bring him home during the day tomorrow with the hospitals blessing. The most likely culprit is the wound.

In truth both Allison and I are a little ‘punch drunk’ by all this. We do feel we are in a battle and today has been a significant step forward – but we remain vigilant as we move ahead.

Update 10th December 2009

Luke remains well and we are getting roughly the same amount of output out of the wound and his bottom. At some point this PM he will be meeting players from Southampton FC on the ward – strangely he is not too bothered…

I pressed the consultant this morning to get a realistic probability of the hole now healing itself – we are around the 20% mark.

As I stated in the last update, they will almost certainly make a decision on Monday if there is no change to go ahead with a new stoma. It is likely that the surgery would be on Tuesday AM which would coincide with Nathan’s 16^th birthday and also the Christmas Concert on the ward that Luke has been rehearsing his violin so hard for.

A number of you have asked why they won’t go back in and try to patch up the hole. The reason is that it will be too mucky in there and the bowel too damaged after having his output flow over it for a week or so.

We really do need a miracle now.

Update 9th December 2009

Ho hum. We still have output coming from both his rear end and the through the wound – with no sign of the later abating.

There is real concern that the wound will get infected and they are well aware that this can’t go on for too much longer. So it looks like we will have a formal review next Monday and if the losses are still the same from the wound then they will have to put Luke through another 6 hour operation plus a new stoma. It would be at least a year before we could try for a reversal then. If the wound does get infected then this would mean operation would be almost immediately.

Luke remains very well and is currently in the school room making pizza.

We (once again) feel like we are living on a knife edge – we really are desperate to see this little hole heal. The impact of this failing is just too horrendous – it is a real limiting factor on Luke’s life. Plus the thoughts of another year of managing a stoma and not having a full nights sleep are just too awful.

Update 6th December 2009

Luke remains clinically well but the leak is still very much flowing. He is back on his favourite Paim Brown ward and so is very happy as he is well known and loved – plus he has the excellent school room to look forward to tomorrow.

The expectation is that at some point the leak will heal itself and all will be well. We did become aware today that Luke is on a very high dosage of steroids. The surgeons upped the amount at the time of his operation as ‘cover’ but he is still on this high dosage 11 days on. Steroids are very wonderful as they slow everything down – including healing… We are now very much on the case about this.

Update 5th December 2009

News is mixed.

The output through Luke’s wound increased last night but is still really a trickle.

But Luke remains very well and with it – no temperatures and there was output through the right place as well. The ‘free drainage’ from his stomach would also seem to have just about dried up and the excess water which was making his feet, hands and other places swell up is also much more under control.

So it does look like all the bits are kicking back in – BUT we still have this leak that could mean another operation – a new stoma – and back to square one.

Update 4th December 2009 (evening)

A mixed and actually rather worrying day. Around 2pm this afternoon Luke had a clear leak through his wound – showing that there is a leak in the bowel (probably where it was all sown up). If this becomes serious then we are looking at another 6 hour operation and a new stoma.

BUT since then there has been no more leaks (6 hours), he remains well and seems to be in a bit less pain after producing a huge ‘output’.

So we are all (parents and doctors) on a watch it and see over the next few days. If he becomes unwell or the leakage becomes much worse (takes us back to Nathan and his b-cell lymphoma almost 13 years ago) then they will quickly operate. Please pray.

Update 4th December 2009

The breakthrough! Yesterday afternoon Luke finally delivered the goods. They were two ‘starvation poos’ which are exactly the same as a new born baby would first produce. And this morning another formed delivery. Huge relief!

Luke is still in intense pain though. Indeed, it is probably the worst pain that any of the boys have experienced over the last 6 exhausting years. And there is little medicine that they can give as it comes in waves. Luke has realized that the morphine self administered pump doesn’t help and the only medicine which is called buscapan – but even this isn’t really touching it. He is exhausted as well from the constant waves of pain. We do expect this to fall off as his output increases but it really isn’t pleasant and can’t come quick enough.

Added to this he still has excessive fluids in the wrong place – although with a full does of Albiumin under way this should start to see this come back into line.

Thanks for praying – it makes the difference.

Update 2nd December 2009

The news on Luke is kind of mixed but moving forward – but not at the rate any of us would like.

His lungs seem much better and any ‘rattle’ has now disappeared and he is able to cough well and take on some simple physio. Also, we found out that the fluid retention was caused by him having low Albium which is currently being put right.

There were concerns on Monday that there was a ‘mass’ detected on an x-ray which could have indicated that there was a leak where the bowel was repaired. If this is the case then it could be life threatening and require immediate action – with the outcome being a new stoma. But as Luke remains clinically well this now seems highly unlikely – he would be a pretty ill young man now if this was the case. It is possibly that this mass is the fluid that the current procedures should put right.

And last night the doctors detected ‘bowel sounds’ which suggests that things are starting to work. Luke is getting the ‘call’ now quite regularly but isn’t yet able to push anything significant out. But these are signs that things are slowly awakening.

It is possible that the delay in Luke’s stomach and bowel kicking back in is because of the steroids that he is on to hold back the Graft vs. Host Disease (the main cause of the ruptured bowel a year ago). So it is very much a waiting game at the moment.

Luke is still in considerable discomfort but to show his positive nature, requested that his violin was taken in yesterday so he can rehearse for the Christmas party in main ward that looks after him. Not sure of what the other children will make of this.

We will keep you informed – please keep praying for (literally) a ‘break through’.

Update 30th November 2009

Well once again we face some interesting times.

The initial plan we had for Luke’s stoma reversal was that he should be home now – but he remains in Southampton General Hospital for the foreseeable future.

As I reported before, the procedure went well but things haven’t been so good over the last 48 hours.

At the moment Luke’s stomach and bowel are not working properly. We know that it can take some time for these to ‘kick back in’ but we obviously do need to see this. That being said, Luke has passed several ‘poos’ of mainly green bile so it does show that there is some activity. This action and also some pain from the wound mean that he is also now back on the morphine pump. This did enable him to get a good sleep last night.

Luke also has a partial collapsed lung which is also good news. We were aware that there was a risk of this and you may recall that this was one reason why they delayed 2 weeks ago. At the moment there is no sign of any lung infections and he is having a good spread of antibiotics. He is also doing some simple physio and this seems to be helping.

But he also has some water retention within his stomach which the doctors are concerned about. The initial reaction was that there might be a small hole in the bowel, but as he has pooed and is clinically quite well this is a bit of a mystery at the moment. Indeed Luke wanted to go to the school room this morning. We welcome you prayers.

Today marks the 6 year anniversary of the diagnosis of XLP. It is hard to think that it has been that long. And for the last 3 ‘anniversaries’ Luke has been in hospital: 2 years ago for his second BMT, a year ago with the ruptured bowel and today because of the stoma reversal. But we are still all very much here!

Update 26th November 2009

Yesterday’s operation to reverse Luke’s stoma went well – and he now has a normal looking belly!

Luke is very well and for those of you who are friends with him on Facebook, you will have seen that he wrote up an entry within 30 minutes of coming back to the ward! He is still in some pain and has a morphine pump which he can use as required. It is likely that he will be in hospital for a few more days.

Huge relief here!

Update 25th November 2009

Luke is now in hospital ready for his stoma reversal which should be late this afternoon. He has already been in to see the lung consultant who gave him the all clear (and was surprised that they didn’t go ahead two weeks ago!).

He is really looking forward to see the end of endless bags – but we know that this is still a serious operation and that it will take some time for ‘things’ to get back to normal. So please continue to pray for him.

Update 11th November 2009

Luke’s stoma reversal has been cancelled as he has a ’fruity cough’ with a concern that this could end up becoming a chest infection. It’s a shame that it took the medical team until after he had completed the bowel prep to discover this.

So he is rescheduled for two weeks from today and will be coming home with an antibiotic nebulizer which hopefully should do the trick. We will insist that they assess him before starting the bowel prep.

We are obviously all hugely disappointed.

Update 6th November 2009

It’s amazing to think that we first received the diagnosis of XLP almost 6 years ago. It’s been a couple of weeks of good news.

Luke will be going into Southampton General Hospital next Tuesday to start the prep for his stoma reversal next Wednesday PM. He is expected to be in hospital for a further 3 to 5 days – depending on how things go. He is very relaxed about this and is organizing his time in there. So after almost a year of sleepless nights (because of the need to empty his stoma bag) we can start to look forward to 7 hours sleep a night! Although we know that it will take some time for his ‘functions’ to kick in again. He is also almost off the overnight TPN (replaced with an overnight milk feed) but we will now hold back on totally stopping this until he is home after his op.

Update 9th October 2009

We saw the gastro consultant about Luke’s bowel first thing on Monday morning at Southampton General Hospital – and he said that as long as Luke could tolerate more overnight feed (Modulin) of up to 500mls, he would be happy to recommend that they reverse the stoma. On Thursday night we managed to get just under this amount into Luke overnight – with no increase in output! So the internal communication has now gone through the hospital to get a date to reverse Luke’s stoma… it really can’t come too quickly for us all.

Thanks to everyone who has sponsored us for the walk this Sunday; we have smashed through the initial target we set! All the money raised will be going into medical research into this awful condition – and a BIG THANK YOU to all who have supported us this far. For those of you who are early birds, I will be live on BBC Radio Solent giving an update on the boys.

Update 18th September 2009

Luke was ‘scoped’ yesterday at Southampton General and the consultant who undertook it was pleasantly surprised at how well the bowel has recovered.

Indeed we will be meeting him in 3 weeks time with the view to agreeing a date to have the colostomy reversed – he sees no reason why this shouldn’t be the case.

Whilst we appreciate that it will be a battle to work with Luke to recover ‘bowel control’ this is a great positive step forward.

Some of you who have seen Luke recently will be aware that he is now practically bald. Over the summer his beautiful curly brown hair started to come out, almost certainly as a result of the ‘end’ of skin GVHD (Graft vs. Host Disease). He has coped well with this and there are some signs of re-growth.

Update 19th August 2009

Luke recovered well last week after his replacement hickman line, so we happily set off on Sunday morning to drop them all at their ‘Over The Wall’ camp, allowing Allison and I to book 3 nights in a relatively local hotel.

Monday morning we had a call from the camp to say that Luke’s TPN pump had given up the ghost so we had to drive home to pick up the replacement and zoom back down there again.

Tuesday morning, as we were just sitting on the beach, we had a call to say that Nathan was in Dorchester Hospital with a suspected broken neck after a rather exuberant ‘rugby’ tackle by one of his fellow campers. So we zoomed down to Dorchester and he was of course fine and then dropped back off at camp.

Wednesday morning, we had another call to say that Luke was exhausted and we would need to pick him up. He is now back home and fast asleep.

So (assuming we don’t get a call on Thursday) the car can be put into remote for the pick up trip for the rest of the boys on Friday.

Update 10th August 2009

As expected, Luke will be going into Southampton General Hospital for a replacement Hickman line. Come Saturday morning he was in a lot of pain as the TPN went in – and despite initial assurances from the weekend doctor that it was probably an infection, our initial suspicions were proven right.

This seems that the scheduled scope that was planned for this Thursday PM will now be postponed until early September – as it is too early after the anesthetic which he will have tomorrow. At least the doctor gave us the reassurance today that it is a matter of time before they reverse the colonoscopy (stoma)… role on that day.

Tomorrow they will also do ‘wash out’ on his left lung to get rid of the last bit of infection which may be still lurking, but his lungs have definitely got much better over the last few weeks.

We are still hoping that Luke will be joining his brothers at a special camp next Sunday for a few days, which will give Allison and I some respite and allow us to take a deep breath again…

Update 27th July 2009

Luke now has a scope penciled in for just over two weeks time, which should give us a better idea if he is ready to have his bowel put back in him. There are still some strange things going on – for example he doesn’t appear to tolerate ‘dioralyte’ (which he is expected to have on the two nights a week when he is not on TPN) and he started a course of oral antibiotics last Thursday (because of a lung infection) but we had to stop them because he was having severe stomach pain. Yet he is digesting the little food he takes on and absorbing the rest of his meds.

Update 8th July 2009

Luke is currently off school because there is a suspected Swine Flu case in his class. The UK Governments advice has been not to panic as the only fatalities have been amongst people with ‘underlying medical conditions’. I guess this includes being immune suppressed. If he is going to manifest any symptoms then that should be 2 to 5 days after exposure which would mean he would be ‘all clear’ by Saturday. At this time he is very well and the output is well under control. So, we are taking the governments advice. Luke was gutted to miss school and particularly at not being able to do his violin solo at the schools concert tomorrow night.

Update 16th June 2009

Slow progress on Luke. He is still coming down on the steroids (25mls now every 48 hours) which is positive news and the ‘flow’ seems to be under control. Amazingly it has been over 6 months since the trauma of the ruptured bowel but still no idea when the stoma will be ‘put away’.

Due to Luke being on steroids now for such a long time, he will start some IV ‘bone strengthening’ medicine next Monday. Strangely, it is over 3 hours for 3 days each month. Luke has been complaining of lower back pain and it is almost certainly a result of the steroids. This is a medicine called Permidronate. Joshua has also had this and it did help.

We also found out that Luke has Adenovirus on board and as it is at such a level (212,000) they will start him this Thursday on an IV drug to help him fight this. Bizarrely, this virus has probably come on because they are lowering his steroids. Luke remains very well in himself though.

So one step forward and maybe half a step backwards…

Update 17th May 2009

As it’s been some time since I last sent an update out, you have probably guessed that all is pretty well in our household.

Both Luke and Joshua are on extremely slow steroid tales (but critically they are coming down) and this is going well.

Luke still has the stoma but the output has settled. We have been able to reduce his overnight TPN (and we actually get Saturday night off!). He loves being back at school and isn’t showing any signs of being too tired at the end of the school day. He is absolutely Mr. enthusiastic and last week joined the school orchestra in second violins.

Joshua is doing well at college and seems to want to stay on for another two years. Despite only making about 60% of lessons due to his heavy commitment to various hospitals (St Thomas in London for two days every two weeks, GOSH for a check up every 6 weeks and Southampton General for Immugloblins every 3 weeks, plus eye clinic visits etc.) he actually got 100% in his hardware course – amazing!

Nathan and Daniel are well and Nathan in particular is growing at an amazing rate. And Mum and Dad can now occasionally get out and start to restore a social life!

Update 9th March 2009

Another historic day as Luke went back to school (again!). It was hard to express just how excited he was about this and he was desperate to get back. Initially he will only be going mornings as he builds up his strength again. Hi ‘output’ is still up and down and we are still some way from having an idea when the stoma can be reversed.

An emotional and rather wonderful day.

Update 2nd March 2009

Yesterday was a special day. Daniel became a teenager. Whilst that is special in its own right, it is extra special for an XLP affected boy – as without a bone marrow transplant few make it into their teen years. He is doing really well.

Luke has now been home for just over two weeks and is loving life back home. We took him in to see the gastro consultant on Friday morning and he was delighted with the progress. It was quite stark in that either with new treatments they work or they don’t and they have clearly worked with Luke. So we were even able to reduce the TPN by a quarter although for our own sanity it is still going over 12 hours. Luke bristles with life and fun and we are very proud of him.

Update 13th February 2009

Luke finally came home yesterday – he was elated as we all were.

It has been another difficult 2 months for us, but we are just grateful that he made it back home. He has been incredibly brave and up beat through all this – a true inspiration.

Along with Luke arrived mass of extra equipment including a NHS provided fridge to keep his TPN in! We are also being supported morning and evening by nursing staff to ensure that we set the TPN up correctly – not sure if we get a certificate at the end…

Update 8th February 2009

Luke should come home on Tuesday after 2 months in Southampton General Hospital. He has really been very well for all of this year and so there seems to be no reason for him to stay in.

He will however becoming home on ‘home TPN’ so both Allison and myself have now acquired another IV skill – and the NHS will be dropping off a fridge on Monday as this is needed before the 2 week supply arrives on Tuesday! The TPN will run for 12 hours over night.

He will also be coming home with the stoma and at the moment there are no plans to reverse it. His output continues at around a litre a day (some days better than others) but they seem happy with this as a level and have told us it will just take time (although no one is saying how long) for everything to come back together.

Luke is well in himself and has taken all this in his stride. He has now recovered all of his weight (in fact he is now the heaviest he has ever been) and all his bloods and other measures are in the ‘normal’ range and stable.

Allison and myself are really looking forward to Luke coming home but there is a little trepidation – plus I have volunteered to set my alarm for 3am every morning to give him one of his drugs… but we really do want us all back together again.

Update 28th January 2009

Luke is doing well – although his ‘output’ continues to mystify us all. Earlier this week there was a clear downward trend and we were getting quite excited and now, for some unknown reason, in the last 24 hours it doubled in size again and we are nearly back to where we were at the start – frustrating would be a huge understatement! At least colour and texture are better (sorry – we spend a lot of time looking at this).

The plan now is for Luke to come home on TPN (an IV ‘food’ which is given over 12 hours and delivers everything he needs to thrive.) Allison and myself will be trained in setting this up next Wednesday and then assuming (and it is a big assuming!) that the output settles down he will come home as soon as arrangements can be made for a new fridge and the TPN to be delivered. But we are totally dependant on seeing the output settle and until this happens we are ‘stuck’ in no-man’s-land.

We have been told that it will be a significant amount of time before things return to normal and he can have the stoma reversed. No one wants to have a guess at significant but we are assuming months rather than week… but things can change quickly.

I mentioned that Luke is actually doing well. All his bloods and other measures are all slap bang in the ‘normal’ range and he toddles off to the hospitals school room at 9.30 and 1.30 every day. He is ‘nibbling’ on some high fibre foods again to help him stem the losses but has no real appetite and is full of mischief (although after two months in hospital he really does want to get home). We enjoy a kick around in the ward corridor early evenings and he has developed a liking to Burger King onion rings (for some reason that escapes me there is a Burger King take away in the hospital). He definitely enjoyed his birthday at home (at least for part of the day) and thanks to everyone who sent him a card.

Please continue to pray that we will see a break through in this – Allison and myself are naturally shattered after alternating spending time with him in hospital overnight plus taking care of our other wonderful sons. You know you have been in hospital too long when the cashiers in the hospital restaurant assume we are staff!

Update 18th January 2009

Luke is doing well but is still in Southampton General (we are rapidly approaching the 2 month mark) but is allowed home for around 7 hours on Saturdays and Sundays which is a great boost.

The EBV load has disappeared after just one treatment of the IV medicine (Rituximab) which is great news. Plus he has put back on all the weight he lost – when he was admitted his weight went down quickly to 19.5kg but is now up to 23.3kg – again showing that he is absorbing some of the milk feed he has.

We are still however struggling to get his gut working properly again and are still seeing high stool losses on some days. This is really what is keeping Luke in now and what we (desperately) need to see the end of.

Luke remains extremely well and positive despite yet another extended time in hospital and is very much looking forward to his 9^th birthday a week today.

Update 6th January 2009

We are still in somewhat of a waiting situation with Luke, as each day the diarrhea continues at somewhere between 1 and 2 litres a day. There are encouraging ‘blips’ where he seems to dry up but then the stoma bag fills up again. At least his U+E’s seem to be OK although he is having replacement fluid for his losses which includes a good whack of potassium.

Late last week we were also told that the old enemy EBV was back on board. They have examined Luke and yesterday he had an x-ray and ultrasound which showed no raised lymph nodes and we should have the virus load numbers back today – depending on whether the numbers are up or down will determine the course of action.

Through this Luke has remained positive and just got on with it. He has been a real testament of how just to get on with life when everything around seems dark and gloomy. He is much more mobile now and his weight is slightly up again (although well short of where he was before this episode started – he has recovered about 50% of the weight loss) and was able to come home for part of the day last weekend. He was very excited about the wards school starting again yesterday and was the only child there at the stroke of 9.30am. And as the subject this weekend is ‘penguins’ he is very much in his element.

We really do need to see this diarrhea stop now though so that we know that the bowel is viable – this is our New Year prayer.

Update 28th December 2008

Well, Luke did make it home on Christmas Day, and Boxing Day and yesterday… and it was a huge lift for us all. He is very well in himself but unfortunately we have still not seen the diarrhea settle back down – he is loosing about a litre a day. We have now stopped the modulin feed with the hope that his gut may settle and then allow us to recommence the feed. He has gained a little weight but he is still all skin and bones.

Tomorrow Luke will receive the first infusion of the drug that should allow us to both reduce the high dosage of steroids that he is currently on (they will be halved tomorrow) and which should settle the gut further. Apparently it has an 85% success rate and we should see results towards the end of next week – we’ll keep you informed.

To add to our fun and games, both Nathan and Daniel have caught a bug or some food poisoning and were ‘big sick’ last night – although Nathan claims to be much better now (Daniel has gone to bed). I am due to take Joshua up for his ECP treatment at St Thomas in London tomorrow so it will once again be a difficult juggling act for us all.

So our New Years prayers are really simple – to see Luke through this difficult period with his gut and restored back to where he was and specifically GVHD free and to continue to see the huge improvements with Joshua that we have seen in his mobility and skin in the last two months. Amen.

Our blessings to you all.

Update 24th December 2008

Luke is doing pretty well. About 4 days ago we started to see ‘normal output’ from his stoma bag, but unfortunately we are back in diarrhea city due to an oral antibiotic which was started 2 days ago and has now been stopped! So hopefully in the next 24 hours we should see things go back to where they were 2 days ago… and Luke has started to put on some weight and is more mobile now.

Tomorrow, if all goes to plan, Luke should be able to come home at 10am and then be back in the hospital for 6pm (in time for Dr Who on the BBC!). As you can imagine he is pretty excited about this as we all are. There will be a juggling of presents and I think lunch will be crackers and cheese (never did like turkey much) but hopefully if only for a few short hours we will be together.

So it does look like Luke is responding to the high dosage steroid treatment he is on. The plan now is to start reducing this next Monday and introduce a new IV drug that should also help what is going on in his bowel. This is pretty critical as Luke can’t stay on the high dosage steroids for too long, plus we don’t want to see any further inflammation.

Seasons greetings to you all. We pray that this time will be a time of peace and joy as we remember the reason for the season.

Update 17th December 2008

Luke is much better and had a really good day yesterday. He woke ‘full of beans’ and launched back into making Christmas decorations. He was quite literally blown away by the parcels and letters that arrived for him yesterday from our friends at Post Pals – he loved the balloons in particular. Thanks guys. He even made it to the Piam Brown Christmas Carol concert (the ward he is on) and he took his first few steps yesterday as well. He should also commence his milk feeds today.

We have been told that we will still have the possibility of a bleed in his bowel for the next 10 days or so – please keep on praying that his bowel will fully heal and that he will go from strength to strength.

Update 14th December 2008

Yesterday was another real fight for Luke. He had a great morning and early afternoon making Christmas cards and decorations when we noticed that the output in his stoma bag had turned red. Shortly afterwards he passed a huge blood clot and several more followed – it was clear that he was bleeding into his bowel.

The team at Southampton came together and took him down to see if they could ‘clip’ the offending artery. By this stage he had lost approximately 50% of his body’s blood. But as he was going into theatre we noticed that he was now only passing dried blood and stool. And sure enough they were unable to find the cause – the offending artery had clotted. Whether this was due to God, the drugs they gave him, or his own platelets, we will never (in this life) know – we are just grateful that it did.

It was agreed that Luke would stay the night sedated in intensive care to give him rest so we duly left him and went home (we live a 15 minute drive from the hospital). At just gone 1.30 am we had a call to say that all was well but Luke would like to speak to his Mummy! Our brave little man had fought the respirator and won and then started to negotiate with Allison as to when she would come in.

I cannot describe how good and positive Luke has been through all this. There have been lots of tears of pain but never a question as to why? God has indeed put a fighter in him.

Update 12th December 2008

This afternoon I spoke to the consultant who is looking after Luke overall and he is now fully convinced that the underlying cause is the dreaded Graft vs. Host Disease which has so plagued the boys post their bone marrow transplants.

Positively, a course of intravenous steroids should get on top of it and start the process of getting Luke back on the road to recovery.

This will mean that Luke will be hospitalized for about another four and a half weeks – meaning that we will have our second successive Christmas courtesy of the National Health Service (and indeed the last 3 out of 4). Ironically this Monday we will feature in a leading UK women’s magazine saying how much we are looking forward to Christmas at home.

Luke finally had his Hickman line put in yesterday; it was held up by intermittent temperatures. He is still very weak (he can hardly walk), and still prone to lots of sleep. However, his old bubbly personality continues to shine through and he is spending much of his waking hours making endless Christmas decorations.

Thanks for your support, it means the world!

Update 8th December 2008

Post can now be sent directly to the hospital for Luke:

Luke Hartley,

Piam Brown Ward,

Tremona Road,

Southampton,

Hampshire,

SO16 6YD

Update 7th December 2008

It has been a pretty good day for Luke. All of his counts – blood and u’s and e’s are now back in normal range and there is an overall sense that things are ‘moving in the right direction’. Yesterday his platelets were down to just 25 but this morning 122 – suggesting that his body is coming back to normal after the trauma of the last week. His stomach is soft to the touch and certainly much better on a daily basis – and (sorry for the detail) the colour of the losses from his stoma bag has also started to change from green towards brown suggesting that he is absorbing his Modullin (in hospital you spend a lot of time looking at this).

The only downer is that for the last two nights he has had some spikes in his temperature although he has a good spread of antibiotics being onboard.

Luke is now negative of any detected viruses, which hopefully means that tomorrow (Monday) he can have his Hickman line put in. It will depend on there being no show of the temperature again though.

So it is pretty good news. Luke is certainly in a much better place than a week ago. But he still has a long road back to recovery – he is still very weak, reliant on TPN and is now simply ‘skin and bones’.

Please continue to hold him up in prayer and the rest of us – we are pretty exhausted.

Update 5th December 2008

Unfortunately Luke didn’t have a new Hickman line yesterday as they detected a ‘skin bug’ which meant that the new line could quickly become infected. However as the ‘temporary neck line’ was only expected to be in for 7 days they are putting a second replacement neck line in about now. The plan is to then replace this with a Hickman line probably on Monday… Luke was not very happy about this as the neck lines definitely restrict movement.

Aside from this he appears to be doing very well. There have been a steady stream of different doctors and nurses visiting him to check various things out and he has a smile and cheeky comment for them all. He seems to be tolerating the Modullin (milk type) feed well which is encouraging. Unfortunately the pleasure of being with him has rested on Allison as I am just starting to recover from one of the many flu type bugs that are around at the moment. Hopefully I will be able to visit my brave boy over the weekend.

Please continue to pray for Luke – still a long way to go.

Update 3rd December 2008

It is all good news today with Luke.

His bowel and gut is now working and this seems to have resolved the issue with his potassium levels. Also, much to our amazement, they have switched some of his drugs to oral and are starting him on a small milk type feed (he is on TPN at the moment – a special bag of minerals and fats that goes into his blood and gives him all he needs in terms of food).

He is out of isolation as he seems now to be bug free and so hopefully tomorrow morning we will get him down to the play and school room. He is still very frail and so he also has regular visits from a physiotherapist.

Allison has been trained in the use of the stoma bag and I have this joy tomorrow.

Tomorrow PM he will however be going down to theatre for a replacement Hickman line. Last week we knew that they had only put a temporary line in (his neck) as the idea was to have it located as far away from the ‘stuff’ coming out of his perforated bowel as possible. The line works well in terms of delivering his IV medicines, pain relief and TPN, but doesn’t bleed back which means that they have to prick him where ever they can to get blood to test. As he is doing so well they have decided to move this forward – it was planned for early next week.

So we are ‘getting there’ but it still has been a shocking week and not one we will ever forget.

Please continue to pray for Luke.

Update 2nd December 2008

Luke spent last night in intensive care to monitor his potassium levels but has returned to the Piam Brown ward this morning. They were concerned that overnight on PB they could not ensure they were on top of this due to staffing. The encouraging news is that these levels are now within normal range and he is only receiving ‘normal’ potassium top-ups. So we expect Luke to be pretty shattered today as ICU is very busy and bright over night.

We are still waiting for the gut to kick back in although the ‘output’ coming into his new stoma bag is changing consistency positively – but please continue to pray for this.

Update 1st December 2008

Luke had a settled night with little pain – although he was up weeing every hour which meant that both Dad and son were exhausted this morning.

Positive news is that there are no signs of infection.

Less positive is that his potassium levels have plummeted which could be very serious indeed. They are now giving him regular infusions of potassium and closely looking at his levels. It seems likely that the IV steroid that they are giving him is responsible for this.

And so far he has no ‘bowel sounds’ which suggests that the bowel is as yet not back working. This could be due to the low levels of potassium.

So we once again face some significant issues… we welcome your prayers.

Update 30th November 2008

Luke is doing well and had a pretty settled night. He managed to successfully pull his catheter out last night (which should not now be replaced) and also there were signs that the bowel is starting to work again as the colostomy bag had to be fitted. He has not spiked any temperatures but is desperate for a drink of water and some ice – we are only able to give him a little water on a sponge.

He is still very weak and sleepy and did start to have some pains last night but was able to push the button on his morphine pump to give himself a dose.

Thanks for standing with us on this. Later this morning I will take his brothers in to see him and then we will start the alternate days of Mum and Dad staying with him.

Today marks the fifth anniversary of the original diagnosis of XLP. Against the odds we are all still here and very much fighting and we know that through encouraging more people to become bone marrow donors and establishing the XLP Research Trust to help other families that we are making a real difference to others in similar dire circumstances.

Thanks for walking through the Valley with us.

Update 29th November 2008

We’re pleased to report that Luke is out of Intensive Care and back on Piam Brown Ward at Southampton General. He is doing very well but is still tired and has yet to feel the pain from the surgery. All the doctors are really pleased at the moment…

The suspicion now is that this is a fungal infection with an underlying bout of Graft vs. Host Disease added in. Luke has a steady regime of antibiotics being pumped through him.

Please continue to pray specifically that he will be infection free – the next 24 hours are crucial.

Update 28th November (evening)

Luke is in Intensive Care after a successful 3 and a half hour operation. They were not able to repair the hole in the bowel but have fitted a temporary colostomy bag which they expect him to have for 6 weeks. He is sedated and all the signs so far are positive.

The next 48 hours are pretty crucial as there is a risk of infection – and whilst Luke is a year post transplant he is still relatively immune suppressed – so please pray about this specifically as he has had ‘poo leakage’ from the hole in his bowel.

Update 28th November 2008 (afternoon)

Luke is due to go for surgery within the next couple of hours to repair his bowel.

Please pray.

Update 28th November 2008

Luke went into GOSH for his scope on Wednesday which showed very acute inflammation in his bowel. The consultant was very surprised about this and it is unclear what is causing it. By last night Luke was unable to hold down any water of milk feed so we took him into Southampton General Hospital this morning. It would appear that he now has a perforated bowel possibly brought on by the scope. Unfortunately Luke does not have a line in for medicines and has few viable veins, but they were able to get a cannula in this morning. However as he has deteriorated so rapidly they are not happy to anesthetize him to put a line in – so this is currently all the access they have. Not good.

He is starting a course of antibiotics which they hope over the next few days will perk him up again and start to sort out this problem, if not then it will be surgery which is just a new dimension.

This is a huge shock considering just how well he had been doing – this Sunday we will reach the 5 year mile stone of being diagnosed with XLP and we had hoped to be over the very worse.

Update 22nd November 2008

Luke will be going into Great Ormond Street Hospital on Tuesday for a scope on the Wednesday. He has been having stomach pains now for about 2 months along with diarrhea and it is either an infection or more likely a bout of Graft Vs. Hast Disease (GVHD) but because this has been going on for so long he has lost weight and he is now pretty exhausted. Fortunately, he is still absorbing food (although he has little appetitive) but we are able to load him up with Modulin feed and diarolite through his PEG. He has been off school for the last two weeks now. But the hope is that by Friday we should know what is going on and the treatment can start – it is just such a shame that it has taken so long. Luke is now just over a year post his bone marrow transplant. We welcome prayer support for this – a scope is not pleasant.

Update 12th October 2008

On Saturday morning Luke was in Southampton General Hospital having a cyst on his ‘derrière’ burst and drained. We don’t believe that this is directly related to XLP, his bone marrow transplant or anything post – just sheer bad luck! Luke was back home last night but was still sore.

Today we as a family participated in ‘Walking The Test Way’ both to celebrate having four sons come through successful bone marrow transplants and to raise funds to support the work of the charity. You could choose from 6 walks from 2.5 miles through to 26 miles along the beautiful countryside alongside the River Test. Unfortunately only three of us completed the walk and Luke was gutted that he missed the walk – as he has worked really hard filling up his sponsorship.

Update 2nd September 2008

Luke has been very well since coming home. He still has occasional flares of skin GVHD but is doing really well. Indeed, this weekend was kind of historic as I took him to Cardiff Bay to see the theatre production of Chitty Chitty Bang Bang. Last year we were all special guests at the Mayflower Theatre in Southampton for one of their nights and they also collected a large sum of money for the charity we run. But Luke was between transplants and didn’t have the immunity to allow him to go. So I made a promise that when he was well we would take him to see the production. Shortly after coming home in January Luke asked me where ‘Kuala Lumpur is?’ – he is using the internet well now and had tracked down where the theatre company had moved to. Fortunately the show has just finished a stint in Wales. It was wonderful and kind of historic that after everything he has been through that we should make it.

Update 24th July 2008

All is well in our household and with summer now upon us things are definitely looking up.

Luke has loved going back to school and was probably the only pupil disappointed that the school broke up for the summer holidays today! Although the initial plan was for him to go in for mornings only he actually has done full days since a week last Monday. His bloods and other results are all good.

Joshua is also doing well although driving us all slightly insane trying to find a summer job! He started to decrease the steroids today and the positive news is that his coughing has all but gone. He will be reviewed in clinic a week today.

Both Nathan and Daniel are doing well and Allison and Dave are actually sending some time together (although it will be some time before we can leave them for any period).

Update 14th June 2008

Luke came home today.

He is now very well with no sign of temperatures and is gradually starting to eat solid food again. It has been a difficult week though. On Tuesday Luke had two seizures, the second was extremely frightening and there were about a dozen doctors and nurses in his isolation room at one stage. They stabilized Luke over about 30 minutes (although I lost all sense of time) and then put him into a deep sleep. It appears that Luke has suffered from ‘Reversible Posterior Leucoencephalopathy Syndrome’ which is induced by one of the key immune suppression drugs – cyclosporine. Indeed Nathan also had this during his BMT.

The key is the first word of the syndrome – Reversible – and indeed by Wednesday morning Luke was out with me kicking a small football up and down the corridor. After an MRI and CT scan, plus a rather long and messy ECG, they have concluded that it has resolved itself. It was incredibly frightening to see Luke become so unresponsive and to see so many people rush around him. We are just grateful that he has recovered so well and so quickly and we are amazed that he is now home.

However even this was over shadowed by the loss of a very dear friend this week. We are still in a state of shock that this great man who has been such a great support to us over the years is no longer on this Earth. At least we have the comfort that now he is in Glory. Please pray for David’s family for peace at this time.

Please continue to pray for the boys for fullness of health. We take Joshua back to GOSH on Monday for a lung biopsy to see if he has some GVHD there – his battle is far from over.

Update 7th June 2008

Things have moved faster with Luke than expected. After his first dose of steroids at around 6pm on Friday he has not had a temperature! Indeed, Allison thought that he was actually cold today. Great news!

Update 6th June 2008

It has been another ‘rollercoaster’ week for Luke as he has continued to have regular soaring temperatures and at times go through extreme pain (thank you CT scan team at GOSH!) to find out what’s going on…

The conclusion is, after discounting just about every type of infection and fungus known to man, that Luke has GVHD (Graft vs. Host Disease) which actually isn’t a surprise.

So the plan is to start him on a course of steroids (hopefully tonight) which should bring the temperatures down quickly – within the first 48 hours. Assuming that this happens, the consultant suggested that he should be home ‘soonish’.

It’s been another long haul and Allison and myself are both shattered in every way possible.

Update 1st June 2008

It’s now been two months to the day that Luke went into hospital initially with rotavirus. He is still regularly spiking temperatures but has now gone through the full suite of antibiotics and last Friday he was ‘scoped’ to see if there was any sign of GVHD (Graft Vs. Host Disease) going on in his gut and stomach. The initial comment back was that ‘it wasn’t obvious’ but we should get the first results back on the biopsies taken tomorrow. If it is GVHD we would expect a short course of steroids to sort it out!

Otherwise Luke is well, tolerating his milk feed well and when the temps are not soaring, back to normal. However two months of being laid in bed is far from ideal and we know that when he is home we will (again) need to work on his fitness.

So we are in a strange, horrid, holding pattern that we need to see broken as soon as possible – for all of our sanities.

Update 26th May 2008

Thanks for all the encouraging messages.

Some more positive news on Luke… As of his tests yesterday, both the EBV and Adenovirus results show counts of zero! We are very grateful to the makers of Rituximab which has wiped out his B cells where the EBV live. Earlier this week we were sweating on a CT and ultrasound scan to see if Luke has lymproliferative disease (i.e. lymphoma) but he is now clear on this. He is now much better in himself and tolerating the milk feed well.

The temperature spikes persist though and we are (slowly) going through tests and treatment to try and get on top of this. He has just completed a course of antibiotics and will tomorrow start a course of treatment for anti fungal and then if that doesn’t have any impact, at some point next week they may consider ‘scoping’ him for GVHD (Graft vs. Host Disease) and then maybe looking at EBV in other cells other than B cells – it just seems to go on and on and on. However if they can get on top of these temperatures he will be on his way home.

Amazingly Luke has now been in hospital (Southampton now GOSH) since the 1^st April. Adding in the two bone marrow transplants he has been in hospital pretty much half of the last year – and of course has now been away from school for over a year as well.

But Luke (like the rest of us) is pretty resilient and when the temperatures are not souring he is back to his old chirpy cheeky self.

I’m often asked by kind enquiring folk what impact XLP has had on the family beyond the obvious of going through a bone marrow transplant and the associated aftermath of this treatment. Well there have been some positives in that we have been able to encourage more folks to become bone marrow donors as well as the medical research that the charity we formed has started into this awful condition. As a family it has drawn us together and given us unbreakable bonds – despite the relative lack of time we have been able to spend together.

But there are significant disadvantages as well… The time apart has been hard on us all, the kids have had to put up with dads cooking and it has been hard to look beyond what is happening in hospital on that day – when the boys at home need help and attention as well, particularly for Joshua with his GCSE’s. Personally, I long to have an adult conversation that is not centered just on the boys and to spend a day when the consequences of this dreadful condition are not before me. It would be nice to be free to go back to work full time, and to be able to commit to a diary and not have to cancel and leave others in the lurch when a medical emergency raises its head. And it would be great to have a relaxing holiday together… and maybe spend some quality time with my wife.

All this makes me even more determined to help ensure that in the future others don’t need to go through this valley experience and to ensure that the work of the XLP Research Trust is successful (www.xlpresearchtrust.org).

Update 14th May 2008

I have much better news on Luke. Following the first dose of Rituximab last Friday we saw a sharp drop in his temperatures over the weekend – on Saturday Luke commented to me that he wasn’t shivering anymore. We also found out last night that his EBV load has dropped from over 2 million to 117,000. This is still high but a very significant drop (although it is logarithmic rather than linear for any statisticians out there!) The consultant was very pleased at ward round today and the plan is for Luke to have his second dose of Rituximab this Friday.

Luke is still very tired and jaded and is still having regular temps heading into the late 38’s – but these are controlled well by IV paracetamol. He has had 5 days on nothing but fluid and is now on TPN (an IV food that gives him all his calories and vitamins he needs) but is still having regular but reduced stomach pains. The team at GOSH believe that this will subside as the lymph nodes shrink.

Other good news is that the second virus which was detected, Adenovirus, has also come down significantly from last week – 13,000 to 3,000. His own immune system is handling this and it bears well for the future.

So the news is good and positive – although still some way to go.

We ‘soldier on’ in our household trying to keep everything ‘normal’ back here whilst Luke is back up in London. Joshua starts his GCSE’s next week and we are so pleased at how well he has done so far.

Thanks for your support and love.

Update 6th May 2008

Update 14th April 2008

Luke has now been in Southampton General Hospital with rotavirus for just over two weeks. He caught it from his brothers who ironically we think picked it up at the same hospital (Daniel was in for two nights with an eye infection just before Easter). At the moment there is no clear end to the rotavirus – his ‘output’ is still very significant each day. Last week was a very worrying time when he appeared to have an autoimmune reaction to either one of the drugs or the virus itself, leaving him with low blood pressure, high temperatures (40 degrees at 1pm on Saturday) and a fast heart rate as well as fluid retention around his hands, feet and face. Yet on Saturday PM this all wonderfully resolved itself leaving medics at Southampton and GOSH a bit flummoxed. He still has some puffiness on the feet and face but nothing compared to what it has been.

He has been his usual brave self through all this and a model patient but he was quite quiet this morning – too long in a small isolation room.

Update 1st March 2008

Luke reached D+100 today. This means that he is 100 days post his second bone marrow transplant and the ‘rule of thumb’ is that if you reach this date with nothing major going on, then all should be well moving forward. Luke is well despite a short stay in Southampton General Hospital last week. It will still be a couple of months until Luke’s immune system is sufficiently recovered that he can return to school, but this is a real milestone.

Update 22nd January 2008

Luke is home!

He actually came home on Sunday and has started to settle in right away. He is very well and is enjoying being out of hospital and having the relative freedom of the house.

I don’t think that it has yet sunk in that we have now had all 4 boys go through successful bone marrow transplants. It seemed such a long journey through this valley that the end seemed to be just so far in the future. Whilst Luke still has a way to go to get back to full health – being able to go back to school is very much the end game – it is really wonderful news.

We have been so grateful for the wonderful support we have had – it has made such a difference to us.

Update 13th January 2008

Luke continues to do well and if all there are no hick ups then he should be home within the next 10 days or so.

I was able to take Luke out of the hospital briefly today which was yet another boost for him. Luke’s appetite is starting to return and he is also drinking more. He continues to be extremely cheerful and full of mischief.

Thanks again for your prayerful support we know that this makes the difference.

Update 3rd January 2008

Luke continues to show that his immune system is coming on well – blood counts are good and getting stronger. He still has this pesky adenovirus around although it is at low levels in his blood (almost can’t track it) but is in his stools, urine and the snot in his nose. So the diarrhoea is continuing and this is stopping him get back on a milk feed or solids. He is allowed off the ward now but we have to choose carefully the times to venture out so that there are not many people around (shame hospitals are full of ill people!) Our record for ‘Spot the Christmas Tree’ is 18. He is full of life and joy – really amazing. And the week before Christmas he had a special visitor – Sir Paul McCartney live in Luke’s room! In truth Luke didn’t know who he was but he was wonderful with Luke spending about 20 minutes with him.

Allison unfortunately has broken two small bones in her foot on Christmas morning whilst preparing to travel up to London with the boys. She is now in a plaster caste and is expected to be pretty immobile for the next 4 weeks or so. This means that David will be staying with Luke at GOSH full time with Allison based back home – not what was planned!

We spent Christmas together on Fox Ward at GOSH and it was really wonderful and a true lift for all of us – especially Luke being able to spend almost three days with his brothers. The staff on Fox Ward were amazing, putting on a wonderful buffet lunch and joining in with the fun.

As we approach the New Year we see real hope that we should be moving away from spending long times in hospital. Thanks for your continued support – it means the world.

Update 14th December 2007

The news we so wanted – Luke has 100% donor engraftment which means that the immune system which is emerging is all from the donor – and his old, faulty, immune system is being replaced. This is wonderful news and we are just so pleased and relieved. Luke’s blood counts continue to get better each day, again showing that the new bone marrow is doing its job.

Luke is still eating very little but they have just started to reintroduce a ‘milk type’ feed. He remains in wonderful spirits and wants to be played with or entertained every waking hour. Our favorite game is ‘throw teddy around the room’ which is pretty much what it says. It does give him a work out as such and has improved my catch skills considerably.

Luke still has adenovirus onboard at very low levels but the huge diarrhea we were seeing a week or so ago is slowly, daily, on the way down.

We know that it is still early days but all the signs are good.

Update 9th December 2007

Luke is doing well. It is now clear that the adult stem cells have engrafted and his immunity is slowly returning. His white cell count, hb and platelets are all up and the doctors are very pleased with him. He has shown a clear graft vs. host rash which the doctors wanted to see but now with a steroid dose this has faded.

He does however have adenovirus in his stools (but fortunately not in his blood) which means that he is having regular bouts of extreme diarrhea which is far from pleasant and is giving lots of sleepless nights – particularly for Allison and myself – Luke is able to fall back to sleep immediately.

It is very early days but very encouraging!

Update 23rd November 2007

Yesterday (22^nd) was Luke’s bone marrow transplant day. The adult stem cell dose was very high (over twice what was needed) and we will be eternally grateful to the wonderful unknown stranger who has given Luke the potential gift of life. The transfusion (it is NOT an operation!) of the adult stem cells took about 4 hours.

Up to the time of the transplant Luke had been very well indeed with only minor sickness. However because of the volume of stem cells, and the fact that his wonderful donor is a different blood type, he did have a reaction to the transplant (temperatures and vomit) and it is only about 24 hours later that he is starting to get back to his old chirpy self.

So now the wait starts. We would hope to see the first white cells come through in about two week’s time…

Thanks to everyone who has written to Luke so far. He does enjoy getting post so please do drop him a note. His address is:

Luke Hartley

Fox Ward

Great Ormond Street Hospital

Great Ormond Street

London

WC1N 3JH

Update 8th November 2007

Well it’s just a few short days before Luke goes back up to Great Ormond Street Hospital for his second bone marrow transplant. We have been fortunate to find a new donor and we are reverting back to the same BMT regime that worked so successfully for Joshua, Nathan and Daniel.

Luke remains very well. I’m not sure how many times I have written that but he really has rebounded well since coming home. Indeed in the four months that he has been back from hospital he has not had a temperature or any sign of illness. His blood counts have gradually recovered with his old immune system kicking back in. He is actually positive about going back in – looking forward to see the band of folks that looked after him so well and particularly the clown doctors that visit the ward on Tuesday PM (the highlight of the week). Luke really is an exceptional young man.

We are obviously extremely grateful to this new wonderful person who has agreed to give adult stem cells to help save Luke’s life. And just this morning in my inbox was a great email from the UK’s Anthony Nolan Trust bone marrow registry giving a link to a web site that they have developed to help recruit young men aged 18 to 25. If you are in that category or you know men that are then please forward on. The web URL is www.ilovemarrow.com

We go into next week very positive that Luke will receive a new immune system that will replace his own XLP faulty one. We know that the transplant procedure and the subsequent recovery is not without risk and so have prepared a specific prayer sheet – if you would like a copy please email me. It will also shortly be available on the teamhartley.co.uk web site. We have seen real event changing events that defy explanation happen for each of the boys during their transplants – and are looking for God to continue to walk with us through the valley.

Update 20th September 2007

It now looks like we have a new donor for Luke and we are likely to go back up to GOSH at the start of November – probably meaning a second Christmas in hospital. Luke remains very well indeed although he now has EBV on board at a reasonably high level – he is receiving treatment for this and we are expecting the levels to decrease over the next two weeks. With his brothers now all back at school it is hard to keep him amused. Unfortunately the Hampshire ‘home tutoring system’ doesn’t appear to have kicked in this time and we see no signs of it doing so and therefore we are having to home tutor. With everything else going on this is quite hard.

Thanks for continuing to hold us in your prayers. Over the last few months this has been especially important for us – it has been hard when we thought that we were almost at the end of this journey – we are resigned now to having to go through a fifth bone marrow transplant with all the issues this raises.

Update 11th August 2007

The news is to say just how well Luke remains. He has been home now over 3 weeks and he has been full of life, mischief and fun. He still needs regular platelets, bloods and GCSF (a drug which stimulates the production of white cells) which means regular visits to Southampton General Hospital, but gradually the days are lengthening between transfusions.

We have now had three engraftment results back – with no sign of any donor cells and unfortunately we were told that there was a new overseas donor available but for some reason (which we cannot comprehend) they have decided not to donate…

So the future remains uncertain for Luke but we continue to hold on that we will come through this dark valley.

Thank you for all of your encouraging emails and words since we have been home. We really appreciate them.

Update 19th July 2007

Luke came home last night and it has been a great joy to have him home. He has come back very well and spent some of yesterday evening playing football and bouncing on the trampoline. He is of course still immune suppressed so we have to be very careful and he will require regular visits to Southampton General Hospital for platelets and some other infusions and it will be a considerable time before he is able to mix freely again.

We found out this morning that the second engraftment study taken last week has also come back showing zero donor cells. We now just have to wait and see how things unfold over the coming weeks and months – not easy.

Can we please ask you to continue to encourage your friends (under 40) to join the Anthony Nolan Trust bone marrow registry (https://www.anthonynolan.org.uk/) or if you live outside of the UK then your local registry – you can find a full list of registries here (https://www.bmdw.org/).

Please also look to support the essential work we are now undertaking with the XLP Research Trust we set up 20 months ago. We will shortly be announcing the international research projects into XLP we will be funding. www.xlpresearchtrust.org

And we are still expecting that God moment for Luke.

Update 12th July 2007

The engraftment result came back late on Monday night and unfortunately showed that the cells which have come back strongly are all from Luke’s old immune system – none from the donor (Nathan). This means that the transplant has failed – Luke is still susceptible to EBV. The consultants believe that the cells from Nathan were too perfect and because of this there was not enough ‘fight’ in them entering Luke body to make them engraft.

This is awful news – after such a long and hard road to get so close to what seems like the end of the valley only to fall flat on our faces at the very last key result… devastating.

But Luke remains very well indeed. He is full of energy and life and it is hoped that he will be home this Saturday. He is now on just one IV drug and assuming he can come off this I will be driving up to London to bring him home early Saturday morning. He will then need to go through the bone marrow recovery process that our other three brave sons have gone through including semi isolation at home but it is expected that his old immune system will come back quickly.

There is still, according to the consultant, a very small chance that some of the donor cells could still emerge but he thinks this highly unlikely. A mixed immune system (old and new) could work perfectly well and would give Luke protection against EBV. The team at GOSH have taken a new engraftment study and we should have the results back on this either late Friday or next Monday.

So now we need a ‘God moment’ – we have seen a number of these as we have battled against XLP but never more so than now. Please pray for this – the prospect of having to go through another transplant is just too daunting at the moment but without seeing a miracle this is what we would need to do.

Update 6th July 2007

Luke is doing very well. Indeed the expectation is that he will be home now the week after next; if not earlier. He will come out of isolation this weekend, which after 38 days in a small room will be brilliant and assuming he stays well, he’ll be back home!

His blood counts have recovered relatively slowly compared to the other boys but they have picked up over the last week and his neutrophils climbed above the magic 0.5 measure today. We are still waiting for the all important engraftment study which will show us what percentage of the new immune system is Nathan’s – we are hoping for 100% (the other 3 boys have all been 100% engrafted) and will now get this late Monday.

Luke is extremely well – full of life, fun and joy…

Update 20th June 2007

Luke remains well with no temperatures (normal for the last 5 days), good blood pressure and virus free. There are early signs that the graft is starting to take hold but the next week is pretty crucial.

Please continue to hold Luke in your prayers as it’s a crucial time.

Update 10th June 2007

Luke’s transplant went very well with a good dosage of bone marrow from Nathan. Nathan was a total hero and although he was in some discomfort immediately afterwards, he is now fighting fit and will be back at school tomorrow.

Luke is doing very well to date although he is now not eating much. Yesterday I shaved his head as he started to pull lumps of it out. He is sleeping reasonably well at night and is extremely polite to everyone who comes into his isolation room.

Unfortunately though, we are back down to one effective carer as Allison now has what looks like the start of a cold.

Thanks to everyone who has sent Luke cards and gifts – they have really lifted him.

Update 5th June 2007

So here we are on the last transplant round. Tomorrow Nathan will travel up to Great Ormond Street Hospital in London with me and on Wednesday morning he will donate some of his own bone marrow to save the life of his brother.

All is going to plan for Luke. He finished his chemotherapy yesterday (Sunday) and has tolerated it well. That being said, he did have a reaction last week to one of his chemo drugs that gave him a seizure. In 15 minutes he was 100% again though and a subsequent CT scan showed no effects on the brain – but obviously very frightening for Allison at the time.

Luke is not enjoying being in isolation. This is partly to do with the extensive hydration that he has just finished (4 days of being hooked up to a machine infusion 100ml of ‘water’ every hour) partly because the chemo is now kicking in and also because he had enjoyed playing with the other boys on the ward who were also allowed out. The chemo is kicking in now – he was sick a little this morning (first time) and will need a blood transfusion today as well – but all is going to plan.

Please pray that this transplant is a success and that Luke is not traumatized by the whole experience – he is very ‘flat’ at the moment.

Update 27th May 2007

Luke is safely at GOSH now and settling in well. He had his central line in on Friday and this went without a hitch. He is not yet in isolation so is spending time wandering the corridor talking with everyone – and has even made friends with a boy the same age via the intercom system (he is in isolation). Luke was very excited on his first evening in and chatted with the nurse who looked after him all through the night… much to Allison’s annoyance (she is currently sleeping with him). All looking good and he tolerated the first course of chemo well today. But early days…

Back home things are not so good. On Thursday on my way to pick up some drugs for the boys I went over the handle bars of my trusty bike and broke my arm. Ironically this is the first time I have ever been to hospital for myself. I’m in a fair amount of pain, but better with the plaster caste. This does mean that I am unlikely to be able to stay with Luke once he goes into isolation (we have to scrub ourselves clean and have new clean clothes once he goes into isolation) and at the moment I would not be able to lift him. Typical! I will be at the fracture clinic this coming Thursday and we are praying for (another) miracle. I’m going up to GOSH for the day tomorrow to take some supplies for Allison…

Update 23rd May 2007

And so tomorrow we start on what we expect to be the last leg of this epic journey through the valley (Psalm 23).

Allison will take Luke up to Great Ormond Street Hospital around lunchtime and after having a central line fitted on Friday he will start chemotherapy on Saturday. Nathan will be traveling up to GOSH to give his bone marrow on the 5^th June – Luke will receive it the same day. And then it’s the critical waiting time to see the new immune system start to emerge…

Although this will be our fourth such procedure (sometimes I have to pinch myself about where we are in the walk and how far we have come) we know that there are significant risks involved for Luke. We need to ensure that he (and Allison and myself) stay well and that he doesn’t contract any nasty bugs – particularly during the days immediately before and after his transplant when he will be at his most vulnerable. Please continue to pray for us all – for strength for this last leg and that Luke will quickly be home. If all goes well then we would expect Luke to be back at school around October or November time – this will be the significant event when we will know that we have come through…

Luke is wonderfully well – we played football in the garden last night for what seemed like hours. Despite only being 7 he has a ‘Francis Lee’ type terrier thrust when he has the ball and faces down on goal (those from overseas should goggle ‘Franny Lee’ – the best centre forward I have ever seen in a Man City shirt). Knowing that he is so well has made the timing of Luke going in so much harder. But we also know from our own experience with Joshua, Nathan and Daniel, plus the now hundreds of cases we have now come across because of the XLP Research Trust, that XLP is a devastating time bomb just waiting to go off.

If you could then please do write to Luke. The arrival of the post every day is definitely one of the highlights and brings great cheer. His address will be:

Luke Hartley

Fox Ward

Great Ormond Street Hospital

Great Ormond Street

London

WC1N 3JH

Update 4th May 2007

The final countdown… It was confirmed yesterday that Luke will go in to start his BMT on Thursday 24^th May – less than 3 short weeks away. So the last major hurdle is now in sight…

Update 28th April 2007

We are now on a 2 to 3 week count down for Luke to go up to Great Ormond Street Hospital in London for his BMT. It seems to have taken an age to get there – but we are now at what we hope will be the major hurdle. About 10 days ago we took both boys (Nathan as well – as he will be Luke’s donor) up to GOSH for the standard day of tests. All went well except that when they did the heart echo they found that Luke has a slightly ‘leaky’ valve. This was a big shock but as their advice would be to come back in 2 to 3 years we are now not overtly concerned. Luke remains well and is almost looking forward to go up to GOSH. Indeed he feels the ‘odd one out’ as he has not had a BMT whilst his brothers have… He remains very well indeed.

And this Sunday should be a wonderful day (all of the Hartleys will be there) as we have the first XLP RT Triathlon. We have 27 teams battling it out over a 3.5 mile run, 16 mile cycle ride and a 0.5 mile swim!

Update 23rd March 2007

Luke now has a confirmed admission date to GOSH of the 16^th May – we are on a two month count down now. This could slip but we are now formally in the planning process. Luke remains very well.

Update 28th February 2007

Luke’s transplant has been put back to May; he remains very well and full of beans.

Update 16th January 2007

Luke is now booked in for his bone marrow transplant in the middle of March. The big news however is that Nathan will be his donor. The good folks at Great Ormond Street Hospital learnt last week that the donor we were expecting to use is now no longer available – we know no more than this. The consultants at GOSH are ‘not worried’ by this and have already carried out a number of ‘piggy back’ BMT’s. Nathan is delighted that he will have the chance to save the life of his brother! Please continue to pray that the boys stay well as we approach this last hurdle.

Update 30th December 2006

Luke’s transplant has been delayed until March 2007 as he remains so well and there are other children who need a transplant more urgently.

Once again, thank you to our elves, they made christmas that more special.

Update 17th December 2006

Luke is now actively looking forward to his BMT! He is scheduled to go up at the end of January next year and should be in hospital then for two months or so. He remains very well and full of life – and never stops talking – and cannot loose any argument. He will make a great politician.

We have literally just returned from a remarkable holiday in Lapland courtesy of the ‘Make A Wish’ foundation. The highlight for Allison and Dave was riding a team of 7 husky’s each through a snowy forest with the most amazing pink sky – breath taking and truly amazing. Dave also managed to turn a snow mobile over with Allison on the back…

Indeed with things being relatively ‘normal’ in the Hartley household we have also enjoyed a memorable trip to Disneyland Paris and a week on a farm in beautiful Devon.

Update 19th November 2006

Next Sunday and Monday Luke goes up to GOSH to have his PEG inserted. This is to allow us to start him on an overnight ‘milk’ feed to add some weight to him before he goes for his BMT at the end of January.

Update 15th October 2006

We are going to Disneyland Paris soon – this is Luke’s ‘Make A Wish’ wish – he wants to meet Mickey Mouse before his transplant…

Update 21st September 2006

We are still ‘on’ for Luke going for transplant – probably in February next year.

Update 23rd June 2006

Luke remains well. At some point soon we will be firming up with GOSH when he will go in for his BMT.

Update 15th May 2006

Luke remains well and full of talk!

Thanks for your continued support and prayers.

Update 18th March 2006

Luke is well and we took him for his check up at GOSH yesterday. He remains well and EBV free. The plan is still on for him to go to transplant early next year.

Update 18th February 2006

Luke is doing fine. He is keeping well and also started tennis lessons today. It is likely that his transplant will be early next year.

Update 1st February 2006

The plan now is for Luke to have his transplant in early 2007. This assumes that he stays well and it will give us a chance to see Nathan, and Joshua back to something like normality…

For those in the UK please don’t forget to watch Songs of Praise this Sunday (BBC1 – 18:10 GMT) – it should be interesting!

Update 1st January 2006

We are yet unsure as to when Luke will go up for transplant – possibly Q4 next year. Luke will share the same donor as Nathan so we have to wait a year – although we have the ‘luxury’ that Nathan could now become Luke’s donor! He loves his school with a passion, has already broken many hears (he had two marriage proposals in the same day at school) and is into everything (and everyone). He is great fun, a wonderful strong little man and very, very determined.

Update 23rd November 2005

For those that live local to Romsey, this Saturday at 6pm ish we will be turning the Christmas lights on in our home town – a real privilege (although it is likely that there will only be 3 of us there). Come along if you can and also support the XLP Research Trust who will be having a stall in the town hall.

Update 24th October 2005

I (Ian-family friend) have just had a brief telephone discussion with David and he asked me to post a brief update. Nathan has had his transplant and is doing well and has started the process of recovery. Joshua is improving and it is hoped although still has some way to go before he will be able to return home. Unfortunately Daniel has broken his wrist and now has a lovely red plaster on his arm! Please pray for the family, especially both parents as they are finding it a particularly stressful time.

Update 8th July 2005

The events in London yesterday were truly horrific – our hearts and prayers go out to all those affected. Only 24 hours earlier we were driving up to Great Ormond Street Hospital along the road where the bus was bombed and past Great Ormond Street Hospital. And the bone marrow transplant team who look after our sons heard the two blasts.

Luke remains well.

Update 11th June 2005

Nathan and Luke remain well. And Mum and Dad actually managed their first evening out on their own for well over a year. It was a little strange (but very nice as well).

Update 18th March 2005

Nathan’s forthcoming transplant has been put back further to the middle of June. This is because there is a strong chance of a second donor being found and this would help us in terms of Luke’s transplant. At the moment we have one wonderful mystery donor who is a good match for both Nathan and Luke (the boys are identical twins apart from being born 6 years apart). When the ‘definite’ donor gives stem cells if there are not enough for both Nathan and Luke (they would freeze some for Luke) then we would have to wait 12 months before going through the process with the same donor again. If we have confirmed that there is a second donor then Nathan can have all the stem cells from the first donor… Plus it gives us another month to ensure that Daniel is good…

So the roller coaster ride continues.

Update 31st December 2004

The elves and secret Santa’s went down very well in our household(s).

Daniel had a great day at GOSH opening presents and now has more than enough activities to keep him set up before returning home – hopefully in around 2 weeks time.

And Joshua, Nathan and Luke were also really blessed by the torrent of gifts…

We do have some photos and will send on to the elf’s ‘soon’ – with one parent at GOSH and the other at home we are not the best co-ordinated at the moment.

But sincerely ‘thank you’.

Update December 2004

Next Friday we take Nathan and Luke up to GOSH for their regular 6 month clinic so they should be able to see Daniel then. We will be talking dates for the next one…

Josh and his family are committed Christians.

Story written 2004

Joshua and his brothers were diagnosed with X-linked Lymphoproliferative Syndrome (XLP) back in November 2003 after suffering from acute anaemia.

The only cure is a bone marrow transplant which Joshua started in June 2004. The transplant has gone well with a few hick ups along the way and it is hoped that he will be home in the next few weeks. The transplant is a cure and once stable it is expected that Joshua will go on to live a full and reasonably normal life.

Joshua is the first of the 4 brothers that have to go through this procedure and he has been extremely brave and courageous. Indeed he has already volunteered to be the ‘third adult’ on the transplant ward during the subsequent transplants for his brothers.

He is very fun loving, has a great sense of humour and is extremely caring and gracious to others. Indeed it has been a privilege being with him during this difficult time. Always a smile on his face.

Update 20th February 2008

We sat down last night over tea and talked about the wonderful support we have had from you guys over the last 4 years. There was agreement that as Joshua, Nathan and Daniel are so well now that they should move on to the ‘Moved On’ status. It would be good to keep Luke on for a few more months – he remains well but still a good way to go.

Sincerely thanks for your support.

Update 20th September 2007

Positive news for Joshua in that his bone density is now almost back at the bottom of the normal range. There is now also a plan being put forward to take him off the last small dose of steroids he is on. However, his scleroderma is still active – particularly on some areas of skin. Joshua has definite better joint movement in the hips and knees compared to six months ago, but there has been no improvement on the ankles and arms.

Update 4th May 2007

Very positive news for Joshua after his ‘scare’ last week – his bloods on Tuesday show that everything is back to normal – a great relief. We are still no wiser though to knowing why his Hb level dropped so quickly. The plan now is that he will have bloods every two weeks and they will monitor the levels. Encouragingly, last week Joshua did have EBV in board (EBV is the glandular fever virus – which XLP boys have no resistance to and is life taking) with a moderate count of 15,000. This week it is gone – the new immune system is working well.

Update 28th April 2007

Joshua went up to GOSH for his 3 monthly check up on Wednesday. They were very pleased with the progress he was making – both his skin and his mobility are improving and his hair loss seems to have stopped. However on returning home Allison picked up an urgent telephone call from GOSH to say that his Hb level had dropped significantly (Hb is the main component of red blood cells). New bloods were taken at Southampton General Hospital yesterday morning (to check that it wasn’t a dodgy sample) and this also confirmed this fall. So last night Joshua had a blood transfusion, came home and is back at school today. The Doctors are unsure as to why this has happened – there are no obvious bleeds, nor any blood in his ‘outputs’. Joshua will go back into Southampton on Tuesday for a recheck…

And this Sunday should be a wonderful day (all of the Hartleys will be there) as we have the first XLP RT Triathlon. We have 27 teams battling it out over a 3.5 mile run, 16 mile cycle ride and a 0.5 mile swim!

Update 23rd March 2007

Joshua has recovered well from his infection and has been back at school all week.

Update 22nd January 2007

Joshua is doing well – seems to be over both the chicken pox and shingles. The hospital promised that he could come home for his birthday tea tomorrow evening – hopefully home to stay. Please send birthday emails to joshua@teamhartley.co.uk

We should be live on GMTV (ITV1) after 9am tomorrow (Monday) morning. Sorry its short notice.

And we should be in the medical section of the Daily Mail this Tuesday.

Update 20th January 2007

Joshua is in hospital (Southampton) – he was admitted on Thursday morning. It looks like he has both shingles and chicken pox…

However, he is much better today and we hope that he will be able to come home on Monday – his 15^th Birthday. Unfortunately it does mean that he will miss his birthday treat, we had booked tickets for us all to go to see the ‘Lion King’ in London, Allison will be taking the other 3 boys up.

Update 16th January 2007

Joshua’s battle continues. He now has shingles on a painful part of his bottom. Shingles can be very serious for a child shortly after their BMT but are not serious for Josh – just very, very painful. He is now back on our favourite drug aciclovir and he does seem a little better this morning.

Update 30th December 2006

Once again, thank you to our elves, they made christmas that more special.

Update 17th December 2006

It’s definitely been a better year for Joshua this than last. He did very well in his SAT’s over the summer and has now settled in to start working on his GCSE’s. Medically there has only been a little sign of GVHD – Joshua now has some hair loss – but the main development is that he now has ‘Scleroderma’ also known as ‘hard skin’. This is excess collagen deposits that can affect the skin and joints and is caused by GVHD, Joshua starts a new once a week course of medication – which could go on for… 2 years! This is limiting his mobility and can be very painful at times.

We have literally just returned from a remarkable holiday in Lapland courtesy of the ‘Make A Wish’ foundation. The highlight for Allison and Dave was riding a team of 7 husky’s each through a snowy forest with the most amazing pink sky – breath taking and truly amazing. Dave also managed to turn a snow mobile over with Allison on the back…

Indeed with things being relatively ‘normal’ in the Hartley household we have also enjoyed a memorable trip to Disneyland Paris and a week on a farm in beautiful Devon and Dave took Joshua and Nathan camping at ‘Soul Survivor’.

Update 19th November 2006

Joshua is doing OK. He is now well into his course of treatment for Scleroderma and he believes that his ankles and knees feel better. Allison took him up to GOSH last Wednesday and they were very pleased with the initial progress made, his skin looks much better. He continues to enjoy school although changing classrooms is a real battle for him with the push and shove of the crowds. Joshua should also restart his immunisations in January – proof that his immunity is very good now.

Update 15th October 2006

Joshua has now started his new medicine to help with the Scleroderma – we have been told to wait from 3 to 6 weeks to see some improvement, so it’s still early days. Mobility remains the big issue. He will also be going to GOSH this Wednesday as his port is not bleeding back properly.

On Thursday 26th October our Home Church will be holding a day of prayer and fasting for Joshua. We now need to see a final end to this long journey he has been on. Members of New Life Church in Romsey will receive a pray sheet next Sunday – but if you would like to join with our local church family, then please email and I will gladly send you a sheet.

Update 7th October 2006

The saga for Joshua continues. He went up to Great Ormond Street Hospital on Wednesday and was diagnosed with ‘Scleroderma’ also known as ‘hard skin’. This is excess collagen deposits that can affect the skin and joints and is caused by GVHD. Joshua starts a new once a week course of medication which could go on for… 2 years! Interestingly he has been better this week although is still complaining of sore joints in his ankles and knees…

A number of folks have emailed me back asking why only Nathan and Daniel are ‘Walking the Test Way’ this weekend – this is the reason for Joshua and we expect 6 year old Luke would spend at least 4 miles on his Dads shoulders. The forecast for the weekend is good and once again thanks to all you kind folks who have sponsored the boys!

Update 1st October 2006

The battle going on in Joshua continues. Over the last few months we had not seen any great signs of the chronic GVHD (Graft vs. Host Disease) which has been plaguing Josh over the last 28 months since his transplant. Unfortunately it would appear to have flared up again on his skin with some bright red patches on his lower legs. Fortunately one of the many creams that we have seems to have dulled this and it has not spread further. However one side effect on this is that his knees are now very sore – this is possibly due to the tightening of the skin following the GVHD. So he is back up to GOSH next Wednesday.

Update 21st September 2006

Allison took Joshua to GOSH today and again all is moving in the right direction. Because he is now on a very low dose of steroids his immunity has recovered enough so that he now no longer needs to have his immugloblins. Good news on his bone density too – a year on from being diagnosed as being 3 standard deviations from the norm, his bone density is now just below normal. Wonderful. So he will hopefully shortly stop these infusions as well and possibly cut down on the calcium supplements he has to take with every meal. Just 6 more weeks of steroids to go and then hopefully off those too – for ever!

Update 7th August 2006

Joshua is now much more mobile with the weight still coming slowly off. Next week should be an interesting challenge as I am taking Nathan and Joshua camping for a week.

Update 23rd June 2006

Joshua is now finally loosing weight – down 4kg over the last two weeks. This is about the right rate of decent in terms of weight loss – we don’t want to see it come off too quickly – but we do want to see it come off! He is now just on a small dose of steroids every second day and there is no visible sign of the graft vs. host disease.

Update 15th May 2006

Joshua is still on a small dose of steroids but disappointingly we have not seen the weight drop off as we have seen previously. Last week he went in to hospital to have his 3 weekly immugloblins and because he has been so ‘pricked’ and he has put on so much weight it took them a very painful hour to find a vein. So this Wednesday afternoon he will be going up to GOSH to have a ‘port’ put in. This will be a small operation to put a skin type port under his arm to make access to veins that much easier. Typically I am away on business that I cannot move so the responsibility again rests with Allison to get him up to London and back… However his GVHD seems to be OK at the moment, although he does now appear to have some hair loss. It’s been a tough 2 years for Joshua.

Thanks for your continued support and prayers.

Update 15th April 2006

Joshua is now on a low load of steroids following last Autumns flair up of Graft Vs Host Disease (GVHD). He still has a lot of weight to loose and it is still not clear if the graft has finally settled or not.

Update 18th March 2006

Joshua is getting better. Importantly he is now coming slowly off the steroids and he is starting to loose weight. His weight gain has been the big issue this time around. At the bottom of his transplant he was just 28kb, at the top of having steroids his body weight has nearly doubled. This had impaired movement, and just generally grotty feeling. He was also having some pain in his knuckles when he wrote but a new medicine seems to have knocked this on his head.

Update 18th February 2006

Josh is doing ok. He had his MRI scan at GOSH last Monday and we finally heard the results last Thursday. There has been little change to the inflammation (possibly a slight increase in one area) but as neurologically he is 100% ok they have started to reduce the steroid dose he is on. This is positive news for Joshua as the steroids have meant that he has put a huge amount of weight on – he should start to lose this now. He does now occasionally have problems with what seems to be arthritis in his knuckles on both hands – at times almost bringing him to tears with the pain. He is seeing a specialist on this in just over a week’s time. Joshua will also be in hospital during the day on Monday to Wednesday when he has his regular 6 week infusions to help increase his bone density (the other bad side effect of his steroids). He remains though the most wonderfully positive young man.

Update 1st February 2006

For those in the UK please don’t forget to watch Songs of Praise this Sunday (BBC1 – 18:10 GMT) – it should be interesting!

Update 25th January 2006

Joshua has been back to school for a few weeks now. At parents evening last night all of his teachers commented on just what a positive attitude he has…

Update 1st January 2006

It’s been a real mixed year for Joshua. He had the joy of returning to school in the spring, a wonderful summer and then it all went ‘pear shape’ in the autumn. Joshua is now back on steroids and this has really helped settle down the Chronic GVHD (which he has now been officially diagnosed with) but we also know that this is ‘bad news’ for his bone density. He is pretty good at the moment and has enjoyed having all his brothers around him over the festive period and he is now the very proud owner of a Hornby Train Set! The hope for early 2006 is that we can reduce the steroid dosage, see the chronic GVHD go away and build his bones back up – and then importantly get him back to school. I have never met a young man so desperate to get back to school! Joshua remains a young man of deep faith, undying love and great courage.

Update 18th December 2005

Joshua went to have a follow up MRI. The bad news is that there would appear to be some new areas of infection/inflammation in his brain. But the areas that were there before have largely dissipated. In himself he remains well, his memory appears fine – although is very stiff (particularly around the knees) due to his low bone density (caused by being on steroids for such a long time – 2.5 standard deviations from the norm for his age for those with a maths background). He will be coming with Nathan to see the consultant at GOSH on Wednesday for an assessment…

Can I take this opportunity, a week before Christmas Day, to wish you all a peaceful Christmas. We are so grateful for the wonderful support over the last few months – it means so much!

Update 7th December 2005

Please continue to pray for Joshua – he has ongoing problems with his joints due to low bone density – it can be very uncomfortable at times.

Update 30th November 2005

The great news is that Joshua came home on Monday evening from Southampton General Hospital. He is doing very well but is unlikely to return to school until January – when we know that his immune system is back up functioning properly after the recent steroids he had to take.

Update 23rd November 2005

Joshua is in the process of overcoming the next hurdle. Unfortunately last Sunday it was clear that he was getting short of breath and so has been admitted to Southampton General. He has ‘PCP’ which is the fore runner to pneumonia. He became susceptible to this after the last dose of steroids – and he is now on steroids to help dampen down the situation. The +ve news is that he would appear to be over the worst – the Drs are pleased with him.

So we now have to deal with having two sons in two separate hospitals – although we have been told that Joshua should be home again shortly. When he comes home he will be placed in bubble wrap and never allowed out again (joke).

Thanks for your prayers – the messages we get from everyone across the globe are a real encouragement and blessing.

Update 9th November 2005

Joshua is doing great at home and will hopefully be going back to school for the morning this thursday.

Update 4th November 2005

Joshua is now home and just about fully recovered from his infection. It would appear to have been a flair up of the virus on the brain that he had during his transplant. The medicine used to ‘put him right’ was stopped about 3 weeks before the flair up. His short term memory has recovered and we are now working on replacing some of the lost muscle that the steroids he has been on have wasted away. We hope he will be fit enough to return to school in a few weeks.

Update 24th October 2005

I (Ian-family friend) have just had a brief telephone discussion with David and he asked me to post a brief update. Nathan has had his transplant and is doing well and has started the process of recovery. Joshua is improving and it is hoped although still has some way to go before he will be able to return home. Unfortunately Daniel has broken his wrist and now has a lovely red plaster on his arm! Please pray for the family, especially both parents as they are finding it a particularly stressful time.

Update 5th October 2005

Joshua is not well. Indeed he is also now staying up at GOSH. Allison brought him up for the BMT clinic today and when they saw how he had lost some of his cognitive reasoning (can’t finish sentences, short term memory loss) they immediately readmitted him. It is highly likely that he has a virus in the head similar to what he went through 15 months ago during his transplant. 5 weeks ago we stopped a drug that specifically addresses this so this is the likely outcome and if it is should respond well to treatment. They are restarting the drug this evening and he will have an MRI and other tests in the next 48 hours. They also found out that he has low bone density which is probably what is causing the ankle and kneed aches. This is due to the length of time he has been on steroids but is treatable by a regular 9 week IV infusion.

Update 25th September 2005

There was some ‘good news’ mixed in with some bad for Joshua. The ‘good news’ is that the problems that Joshua was having with his liver and graft versus host disease has passed – these results were all good. But he is now back on steroids (quite a small does) as x-rays taken earlier this week at GOSH showed that he had some inflammation around his knees and ankles – part of skin graft vs. host disease. We do need this to ‘go away’ so that we can concentrate on Nathan’s transplant.

Update 19th September 2005

Unfortunately Joshua is also far from being himself. For the last 10 days or so he has found it difficult to sleep and so has had a few days off school. He also has some stiffness in his knees which we understand is due to him being on steroids for such a long time. Plus he also has a urine infection. So this Wednesday he will go up to GOSH for a full review to try and work out what is happening. His blood and other measures look good through – we don’t think it is the liver GVHD but do need to get him right before Nathan goes in.

Update 3rd September 2005

On Monday Joshua will be in GOSH having his PEG removed (this is a tube that goes straight into his stomach and through which we can push drugs etc.) As of today he is now down to just two drugs twice a day – and happily takes them orally.

And for those who can, we will be featured on BBC Breakfast News this Monday morning – it was recorded yesterday so we don’t know what time!

Update 29th July 2005

Joshua remains well and is coming off his steroids very slowly (again). At his last day of school he was somewhat shocked to be called forward by the Head Master and awarded him the Diana, Princess of Wales Memorial Award for Young People.

This Sunday both Joshua and Nathan are going ‘Over The Wall’ for a week at a special camp which they are looking forward to.

Update 8th July 2005

The events in London yesterday were truly horrific – our hearts and prayers go out to all those affected. Only 24 hours earlier we were driving up to Great Ormond Street Hospital along the road where the bus was bombed and past Great Ormond Street Hospital. And the bone marrow transplant team who look after our sons heard the two blasts.

Joshua has responded well to the small dosage of steroids and his liver function tests are all heading back towards ‘normal’ again. He has been back at school for the last two weeks. Ironically he was on a school coach yesterday heading for a visit to the Bank of England and St Pauls Cathedral – but the coach turned round.

Update 22nd June 2005

The swings and roundabouts continue…

We had hoped that Joshua would have finished the majority of his meds two days ago. But at the start of the week he clearly was not too good, hasn’t made school and it has been confirmed today that the Graft Vs Host Disease of the liver is back. So he is back now on the steroids, although a relatively mild dose, and hopefully this will clear it up.

It reminds us that whilst the bone marrow transplants are life savers and a cure for XLP, they do leave the new immune system very open to disease and infection… It will take 2 plus years for their immune systems to relearn – an awful long time.

Update 11th June 2005

We have been waiting for a while now to get Joshua’s CD4 counts up (a subset of his white cells). Once these reached the magic figure of 300 we can reduce his medication dramatically… And this week they shot past the winning post at over 500! So a week this coming Wednesday he stops the majority of his medicines and his 3 weekly immugloblins – wonderful. And last Wednesday (8th) was the anniversary of his transplant.

Update 24th May 2005

The Walk 4 Life on Sunday was fabulous. Some 700 folks turned out and the estimate is that a total of over £30,000 has been raised for the Anthony Nolan Trust. We are made it around although it was a bit telling on the kids towards the end. Joshua was a complete star and recorded several interviews on his way around – for those in the South of England he should be on BBC South Today tonight.

Update 3rd May 2005

Joshua went to clinic at GOSH today, they are very pleased with his progress and immunity and we now have a plan to get him of the majority of his meds in 5 weeks time – which will nearly be the anniversary of his transplant.

Update 7th April 2005

Joshua is great – very happy and loving the freedom of being back in the community.

Update 18th March 2005

Joshua went back to school a week last Monday. He was suppose to just stay for 3 mornings (Monday, Wednesday and Friday) but actually did the full week! and all of this week so far. And the reception he his had from his class mates has been great – a real credit to the school. He is very happy to be back – and very enthusiastic.

So the roller coaster ride continues.

Update 7th March 2005

Joshua has gone back to school today… He has continued to recover well from the GVHD of the liver and last week the Consultant at GOSH said that it would be OK for him to go back. He will be broken back in gradually. He was deliriously happy this morning and left quite early – so was probably the first one there. We remain mindful however that it will take up to 2 years for the immune system to relearn and catch up.

Update 5th February 2005

Joshua is currently at home so he has got time on his hands, he says he is very happy to send thank you cards and e-mails so if you are sending a letter/ card can you please if you have one write down your e-mail address.

Update 4th February 2005

Joshua is doing very well in regards to the liver infection. The key measure has now fallen by two thirds in less than a week, and although it is still high it is clearly on the way down. And he is in great form at the moment…

Please continue to pray for us all – especially for Allison who has lost some hearing in one of her ears. She puts this down to the whirring of machines in hospital, but 3 weeks after Daniel coming home from GOSH she has not recovered.

Thanks again for your support and prayers.

Update 30th January 2005

Joshua seems to have made a good recovery from the liver infection that he had. His eyes are ‘near normal’ now and he is eating and drinking well.

Update 22nd January 2005

Today Joshua became a TEENAGER. No sudden change but a joy that he has reached this land mark age. Amongst his presents was the start of a new course of steroids as yesterday it was diagnosed that he has Graft vs. Host Disease of the liver. The consultants are confident that this will clear it up but it does unfortunately mean that going back to school is now on hold – with a proposed target date from the consultant being after Feb half term… The good news is that his new immune system is ‘excellent’ but this new course of steroids will ‘dampen’ it down. Already after just one set he seems better… swings and roundabouts.

Update 19th January 2005

Some of you will have seen the fantastic news that Joshua returned to school today…

The down side is that it looks like he has a liver infection which is making him very tired – so he came home just after lunch. But he is deliriously happy about being back. We are waiting a call from the team at GOSH to agree next steps re the liver infection (bloods were taken today) – we should get this tomorrow.

Update 16th January 2005

Daniel is home and doing extremely well.

We now have a (hopefully) normal period of family life before Nathan goes in for his transplant – scheduled at the moment for the end of April. Joshua is still on target to return to school fully in the next 2 weeks.

Thanks for the wonderful support.

Update 11th January 2005

Joshua should be back to school middle of next week 🙂

Update 7th January 2005

It is almost certain that Joshua will be returning to school on the 18th of this month. This very much signifies the ‘end of the road’ for Joshua with regards to XLP. Whilst he will still be on some medicines and immugloblins for some time yet, going back to school signifies that he can get back in to public life. He will have to have all of his baby and child jabs again and now actually needs to loose some weight. The later I never thought I would say about Joshua, as he has always been a rake. At the bottom of his transplant he was down to 28.5kgs but is now up to 45kgs, even putting on weight after finishing steroids.

Update 31st December 2004

The elf’s and secret Santa’s went down very well in our household(s).

Daniel had a great day at GOSH opening presents and now has more than enough activities to keep him set up before returning home – hopefully in around 2 weeks time.

And Joshua, Nathan and Luke were also really blessed by the torrent of gifts…

We do have some photos and will send on to the elf’s ‘soon’ – with one parent at GOSH and the other at home we are not the best co-ordinated at the moment.

But sincerely ‘thank you’.

Update 8th December 2004

Joshua remains well and we have just had confirmation that his graft remain 100% donor (hurray). It is now planned that he will go back to school in the New Year.

Update 5th December 2004

Joshua is still very very well – and is contemplating setting up a cakebaking business (follows from his Mum). Any orders please email him, link is above.

Update 23rd November 2004

Joshua remains well and we will be taking him to clinic at GOSH tomorrow (Wednesday) and he should also be able to see Daniel for a short time.

Update 1st November 2004

Joshua is doing very well. His weight continues to go on and he is full of life. The steroids are being reduced now, they are now half the dosage he was originally prescribed and the dosage will fall again weekly.

Thanks again for your great support and encouragement.

Update 15th November 2004

Joshua continues to go from strength to strength – looking very well. The steroids have been reduced significantly and most notably he now has T-cells and no signs of EBV! Although he is a little slower today, probably due to the effect of coming off the steroids…

Update 27th October 2004

We went up to see the consultants at Great Ormond Street today and all pretty positive.

Joshua continues to do well and all are hopeful that he could be back at school just before Christmas (it will be 6 months since his transplant on 9th December). He has put on a lot of weight in the last two weeks, no temperatures, sickness or signs of Graft vs. Host disease and is very bright. He is the best we have seen him for – well as long as we can remember…

Update 16th October 2004

Joshua is now home and doing really well. It was as suspected a case of Graft vs. Host Disease – very mild.

He is now being reintroduced to solid food again – chicken and rice cakes have never tasted so good. It is hoped in 2 weeks that he will be back on a full solid diet…

Update 13th October 2004

Joshua should be home tomorrow (Thursday) as we had a mad dash to London from Southampton General last Friday to confirm that it is Graft vs. Host Disease and now that he is well through a course of steroids he is very much better – big relief. He will have a check up at GOSH on Wednesday.

Update 29th September 2004

Joshua remains in Southampton General with the diagnosis unchanged. Tomorrow (Thursday) he will go down to theatre for a minor op to reinsert his Hickman line as it has slipped and they are having problems drawing back blood. They also intend to take some biopsies of his bowel at the same time.

Update 28th September 2004

Joshua is still in Southampton General and is likely to be there for a few days more. After an initial (wrong) diagnosis of constipation they believe that what is going on in the stomach and bowel is likely to be a combination of GVHD and the ongoing soreness of his stomach – possibly with a bug on board. He is OK but very tired (nights are quite disturbed). Please pray that we will see an end to this and that he can get back on with his recovery.

Update 24th September 2004

We always knew there would be ‘swings and roundabout’.

Joshua was doing very well until Wednesday morning when there was clear swelling in his hands, some high temperatures and the sign of the Graft Vs Host Disease rash…. So he is tonight in Southampton General Hospital so they can keep an eye on him. Hopefully he should be out over the weekend. It has been confirmed that he has mild GVHD. It’s a real shame as it seemed to us that Joshua had ‘turned a corner’ the week before with only occasional sickness and great weight gain.

Nathan may also be admitted due to EBV virus, and Daniel is having his treatments at GOSH.

So it’s a real battle in our household at the moment – I think we feel that we can fight one battle at a time but not two or three together … welcome your prayers as always.

Update 15th September 2004

Written by Josh’s Dad.

It’s now an amazing 14 weeks since Joshua’s transplant (D+98) and he continues to do well, with some sickness still. We’ve just got back from clinic in London and they are pleased with the progress he is making. He has put a little weight on (although still some way to go) and the various levels remain good. They are gradually reducing Joshua off some of his meds as well.

Next Wednesday we take Daniel up to GOSH for his pre transplant treatment. He is still scheduled to go for transplant around the end of October…

Other news…

The boys received the highest award possible recently. (Sorry this won’t work for non BBC viewers!). Last summer the three oldest appeared live on Blue Peter after Joshua won a competition (before all this XLP stuff kicked off) and they were duly awarded the important blue bade. Thanks to our friends at PostPals all of the boys were awarded the gold Blue Peter badge…

https://www.bbc.co.uk/cbbc/bluepeter/contact/badges/ Joshua in particular is ‘over the moon’ and rumour has it that he even wears it on his pyjamas (only joking Joshua).

Update 2nd September 2004

Joshua has been home now almost two weeks and continues to do well and put some weight on. He still needs a fair amount of nagging to get his special diet down him – no change. Yesterday we drove up to clinic in London (we thought the drive would be a nightmare but amazingly we arrived 1.5 hours early!). This also went well – overall very +ve. There are some concerns that he might have a slight ‘bug on board’ which is contributing to occasional sickness so he now has additional anti biotics.

Update 23rd August 2004

Just wanted to let you all know that Joshua arrived home on Friday. He is very well, very pleased to be home and fitting right back in with the other boys.

The Consultants at GOSH were very pleased with the way the transplant went, that he remains EBV free and that the ‘milk type’ diet he is now on will give him all the nourishment he needs plus also help soothe his inflamed stomach.

It is really impossible to put into words the emotions that we went through driving him back from London on Friday. We are just so grateful that he has come through and that he is doing so well. For the next 4 to 5 months Joshua has to stay away from places where there are lots of people – church, school, restaurants (all food once he is back on has to be tightly controlled), cinemas although Dad has said that he can go and see Southampton FC play as there are not many people there.

Before Joshua came home he received a special injection of T Cells from Texas! Before transplant Allison (his Mum and his donor) gave blood which was sent from the UK to Baylor College of Medicine who were then able to extract from Allison’s blood the T Cells that she has to combat EBV and basically they replicated them and made more! Whilst it is still at test stage none of the 70+ test subjects have gone on to develop Lymphoproliferative Disease (LPD) post transplant.

There will be a fair amount of UK media interest – we should feature on BBC South Today and Meridian Tonight and we also know that Sky News have asked for footage as well…… and there should be a large feature in the Baptist Times this Thursday!

To those of you who have stood in the gap with us over the last few months – thank you. We have known the almost physical manifestation of prayer support helping us through some tough times, and the hand of the Master soothing our brow. As Joshua nears the end of his journey we know that we are very much as a family still at the start – with the other three boys still awaiting their ‘call up’.

Thank you

Blessings

David

Update 28th July 2004

Just heard that Joshua has gone ‘into reds’ which unfortunately means back in his isolation room as he has a bug…

However it is not very serious and a decision will be taken on Thursday afternoon about him coming home – it will either be Friday or early next week.

Our house is probably the cleanest it’s ever been – and a large gazebo now stands in the garden to allow Joshua to go out without being in sun light.

Update 1st August 2004

Afraid further disappointing news.

Joshua is likely to be in GOSH for around another 21 days. Since he came in over 2 months ago he has lost a lot of weight and they are keen to get to the bottom of it. He will be ‘scoped’ on Wednesday with results back by the end of the week. A course of treatment will then probably take 2 weeks. We were all hugely disappointed with plans at home for the homecoming well developed – now well on ice.

Joshua remains in good spirits though and was pleased 2 see me (I’m sat with him now). He knows how important it is to come home well.

Please also spare a thought for Daniel who will be come a ‘true Jew’ on Wednesday – necessary preparation before his transplant later this year.

Story written 2004

Daniel and his brothers were diagnosed with X-linked Lymphoproliferative Syndrome (XLP) back in November 2003 after suffering from acute anaemia.

The only cure is a bone marrow transplant which Daniel is due to start in October 04.

Daniel will be the 2nd of his brothers to go through this – the reason he is next is that a few months ago Daniel had EBV virus (that causes glandular fever) and the XLP condition means that he can not fight this – and it is potentially life taking.

Fortunately Daniel was diagnosed quickly and a combination of drugs has seemingly irradiated it from his system. However, there is fear that it could come back quickly.

Daniel is great fun. Very quite with people he doesn’t know but a ‘riot’ once he gets to know them. He is described as an angel at school which confounds his parents and his brothers who know what he is like at home. Daniel is definitely the sporty boy and has a seemingly unendingly unfilled stomach.

Update 20th February 2008

We sat down last night over tea and talked about the wonderful support we have had from you guys over the last 4 years. There was agreement that as Joshua, Nathan and Daniel are so well now that they should move on to the ‘Moved On’ status. It would be good to keep Luke on for a few more months – he remains well but still a good way to go.

Sincerely thanks for your support.

Update 20th September 2007

Daniel has taken secondary school very well – especially the PE and after school games clubs. He is doing very well.

Update 28th April 2007

This Sunday should be a wonderful day (all of the Hartleys will be there) as we have the first XLP RT Triathlon. We have 27 teams battling it out over a 3.5 mile run, 16 mile cycle ride and a 0.5 mile swim!

Update 30th December 2006

Once again, thank you to our elves, they made Christmas that more special.

Update 17th December 2006

Daniel is extremely well, growing ‘brilliantly’ (the later being his favourite word) and now over two years post his BMT. He has struggled at school from the work perspective but is looking forward to go to a new school this summer.

We have literally just returned from a remarkable holiday in Lapland courtesy of the ‘Make A Wish’ foundation. The highlight for Allison and Dave was riding a team of 7 husky’s each through a snowy forest with the most amazing pink sky – breath taking and truly amazing. Dave also managed to turn a snow mobile over with Allison on the back…

Indeed with things being relatively ‘normal’ in the Hartley household we have also enjoyed a memorable trip to Disneyland Paris and a week on a farm in beautiful Devon.

Update 1st October 2006

Thanks to all those who have supported Nathan and Daniel in their forthcoming walk (9 days time). This will definitely be the longest that either of the boys have ever walked so please sponsor them…

And if you ever wondered where all this money is going to I am pleased to announce that today we have an advert in the prestigious medical journal ‘Nature’ with a call for research proposals – just a year on we now have sufficient funds to start funding research into this cruel condition.

Update 21st September 2006

Thanks for those of you who have sponsored Nathan and Daniel on their ‘celebration of finishing their bone marrow transplant walk’ they are now 10% of the way to their self imposed target., so thank you!

Update 23rd June 2006

Daniel remains well. He was a little off colour yesterday but made it to school today.

Update 15th May 2006

Daniel also continues to improve. This afternoon he has the first of his childhood immunizations again. With a new immune system they have to go through all their jabs again and can catch chickenpox again…

Thanks for your continued support and prayers.

Update 18th March 2006

Daniel is well and we took him for his check up at GOSH yesterday. Daniel will shortly restart having all his child hood immunizations and this marks the near the end of his journey. He is gaining weight and growing well (0.6cm since Jan). Last Saturday I picked Daniel up after his two hours of tennis. The wonderful coach came over and told me how well he was doing, and asked when Daniel was going for his transplant – when I told him that he had it 14 months ago he was quite amazed. Daniel is that well (and at tennis as I write this).

Update 18th February 2006

Daniel is doing brilliantly. He started tennis lessons today as the natural athlete of the family. Growing well, full of energy – you would not know that he has been through a bone marrow transplant.

Update 1st February 2006

For those in the UK please don’t forget to watch Songs of Praise this Sunday (BBC1 – 18:10 GMT) – it should be interesting!

Update 1st January 2006

It has been a great year for Daniel. Returning home from transplant in the middle of January (seems like a life time ago) and then back to school in September since when he has not looked back. He did his best to add to the chaos of October (when both Joshua and Nathan were in GOSH) by breaking his wrist whilst skateboarding (the skateboard is having a winter break). He is back to ‘bouncy Dan’, eating us out of house and home and very happy.

Update 23rd November 2005

For those that live local to Romsey, this Saturday at 6pm ish we will be turning the Christmas lights on in our home town – a real privilege (although it is likely that there will only be 3 of us there). Come along if you can and also support the XLP Research Trust who will be having a stall in the town hall.

Update 4th November 2005

Daniel’s plaster caste will come off next Wednesday…

Update 24th October 2005

I (Ian-family friend) have just had a brief telephone discussion with David and he asked me to post a brief update. Nathan has had his transplant and is doing well and has started the process of recovery. Joshua is improving and it is hoped although still has some way to go before he will be able to return home. Unfortunately Daniel has broken his wrist and now has a lovely red plaster on his arm! Please pray for the family, especially both parents as they are finding it a particularly stressful time.

Update 5th October 2005

Daniel was seen at clinic and they remarked how well he looks. He should come off all his meds bar Penicillin (which he should keep taking for life) this Friday.

Update 3rd September 2005

Daniel returned to School and had a fantastic day. Everyone was so pleased to see him back at school after such a long time away. Truly wonderful.

And for those who can, we will be featured on BBC Breakfast News this Monday morning – it was recorded yesterday so we don’t know what time!

Update 24th August 2005

Great news – Daniel can go back to school on the 2nd September! He is absolutely delighted about this (as are we) and is really looking forward to it. Not certain if the school will be ready for the return of the whirlwind though.

Update 29th July 2005

Daniel remains very well – and very loud. This is a very good sign. Each week we reduce his Cyclosporan dosage (this is a drug that suppresses his new immune system – stops it becoming a ‘wild horse’). And immediately afterwards we see a slight flair up of his skin graft vs. host disease but the steroid cream we apply has it under control after a couple of days – may this pattern continue! This Sunday both Joshua and Nathan are going ‘Over The Wall’ for a week at a special camp which they are looking forward to.

Update 8th July 2005

Daniel is very much back to himself. He is eating enormous amounts of food (far more than he did when he was on steroids) and has lots of energy. If things continue he should be back at school at the start of the next school year in September but still a long way to go.

Update 22nd June 2005

The swings and roundabouts continue…

Daniel had his central line removed on Thursday. This was mainly because it was only being used to take bloods every week or so and is a potential infection point. The small 15 minute operation went without a hitch and Daniel was very pleased to see the line go. However just tonight he showed that he had a small temperature…

It reminds us that whilst the bone marrow transplants are life savers and a cure for XLP, they do leave the new immune system very open to disease and infection… It will take 2 plus years for their immune systems to relearn – an awful long time.

Update 11th June 2005

Daniel is doing OK as well. His blood counts have been good and climbing over the last few weeks. He does however have a line infection (again) – so is receiving an extra antibiotic for 5 days. The plan now is to take his central line out as soon as possible as it is only used for bloods – another good step forward.

Update 24th May 2005

Very pleased to say that Daniel is home… and will be sleeping here tonight. Amazingly the jaundice has disappeared (there apparently are some possible medical reasons for this – when we know them we will let you know!). He has come back on a gluten and dairy free diet – and as he is now receiving no supplements then has to keep his weight up. We are off to GOSH tomorrow for them to check him over and try and unravel what has happened.

Update 19th May 2005

Some steps forward – some back…

Daniel is now allowed home for around 4 to 5 hours a day. This has made a huge difference as he can see his brothers when they come home from school and we can all have tea together. He is on a non-diary, gluten free etc. diet. This means only very simple things – but it is now amazing what the supermarkets stock… For the last few days we have seen the start of the end of the watery poos and indeed some solid ones yesterday (but not today). He is now passing about 0.6 litres a day… (sorry for the detail)

He finally stopped the steroids on Monday after what has felt like an eternity. This should mean that his white cell count should now start to rise and get rid of the blessed Astrovirus…

However – it does now look like he has GVHD of the Liver – there is a definite yellow tinge to his eyes… And the solution to this? Back on the steroids… You may recall that Joshua was diagnosed with this way back in January – he is due to stop his steroids for this next month!!!

So it looks like we are in for a long hard fight over the summer…

Please continue to pray for the break through we so desperately need…

Update 13th May 2005

Daniel remains in hospital in Southampton. The biopsies came back showing that he does only have Astrovirus so he is now being weaned off steroids. Unfortunately he had a rash this morning suggesting that the GVHD of the skin might be reappearing (we have been in this cycle for about 3 months now!). Daniel is well in himself, laughing a lot again (playing a few jokes on the nurses and his parents) eating a plain diet but also still having TPN – so is restricted in movement. However the room he is in is ‘palatial’ compared to most rooms – with his own bathroom, PS2 and desk top computer…

Update 3rd May 2005

Daniel is back at Southampton General from GOSH. He is doing well – and is starting to eat some plain foods again (already put back on 1kg). We should get the results of the biopsies back tomorrow.

Update 1st May 2005

Daniel’s scope went well at Great Ormond Street on Friday. The consultant said that it looks like it is just Astrovirus and not GVHD of the gut – but this will be confirmed when they get the results of the biopsies back – but not until Thursday. Daniel is now on TPN (that provides all of the fluid and the essential nutrients and electrolytes when they are unable to feed themselves by mouth) with a plan being hatched to reintroduce food gradually. The consultant was confident that there has been do significant damage to either the stomach or gut.

The team at GOSH were happy to allow Daniel to come back to hospital in Southampton on Saturday – but they cannot arrange a bed until this Wednesday, the day that Joshua is meant to go up to GOSH for clinic ! More pressure…

Update 26th April 2005

Daniel is unfortunately still in hospital. Whilst they are well on top of the line bug , the Astrovirus and possibly other ‘things’ mean that he is loosing a lot of body fluid and consequently weight, minerals etc.. Last night he passed over 1.2 litres… a lot for a 9 year old boy. It is likely that he will go to Great Ormond Street Hospital in London for further tests on Thursday afternoon.

Update 24th April 2005

Daniel is still in Southampton General Hospital – although we hope he will come home tomorrow (Monday). He did indeed have an infection around his central line which then spread to his blood. It would however appear to be sensitive to an antibiotic so he is on the mend. However he will need the antibiotic 4 times a day for the next 2 to 3 weeks – and it will be IV – so tomorrow one or both of Allison and myself will be trained in this – yet another new skill. The Dr’s are concerned that he is basically now ‘skin and bones’ after around 10 weeks of acute diarrhoea courtesy of the Astrovirus – although amazingly his weight has stayed pretty stable.

The knock on effect is that it is now unlikely that we will have Daniel well enough for Nathan to go up for his transplant in mid June – so this is likely to be pushed out yet again. Both Nathan and Luke remain well (and EBV free).

Update 19th April 2005

Daniel has just gone into hospital again – unfortunately he had high temperatures this morning and vomiting. We suspect that it is a line infection (where his Hickman line goes into his skin) as there was some oozing there late last week, a swab was taken and a bug identified, and we were to go in and pick some antibiotics up this morning anyhow! Will let you know how thinks progress. The good news is that his blood results last week were excellent – so the transplant side is at least going well.

Update 7th April 2005

Daniel seems to be going through a cycle of ‘Graft Vs. Host Disease of the skin’ and ‘Astrovirus’. Unfortunately the answer to one stops the other from healing! So we still have bad stools and bad skin! But overall he is OK now, it will just takes time.

Update 23rd March 2005

Good news – Daniel is home 🙂

His blood levels went up yesterday, although they have fallen back again today. He has had no temperatures and they haven’t grown anything so we are off tomorrow (Wednesday) to GOSH to see the team there to see if they can shed any light.

Daniel seems good but the astrovirus continues to make things flow. There is a great poem about Astrovirus here.

Update 20th March 2005

A worrying development with Daniel is that his blood counts have rapidly declined since going into hospital – he is now neutropenic, his blood counts for his white cell count and neutraphils have fallen very quickly. And they have been so good for the last couple of months. At least he was in hospital when this happened.

So very unlikely to be out tomorrow as planned, he remains visibly well and in good spirits – although ‘fed up’ with being in isolation.

Update 18th March 2005

Daniel was admitted back to Southampton General last night. The Astrovirus he has had on board for a few weeks now means that his body has not been absorbing some the medicines and minerals necessary. This is being treated now and he should be out by Sunday evening. His ‘blood’ results still look good and he remains 100% donor engrafted.

So the roller coaster ride continues.

Update 1st March 2005

Daniel is OK. Last week it was diagnosed that he has ‘astrovirus’. This is not serious in itself but does account for the diarrhoea and some sickness he has had. We have to just wait for his fledgling immune system to drive it out. His blood levels remain good and as I write this is downstairs with his home tutor working on English.

Update 4th February 2005

Very Pleased to announce that Daniel is now home from hospital. It would appear that he is over the worse that the bug could throw at him – although it will take time for his stomach and bowel to settle down.

Please continue to pray for us all – especially for Allison who has lost some hearing in one of her ears. She puts this down to the whirring of machines in hospital, but 3 weeks after Daniel coming home from GOSH she has not recovered.

Update 31st January 2005

Quick update to let you all know that Daniel is now in Southampton General Hospital…

Over the last week he has been struggling to keep down any food and liquids – and whilst there is no positive conclusive sign of graft vs. host disease it has been agreed that he should go in for a couple of days to get liquids intravenously – resting his stomach.

Puts more pressure on at home…

Update 30th January 2005

Daniel however has picked up an infection in the last week. He is struggling to eat and drink and has a slight rash on his torso. When we visited Great Ormond Street Hospital last Tuesday they didn’t think it was Graft vs. Host Disease. We are back this Wednesday and in daily contact with them. Fortunately Daniel had already regained all the weight he had lost during transplant – but he is not a ‘happy bunny’ and at the tender age of 8 wears his heart on his sleeve. We are feeding him via his PEG at the moment.

Update 22nd January 2005

Daniel is doing great with platelet and white cell counts in the ‘normal’ range….. they were very pleased with him yesterday.

Update 20th January 2005

Daniel continues to do well – including playing badminton in the garden this afternoon (before the rain). He is loving having a home tutor and the relative freedom of home compared to his isolation room.

Update 19th January 2005

Daniel continues to do well – including playing badminton in the garden this afternoon (before the rain). He is loving having a home tutor and the relative freedom of home compared to his isolation room.

Update 16th January 2005

Daniel is home and doing extremely well. He has settled right back in to family life and is absolutely delighted to be home with his brothers – he is literally beaming. Daniel is now virus free and remarkably is the same weight as he went in to transplant – he is eating very well.

We now have a (hopefully) normal period of family life before Nathan goes in for his transplant – scheduled at the moment for the end of April. Joshua is still on target to return to school fully in the next 2 weeks.

Thanks for the wonderful support.

Update 11th January 2005

Quick update to let you know that Daniel will now be coming home this Friday (14th).

Update 7th January 2005

The good news is that Daniel may be coming home next week… He has been very well in hospital over Christmas and the New Year and has put back on almost all the weight he has lost. He does have a very mild Graft vs. Host Disease rash but nothing to be concerned about. And Daniel has had the odd temperature spike that has been treated as a line infection. He is very good in himself, although still in isolation. The ‘bug’ that he has been struggling to get rid of has almost gone – the load on Monday was 48 (one of the lowest ever recorded – usually in the 10,000’s). In his room he now has a mini trampoline and electronic keyboard. We swapped over today and I left him baking cookies with his teacher.

Update 31st December 2004

The elves and secret Santa’s went down very well in our household(s).

Daniel had a great day at GOSH opening presents and now has more than enough activities to keep him set up before returning home – hopefully in around 2 weeks time.

And Joshua, Nathan and Luke were also really blessed by the torrent of gifts…

We do have some photos and will send on to the elf’s ‘soon’ – with one parent at GOSH and the other at home we are not the best co-ordinated at the moment.

But sincerely ‘thank you’.

Update 22nd December 2004

Daniel continues to do well at Great Ormond Street Hospital. They are very pleased about how the transplant has gone, and the graft remains very strong. He remains full of mischief and regularly has us all falling about with laughter. It has been amazing just how good he has been in the isolation room.

He does however still have the adanode virus which means that he is still in isolation and will be until the at least the week after next. It continues just to ‘hang’ there – at the very lowest levels. It does mean that he still has bad diarrhoea but he is now eating again.

So this Christmas Daniel and myself will be celebrating in London with Allison and the other 3 boys down in Romsey. It will be tough but Daniel has already received the very best Christmas present possible – a new immune system.

Update 13th December 2004

Little to update on Daniel…

The key is that the graft holds strong and as reported in the last email is 100% donor…

He had gone 5 days without temperatures but they reappeared tonight. We seem to be on top of his sickness now but he is still nil by mouth whilst he stays on TPN

He remains in good spirits and is becoming a mean card player. He seems happy enough that we will be in hospital over Christmas (unlikely now that we will get out in time).

Update 8th December 2004

Good news on Daniels graft it is 100% donor. The temperatures have come down somewhat from recent highs but the diarrhoea continues. Daniel is likely to go onto TPN tonight as he continues to loose weight. So overall good news, but still someway to go.

For those in the UK u might like to pick up a copy of this weeks ‘Woman’s Own’ which has a 3 page diary piece on us entitled ‘Our Year of Hope’.

Update 2nd December 2004

Daniel is doing well. He has moved into ‘greens’ which basically mean that the hospital considers him ‘post transplant’. This is very good news indeed. His blood counts are very good and he is now producing his own platelets which is very quick…

However he is still suffering from high temperatures (up to 40.5 one night last week) which is caused by the new graft (because it is so strong) and has a bug on board which means that he isn’t eating much (for those of you that know Daniel this is quite unlike him) and he has bad diarrhoea.

Overall the team at GOSH are very pleased with his progress.

Thanks to all that have written to him and sent presents. He is definitely now a ‘Lego addict’.

Update 29th November 2004

Good news on the transplant side. Daniel first showed a white cell count on Saturday and his neutophil count has risen sharply today – so all is well with the transplant – although we know there is a long way to go yet.

Unfortunately Daniel has developed very high temperatures over the weekend and so is on a course of antibiotics to get what they suspect is a ‘line bug’ away. As he is not used to being well he is at times like a wet blanket and then after some paracetamal the old Daniel re-emerges. It does mean very restless nights for Daniel and Dad at the moment….

I had to tackle one of my worst nightmares this morning – shaving Daniels head. Over the weekend Daniels party piece was to pull huge chunks of hair from his head – which he thought was very funny. But last night it started to come out on its own and so this morning there was literally hair everywhere… He now looks very trendy (or so we are telling him).

Update 23rd November 2004

Daniel is doing well and remains bug free. No temperatures, shakes etc. and just a small amount of sickness. His huge appetite has diminished (as expected) but he is still eating and drinking which is of course all positive. He still has not taken a ‘nap’ during the day and Allison reported that he was leaping from one bed to the other last night – quite amazing.

Now the waiting game starts to see the stem cells develop into his new immune system. This can take a few weeks so each morning we will be looking carefully at his ‘blood’ results until we see his white cell count rise above 0.0.

Update 18th November 2004

Transplant went smoothly last night and Daniel is doing well. He is in his isolation room with his teacher working on the computer at the moment.

It was very humbling to see the bag of stem cells infused into Daniel last night – and to think that somewhere out there is a wonderful stranger who has provided this potential gift of life 4 him.

Update 13th November 2004

Just back from GOSH… Daniel is doing ‘fine’ (his favourite word when you ask him how he is) and tolerating the chemo very well at the moment. He has settled in well but moves into isolation tomorrow (Sunday) so will be confined to a room until his new immune system kicks in. Transplant is lined up for next Wednesday (17th) as planned. It is interesting to note how well he is doing compared to Joshua at this stage – one big advantage for Daniel is that he goes into transplant very well. This has positives and negatives for us. The positives include that he is able to eat and drink well. So for example to flush the chemo through he needs to drink 1.65 litres of fluid a day (it’s a lot for a boy of eight) which he has easily done… the negatives are seeing him go in well – on the ‘surface’ there is nothing wrong… With Joshua we have seen an amazing recovery post transplant – with Daniel we need to see him get back to where he is now (but becoming a X-XLP boy).

It is hard for the family at the moment with one exhausted parent at home and another up at GOSH – please continue to pray for us as a family.

Update 5th November 2004

Daniel is now home – the small operation to insert the PEG went without a hitch and he was very brave. It also worked well as amazingly these were the first two nights that Daniel has ever stayed in hospital – so also good preparation. He seems to have the knack of charming everyone around him – he uses the same ‘big eyes’ technique that Puss In Boots uses from Shrek 2 does (indeed there are many striking resemblances)…

So Sunday afternoon we go back to GOSH for Daniel to start his pre medications before his transplant. Thanks for all the encouragement and prayers – as we face the ‘next challenge’ we know we do not go alone 🙂

Update 1st November 2004

Daniel is great at the moment. Tomorrow (Tuesday) he goes into GOSH to receive his Gastrostomy fitted. This will allow medicines and if necessary food supplements to go straight in to his stomach. This worked very well for Joshua. For info on Gastrostomy please check out this page. We expect Daniel to come back home on Thursday before going back to GOSH next Sunday (7th November) to start the pre transplant treatment (conditioning) before his transplant.

One key word for us at church yesterday was not to be complacent. We have seen Joshua come through transplant cured and now wonderfully well and whilst Daniel goes into transplant in great shape (Joshua wasn’t – he had a very high EBV load) we are reminded that this is a very serious procedure and that there are real mortality and rejection risks. We need to maintain the strict regime of cleanliness and importantly we know that prayer is what will bring him home cured. Please continue to pray – give thanks for Joshua but pray hard for Daniel.

Update 27th October 2004

Daniel is due to go up for transplant shortly. We can’t be too exact with dates as it would be unfair to make it too obvious for the donor that they are donating for Daniel. Part of the ‘rules’ is that both parties don’t know who they are donating for and receiving from for 18 months and we wish to abide by this – and protect the identity of the donor. So we will be asking our media friends not to be too exact about
announcing any details of Daniel before his transplant. We are likely to do some limited media ‘stuff’ after transplant day… Daniel will follow the exact same protocol as Joshua.

Daniel had a fabulous time yesterday when he was a zoo keeper for the day at Monkey World (https://www.monkeyworld.org/) indeed this way well have defined his future career… (zoo keeper not monkey)

Update 16th October 2004

Next Monday Daniel goes in to hospital for the day to have his Hickman line inserted.

Update 13th October 2004

Daniel will now go up to GOSH in early November and whilst it is possible that he could be out before Christmas it is more than likely that he will be in over Christmas and in to the early New Year – depends just how things go (could be 6 weeks to 3 months…)

Update 24th September 2004

Josh is in hospital with GVHD, and Nathan may also be admitted with a EVB, as well as this Daniel went to Great Ormond Street Hospital for his pre BMT meds on Wednesday (whilst Joshua was going into Southampton). He did very well indeed and everything looks set for the transplant at the end of October – assuming the other boys are doing well…

So it’s a real battle in the our household at the moment – I think we feel that we can fight one battle at a time but not two or three together… welcome your prayers as always.

Blessings, David

Update 15th September 2004

Next Wednesday Daniel is going up to GOSH for his pre transplant treatment. He is still scheduled to go for transplant around the end of October.

Update 2nd September 2004

Daniel will be starting the Bone Marrow Transplant process in October, and will be requiring lots of TLC through out. As he will have to spend a lot of time in an isolation room, Post Pals is sending him a Play Station 2, kindly donated by Colin Grimshaw. Thank you Colin.