Our Pal - Luke H

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 (http://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 (http://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…