Hi there

All is well in the TeamHartley 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 spending some time together (although it will be some time before we can leave them for any period).

Rather than bung up your inboxes we thought that it might be more friendly to ask you to download the latest newsletter from the XLP Research Trust, the charity we run. In the newsletter is an update on the triathlon, news on literature we are publishing about XLP and also some easy ideas to fund raise at no cost to yourselves. Please download ‘Summer08’ from this page:-

http://www.xlpresearchtrust.org/literature.asp

Thanks again for your love and support.

Blessings

David

Hi there

Wednesday will be a historic day for us as Luke will be able to return to his school after 14 months away. His immune system has recovered sufficiently that the medical team looking after him agreed that he could go back part time. Naturally he is very excited indeed! This hopefully now marks the near end of Luke’s trials – we’ll let you know how the day goes.

We also had a positive diagnosis for Joshua. Fortunately he does not have lung GVHD as initial proposed (which would have meant another protracted course of steroids) but LIP (Lymphoid Interstitial Pneumonia) which should be treated with a 2 week course of steroids plus a 4 week course of our favourite drug Rituximab (Joshua has some EBV in his lungs). The hope is that his lungs will be back to normal within a month!

And Joshua had a new Hickman line successfully inserted last Tuesday and started his treatment of ECP (Extracorporeal Photochemotherapy) at St Thomas hospital in London which went well. This will be an initial 6 month course of treatment meaning a two day visit every two weeks.

So things are moving onwards and upwards. Well almost, just as we start to see the possible end for the boys their Grandmother (Allison’s Mum) had a knee replacement operation this afternoon – Allison is there now - as though we haven’t seen enough of hospitals.

Thanks for your ongoing support.

Blessings

David

Hi there.

Luke came home today.

He is now very well, no sign of temperatures and gradually starting to eat solid food again. But it’s been a difficult week. 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 stablised 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 tat 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.

Blessings

David

Dear all

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.

Note to media – please do not report anything in the next paragraph. It looks like we will also have to find a new donor. Nathan is seen as ‘too perfect’ for Luke and the only way we would not see a repeat of what happened this time would be to use extreme conditioning on Luke – which we would want to avoid unless we have no choice. This time around in addition to Nathan’s bone marrow, there was also some bone marrow from Nathan’s wonderful donor given to Luke. This was to ‘back up’ the bone marrow that Nathan gave. Nathan’s donor came from a non UK bone marrow registry which has very strict criteria including that a donor can only give to one patient but fortunately they did change this for Luke’s transplant. What seems to be the best option now for Luke is to use this donor again but to go through the same successful conditioning regime that Nathan went through (i.e. stem cells) but it looks unlikely that this overseas bone marrow registry will allow, this although the team from GOSH will make the case. At this time there are no further good matches for Luke on any of the bone marrow registries worldwide.

Blessings

David

Hi all

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

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……..

Blessings

David