We wish you a Merry Christmas

Its been another month since I updated and such a lot has happened and its all good.

Our holiday in Euro-Disney was just perfect from the journey, to the hotel, to the weather whilst we were at the park and the way we were looked after while we were there.  Ryan had a wonderful time, we all did.  Miles, the owner of Peak Performance Gym that gave us the break, had a friend at the park Karen, and with the help of her friends and contacts within the park she arranged some special surprises for us like a night at the Wild West Show, a personal meet and greet with the character Stitch and a pass which allowed us to avoid the queues - for once we really did feel very lucky and very spoilt.  

When we left Euro-disney Ryan's adventure wasn't over as then it was an overnight stay in a hotel near London ready to meet Father Christmas at Lapland UK - Ryan thoroughly enjoyed the day, helping the elves in the toy workshop, decorating gingerbread men with Mother Christmas, ice-skating and then meeting the big man himself, Father Christmas.  He had been a little nervous, worrying that he would be disappointed and that it wouldn't be the REAL Father Christmas and only a 'helper' but as soon as he left the cabin in the woods his smile said it all - It was the REAL Santa - he has finally met him!!  If you follow us on Facebook you will have seen our photo's but if not click here and here for the Euro-Disney photos and here for the Lapland UK photo's. (I hope the links work!)

Ryan is excited for Christmas, he has been counting down the days, his sack is ready and there are presents under the tree ready to be ripped open on Christmas morning.  Just as it should be.  But for us just being here at home with Ryan feels like a miracle.  With the complications that Ryan experienced things could have been so different and although we don't like to dwell on that it is the harsh reality.

At this time of year our thoughts are especially with the families that we have met over the course of Ryan's treatment, we feel privileged to have met these families but especially to have known their remarkable children.

We wish everyone following our story a very Happy Christmas, cherish all the important things this year and focus on what makes Christmas really special, your family and friends.  

Ryan's even bigger Christmas Surprise!

Gareth, Ryan and I meet Miles Leeson, Beccy Shields and Luke Tillen at Peak Performance Gym in Torquay

Yesterday Ryan met Miles Leeson of Peak Performance Gym Torquay, it is a meeting he wont forget for a long time, as Miles is giving the three of us a trip to EURO-DISNEY! I dont think Ryan quite believes it yet but we go on Friday so it will soon be real for him.

There is a bit of a story behind this amazingly generous gift: Miles ran a 12 week challenge at his gym with a chance for the winner to take a trip to LA to train in Gold's Gym. Unfortunately the winner, Beccy Shields, was unable to take the holiday and so Luke suggested to Beccy that they change the holiday to Euro-disney and offer it to a family through Luke Tillen's charity Torbay Holiday Helpers Network. The charity then got in touch with the CLIC Sargent team at the Royal Devon and Exeter hospital and Ryan's name was put forward....

We found out that our family had been chosen for this wonderful holiday about 6 weeks ago but managed to keep it secret from Ryan, just in case something happened and we couldnt go. We have been having secret phone conversations with Miles and Luke over the past few weeks but yesterday Miles got to tell Ryan that he was going to Euro-disney this Friday! 

For once Ryan was lost for words - he said Thank You lots of times but you could tell it hadnt really sunk in, he has heard his friends talk about disneyland and has asked before if he could go one day but there had never really been the opportunity. Miles showed Ryan lots of photo's of Euro-disney on the website and when he was asked what he was looking forward to the most his reply was 'All of it!' 

In the car on the way home I asked Ryan how excited he was as he was a bit quiet and he said he was afraid to get too excited as 'things dont always work out as planned'. Dear little man has been so just to disappointment but I reassured him that he could get wildly excited as this was definitely going to happen! (And I promise to post some photo's while we are there!)

And now we are counting down the sleeps! 3 sleeps to go!!  Huge thanks to Miles, Beccy and Luke for making our Christmas magical and Ryan's his best EVER!! We really can't thank you enough xx

Ryan's Christmas surprise

Ryan was very excited to find a letter from Father Christmas waiting for him under the Christmas Tree inviting him to visit him in his snowy woodland home in Lapland UK (http://www.laplanduk.co.uk). He is very excited, and a bit nervous!, as has never had the opportunity to go to see Father Christmas before and has spent most of his Christmas's in hospital. Big thank you to the local charity Dream-A-Way for treating Ryan to this magical experience and to my lovely friend Shelley for putting Ryan forward for this Christmas time treat. Another tear jerking experience for me but it will be happy tears!

Next week Ryan finds out another big surprise! 

Another month closer to Christmas!

Well the past month seems to have flown by. Ryan is well and we have only had one hospital visit since the last update, when Ryan had venesection (blood draw to reduce the over load of iron) and immunoglobulin (to support his rubbish immune system).  We now only get blood results every four weeks - when we came home in July it was twice weekly, you get dependent on these results, its like a full MOT and having such a long period between results has taken some getting used to.  The most recent results have all been good - Ryan's ferratin level (which measure the iron overload) and liver enzymes (which reflect the inflammation in his liver due to toxicity of medication and iron overload) are both at a record low since our return home.  Both still have some way to go before they are in normal ranges but we are getting there.  His t-cells are slowly increasing but are unfortunately still very low and not high enough to stop preventative anti-virals and anti-biotics.  His lung function is again improving although the decision was made not to stop his inhaler or the medication for his airways at the moment.  We are just waiting for the latest result from Tübingen to give an up to date picture of his autologous t-cells, the last test was 2 months ago.....

All in all we are experiencing the longest period of normality that we have had in a very long time, which is both wonderful and weird at the same time.  Ryan is enjoying his sessions in school and as of this week he will go into school for 2 hours on a Tuesday and Wednesday morning.  He is joined by a small group of children from his class and really enjoys the sessions.  We have also incorporated the break time into his time at school although he is finding this a lot harder.  He is not used to being in the company of so many children, who are running around burning off some excess energy.  To him the running and chasing is frightening, as he is worried that if he joins in someone may grab his t-shirt and pull his central line.  And so he mostly observes what the other children are doing until a time that he feels happy to get more involved, but small steps in the right direction.

It hasn't helped that he doesnt really know anyone in his year and so when a couple of the boys asked Ryan is he was coming 'football after school' we found out about the after school football club and joined.  When I started this blog I said that playing in a football team is something that Ryan has always wanted to do - and he has always wanted to play on the school football field that we pass whenever we leave our house.  I felt very emotional seeing this become a reality, seeing his excitement running around the pitch, seeing him push himself to keep up with the others and managing to join in for the whole hour long session, he has even scored a goal!  He still amazes Gareth and I, and his consultants too!

It is hard to imagine that this week it is a year ago that, thanks to Make A Wish, we took him to meet his hero Travis Pastrana in Dusseldorf.  He now has the framed signed poster proudly on his bedroom wall.  Such a lot has happened since then and we feel very lucky to be where we are today.  This is the first Christmas in many years that we can actually look forward to, Ryan has spent the majority of Christmas' in hospital. And so this year, thanks to the generosity of 2 wonderful local charities, we are making plans to give Ryan a Christmas to remember, we are keeping the plans secret from Ryan until nearer the time but will be sure to share photo's.

Finally, and on a separate note, the charity that have supported us and that we fundraise with, The NCCA UK, has a national event starting in Exeter with Ryan.  Please come along and show your support to the NCCA UK's Cycling Santa's who start their 800 mile cycling challenge by delivering Christmas presents to children fundraising with the charity, starting with Ryan at the Toby Inn, Middlemoor, Exeter on Sunday the 1st December. The Santa's will be meeting Ryan between 9.30-10 am and we would love as many as possible to come along and see them off and if you needed another reason to get out of bed early on a winter's morning then how about a Toby 'all you can eat' cooked breakfast?  Hope to see you there......

All is well here, Ryan has recovered from pneumonia although still has a slightly lesser lung function than before he became ill but hopefully this will continue to improve in the weeks to come.  He has regained his appetite and put on some badly needed weight so looks and feels good again.  He had his first hour session at school on the 2nd October and really enjoyed it.  It was just him and his tutor working together in a room within the school but it was the closest to school that Ryan has ever had and a huge milestone for all of us.  There were no tears (from either of us) I just felt so proud of him as he walked to school with his tutor.  The following week he was less enthusiastic but still enjoyed the session and has now been four times and seems happy to finally be part of school.  He is joined by a couple of children from his class when possible and this will help him greatly, as working alone can feel like all work and no play! 

Here is the photo I posted on Facebook for those of you that didn't get to see it: 

Ryan still has the same issues that he came home from Germany with: an inflamed liver due to the toxicity of medication -  some of which he still needs; iron overload due to the numerous blood transfusions he has had over the years and still some of his own autologous t-cells lurking around; but none of these things have an impact on how he feels.  His full blood count has been stable and strong for the past few months and his lymphocytes are slowly, slowly increasing which should mean that his immune system is starting to recover.  All in all everything is good.

When we returned from Germany we brought with us a large folder containing copies of Ryan's medical expenses since the start of the year.  These have now been fully audited by NCCA UK, the charity that hold the funds for Ryan's appeal and without the support of whom our lives would have been so very different.  They have confirmed that Ryan's total treatment costs to date amount to £214,007. The total amount shown on just giving is showing as slightly more than this but just giving fees need to be deducted and some monies are showing on just giving but have not yet been received by the charity but the long and short of it is that we are very close to have raised enough to cover the monies outlaid by the charity.  This is a truly wonderful feeling and without the overwhelming support we have had from family, friends and everyone out there that was touched by Ryan's story this would not have been possible.  Gareth and I never dreamed that we would be able to raise this much money since March 2012 and I for one have been brought to tears on a number of occasions by the generosity of total strangers.  Ryan still finds it funny seeing his face on tins and posters in the local area and often points and says to people 'That's me!'  Thank you to everyone that has helped us, from the bottom of all of our hearts.

So what now? Any money raised in excess of Ryan's existing medical expenses, and any money raised in the future will remain in his appeal, to be used exclusively for any future treatment that Ryan might need.   We know that there are treatment options available in Tübingen and the US and that these would cost money.  But when the medical opinion is that Ryan is out of danger of relapse (say 5-10 years time) the money will be used by the charity to pay for the treatment of another child that is in urgent need of treatment outside of the UK.  Gareth referred to it like an insurance policy for Ryan, this is a good way of looking at it.  No one wants to claim on their insurance but everyone needs the security of knowing it is there, just in case.  

No one can predict the future and sadly there are no guarantees that this will be the end of treatment, that the vile cancer neuroblastoma will never rear it's head again.  After all Ryan has relapsed twice and we will never again take life for granted.

So our appeal for Ryan will continue, albeit without the urgency that we had at the beginning but knowing that we are giving him options should be need them. And, in the hope that he never will need more treatment, in the knowledge that the money raised in his name will help give a future to another 'Ryan' in the future.

Ever since Ryan was diagnosed we have learnt to live life by the day and enjoy every minute and that is what we fully intend to do. Here is Ryan today, seeing him looking so well is absolutely priceless:

More good news and a set back....

First the good news: On Monday 16th September Ryan had bone marrow taken for analysis.  Today we heard from our consultants that Vienna have reported that both the bone marrow aspirates and trephines remain clear of disease.  They tested over 4 million cells and found no neuroblastoma.  This is again fantastic news.

Ryan had another venesection on the 12th September and blood tests show that his iron overload is starting to reduce.  Although his inflammation marker (ferratin) is still massively elevated it is a great sign that it has started to reduce after just 3 venesections.

And then the set back; I noticed over the weekend following my last update that Ryan seemed to be getting out of breath on his scooter and running around.  When he was in hospital for his GA the following monday for the bone marrow tests he was given a thorough examination and although his lungs sounded clear, his lung function had reduced compared to his previous reading.  He had no fever to speak of, although was a little warmer than usual and was off his food a bit too.  It was suspected that he had picked up a virus and that hopefully he would improve in a week or so.

Over the next week his symptoms seems to get a little worse in that even going up the stairs at home caused him to become out of breath and sit down to recover his breathing.  I also noticed that his rate of breathing in the night was much faster than usual but still no fever etc.  By last Monday I was feeling really worried and asked to take him in for another examination.  His consultant decided to send him for an x-ray and agreed that he was puffing quite hard even at rest.  He still didn't have a fever to speak of and as long as he didnt do more than sit on the sofa or walk around he seemed ok and was in good spirits.

Ryan's consultant gave it some thought and decided that it was most likely that Ryan had developed a form of pneumonia caused by a parasite, which can be common in transplant patients.  He immediately started him a high dose of anti-biotic and thought that he would need between 14-21 days of treatment depending on how he responded. He has now been on the anti-biotics for a week and in the past couple days does seem to be starting to become less breathless which is great to see.

This set back has meant that Ryan didnt get to attend his first hour session at school but we are really hoping that can be re-arranged in the near future.

It's been quite a while since I last updated and that is partly because I hate writing an update when we are waiting for scan results, I am afraid if I write something positive I will be 'jinxing' the next thing I write but yesterday we got the news we had been waiting for.

Our consultants here in Exeter made contact with the team in Tübingen a few weeks ago and provided them with an update on Ryan and at our request asked if they were happy for the review planned for next week in Germany to be carried out in Exeter/Bristol - they agreed and so we can stay at home.  Ryan was really excited to hear this news although did say 'but that means that I won't get to see my doctors and nurses in Germany'.  We really do have a very good relationship with the team there and have every intention of keeping them up to date with Ryan's progress.  

Ryan has celebrated his 7th birthday and in his usual extravagant style decided on a laser combat party which we arranged for the saturday after his actual birthday.  Then the day before his birthday he decided he wanted to do something on the actual day and so off to Clip and Climb with a few friends we went.  He had a great birthday and really enjoyed his laser combat party despite the fact that it rained the whole time.  We were surprised at how well he coped running around the woods carrying a laser gun but he surprised us all - although was understandably exhausted the following day!

In between his birthday and the party we had to go to Bristol Children's Hospital for an MIBG Scan (to detect the presence of any abnormal cells or bones.) This scan is done over 2 days and so we spent the night in our usual room in Sam's House (a CLIC sergeant home from home) Ryan feels really at home in Sam's house and seemed to quite enjoy being there again.  Yesterday we heard that the scan was clear which, for me at least, was a huge relief.  Nothing can be taken for granted and never will be.

We have also had some results from Tübingen about Ryan's autologous t-cells: after two lost samples the third sample was processed and confirmed that they have reduced from 10% to 8%, another positive step.  

Another really positive step, well huge milestone, is that we have had a meeting with Ryan's school to discuss how he can be integrated into school.  To summarise a very productive meeting between the school, the hospital and myself it was decided that Ryan will not be able to join his classroom at the present time due to risk of infection from the 90 children he would be exposed to in the open style classrooms and the school policy regarding children with fevers.  However the school were keen to include him and are looking to find a suitable room where he can have his one-to-one tutor sessions with this current tutor.  Currently all such rooms are fully utilised but it is hoped that over the next weeks/months this will change and Ryan and his tutor can use a room at the school and be joined by a small group of hand picked children from his class.  He currently sees his tutor 2-3 times a week for an hour and a half each time.  So we have a long way to go before a full day at school can be achieved but at least we are again making small steps in the right direction.

Ryan really wants to go to school and be 'normal' but understands why this is not currently possible.  He is missing his friends now that the school holidays have finished but has learnt to tell the time and plans his day around 'when the kids come home'! Gareth has been back to work since we came home and I try and keep Ryan occupied at home, which is not always easy as we don't like him to be on his x-box too long.  But from a selfish point of view I feel very lucky to have this extra time at home with Ryan and plan to make the most of it. 

Lucky

Ryan clearly isn't afraid of heights!

Lately I have been very aware how lucky we are, so very lucky to be at home with our gorgeous boy.  

It has been wonderful to see Ryan doing normal things, he has been catching up with friends and family, although we still haven't managed to see everyone yet.  He has been enjoying days out at the beach, at Crealy, Pecorama, and the Minature Pony Centre, has visited his cousins in London and Southsea, visited Adam's family, as well as enjoying simply being home.  

He has gained almost 2kg since coming home in June and looks much better for it and his energy levels are slowly increasing.  He is loving his trampoline, his pushbike and scooter and has even been out on his motorbike a couple times.  I feel quite emotional seeing him doing these normal things - he has missed out on so much and we so want him to have a normal life.  It is easy to take things for granted when you start to get into a 'normal' routine but I am very aware how quickly things can change and how quickly your life can again be thrown into turmoil.  

In action on the zip wire

Medically Ryan is also doing well; his liver enzymes, although still elevated, are still gradually reducing, the full body MRI was clear, the CT scan of his lungs had improved and his diarrhoea has stopped.  His full blood counts are all stable in the normal ranges.  

Since the last update we haven't had any more autologus t-cell results as the cells had died by the time they were analysed in the lab in Tübingen and the next sample was not received - a further sample was sent off yesterday and hopefully we will have the results next week sometime.

Ryan has had 2 venesections (the removal of 120ml of blood to reduce the iron levels) and has had no adverse effects.  He is having immunoglobulin every 3 weeks (giving him an essential part of his recovering immune system as he is not yet producing any B cells) but apart from this is not receiving any other treatment.

Next month we are due to return to Tübingen for a review as Ryan will be one year post transplant.  Full re-staging is being arranged in Exeter and Bristol.  Re-staging is a horrid time.  Most of the time I can be rational and only worry about the things I know and not the things that might be, but sadly the fear of relapse never completely goes away.  

But next month is also Ryan's 7th birthday and so we are busy making plans for his party and looking forward to making his birthday a special one.

 

An update on Ryan

Our trip to Tübingen went according to plan with no surprises; we didn't wait for the results of t-cell analysis or virology before heading back for home but the night before we left we were lucky enough to have timed it right so that it was the 'Thursday Parents Meal' but this time the meal was prepared by a turkish family, whose daughter had recently had a haplo identical stem cell transplant.  They prepared a feast of wonderful food and invited the doctors and nurses from the transplant unit.  Ryan stayed up late so that he could try some of the food and was pleased to see his favourite male nurse Luca again.  The following day we managed to squeeze the rest of our belongings, including 2 toboggans, into the car and headed home.

We received an e-mail from Tübingen last week confirming that the HHV-6 virus remains in the leukocytes, adenovirus is still positive in the stool, liver enzymes continue to gradually reduce and that autologous t-cells were stable at 11%.  Nothing new but no real improvements either.  However Ryan's bowels started to improve in Tübingen and have continued to do so which is a good sign that his body is starting to recover.

Whilst in Germany we discussed what happens next for Ryan.  The plan from the start was for Ryan to have the haplo stem cell transplant and then to have 6 cycles of anti-body therapy. The same antibody that he had in Greifswald, Germany in 2011 but with a new immune system.  However the optimum period to start the antibody therapy is between 60-180 days after transplant.  Today Ryan is day +301.

In addition he is the only child whose own autologous t-cells survived the high dose chemotherapy, the conditioning part of the transplant; he is the only child to have had donor lymphocyte infusions which caused grade 3/4 GvHD and on top of that he has an inflamed liver and 2 viruses present in his body.  After thinking long and hard about the implications of this Gareth and I have decided not to take the anti-body treatment.  We feel that the risks are simply too high and that Ryan has been through enough.  We have done everything we can to try and stop this horrendous cancer from returning, his body has had the anti-body before and there is insufficient evidence to prove that he would benefit from receiving it now (even if that were possible).  And so that does mean the end of treatment, again.

The anti-body therapy itself was free, there was no charge for this as it was a clinical trial, although we would have had to pay for his supporting drugs and hospital admission.  However the complications Ryan experienced with GvHD and the extended hospital admissions and investigations have meant that our total medical bill is expected to be in the region of £300,000.  At present the charity, NCCA UK, are working through the paperwork for Ryan's medical care since the start of the year.  We will still need to return to Tübingen periodically; we are next due to return mid September and full re-staging will be carried out at the same time, but the major expenses are now, hopefully, in the past.  

We have been absolutely blown away to see our fundraising target has exceeded the £200,000 mark and are so very grateful for everyones support in keeping our fundraising going while we have been away.

But what does 'end of treatment' really mean? Ryan still has a number of issues requiring careful monitoring by the hospital but hopefully time in hospital will be limited to once a week, sometimes a quick visit, sometimes a long day, but still much more time at home which is exactly what he needs after so long away from home.

Every 2 weeks we will be sending blood to Tübingen in order for them to do the specialist blood tests to monitor the level of autologous t-cells.  It is hoped that in time these will slowly reduce and disappear altogether.

Every week Ryan's liver enzymes will be checked to ensure they continue to fall, or at least remain stable.  This week we will begin drawing blood from Ryan (about 200ml) and replacing it with saline, as this is the standard way to reduce the level of iron in his body.  Lowering the level of HB should allow the deposits of iron in the liver, and any other organs, to leak back into the blood, therefore reducing the liver inflammation. This will be repeated every 4-6 weeks and unfortunately can take anything from 12-18 months. This will mean that Ryan's hickman line cannot be removed unless he wants one rather large cannula's in each of his arms each time - A BIG 'NO' from Ryan in answer to that question!

His electrolytes are also checked weekly and last week we managed to stop the sodium supplement, which means that he is now only taking potassium.  This is a great sign that his kidneys are recovering.  His drugs have reduced to about 12 doses of medicine daily as opposed to almost 50 at the highest point!

His blood will be checked to ensure that it is negative to adenovirus, as while it remains in the stool there is a risk that it could appear in the blood.   

His lungs are also checked and have been improving so in about 2-3 weeks he will have a CT scan.  We are hoping this will show the lungs are clear which will mean that the steroid inhaler and drug to open his airways can be stopped.

Last week Ryan had a full body MRI, as it had been over 3 months since the last one.  Again he was amazing and lied still, covered in strapping and a head brace, for almost an hour. We are awaiting the results.

All in all things continue to slowly move in the right direction and Ryan is certainly feeling better as each week passes.  

He was very upset in the days after his friend Adam died.  We have spent lots of time talking about things with him and answering his questions; we are very lucky that he is so good at communicating his feelings and expressing himself.  Gareth and I usually have to wear a mask to hide our own feelings and so it has helped us greatly too being able to talk about things.

As the days pass the upset has turned to quiet times where he is obviously thinking about Adam but he doesn't always want to talk, and we don't force him as after all no words can really make the situation better.  We have been keeping him busy seeing people and doing fun things and at the end of one day when I asked him what the best bit had been he simply said 'I talked to Adam 3 times today, quietly so no one else could hear' and he gave me a big cuddle and said he felt happy.

Last week Ryan also asked us directly how his friend Jamie was and it felt wrong not to tell him the truth, that Jamie too had died after not being able to wake up from the special sleep that Ryan knew he was in.  We explained that we hadn't wanted to tell him at the time as he was feeling so poorly himself and he had enough to deal with.  He was very quiet again but said he understood why we hadn't told him and was glad that we told him now.  Such a lot to deal with at such a young age but in time we know that he is going to be ok.

Adam

          

We are very sad to tell you that yesterday Ryan's best friend Adam Bird passed away peacefully at home with his parents Nick and Alison holding his hands. 

We respect them both so much for always being able to make the right decisions for Adam and cannot begin to imagine how much the whole family will miss him. Our thoughts and prayers are with Nick and Alison and his big brother and sister Jake and Jessica x x x x 

Nick's blog

                      

Back in Tübingen

Our journey back to Tübingen didnt start well; we hadnt realised it coincided with the end of Glastonbury festival and so the traffic was at stand still several times on the trip to Dover. Then there was another jam due to an overturned lorry.... Needless to say we missed our train on the Eurotunnel and had another hour wait before boarding finally arrived at the parents house at 1.30am instead of between 9-10pm.

Whilst stuck in traffic we received a call from the Klinik asking if we could come for review on wednesday instead of tuesday as they were so busy. And so our first day was a free day and as the sun was shining, and we hadnt previously had much opportunity to enjoy the lovely city of Tübingen, we hired a paddle boat and spent an hour on the river Neckar in the beautiful sunshine.

Today we have klinik at midday and hope to get the results of Ryan's t-cells before returning home later this week. 

It's been ages since I did a proper update but thankfully this time no news is good news.  Overall everything is going well, Ryan is really loving being home and the difference in his energy levels has been noticeable.  It's going to be a very long time before Ryan has the energy levels of 'normal' children his age but the fact that he has wanted to use his new trampoline for 10 minutes most days and sometimes wants to go to the park for half hour once his friends leave school has been great to see.  

For those that like the detail there is a lot to report but I will try and summarise what has been going on:

The week that we found out that we could come back to the UK we also found out that Ryan's liver enzymes had again become very elevated.  This time it was felt it was a combination of toxicity of medication and a condition called siderosis of the liver or iron overload.  Evidence of this was found in the biopsy done in April and together with a highly increased iron marker is now considered to be contributing to the inflammation of the liver.  This has occurred because of the high number of blood transfusions that Ryan has needed over the past years now combined with his own strong blood count.

SO… it was decided to immediately stop the twice weekly IV infusion of anit-fungal, stop the immune suppressant, stop one prophylactic anti-biotic and half the anti-viral medication to a prophylactic dose.  All quite a shock really but we are lucky to be in the position where these drugs can be stopped.  At the time of leaving Tübingen Ryan no longer had HHV-6 or Adenovirus and autologous t-cells had reduced from 15% to 12%.

Ryan got a great welcome home from his local hospital and for the first hour, while we were updating his consultant, we didn't see him as he was off saying his Hello's to everyone.  He is having his blood counts checked every week and unfortunately the  Adenovirus has returned in his stool and HHV-6 has returned in his blood.  His liver enzymes have started to reduce and his blood counts have been stable in the normal range.  His cough has improved significantly too.  We have also been able to reduce some of his electrolyte supplements as his kidney function is returning to normal.

All in all he is slowly getting better.  Being home has been the best tonic, he really is so happy to be home.

Next week we are returning to Germany to discuss future treatment for Ryan (and to collect the boot full of belongings we had to leave at the parent's house!)  All being well we will be home again within the week.

Home for 3 weeks!

Later today Ryan will arrive home for 3 whole weeks! One very happy boy in the back of the car I can tell you. He still has several issues to deal with; some GvHD symptoms, raised liver enzymes again and Autologus t cells but he feels ok and is looking forward to seeing friends and family

Last weekend we got to meet up with our friends for a couple of hours as we hoped and had a day out and about in the black forest.

On Tuesday it was back into clinic for IV medicine and, as a 'low level crackle' could be heard in Ryan's lungs, he also had a CT scan.

On Wednesday Ryan had more IV medicine in clinic and we were told that the the CT showed slight changes since the one they did at the end of March, which was an indication of GvHD.  Not the news that we wanted to hear by any means.  Ryan was therefore started on a steroid inhaler, more prophylactic antibiotics and a drug to open his airways.  

Today it was back into clinic again for more IV medicines and we got the results of the autologus t-cells which showed an increase from 9% last week to 15%.  So a week of disappointing news really, although looking at Ryan we can only see improvements.  He is eating and sleeping well, slowly gaining a little weight and gradually feeling stronger and we are very thankful for that.

We are hopeful that we may be able to return home for a couple weeks sometime in June and will be talking to our consultants here and in the UK next week to see if this weeks developments will change things.

On Wednesday i updated our facebook page with the following update: (don't get too excited as the news changed on Friday).

Last week we didn't get any t-cell results but were shocked to learn that adenovirus had showed up again in Ryan's blood. They repeated the test on Friday and it felt like a long wait until Tuesday when we learned the test was negative again. The virus is obviously trying to rear its head but the t-cells are keeping it at bay at present. We were also told that the virus was no longer detectable in the stool which is the first time in months. 

But the best news was that all t-cells are now 100% donor, no autologus t-cells exist. Now we just want this weeks blood, stool and t-cell test results to show the same great results! 

In himself Ryan is feeling ok, he still gets tired easily but there is still so much going on in his body. But at the weekend we took him to a theme park for a few hours and he had a great time on the rides, and is still a bit of a speed demon! It was a great feeling to see him having some fun.

That was the update i was going to put on here too but then on Friday we got the results from Tuesday's blood and t-cell analysis:

'The adenovirus is negative and autologus t-cells are 9%....'
'Sorry did you say 9% - last week it was 100% donor?.......' 

Apparently, on closer inspection, errors were found in the results and the analysis had 'failed' or so they think. It is a sensitive test and we knew it could sometimes 'fail' but still. 
And so 9% is an improvement on the result of 11% 2 weeks ago so we have to be thankful for that. 

As Ryan's GvHD symptoms are under control the immune suppression has been reduced again and so we simply have to wait until the end of next week for the next results. I forgot to state in the last update that the virus HHV-6 remains in the leukocytes but not in the blood plasma.

We have had some hospital free days but the weather has changed so we haven't been up to much. Tomorrow though we hope to meet up with friends who our touring the area on their motorbikes, weather permitting.  Not posted a photo for a while so here is a recent one:

Ryan has felt quite well the past week and has been a bit more active, like walking from the room to the car, from the car park to the hospital, nothing spectacular but an improvement none the less.  He had 2 sessions of ECP (extra corporal photophyreresis) which makes him feel tired for the rest of the day but other than that seems to have been feeling ok, in fact better than he has in a long while.  The ECP treatment had been 2 sessions every fortnight but will now be 2 sessions every 4 weeks.  We are slowly reducing the immune suppressant and Ryan's t-cells are increasing without any adverse effects, at present anyway.  His blood counts are also increasing and his white blood cell count and neutrophils are now stable in the normal range for the first time in what feels like years.

This is obviously great news but although I am happy about this, I don't feel happy.  I have an overwhelming feeling of sadness because of circumstances that two families close to our hearts find themselves in.  I have thought so much about what I want to say here in this blog as saying nothing feels wrong, it has kept me awake at night, but it is simply too painful to truly convey how upset we feel about what is happening to both families.

Ryan has been very close to his friend Adam since meeting him in Greifswald in 2011, the boys had a connection right away and this has remained despite the fact that they don't get to see each other very often.  Adam has had to return home from the US due to complications with treatment.  His disease has not responded.  There is no more treatment out there.  In himself Adam is doing ok, he still has his defiant streak, his quick witted put downs to his Dad and Gareth if they monopolise FaceTime.  He and his family are constantly in our thoughts. 

During our time in Tübingen we have become friends with another family who are now in similar circumstances. This weekend we went to Munich to meet up with Tobias and his family and another family that we have met here.  Tobias relapsed a couple weeks ago, his treatment also failed after what looked like really promising news a couple weeks earlier.  Tobias is 12 and understands his situation, again there are no more treatment options.  I can't really put into words the courage that he has shown dealing with this news, he has a list of things he wants to do and has had a busy few weeks.  This Saturday he watched a football match in Munich stadium, something he has wanted to for a long time. 

Please keep Adam and Tobias, and their families, in your thoughts and prayers.

It's been an eventful week again; I wrote the last update while Ryan was having ECP, he was having a lesson with this teacher and feeling fine. Within minutes of posting the update things changed suddenly.  Ryan eyes were wide and he started to wretch, he went as white as a ghost and he lips went blue.  We called for the nurse and she took his blood pressure which was dangerously low.  She called for help and 2 doctors and more nurses came rushing in.  His bed was tilted backwards so his head was lower than his heart and we managed to keep Ryan awake by talking to him while the doctors interrupted the ECP machine and made it start to return his blood quicker than it had planned to.  Apparently 'this sometimes happens' as too much of Ryan's blood was in the machine and not in his body causing his blood pressure to plummet.  As soon as the blood was returned he started to regain colour and his blood pressure started to improve.  Panic over.  

He was tired for the rest of the day and then during the evening and night his temperature started to rise and he woke several times with diarrhoea.  We spoke to the doctor in the Day Clinic the next day and he said that provided that Ryan was ok in himself we should just monitor the situation and 'see what happens'.  As the levels of steroid had been reduced earlier in the week it was not a complete surprise that Ryan would get a temperature and the upset tummy could be a flare up of GvHD.  It was the first time we had 3 hospital free days since January and so none of us were keen to go into hospital unless it was absolutely necessary, and as he was well in himself we just stayed at the parents house.  However on the Tuesday morning it was clear that Ryan was not right, he woke with a headache and felt sick and was quite sleepy.  When we got to clinic as planned for an infusion of anti-fungal medicine he fell asleep.  His blood pressure was fine, his blood results showed he had a raised infection marker but nothing more than that. We talked through the last few days with the doctor and he suggested that Ryan needed a higher level of hydrocortisone due to possible poor absorption due to the diarrhoea.  We gave him a higher dose and within an hour he was his usual self!  The temperature and diarrhoea have both settled again and tests on the stool have shown no pathological cause.

The next day, Wednesday, they suggested we had a check up to see what the infection marker was doing and it had halved.  However they then had the results of the previous Thursday's t-cell analysis which showed over 30% of t-cells originated from Ryan and so they urgently got the results of the test done the previous day which showed 12%.  It is therefore possible that the fever and diarrhoea over the weekend were a result of the two immune systems 'battling'.  No one will know for sure, but either way the increase in autologus t-cells is not a good thing.  However as Ryan was again feeling well the consultant was able to reduce the immune suppressant, Cellcept, by 25% to try and allow Ryan's new donor derived t-cells to become more active and overpower his original ones.  We have to wait until Monday before another analysis of the t-cells is done and hope to get the results soon after.

All in all the past week started badly but has ended with Ryan feeling ok again.  We have no hospital this weekend and hope that he will continue to feel ok although he still has very little energy so we won't be doing very much.  What we really need is to see the back of the autologus once and for all, but at the same time we don't want a flare up of GvHD - such a fine balance to get things right and such a long time to get there but it will be worth it.

When I start to write these updates I always try to keep them short, but that never seems to happen lately. Ryan has had so many different issues going on, all of them equally significant.  But gradually Ryan's condition is improving.

Since the last update his liver enzymes continue to slowly reduce, indicating that the inflammation due to toxicity is reducing, his blood pressure medication was stopped and his blood pressure has remained within the normal range, his electrolytes have been stable without the continuous pump, the oral medication to supplement electrolytes is slowly being reduced, his hydrocortisone is now back to the normal level and the autologous t-cells (originating from Ryan's original immune system) have reduced from 13% to 6%, and as at Monday's blood test there are no viruses in his leukocytes or blood plasma - all of these things are huge steps in the right direction.  

Loosing the pump has given Ryan a real boost, he has the freedom to move around although not the energy to do so.  He has lost a total of 2.5 kg, he never had much fat to start with and so most of the loss is muscle.  He is painfully thin and due to the GvHD of the stomach we have had to give him low fat food.  Now though his stool has returned to normal and so we can start to introduce foods to help him gain weight, it is a case of try it and see what his stomach thinks of it.  

We have come to realise that the recovery from GvHD is going to be a slow process.  We don't see improvement in Ryan daily but as each week goes by he is better overall than the last.

His mouth and skin still show signs of GvHD although both are much improved. He still has the HHV-6 virus in his mouth which causes some inflammation and blisters but surprisingly these don't bother him too much. He still has a cough too, it now sounds very loose but there is nothing in his lungs, the doctors think it could be inflammation of the mucosa (lining of the throat) but the cause is not known.  As he has no fever or infection marker it is currently not being further investigated (as the original CT scan was clear and his lungs are listened to daily) 

In himself Ryan is a lot happier than a few weeks ago, the visit from his auntie and cousins had the desired effect and really cheered him up, and although he still desperately wants to go home he has stopped being tearful about it.  The weather has improved and the sun has come out and so it hopefully won't be too long before he feels up to doing some gentle activity outside.  

Life is a roller coaster......

A few days after my last update we had some unexpected bad news during a routine clinic visit: the Adenovirus and HHV-6 viruses had both reappeared in the leukocytes as at the blood taken 1st April (within the white blood cells). 

A few weeks ago we learned that the company that manufactures the only drug available to contain the virus has had to stop production due to microbiology problems and it was not available for the foreseeable future.  This is a worldwide problem and has very serious implications for patients with the virus in the blood.  After discussions with the consultant here Gareth and I were tested to see which of us had the most effective t-cells against the virus and yesterday Gareth had about 500ml collected to be prepared ready to be given to Ryan should the copies of the virus in the blood increase.

In addition we were shocked to learn that although there are now good numbers of T-Cells 13% of these were now Ryan's.  Apparently they had first appeared at the end of February and had been increasing 'a couple percent each week'.  I think the doctors in the day clinic had thought we had known this already and so had not mentioned it, they said it was very unusual to see reemergence of autologous t-cells with active GvHD and could not explain it other than to say Ryan's were 'incredibly strong'. 

At this level they are not a huge threat, but obviously have potential to be a massive problem.  Currently as the GvHD of the gut and liver has improved and the mouth and skin are improving slowly we can start to reduce the remaining immune suppressant and so on Monday we reduced hydrocortisone from 3 x 15mg to 3 x 10mg (Ryan's usual dose is 3 x 5mg). 

The only adverse reaction so far has been an increase in temperature and so hopefully next Monday we can reduce again.  Then the remaining immune suppressant, Cellcept, will start to be reduced.  The reduction has to be done slowly as doing this too quickly could allow the T-cells to become too aggressive again and trigger GvHD.  It is a balancing act, with huge consequences either way.  The outcome we are looking for is obviously for the immune suppressant to be gradually weaned off allowing the donor derived t-cells to destroy the  autologous t-cells and viruses but not causing Ryan problems.  

On Wednesday afternoon we finally got the results of the outstanding virology tests of the liver biopsy which confirmed that the inflammation of the liver was caused by toxicity of medication.  As there was no evidence of virus infection this meant that yesterday Ryan was not given the daily 2 hour IV infusion of anti-viral medicine.  This is very toxic and makes Ryan feel tired as well as suppressing his bone marrow and we were pleased that this could stop.

We also got the results of the re-staging scans and were thrilled to learn that both the full body MRI and MIBG scan showed no evidence of disease.  Ryan has bone marrow tests planned for the week after next for completeness. 

And today we got the results of this Monday's blood virology and were really pleased to learn that the ADV virus is again gone for the leukocytes, although the HHV-6 virus is still present.  The adenovirus is however still present in the stool.  

This week Ryan has had two 4 hour sessions of ECP (the UV light treatment for GvHD).  It usually makes him feel very tired but he has fought off the tiredness well as his auntie and cousins are here visiting. Despite spending so much time in hospital it has really lifted his spirits to see them all which is just what we were hoping.  

For the past few days the doctors have been reducing the levels of electrolytes in the portable pump.  It is the only way to see if the kidney has recovered as the levels will not become high, they will just become stable within the normal ranges.  Since the reduction most have remained stable within normal ranges although a few more tablets have been added until we are sure that they are not needed intravenously.  Today the pump was disconnected and the next few days will show whether his kidneys have stopped 'leaking'.  Ryan is so happy to be free and not connected, it is the first time since being admitted to hospital on the 1st February that he can move around freely without worrying about being connected and it is truly lovely to see him enjoy his freedom.

Improvement

Things have improved here since my last update. Most noticeably Ryan himself. The increase in hydrocortisone has greatly helped the GvHD of the skin and he has walked more this week than he has in the past month. There has also been an improvement in his appetite and he has started to feel hungry at times and is eating willingly again. His stool also seems to have begun to improve, hopefully this improvement will also continue. 

The liver biopsy didn't reveal any signs of infection or GvHD and is indicating toxicity of medications. We are still awaiting final results of virology tests, which will be a few more days, but it is hoped that the liver will gradually recover by itself. One of Ryan's medicines which is particularly toxic to the liver was stopped as soon as the enzymes started to rise, over the past few days these have started to fall and will hopefully continue to do so. 

The other change to medication is that Tacrolimus (the immune suppresent drug that is most effective against GvHD) was being gradually reduced and has now been stopped and replaced with a less aggressive immune suppresent (Cellcept).  This will hopefully mean that Ryan's blood pressure and kidney problems should start to improve - in fact just days after stopping the drug his blood pressure medication has been reduced from 4 times daily to twice daily which is a really positive sign.  Once his kidney's start to function properly he will no longer need the continuous infusion of electrolytes.  For Ryan this will be a huge benefit as the bag contains one litre of fluid when it is replaced each day and is simply too heavy for him to carry around in the special backpack provided with the pump.  Fingers crossed we will see this improvement soon.

The IV anti-virals will continue until the full virology is reported which means that Ryan has a 2 hour long infusion each day when we come to clinic for blood tests and a new bag of electrolytes. At best we spend 4 hours in clinic. Ryan is totally fed up of hospital, he hasn't had one hospital free day for over 2 months and it is taking its toll. He is not his usual happy go lucky self and is tearful about being connected which is most unlike him. Some days, when additional medicines are required, we can be in clinic for as long as five hours, combine that with a scan or ECP therapy and the whole day is gone. 

But next week Ryan's eldest cousins arrive for a visit with my sister Jackie and this is something that he is really looking forward to.  Fingers and toes crossed that the improvements we have seen this week continue and he enjoys his time with his cousins, despite the fact that most of the days will be spent in hospital.

A lot has happened in the ten days since my last update.  Some significant good news in that the adenovirus that was in the blood has gone (as at the test taken 18th March) although it remains in the stool.  However the HHV-6 virus remains in the leukocytes.

Ryan developed a cough a couple weeks ago, a dry non-productive one, but as it continued a CT scan was done as this could be a sign of GvHD of the lungs.  Nothing showed on the scan but the cough is no better and a further CT will be done if it doesn't resolve.  His skin has also continued to get worse, especially the palms of his hands and soles of his feet which are bright red and inflamed, so much so that it is often too painful for him to walk.  This is being treated with steroid and immune-suppressant cream but isn't improving. His arms and legs are also covered in a red bumpy rash and really looks quite awful.

Unfortunately Ryan has continued to have blood pressure problems, needing an echocardiogram to check there was no fluid around his heart. His liver enzymes have become elevated again and not stabilised resulting in an ultrasound to check for veno occlusive disease and then, as the enzymes continued to rise, an emergency liver biopsy yesterday.  Ryan needed to stay in the ward overnight as there is a significant risk of secondary bleeding, luckily there are no signs of this having happened.

The results of the liver biopsy yesterday won't be known until next week due to the Easter break.  The consultants believe the cause of the problems to be either a virus attacking the liver or a return of GvHD although have said it could be toxicity of medication or even a relapse of neuroblastoma.... To try and stop further inflammation of the liver Ryan has been started on IV anti-virals again and his hydro-cortisone dose has been increased.

On top of all this we are at the time for the dreaded re-staging which means full body MRI and MIBG scans.  The hour long MRI took place earlier this week and the MIBG scan is scheduled for next Wednesday/Thursday.  Bone marrow tests may also be needed.

All in all it has been a rubbish couple of weeks for Ryan and a stressful time for Gareth and I.  My parents arrived for a visit this week and we had hoped to spend time with them at the apartment they rented but that hasn't happened as the majority of time has been spent in hospital.  But Ryan has enjoyed seeing his Nanny and Grandad and hopefully once he is discharged today he will be able to see a little more of them tomorrow and before they leave on Sunday, although he still has to spend a few hours each day in clinic.

Ryan has managed to sleep at the parents house since being discharged to the day clinic last Wednesday.  It has meant a trip into clinic each day, the first morning after being discharged we had to be in at 9 and were still there at 1, other days have been shorter visits although nearly always an hour or two.  

Ryan blood counts seem to have stabilised again, and the need for GCSF (to stimulate the white blood cells) is reducing.  His platelets and HB, both of which are effected by ECP, have also stabilised but its possible that he will need transfusions of both next week when he has ECP again.

We had good news on Friday, based on the previous monday's blood test, that the level's of adenovirus in the blood plasma had reduced to 'less than 1,000' and the HHV-6 virus is now only in the leukocytes and not in the blood plasma.  Both of this is great news and shows that Ryan's bone marrow is slowly recovering and fighting against the viruses, together with the anti-viral medications.

Ryan is managing to eat sufficient to maintain his weight, his appetite still isn't really there yet but he understands that he has to eat if he wants to stay out of hospital.  His tummy is still effected by GvHD and so we have to be careful what foods he eats but he has found some things that both he and his tummy like.  

As the levels of the immunosuppressent in his blood reduce the GvHD of the skin has become more noticeable again.  His skin looks 'irritated' more than anything.  Sometimes a rash is clearly visible, then other times it is gone again.  His blood pressure caused problems again this weekend, causing him to wake up with a headache and nausea and as soon as we got to clinic he was sick and then fell asleep.  Apart from this he has mostly felt ok since leaving hospital, he is still exhausted and very ready for bed at 7.30pm,  in fact if it wasn't for the schedule of medicine that runs from 7am until 7.30pm he would be asleep much earlier i'm sure.

Here is a photo of the tablets he is taking each day - in addition to this there are 8 syringes of medicine and a steroid mouthwash…..

We had expected an overnight stay in hospital yesterday (for the drug meant to contain the adenovirus) but when we arrived at the ward at 9am laden with suitcase containing the x-box and clothes and bags of food and medicines we were told that an emergency admission during the night meant that Ryan's room had to be allocated (although not sure why no one rung us) and so we are now expecting to be admitted tomorrow……

Today Ryan is being discharged to the parents house here in Germany.  Good news I know but for some reason I feel more anxious than excited.  Now he has to eat to maintain his weight, now he has to drink a minimum of 600ml a day.  He has a continuous infusion giving him the essential vitamins and minerals that he is loosing through his kidneys, via a portable pump.  He is far from being well but he is pleased to be leaving hospital after 6 weeks, although the doctors and nurses have all been fantastic.  Every day we will need to come into hospital to the day clinic for a new bag of fluid, for IV antibiotics, GCSF and blood tests.

On Monday we were told that the adenovirus that was in the stool has now also been found in the blood.  The blood test was taken last monday, and at that time the levels of virus were classed as low.  This is a huge concern, as this can be a very aggressive virus, and Ryan's t-cells (which are needed to fight the virus) are still being very suppressed by the numerous medications he is on.   The result of virology tests take several days to analyse and so you only know the situation days ago and not the current situation. 

Ryan blood counts, that had been stable, have started falling again.  We are struggling to get the levels of the immunosuppressant drug (tacrolimus) right, we have to reduce this drug slowly but currently it is still having too much of an impact on his blood counts.  He has need a blood transfusion and platelets in preparation for a further treatment of Extra Corporal Photopheresis (he now has two 4 hour sessions a fort night).

The doctors therefore took the decision to stop the daily IV anti-viral, as this also has an immune suppressing effect, and have further reduced tacrolimus.  Over the course of the next week this should allow the t-cells to multiply, they simply have to.  There is obviously a risk involved in stopping the anti-viral as this targets the HHV-6 virus that is also present in the blood.  The other worry is that if the T-cells multiply too quickly the GvHD will flare up. 

He has been having a drug targeting the adenovirus, every fortnight, and this involves an overnight stay in hospital as he has to have fluids before and after the drug to flush it out of his body.  His next treatment is next Monday and so he knows that he has to come back into hospital then but just for one night. 

Ryan has been feeling very homesick lately, he is tearful at some point most days, the last thing we need is for to get discharged and then to have to be re-admitted and so we are not making too much fuss about him staying at the parents house.  As you can imagine this is a very stressful time; I am trying to stop my insides freaking out but end up feeling like I am holding my breath.  We are trying to focus only on today, deal with what we know today, how Ryan feels today and not think about anything else.