Ryan's story

Ryan was diagnosed with Stage 4 High Risk Neuroblastoma N-MYC amplified in July 2008. He was out of treatment for just over a year when in November 2010 our lives were again turned upside down when we discovered the disease had returned in his bones and bone marrow. After a year of treatment re-staging scans in November 2011 showed that he had again achieved remission.

However just 3 months later, in February 2012, end of treatment scans gave us the devastating news that the disease has returned, this time with a tumour near his spine. In March Ryan started 4 weeks of radiotherapy followed by chemotherapy which is on-going. Re-staging scans in May and August showed no evidence of disease. On the 4th September 2012 Ryan received treatment in Tubingen for a haplo-identical stem cell transplant. He is a happy, active boy who is now enjoying life to the full.

Monday, 29 October 2012

It's not good news

Today's clinic appointment did not bring good news. Ryan's counts have all dropped significantly. His WBC is now only 0.5 when only a week ago it was 4.79, his neutrophils are 0.2 and HB and platelets have also dropped. Not the news we wanted but what we were trying to prepare ourselves for.

Gareth had a series of tests and examinations today, along with 24 phials of blood taken to check he is fit and healthy enough to be the donor. He will get the results tomorrow and I think he is pleased that he can finally help although didnt want these to be the circumstances.

Plan B as it stands at present is to admit Ryan to the transplant unit on monday to begin re-conditioning. This will involve a regime of chemotherapy and radiotherapy to his lymph glands, the latter targeting his t-cells. Ryan having to have more treatment is what upsets me the most, i cant even begin to put into words how i feel. I think part of how i feel is due to the fact that it was my stem cells that havent done the job that was intended, I know I am not to blame but still cant help that feeling of utter disappointment.

However Plan B will not commence until everyone is certain that the T-cells infused on the 17th have had opportunity to establish themselves and so a blood test to determine the origin of Ryan's t-cells will be done on Friday and urgently on Monday. The results usually take 5 days but I believe they will be expedited.

In himself Ryan remains well, still an x-box addict but we managed to steal him away from it on sunday and took him to the mountains to visit a castle where there was loads of snow! We had a tour and saw the armoury and the crown of the Prince of Prussia and Ryan seemed to find it interesting. After clinic today we went to a park and fed the ducks - always a favourite and Ryan scooted around the lake oblivious to everything going on. Exactly as it should be.

Friday, 26 October 2012

The waiting game continues...

Ryan counts have responded to GCSF and so have not dropped further. Nothing can be read into this, but it does mean there is still a glimmer of hope. In the meantime plans are being made for gareth to be the donor but we will not be using the frozen stem cells in the interim period. Assuming he continues to reject the stem cells Ryan will start re-conditioning on 6th nov but the full details of what treatment he needs cannot be decided until nearer the time, it depends on the level of bone marrow function, if any. So the waiting game continues and next blood count is monday.

In the meantime plans are in place for Gareth to undergo the necessary tests for him to be the donor, starting with blood tests on Monday....

Thursday, 25 October 2012

Our worst fear.....

We had another clinic appointment yesterday and it's not good news. All Ryan's counts have dropped again. The doctors say there is now only a slim chance that Ryan will not reject the stem cells. This had always been our biggest fear. 

The reason for this is that somehow some of Ryan's own T-cell survived the conditioning treatment and are essentially attacking the bone marrow that originated from the new stem cells, and are destroying it.  The centre here in Tübingen have been doing haplo-identical bone marrow transplants for over 15 years and we had been told that for other cancers the host rejects the donor stem cells in 10% of cases.  However they have been doing haplo-transplantation for neuroblastoma for over 2 years and have not have a rejection for the 30+ children that have so far had the treatment.  Why oh why does Ryan have to be the exception the rule again!?! 

The doctors and consultants here need time to get together and discuss the best plan for Ryan.Yesterday they gave him GCSF to try and force the new stem cells to reproduce.  The T-cells that were infused last week will not be effective until next week at the earliest, the hope was that they would be stronger than Ryan's T-cells and be able to destroy them before they destroy the new bone marrow. However now there is only a very small chance that the new bone marrow will survive long enough for my T-cells to establish themselves enough to destroy Ryan's, it is only a tiny glimmer of hope.

However there are options, and the doctors say there are good options.  One option would be that Ryan be given back his own cells however although Ryan has a back up of his own cells but they were collected when he had 20% contamination with neuroblastoma cells and so we never want to use these. He also has a small number of his own stem cells that were collected when his marrow was clear. These are not enough for a successful transplant but may give him some bone marrow function.  This option would only be used should the powers that be decide that they want Ryan to go through the conditioning again and then for Gareth to be the donor.  The other option would be that Ryan is re-transplanted with my stem cells.  He would need immunological conditioning to destroy his surviving T-cells and should not need chemotherapy.  But at the moment this is all just hypothetical as the decision will not be ours. But we will ensure that we understand fully the reasons behind any plan that is made.  We may get a plan tomorrow but it may be early next week.
We are obviously devastated, and very shocked by this news.  I swing from feeling really upset to really pissed off.  The thought of putting Ryan through more treatment when he already had more than 99% people would have in a lifetime breaks my heart, it really does. But we have to choice as he without a functioning bone marrow he cannot survive.  
Ryan is totally oblivious to all this stress, he is happy and active, eating, drinking and sleeping well and he looks great.  He is the heaviest he has ever been and is really enjoying his food, although still only really wanting my home made spag bol most nights! The likely hood is that he will very soon have to be admitted back into the bone marrow transplant ward and so we have tried to explain to Ryan that his body is fighting mummy's new stem cells and that the bad news is that his body is winning.  We have told him the doctors will have a plan but that he will need to go back into isolation.  His first concern was would he need to have a wash every day? and his next was that he had better not have a small room like he did the first time!  
Anyway onwards and upwards! What other choice is there, sitting in a corner and crying isnt going to help Ryan one bit and wont do me any good either so after we heard this devastating news yesterday I went food shopping, just like I had planned to do before I found out.  It did take a little longer than usual as I kept forgetting what I was looking for but hey.
I promised to try and upload the video of him screaming all the way down the roller coaster and here it is for your enjoyment but best not have a cup of tea in your hand as you might spill it laughing!

Tuesday, 23 October 2012

Playing the waiting game....

I hate waiting, thoughts of what might happen are sometimes hard to keep at bay.  But we have been keeping as busy as we can and making the most of Ryan feeling well while the sun is shining.

After clinic on Friday we came back to the parent’s house and there were some children playing at the house, 2 girls which were sisters of children that were having treatment.  Ryan didn’t waste any time and didn’t need the usual encouragement to join in.  He ran outside and didn’t stop playing for a good couple hours, scooting, playing football and running around the house and garden.  It really was great to see him being so active, the most active that he had been in absolutely ages.

Saturday though he woke up complaining that his leg hurt when he walked, my stomach always turns when he complains of pain.  But realistically he had used his muscles more the day before than he had in weeks and so we tried to encourage him to try and walk properly when he needed to but he spent all the morning playing x-box on-line with friends and sure enough his leg improved (and I could breath again).

On Sunday any aches and pains from the day before had gone and the sun was shining so we told Ryan we were going out for the day.  Gareth had been looking on the internet for something to do and had found that the second longest dry toboggan run in Europe was within 2 hours drive.  This is the website www.hasenhorn-rodelbahn.de if you are ever in this area! It really was worth the drive just to see Todtnau, the beautiful village that it was in.  The toboggan or coaster was set on the side of a mountain, we caught a chair lift to the top and the views on the way up were spectacular. If you can zoom in on the photo you will see Gareth and Ryan.

Then the coaster took you back to the bottom – 2.9km of railed tracked to be precise and Ryan screamed all the way the first time down.  It took about 4 minutes even going ‘flat out’!  Gareth and Ryan were together with me trying to keep up behind, it was great fun.  Gareth videoed the ride and we will try and upload it to YouTube – but be warned there is lots of screaming, Ryan did enjoy it although he does sound a bit terrified at times!  He loved the second time more I think when he knew what to expect.


Then yesterday it was back to reality and a clinic appointment at 9.30a.m.  Ryan blood counts have all dropped.  Not plummeted, just dropped.  They are allowed to drop, they usually peak and trough apparently but the question is are Ryan’s dropping because of the situation that he is in or are they behaving normally and dipping before they pick up again?  No one can answer this question today – we have to wait until we get the next blood results on Wednesday……..

Vanessa is back at the house today with her mum and dad as she has scans and tests this week to see if she can start the next phase of treatment.  Ryan didn’t get to meet Vanessa in person as he was in isolation when she was at the house and was unusually shy when he saw her yesterday morning but soon got over that and has already introduced her to the joys of minecraft!

Wednesday, 17 October 2012

I spoke too soon

After writing yesterday's update we had some unexpected bad news, which has been confirmed today.

The dynamic of Ryan's t-cells have changed so that the majority are now his. This is a concern as his t-cells could be capable of causing his body to reject the new stem cells. However usually when the majority of t-cells are the host's the normal blood counts are low and not stable but Ryan's counts are still all great. So at present he is an exception to the rule but this may change.

The doctors are concerned but not panicking, they have seen this before but cannot predict the outcome or how long it will take to try suppress Ryans T-cells.

Yesterday the steroid and immune suppressant drugs were stopped to 'take the brake off' the new cells. Today Ryan was given an infusion of my t-cells which were frozen in the lab. (I wasn't even aware there were any frozen!) This will be repeated again every 2-4 weeks to try get control again. The flip side of this is that being off the steroid could trigger the fevers to return and the new T-cell activity could trigger Graft Versus Host disease. We will be monitoring him very closely.

This will delay immunotherapy and our return home but we can deal with both of these things.

We are in the best place to deal with this situation. Probably one of the best places in the world. We trust the doctors and know that they will be closely monitoring Ryan. But equally they respect us and dont hide anything from us.

We are obviously very concerned but will deal with this day by day and not assume the worse. There is a plan b, if plan b is needed.

Ryan is looking really great and i think is feeling less tired. Today he was a little upset that Jamie was going home, albeit only for about 10 days, and he wanted to know when he could go home too. So this afternoon we had to tell him we couldn't go home as soon as we thought, in fact we didnt know when we could go. We expected him to be upset or even kick off but instead he simply said 'That's fine, dont worry, after all there's nothing stopping people coming to see me is there!' We had to explain about school etc but you have to love his attitude. His spirit really does keep me going.

Tuesday, 16 October 2012

There really isnt so much to report at the moment - which is usually a good sign.  Ryan seems to be  gradually getting stronger and willing to do a bit more outside of the parents house.  I have realised that one of the reasons that he doesnt like to go out is that he doesnt like to wear his mask.  It has to be said that people stare.  I am sure they are wondering why he wears it and whether he is contagious!  It actually suits us that people move away when he is playing outside so we just smile at them.

Ryan continues to attend Day Clinic twice a week to be reviewed by a doctor, have his weight checked and routine blood tests.  This week the plan is to try and reduce the steroid that was prescribed to stop his high temperatures.  It was originally 20mg twice a day and this week we are down to 7.5mg twice a day so it's a case of monitoring his temperature to see what happens.  If his temperature goes above 38.0 we have to take him into hospital so that bloods can be taken and he can be seen by a doctor - as annoying as this is to us it is not safe to assume that a rise in temperature is purely down to the reduction in steroid as Ryan is obviously very susceptible to infection at the moment.  We discussed this with the doctor and he said that providing his blood tests did not show any infection and his infection/inflammation marker was not raised then we could simply increase the steroid and try to reduce it again the following week.

Since the last update we have taken Ryan to a dry toboggan run for half an hour - he had great fun zooming down the run screaming to his Dad to go faster and 'don't brake!' - good to see that he hasn't lost his davedevil streak!  He even had a go with me and said 'Mum you weren't as fast as Dad but you did great' - high praise for me then!

Then the following day, as the sun was shining (and the wifi was down at the parents house), we drove down towards Lake Constance, in Switzerland but as Ryan was suitably unimpressed with the enormous lake we didnt drive into Switzerland but luckily found a wildlife park with children's play areas and Gareth and I were both pleasantly surprised to see Ryan leap out of the buggy and head for the trampoline.  It was a lovely afternoon and we packed a lot into a couple hours.

Friday, 12 October 2012

Yesterday's clinic appointment went well, all of Ryan's bloods are good and he has started to produce T-cells, an essential part of his new immune system. We feel so lucky that things are moving in the right direction, and I really do mean that, there is always someone dealing with something worse and that is very much the case at the moment.

Mia, a little girl we see at the parents house who also has neuroblastoma, had her planned surgery 3 days ago to remove the residual tumour but after the operation a blockage in an artery cause her heart to stop. She was immediately opened up again so the surgeons could massage her heart while they looked for the blockage. This happened twice. This kind of news is devastating, she was always running around and was doing really well. Now her mum is waiting to hear if she will make it. When I woke in the night I thought of her straight away and said a prayer.

We also know that a teenage boy who had his transplant, from a matched donor, on the same day as Ryan is still waiting for his counts to increase to repair his ulcerated mouth and stomach and stop the attack of shingles that has reactivated.

Then there is the little girl in the isolation room next to Ryan's. She had a haplo transplant the week before Ryan but failed to graft and so she was given her own stem cells back and is still waiting for her own cells to graft. And a little boy from Ireland who I saw photo's of sitting in the pilot's seat on his flight to the USA to start immunotherapy but due to complications was immediately rushed to intensive care where he is stable but fighting his hardest fight ever.

So much awful, sad news which I cannot help but be effected by. We protect Ryan from this news and smile and joke like things are normal but right now lots of good news would be nice to hear.

Tuesday, 9 October 2012

Ryan has been discharged from hospital

Since my last update lots has happened but mostly good things; mainly Ryan being discharged just 19 days after having his transplant, which meant a hospital stay of 31 days.  Even as I write this I can't really believe that it is true, it is totally amazing, he is totally amazing.  He walked out of the hospital back to the car while I walked behind with the empty buggy!

I wont bore you with the details of our stay on Station 14 - the oncology ward.  It was stressful, with poor Ryan having to wear his mask all day, and was not how we envisaged his stay in hospital would end but we have to be thankful that Ryan only needed to be there a few day while he was weaned off morphine and the IV drugs were changed to oral ones.  At least we were fortunate enough to be able to take him back to the parents house in the evening before returning to hospital early the next morning.  Luckily the little boy that we shared the room with in the day was lovely and they understood our concerns and reasons for not wanting Ryan to share a room.  The two boys got on well despite the language barrier and both enjoyed watching Tom and Jerry DVD's.  On Sunday he was discharged to the parents house after a review with the doctor in charge of the ward.  The doctor said that Ryan has done amazingly well and was in very good shape, he just needed to start eating.

Ryan has been very happy back at the parents house, the first thing that we had to do was set up the x-box of course but he has happily prized himself away to play with Jamie who is a couple weeks ahead of Ryan and also now doing really well.  Both boys love Power Rangers so run around the ground floor of the house fighting invisible baddies - it really is great to see them having fun together.

Yesterday Ryan had to attend his first appointment at the Day Clinic where he is seen by more new doctors and nurses.  His blood results were all good and for those interested his counts are now: WBC 3.42, HB 10.8, Platelets 234 and CRP 0.6! All of which are fantastic.  We have to attend clinic twice a week and so now have 2 whole hospital free days which feels fantastic.

In himself Ryan is happy and feels well BUT is noticeably tired after short bursts of energy.  This is obviously to be expected but hard to convey.  He does run around, go on his scooter etc but then needs to rest which is something that Ryan finds very hard to do!  Over the next week or so, as he gets stronger we hope to take him out to see a little bit of the local area but at the moment it is more important that we focus on eating, drinking and medications.  He has to drink a minimum of 600ml each day, ideally 1 litre, as some of the anti-biotics, anti-fungal, anti-viral and anti-rejection drugs are toxic to the kidneys but he is managing this and is also eating 3, albeit small, meals a day.  Each day he has 9 medicines to take, most of them he has to take morning and evening!  I'm surprised he has any appetite with that lot sitting in his stomach.  All in all he is doing amazingly well and we can't be more proud of him than we are today.

Thursday, 4 October 2012

Sorry it's a long one ........

The past couple days have been very mixed; Ryan continues to do well in that his mouth and throat are healing and his morphine is being reduced, his counts are good, his CRP has started to reduce but his fevers are persisting and when they come Ryan is a difference boy, shivering, tired and feels very unwell. We took him to the parents house the day before yesterday and he had a bit of a kick about and went on his scooter a bit but then asked to go inside and sure enough his fever was returning.

The doctors believed the fevers were either engraftment syndrome but lasting longer than normal, an auto immune response or possibly an infection.

So to ensure they know what they are dealing with the consultant in charge of Ryan requested a CT scan to rule out any infection in his lungs, this was carried out on Tuesday. It was quite a stressful day.

Yesterday morning we were told the good news that the CT scan was clear and that they had done many tests on blood, urine and stool samples to rule out infection of any kind. All tests were negative, this was a huge relief.

And so the doctor said they felt sure that Ryan fevers were caused by prolonged engraftment syndrome, his body reacting to the new cells. The plan was to give Ryan a steroid to suppress his response they felt sure this would stop the fevers.

Then came the news that as he was doing so well in all other respects, and they had a new admission coming in, Ryan would be moved to the oncology ward.....later that day, yesterday. We asked if he would be in his own room and this was confirmed.

We started packing up the room and had almost finished when the doctor came back to say that unfortunately there was no longer a single room and that Ryan would have to share with another child. I think we felt so shocked that we initially accepted this news, as we understand the pressures on doctor and nurses and knew it wasn't the doctors fault. He assured us the child had no infections and was having chemotherapy.

Gareth and I went to see the room without Ryan to see how much room we had for our things. The room had been lived in by the other family for a while and so things were everywhere, after all they must have only just been told too. The realisation hit us that we were moving from a room where we had to strip ryan from his outdoor clothes as soon as he returned to his room to exposing him to 3 other people (child and parents) and their belongings. Gareth took some of our things back to the parents house and whilst he was away from the hospital had time to think about what we were being asked to do.

I felt very uncomfortable but felt we had no choice. Gareth decided we did have a choice, we would simply refuse to move unless it was to a single room. And so after many lengthy conversations, with nurses and doctors who were called in from home, ryan was transferred to his own room in the oncology ward, just for the night. In the morning he would have to move into the room with the other child. We accepted this as in the day Ryan can wear his 'industrial' looking mask that we were told he has to wear everywhere that he is in the company of others, with the exception of close family and friends that know how important it is to be well.

And so today we are sharing a room with Marco and his mum, Ryan is wearing his mask and being really good about it. There is no single room tonight so part of the negotiating that Gareth did was to ensure that whatever needed to happen to get Ryan back to the parents house tonight happened.

The conditions are Ryan must take the majority of drugs orally, must demonstrate he is eating albeit small amounts and must drink a minimum of 600ml as some of the medications are toxic to the kidneys. He is also withdrawing from morphine which can sometimes be a slow process and depends how the individual feels.

In addition his blood results had to be good. Luckily they were better than good - his platelets are in the normal range for the first time in over a year! His WBC is stable at 2400, his HB has increased but most importantly his CRP ( infection marker) has dropped meaning that 2 of the 4 iv antibiotics can stop.

And so the plan is that we can leave the hospital tonight at 9pm and Ryan can sleep with us at the parents house, mask free, and return to hospital tomorrow for 8am. We will have to wake him during the night to give him medicines but he understands and doesnt mind.

This time tomorrow we may be one step closer to being discharged to the parents house.......

Monday, 1 October 2012

Day +13

He got out and here is the photo to prove it:

He actually enjoyed the hour and I cannot begin to explain the happiness I felt watching him walk out the transplant unit. There is still a long way to go, he still has a fever, CRP and is on morphine and many other iv drugs but this hour was a momentous achievement. But I know Gareth felt the same. When Ryan left isolation following his autoglous bone marrow transplant transplant at the age of 2 he cried in pain when he tried to bear his weight as he had been in bed so long. With our help he managed to walk the 30 paces from isolation to non-isolation before getting back into bed. It was heart breaking. This time though he wanted to walk, we visited the small play areas within the empty hospital (it was deserted as it was a sunday) and then ventured outside to the slide and basket swing. He did need the buggy on the way back to the room but it was still amazing to see him.

He was as good as gold coming back into the room. We had to remove the clothes he had worn outside, wash him and get him reconnected to his iv machines.

His temperature is persisting and with only paracetamol it isnt going below 38.0 so this morning, he is very tired. The consultants here have never known anyone be allergic to novageen and as it is so effective against fever they wanted to be 100% sure that Ryan was reacting to it. So this morning when his fever reached 39.1 they wanted to give him a half dose of novageen and monitor him, with anti-histamine at the ready. Ryan was not impressed and told the nurse they were really mean making him get hives.....  Sure enough within 5 minutes he started coughing, went pink and got hives! But even with a half dose his fever reduced to 38.2!

Today, without the help of GCSF, his WBC is 4,740 and so today he has 2 hours of freedom. His CRP has also increased but we are told this happens during engraftment but as his temperature is persisting unfortunately also means he will change to 4th line iv anti-biotics until the CRP is less than 1 (today it has increased to 3.0 or 30 as it reported in the UK)

He has to wear a mask everywhere except for in our room at the parents house, the car or in his room in isolation and will need to do so until Day +100 but so far isn't complaining. His 'clean diet' has to continue for the same period.