Ryan had a blood count last Monday morning and luckily his neutrophils, with a bit of help from GCF on Friday, had recovered sufficiently to start his third cycle of chemo.  This time he is having irinotecan and vincristine in the hope that his bone marrow will not be so knocked by the drugs.  This meant a trip to hospital every day, on the Monday he had both drugs and Tuesday to Friday he had irinotecan.  He was noticeably tired during the week but still refusing to sleep in the day or go to bed early. He has felt quite sick at times and so really wasn’t keen to eat anything much despite 3 anti sickness but they must have worked because he wasn’t actually sick.  Usually by tea time though he would be able to eat his dinner, which is normally spaghetti bolognese – thank goodness that he still enjoys his old favourite.  This time he also experienced jaw and back pain which are side effects of vincristine and occasional tummy ache. We continue the anti sickness on a couple days after he finishes the chemo and stop it on the Monday, today.

This weekend though he had his friend Josh’s birthday party treasure hunt at Haldon Forest and Ryan really surprised us by running around the forest looking for clues just like the other children.  He couldn’t always keep up but didn’t want a carry until almost the end, about 2 miles later!  Seeing him having fun is always wonderful and being able to go to parties is even more special as Ryan has missed out on so so many.

Then right after the party his mate Adam and family arrived for the weekend which was great, as Adam has wanted to check out all of Ryan’s toys for a long time.  We had thought it might be a quiet weekend, as both boys are having chemotherapy, but they crammed quite a lot into their time together; power rangers, x-box, wii, bouncy castle in the garden, footie in the park and a trip to the beach for crazy golf and a game of dodge the wave. Shame we didn't capture the moment where Adam got soaked!  It was a great weekend and we hope that we can get together again before too long.

This week should be a better one as far as side effects go but will probably mean GCSF injections to boost Ryan’s neutrophil count and maybe a couple trips to hospital for a blood and/or platelet transfusion.  The only problem is that now Ryan is feeling like doing something and seeing people nearly all of his friends are at school and so he doesn’t really have any one his own age to play with and regretfully I have to say that playing with mum or dad just doesn’t cut it anymore.

The meeting...

Ryan thoroughly enjoyed himself at the circus and sleeping over at his cousin James’ house.  He was understandably a bit tearful at bedtime but was perfectly happy in the morning and snuck into his cousin’s bed to wake him up!  Here is James and the cheeky chappy.

Our trip to Tuebingen was straightforward enough I guess, the only direct flight we could get was from Heathrow (or even worse Stanstead).  Unfortunately there were 2 full flights checking in on just 2 desks and it was a bit chaotic to say the least and so despite arriving with plenty of time to spare by the time we checked in there was only enough time to make a quick dash through to the departure gates.  Then, as people were boarding, an announcement was made asking for 2 volunteers with hand luggage only to fly out the following day as the flight was over booked??!!  Madness really but I am assuming someone volunteered and took the 250 euros compensation and over night stay in a 5 star hotel….

Our hotel was near the airport, in an industrial estate actually and being a Sunday the restaurant had closed 3 minutes prior to our arrival, perfect.  The helpful receptionist did give us directions to an Indian take-away which also did Pizza – so that was tea sorted!

The next morning we arrived at the hospital and, in the way that we had become accustomed for our treatment in Greifswald, we first had to complete registration and then we had to pay for our consultation and then we got to meet with the consultant.  It is during registration that you wished you knew more languages, especially German!  Dr Lang was a very pleasant gentleman but made us no promises of a wonder cure.  He talked us through the treatment, the intended purpose and the risks involved.  It is difficult to describe how you feel during such a meeting.  You almost have to detach yourself from the situation and not think that you are talking about what could happen to your precious child.  The risks, and there are many, include heart, liver and kidney problems, rejection of the donor stem cells and graft versus host disease to name but a few.  But the greatest risk is of infection during the period where Ryan’s bone marrow has been wiped out completely and the period where the new stem cells are grafting in his body, which can take approximately 2 weeks before we begin to see signs of engraftment.  All of these risks can be fatal. 

During this time Ryan will be kept in isolation in the transplant ward of the hospital.  There is an air filter fitted in each room and the ward can only be accessed through a changing room where you must remove your outdoor clothes and shoes and put on clean ones. He will be on numerous anti biotics, anti-virals and anti-fungals all to try and prevent any infection.  Ryan will have a restricted diet for 100 days so that there is no risk of infection through the food that he eats.  Although during this time he will have mucusitus, and so eating will not initially be a concern, it will be managing his pain while we wait for the new cells to graft and repair his body.  He will not be forced to have an ng tube and he will be supported by TPN (IV Nutrition) both of which was our request.  Ryan had an ng tube for 9 months between age 2 and 3 and as he remembers this he has no desire to have one again, so when he can eat he does eat, with a lot of encouragement from me and Gareth! 

Strangely the hospital do not have a policy of having a parent sleep in hospital with the child, we had been warned of this by another family who had already made the trip out, and so we asked about this and made it clear that we would not be leaving Ryan at any point and so would sleep in a chair if necessary.  This did not seem to be a problem and we were told we could sleep at the hospital with Ryan, thank goodness!  After the meeting Gareth and I had blood taken which will be tested to see who  will be Ryan’s donor.  A sample of Ryan’s blood will be sent to the hospital and then we will find out the results.  We also discovered that the stem cells will be taken from us peripherally, which involves being connected to a harvesting machine for many hours with IV lines in both arms – something else to look forward to!  This will be during the period when Ryan is receiving high dose chemotherapy prior to his transplant.  We will also have to have GCSF injections twice a day for 5 days prior to the 2 day procedure! Little price to pay though although I will probably pass out based on my track record!  

Luckily the actual transplant for Ryan is nothing more than a blood transfusion into his central line, usually several small bags of stem cells which look just like blood depending on the number of cells harvested, it does not involve surgery as the name suggests.  Overall the trip was a success and although we both felt rather subdued after the meeting our minds are still made up.  The only problem being that the hospital does not have a bed available in the transplant ward until the 12^th August.  This will mean that Ryan will need to continue to have chemotherapy until that time.  This is quite a worry at the moment as his counts are still not recovering from the 2^nd cycle and he is now 3 weeks late starting the 3^rd cycle.  This is not unexpected in view of the extensive amount of treatment he has had since relapsing in November 2010 and is a sign of how very weak his bone marrow has become.

Ryan will have another count on Monday and we have everything crossed that his counts are sufficient to start.  The type of chemotherapy has been changed to one that is not so toxic to the bone marrow to see if Ryan is able to tolerate this better.

Ryan has been well the past week despite needing a blood transfusion and regular injections of GCSF to boost his neutrophils.  With it being half term he managed to catch up with some of his friends although the weather limited any outdoor activities.  

Here he is scooting with his cousins Gabriel and Archie:

Later today Gareth and I will be driving to Heathrow so that we can be in Tubingen early tomorrow morning for our meeting with Dr Lang, the consultant in the charge of the facility where they will do the haplo-identical transplant.  We have not been apart from Ryan since he relapsed in November 2010 and so it will be very strange to leave him, Ryan still has mixed feelings about it.  At least it is only the one night but there is more to having Ryan to stay then just an over night bag – we have to explain what to do in an emergency with his central line and how to administer an injection of cortisone should he become unwell and make sure that my sister Jackie has the number of the local hospital!  But our lovely CLIC nurse lives fairly close to Jackie and has given us her personal mobile number and has said that Jackie can get in touch at anytime if she has any concerns about Ryan.  Best to cover all angles that way we know that he will be just fine!

But we have tried to make it as much of an adventure for Ryan as possible and so this afternoon he is going to the Circus, courtesy of CLIC Sargent.  He is going with his auntie Jackie and cousin James and is very excited about it.

Yesterday his cousins from London came to visit which was great and the boys played football for hours! There have been lots of fund raising events taking place this weekend; the little ones sky dive team did their jump, Uncle Paul ran his marathon, Donna Hawkins held a Waxing event (Gareth had to have his chest waxed!), Uncle David and Team did their 50 mile cycle ride, Alex and Ben's fun day and auction in Portsmouth is being held today and the 4 soldiers in Germany from 64 Fuel Sqn’s are currently in the middle of their 24hour event – details of all these events are on the Events page.  Thanks to everyone for all the hard work that has gone into making these events happen.  I hope to update the events page with photo’s from all these events over the next week.

I meant to say in my last post that we have heard from NB Alliance, the charity that we are fund raising with, and they have confirmed that they have sufficient money in their charity reserves to make the advance payment for Ryan's treatment.  This is a big weight off our minds, as you can imagine.  As we have said it has always been our intention to try and raise the money and this is still our aim so that the Charity can do the same for another family in the future.

Ryan has a busy long weekend planned including a visit to clip and climb with his girlfriend Ella to celebrate her birthday, this is a bit of a risk with his poor immune system but likely to be last time he has the opportunity to do something like this in a long time. He also wants to get out on his motorbike with his mate Jack, which is good timing following a platelet transfusion yesterday, we have a BBQ garden party to go to and hope to get out on the push bikes again too - although it's a bank holiday so it's bound to rain!

This weekend's events that I know about:
Saturday 2nd June: Suzanne Yeo is doing a 15,000ft tandem sky dive at Dunkeswell Airfield, Honiton
Sunday 3rd June: Daniel and Arron are running the Plymouth half marathon
Monday 4th June: Tracy Derges has a stall at the Exminster Village Fete selling cakes, wristbands, cards and great raffle prizes.
Tuesday 5th June: Jackie Chalk has a stall at the Waterfront Jubilee Street Party selling wristbands and raising awareness of Ryan's appeal.

Thank you all so much for giving up your time to raise funds and awareness of Ryan's appeal.

Finally my brother has an event planned next Saturday 9th June: a 50 mile cycle ride from Exeter to Okehampton and back again.  He has made the event open to anyone and everyone, with or without sponsorship, and will set the pace on the slowest riders ability.  But so that he can plan the event and so that they don't leave without you, if you would like to join the team on the ride please contact Dave on 07809 295380 or 01363 82918.  For more details go to http://www.justgiving.com/dave-smith4ryanedwards

The weekend after learning Ryan was in remission was very emitional. We felt elated, and so very very lucky that we were in this position again. But then also very apprehensive about putting him through the haplo-transplant. This doesnt mean we are having doubts. But Ryan has had a transplant before, we know the risks, how poorly he will be and it also commits us to another 9-12 months of hospital treatment, in another country when what we would love to do is take him away on holiday and let him enjoy life. We like to think that we fill every day when he is feeling well with fun things to do but it obviously isn't the same. But we know what we have to do and why we are doing it.

We have arranged to have a consultation with Dr Lang in Tubingen on Monday 11th June. We need to fly from Heathrow the evening before for a morning appointment. It is not mandatory for Ryan to come, and as we never really like to talk about treatment in front of Ryan, we have decided he can have his first sleep over at his cousin's house. He was excited about this but after some thought has decided he will miss us! After all we are never apart from each other so its only natural but hopefully he will enjoy it.

This week Ryan should have started his 3rd cycle of chemo but his blood counts, platelets and neutrophils, were too low. He has continued to need GCSF injections to boost his neutrophils and his platelets today are only 13 so he will be having a transfusion tomorrow.

This morning we were in hospital for a synacthen test. Ryan stopped producing the steroid cortisone as a side effect of dexamethasone. This has meant we need to give him hydrocortisone 3 times a day and carry an injection of cortisone with us at all times in case he become unwell as the body needs cortisone to respond to a crisis situation. In such a situation the pituitary gland in the brain releases a hormone which gives a signal to the adrenal gland to produce cortisone.

Today we didnt give him his morning dose of hydrocortisone but instead went to hospital at 9 o'clock for a special test. Ryan was injected with a synthetic form of the hormone released by the pituitary gland and then blood taken at regular intervals to see if his body could respond appropriately.

We also had a review with Ryan's lead consultant from Bristol and he is going to start the process of applying for funding for treatment, although it is expected it will be declined!

This afternoon Ryan has a review with his tutor and her manager to assess how his home tuition is going. We are really pleased with his progress and despite the fact he only sees his tutor twice a week for one and a half hours, she says he is doing really well. He didnt see her at all for 5 weeks when he had radiotherapy but she says he had remembered everything she had taught him so she could pick up where she left off. I have to be honest though and say that he still thinks learning is boring and would rather be learning stunts!

A bit about our fund raising; THANK YOU, THANK YOU, THANK YOU!! We cant believe that in the last month the fund has increased from £30,000 to just over £60,000! Its just amazing and we are so so thankful to everyone helping us.

I have spent some time updating the events page with all the public or sponsored events I know about and will try and keep it up to date. Thank you to everyone supporting these events. We try and attend any that we can and I'm sorry that we can't make them all but this past weekend we managed to catch up with the Robinson Family Cycle Relay team after they finished their 80 mile relay. I've added some photo's and news of amounts raised at recent events to the events page, please take a look.

Better news than we could ever have hoped for....

Ryan’s MRI scan is clear, there is no sign of the tumour that had been in his spine encasing his spinal cord.  It has gone.  Gareth and I didn’t dare believe this was true when we heard it on the phone on Thursday evening.  We didn’t allow ourselves to believe this.  There must have been a mistake, it can’t have vanished, they told us it was a possibility but extremely unlikely.  We needed to wait until we met with the consultant, face to face, and saw the images.

But it is true.  Even typing this makes me cry, happy tears though.  We asked if they were sure, really sure, that it has gone.  Yes it was double checked and we were shown the MRI from February and then the MRI from last week.  They are noticeably different. There really is nothing left.  This is truly the best news we have ever had and feels like we have won the lottery. But at the same time I know that we both feel a sadness for the other families that we know that have recently lost their beautiful children to the disease or those that are still fighting, and fighting hard.  We, like them, will never take a day with our child for granted, and will continue to live life day by day, enjoying every single moment.

What does this mean for Ryan?  Well this means that there is now no visible sign of neuroblastoma in his body but the key word is ‘visible’ sign.  This is not a guarantee that there are no individual cells, dormant, waiting.  When Ryan originally received remission he was 2, he had had 3 months of aggressive chemotherapy followed by an 11 hour surgery to remove the remaining tumour from his tummy.  The scans after this were clear as they are now.  He then had high dose chemotherapy, a stem cell transplant (a type of bone marrow transplant where you receive your own cells back) 14 fractions of radiotherapy and 6 months of cis-retonolic acid (similar to oral chemo). Despite all this treatment he went on to relapse at the age of 4, as unfortunately most children with this disease do.  We had been told right at the start of treatment that Ryan’s chance of survival 2 years past diagnosis was 30%.  We have never been under any illusions that this was an easy cancer to beat.

As you know he then endured 6 further cycles of chemo, and MIBG therapy (internal radiation treatment) before going to Greifswald, Germany for immunotherapy.  After the 4th cycle of treatment again his body was again clear of disease but just 3 months later a new tumour had appeared in his spine.  No one saw this coming but when his original scans were examined we were told that the site of the latest tumour had showed signs of disease back in 2008 and so it was likely that cancer cells had remained dormant until this year, when they started to grow again.

Now we have several choices.  This time it is different.  We have never had choices before, not really.  Sorry this is going to be a long one…..

Our first choice is we can do nothing, we can stop treatment.  There is a possibility that the disease has really gone this time, gone for good.  We can try and resume our normal life and wait and see…. This is so very tempting as Ryan, our beautiful only son, has surely been through enough, more than most people would have to experience in a lifetime. But if it comes back again…. If it comes back again it is very unlikely to come back as an isolated tumour, it is more likely to come back with a vengeance in his bones and marrow as it did before.  He would need chemotherapy to take control of the disease but he has now had every type of chemotherapy known to work on the disease.  He would need a strong bone marrow to allow his body to tolerate such treatment, but Ryan does not have a strong bone marrow.  His counts have not been in the normal ranges since November 2010 and it is likely that it will be many years before they would return to normal.  His options would be to try whatever new trial treatments were available at the time, most likely abroad, but you need to meet criteria to join these trials and most need a strong blood count at the start of treatment.

The other options that we have been given are to put Ryan through another bone marrow transplant, originally it was suggested with his own cells but unfortunately we have been unable to collect sufficient stem cells for him to have a second autologous transplant (his own cells returned).
So we could choose to have a matched bone marrow transplant from an unrelated donor.  To minimize potential side effects, this type of transplant would use transplanted stem cells that match Ryan’s own stem cells as closely as possible. ‘People have different sets of proteins, called humanleukocyte-associated (HLA) antigens, on the surface of their cells. The set of proteins, called the HLA type, is identified by a special blood test’.

‘In most cases, the success of allogeneic transplantation depends in part on how well the HLA antigens of the donor’s stem cells match those of the recipient’s stem cells. The higher the number of matching HLA antigens, the greater the chance that the patient’s body will accept the donor’s stem cells. In general, patients are less likely to develop a complication known as graft-versus-host disease (GVHD) if the stem cells of the donor and patient are closely matched’.

But this option is not proven to offer a chance of a cure, this option is what it is, an option based on the knowledge and experience of Ryan’s consultants.  Ryan would have a new immune system, which over time would grow stronger and the hope would be that the cancer would never return.

And then we have the option to go to Tubingen for a Haplo-identical transplant.  In Tubingen, they have a specialist clinic where they have been treating children with neuroblastoma for many years, trying to find a cure.  They use the stem cells from a parent but they are not looking for an exact match.  The idea (in layman’s terms) is that the new cells have an anti-cancer response when infused into the body, they destroy any remaining cells, which is the job that should have been done by Ryan’s own immune system but it is not able to. The flip side to this is that the donor cells also attack healthy cells in Ryan’s body, this is known as Graft Versus Host Disease.  GVHD sometimes develops when white blood cells from the donor (the graft) identify cells in the patient’s body (the host) as foreign and attack them. The most commonly damaged organs are the skin, liver, and intestines. This is a serious condition and if it cannot be controlled can effect quality of life. But in Tubingen they prepare the donor cells in a way to maximise the anti-tumour effect and minimise the GVHD.  GVHD is only one of the many risks associated with this, and other, types of bone marrow transplant.

Our consultants have talked through the options and possibilities with us and are genuinely not able to advise us the option that is best for Ryan.  The ultimate decision lies with us. Our decision is unchanged; we intend to take Ryan to Germany for further treatment.  Again there are no guarantees that this will cure Ryan but there is a chance.  This is the hardest decision we have ever made, but it also feels like the right one.  To do nothing feels wrong and the other options have no evidence to support there success or otherwise.

So we have contacted Tubingen to make an appointment to discuss the treatment further, to ask questions and to understand the risks involved.  At the same time our consultants will do the same and within the next few weeks we should have a timescale for the transplant.

Then to the financial side of things; we are hoping to raise the full amount of money, but it is now looking unlikely that we will raise it in time. The charity that we are fundraising with, The Neuroblastoma Alliance UK has kindly agreed to step in to provide the additional money from its charity reserves if there is enough available when we need it.  However we are committed to continuing our fund raising efforts to try and cover all the costs of Ryan's treatment.  This means when another family comes forward, there will be funds available for the next child that needs help.  The Charity has said that they will confirm whether they can support us early next week.

A bit about Ryan.....

Ryan is still well; after an enjoyable weekend Ryan has had a fairly good week so far.  On Monday his girl friend Ella was allowed to take the afternoon off school to see Ryan - the two of them had a great time making a den in her back garden and generally had great fun seeing each other.  While we were at Ella's house we had the good news that his MIBG scan was clear but also learnt that his neutrophils were very low (0.4 when the normal range is 1.5-5) This means that Ryan is classed as profoundly neutropenic and has almost no immune system.  His consultant therefore decided that he should start GCSF - this can be given as either a subcutaneous injection (into the skin) or IV (into his central line).  The IV infusion takes 1.5 hours in hospital whereas the injection can be given at home (by his nasty Dad).  We opted for the injection, much to Ryan's upset and so when we got home from Ella's he had to have numbing cream on his leg and then an hour later at 7.15pm Gareth had to give him an injection.  Despite having had 60 injections in Germany, he is only 5 years old and HATES injections. Unfortunately Ryan can remember that GCSF stings a lot when it is injected, the cream only helps with the pain of the needle. He cried as soon as the cream came off and continued to do so, asking why we were trying to ruin his lovely afternoon? He soon calmed down but insisted on being allowed a late night to recover from the trauma of the injection!

Then on Tuesday we had a sitting with a photography studio and had photo's taken of the three of us together.  The studio had offered the sitting via the hospital and we can't wait to see the photo's as we have so few of us all together!  It is a perfect time to have the photo's taken as Ryan is looking well and although he is loosing his hair it is not falling out as fast as we originally thought and so he had a trendy new haircut and is looking really cool.  We also had some new photo's taken with the Express and Echo, as the first photo they had was taken by us when we were at Sam's House in Bristol.  It was very funny when the photographer was here, Ryan had just come back from the photography studio and was under the impression that he was a professional model!  He kept suggesting new poses to the photographer, 'Now I'll get on my trampoline, Now on my scooter, on my bike, with my toys....' It was so funny and in the end he politely had to explain that he had to leave and go to his next job!  The day ended again with an injection - the injection has to be given at a similar time each day but we did manage to bring it forward an hour or so but had to agree to another late night!

Yesterday and today Ryan saw his tutor and she says he is doing really well. He is still a bit reluctant to learn reading and writing because obviously it is 'boring' but she is great at getting him involved and he works well with her - we are very lucky to have her.  He had a blood test this morning to see what effect the 3 days of GCSF had had on his blood counts and were a little disappointed to find out that his neutrophils were only 1.0 and his platelets were only 10! (normal range 150-600).  So platelets were ordered urgently.  Exeter hospital were told they would arrive between 3-4 and so we cancelled his mate Ben coming for tea and instead told a very unhappy Ryan that he had to go to hospital for platelets. He was not impressed.

At 4.15pm we got a call from the ward to tell us that there had been a problem washing the platelets and they had in fact only left Bristol at 3.45..... In brief we ended up going into hospital at 6pm (after yet another injection) and the platelets were connected at 7.45, way past Ryan's bedtime.  He was exhausted and completely over tired by the time we got him home and didnt get to sleep until 9pm :-( It is not looking likely that Ryan's counts will be strong enough for him to start the 3rd cycle of chemo on Monday but a decision will be made on the day.

Tomorrow we have a meeting with his consultants in Exeter and after this will know full details of the scan results and hopefully have an outline of a plan for the coming weeks.