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.

Wednesday, 30 January 2013

Why does bad news always follow good news??

First the good news: as at this week Ryan's t-cells are now 100% donor derived, none of Ryan's t-cells can be detected.  The GVHD rash is being controlled well with cellcept (a drug which 'puts the brake' on the new t-cells) and the steroid and immune suppressant creams and his temperature was consistent but quite high at around 38.0.   Over the past week he has been feeling quite well and enjoying a bit of fun in the snow and even a bit more ski-ing!

The plan was to begin reducing the cellcept on Monday to half a dose and monitor the rash and temperature. 

On Monday we were told that Ryan's situation was discussed at the weekly meeting and the feeling was that a stem cell boost should be planned sooner rather than later.  The purpose of the stem cell boost is to give Ryan a strong bone marrow as his newly grafted marrow has been attacked by his own t-cells for so many months that no-one can predict how long it would take to recover.  Until his counts recover he will remain blood products and GCSF dependent and be at great risk of infection. The beauty of a haplo transplant is that Ryan's blood and mine originate from the same stem cells, mine! And so on Friday I start a 5 day course of injections to force my body to produce stem cells and next Tuesday they will be harvested in the same way that they were before over a six hour period.  And so on Monday I underwent a series of blood tests and investigations to check I am fit enough to undergo the harvesting process.  Although this is not something I am looking forward to it is amazing to think that I can help Ryan recover after all he has been through and I will do it gladly.

And here is the bad news : by Monday evening his temperature had increased and continue to increase reaching 39.4 at it's highest today.  On arrival at clinic we were told that yesterday's blood results showed that his liver enzymes were elevated and this concerned the Dr's as it was an indication of inflammation of the liver - the cause of which they suspect to be either GVHD or a virus. His CRP marker was also above normal range today and Ryan was very tired and slept for hours in clinic.   To give a complete picture an ultrasound of his liver was performed which confirmed it was enlarged and showed signs of inflammation.  And so the consultant in charge of Ryan decided to treat both possible causes immediately by starting him on high dose steroids to address the GVHD and IV anti-biotics and anti-vitals until the result of the relevant tests are known.  They are also considering doing a needle biopsy of the liver if the liver enzymes are further elevated tomorrow.

This evening his temperature in closer to 38 again and he is feeling a little better although hasn't felt like eating all day.  Hopefully tomorrow things will look a little better all round.

Monday, 21 January 2013

T-cell update

I realised it's been a while since i updated about Ryan:
As at friday donor derived t-cells have reached an unbelievable 93%! The total number of t-cells per microlitre has risen from 50 to 205 in under ten days!
This is the best news we have had in a very long time and totally reverses the situation Ryan was in. The Dr now say there is very little chance of Ryan's remaining t-cells fighting back.

As usual this good news comes at a price as this massive surge in t-cells has caused Graft Versus Host Disease (GVHD) of the skin. At present this is being managed by both steroid and immune suppressant creams. The rash, which is on the front and back of his torso, is not much to look at but is itching so very annoying for Ryan. The Dr's had obviously warned us that this might happen as a result of the t-cell therapy and although a worry the thought of a further transplant was much more scary and unpredictable.

Although ideally we need to reach 100% donor derived t-cells before the Dr's can say categorically that the transplant can not be rejected, it is clear that the tables have now turned and no-one expects to see Ryan's t-cells for much longer.

The Dr's are keeping a close eye on Ryan's blood results and skin condition. His bone marrow is still very weak but once there are none of Ryan's t-cells left it is hoped that his bone marrow will recover. If it doesnt the plan would be for me to donate further stem cells.

Wednesday, 16 January 2013

It is with great sadness that I have to tell you that our little friend Jamie's struggle ended, peacefully, yesterday. He was a funny, energetic little boy with a wicked sense of humour (and obsession with spiders!) and i'm sure that he will not be forgotten by anyone who had the privilege of meeting him. We have nothing but admiration for John and Vicky his parents....

http://www.facebook.com/keepjamiesmiling

Saturday, 12 January 2013

Ryan and the instructor in a 'race'
After having platelets on Wednesday we decided that we would take Ryan back to Feldberg in the Black Forest for a ski lesson on Thursday. He was very keen and wanted the lesson as soon as we arrived! 

As most of the other children there were not complete beginners they suggested a one on one lesson for an hour with an instructor. It soon became apparent that Ryan didnt have the physical strength of most children his age as he couldnt do a snow plough which is where the front of the ski's touch and back are wide apart and forced into the snow - this move is the brake and therefore an essential basic control. However that didnt bother Ryan as he was quite happy with both ski's pointing straight now the hill! (So much like his Dad!) He had great balance and held a good position on the ski's and amazingly didnt fall over. It just meant we had to catch him at the bottom to stop him going into the fence!  He had a great time but was exhausted afterwards and we even thought he might fall asleep at lunchtime but he didn't. He is now the proud owner of a ski-ing medal and certificate which says 'Super Fast'! 

Then yesterday it was back to clinic for an infusion of immunoglobulin (to give him protection against infection while his immune system is compromised). 

Whilst in clinic we got told the results of wednesday's t-cell analysis. 21.47% now originate from my donated stem cells! A very significant increase which the doctors are very happy with and our glimmer of hope feels a bit brighter! Needless to say we are (cautiously) delighted to have this good news and have fingers and toes crossed that the new cells continue to multiply. 

Our hope too is that Jamie's parents John and Vicky also get some good news very soon, Jamie remains critical but after a very worrying start to the week has remained stable on the ECMO machine which is oxygenating his blood. There have been many ups and downs this week but he has a CT scan of his lungs on Monday and we hope and pray it shows improvement. 

Monday, 7 January 2013

On Friday we got the results of the latest T-cell analysis and for the first time this showed that a small percentage (2.7% to be precise) originate from me! This is hopefully the first small sign that something is happening. Unfortunately this doesn't necessarily mean that my T-cells will overpower Ryan's, it is too early to know what the outcome will be and until the large majority of t-cells are mine the possibility will remain that Ryan can reject the transplant but this is still undoubtably good news.


Also on Friday Ryan was given a further infusion of my T-cells, the same amount as last time: 100,000 t-cells per kilo which actually means 1,900,000 cells! He also needed a blood and platelet transfusion and GCSF so it was a long afternoon in hospital. 

It is hoped that as Ryan's t-cells are destroyed that his bone marrow will recover and his need for blood products will gradually reduce as currently platelets are only lasting 3-5 days and blood is lasting 4-5 weeks. He is also needing GCSF injections every other day at the moment. As his consultant stated "improvement is needed".


Yesterday, as he was topped up with platelets and blood, we drove to the black forest for the afternoon. It is an hour and a half drive to the town of Feldberg and when we arrived we were a little disappointed at the piles of melting snow. But after asking a local we were directed another 10km up the mountain to a ski area where they had loads of snow! The difference in the landscape in ten minutes was amazing. Ryan spent a couple hours playing in the snow and tobogganing and after watching children in a ski school, has asked if he can have lessons. We've agreed to take him back later in the week when it's quiet and see if we can get him an hours session. 

Gareth is a keen skier and has always wanted to take Ryan ski-ing but when he was old enough he had relapsed. To miss this opportunity when it's so accessible would be foolish so as long as Ryan feels well and has sufficient platelets that is the next thing he can look forward to. 
The fenced off ski-school at the ski area in Feldberg

Thursday, 3 January 2013

Here's to 2013

Every New Year since 2008 we have had only one wish: that the forthcoming year will be the year that Ryan beat's this disease once and for all.  The hope remains as we start this year. 

We travelled back to Tübingen on the 1st January, after spending the night at the home of Family Bird as Adam was also home for Christmas.  It was lovely to see Adam and his family.  Adam is recovering from a painful surgery for an abscess on his bottom, it is so painful that he has to lie on his side, as he can't sit or walk without feeling pain but not once did he complain.  The boys enjoyed their time together and it gave Ryan something to look forward to when we had to leave home.  He felt sad about coming back to Germany not because of going to hospital or the travelling but because he will miss his friends. He said x-box live and Facetime are great but it's just not the same.  He has every right to feel that way but he didn't dwell on it and we hardly heard a peep out of him in the back of the car as he watched Tom and Jerry, Sky Kids and Home Alone.

We were due in clinic yesterday but when we called to ask what time to come in we were told that the blood test machine had broken and that they had 20 patients already waiting to be reviewed by a doctor and so they asked if we felt we could wait until tomorrow and so we are going in today at 1.30 which gave us plenty of time to unpack, settle back into our room in the Parents house and get some food shopping done.  

Ryan was pleased to see Vanessa Riddle who also came back to Tübingen yesterday for her third cycle of antibody treatment -she looks and feels great which is wonderful to see.  Within minutes he had her playing x-box with him!

Sadly our little friend Jamie deteriorated over Christmas, despite having fought to recover from the encephalitis he then got a second pneumonia which caused devastating damage to his already damaged lungs.  He is now on full life support with a machine oxygenated his blood outside of his body.  Over the coming days we hope and pray that his lungs will heal, they simply have to, it is not Jamie's time, he has fought so hard. It is absolutely  heartbreaking.