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.

Thursday, 25 April 2013

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.  

Friday, 12 April 2013

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.

Thursday, 4 April 2013


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.