Not quite ...

http://www.epsomguardian.co.uk/news/9115287.Seven_year_old_cancer_boy_launches_appeal_to_fund_treatment/

Unfortunately the above article that appeared on the Epsom Guardian website today is a little different to the press release that I agreed with the charity. Small differences, but significant nonetheless.

I don't want to do the Epsom Guardian a disservice because they have been nothing but supportive of Adam's Appeal. In one instance last year they even contributed to saving us £26,000 during an impasse with Surrey PCT over funding for some of Adam's treatment. And of course I don't want them to stop writing stories about Adam!

However, I also don't want people to read the story and think we're doing something we are not, or that we're attempting to fool anybody. We are not launching an appeal, we did that last year as most people will know. As the actual press release said, it has been fantastically well supported. Fundraising is still ongoing albeit with less momentum than this time last year, because the truth is Adam's future is still as uncertain as ever. I also hoped it would make clear that we are still being supported by the same charity, it just has a different name now.

Perhaps I am being over-sensitive, but all I wanted was for the charity to make their appeal for runners, accompanied by a little update on Adam's progress. Not quite how it comes across ...

Sports Day ...

Adam is having a lazy day today, not going into school until after lunch. As nice as it is to see him having some small semblance of a normal childhood again, we don't want to overdo it. Especially as tomorrow is school sports day (well maybe, it's weather permitting of course)! Needless to say Adam missed sports day last year; it would appear from looking back on here that he was in between rounds of chemo. (It's actually proven quite useful having this online diary to look back on; it means I make fewer factual errors, which in turn means I get less of Alison telling me "that's wrong" and "you've got the date of that completely mixed-up" and "that never happened, don't you remember ... ".)

Sports day 2009 was just a couple of weeks before Adam fell ill, and we spiralled into repeat doctor and hospital visits that eventually culminated with Adam's cancer diagnosis. I had very recently started a new job back then, based locally so I managed to get along to watch. How little did we know that day what was to follow so shortly afterwards ...

Me and Adam at School Sports Day, June 2009

Morning ...

Morning friends of Adam! This is a picture of my little superstar taken this morning. Smiling as usual. If you look closely you can see his skin shedding. This is the retinoic acid's doing. It's a little disconcerting at times; last night as Adam was going to bed I noticed a tiny thread of skin hanging from his chin, so naturally my instinct was to pull it off ... and a much larger area of skin started to peal away along with it. I must learn to leave well alone in future.

We go to Germany ...

On Thursday evening Adam was sitting in the bath when he looked up at me and announced "I want to go into school for longer tomorrow."

"How much longer?"

"The whole day."

"Do you not think it might be little difficult for you to get up, get dressed, have your Avemar, NAC, Life Mel, wait an hour, have your breakfast, have all the rest of your pills, have your vitamin supplement, have your smoothie (a blended drink loaded with all sorts of stuff that Adam has twice daily) AND get to school by 9 o'clock?"

He paused to give this due consideration. "Ok then, I'll go in at the start of first playtime." First playtime is 10:30am.

At 7:30am the following morning Adam appeared downstairs dressed in his school uniform and clearly on a mission. At 10am he was ready to rock and roll. We would never have bet that it would turn out to be Adam waiting on his Mum to finish packing his lunch before he could go to school! He made it just as playtime started and remained for the rest of the day. He even had a friend round after school as well. It was ... almost ... normality. Normal people's normality I mean, rather than the normality to which we have become accustomed these past two years.

Monday was a staff training day at school, but this morning the process was repeated (well except that he didn't get himself dressed the minute he woke up), and he spent another enjoyable day at school. Although he did get told off today ... "and it wasn't even me that was doing it!" he told me indignantly.

On Saturday our road held a residents party. Adam spent hours on the bouncy castle and inflatable slide, up and down over and over again, along with Jake, Jessica and other children who live near us. I spent hours watching intently, waiting for the accident that (thankfully) never happened, telling him not to bounce down on his front as it wasn't a good idea when you've got a central line into your chest. There were a few bumps and a couple of upsets, but nothing too serious. Whilst we were at home having dinner they took down the inflatables, but that didn't stop Adam. He carried on playing; first it was 'it', next '40 40 home', then throw and catch, and finally football in the twilight hours. We finally dragged the kids away and put them wearily to bed at some very late hour, but they all had a great time. Adam so much so that he keeps asking us when the next one is!

Oddly enough Sunday was a lazy day. A very lazy day; aside from eating and watching television Adam did very little else.

Over the weekend I had a good talk with the professor in charge of the children's unit at the Royal Marsden. I'm pleased to report my head is now properly realigned and facing forward once more. We go to Germany.

(Just for Alison: that reminds me of the BBC1 production of Robin Hood from a few years ago. In every episode somebody would be captured by the sheriff and it would fall to Little John (in the shape of Gordon Kennedy) to rally the band of merry men to embark on yet another rescue mission. "We go to Nottingham". Cue collective tub-thumping, air punching and enthusiastic waving of bows, clubs and various other weapons of choice).

Getting back to the point; I don't doubt there'll be further doubts, because we are now completely off the beaten track. It isn't that there are no other children like Adam because there are. Actually I should caveat that. (Hint: You need to read the next bit slowly). There are other children that are how we hope Adam will turn out to be, as it remains to be seen in the fullness of time how our particular story unfolds. There are children who are 5, 10, 15+ years out from treatment who under diagnostic imaging present as stage 4 neuroblastoma. One day medical technology might catch up and find a better way of categorising them, but in the meantime they are anomalies. In the main, because these children fall outside trials and off standard protocol, they're a hitherto under-studied group. There is anecdotal evidence, but very little to be found in medical literature apart from an occasional retrospective study. Some of these children are on long-term low-dose therapy, but nobody knows whether or not that plays any part in their continued lack of disease progression. I can imagine that in the minds of parents, doing something must always be better than doing nothing, right? I daren't let myself hope (too much) that Adam is (or rather will turn out to be) one of these children. I know just how quickly and brutally this disease can destroy such hopes, and I know that for as long as both Adam and I are alive such thoughts will always be lurking in the shadows at the back of my mind.

Of the treatments we could give Adam now the one that clearly makes the most sense is retinoic acid, which he started last month. Normally it is given for 6 months following the end of treatment, but I'm very keen for Adam to continue (in the absence of intolerable side-effects) for a full year. There is a trial currently running in the UK to test children on retinoic acid to ensure they are retaining sufficient concentrations in their blood. It seems sensible to me to get Adam's levels tested, so I am trying to get him enrolled on this study even though he's already started the second cycle. If we're unable to do so I'll push to do the testing anyway outside of a trial setting. Generally speaking the biggest problem with retinoic acid levels is in younger infants who don't swallow capsules and therefore require the contents be extracted and mixed with food and drinks. The collective evidence of previous clinical trials is that the efficacy of retinoic acid is related to dosing. We know how much Adam is being given, but we don't know how much is being retained. So let's verify that he's getting to the levels that are thought to be necessary for the treatment to work.

After retinoic acid the next logical treatment is immunotherapy. He may need it, he may not. It may make all the difference, it may make no difference whatsoever. The side-effects are horrible, but generally manageable and reversible once treatment ceases. If they become too horrible, or unmanageable then we will stop.

After much consideration, I have come to realise that I have to believe - I mean really believe - that there is a chance that Adam would be 'cured' if we gave him no more treatment. How much of a chance? Not huge is my instinct, but not zero; if I tried to be more specific it would be entirely baseless. I also have to believe that if he's not 'cured' now then giving him retinoic acid might mature those remaining cells which if left will cause Adam's cancer to start growing again. And lastly I have to believe that if he's not 'cured' now, and the retinoic acid doesn't do the trick, then immunotherapy might cause Adam's own immune system to mop up whatever cells are left and thus prevent his cancer from returning.

When I say 'I have to believe' it's because that's the key to everything making sense. See how I've also conveniently avoided all mention of everything that lights up on Adam's mIBG scan. That's something I simply cannot make sense of; to think about it brings chaos to my well ordered mind.

And what does it even mean to say Adam is 'cured'. It's not a word we'll ever be able to use with certainty. Whether or not Adam is cured can never be proven, only disproven. ad infinitum. The longer he stays like he is the better the chance. Considering the long-term survival rate for children with high-risk neuroblastoma is around 30% I have to be thankful to still be able to talk about such an eventuality. If the neuroblastoma stays away or remains dormant, and Adam grows up to have a family of his own, then he probably has been cured. But it will be a long wait. And that won't be a biological family of his own by the way; that's all but impossible due to the treatments he's had. It's one of the things you sign away at the very beginning with the consent forms ...

Aarghh …. !

Having a major dose of the collywobbles over going to Germany for antibody treatment. Not the practical stuff, whatever it takes we will get on and do in spite of it being an all round major league pain in the arse. No, my problem is much more fundamental than that. My problem is whether or not this is the right thing to do full stop.  My problem is we cannot afford to get this decision wrong, to do the wrong thing. My problem is it might not make a blind bit of difference what we do, any of it. For all I know, this problem is insoluble. Or maybe it isn’t a problem at all.

There is just nothing concrete to go on (at least not that I’ve seen so far) other than opinions and instincts. And whilst these are opinions of doctors that I respect and for whom I have a high regard, they are still not the same as cold hard facts. In the meantime I keep hearing of kids relapsing either whilst in Germany having this treatment, or soon after completion. I’m not ignorant of the statistics regarding relapse in neuroblastoma, but it’s starting to get to me all the same. I need to hear the other side of the coin, some stories of children who have had this and haven’t relapsed. Or at least some numbers.

By definition this trial includes difficult to treat cases, who have failed frontline therapy, or at least taken longer to respond and therefore cannot have antibodies elsewhere. So maybe the success rate was always going to be poor (I’m just speculating here)? It’s not a treatment that was designed for an extensive disease burden such as Adam has that’s for sure. Or is it an extensive disease burden? Maybe not. We still don’t know what it is; only ever will if it starts growing again I guess. In all likelihood what lights up on the MIBG isn’t going to be touched by antibody therapy, so what if it does start growing again at some point in the next 6 or 7 months? Then we’ve blown it.

Give me strength. Please. I think I’m heading for a breakdown.

Onwards and eastwards ...

Howdy folks!

Adam is doing okay at the moment. He's off the retinoic acid for a couple of weeks and the skin peeling is only minor now, although there's still a little bit affecting his hands and feet. He's been going into school each afternoon and is really enjoying it. It wouldn't be practical for him to go in for any longer as he spends the entire morning alternating between eating and taking supplements! He goes in after lunchtime and leaves along with everybody else and that's working very well at the moment. There are certainly no ill-effects from it that we've noticed.

Adam’s MRI scan came back clear. Nothing showing whatsoever. Last Thursday the Marsden/St George’s joint tumour board discussed Adam at their weekly meeting. The definitive view was that there is nothing visible in any of the scans that would be suitable for biopsy. Just to recap:

• MIBG scan has extensive diffuse uptake in spine, pelvis, and femurs. Classification is stable disease, no change since the previous scans. There are no well-defined lesions, no actual solid tumours. Just lots of little tiny bits of disease. Lots. :-(
• CT scan is clear. :-)
• Bi-lateral bone-marrow aspirates are clear. :-)
• Bi-lateral bone-marrow trephines are clear for all standard and acknowledged tests for neuroblastoma. However, about 1% of the cells are ‘abnormal’ based on particular immunological testing. The significance of this result in unknown, but it’s not neuroblastoma. :-/
• FDG-PET scan is clear. :-)
• Gallium Octreotide scan is clear apart from some faint uptake in the right femur. However, the uptake does not correlate completely with what lights up in the same area on the MIBG scan. :-/
• MRI scan of left pelvis/hip/femur is clear. :-)

Add it all up and it means there isn’t anywhere for them to go in and do a biopsy, and the consequence of that is we cannot enrol Adam on the U.S. hu14.18 antibody trial as we had hoped to. Instead we are waiting for a date when we can start treatment in Germany. Now we have settled on a course of action the sooner we get started the better as far as I’m concerned.

So in the next few weeks we will be joining the ever growing list of UK families having to travel abroad for treatment this country is not capable of providing. Regardless of who you know, what you know, or how much money you have at your disposal there is so much that is not accessible in the UK at the moment. Sure the vast majority of it is experimental, but it is also an indisputable truth that some of yesterday's experimental treatments are today's standard of care. In many cases these early phase clinical trials give hope to families that would otherwise have none, and for some they do make the ultimate difference, prolonging life, or even leading to remission, or long-term stable disease. The only way for UK parents to access any of them right now is to take their child to a foreign country; splitting families up for long periods in the process, and heaping even more stress and anxiety into lives that are already difficult enough.

But what choice do we have? We just have to get on and do whatever's necessary. In many respects we are among the lucky ones; we know there is more out there than the UK has to offer. Believe me I have seen the look of utter disbelief in people’s faces who simply had no idea. Who, in blissful ignorance, honestly believed there was nothing over and above that which was available at the leading hospitals in the UK. I remember one day Adam innocently telling people seated round the colouring table in the playroom at the Royal Marsden that he was going to hospital in America for 6 months (this is back when we originally thought that’s where we would end up taking him). The incredulity of one poor chap in particular – he came into the parents room and asked me “What’s all this about going to America for treatment? Why do you need to do that?” He literally had no idea, it had never occurred to him. Then again I guess I was exactly the same before this whole nightmare began. I was just fortunate to meet some people (early enough) along the way who helped enlighten me. Without them I’d probably still be none the wiser; and Adam’s Appeal certainly wouldn't exist.

The trial that Adam will be on in Germany is a pre-cursor for a second antibody trial that will be opening in the UK, one that is all-inclusive to those who fail the eligibility criteria for the original trial. This second trial was conceived around this time last year, when the first child in the UK failed to reach high-dose within nine months of diagnosis and was therefore told they couldn't have the antibody in the UK. They ended up going to Germany and set the precedent for similar children to follow. One year on and the second UK antibody trial still hasn't opened. The last I heard, which was a few weeks ago, there wasn't even a date for when it would be open. It's easy to cast blame, point the finger, or shoot the messenger. I don't know why things are like they are in this country, but what I do know is we need to do so much better by our children.

Getting back to practical matters; Adam's treatment will be conducted in Greifswald (indicated on the map below) a town in northeastern Germany. It doesn't matter what mode of transport you choose there is no easy or quick way to get there.

View Larger Map

We are determined to maintain, as much as we can, an environment that is as close as possible to home. This means eating the same foods, taking the same supplements, having the same routine. It also means avoiding the same things. If Adam's appetite wains, if his habits change, we want it to be solely because of the treatment, not because of anything else. We know that's only going to make our lives more difficult, but we are totally committed to it. We'll be driving there for starters; a journey that takes somewhere in the region of a 16-hours.

The antibody treatment itself is no walk-in-the-park. Nothing ever is with neuroblastoma. It is, after all, one of the most difficult to treat of all childhood cancers. Using the experience of others as a guide, the first couple of rounds are likely to be the worst. There are some side-effects that are inevitable; loss of appetite, pain, fluid retention. And then there other far more serious, even life-threatening, complications. We simply won't know how well Adam will tolerate the treatment until we get started (and I'm sure it's not the first time I've said that on here). The schedule is roughly 2.5 weeks in Germany then 2.5 weeks back at home, repeating for 5 cycles.

Our doctors have already cautioned us that if Adam starts to become seriously unwell due the antibodies the best thing would be to lower the dosage, and ultimately to stop completely. We don't even know for certain that Adam needs this treatment. We think he does, the balance of probability is overwhelmingly that he does. But we don't know for certain. As it was put to us bluntly, but honestly, 'we don't want to kill Adam by giving him lots of treatments that he might not even need.' We are putting Adam through immunotherapy because we perceive the risk of doing nothing right now outweighs the risks associated with the treatment. It's not a very scientific assessment I know, but it's the best we've got. Honestly, I'm still not sure if we're doing the right thing. But neither would I be if we were doing something different. Or if we were doing nothing at all. I don't think I ever will be. For want of a more eloquent means of expression, it does my bloody head in.

To end on a lighter note there is one final piece of exciting news … as you can just about see from the photo, Adam's hair is really starting to grow back again now! Another week or two at this rate and he'll have some quite decent coverage. Not that he seems altogether bothered about it mind. He couldn't really give two hoots about being bald anymore.