Monday, 16 April 2012

Been a long time ...

Adam is doing okay. Last week we had a repeat MRI at the Royal Marsden. It had been six weeks since the previous one which had shown no change five weeks after the MRI in Germany on which the small lesion/node/tumour in Adam's abdomen first appeared. Result this time was also no change. Still there, still the same size, hasn't gone away but most importantly hasn't grown at all either.

That's eleven weeks since 'it' first appeared on 23 January during which time there has been no change at all. It remains impossible to know what it is. However, the longer it remains unchanged, and remembering it did not appear on the last MIBG SPECT scan, the greater the likelihood that it isn't neuroblastoma. We know that it first became apparent following the end of antibody treatment. Therefore were it to be neuroblastoma it grew whilst Adam was on ch14.18 immunotherapy and 13-cis-retinoic acid. For the past eleven weeks it has not grown at all, during which time Adam has been on 13-cis-retinoic acid alone. So either it's not neuroblastoma, 13-cis-RA is more effective alone than when combined with immunotherapy, something innate within Adam's body is stopping it growing further, or something equally weird is occurring.

Come the start of May it will be 3 months since the last MIBG scan (the one that we feared would be devastatingly bad, and wasn't). So we have to decide whether to continue on the same scanning schedule, bearing in mind every one of these scans exposes Adam to yet more radiation. And the next question after that is how long to leave it now before doing another MRI? What do you reckon? I'm thinking somewhere between 6 weeks and 3 months? What would you do?

The start of May will also see the end of Adam's 12th cycle of 13-cis-retinoic acid. I've had a couple of meetings about what to do after, but nothing is settled yet. Nothing is even close to being settled. There isn't a right or wrong answer. We could do nothing and see what happens. Adam has lots of bone and bone marrow disease, an abnormal lymph node imageable by MRI, but despite that there is still a chance he could still be fine if we did nothing else. Problem is we'll only know whether Adam is in that very small group of children who do well despite seemingly responding badly after the fact. There isn't enough known about the biology of those patients whose disease matures and takes a benign course to be able to predict who they are in advance. Current imaging techniques are unable to provide differential diagnoses. Biopsy is almost sure to fail in the case of Adam's bone disease, and the risks outweigh the benefits where the suspicious lesion/lymph node is concerned.

At the other end of the treatment spectrum we could take Adam to America for 6-8 months and enrol him on an early phase clinical trial of an unproven treatment that may (if we had the benefit of hindsight) be completely unnecessary, may (years from now once it's completed and results are published) turn out to be ineffective at treating neuroblastoma, and may in the worst case scenario even end up doing far more harm than good. On the other hand we could get lucky and end up have a more prolonged period of stability by continuing treat. And maybe we hit the jackpot and Adam's disease is miraculous resolved (not that I actually believe that will ever happen).

In the middle is what I'm shooting for, and trying to figure out. Something that doesn't continue to impact too severely on the life of a child who's already been in treatment continuously for nearly three years. Something that let's us be a proper family, at least for a while. Something that has some scientific evidence to support it. Something that has objective reasoning behind it, rather than simply giving us a warm feeling because we're doing something rather than nothing.

If we happened to live reasonably near an institution in America that was doing a Phase I or II clinical trial that had showed early promise I would seriously consider it for Adam, even if it meant one or two days a week in the hospital rather than the classroom. But, of course, we don't. There is a trial that we could enrol Adam on at the Marsden (an mTOR inhibitor study for those who are interested), but I've looked at it and I just don't see the prospective benefit being worth (a) the amount of time we'd have to spend getting blood drawn for pharmacokinetic and pharmacodynamic testing, and (b) the side-effects of mouth ulcers and low blood counts.

There are a couple of ideas that I need to speak further with the doctors about, so we'll see where that takes us. Clearly we want to avoid going outside the UK if at all possible, and failing that to keep any time spent away to an absolute minimum. The only thing we've really ruled out at this stage is relocating full-time to America for the next 6-12 months. Options that may require us to travel back-and-forth are still on the table, but as yet we haven't figured out what's in Adam's best interests regardless of any such logistics. Answers on a postcard anyone?


  1. Nick, Lovely to read your update on Adam although we can understand your turmoil. Nils had a request from a family in Norway more than 12 months ago asking if we could put them in touch with anyone in the USA. The families young daughter Mia had Neuroblastoma. The hospitals in Norway had done their best for her, so we sugested New York. After treatment Mia is now back in Norway doing very well. The family were very pleased with the Sloan Kettering Centre and the treatment Mia received. Not like the UK, the Norwegian Government paid the expenses for Mia's treatment. If a cure can't be found within Norway, then the government do their very best to help families. Indeed, Mia has been on television over there, and different Norwegians have mentioned this to Nils when he has been both in Stavanger and Oslo. It is wonderful to know that you have helped a child through a very difficult disease, and also the family. Good Luck in making the best of a terrible situation. Sorry I have not been in touch as often as I should, but I am also having a bad time of it. Came out of hospital just two weeks ago yesterday. "Run Balmoral" is coming up again aweek on Saturday. This year I am a "Lucky Black Cat". Will send picture and hopefully it will give Adam a good laugh.
    God Bless - Take care. Sylvia.

  2. Hi Nick (hope this works, never been able to reply to a blog post before!). Thanks for sharing how things are going. What A minefield of options! And how difficult to know which path to take. I hope that in the coming weeks, you will see a sign and that things will become clearer to enable you to make the right decision. Big hugs to Adam, and love to your family. Stella