It occurred to me this morning ...

... that yesterday's update was somewhat incomplete. Not where the facts about Adam are concerned, but in a more general sense regarding where things are at now, and where we might be heading in the future.

That neuroblastoma is deadly is without question, but it also has a certain strangeness. It is one name but many diseases. It's not very well understood, and despite all the advances in medicine over the last couple of decades there remain lots of unknowns. It has an almost entirely benign form, and an ultra-aggressive form at the same time. The spectrum in between is vast, and there are very few absolutes. I find it incredibly difficult trying to understand enough to believe we are making intelligent and informed decisions for Adam, whilst at the same time accepting that I probably know next to nothing.

Here is some basic information to begin with; neuroblastomas are highly malignant tumours that originate from tissue that form the sympathetic nervous system. Ganglioneuromas are benign tumours  that originate from the same. One is a mature form of the other i.e. neuroblastoma cells can mature into ganglioneuroma cells. Ganglioneuroma cells can dedifferentiate to become cancerous. Tumours can be formed by a combination of the two types of cells - this is referred to as ganglioneuroblastoma, where immature neuroblastoma cells are either mixed in with, or form clumps amongst, ganglioneuroma cells. It's already a minefield, and we've barely gotten started.

For many many years all children, including newborns and those very young, were treated the same. As though all neuroblastomas were the same, distinguished only by a few established markers such at N-MYC amplification, and by whether the disease was confined to a localized tumour or had metastasized to distant sites. All children were given intensive treatment, and some undoubtedly died as a result of these medical interventions rather than neuroblastoma itself. Subsequently it was discovered that in a number of very young children (usually < 12 months old) the most appropriate course of action was to do nothing, to adopt a watch-and-see approach. For in many cases amongst this particular group neuroblastoma spontaneously regresses and disappears without any treatment at all. As far as I understand it the why and how of this spontaneous regression remains unknown, but is assumed to be related to their early developmental stage. Of course there are now children for whom a wait-and-see approach is taken whose neuroblastoma does go on to become progressive. For them early intervention or tumour resection would, with the benefit of hindsight, have been the best course of action. This world is not, and never will be, perfect.

There is also another group of neuroblastoma patients; children for whom intensive and continued treatment does nothing to reduce their observable disease load. These children are collectively referred to as refractory. More and more clinical trials in the United States are being opened specifically to target refractory and recurrent/relapsed neuroblastoma. They are hard to treat successfully and the prognosis is generally very poor indeed. Treatment after frontline therapy has failed is an area where our NHS is way behind medical institutions across the Atlantic. Adam is in this refractory group.

It would seem natural and obvious that being in the refractory group is a universally bad thing, but it's not necessarily always the case. Within this group there is another smaller subset of children, albeit the exception rather than the norm, who become long-term survivors despite never ever reaching the panacea of NED (No Evidence of Disease). There are some of these children in the UK, there are plenty more in America. In general they are (re-)diagnosed with chronic neuroblastoma. It is assumed that their 'disease' has either undergone maturation or otherwise taken on a more indolent form. In this state, whilst it will still light up on MIBG scans, it is completely impervious to chemotherapy, radiation, and whatever else may be administered to try and combat it. The one thing these children do have in common with the 'wait-and-see' group is that their lives can be destroyed by endless rounds of unnecessary treatment.

So you can now really begin to see how difficult this really all is. To the point of being practically unfathomable. There are children who have a remarkable response to induction therapy - 80 days of on/off chemotherapy and all their disease completely disappears. And then back it comes, twice as fast, twice as aggressive, and there is nothing that can be done. There are children who respond to frontline therapy, go through high-dose, radiotherapy, immunotherapy having no evidence of disease and yet relapse days, weeks, months or years after. Most relapse within two years, but within five years is not uncommon and even beyond that remains a definite possibility. There are, of course, those who respond to frontline therapy and go on to become long-term survivors. Then there are those who have refractory disease, who remain stable for an extended period of time before eventually succumbing to progressive disease. And then there are long term survivors who move from childhood to adulthood with the same level of 'disease' that they had when they stopped treatment. Who, if they were scanned today would still look identical to a stage 4 neuroblastoma diagnosis.

As time has gone by I've got to wondering how long Adam had been living with neuroblastoma before he was diagnosed. Had it been with him all his life? Are we at this juncture because Adam has had slow growing disease for many years? He's never had a dramatic response to anything that's been pumped into him, and generally speaking cytotoxic agents used in the treatment of cancer tend to work on faster growing immature cells that are rapidly dividing. That's why they cause hair loss and bone marrow suppression because of their indiscriminate nature. Set against that his presentation on diagnosis with extensive bone marrow, lymph node and skeletal disease, coupled with his N-MYC amplification and the fact that his primary tumour wasn't ganglioneuroblastoma, is suggestive of something altogether different. Did he have a slow growing form of neuroblastoma that mutated into an a more aggressive strain? Are we treating the latter so that we remain with just the former? Is it even possible? So many questions, but the only truth is that whatever answers we come up with they are mere supposition, and we'll never ever know the true story.

So where does this leave Adam, and the prognosis for his future? We have to believe he is in that small subset of children who will go on to long-term wellness despite what shows up on his MIBG scan. I think we've come to a point where we can no longer harbour false hope that Adam will ever be clear in the conventional sense. We haven't reached a point where we have no hope, but rather we now have a different hope. Our baseline has moved from being no disease to being no more disease. It doesn't seem such a monumental change when thought about in those terms, does it? Whilst there is no progression, and no new disease in other areas, we can still have hope, we can still believe. It may not be the most probable scenario there is for Adam's future, but it's not one that is entirely beyond the realms of possibility. As one of the nurses at the Marsden said to us when we first started out on this journey and began to learn some of the awful truths about neuroblastoma - "Even if statistically the chance of long-term survivorship was only 1% you have got to believe that Adam will be in that 1%." And she was right. Hope that is not false hope, however small, is a very precious thing indeed.

Scan results …

The last two weeks have been pretty tough; waiting to find out where things are at after Adam’s bone marrow tests, CT and MIBG scans. I’ve lost count of the number of times we’ve been down this road over the past year-and-a-half, but for some reason this time the purgatory was worse. Or maybe it wasn’t really, maybe it’s simply that every time seems worse than before. It’s being in the moment, filled with fear and uncertainty, versus looking back all-knowingly after the fact. Each and every time the fear gnaws away inside your head saying ‘This time it’s going to be bad. This time the luck’s going to run out.’ I’m sure people that have trodden this road themselves will know what I mean.

This morning we received the results.

Stable disease.

Despite all the chemotherapy he’s had; despite two rounds of high-dose radiation therapy, despite high-dose chemotherapy with stem-cell rescue, Adam’s MIBG scan shows the same disease burden he's had for the past year. His scans continue to light up throughout his spine, pelvis and at the top of both femurs. We've been told, more or less, that in all likelihood Adam will never be 'clear' of whatever it is that he has got, and at best whatever it is that lights up on his scans will always be there. Moreover, there is currently no way to determine, other than through the passage of time, what it is. If it’s mature cells he could live with it for many years to come. If Adam is still alive and well in 30 years time we will be able to say with some degree of confidence that it isn’t, and wasn’t, active neuroblastoma. That’s the prospect we are now faced with.

The CT scan of Adam’s abdomen, specifically in the region of his primary tumour, was completely clear. His bone marrow aspirates were also clear. The bone marrow trephines showed a very small percentage of ‘abnormal cells’ on immunological testing. However, their presentation was not consistent with Neuroblastoma, and by all standard measures the results from the trephines are considered clear as well.

Whilst I had hoped everything would somehow miraculously disappear, and as much as I worried that things would show up that weren’t there before, this is the outcome that I very much expected. It means we have to get our heads around the fact that unlike normal cancer patients the ultimate aim of having no evidence of disease is one that we realistically have no possibility of reaching. The goalposts have been moved. At some point we will have to stop treatment and just 'hope' that what's left is not malignant. However, we're not at that stage yet. We’re not even close.

We have begun talking to Adam’s doctors about where we go from here. Our Consultant and Professor Pearson at the Royal Marsden are being supported by experts from other countries. Our most likely next move will be to take Adam to either Germany or America for some form of immunotherapy, and at the same time start him on a course of oral retinoic acid. This does nothing for what lights up on his MIBG scan, it's to try and prevent him relapsing in other areas. It's not beyond the realms of possibility that we will do 6 months in Germany and then transfer to America for 6 months, so that we do two treatments back-to-back. We should have a decision in the next couple of weeks, with treatment starting sometime in May.

In one sense this is an odd update. Normally I have little difficulty with the tone of what I’m writing. I mean I have no idea how it comes across to other people, but (knowing what it is that I’m trying to say) it’s usually clear to me when I look at it. Reading back what I’ve just written I don’t get that. Is this good news? Is it bad news? Well, it’s good and bad news really. Most of it is what, deep down, I expected to be writing. Our most optimistic forward forecast is that we have at least another six to twelve months of difficult times ahead, emotionally, practically and logistically followed by a lifetime of uncertainty. At the same time we’re ever thankful that we have our middle-sized boy still with us, and that at least we do retain some hope for the future when many others do not. Immunotherapy is no walk-in-the-park, intense pain, dangerously low blood pressure, hives, severe fluid retention - all manner of side-effects can, and more often than not do, occur. At the same time Adam has tolerated a multitude of treatments to date remarkably well, with a courage and maturity that belies his years. So it’s a mixed bag I suppose; there are both positives and negatives to be found depending where you look.

Flat - that probably sums it up best.

The Weekly Update ...

Wednesday again (or at least it was when I starting writing), and at the risk of becoming too predictable I figured it was time I posted another update. The short answer is that things seem to have improved since last week. How much of what was going on then was really real, and how much of it was psychological on our part? Who knows? Does it even matter? The only truth is that it always seems real, it grips you like it's real, it consumes you like its real.

Ironically the thing that put our worrying on hold was the bone marrow tests that Adam had last week. After the procedure Adam had his usual post-anaesthetic 'episode' and threatened all who came near him with extreme violence, whilst screaming at the top of his voice that he wanted to go back to sleep, and wanted some more sleepy medicine “NOW! I SAID GIVE ME SOME MORE SLEEPY MEDICINE NOW!" Pity those people sharing a bay with him. Pity Alison who had to smile sweetly through her 7-year-old son's 25 minutes of insanity. As we've both previously discovered to our cost there is absolutely no point in trying to intervene whilst it's all going off.

Alison was warned that Adam might be more sore than usual (!) as they'd had to do some digging around to find any viable bone marrow. A consequence of so much treatment in general, and the recent high-dose chemotherapy in particular. They couldn't even put the really small plasters on that Adam prefers (insists upon) because there was more blood than normal. Sure enough when he arrived home he was both much more sore, and much less mobile, than he had been when he'd left that morning. The first thing he insisted on though was replacing the hospital plasters with nice neat round ones from our medicine cupboard. His 'holes' were definitely over-sized this time round compared to previously, but despite that I was a big brave boy and helped him change the plasters. Never mind how Adam feels about having his holes, I can barely stand to look at them!

Anyway back to the main point of all this - after the bone marrow extraction Adam's movement for the next few days was more of a hunched over shuffle. There was no running, no jumping, no trying to play football ... and consequently no foot pain. And because Adam stopped complaining, we stopped asking. And because we stopped asking, we started to relax a little more. And because we started to relax a little more, we stopped looking for any tiny indication that something was not right. They say no pain, no gain; well in this instance Adam's pain was most definitely our gain. Sorry little fella. I mean middle-sized fella.

By Sunday Adam was getting back to something like his old self again; less conscious of his war wounds, back to playing football outside, bouncing on the rebounder, climbing on, and jumping off, the furniture. He's not back to where he was before high-dose, but sometimes I overlook just how tough that was on his body even though outwardly he tolerated it better than most.

He was given a platelet transfusion before the bone marrow procedure, predominantly to counter the risk of excessive bruising and bleeding in the event something went wrong and emergency action had to be taken. His post-transfusion count was the highest since high-dose, so even if he's still not making too many platelets himself he is at least managing to hold on better to those that are donated to him.

Fast forward to this week and Adam has been back at the Marsden; for a CT scan on Tuesday, and MIBG scan on Wednesday. Our appointment to discuss results with the doctors is next week. I'm not very hopeful that we will be any further forward than we were before Adam went through high-dose and transplant, but I am fearful things will be worse. Seldom hopeful, generally fearfully. It's a permanent state of being really, only more acute than ever at scan time.

Important days ahead ...

All is not entirely well in the house of Bird at the moment. On Sunday Adam began complaining of pain in the bone of his right foot, and has since been moving with a definite limp. There is nothing specific that we can relate it back to; he was quite active the previous week - out playing with Jake several afternoons - but there hasn't been an incident or a fall or anything that we could point at to give us the comfort of an innocent explanation. And so our minds naturally turn to a far darker, and far more sinister, cause. We're taken back to the time before his diagnosis when Adam went from unexplained pain, to excruciating pain, brought about by Neuroblastoma in his spine and coccyx.

And then there are other little things that in and of themselves don't seem like much, but that aren't normal. And not normal is not good. Not normal is worrisome. Adam's temperature has been elevated this week, not feverish but higher than 37.0 normal, and quite a bit higher than his usual readings. Adam's blood pressure today was significantly above what it usually is, not high enough to be of clinical concern, but high enough for us to pay attention to it. Elevated temperature and blood pressure are also symptoms that can be caused by Neuroblastoma, and Adam had both when he was diagnosed.

His bloods are still struggling to recover; platelet count is low but more stable than it had previously been, Hb has been dropping whereas before it was relatively good.

It's difficult not to bundle all these things together and end up with a bad bad feeling. Things are a little tense right now. Tomorrow Adam is at the Marsden for bone marrow tests (aspirates and trephines). Next week he is back for CT and MIBG scans. Only then will we have answers to the questions that are consuming us at this time.

An Even Quicker Update …

Short of going into banal details about what Adam’s been eating, what he’s been watching on television, and what games he’s been playing with his brother and sister, there isn’t a great deal for me to report on at the moment. We’re in a holding pattern until Adam’s scans at the end of this month. We’re doing a pretty good job of not thinking about them too much to be honest. Once we’ve received the results, and held subsequent consultations with experts both here and abroad, we know we will need to make our biggest decisions yet. We know it’s looming on the horizon and whatever we do now isn’t going to make one iota of difference. So rather than tear ourselves up with what-ifs and maybes we are just trying to get on with day-to-day life.

Adam remains well. He’s still requiring regular platelet transfusions, but his white blood cell and haemoglobin counts have both been stable for the past week. There’s nothing to be done other than wait for his platelet production to come back online, however long that takes.

On Sunday we had our first trip to the caravan that last summer provided such a wonderful release from the stresses and strains that have dominated our lives since Adam became ill. Adam himself loves it there and can’t wait to go back. It’s good for him to have something to look forward to, it’s good for all us.

This summer is going to be very different indeed. What a bastard (apologies for the language).

A Quick Update ...

Has it really been over a week since I lasted updated this thing? I guess it must have been. In truth there isn't a huge amount to report. Adam continues to be quite well - he is eating well, drinking well, sleeping well; we are gradually easing him back into his daily routines. He's still not producing his own platelets, which remains the only real problem at the moment.. Most recently he has had double transfusions (two bags of platelets each time) which has meant we have only been going back to the hospital every three days for them. Yesterday before his latest transfusion his platelet count had actually held steady overnight (from 13 to 14), but it remains to be seen if this is the first sign of some positive improvement or just a one-off. His numbers are being checked daily; weekdays the community nurse comes to us, and on Saturday and Sunday we run him up to Epsom General for a quick blood draw. It's been almost a year since we spent anything like this amount of time up at our local hospital; the nurses have all been remarking on how much Adam has grown since then!

Now that the little fellow is back home, our house is back looking like one large playroom. His 'belongings' - I can't even refer to them collectively as toys - have (once again) spread into practically every room. Whilst he was in hospital his Grandads between them decorated his bedroom (to Adam's own colour specifications), so we are just waiting for a new carpet to be fitted before he can move back in.

This afternoon we met with Adam's consultant for the first time since he completed stem cell transplant. His next set of scans are being scheduled for the end of March, to give sufficient time for all the treatment he's had to do what it's going to do.  Once we have those results we'll have a better understanding of where things are at, and what sort of discussions we need to have regarding future courses of action. In the meantime we just have to keep Adam well, build him back up as much as possible, and deal with the after-effects of high-dose which basically means flushing all that nasty toxicity out of his system.

And Again …

Adam’s check-up on Monday was the standard fair, examination by the doctor and a full blood workup. Looking good so far was the verdict – well in himself, eating and drinking at one end, disposing of waste in the normal manner at the other. Haemoglobin at 8.3, platelets at 30, so move to twice weekly bloods and an appointment is booked with Adam’s consultant for next week. Thank you very much and goodbye.

Both Alison and I shook our heads disapprovingly as she recounted this to me. Twice weekly bloods? How can that be right. When he’s having a platelet transfusion on average every two days? Oh well, no point in arguing. Better to just ignore what the doctor said and arrange our own schedule with the Community Nurses and Epsom General.

Fast forward to Wednesday and Adam’s platelets are 5 (x 10⁹ per litre of blood). The normal range is > 150, and generally they will transfuse below 10 (or if there is evidence of bleeding and bruising). So it’s back up to Epsom General this evening for Adam’s 4th platelet transfusion since last Wednesday. I think we’ll be getting his blood redone on Friday now as well. Twice weekly indeed! His haemoglobin has dropped below 8, once it reaches 7 he’ll need a blood transfusion too. And they really are boring, 3+ hours at a time. At least his white blood count is holding pretty steady - I just wish the other stuff would hurry up and do the same!