Reflections...

As the weather turns colder and the festive season draws nearer I've found myself reflecting on the past twelve months.

This time last year we were between cycles of TVD. In total Adam had six cycles of this most hideous of chemotherapy cocktails, and it did very little for him except cause some irreversible heart damage.

I sometimes wonder why he stayed on it for so long, and although I think I know the answer I'd rather believe I'm wrong and it was something else. For although Adam's disease did not respond, it didn't progress either. And so 'stable' is deemed to be a positive response in itself, even though nobody could predict whether this would have been the case without anything at all. If you add to this the fact that the next drug combination on the list requires pre-approval and funding by the PCT, therein lies my answer.

Twelve months ago I was much less cynical, or much more naive, depending how you look at it. I do know I was pushing really hard for each and every cycle, querying any delays and questioning decisions that I didn't like. The longer Adam went without chemo the more I thought we would wake up one day to find him once again in agony. Agony that would almost inevitably signal the beginning of the end. This time last year I was desperate that we would arrange things so that Adam could spend Christmas at home. The reason was simply this; I honestly thought it would be his last. Believe me, it's a horrible place to be mentally.

As it turned out we brought Adam home late afternoon on Christmas Eve. He managed as best he could to enjoy Christmas and Boxing Day, before the effects of the chemotherapy kicked in and we took him up to Epsom General where he spent what remained of 2009.

It wasn't until February/March of this year when it really began to dawn on us that the chemotherapy wasn't having any material effect on Adam's disease and his path was not going to be one that followed that of the other children whose stories we'd come to know. We'd put off launching an appeal, telling ourselves we'd do it once Adam got through the first stage of treatment. By the time we'd completed six months of TVD we realised we needed to act, to ensure that when the time came, and wherever Adam's treatment needed to take us, we would have the money to do what we believed was best.

We will never be able to properly express our thanks to all of those people, to all of you, who
have raised such an enormous amount of money. I could literally spend the next hour reeling off names of people and organisations that have held events, raffles, quiz nights, collections, sponsored events, or made individual donations to the appeal. The list goes on and on. People are still getting in touch, wanting to help and to raise money for the appeal and we are just extremely grateful for every penny that has been, and continues to be, raised.

I sometimes wonder how people perceive us. Given that I have a child with a life-threatening illness I really shouldn't care, and yet I do (I think it all comes back to that question of it being impossible to express our gratitude, the whole thing is just too big). We launched the appeal with a huge fanfare in March, did tremendously well - exceeded every expectation I ever had - and then since the summer holidays we've kind of crawled back under the shell from whence we came, coming out occasionally to post things on Facebook or on here. The only thing I can say is this; when we started the appeal somebody said to me about getting the money raised quickly so I could go back to being a full-time Dad. At the time I kind of dismissed it, I was on a mission, spending hours and hours doing the appeal stuff, staying on top of everything. And then we reached the milestone of £300,000, and the summer holidays came, and almost without knowing it I stopped wanting to push to raise more and more money. Instead I wanted to go away with kids, and spend the time doing mundane, normal, everyday things. I think back to what that person said to me and realised she was right. First and foremost I needed to go back to just being Dad.

One thing I don't do any longer is worry about money. I used to. I used to wonder about using the house to pay for treatment abroad. And then what happens if that's not enough, if after the best options, or indeed only options, are abroad. This disease of Adam's is like that. You can reach the end of the road in the UK and there are possibilities in America that offer real hope still. I am talking about a major commitment and having to live there for as long as it takes. If you go for treatment at Memorial Sloan Kettering (MSK) they require an upfront deposit of $350,000 before they start treatment. If the disease spreads to the brain, MSK are the only place with any proven treatment, one that can still offer the possibility of a cure. Not just a few extra months, but a treatment designed to completely eradicate the cancer. Of course it comes at a price, something in the region of $1,000,000. I used to worry about all this stuff. Not just 'what if it happens?', but 'what if there is absolutely nothing we can do about it because we haven't got the money?'. There are other reasons why that might still be true; for example the hospital refuse to accept Adam as a patient, or he is unfit to travel. But the money one was always an issue for me as I thought it was something we could do something about. And that's what the appeal has done. In March, I didn't think we'd raise £300,000. Now I feel as though whatever we need to raise, whenever we need to raise it, there are people looking out for Adam who will do all they can to make it happen. Of course my greatest hope is that we are never faced with that situation.

So we approach December in a not totally dissimilar position to last year. Adam had his first ever clear bone marrow biopsy in June, and tumour surgery in September. The bulk of his metastatic disease, however, remains largely unchanged. Despite eighteen rounds of chemotherapy and high-dose internal radiation (¹³¹I-MIBG) therapy he still has widespread disease in his spine, pelvis and femurs.

On Friday and Saturday Adam will be in the Marsden for IV cyclophosphamide, both as a holding measure and to prime for a third and final stem-cell harvest. If we only get the same number of cells as the previous two harvests (~ 2 million) this will give us enough for a second transplant procedure in the future should that become necessary. If we don't do this now we will never have the opportunity to do so again unless Adam is completely cancer free. On Sunday 5th December he will go in for 3 or 4 days for the harvest itself. After that we await to hear what the plan is in terms of 2nd MIBG therapy followed by high-dose chemotherapy and transplant.

It could well be that this year Adam will be in hospital for Christmas. If he is we will just have to get on with making his Christmas, and that of his brother and sister, as good as we possibly can.

Finally...

Adam's blood counts have held steady this week, and so we can go forward with the plan to have chemotherapy this weekend in preparation for stem-cell harvest the week after next.

We knew it, we just bloody well knew it...

Adam's blood counts on Wednesday were up. Platelets at 161 and Neutrophils at 0.9. Given what we know about Adam's counts this is good enough to go forward to chemotherapy followed by stem cell harvest.

But...

Adam can't have chemotherapy this weekend because the stem cell collection machines are booked out the week after next. Which means that although his blood counts are now good enough, we still cannot proceed.

Which means that he will now have gone two months without any treatment (since MIBG therapy). And his last chemotherapy will have been in August.

Which means we have to do it all again with his blood counts next Wednesday. And we know Adam's blood counts come with the same warning as investment products - they can go down as well as up.

Which means we will have had a delayed stem cell collection at The Royal Marsden in August because of scheduling and PCT funding, delayed tumour resection at St George's in September due to the surgeon being on holiday, delayed MIBG therapy at UCH in September due to staff shortages and now delayed chemotherapy and stem cell harvest The Royal Marsden due to availability of equipment.

None of which has anything to do with Adam, or his condition, or his treatment, or standard-of-care. For a little boy with an advanced form of one of the most aggressive cancers that's an awful lot of delays. For the parents of said little boy that's an awful lot of extra worry and anguish whilst their child isn't getting any of the treatment they know he needs.

The mystery of the missing Neutrophils...

Adam's blood counts have always been something of a mystery to me. The number of times we've waited for his Neutrophils to climb back up above 1 before being able to continue treatment. The 'magic' numbers are platelets above 100 and Neutrophils above 1. MIBG therapy supposedly has most impact on platelets and yet these recovered whilst the Neuts were still on the decline, down to 0.4 as of last Wednesday. This low count was responsible for Adam missing his planned slot for priming chemotherapy last weekend.

One could speculate that he has no immune system. His white blood cell count is just dormant. We had a complete blood work-up done on Adam in October (not on the NHS) before starting MIBG therapy. As part of this the lab performed 88 different food intolerance tests, each scored from 0 (no intolerance) to 4 (extreme intolerance). The tests are conducted by detection of an immune response to the introduction of each different food stuff. Adam scored zeros right across the board, 88 of them. Such results are extraordinarily rare. Our practitioner even asked the lab to double-check them. So maybe this lends weight to the hypothesis that Adam has no immune system. But then wouldn't we expect him to get ill more often? We don't live in a sterile bubble by any means, and yet Adam has only had one short-lived line infection since he was diagnosed last July. Bit of a sniffle now and again and a sickness bug that he caught off his sister, but only one proper infection.

So basically I just don't get it. I do know around 0.8 or 0.9 is Adam's ambient level post his induction chemotherapy. On more than one occasion I've found myself asking why 1 is good enough to have treatment, but 0.8 or 0.9 isn't, because I had little expectation that if Adam missed treatment because of such that the situation would be any different a week later. Of course, the doctors couldn't give me an answer because none of them knew. You get to realise when it's just one of those things that are documented in the protocol. An indicator that's been assigned a number by somebody somewhere, not a scientific rule. In the end I'd get the head of the unit to agree to proceed at .8 or .9. It's one of those times when knowing your child, and being able to advocate for them, makes a tangible difference. A shot of GCSF (growth factor that stimulates the bone marrow) and they'd immediately shoot up into the teens; like they were just waiting to be flushed out.

Like I said - a mystery to me.

Quick Update...

Adam remains quite well in himself and had the most brilliant birthday celebrations - more on those another day. His blood counts, or more specifically his neutrophils, remain too low for him to go forward for chemotherapy this weekend. His bloods will be checked again next Wednesday and we'll go from there.

Unfortunately nothing is ever as straight forward as it might seem. The hospital are effectively fully booked for procedures to the end of the year and Adam was being 'squeezed in' as one extra. Which was fine when the plan was for him to go in this coming weekend, things had been scheduled accordingly. But now everything will have to be shuffled around again to accommodate him. Oh well, not my problem I guess. At least not unless things get delayed because of something other than blood counts.

Scan, scan, scan, scan. Scan, scan, scan, scan. ...

For those of you wishing to find out how Adam's doing without having to suffer my narrative style, there is a much shortened update below.

The second part of this particular update has been waiting in the wings for a while. The reason I've not published anything until now is that we met with Adam's consultant yesterday to discuss the results of his latest set of diagnostic scans. His previous scan was back at the end of June so there has been a prolonged gap between then and now. Of course there was plenty going on in said gap. Chemotherapy, two stem cell collections, tumour surgery, MIBG therapy. But no diagnostic scans.

The (MIBG) scan itself was last week. Radioactive isotope injection on Wednesday, imaging on Thursday. I tried to figure out how many of these one-hour full body scan's Adam has been through and I think the total, including the latest one, is nine. No doubt he'll be into double figures before too long. Alison and Adam arrived home from the hospital on Thursday to find a message waiting for them on the answer phone. Could Adam please go back so they could re-scan his abdomen, the images they'd just taken were blurry. I wouldn't say panic set in but it was, despite the apparently innocent explanation, a bit unnerving. This was because (1) nothing like this had ever happened before, (2) the area in question encompasses the majority of Adam's imageable disease, and (3) the scans were, as always, taken away to be spot-checked before Adam was given the okay to leave in the first place. Thankfully the return trip was uneventful and they quickly squeezed Adam's re-scan in so there wasn't any waiting around. When I asked Adam later what had happened he told me "My winkle was itchy so I had to scratch it". So there you have it, a perfectly plausible, and quite innocent, explanation. Or was it? Was there something more to it? Were we just looking for a way-out? When anything unexpected happens there's always this nagging suspicion in the back of your mind that this is it, this is bad. To fully appreciate this mentality I think you either have to be a parent of a child with this kind of life-threatening illness - or a paranoid worrywart.

Last Tuesday Adam did his usual trick of attending school just as it was about to close for the holidays. He went in first thing in the morning, got told off in assembly for talking, and ran out crying. The teacher in question didn't actually realise it was Adam who she was telling off and to be fair it was his own fault for talking when he should've been listening! He's lucky I didn't give him a clip round the ears when he got home. He stayed for the whole morning and thoroughly enjoyed himself. I took his pills in for him to have at lunchtime and he told me he was 'staying until last playtime, at least'. Shortly after I arrived back home we got a call from the school to say Adam was in the office and he'd like to come home now. Clearly his desire to stay for another playtime was outweighed by his desire not to sit through anymore lesson time. Unsurprising given how much school he's missed. His school are fantastic though, they are happy to accommodate him whenever he wants to go in, for however long or short his visits might be. It's especially nice given that his appearances often cause excitement amongst some of the other children and a bit of disruption to normal classroom activities.

The radioactive iodine used in the MIBG scans, though much weaker than that used for therapeutic purposes, has a half-life of 14 hours and so he wasn't able to go back into school after his hospital visits on Wednesday or Thursday. But mark my words, one of these days he WILL go in on two consecutive days!

With hospital visits out of the way we went to the caravan for some more fresh air, cycling and feeding of ducks. We always planned to return on Wednesday so that Adam could have weekly bloods taken and have his central-line dressing changed. On Tuesday the hospital called to say we'd been booked into Wednesday clinic and could we please make sure we attended. It's impossible not to get worried on the eve of a post-scan visit. We've stopped hoping that Adam's scans will show marked improvement; instead we now hope they do not show any marked progression or areas of new disease. We've been reconciled for some time to the fact that there's no quick way out of this for Adam, or for us. Well not one with a happy ending anyway. What we hope instead is that we're in for the long haul. At the end of which we will, finally, prevail.

After building works that have been on-going since before our first visit last July, the new children's unit at the Royal Marsden has finally opened. It won't though be fully operational until the existing building has been renovated to accommodate the daycare unit. At the moment daycare and consultation rooms occupy the ground floor of the new building, and inpatients the upper floor. In it's final configuration, once daycare have moved back out again, the inpatient ward will occupy the whole of the new building; ground floor for infants and young children, and the first floor for teenagers and adolescents.

After a quick checkup Adam went off to play leaving Alison and I to speak to his consultant. Queue rising tension. "The latest MIBG scan remains much the same as before. If anything there is some slight improvement in the femur." And relax, it's okay. It means I can give you all the information I couldn't give you before, in case the results of the scan rendered it all meaningless (plus I didn't want to tempt fate). One final thing that's worth mentioning is that these results do not mean the MIBG therapy has failed. It often takes longer than four weeks for the full effects of such treatment to become apparent. Sometimes considerably longer. His blood counts continue to drop, though not to levels that require transfusions. We're hopeful that scenario will be avoided altogether.

So, to the update that's been waiting in the wings.

Last week I had a very good discussion with Prof. Pearson, head of the children's unit at the Royal Marsden and the leading UK expert in Neuroblastoma. A very good person to have reviewing your child's treatment plans. Also a person very much in demand and with a very busy schedule. Nonetheless he's always been happy to discuss Adam with me, and has returned my calls on numerous occasions when I've felt that high-level intervention has been required.

Until now the feeling has always been that the best way to proceed has been to try and get a clear scan before going into high-dose chemotherapy and transplant. However, Prof. Pearson feels strongly that the time has come when the benefits of that approach are out-weighed by the risks that the NB cells targeted by high-dose may become resistant to treatment and eventually lead to a relapse. Going through with high-dose and the remainder of the upfront treatment now, and trying to deal with any remaining disease afterwards is, in his opinion, the right approach. Obviously I am summarising somewhat but I left feeling much more confident than when I went in. Not about what treatment would or wouldn't do for, and to, Adam. But about the whole 'plan' going forward.

Providing Adam remains at least as well as he is now clinically this is the plan. First we are going to attempt a third and final stem cell harvest. I have always been perturbed by the fact that we don't really have enough stem cells for two transplant procedures. It's borderline at best. And once Adam goes through high-dose it will be a long time before we could even contemplate another harvest. Should things take a turn for the worse, having no stem cells definitely limits the treatment options that are available. So we are going for a peripheral blood stem cell harvest and then, if that fails, a bone marrow harvest which is something the Marsden have not actually needed to do for a number of years. Adam is pencilled in for cyclophosphamide over the weekend of 13th/14th November. The exact date depends on his blood counts recovering sufficiently from the MIBG therapy. The cyclophosphamide is to prime him for the harvest as well as acting as a holding chemotherapy. It takes around 10 days for blood counts to drop and then begin to recover again - which is when the harvest is conducted. So that should be from 23rd November (harvest always begins on a Tuesday), assuming 13th/14th for priming.

After that the sequence looks like it will be; 2nd MIBG therapy, either at the Marsden or back at UCH; straight into high-dose chemotherapy and transplant; (possible) 3rd MIBG therapy; radiotherapy and then antibody/immunotherapy treatment in the former East Germany. There is a possibility that by the time Adam reaches the final stage a new antibody trial, for which he would be eligible, will have opened in the UK. As things stand, however, it's still most likely that we'll need to go to Germany. America is no longer an option (unless a new trial also opens up there) as he does not meet their entry criteria of having reached high-dose no later than 9 months after initial diagnosis.

It looks likely that Adam will not be able to escape Christmas in hospital this year. Last year we brought him home on the afternoon of Christmas Eve. This year Adam could well be in the midst of high-dose/transplant… which means he'll either be sick in the Marsden, or even sicker in the PICU at St George's. Nice. He doesn't know any of this yet. No point in telling him until possibly becomes definitely. Working on some rough timescales immunotherapy (Germany) would begin sometime in the first quarter of next year. And the treatment would be completed towards the end of next summer. At which time we'll know whether or not all the treatment has had any effect on his existing disease burden. Anyway there is a long way to go between now and then, and we'd be getting very much ahead of ourselves to even think beyond the end of this year. It's good to know and understand what might lie ahead, but the focus has to be on what comes next.

Adam turns 7 on 9th November and we've organised a party for him on the Saturday before. On Friday afternoon before school finished for half-term he took in all his invites. Fingers crossed nothing crops up to spoil it and he has a fantastic day. He deserves to. There's an even bigger treat lined up for him on his actual birthday… but that's a story for another day.

Scan, scan, scan, scan. (The Shortened Version)

Adam's MIBG scan from last week showed little change from his scan at the end of June. If anything there is some slight improvement in his femur. The plan now is for Adam to undergo a third stem cell harvest, 2nd MIBG internal radiation therapy, high-dose chemotherapy with stem cell transplant, 3rd MIBG internal radiation therapy, radiotherapy and immunotherapy in Germany. All that will take us up to the end of next summer.