To Grand Rapids ...

Me again! Those of you who regularly read my updates will know by now that I do like to use the correct terminology wherever possible. So, in that vain, I will summarise the last week. Adam has been ok in himself and generally stuffing his face at everybody conceivable opportunity. His blood counts are shit, and thus far have shown no sign of picking up to any great extent. End of summary.

We've been giving Adam daily GCSF shots to try and stimulate is bone marrow to produce infection-fighting white blood cells, but to date they remain rock bottom. He was up at Epsom General on Friday for a Platelet infusion, and his Haemoglobin is sitting a little higher than the level at which they transfuse so it could go either way in the next day or two. We decided, in consult with Adam, to do GCSF by daily injection rather than IV. He's had so many injections now (50+ out in Germany last year) that putting some Emla cream on his thigh and one of us sticking him with a needle an hour later is so much easier (all round) than having his port accessed daily and being hooked up to an IV infusion, a process which can take an hour or more including the messing about with his port, line flushes, etc. Notwithstanding the fact that the Community Nurse team don't work weekends so we'd have to drive to hospital for it.

On Wednesday of last week I had a meeting at the Marsden to tell Adam's oncologist about our plans for the next phase of his treatment. So here goes …

Over the course of this week we're hoping Adam will continue to recover from the cyclophosphamide and topotecan, and that his blood counts will begin to pick up. On Thursday he's been selected, along with three other children, to help officially open Hobbledown, a new children's activity centre in Epsom (read here for the details). If his white blood count doesn't improve before then he won't be able to attend unfortunately, as we can't afford for him to be around others whilst at increased risk of infection that his body can't fight. In any case we'd like to thank those people who put Adam's name forward. We know he's a superstar, but no more so than any parent knows their child's a superstar as far as their concerned. That others see him thus, and recognise what he's been through, is something different. Not that I can quite explain it properly. Pride is the wrong word. I can't think of the right one.

Next week we're hopefully going to have a few days away; Jake won't be playing cricket every minute of every day so we can spend some family time together before Adam goes off to the U.S. for treatment …

Alison and Adam will be flying out to Chicago, and then on to Grand Rapids, Michigan on Monday August 6th to see Dr Giselle Sholler, Chair of the Neuroblastoma and Medulloblastoma Translational Research Consortium (NMTRC). The rest of that week Adam will undergo a battery of scans and tests; MIBG scan, MRI scan, PET scan, bone marrow aspirates and biopsies. It's going to be a very long week.

The following week he's going to start on a Phase I trial, of what precisely we don't yet know. It depends, to a certain extent, on the results of the scans. Most likely scenario is we do another round of cyclophosphamide and topotecan with the addition of oral nifurtimox, a combination that is running as a Phase I trial out there. Like almost all the drugs one encounters along this journey nifurtimox comes with side-effects, the most common being neurological and the most severe being seizures. Not a pleasant prospect, but the side-effects are temporal; they do not continue after treatment ceases. It's a unpleasant fact that nothing we've put Adam through over the past (almost exactly to the day) three years has come without risk of serious consequences. Whatever treatment we do start him on out in Grand Rapids, one things for sure. It's going to be another very long week.

If, and by that I mean if, everything goes according to plan we've booked flights home for the weekend immediately after treatment ends. The idea being that we want to fly Adam back to the UK whilst he is well enough before the inevitable, but routine, side-effects such as low blood counts occur. For one it's better for him to be at home as much as possible, and away as little as possible. For two, it's better for us to be together as a family as often as possible, and separated by four thousand miles as seldom as possible. And for three, it's pointless us having to pay for supportive care, transfusions, GCSF, etc. in the US when we can receive exactly the same on the NHS, and only have to pay for the parking.

There is slightly more to our plan than going out there, having a week of scans, having a week of treatment, and flying home. As long as The Royal Marsden give us their support we should be able to continue the U.S. trial in the UK. After doing the first round in Grand Rapids, subsequent rounds can be done back here; the intravenous chemotherapy backbone at the Marsden, and the oral drugs shipped in from the U.S. to be administered by us. The initial meeting with Adam's oncologist to discuss this went well, but I am sure there are other hurdles to be overcome before it can become a reality. Having said that we are talking about a properly regulated, recognised, clinical trial so hopefully it should simply be a case of ensuring there is sufficient information flow for the Marsden to be comfortable overseeing Adam's care whilst he is participating in another institution's trial.

The final element concerns Adam's bone marrow, which we know is an area where the disease has come back strongly. The NMTRC are running a personalised medicine trial which aims to biopsy a tumour site, determine the precise molecular make-up of those cells, and use the information against a database of therapeutic agents to make real-time, patient-specific, treatment decisions. Because of the nature of Adam's disease this isn't feasible in his case. However, they can take bone marrow samples containing tumour cells and attempt to grow them in the lab in order to do something similar. The difference being it might not be successful, it will in any case take longer, and it's not part of an official trial with strict adherence criteria. The upside is it might, and by that I mean might, provide us with some useful information that could guide our decision making in the future. It has to be worth a shot.

We also hope to be able to send a sample of Adam's bone marrow to Children's Hospital of Philadelphia (CHOP), for testing for known mutations that occur in a (small) subset of neuroblastoma patients (ALK and PHOX2B). It's not feasible to do this from the UK, as a fresh sample is required. Again we don't even know if the test will provide any conclusive result one way or the other. For bone marrow testing at least 15% of the sample must contain tumour cells. We know Adam had much more than this when he was last tested back in May, and we know also that his most recent MIBG showed further widespread and more avid uptake. Putting these together would infer he still has at least the same amount of bone marrow involvement, if not more. However, things are not always what they seem, he's had another round of heavy chemo since then, and the more times Adam's bone marrow get's taken the harder it becomes to get a good sample. The bottom line is that once again it's worth a shot. What's to lose? Except for a few thousand dollars? For me the bigger downside is the fact that bone marrow procedures are horrible and Adam is doing to be in pain and discomfort. Again. But I also know he'll soon get over it and be back to his old self. Again. And so we'll do it, and we'll hate it, and we'll move on, and we'll keep our fingers crossed it gives us some information that we didn't have before … and with it some more options, good ones.

That was the week that was ...

The first round of the new chemo combination is done and dusted, and we now wait for the inevitable downturn as Adam's blood counts drop. Almost certainly he'll become neutropenic over the next few days, meaning he has no natural immunity to fight bacterial infections, and will have to be admitted for a minimum of 48 hours intravenous antibiotics should he develop a fever. It's also highly likely that he'll require blood and platelet transfusions later in the week. He hasn't received any transfusion since March last year; it's obviously not great to be back in this situation again, but we knew before we started on this course of action what it would entail. Due to the significant amount of bone marrow disease Adam has his body's ability to produce blood cells is already weakened. The additive effect of the chemotherapy is almost certain to drive them below the thresholds at which transfusions are required.

Adam tolerated the chemo itself quite well. He had a reduced appetite and was noticeably more tired than usual, but he was never nauseous or feeling sick. He did have constipation earlier in the week, but that was rectified with the help of some docusate sodium. It was a long week though, long and tedious. Thirty minutes of cyclophosphamide, followed by thirty minutes of topotecan, followed by six hours of hydration. With the wait to get started, flushes, change overs, etc. it was eight hours at the hospital each day, leaving after breakfast and not arriving back home again until after 6pm. By the time he did get home Adam was really tired and washed out … literally I think from all that fluid.

The combination of chemotherapy drugs Adam's had has been well used previously in the US, and forms the backbone of several ongoing trials there where it's administered in combination with a novel third agent. However, it's not often given in the UK to neuroblastoma patients, though I don't know why. I have a suspicion that Adam might even be the first child with neuroblastoma to receive it at the Royal Marsden. Adam's had both of the drugs before, so unfortunately his disease is not being attacked by anything it hasn't seen before. However, he's not had them as a direct combination, so we hope they will succeed in arresting the progression in his bones and bone marrow.

Whilst there isn't anything too dramatic to report, the week was not without it's ups and downs. Monday is always a pain no matter what because it's the first day of treatment. Consent forms to sign, blood counts to do, assessment by a doctor required, Adam's port needing to be accessed. It was a long day, and set the pattern for the rest of the week; daycare bustling with patients, nurses and doctors when Adam went in, and empty except for two or three nurses by the time he left.

Having got Monday out of the way it was all supposed to be plain sailing from Tuesday. Straight in, chemo straight up, clock ticking, out in time to get home and cook tea (so I didn't have to). Err, no. From the minute the nurse first accessed Adam's port on Tuesday morning it was clear a problem had developed overnight.

Adam's port is a central venous access device that is installed beneath his skin and runs into a main vein providing a route via which IV medications can be administered. There is an access point on Adam's ribcage, - it juts out like he's got half a table tennis ball stuffed under there. This is the 'docking station' into which a needle is inserted to provide an external line on to which chemotherapy etc. can be connected. Ordinarily there is no external line (which is why Adam can bath and swim freely with a port, which he couldn't with a hickman line). When he's having a week of treatment we put emla cream over the access point on Monday morning and about an hour later a needle with external line attached is inserted by a nurse, and it remains there until treatment finishes on Friday when it's removed and Adam goes back to just carrying this lump around in his ribcage.

Unfortunately on Tuesday, the line wasn't behaving as it should, and when the first chemo infusion was started pressure in the line built up and triggered the pump's automatic cut-off. Repeatedly. To the point where the only option was to remove the line and needle from the access point on Adam's chest, and replace it with a new one. Immediately, so the infusion could continue. Without time for emla cream to be applied and an hours wait for it to numb the area. I wasn't there, but it wasn't pleasant for Alison to stand helplessly by and watch Adam cry out as the old needle came out and a new one went straight back in again. What should have been a less stressful and quicker day had become quite the opposite.

If Tuesday was a low point in the week, Wednesday turned out to be the high point. Although I did get rather annoyed at being threatened with a ticket for waiting outside the children's unit for a parking space. I have a child inside being poisoned (yet again) with toxic drugs at my own request, Alison is parked in the main car park using an annual parking ticket that cost us £100, and I've got this muppet telling me 'you can't wait there, you will get a ticket' in his softly-softly-I-am-only-doing-my-job voice. I didn't swear. I didn't even raise my voice (well not much). I just told him that I didn't want to hear another word come out of his mouth. And when he proceeded to tell me 'I am telling you how you can … ', I cut him off and reminded him of what I'd just said. Being in a situation like ours is incredible stressful and sometimes, just sometimes, you snap. I think it's disgraceful that parking in hospitals is so expensive in the first place, but taking it to this extreme is crazy … and asking for trouble. I'm sure there'll be other incidents if he keeps on like that.

Anyway, what Wednesday was really about was a visit by Allan Pickett, head chef at Plateau restaurant in London, who came into the hospital and showed the children in the school room how to make their own pizzas, which were then cooked in the children's ward kitchen and served for lunch. I don't know about the others, but I can tell you Adam's pizza was pretty good. All of us had some, apart from Jake who doesn't eat pizza at all. Visits like this really do brighten up everybody's day, children and parents alike. Adam has something positive to do, we all have something positive to talk about, and the day is about something other than cancer and chemotherapy.

By Friday Adam had just about had enough of the monotony of the daily routine. Even the afternoon arts and crafts session in the school room had become too familiar. Home-time couldn't come soon enough. Which made it all the more surprising to see him more active on Friday evening than he had been at any other time during the week. Whether having the port de-accessed and being line free made a difference I don't know. Or perhaps knowing he was finished for this round gave him a fillip.

He's carried those good spirits, and general wellness into the weekend. Yesterday we went into town and got those haircuts I talked about. In the end Adam opted for a short look for himself, and a sensible trim for his Dad. His reasoning, he said, was that he didn't want to be embarrassed by being seen out with me sporting a ridiculous hairdo. All week coming home in the car, and again over the weekend, he's been tugging his hair to see if it's starting coming out. So far he's come away empty handed each time. I fear it may be too much to hope for it to stay that way until at least after the next round …

A new plan (of sorts) ...

After much consideration we've got (the start of) a new plan (of sorts) worked out. Adam will head to the Marsden Monday to Friday next week for a different chemo combination - cyclophosphamide and topotecan for those who are interested in such details. This is a more potent mix than the combination we've just done two rounds of, but at this point we don't have much choice. We don't quite know what effect it will have on Adam's already fragile bone marrow, but it's possible he'll end up being neutropenic and in need of blood and/or platelet transfusions. Any fever and we'll be back up to Epsom General waiting on blood cultures. Feels like we're going back to the dark ages. I guess we are. Adam's bone marrow is a particular source of worry, since his blood counts are likely being affected by the amount of disease. Any further progression could have profound consequences, notwithstanding the bone marrow suppression caused by the chemotherapy. We need adequate blood counts for Adam to be eligible for clinical trials. As Phase I trials evaluate side-effects rather than efficacy they exclude children whose counts are already low, as it would then be impossible to assess the impact of the new treatment.

We're planning to use the recovery period after this chemo to decide where to go, and what to do, next. There are a few options, but it's not exactly easy interacting with doctors across the Atlantic. At least I've not been finding it easy, but maybe it's just me. So Plan B is to simply put Adam on a plane, take him to the US, visit a few doctors and make a decision after that.

There is a chance we'll stay in the UK. For a 2nd round of this chemotherapy combination, and possibly for a new Phase I trial that is due to 'open soon' at the Marsden. There are 3 existing Phase I trials, none of which sound particularly promising to me. The new trial is a combination of drugs on 2 of those 3 trials. The only problem is 'open soon', which in oncology speak translates to 'is planned and will be open when it's open'. It's a drug company sponsored trial across multiple centres worldwide, so I think the first the Marsden will know it's open for enrolment is when they get notification from the central coordination unit. It's not actually something over which they have any direct control.

Last night, after he came out of the bath, I told Adam he would be starting treatment again at the Marsden on Monday. He counted out how many days that was, and was overjoyed that he had four whole days left until he had to go back to hospital. His reaction when I told him his hair was likely to fall out again was the polar opposite. He cried. He doesn't want his hair to fall out. He loves his hair. We'd been chatting a few minutes earlier about how Wednesday would be his shower night. Our broken shower unit had (finally) been fixed yesterday, and it was the first time he'd used the 'disco' shower head that Ryan bought him a while back. Now you may be asking yourself what I was doing telling him he was probably going to lose his hair straight after he'd told me how great it was that Wednesday night was going to be shower night from now on. I simply say to you that there is no good time to deliver shit fucking news. It 'felt' like an opportunity to broach the subject. Or maybe I just couldn't bring myself to play along with him at that particular moment. I felt awful of course, but somebody had to do it.

Adam being Adam, and me being me, his state of deep despair lasted approximately two minutes. What he hates most about his hair coming out, he said, is that bits get left on his pillow and it feels itchy. So Mum has been instructed to buy a dozen new pillowcases and Adam will be having a fresh one every night for as long as he pleases. He'll also be selecting a new range of headwear to add to his already burgeoning collection. And last, but by no means least, he gets to cut his Dad's hair any way he wants. And that, though I'm not quite sure how given he doesn't watch football, means you should not be surprised to find me sporting a Mario Balotelli look this time next week. And do you know what? The prospect does not bother me one iota …

Results ….

Adam's scan results were not those that we had hoped for. His response to the two rounds of chemotherapy has been mixed, and mixed simply isn't good enough. Although the diseased soft-tissue lymph node has shrunk, the widespread skeletal disease has further worsened. We are in the midst of formulating a new plan. As I type Alison is at the US Embassy in London trying to secure a visa for herself and Adam. At least nobody will be stealing her phone on this trip into London … she's not allowed to take one. Or any other electrical equipment for that matter.

Adam remains well in himself. He is pain-free, eating and sleeping is pretty normal. For that, I suppose, we must be thankful.