We're sneaking in a few days away...

Adam is beginning to feel some of the after effects of the latest round of chemotherapy. He’s had half a dozen trips to the toilet today.  Alison has started him on Imodium to try to keep it under control. He’s also been a little off his food, and was sick once, though only after downing two fish oil capsules and a vitamin pill a bit too quickly.

We have made arrangement to spend a few days away on the south coast. One of our friends has very kindly offered us the use of their house. I know I keep going on about it, but we are so very lucky. This will be the first time since last May that we’ve been away. Seems longer. Saturday will be one year since we sat in that small pokey little room at St Georges and were told the lymph node biopsy had confirmed Neuroblastoma, Stage 4. Until then we’d still clung to the hope that there’d been a mistake, that the diagnosis would come back different. It wasn’t to be.

The day after we return Adam goes into hospital for a CT scan. He hasn’t had one for a while as the focus has been on his bone marrow disease and bone lesions. The purpose is to get an up-to-date picture of the primary tumour for the surgeon as St Georges who will operate on Adam next month.  There’s always a worry lurking not to far away that these scans will show up something they’re not supposed to. It’s human nature and, very sadly, it sometimes turns out to be true. Hopefully all they will find is a heavily calcified, shrunken, tumour ready to drop off into the surgeon's hands.

Anyway we’re going to try to enjoy the first little bit of the summer holidays that we have together before it all gets consumed by hospitals and cancer treatments. We’re going to try.

Adam's Appeal has now raised more than £300,000 since we started back in March. This is an unbelievable achievement and has happened because of the kindness, generosity, hard work and determination of so many people. 

A MASSIVE THANK YOU to everyone that has helped to make this happen.

I’d especially like to thank Debbie who has been managing the appeal, responding to emails, keeping tabs on all the collection pots, buying flyers & stickers, and running all over the county with t-shirts, banners and collection boxes. She has done a fantastic job (as I knew she would) and we can’t thank her enough. Thanks also to Amanda and Sheena for their continued help and support.

When we started the appeal in March our goal was to raise £300,000 to enable us to fund immunotherapy treatment for Adam in America around September time. We never imagined for one moment we’d be sitting here in July having already passed that figure.

The fact that Adam subsequently stopped responding to chemotherapy forced our plans to change, putting back timescales and narrowing our treatment options. At the beginning of July when Adam’s bone marrow tests came back clear it gave us renewed hope. He will shortly be undergoing a second stem cell harvest followed by surgery at the start of August and MIBG (internal radiation) therapy at the end of August. 

If the MIBG therapy clears Adam’s bone lesions then he will move forward to high-dose chemotherapy and stem cell rescue. If this goes well it’s possible we will be able to take Adam for immunotherapy towards the end of the year - November/December. The current situation is that our destination will be Germany or New York. The amount of time that Adam has been in treatment means he has become ineligible for trials in the UK and elsewhere in America. Ineligible for potentially life-saving treatment, not because there is evidence that it would be non-effective for a slow responder like Adam, but because a) the trial needs a degree of uniformity for those entering it, b) nobody considered the children that fell outside the trial, and c) there is limited supply of the antibody that would run out sooner if all the children are given access to it.

If the MIBG therapy fails then we may have reached the point where UK treatment options are exhausted. In this case we would have to look abroad to continue to fight this disease, again the most likely in Germany or New York. We would then be faced with the situation of having to spend hundreds of thousands of pounds just to get Adam to a position where he can go for immunotherapy treatment. 

Even after everything, immunotherapy included there’s still a more than even chance of relapse, for which there are no curative protocols in the UK.

With all we have learnt since we began this journey one year ago we know that no amount of money can buy a cure for this disease. We also know, however, that a lack of money can bring a premature end to the fight. We’ve heard such stories, and we’ve also heard stories of people spending a million dollars and more. Of people leaving their homes in the UK for years at a time. This could be our fate, we just don’t know. We will do whatever it takes, and use everything we have, to try to win this fight.

With the future so uncertain we’ve decided to continue fundraising for Adam’s treatment despite having now reached our initial target. The summer holidays will bring a natural lull in activities and by the time school starts back Adam should have had his tumour surgery and possibly MIBG therapy too. We should be closer to knowing where our journey will take us next.

It is our sincerest hope that the only money we ever spend is to take Adam for the immunotherapy, and that anything we raise above that can be used to help another child or fund research and projects to bring new treatments into the UK. However, we are realistic enough to know that there a better-than-even chance that we will have to take Adam outside the UK more than once, or for an extended period.

So the appeal will stay open. We know it’s the right thing to do. It is our intention to open a second appeal account, alongside the current one, with fledgling charity Families Against Neuroblastoma (FAN). FAN will allow us to fundraise for a broader range of things such as alternative therapies, travel, accommodation and subsistence. Having embraced an organic diet, fresh juicing every day, wooden beds, non-sprung mattresses, water filtration, non-plastic cooking and storage, and a variety of supplementation and alternative therapies in our pursuit of a cure for Adam's cancer we are committed to replicating all this wherever he travels for treatment. Clearly this will be both costly and time-consuming, but we know how hard this fight is to win. Nothing is going to be easy.

FAN is trying to grow sufficiently to be able to offer real support for parents of children diagnoised with Neuroblastoma in the UK. We know from experience that currently when a family receives the same devastating news that we did there is no clear organisation to turn to for information, help and advice. FAN aims to be that organisation.

We hope that by associating Adam's Appeal with FAN, and thereby introducing it to our friends and supporters, we might in some small way help it to become more widely recognised and supported.

It only remains to say that if we do not raise another penny we won’t be any less grateful for the efforts of so many people; friends, family, colleagues and complete strangers alike that have got us to this position. 

Thank you all so much. We wish you a safe and happy summer holidays.

Nick, Alison, Jake, Jess & Adam

15th July 2009...

The day Alison took Adam to A&E because the pain had returned in his neck and at the base of his spine. 

The day I took Jessica to her infant school leavers party. On the way I took a couple of photos on my mobile phone to show Alison when she returned from the hospital. It wasn’t until we were setting up the appeal in March 2010 that she finally got to see those photos.

The day I walked back through the car park having bought Jake those new boots for his football trial. Just before I reached the car my phone rang. When I answered all I could hear was Alison crying.

The day the doctors found Adam's tumour.

Another one bites the dust...

Adam is back on the chemotherapy whilst we wait to hear what happens next regarding stem cell collection. They harvested a total of 2.5 million cells last week over the two days. That’s barely enough for one procedure, but it’s sure better than none. The battle to get Adam to take his six capsules that we had last time was seemingly a result of his stomach bug and feeling unwell. Yesterday and today he took them all within a couple of minutes. Job done. The pre-treatment assessment at the Royal Marsden and his IV chemotherapy have also been unremarkable. His weight is back to a steady 23.4 kilos.

Things couldn’t really be that simple for once, could they? Of course they couldn’t. On Sunday Jake went off to a birthday party and less than an hour later we got a call to say he’d had an accident. It transpires that he was having a duel on the ‘Gladiators’ bouncy castle, and in the process of being knocked off his podium he fell backwards over the edge of the bouncy castle. We arrived to find him being attended by the paramedic and having entinox (gas and air) for pain relief. One look at his arm above the elbow and it was pretty obvious it was broken. The paramedic pointed out the clear deformity in it. He’d already called for an ambulance to transfer Jake to hospital. 

So we went to a new destination, St Hellier, the only hospital in the area that we’re not intimate familiar with. I rode with him in the ambulance whilst Alison followed behind in the car. He was in some degree of pain, interspersed with periods of spaced out delirium when he’d puffed too many times on the entinox.  

I must confess this was all new to me, I have no experience of broken bones. Ignorantly I thought a broken arm was a broken arm. I was even a little blasé about it. When we got to the hospital the attending doctor was brilliant with Jake. We couldn’t have asked for better care whilst we were in A&E. The first thing that happened was they stuffed him full of drugs; ibuprofen, morphine and another whose name escapes me. Then two quite unpleasant things happened. 

The first was the x-rays. The A&E doctor stayed with Jake whilst we had to stand behind the protective screen. The screams of pain as they put his arm into the necessary position to get a clear picture were horrible. The second was the orthopedic consultant telling us that it was a very bad break and they needed to operate within 6 hours because they were concerned about nerve damage. At the moment his radial pulse was okay and Jake still had feeling in all his fingers, but the pressure of the misplaced bone and associated tissue swelling risked damaging the nerves that ran through the area. I got a little more worried at that point!

They operated around 10.30pm and he was up on the ward by midnight. They straightened out and realigned the bone and inserted two wires to hold everything in place. It made a change for Alison to be spending the night in hospital with her other son. Apparently he woke up all chatty at 2am, 3.45am and 6am. She gets all the best gigs. The following morning he was discharged with a course of antibiotics as prevention against infection. He’s back on Thursday for another set of x-rays to assess how things look post-surgery. We were warned by the consultant in A&E that it was not uncommon with this type of break to require a second operation in the future.

It’s going to be a long summer holidays for Jake. At least six weeks in plaster. No cricket camp, no football training or in the park, no swimming, no cycling. He is going to be very bored indeed, and probably quite unpleasant to live with as a result. He even has some problems playing the xbox at the moment. That would be a total disaster if it persists!

I suppose some good came out of the whole sorry episode; at least I didn’t have to sit through yet another terribly dull World Cup final. Cheers Jake, mate. High fives!

Adam's Appeal Show

Today we all went to Adam’s Appeal Community Show. What an amazing town we live in. What amazing friends, neighbours, fellow parents, and others besides we have fighting for us.

The concert was great, the choir were fantastic (seriously they were REALLY GOOD). I’m sure when all the totting up has finished a massive amount will have been raised for the appeal.

Even more than all that though, it’s hard to believe so many people got up and sung for our little boy. A good number of them would never have envisaged doing anything like singing in a choir.  Then to see all the people that had come along to support the show, well it’s all very humbling really. It's amazing how one little boy, our little boy, has brought so many people together, and generated so much positive energy throughout the whole of the local community. 

We’ve been doing this for a few months now and you might think we should be used it. We’re so not. I’ve come to the conclusion that sometimes it’s better not to think too hard about it. So today I just wanted to go along and enjoy the show. Thanks to all the dancers, singers, musicians, and the 58 ladies and 2 men in the choir I did precisely that. 

P.S. When Adam's year one school friends came on and did their bit Adam was singing along with them from his seat. It was definitely the highlight of the show as far as he was concerned.

Stem Cell Harvest (3)

Adam’s first stem cell harvest on Thursday collected 1.5 million cells, and Friday’s was expected to obtain fewer because his CD34 count had dropped. Together the total number of cells will be barely sufficient for one transplant procedure. We - and by we I mean the hospital as it was all sorted out before I even opened my mouth to speak - have a plan. Next week Adam goes back on to the chemotherapy. Six capsules each day (don’t get me started about that again) and a 30 minute infusion. In a couple of weeks time we will try for another harvest, this time using Plerixafor, which has already been applied for. The only slight hiccup is that Adam’s platelets have fallen below the level where he can start chemo. They get consumed inside the harvesting machine. Fingers crossed they won’t take long to come back up to a good level. We're taking him up to Epsom General for a full blood count tomorrow.

They removed the vas-cath from Adam’s leg yesterday afternoon before we came home. The little fellow helped them by taking off the dressing and pulling out the stitches himself. ‘It hurts, it hurts’ he kept saying, but the nurse was very patient and just let him do it in his own time. Below is a photo of the line after they’d removed it. 

The stitches were in the green plastic pieces to hold the line in place and then everything after the lighter of the two was inserted into Adam’s groin and up into his femoral artery. Little wonder it felt uncomfortable! He’s felt no ill-effects though since it was removed. Been running around in the garden throwing water balloons everywhere for the last couple of evenings, having great fun. 

Stem Cell Harvest (2)

Adam’s CD34 count went up to 13 this morning so they decided to press on and try for a stem cell harvest. He went into surgery late morning and came out with a new line running up through his groin area and into his femoral vein. Adam’s first action upon waking from the general anesthetic was to whip down his pyjama shorts and assess the damage. After a few moments consideration he decided it wasn’t too bad, his leg just ached a little.

A blood count of 13 is still very low and the most probable outcome will be that they get insufficient cells. The worst possible outcome is that they get too many to justify (the cost of) another stem cell harvest but not comfortably enough for two transplant procedures plus some extra just in case we need them in the future. The cells are stored for something like five years and we know (from other peoples’ experiences) that having extra stored away can never be a bad thing. So it’s possible we may need to explore how we can fund an extra stem cell collection and storage. I don’t ever want to get to the stage when one day we look back and wish we had taken more stem cells when we had the chance. The other problem is that because Adam has mobilised without the support of Plerixafor I’m guessing that it’s now less likely they would agree to fund that either, even though it all but guarantees a much better harvest. Again my view is that if all it comes down to is money, however many thousands of pounds it costs to pay for the drug and the procedure and the patient care and the hospital stays, then we should opt for the most effective way possible.

The machine that collects the stem cells is a rather cumbersome looking beast. Doesn’t really look as though it belongs in a modern hospital. However, we’re all old enough to know that looks can be deceiving and what it does underneath the hood is really rather cool. 

Blood is drawn out of one of Adam’s lines and enters a centrifuge where is it spun and separated into different components. The bone marrow cells are drawn off into a bag and the remainder of the blood is warmed and returned to Adam’s body using the second line. (Clearly I have oversimplified somewhat but you get the idea). Children often get a strange tingling sensation whilst the machine is operating, caused by the anticoagulant that is added to the blood to stop it from clotting inside the machine. When it reenters the body it lowers the level of calcium by bonding with it. Adam didn’t seem to exhibit this side-effect but we gave him some milk to drink just in case.

The most notable thing about the whole process is just how boring it is. Adam had to lie on the bed generally motionless for several hours whilst three times the volume of his blood was extracted, filtered and returned three times over. 

Apart from a shuffle every now and then to get into a more comfortable position the only other noteworthy fact is that it was me on duty when Adam decided he wanted the toilet. Now I don’t know if there is a recommended approach to arranging a child so that they can have a poo in a cardboard tray on top of the bed. Or whether there are any instructions on how best to clean their bottom thereafter, whilst maintaining white bedsheets and retaining all doings in said cardboard tray. All I do know is it’s a skill that I haven’t quite mastered yet. Still we got it sorted out without the need for clean bed linen, which hasn’t always been the case. A quick trip to the sluice, thorough washing of the hands and an apology for the smell to the parents and two sets of grandparents sitting in the bay opposite, and we were back to boring again.

Tomorrow morning we will redo the stem cell harvest. Once that is complete and Adam’s vas-cath has been taken out we’ll be free to go home. All that will remain is to find out how many stem cells we got and how close we are to the target of six million. Not quite enough is my guess, but too many to call it a complete failure. I will let you know...

Five days is a long time

Wow. Five days is a long time.

Saturday night was an incredible evening. Adam’s Appeal Ball at Epsom College was a fantastic event and both Alison and I thoroughly enjoyed ourselves. I deliberately held back the news about Adam’s bone marrow for a few days because I wanted to announce it on the night. It went down well.

That was last week. We’ve moved on. We are on the verge of a (not totally unexpected) failed stem cell harvest. We’ve been discussing with the doctors what happens next; chemotherapy, another stem cell harvest attempt, surgery, MIBG therapy.  

I’m not sure that I’ve previously managed to convey just how critical every procedure is from now on. Perhaps the easiest way is to tell you about the exchange we had today with one of the doctors. Adam’s treatment plan is now off-protocol. It is no longer standard-of-care. So I asked what was the standard for a child in Adam’s position. The answer? Palliative care. It has served to focus my mind. It’s not like chemo where they keep going, change the mix. If Adam’s stem cell harvest fails again the likelihood is we are done as far as the UK is concerned. Major tumour surgery obviously carries with it certain inherent risks. If MIBG therapy fails to clear the bone disease, again we’re probably done as far as the UK is concerned. There will be no high-dose chemotherapy and no stem cell rescue (transplant) for Adam.

For those of you reading this that have seen Adam over the past few months; take a moment to reflect on how he has appeared to you, and then reconcile that with how best-practice medicine would deal with him. It’s almost impossible to believe. Yet that is the reality of the situation. 

Of course we can’t, and won’t, give up. We retain a positive attitude towards the challenges ahead. We are going to get the stem cells we need. The chemotherapy is going to keep the disease at bay. Surgery will be a complete success. MIBG therapy will clear enough bone disease to get Adam to minimal residual disease and allow him to progress to high-dose and transplant. In 6 months or so we’ll be ready to take Adam for immunotherapy.

However if things don’t go to plan; if the UK has no options left for us; we will spend every penny we have raised and everything else we have besides to try to beat this thing. That my beautiful little boy, Adam B, is a promise.

Stem Cell Harvest

The chance of the stem cell harvest going ahead are now very slim indeed. The procedure to insert the vas-cath has been postponed - it was supposed to be this morning. We will know for definite tomorrow morning that the harvest is cancelled. 

Adam has now had 4 days of GCSF and had his second CD34+ blood count this morning. The magic number he needs to reach to have a chance of a successful harvest is 10. He has gone from 2 yesterday to 5 this morning. The plan is to give GCSF tonight and more hydration (swells the blood vessels in preparation for the procedure to insert the line). His blood will be tested again tomorrow morning and if, as is likely, it remains well below 10 he’ll be discharged. We are anticipating that chemotherapy will recommence instead in the next few days.

This was always the probable outcome. It would seem logical that a failed harvest is a prerequisite to gaining funding for the use of Plerixafor to mobilise Adam’s stem cells. So unless something miraculous happens in the next 24 hours we will be retrying the stem cell harvest after another course of chemotherapy, and once funding approval has been sought and received.

Looking back and looking forward

When Adam was diagnosed with Neuroblastoma he could barely walk. He was given pain relief to allow him to lie comfortably in his bed. He was drinking only sporadically and was put on IV fluids because of dehydration. He stopped eating and very quickly became frightfully thin. He was severely constipated, and manoeuvring him on to a bed pan so he could try to go to the toilet brought frequent cries of pain. His bone marrow was stuffed full of Neuroblastoma, 85% of it had been eaten up by cancer cells. 

That was July 2009.

During the next 40 days we witnessed changes in both Adam's appearance and his general disposition. His hair fell out; he lost his eyebrows and finally his eyelashes. His face became bloated by the steroids he was on. There are photos of Adam that are not our son as we recognise him. He grew stronger almost by the day, and his day 40 CT scan showed that the tumour had shrunk significantly and his bone marrow aspirates were clear. It was fantastic news. 

Adam continued to improve. He spent less time lying around and more time on his feet. Less time watching DVDs and more time playing. I remember the first time he went back outside and played in the garden. It was late and almost dark. Adam was in pyjamas, a jacket and flip-flops. He was still a little unsteady on his feet, but there had been a time when we doubted we would ever see him running around outside again.

When induction therapy ended at day 80 we expected Adam to be clear of disease. Not hoped, expected. Looking back we must have been stupid to think this way, but the changes in him had been so profound since those dark days in mid to late July. Mentally we had already begun preparing ourselves for stem cell harvest and surgery. Dates for surgery at St Georges had been pencilled in for the week commencing 9th November. When we sat before our consultant and she told us that his bone marrow had come back positive for disease it was a huge blow. That was when we truly realised that how Adam is visually, and how Adam is medically, are not necessarily the same thing at all. We went home, held hands, and sobbed.

That was October 2009.

Adam moved on to a new type of chemotherapy - TVD. One cycle per month, re-tests after every two cycles. For the first four cycles he responded to treatment but his bone scans continued to show widespread disease, and his bone marrow results remained positive for Neuroblastoma. Our consultations began to become repetitive. We started the appeal in March as Adam started his final two cycles of TVD. A full 6 months after we received the day 80 results and this time the news was even worse. Bone scans and bone marrow results the same as two months previously. Adam was no longer responding to treatment, he now had 'stable' disease.
    
That was April 2010.

Adam moved to another new type of chemotherapy - temozolomide and irinotecan. The use of these non-approved drugs required written submissions by Adam's consultant and the funding for them had to be signed-off by our local NHS trust. Adam was approved for two cycles, with further approval required for any continuation, clearly dependent on how he was seen to respond. He underwent an MIBG scan, followed by bone marrow aspirates and trephines the next day. We were told within 24 hours that his bone scans had come back unchanged. As there was no clear evidence of progression, approval had already been sought and granted for another two cycles. Bone marrow results would take a week to process. Last Wednesday we attended clinic expecting to once again hear that the situation remained the same. Wrong again. We were told his bone marrow had come back clear in both samples. For the first time, after battling this disease for over 11 months Adam had reached the first rung on the ladder.

That was June 2010.

The clear bone marrow result means we now have a plan, a way forward. There are no certainties, nothing is straight-forward and believe me when I say the worst is still ahead. This week Adam will undergo a stem-cell harvest. On Wednesday morning he will have a vas-cath fitted into his groin under general anaesthetic. This operates like a dialysis machine. Blood is extracted, the stem-cells are filtered out and stored, and the blood is put back in again. They are attempting to harvest 6 million cells over the course of Weds to Friday (two hours each day), enough for two (transplant) procedures. Often this proves to be difficult in children that have been heavily pre-treated... and they don't come more heavily pre-treated than Adam. If they fail to get the necessary stem cells the plan is to give Adam another course of chemotherapy - he still has active disease as shown on his bone scans; and retry harvesting using a new supporting drug called plerexifor. This acts to mobilise the stem cells and has proven to be successful where natural production has been suppressed. It's also unapproved and unfunded, but I can't see Adam being denied access to it.

Even if the stem cell harvest is successful first time round another course of chemotherapy will still be given before Adam goes for surgery. After surgery the plan is to do MIBG radiotherapy, where Adam will be injected with the same active agent as with his scans but in a vastly higher dose. He'll then have to spend a week or more in the special lead-lined room, designed to protect the rest of us from radioactive contamination.

After MIBG therapy is high-dose chemotherapy; then stem-cell rescue (transplantation by one's own stem cells), radiotherapy, and finally immunotherapy and retinoic acid. As of today this will be in either America or Germany in November/December 2010.

We can't allow ourselves to get carried away. Adam may have cleared the first hurdle but he has many more to go, much bigger ones too. He is not even at the stage most children reach after 3 months. And the majority of those still don't make it beyond two years from diagnosis. That's the brutal truth of this disease.

Outside the fear and worry, the last few months have been relatively easy on us as a family. Adam has been in hospital only as a day patient and needed very few supporting drugs, none administered by IV. I have almost forgotten the last time he had a blood or platelet transfusion. He has been well enough to enjoy days out, have trips to school, and participate fully in a wide range of activities. It's all about to change. For the next 4 or 5 months Adam will be in hospital more or less full-time. You won't be seeing him running about in the park, or wandering around town nagging me to buy him something. You won't see us all out as a family. That's the price we must pay to try and get Adam better. It's time to transition ourselves back into 'hospital mode'.