Small windows of opportunity

It is odd how the mind works. Over the last few days my rigors and fevers have improved, I have had periods of undisturbed sleep and, other than a couple of humours qualms, I am deemed ‘well’ by the haematology team. And yet, when I sat to type this, tears flowed faster than the words. For no reason. I am not sad, a little tired yes, but not upset. Perhaps the previous months have caught up on me, or perhaps I just found Biffy Clyro particularly sentimental today. I don’t know. But if you can imagine your screen as a sheet from my little black book, there would be small salty droplets marking it. Not as authentic as a coffee ring, I grant you; well I’ll give you creative license to add whatever features you wish. I was hoping to provide an insightful blog post during our last limbo period, you know, an overly descriptive piece regarding my thoughts and feelings on various aspects of medicine and life. One of my sisters simply loves these entries. Unfortunately, I didn’t have the strength or energy to do such a thing so you have all been saved. For now.

Despite my relapse, my situation still fell within the BCSH guidelines. But the lines were a little blurry, the directions a little vague, and by the end you cannot help but think the document is simply shrugging at you. That being said, we were left with three treatment options. The first was to continue with Brentuximab. I was all for this, but my reasoning was purely emotional. I desperately wanted this cool ass drug to work. But it hadn’t. They scanned me to prove it. I’ve been told the unused portion will be transferred to another patient. They may have said this to ease my guilt. The second option was Gemcitabine; another chemotherapy agent. My cells, however, seem suspiciously resistant to chemotherapy, so I imagine this thought pattern was discarded rather quickly.

Seemingly, this left one final option: a double stem cell transplant. But wait, there was an outsider. An option that, travelling feverishly back from the Coromandel, I had not considered. No one, I imagine, had considered it. Around the time I relapsed a drug company began offering a new unregistered drug to refractory Hodgkin’s lymphoma patients for free. I did not meet the criteria but my consultant opted to apply anyhow. It sounds like he had to compose a novel. Upon completion of his manuscript, he returned and said he was not hopeful at all, don’t hold your breath, this will never be accepted. We forgot about it and concentrated on the original final option.

A double stem cell transplant. There are two types of stem cell transplants. Autologous, using my own stem cells, and allogenic using donor cells. The first is a way to administer high dose chemo. The second has further curative aspects. The donor cells work to eliminate the cancerous cells. Because they’re foreign cells, they can recognise that Hodgkin’s cells aren’t normal. It’s like the donor cells walk into a masquerade ball, have a quick look around and cannot believe Hero could be so foolish; that is clearly Don Pedro not Claudio, and they hope Beatrice is leading Benedict on because his disguise really isn’t that great. Then they kill them all. Yes I turned it into a tragedy. Are you lost? I think I am.  

Where was I? Right a back-to-back stem cell transplant. It was finally going ahead. Global emails had been sent; this was the consensus. First up would be the autologous with some BEAM to eliminate as much disease as possible. Then when I recover, say after two months, they would smash me with the allogenic. Hopefully, in that two month period they would find a donor for the allogenic transplant. The donor cells need genetically similar, HLA matched, as we say in the industry. Although I have two sisters, I again landed on the wrong side of statistics. Neither are suitable donors. They tell me somebody in Germany is a full match. Germany have a strong donation culture. Many people donate stem cells more than once in their life. And they donate all around the world. Even with my occupation, I was unaware of this need for donors. I cannot believe I was never on a register. Don’t worry, my stem cell harvest post is imminent, so I can implore everyone to join the donor registry. Anyway, they had two months to sort out Germany. I myself was facing six months of incapacitation, and that is not hyperbolic, it is necessity. Further disease staging was performed, namely another bone marrow, but don’t worry I asked for the gas again. Always ask for the gas.

My stem cell transplant was scheduled for Friday. We had an appointment with my consultant Wednesday for consenting and a final rundown on the procedure. I’d booked my last supper at a classy Wellington restaurant for that evening. But the consultant had a bombshell. You know before the Shakespeare tangent, the HLA tangent, the Germany tangent, there was that drug application made. The one we had all given up on. Yeah that one. Well it had been approved. My consultant explained that we were now in uncharted territory, probably somewhere off Bermuda. In a triangle. The global medical consensus (there had been more emails) was to continue with the transplants. The Wellington team felt they should continue with the transplants. My consultant was neutral, or at least portrayed neutrality, and the decision was ours to make. We had three hours.

I am not an apt enough wordsmith to convey how serious this decision was. It was the biggest of our lives, although, granted, we have had few of those lately. I am often asked if my medical background helps with my treatment. Well it obviously hasn’t helped physically. As far as understanding goes, it has definitely been beneficial when communicating with clinicians, and it has possibly helped me to rationalise my disease, although ignorance would have made relapses much easier emotionally. In this situation, my occupation and my affinity for nerding out, helped substantially. I had heard about this drug before leaving the UK, I knew it was in development, but I never expected it to be available to me. I had read about it, knew the uber cool science behind it, the potential side effects, the results from the small (very small) clinical trial, and to know all this, and to be able to absorb further information rapidly, was priceless. So, our question remained, do we opt for the conventional treatment, the one the experts are suggesting, the one with a known curative possibility; or, or, do we opt for this new drug, with little long-term data, with little data at all, a drug nobody in the haematology department has used, an experiment really.

Well, I am a scientist after all. I am always up for an experiment. And the biology behind it sounds solid. After a three hour stroll around Newtown and far too many coffees, we announced that we would try the new drug. I was so close to having that bloody stem cell transplant I’d nearly declared it to the online world. But this time I turned the bastard down. It was at least on my terms. And I still went out for my classy dinner.

         

There were further reasons that lead us to our conclusion, reasons that may seem less than rational. The drug is offered through a “Compassionate Access Scheme”. It had barely opened for Hodgkin’s patients when I re-re-relapsed, and remained open only a few weeks. I believe it has now closed. Such a narrow window, and yet I had slid through. It was almost my duty to give it go. Those who know me will be well aware of my spiritual and religious beliefs. If anyone thought there was a slim chance that I was agnostic, I am not. The more I am compelled to reflect on my mortality, the more I believe that when I die, that is it. I am not coming back as a cat, I never consider the pearly gates, nor Dante; all I imagine is nothing. Definitely an atheist, sorry guys. So I do not believe in fate, but I do believe that if an opportunity opens up, like this window, well I should probably take it. Ok, maybe I will return as a cat burglar given my apparent window obsession. Obviously all my clinical data will be collected, so I can flatter my ego with the belief that I am contributing to future medical advances. Liv, the ultimate humanitarian. I spoke of my medical background, and clinical trials, and science, and making an informed decision quickly; but it was predominately personal reasons that lead us to our final outcome. I found it quite difficult to go against medical consensus.

A new drug, a distended abdomen and who's that sexy beast in the mirror? 

The drug is in my veins now. It took two weeks of further paperwork before it was cleared, and I was too afraid to update the blog in case everything fell through. As you’ve probably gathered, plans have a tendency to change at the last minute. And I didn’t have the energy to type a retraction. I probably would have Fairfaxed out. During these two weeks my disease progressed rapidly. I suffered unresolvable nocturnal fevers resulting in terrible mornings. The drug infusion itself (one hour, once a fortnight) was an enjoyable anti-climax. Unfortunately, the following day was one of those terrible mornings. I had a pleasant mustard complexion and a yellow glow in my eyes. They admitted me, and to be honest I didn’t argue. They were concerned that these were side effects, however I knew it was all disease. That night my fevers were again unresolvable. This caused quite a stir in the morning. I informed my nurse I had a fever. She knew. She’d been watching me for five minutes. My resting heart rate was over 150. The alert team were called. About six doctors and five nurses streamed into my little cubical. All the while I was mumbling “But this is just normal morning fevers, this isn’t the drug.” I made a joke about dying. It fell flat. Not funny in a cancer ward. They pumped me full fluids, and more fluids, and more fluids. Too many fluids. Eight kilograms too many. And it all ended up in my legs. I won’t be climbing through any windows for a while. I have to lift my elephantine limbs off the bed. My underwear are two sizes too small and I spend my days with elevated feet, wondering if it is acceptable to simply forgo trousers. It is rather comical. Mainly because my fevers have eased and this is probably my biggest ailment. Oh that and my spleen! Yes you get to hear about that again. My spleen is massive and painful, as is my liver. Both are enlarged to the extent that I was used as a training abdomen. Twice. A medical student was ecstatic because one of his goals was to palpate a spleen. What can I say? I aim to please.

So this is long. But it is current. I was only discharged Monday. I know I have been deliberately coy about the drug. I am yet to receive confirmation as to whether I can name it, hence I haven’t. And the New Zealand media are currently a little sensitive about cancer treatment. I am itching to launch into the science behind it; patience Liv. Basically, it is not chemotherapy, it is immunotherapy. It works to turn my immune system against the cancerous cells. This is why I am feeling pretty positive about it all. Chemo wasn’t working, this might. And how else can I feel?

If all goes according to plan

So. It has been a while since we last spoke. As you may have noticed I have retracted into my shell, occasionally sticking my irritable neck out for food and water, but generally content to sit in my own darkness, insulated from the outer world. I think it is my bald head. It kind of makes me look like a turtle. I am pretty distracted and this entry has been the victim of severe procrastination so I am just going to launch into it, skip the descriptions Wellington’s wind, of my fragile emotional state, and just get the words out there. I don’t particularly enjoy writing in this manner but let us see how it goes.

Some weeks ago, after my second round of ICE, I had a CT scan to check my lymphoma status. The results were good; I had achieved a partial response to ICE chemotherapy, only the nodules in my lung remained. BCSH guidelines (yes, I have read them) state that a partial response is required to proceed to the next treatment stage. Mike and I shared another public peck at the good news and preparations for the stem cell transplant began. It was scheduled for December 23^rd, a perfect Christmas present. I had only one round of ICE remaining.

I shall deviate here slightly to nerdily describe the stem cell transplant progress. It is better defined as a ‘blood stem cell transplant’, you know, to remove any controversy. The idea is the bone marrow is stimulated via high dose G-CSF injections administered over a ten day period. This means two injections in the gut each morning. By about day seven the bone marrow is producing so many cells that they do not have time to differentiate within the marrow, so they just remain as stem cells circulating in the blood. These ‘mobilised’ cells are then ‘harvested’ by apheresis: blood leaves the body from one tube, undergoes centrifugation, the stem cells are collected, and the blood is returned back to the body through another tube; a continuous process with only a few hundred milliliters of blood leaving the body at one time. It is similar to dialysis. The collected (haematopoietic) stem cells are then frozen. This is a preservation process as the high-dose chemotherapy (BEAM in my case) is so toxic that it kills the bone marrow and damages stem cells. It also melts away any residual tumours. After the BEAM, the frozen cells are reinfused into my body, take about seven to ten days to work their way into the bone marrow and Hey Presto! I am cured. If all goes according to plan. The stem cell mobilisation was to start the day after my final ICE infusion.

I check into the haem ward cranky, as usual, for my final round of ICE. As I have previously mentioned it is a three night incarceration that I am never eager to attend. And the final round was crap. I was irritable day one, threw up for three consecutive hours day two, refused all hospital culinary delicacies from there out, and spent day three trying to focus on objects situated directly in front of me, failing, and falling asleep. At two a.m. in the morning of my final scheduled night as an inpatient, my temperature spiked above the dreaded 38°C. I am usually pretty clued up when it comes to my fevers, I know when they are coming on, I know how long it will take for my temperature to reach 38°C, and I know when to take paracetamol to calm the bastards down. This particular fever, however, took me by surprise. I knew I would not be discharged that day and I was pretty bloody angry about it. The doctors termed me ‘unwell’, infection was presumed, and broad spectrum IV antibiotics began with a disclaimer: we may not be able to begin your stem cell mobilisation tomorrow if you have an infection.

Well, isn’t that a fun thought to try and get your head around, when you are stuck in a room with a stranger, a stranger who has many different snores (so many I could not count each noise), trying to comprehend that your schedule, the schedule that had taken three months to prepare, could be thrown out the window because of one stupid temperature spike. I had prepared for many scenarios where the transplant would not go ahead, but I had not prepared for failure before the process had even begun. I was angry, down to the depths of my stomach, and there was nothing at all I could do about it. Relief came the following day, a Monday, when the regular haem team were on and assured me that the stem cell mobilisation would go ahead. It was the most reassuring gut injection I have ever received.

Despite the continuous IV antibiotics, my body feverishly pottered along. A couple of tender lymph nodes bulged from my neck, a couple more sprung up in my groin. The fevers became the predictable events I remembered; a rigor one could set a watch to. These were starting to resemble disease fevers rather than infection. This thought comforted me; if I don’t have an infection then they will let me out of hospital and I can at least feel shitty in an environment of my own making. I decided these words of wisdom ought to be conveyed to the haem team.  Unfortunately, they did not share my enthusiasm. I should not be displaying symptoms of disease. If I was, then the transplant would not happen and plans B and C would not only need to be devised, but also actuated.

And it was about then that I completely lost my shit. I had, externally anyway, remained calm when around the clinicians, hid my fears, my worries, and had just concentrated on the information they divulged. I even offered them a sly joke or two. But, at that moment, I lost it. My tears became as uncontrollable as my fevers. It turns out that further relapse was also omitted from my list of possible failures. There was still the slight, very slight, possibility that an infection was causing my symptoms and, as I was losing my shit in more ways than one, further tests were performed. I vaguely recall cheering "I have c.diff, I have c.diff" from my hospital bed and fist-bumping my nurse as he wheeled me into isolation. I am not sure how much of that memory is actually fever. Probably most of it. The general sentiment of the moment remains: I was happy and the clinical team were ‘cautiously optimistic’. The persisting IV antibiotics had wiped out my microflora, my good little bacteria, leaving c.diff to run amok. But it did not take long before I realised that the infection was a false hope. A helpful little night nurse even told me that one doesn’t get fevers with a c.diff infection. I do not know if she understood the implications of her statement.

I remained in isolation to protect my fellow patients, received the daily G-CSF jabs and plunged back into lachrymosity, the tears only amplified by my frequent fevers and general pessimism. Harvest day was looming. The haem team continued to bathe my cells in IV antibiotics. If I did indeed have some superbug with freakishly good hide-and-go-seek skills, they did not want it interfering. Interestingly, if my disease had relapsed it would probably not affect the harvest. Hodgkin’s cells rarely make it into the actual blood, so my stem cells should be mutation free. I know I have used the word ‘rarely’ there. I will emotionally deal with that possibility at a later date. A far later date. Anyway, the only ominous factor, aside from those aforementioned, was the absence of bone pain. I had been on double dose G-CSF for eight days without the slightest orthopaedic discomfort. There is a minimum cell count (CD34) required before the harvest will proceed. That magic number is 20. (They never told me the units, and I confess I never asked.) My count, on the day scheduled to be harvest day, was 2.5. The harvest is planned over a week, I still had four days remaining to reach 20, so really it was no big deal, but having been in hospital for eleven days, and with all the setbacks, and the frequent toilet breaks, I did not have the mental capacity to deal with a number as low as 2.5. So I do you know what I did? I am sure you do. Yup, I cried. I ignored all of Oasis’ advice, and cried my heart out.

The following day, however, I was roused by unrelenting skeletal agony. And I smiled, a sick masochistic smile, as I informed the clinical nurse of the substantial bone pain. She shared my excitement and rushed to tell the team. I imagine she burst into the office, hands in the air singing “She’s got bone pain!”, the remaining nurses and doctors of all ranks tossing their papers in an act of jubilant celebration.  Hmmm, perhaps that was just another fever. When she returned I was back to tears. I was no longer enjoying the pain. My count that day was 10, but they decided to hook me up to the harvester anyway with the hope of collecting the required volume of cells over two days rather than just one. They did not want to risk losing any of my circulating stem cells. The panic was unnecessary. The next day my stem cell count jumped up to a whopping 45 and I was hooked up for a further six hours. I will tell you about it sometime. But not now. This has gone on far too long already. The stem cell harvest is done, they have double the cells required, frozen in a protective pool of DMSO, safe for the next five years.

Harvest time

So that just leaves the fevers. In the final days of this horrendous episode I had a CT scan. The results were damning. The lung nodules had grown, further nodules had popped up in my spleen; pretty much all of my lymph nodes were enlarged. A biopsy wasn’t even required. In the three weeks since my last scan I had once again managed to relapse, relapse with a vengeance. This means, of course, that of my three doses of ICE, one worked, one was dubious, and the final was utterly useless. All it did was increase my reliance on donated red blood cells and platelets. In July I just wanted to make it to Christmas without a relapse, instead, I have relapsed twice. Yes, I am a bit bitter.

My discharge came suddenly. I could not be discharged to the cancer accommodation we had been staying at for the last four months as I was still symptomatic for c.diff and my fellow immunocompromised inmates could contract it from me. Mike called his brother and we made a rapid transfer to his place, and, ah, we kind of haven’t left. I am not going back to the cancer accommodation. It is existence, it is not living.

As the clinicians discussed plans B, C, D, F and probably Z, I was left to battle the fevers myself. I had a column of cuts running down my thumb from the paracetamol packaging. The fevers became more and more frequent and debilitating with each passing day. I was a broken mess, and all the King’s horses and all the King’s men were struggling, really struggling. They have since put me on Prednisone, hence the hyperactive nature of this post, which usually works for ten days. I’ve been on it sixteen now and it is starting to wear off, a few symptoms are sneaking through, but it has given me a fortnight of faux energy and actually feeling alive. Obviously, the transplant did not go ahead, but plan BCDFZ has been written, in pencil, and further treatment is imminent. We are just not sure quite when. There have been, and still are, a few complicating factors, but I will go over those in a later post. I don’t have the energy to discuss them right now. We will be in Wellington for a few more months yet. After a year of treatment, I find myself back at the start.