Game changer

Sometimes, when my health is on the improve, I play a little game. It is a dangerous game. I pretend that all will turn out fine. That at some point, soon even, life will return to normal. We will rent a wee flat in Nelson, The Wood perhaps. Our cat will move back in with us. She will cease her biting ways. The flat will be near the city and we will walk or cycle to work. Ah, to work.  A job. Right. Becoming a contributing member of society again. Tricky business.

Our possessions are scattered throughout New Zealand. Various friends and family members are storing boxes filled with our crap; in garages, closets, chests, under beds, piled high in spare bedrooms. My books, my books are also scattered. I try to infiltrate the bookshelves of loved ones but usually, they too, end up in boxes. The books, not the loved ones. Gosh, things can get morbid mighty fast when grammar is overlooked. I must confess I never expected to open any of those stored boxes. It was all part of the game.

I left you in March (shit, was it that long ago?!) closely monitoring my fevers, platelets, haemoglobin and bilirubin. I started two blog entries but circumstances kept changing. My updates were obsolete before they were published. Like a newspaper. It is difficult to be witty and current. I’ve found opting for neither is the best approach. Anyway, there I was, March, obsessing over my bloods. I must apologise for I wasn’t exactly honest in my March post. Well it wasn’t complete dishonesty, it was more omission. Avoidance rather than evasion. Like Cameron. Allegedly. You see, the immunotherapy arrived at the eleventh hour, like a fairy-tale prince. I was a little too dependent on blood donations. Two bags a day, of both platelets and red cells. My bone marrow wasn’t working. It was packed full with Hodgkin’s cells. The marrow surrounding the Hodgkin’s cells becomes fibrotic and cannot produce any blood cells. My liver wasn’t working. Presumably, it too was packed with Hodgkin’s cells. I would like to thank all the blood donors out there. They kept me alive.

This new drug arrived and promptly terminated my liver failure. The bone marrow response was a little slower, but I have maintained a haemoglobin in the low 90s for at least four weeks now without any transfusions. Go team! Thrombopoietin (TPO) is the hormone that stimulates platelet production in the marrow. In a wicked feedback cycle, the liver produces the majority of TPO. The red blood cell equivalent, Erythropoietin (EPO), is produced by the kidneys. By having liver failure there was minimal production of TPO, which was fine at the time because my bone marrow was also failing and would have done fuck all with such stimulus, but once the two began working again it was interesting to watch my haemoglobin rise whilst my platelets lagged behind. I was reliant on platelet transfusions for about a week longer than red cells. In Wellington, protocol is to keep platelets above 20 for patients with fevers. You are not allowed to shave your legs until your count reaches 50. I am unsure if that is actually documented in the official SOP. These are the pesky issues I worry about now that the chemo is out of my system. Did I hear somebody say first world problem?

My consultant informed me he was rather impressed with my blood. I blushed. This is probably deemed showing off in an anaemic ward. But my cells had done me proud. I still take it personally when my haematological results amaze, fucked up, I know, I know. It is the nerd within me. Or the nerd that is me. Anyway, so impressed was my consultant that during an impromptu meeting he released us from Wellington. Mike and I were free to live in Nelson on a full time basis with me returning to Wellington once a fortnight for treatment. We were shocked. This was completely unexpected. More unexpected than the marrow failure. We had to take a moment or two to recover. Despite the southerly cutting through my now functioning marrow, Wellington had grown on me. Stockholm Syndrome perhaps?

  

Mitre Peak, Milford Sound

This occurred a few weeks back. Since then I have been a little distracted trying to cram the rest of my life into four weeks. Christchurch, Queenstown, Fiordland, Omakau – ok so that one doesn’t feature in Lonely Planet. I am still cramming. How does a Mid-May overnight tramp in Nelson Lakes sound? Great, let’s do it. Now. Let’s do it now. Tramping has a different definition in New Zealand, although both involve a sleeping bag and no showers. My fevers persist, however monitoring these has been complicated by menopausal flushes. Fun fact: your temperature does not rise during a hot flush. It does with a fever. Initially I was pleased; menopause is a process all women go through, I felt it was my duty as a woman to experience it. After a month of continual hot flushes I declared it unfair ­– the first time throughout my journey I have said such a thing. Still, my glowing red face does provide a conversation piece with women over forty-five.

It turns out merging back into reality is time consuming, exhausting, and rather difficult. My procrastinations are interrupted by self-imposed distractions. Looking for a flat in The Wood is not as romantic as it sounds. My cat still bites. And a job? Ha! I can’t even commit to a haircut. And I need to. I really need to.

     

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?

My Brentuximab Fling

Clinicians and patients differ in their opinions about Prednisone. Clinicians seem to view the drug as a last resort, a temporary fix to be used sparingly for a short duration. As short as possible. We patients, however, love the stuff. It makes us feel wonderful. We can frolic in the sunshine, climb hills – slowly, but we make it – talk incessantly and feed constantly. In short, we feel well and like to stay on the steroid for as long as we can.  Oh there are some negatives; degrading quad muscles, degrading biceps, minor sleep impairment, facial puffiness, and some funky liver enzyme readings, but did I mention I sunshine frolicking? I was allowed Prednisone over the Christmas period. It was wonderful. It even offers me temporary reprieve from my rigors. Simply wonderful.

My ICE relapse complicated further treatment plans. Actually, it put a complete halt to them. Other options were required. The easiest was to use a different strain of chemo, but I am kind of running out of those. I have been exposed to most chemo classes, therefore the chance that a different agent will actually help me is limited. The resistant cells will merely grow stronger. I think there is one chemotherapy class that I have not yet tried. Another option was a targeted chemotherapy drug called Brentuximab. Hodgkin’s cells express a cell marker called CD30. It sticks out on the cell like a little flag declaring its individuality from those surrounding it. Brentuximab contains an antibody complex that targets CD30. The drug floats around the body searching for any CD30 flags. When it finds one, the antibody attaches to the cell and pumps it full of a chemotherapy agent. It is a lock and key approach. Imagine you are standing in a hotel corridor with a key but no room number. The only way to find out which room you are in is to try all the locks. Oh look, the key fits in this lock, this must be your room. But wait, someone else is already in the room. Well you’d better punch them in the face, Bruce Lee style. That is pretty much how Brentuximab works.

This drug had been around for a few years now, but it is usually only used for patients who relapse after their autologous stem cell transplant. There have been a couple of trials using it on patients pre-transplant, but they are not sufficient for the New Zealand drug agency, Pharmac, to automatically fund Brentuximab. Strangely enough, the New York blogger I mentioned, probably a year ago, was a participant one of those trials. It is a small Hodgkin’s world. Brentuximab was the drug my consultant wanted to use so he asked Pharmac to fund it for me. This required a bit of research on his behalf, a named application followed by a cost benefit analysis. I don’t know if they attached a personal reference and a photo as well; it did sound complicated. For my non-New Zealand readers Pharmac is deemed a bit of baddie. They only make negative headlines over here. Nobody wishes to be denied treatment for financial reasons, and media outlets, well, they eat those stories up. In a Christmas pantomime, Pharmac would be booed on entry.

The application was two weeks into another limbo period. One would think that with all my prior practice in waiting, my patience would now be pretty good. It is not. Well it is to a certain extent. Tell me there will be no news for a week and I will be fine for that week. But once those seven days elapse I start to become restless, distracted, cantankerous. Initially the wait for Pharmac was to be three days, then seven, then ten. I did not expect the funding to come through. I expected them to reply “Try more chemo. If that fails get back to us.” In an odd ironic twist, if they didn’t respond before a certain date, I was going to require the chemo anyway. One can’t help but smile at that. But Pharmac responded in time. They responded with a yes, which leaves me a little confused – do pantomime villains actually have layers? Our families were ecstatic with the news, but I must confess I felt indifferent.

The ten day wait for Pharmac was nothing compared to the wait for the actual drug. There are no stocks in New Zealand. An Australian company said they could deliver it to us in a week, with it arriving Christmas Eve. I was to attend the day ward the Tuesday after Christmas ready for my Brentuximab. But it hadn’t arrived. It was “in transit”. Transit from where, no one knew. They weren’t even sure if it was in transit to Wellington, or to the Australian company. Just that it was in transit. For the next two weeks we spent our days waiting for a phone call announcing its arrival. Eventually we got a call “Ah it has left Australia”. What? It was only now in Australia? A call a few days later “It is in Auckland. We will try to get an overnight courier, so come in tomorrow afternoon.” It turns out it was held up in customs in Auckland as it cost (far) more than the GST import threshold, and customs would like some tax please. I am not sure how that was resolved, but I know it infuriated my clinical team. It took three weeks for the Brentuximab to get to Wellington. Some say it was sent by kayak.

During this waiting period Mike and I binged on the second season of Fargo. It was a bit fucked up. Not the scene where they bury a guy alive in molten asphalt, I can handle that, but the thirty year old wife who has lymphoma and is on experimental drugs. And we know she dies. We know because Molly’s mother was dead in the first season, and she died of cancer. Ah but we’ll just let the senseless killing around her, around us, distract us from battles that need to be fought, fought without killing anyone. That and Kirsten Dunst’s performance. Which was awesome. 

Anyway, after my Penelope inspired patience (and weeping), and some excitable distractions courtesy of a surprise visit by my Scottish sister, I was ready for this Brentuximab shit. I was all psyched up, prepared for all the worst side effects, and it was totally anti-climactic. A little fuss and excitement was had in the day ward, but physically nothing. Nausea: nil. Cell counts: completely normal. Hair loss? Nope, more like super hair growth, which is good because I was looking like a humanoid cylon prototype, with tubes under my skin and a shiny skull. So there were no side effects. Maybe I could imagine some fatigue. But that could also be the steroid withdrawal. And the infusion only lasted thirty minutes. I had it in the day ward. No overnight stays.

Three weeks of Brentuximab, a week off, then three further weeks. That was the plan. After the first three weeks I was finally fever free. My heart rate was a charming 79 and I was starting to feel much better. Granted I still had prominent lymph nodes in my neck and groin, I was still experiencing night sweats, and my spleen was causing me grief, but overall I felt nearly well. My consultant allowed us an anniversary trip, which we’d kind of already partially booked. The trip took us to the northern Coromandel, completely off the grid. The plans were for walking, swimming (paddling for Liv), nature and books. Maybe even some beer. And no contact with the outer world. We were to forget our current worries.

My rigors started on the second day. The lymph nodes burst through later that evening.  You can ignore, or at least weakly justify, the changes for a couple of days, but once the rigors become regular and the fevers creep higher, well we knew, we both knew, what was going on. It was fortunate that we were completely alone, in our hired bach, sitting on the deck listening to the kiwi and the morepork chat to each other – the morepork always has the final say – as it gave us a peaceful setting to contemplate my fairly obvious relapse. I set myself to weeping. I was glad that we were off the grid. It is difficult to correspond with people when everything is getting worse and our future is becoming uncertain and bleak. Since November we have had three weeks, those first three weeks of Brentuximab, where we have definitively known what was going on. So if you’re wondering how I am, be rest assured that I am shit. Except, of course, for that one Thursday when I saw my consultant and I was well. There is not much point in asking how I am. It is strenuous and exhausting responding to multiple messages.

Ok, so it wasn't all doom and gloom

The day before we left my temperature shot up to 40°C. We made an obligatory call, which went unanswered, to my clinical nurse who is holidaying in the North of England. I hear it is charming there at this time of year. Really the phone call was so we could claim that we hadn’t broken all the rules. Only the remoteness, the untreated drinking water, the unpasteurised cheese… They were not overly impressed at the day ward when I admitted the 40°C fevers.

I have started Prednisone again. I pretty much begged them for it. It doesn’t seem as effective this time. There has been no frolicking, and although the rigors have gone, the fevers still remain. In my gut I had believed Brentuximab would work. But it didn't. It is another limbo period treatment wise. We don’t know what the next step is. No one does. Emails are being fired between here and Australia, opinions are being sought, but a decision is yet to be made. I now know that these limbo periods need to be covered by steroids because Liv’s body and mind and husband cannot deal with long frequent rigors. They are debilitating. And, during this current period of high grade fevers, Wellington decides to throw out a fortnight of scorching weather. But at least now I am now grateful for the wind.