I was diagnosed with stage IV leiomyosarcoma in February 2014, in the week after giving birth to my second daughter.
Sarcomas are cancers of soft tissue and bone. Leiomyo refers to smooth muscle, in my case, the muscles of the wall of a vein or artery. My primary tumour is in my retroperitoneal cavity (the back, right hand side of my pelvis). It was measured at about 11cm on my last CT scan, but tumours are such irregular shapes that this might be best thought of as notional rather than literal. I have multiple secondaries (metastases) in both lobes of my liver, the largest of these was 13cm a few months ago. I think there's three or four tumours there, I can't quite remember. And finally, I have 'numerous' tumours in my lungs - numerous meaning that no one has counted them all. The largest is 5cm.
Cancer that has spread this far would previously have been considered a life-limiting or terminal diagnosis, and there is certainly no established treatment pathway out of this place. But my oncologist says that she no longer draws a line between treatable and non-treatable disease. New treatments are developed continually, and in any case, it is never possible to determine an individual's trajectory from statistical information. "You are already rare", she says to me.
The upside of leiomyosarcoma being rare, my oncologist says, is that rare cancers are the simplest ones: they typically develop as a result of only one or two random gene mutations. There are research labs that target rare cancers because they are more likely to get a 'hit' with a drug that targets a single gene mutation. I haven't identified any labs that are actively working on leimyosarcoma, but if anyone wants to look them up for me, I'd be very appreciative. The downside of having a rare cancer is that research funders aim (quite rationally) to benefit the largest number of people possible. So most research labs focus on common and high profile cancers, even though these develop through multiple metabolic pathways and are affected by all kinds of complex factors (toxins, hormones, lifestyle and so on). It is remarkable that there are now refined, targeted and effective treatments for a number of common and high profile cancers. And something relevant to a rare and low-profile cancer could theoretically happen at any time.
Overall treatment strategy
I gathered opinions about my main oncologist from a pretty good selection of oncologists and researchers and once I'd done that, I decided just to trust her. And I think she's great. She is sharp and perceptive, compassionate, extremely experienced in my kind of cancer and involved in the development of new treatments. I'm being treated now through the Peter MacCallum Cancer Centre, which has 400 full time researchers and the most integrated research and treatment capacity in the country. I've read everything I can about alternative and complementary treatments and I'm pretty convinced that the people who most know what they are talking about are specialised oncologists.
So I am essentially doing what my oncologist tells me to. I'm open to emergent and alternative therapies on the condition that they can be assessed on the same sorts criteria used to evaluate chemo or radiotherapy. What is the evidence that this works and is relevant to my particular situation? Does it interfere with other treatment? Can I weigh the likelihood of it being successful against the degree to which it will distract me from my family and my process of coming to terms with my situation? And so far, the only additional 'treatments' that seem useful to me are the most standard, general health aspirations: vegetables; unprocessed food; exercise; a peaceful outlook. I think the evidence for each of these measures is really significant (not cancer-curing strong, but worthwhile-in-everyday-life strong). In any case, I don't want to try any alternative therapy in order "to do something", "to have a sense of control" or "because I have nothing to lose". To me, these are emotional matters that probably each need to be faced directly, analytically, and with plenty of support. They're not helped by taking vitamins or supplements, and in too many cases, those supplements interact unpredictably - or badly - with conventional treatment, especially chemotherapy.
My oncologist aims "to keep patients alive, without trashing their bone marrow, or their liver or kidney function, in the hope that new treatment options come along." Initially I'd struggled with the lack of structure of just treating symptoms as they arise. I'd wished for a plan and a path and someone to manage it. But as I gradually become more familiar with my diagnosis and the available treatment options, I'm thinking that this is a rational response to uncertainty and complexity. Just a bit of an uncomfortable one.
Causes and treatments, generally
I give a lot of credit to the role of general health. I believe that there's great value in everyday actions like: eating plenty of vegetables and unprocessed food; getting enough exercise and sleep; maintaining a low sun exposure; aiming for a calm positive outlook and so on. But trying to stretch the idea of any lifestyle measure to account for an instance of an extremely rare cancer is unhelpful. I might be a bit sensitive, but people have sometimes seemed to suggest that my illness is my fault because, for example, I was stressed, I had babies, I ate meat or didn't eat meat (I was vegetarian for twenty years), and so on. I tell myself that people might be seeking to reduce their own anxiety, or to shore up their sense of reason and order in the world. Their hypotheses might be linked to our cultural narrative (our fantasy, frankly) that we can truly control our own health and mortality. But people tell me these things and imply that I bear the blame for having cancer, then it hurts.
The only 'cause' that I really take seriously is my oncologist's explanation that rare cancers are chance events, typically involving only two or three gene mutations (as compared to cancers that are linked to lifestyle, genetic or hormonal factors). I try to focus on the fact that gene mutation is a necessary quirk of biology, a key mechanism that enables the abundance, complexity and gorgeousness of evolution itself. Although, of course, evolution is pretty slow, and sometimes nasty, not least because most random gene mutations turn out to be no help at all.
I've reached my 'comfort in randomness' position through lots of hard thinking, and of course I wobble at times. I've had my own attempts at making a narrative out of all this by looking for causes. There's not much evidence anywhere, but leiomyosarcomas might be associated with exposure to PVC (flexible plastic) and several agricultural chemicals. These are common, known carcinogens, that we are all exposed to at low levels in everyday life. For what it's worth, I'd encourage everyone to reduce their exposure to known carcinogens where possible, perhaps by using glass or stainless steel water bottles rather than plastic, or trying to eat food that is relatively low in pesticide residue. But again, these measures can't guarantee anything and shouldn't be expected to. For my own part, I just try to keep living with uncertainty, and to keep healthy even when that seems bitterly paradoxical.
So it follows that - after months of thinking about this - I don't really accept that any lifestyle measures could be a meaningful treatment (in and of themselves) for Stage IV cancer. I do try to keep healthy because it keeps me steadier, but I've no expectation of lifestyle measures or alternative 'treatments' changing things in a very significant way. This is very difficult to write, because people suggest alternative 'treatments' to me very frequently, with very good intentions, and I don't want to hurt their feelings or make them feel like I am callously ignoring their advice, or that I am wilfully contributing to my own demise. I'm not up for wild experimentation because I've seen too much data on something that works well for one kind of cancer being problematic in another instance. But I'm all for empiricism and evidence and flexible thinking, and I will follow treatment protocols - including extremely tough measures - if there's good evidence to back them up. I'll talk about this more below.
February - July 2014.
My first line of treatment was chemotherapy and I had an eighteen week course of doxorubicin. Initially the plan had been to give me combined doxorubicin and ifosfomide, but we weren't able to proceed because of my reduced liver function. There had been a theory that these drugs in combination were the most powerful treatment available for leiomyosarcoma, but a study showed that longer term survival rates didn't vary whether the drugs were combined or given sequentially. And by having them separately, I can have larger doses of each.
Doxorubicin is an almost-glowing, profoundly unnatural, orange colour and it's side effects are consistent with it's appearance. It really does feel a bit deathly when it comes in through the port. My oncologist made a pretty accurate guess of the maximum dose I could withstand and gave me the best drugs that they have for managing the side effects. I was hospitalised on three of the six cycles because of various complications, and on the last cycle I had severe neutropenia and had to be isolated for a week. It was a difficult time but I was convinced that we were giving the drug it's best chance of working. I can't have doxorubicin again because the theoretical risk of heart failure is too great.
In the end, the doxorubicin reduced my tumours by an average of 20% (a somewhat successful result), and that meant that I was virtually symptom-free during August and September. By October I had significant symptoms, but I delayed treatment by a few days (with doctor's consent) to get married :-)
I had a three week course of radiotherapy to my liver and pelvis. I changed hospital from the Royal Women's to the Peter MacCallum Cancer Centre to have radiotherapy, as per my oncologist's recommendation (she is a consultant at both). The radiotherapy was pretty precise. Up until recently, they did not irradiate livers because it was considered too dangerous, but the Peter Mac has specialises in planning highly 'conformal' radiotherapy (i.e. in the same shape as the tumour) and therefore can give more powerful doses to more places than previously possible. Overall, the radiotherapy caused far less trouble than chemotherapy (which is not to say that it didn't have side effects). I'm booked in to have a CT scan in December to see how effective it was.
The point about giving radiotherapy to the liver is particularly poignant for me, because one of the first 'cancer' articles I read was Atul Gawnede's piece on 'Letting Go': http://www.newyorker.com/magazine/2010/08/02/letting-go-2
Probable future treatments
The next treatment I'm likely to have is an eighteen week course of ifosfomide, also a chemotherapy drug.
A round up of treatment options
Surgery. I've been told by more people than I care to remember that my tumours are definitively inoperable. Some are individually inoperable, and in any case, there are simply too many, spread too far, affecting too many organs, to too great an extent. The doctors say that if they attempt surgery in one place, tumours elsewhere will overwhelm me during the recovery period.
Radiotherapy. Peter Mac is a pretty advanced centre for developing and delivering radiotherapy. My radiation oncologist (not my main oncologist) is involved in developing new, intensive radiotherapy techniques (e.g. SABR), but these are used most commonly in conjunction with surgery. Nonetheless, he knows I'm interested in having more radiotherapy and is continuing to monitor my scans.
Chemotherapy. Chemotherapy is obviously a very blunt response to a fine-grained problem, but it is likely to be my predominant treatment option because it is the only thing available that is systemic. The chemo drug protocols have barely changed in forty years. This was quite distressing to me until I realised that all efforts are going into more tailored treatments; they just haven't got anything for me yet. So the reality is that I have to try to have enough chemo to keep my tumours in check, while minimising the inevitable damage, and to wait.
The strategy with chemo is that my oncologist estimates which drug and dosage might be the most effective with the least side effects for my specific situation. The chemo not only kills fast dividing cells, but also triggers an immune response, and this is probably one of the reasons it's efficacy varies so widely - for a few people, common chemo drugs can be ludicrously effective (not for me, so far). When I've had as much of one drug as possible or worthwhile, we move onto the next likely candidate, a little less likely to be useful than the last drug. It is inevitably a narrowing field.
Genetic testing. We're seeing if the lab at the Peter Mac can get meaningful information about genetic mutations from the biopsy that I had done for diagnostic purposes. This isn't routine practice, because it's extremely unlikely that there'll actually be any drug that can address the specific mutation(s) that caused my tumours. But there is a chance that we'll find out some useful information that we might be able to use in discussions with cancer researchers. A very long shot, but interesting.
Immunotherapy. Much cancer research now focuses on the immune system, but as with other treatments, the researchers have to work out exactly what the issue is before they can find a treatment that might be relevant. Many of the treatments are very new and available through clinical trials only, I've met several patients and researchers involved in this in Melbourne but there aren't any trials that I'm eligible for at the moment. Let me know if you know of any.
The immune system is clearly relevant to every type of cancer treatment. It is rare, but very well documented, that radiotherapy can trigger an immune system response that decreases tumours outside of the area that was being irradiated. Chemotherapy also partly works by triggering an immune response. But the immune system is extremely complex, and immunotherapy researchers have warned me off doing anything to 'support' its function (for example, taking antioxidant supplements can undermine the damage-and-response cycle of chemo- and radiotherapy). It seems that everyone is interested in the immune system, but there's few available treatments as yet.