480DTG: Planning my death

This post’s title is not intended to be provocative but instead is a realistic exercise I must do. Most of us (including me, until about two months ago) know we’ll die but the details are usually fuzzy and somewhere distant. But now with a diagnosis of a fatal disease I can start getting down to realistic approach to my own death. And don’t kid yourself – there is a lot to think about and so you should approach this issue with the same degree of research and planning as picking a college or a job or a mate when you’re fortunate enough to be able to plan your death (and, yes, it is fortunate since with less uncertainty you can chose alternatives you prefer, the last choices you’ll have).

And this topic took on new meaning to me just this week. My sister-in-law died on Thursday, a shocking event. Not shocking because she died (given she had a diagnostic from my oncologist of stage 4 lung cancer that had metastasized to the brain – i.e. almost certainly fatal), but shocking that it was so quick, less than two months after her diagnosis. She accepted her death but thought she had 1.5 years whereas my quick research indicated 3-9 months was more likely, but two months! – that was unexpected, which, of course, makes you think about the interpretation of survival statistics associated with cancer. While I am immensely saddened by her passing and should be (and normally would be) just focused on her, the coincidence that I too have a fatal diagnosis from the same oncologist makes it impossible for me not to think of my own fate, as callous as that may sound as a reaction to someone else’s death.

I have a fatal disease, myelodysplastic syndrome (MDS) that will almost certainly be the cause of my death (of course, I could still get killed in car wreck or random violence or some unknown disease, but the MDS is about 99% certain as the cause). I’ve learned a lot about MDS: it was poorly defined and identified just about a decade ago, but in the last 10-20 years medical and biological understanding of MDS has significantly increased. It is both more common than originally thought and, interesting, one of the more expensive ways to die (good thing Sarah Palin’s scare tactics about death panels is false since I’d be the candidate to suspend expensive and essentially useless treatments to save money for others). So while this disease is not as well understood as many fatal disease it is possible to make some sensible predictions about it.

Actually MDS is not a disease (although more than diabetes is as per my previous rants of labeling a blood measurement as a disease). MDS is an umbrella term for a variety of very similar diseases that are difficult to clearly categorize. This group of diseases share the common characteristic that your blood cells don’t mature properly and ultimately this leaves you with one or more cytopenias which is what will actually kill you. (btw: It’s amazing how many blood cells we create each day of our lives and how short many of them live, hematopoiesis (development of blood cells) is fascinating)

When I had my previous meeting with my oncologist I only knew I exhibited neutropenia (shortage of one of many types of white blood cells known as neutrophils and -penia because I’m low more than 95% of the normal range). So I studied what might cause this blood test result so I could ask some intelligent questions. My oncologist had gotten my attention when she said she needed to do the bone marrow biopsy explaining “I’m not saying you have leukemia but it’s a possibility”. That wakes you up. So the biopsy went smoothly and then I got my MDS diagnosis. But I hadn’t studied MDS in enough detail (it was one of many possible causes) nor understood what can be learned from the biopsy (for instance, the % blasts in my marrow is a critical diagnostic criteria and I didn’t know that and so I didn’t even ask what that number was, and I actually think my oncologist should have told me, but I think the default doctor assumption is that patients are dumbshits and wouldn’t understand the detailed measurements, like %blast or my exact cytogenetic status (like am I possibly -5q) or what types and how many lineages of dysplasias I have. Now I know so in my next visit in 15 days I’ll be able to get and understand a lot more information, plus teach my oncologist to give me hardcore data since I can understand it even if most patients can’t.

Now while I readily admit I have no medical training as well as not being so stupid as to not put real medical expertise (and scientific results) topmost in my thinking, I am a fairly quick study and smart enough to understand this stuff and I had a decent molecular biology background (despite no degrees or work experience in it, amusingly studying is my hobby and I’ve had 25 years to learn a lot about the mechanics of DNA, epigenetics, mitosis and apoptosis, all of which are at the root of my disease). What I have learned (which careful reading of readily available sources can provide) is that MDS can be sub-categorized (several systems, but the WHO scoring system seems to be the best). And the sub-categories then tie into your prognosis.

Now frankly the WPSS, as an “algorithm” isn’t that hard to do. While some subjective interpretation may be applied by oncologists I can apply the clinical data myself. But, since I didn’t know to ask I’m missing two critical bits of information from my BM biopsy: a) %blasts (more, esp. past a certain threshold) is real bad, and, b) how many lineages show dysplasias (there are basically three “lineages” (families, if you will) within the myeloid “tribe” of blood cell precursors). I do have good data on my peripheral blood cytopenias and (with some experimental/statistical uncertainty) my existing history of progression.

Now from the more vague info my oncologist gave me (again vague because she didn’t tell me, hopefully!, because she figured I wouldn’t understand more detail, not (worse!) that she doesn’t know), She said I had no DNA damage. Presumably that is the summary of the cytogenetic evaluation that was done. But as I’ve dug into this part of the MDS clinical data the cytogenetic study is difficult to do accurately, potentially expensive (various alternative lab techniques, which was done? I don’t know but I’ll bet not the expensive ones since that’s pretty standard diagnosis approach). So can I really be sure there is no chromosomal damage? I’d say I can only be about 67% confident in that result.

Now in terms of %blasts I think I can pin this down. #1, if I were over 20% (which would imply the worst MDS or even already AML) I doubt my oncologist would have just said “watchful waiting” and come back in three months, since I, like my sister-in-law, might have been dead in three months, and, #2, I must be more than 5% or presumably the oncologist following the standard algorithm would not have declared my diagnosis as MDS.

Now while I do have considerable confidence in my oncologist (after all she went to the rather well-known university up the river from mine, i.e. Harvard (and me, MIT) if you can’t guess) I also know that the thought process of doctors is way different from either research scientists or me (rather good big picture systems thinker). Or, importantly as has been another of my post topics, Nate Silver. Nate makes a very big point in his book that nothing can be stated with precision so, in essence, error bounds and/or probabilities should always be supplied with “predictions”. Now I know the Nate Silver and/or systems type thinking does not characterize my oncologist so it doesn’t surprise me she doesn’t present information in that way.

And if you’ve followed all my incessant articles about my weight program and all the experimental error I have attempted to determine and then describe you’ll know that I’m fully into the idea of any real-world measurement having error. So, for instance, when one month apart I get a hemoglobin reading of 13.8 (not abnormal) and 12.8 (abnormal) is this statistically meaningful? What is the error bound of the automated full blood count machine? And different machines were used for those two tests, so what is the machine-to-machine variability? An 8% drop in one month (if I just take the figures literally) extrapolates to I will be dead (or at least need some treatments) in just a couple more months, so the uncertainty in those numbers is not just an academic exercise for me. Now on 30Dec I’ll get new numbers and at least three points is better than two, but again how much can be predicted from such a tiny amount of data, plus is there any scientific evidence to indicate the drop in blood cell counts is even a linear function, and so forth. IOW, I have lousy data, so prediction is going to be almost meaningless.

But with these various caveats I’m going to wade in and play amateur doctor and do my own prediction: I think I have RCMD (Refractory cytopenia with multilineage dysplasia) sub-type (see here for a concise explanation of the types).

Refractory cytopenia with multilineage dysplasia (RCMD) is one of the more common myelodysplastic syndromes (MDSs). MDSs are clonal disorders of myeloid stem cells. These syndromes are characterized by ineffective hematopoiesis manifested in morphologic dysplasia of hematopoietic precursors, 1 or more peripheral blood cytopenias, and a propensity to progress to acute myeloid leukemia (AML).

Since my blood reports clearly say “pancytopenia” (IOW, all of my myeloid cell types are abnormally low: erythrocytes, neutrophils and megakaryocytes) it’s a reasonable inference that I have dysplasia in more than one lineage, hence the MD instead of UD. And I probably don’t have either type of EB (excess blasts) or else, assuming my oncologist is competent, I’d already be in some treatments.

In 15 days I’ll either know if I’m right (because now I can ask the right questions and presumably my oncologist has the answers; if not, I want something more definitive and my confidence in my oncologist will drop a lot). And what my prediction means is that I am definitely not in the low risk or probably even the int-1 category. IOW, I am now in the 2 year median survival group (again, probably not high risk or I’d be getting treatment, plus I’d certainly have some symptoms).

So my original guess (driving the countdown in my title line) of 1.5 years probably isn’t too far off.

Now we start getting into that planning part I started with. What is a realistic guess at my survival?

Now let’s start with the outcomes in MDS, which now I understand a lot better. Basically there are three:

  1. ‘cure’. With cancer ‘cure’ must always be discounted a bit as really there aren’t cures now, just remission. Basically cure for MDS means the MDS will kill you later than something else will, not that MDS is no longer a possible cause of your death. And the only ‘cure’ (with considerable risk (like 10% immediate mortality) and not great efficacy) is a stem cell transplant. While there are many variations of this procedure for MDS it will have to be allogeneic (new cells from a stranger) and I’m ineligible (by the standards of treatment). Now probably some rich and powerful dude could get an exception made but I: a) can’t do that, b) won’t do that. So, IOW, no cure!
  2. progression to AML (acute myeloid leukemia). This pretty much lights-out (especially as I think (now while calm and thinking rather than afraid in a hospital) I won’t do extreme measure treatments. That puts my survival (and agony of dying) in a similar category to my sister-in-law.
  3. watchful waiting and supportive care: Basically this is hope-it-progresses-slowly, but it will progress and when low blood cell counts start having impact use some treatments to try to boost my cells (like drugs jocks get in trouble using or just transfusions or massive doses of antibiotics). But it will just be a matter of time and also then how much the disease affects both my quality of life or even more basically whether I can even function at all.

With RCMD I probably won’t progress to AML since I’ll probably die first so that really puts me in the third bracket above.

So I have two timeframes: 1) how long do I live, and, 2) how long will I be well enough to reasonably function (MDS is clearly going to be a wasting disease where gradually my life to transition over to being incessantly treated, possibly with trips to ER, to avoid dying, but it will be a losing battle. That part of “survival” I will dread (and I may try to avoid, i.e. exercise other options than hopeless treatments) so it’s really the first part I have to plan for.

So at last I’m getting to my main topic, planning the reminder of my life.

Until I got this diagnosis my biggest fear was living too long, i.e. living longer than I can afford. Like most people I accumulated some assets for retirement and like most people I didn’t accumulate enough. On top of that, I think my generation will face inevitable cutbacks in social programs that will put greater financial strain on us (all the Repugs who want the cuts need to decide how they’re actually going to kill the baby boomers, I hope they actually have to get their hands dirty doing it so it’s not something they can hide from). Anyway my previous planning was how to cut our expenditures to match a reasonable guess at longevity and the financial climate off the next two decades.

Now I think that is definitely not going to be my issue. Two decades is way, way longer than my MDS horizon. With some sensible control over our spending and no government repayment of its debts to me (IOW, giving the benefits I spent 35 years paying for, not handouts) I would have lasted about 15 years, with a best case at about 25 (SS doesn’t get turned off to give to the rich) and worst case about 10 (Medicare gets turned off and I get expensive, like MDS, illness).

But now with RCMD I think I’ll beat the median 2 years. Most people with MDS are older and many have other morbidity. Due to my efforts on weight and exercise, plus generally a good health history, I’ll probably beat the median. And since I live in an area with good medical care (and can afford it) even some of the threats I may face probably won’t be fatal. If your MDS doesn’t turn into AML you’ll die from the consequences of the MDS itself. Surprisingly (to me) the most likely is cardiac failure – huh??, why? Well, it turns out anemia (shortage of erythrocytes and/or insufficient hemoglobin per cell) has more impact than merely making you tired (and probably a lump, not a fun prospect for me or for my wife). And this accounts for about 50% of the fatalities and so will probably get me. The next biggest is the neutropenia, my original symptom. But if I’m careful to avoid germy situations and get to ER rapidly with any infection and hope I didn’t get an antibiotic-resistant strain, I’ll probably survive a few of those crisis (those won’t be fun, either, sick for weeks in a hospital, possibly in isolation and hit with the side-effects of massive antibiotic doses). I can probably avoid serious cuts and/or get platelet transfusions so I’m not to worried about bleeding to death, but this does seriously raise the issue of whether I ever go camping again (where I usually sustain some skin penetrations).

Now on 30Dec I will get my third data point on peripheral blood counts and maybe I can begin my own extrapolation of my cytopenias, but in the absence of other information I’d guess I’ve got 12-24 months of relatively-symptom free life and then 12-36 months of increasingly miserable life with significant supportive therapy interventions. Now I’m really hoping I’ll respond to EPO (or other ESA) so I can keep my energy up. I’ll deal with the risk of infection or bleeding by caution, but if I’m just exhausted all the time life will quickly get miserable and maybe an infection won’t look so bad as an end.

And, btw, that has to be part of my planning. I don’t cherish every breath so much that it’s out of the realm of my planning to bring about my own end and I need to have that option (can’t do much in this state if I’m immobilized in a hospital, but at home I might have some choices). I know that when I face this as a reality (instead of abstraction now) it will be hard to do but there is some prospect of being pretty miserable in my last months and even turning off supportive therapy to hasten my end will be a fairly protracted way to go.

So it’s a reasonable projection (now, without more data) to assume somewhere around 3 years (max) with decent quality of life, IOW, more like 1000 days as my countdown.

With 1000 days of “good” life left and having to leave some financial reserves for unexpected costs and some funds for my heirs (but, if you’ve read all my posts you know that too much for them would be like sugar, they’d like it, but it will kill them too) I probably can spend about as much per day as my average spending has been per month which means I can certainly turn those decades of hard work and my stingy lifestyle into some extravagance now and I intend to do exactly that – but how much? and on what?

Now spending money just to blow it is stupid (even if that means after I’m gone my assets finance unhealthy habits of others) but it surely means I shouldn’t bypass something I really want right now.

But actually I’ve always lived a fairly modest, albeit comfortable, life and there just isn’t that much “stuff” I want. Yep, a Lambo might be fun now but my wife would kill me if I tried to get that, plus where do you drive a beautiful machine like that around here (or really anyway). So with no big ticket items in my future, even $100/day, much less $500, may be hard.

Now as an example of my dilemma I was just looking at a Nikon P520 camera, an expenditure approximately equal to one day of my possible discretionary expenditures (or two days if I assume I might have predicted 2X too pessimistic). It’s a really cool device. And I’m a sucker for buying it since the first big purchase in my life once I had a salary was a Nikon F (which I still have and on an inflation adjusted basis is about 8X as expensive as the P520) and the P520 is a fantastic piece of engineering with all sorts of features I used to dream about when I first got into image processing. So it’s a great toy, but what would I do with it? Take pictures of snow-covered fields and barren trees? Or at least what would I do with it until I can take a trip someplace cool. So things like that will have to wait, certainly until after 30Dec and I get to pin down my timeframe a little more precisely, and until I can really use them rather than just acquire them.

So I guess it’s just going to be any iTunes song I find even vaguely interesting and a couple more iPod Touches and a computer just to host iTunes.

But you get the drift of what I’m thinking about and most of the rest of the details I’ll keep in private posts.


About dmill96

old fat (but now getting trim and fit) guy, who used to create software in Silicon Valley (almost before it was called that), who used to go backpacking and bicycling and cross-country skiing and now geodashes, drives AWD in Wyoming, takes pictures, and writes long blog posts and does xizquvjyk.
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