Something I’ve been meaning to say…

Something I’ve been meaning to say…

spoons

I’m writing this now, a few sentences a day, because I don’t think it’ll be very long before I’m not able to type at all.  This is because I’ve gradually deteriorated to the point where doing pretty much anything other than lying quietly in bed makes me worse.  I can’t walk to the bathroom any more or stand for more than a few seconds, I can’t talk for more than a minute or two or comfortably lift a litre bottle of water.  I’m starting to mix up homophobes when I rite, witch eye never yous too due, and I can’t read or think properly any more.  And just as poverty breeds poverty, the more depleted you are the more vulnerable you are to further depletion, and once you reach a certain point a downward spiral becomes very likely.

This is not a self-fulfilling prophecy: it’s a well-informed probability, considering that I don’t know of any available treatment which I haven’t already tried and found ineffective several times over, and that the less you can do or say or read or think or feel, the greater the number of treatments that become unavailable to you.  It’s also not ‘defeatist’ to look reality in the face when the national health service won’t do it for me, especially since my morale, good or bad, has never had any effect upon that reality.  I probably shouldn’t feel I have to explain the severity of my condition as though I’m at fault for not recovering, but I do, and that’s an issue beyond the scope of this post. I would just like to explain my experience of the mechanics of illness that bring this sort of situation about. I’ll be using the term ME rather than CFS (though I don’t like either), because I’m taking CFS to be a more vaguely defined condition than ME, and things which may be true of one are not necessarily true of the other. Before I start, though, it’s only fair to tell you that I’ve found an excellent blog that says most of what I’m going to say here in much less verbose posts of a few hundred words plus graphs, so you might prefer to head to the Just ME blog instead. If you’re staying here then let’s begin.

First of all, the problem with fatigue in ME is not up front, in how much you can do in the first place. The problem is how much you can recover from. The defining characteristic of the illness is exertion intolerance, which may sound like a euphemism for laziness but essentially means you don’t recover properly from even trivial effort, you don’t respond in the normal biological way and pushing your limits makes them worse instead of better. This is different from other kinds of fatigue in which you have a lack of stamina that will however improve with training or you feel exhausted whether you’ve done anything or not or you feel a sluggish lack of motivation to do anything in the first place. It’s not the presence of fatigue that disables me, it’s the absence of energy.  I want to do stuff but, as Byron put it, the sword outwears the sheath.

So for instance, if you put me at the bottom of a flight of stairs, it’s not that I’d feel already drained, or faint, or feeble, or in a deep fug of lethargy that prevents me from summoning up the will to move. Nor would I be paralysed, or shaking, or dizzy, or in some other way unable to initiate normal movement. Some people with ME (henceforth pwME) do get these symptoms, but they’re not necessary for a diagnosis. The problem is that after I’ve got to the top of the stairs I will be spent, there will be an onslaught of disabling symptoms – ‘fatigue’ is a little effete for my taste – and my stamina during this acute period will become even smaller, exponentially so, so that tiny things which would normally be within my powers will now take further huge chunks out of me. And unlike the tiredness healthy people feel after exercise, it isn’t the short-term effect of a process that is overall making you stronger.

This catastrophic response to exertion is usually known as post-exertional ‘malaise’ (PEM), as though patients are feeling kind of languidly unwell like Baudelaire on a fainting couch, but that doesn’t really capture it. Different people experience it in different ways, and for some people there’s a lag or cushion of one or two days before it fully sets in. For me it’s much quicker.  I am a bit unusual in that PEM doesn’t make me feel sleepy or washed out or jetlagged or “brain foggy” or any of the ordinary types of tiredness that sap the average person’s will to do things, nor do I often feel the immobilising exhaustion that your body manufactures in response to a virus in order to get you to conserve energy, though other pwME do get all these things in spades. In fact I don’t think malaise is the word for me at all, because I don’t really feel ill in an immune-activated way most of the time. If I had to link my PEM to any normal kind of fatigue it’s probably like an extremely toxic exercise burn but branching out into various horrible new ways of feeling bad, mostly in my muscles but also in the meat of my brain and nervous system. It feels as though I’m being burnt up by a medium strength acid or poisoned, pulled, peeled, strangled, scrunched, stung, perished, fuzzed or suffocated, on a cellular level, but it doesn’t usually stop me wanting to fidget instead of resting. It can last days, weeks, or, for some people, months.

There is plenty of evidence by now about what might be causing exertion intolerance and unfortunately it’s not merely subjective, i.e. something which feels unpleasant but isn’t harming you. I’m trying to describe how ME works in practice, not write a polemic about its causes, but it helps now that scientists are corroborating my experience in their labs that I can talk about what’s happening to me without worrying that I’m merely anatomising a massive delusion. There are numerous documented biological dysfunctions, though their ultimate cause is still unclear, and you tend to find different dysfunctions in different patients, which suggests that we’re dealing with a category of disease rather than a single illness. But for whatever reason, the end result seems to be that ME sufferers don’t produce enough energy via aerobic respiration (1).  It builds up very slowly, like trickle charging a battery, so there may be enough at rest but with activity it runs out far sooner than it’s supposed to, which probably leads to excessive reliance on anaerobic respiration. Even in healthy people anaerobic respiration is inefficient, messy and painful, and the longer it goes on the more problems it creates for aerobic respiration to mop up afterwards, which healthy people can deal with but pwME cannot.

In addition, researchers have corroborated many pwME’s reports that trying to improve this problem the way you would with someone who was simply unfit or deconditioned, i.e. by challenging yourself with activity, does not work and makes it worse (3).  This is a problem because many UK doctors believe inactivity to be a cause of ME rather than an effect and use a definition of the illness so vague that it doesn’t mandate exertion intolerance or PEM. So we end up with treatments which make sense for people who for any number of different reasons don’t exercise, but which are useless or even harmful to people who can’t exercise. If you’re merely unfit and start exercising of course you’ll find it difficult at first but exertion will increase your capacity and it’ll get easier as you keep going – this is the ‘No Pain, No Gain’ paradigm. But in ME exertion that makes you feel worse really is making you worse. Your exercise capacity diminishes when you challenge it and future exertion becomes even more difficult and so on.  

Far from being a sloth’s charter or the refuge of an ‘exercise phobia’(4), it’s actually very frustrating when you can’t do anything you want to do and when effort is counterproductive to improvement, and most people kick against their limits and do more than is good for them. And like most pwME, and this is the second major point I wanted to make, I find that if I challenge my limits too severely this harm isn’t just confined to PEM which can be convalesced off and left behind. My baseline (the amount I can do without incurring PEM) diminishes, so my limits become even tighter and it’s even easier to fall foul of them and so on (5).

This is the principal cause of deterioration. I don’t know exactly why it happens, but in practice it works a lot like a bank account. You’re receiving a small daily energy income in a current account, which has a low ceiling so that you can never save up very much in it even if you don’t spend anything, and this is funded by the interest from a capital sum in a savings account. If you spend your current account balance you go into the red and incur punitive overdraft charges, even trivial further expenditures become disproportionately costly, and to pay off the debt you have take a chunk out of your savings capital. And of course when you erode your capital in this way it generates less interest for you to live upon so you can’t rebuild your savings and you’re more likely to get overdrawn again and so on and so on.

This downward spiral is not the result of rest and deconditioning. (6).  It’s a separate process, as attested to by people in good condition who suddenly deteriorate and find that they lose capacity much faster than ordinary deconditioning will account for (7). Of course I’m vulnerable to deconditioning just as anybody else is, and of course if I have to rest more I get physically weaker and it makes future exertion more costly.  But PEM is by far the more severe limiting factor, and if I try to ignore it for fear of deconditioning I simply get more ill more quickly. I’m sure I occasionally overestimate how much rest I need (although I think I underestimate it a lot more often); I could do with a device that could scan a muscle and measure the exact amount of metabolic debt and the perfect amount of rest that would pay it off.  But it would only minimise the Catch-22 between ME damage and deconditioning damage, not solve it. Like hangovers, there is no known cure for PEM and its consequences so the only thing you can do is try to avoid it.

Some people do gradually recover from severe setbacks with time and prudent energy management. Other people, including me, do not, and this is point #3 . Maybe I’m just not prudent enough, but my ceiling ratchets down and doesn’t go back up on its own and I can’t make it. I’ve had two significant remissions of my illness in fifteen years, but only as a result of drugs, and nobody knows for sure why they worked and what problem they were addressing.  And I know a lot of people, including many doctors, find the idea of exertion from which you don’t recover very difficult to believe. Whenever I’ve cited X or Y event from which I haven’t recovered as evidence of this phenomenon, my dad has said “But that was a year ago!”, or two years, or three years, and so on through the turtlebacks of infinity, as though that does anything other than illustrate my point. He seems to be saying, “Well, but that was a long time ago, it shouldn’t have a lasting effect.” Well, but it does. I expect sooner or later researchers will confirm its physical reality, as they are confirming all the other weird stuff pwME have consistently reported all these years, and eventually they’ll be able to explain it. Until then I can only guess.

In the mean time, it isn’t helpful, if I’m about to undertake an unavoidable activity such as going to the dentist and I warn you that it will be too much for me, it isn’t helpful to advise me, “Well you’ll just have to try.” I hope I’ve done enough to explain that trying is beside the point. I’m not saying I won’t do it or that it’s impossible, or even that I’m reluctant or afraid. It’s nice to get out of the house, actually – even for dentistry, even when it’s seriously fucked me up, I’ve still enjoyed it as an adventure as much as possible. I’m merely advising you that it will make me a lot worse, and telling me to try feels like telling a diabetic who’s just eaten a box of fudge to “try” not to become hyperglycaemic. Similarly, when I choose to forego a pleasure such as seeing a friend or coming downstairs at Christmas, it’s not because the consequences will be merely subjectively unpleasant for a bit; it’s because I judge they will do me lasting damage.  I can choose to exceed my limits, but I can’t choose to recover from that. I know this because I mess up and get worse quite a lot.

And this brings me to the last point I want to make. Because of the sting-in-the-tail nature of ME, it’s not only possible to make the mistake of doing more than you can recover from, it’s easy. Avoiding it is much harder, especially if you’re generally fidgety and compulsive and when you’re tired you just get more wired and want to keep going well into the red, which is the case with most pwME that I know of. There are unmistakable signs that I’m doing too much, my muscles feel like several kinds of crap and it’s frightening, but I can choose to ignore that and press on against my better judgement and screw the consequences.

This is a problem because it means I’m always having to try and stop myself consciously rather than rely on anything as convenient as collapse. Some people talk about ‘going floppy’ with paralysis or seizures when things get really bad; maybe things have just never got that bad for me, or maybe, more likely, I have a different subtype of the disease, but for whatever reason it’s never happened(8) and it doesn’t have to, because the damage happens without it and I can do without more symptoms that could be considered psychogenic.  I always openly tell doctors that I can enact activity which I choose not to because the consequences would be too severe.  That’s the reality and a faint, fit or paralysis won’t make it any realer.  I presume there is a point of unendurable exhaustion at which I would collapse and not be able to get up again, just as there is with everyone, but the point at which I’ve started to do myself severe lasting damage will have been and gone well before that.

And I get that all this probably seems weird to you. It’s weird to see people keep avoiding things that they seem to you to be able do. It seems weird to the Department of Work and Pensions, which decides that claimants are well enough to cook for themselves every day because it sees them go downstairs once, ignoring the fact that they take more than 24 hours to recover from it. It seems weird to a lot of doctors because yes, the idea of exercise making someone less fit is straight out of Bizarro world and I can understand why at first glance – at first glance – they might think the patient’s response to activity is irrational.  Walking on a broken leg is a lot more blatantly inadvisable than walking on a broken energy metabolism. When you add to that the extremely lax definitions of ‘CFS’, usually devised by psychiatrists, and the mishmash of people who are therefore considered representative of it, I can’t blame non-experts I otherwise respect when they start talking about deconditioning, confirmation bias and ‘the sick role’.

It seems weird to me too. For a long time I wanted to believe that the illness was a neurological illusion, my brain sending me false alarms about my body, so that I could keep going even when I felt like crap. The post I wrote for this blog just two years ago takes this kind of Scrappy-Doo mentality even further by wondering if I couldn’t just force a reset on my faulty neurology (and any knock-on biological dysfunction caused by it) by calling its bluff by getting incredibly much iller than ill and just keeping going till I literally dropped and sort of reductians it ad absurdum. In the end I didn’t try it: the CFS consultant at the RLHIM rightly nixed the proposed hospital admission which formed the substance of this scheme because it would have been too much for me. But of course it doesn’t work. People with ME who do get very very overtaxed, they don’t somehow turn full circle through to recovery, they just get severely and often irreversibly damaged, enter realms of torment that unfortunately I probably can imagine, and in extreme circumstances die. Whether they die directly or indirectly from ME probably nobody knows for sure, but in such circumstances I can’t see it as anything other than a mercy.

Nor have I ever quite let go of the hope that I can improve my physical condition by changing what I do or think. People are very much getting the wrong end of the stick when they think ME patients reject psychological or behavioural interventions because we’re desperately craving the validation of a ‘physical’ illness. By far the quickest and easiest way out of this would be if I or my hypothalamus or my amygdalas were stuck in a loop that I could unstick with rational thought, a reformed attitude and/or careful adjustment of my boundaries.  These approaches have never worked for me, and most of the assumptions behind them are speculative or disproven. But they were sort of consistent with a theory that I liked, which is that ME is the bodily result of brain dysregulation or damage and that at least in some cases people can retune or refashion their nervous system back into normal function.

This could possibly be achieved by all manner of methods, cognitive or behavioural ones included. I’ve tried psychiatry, psychotherapy, ‘sleep hygiene’, occupational therapy, cognitive behavioural therapy, graded activity or exercise therapy, ‘amygdala retraining’, ‘dynamic neural retraining’, every possible relaxation method and a partridge in a pear tree, willingly, on my own initiative and with my own money, over and over again, despite none of them ever having made even the ghost of a difference to my illness, because I’d rather be surprised by success than confirmed by failure. I kept trying on my own once I was too ill to talk to health professionals and it still kept not working, and if a patient is trying their best at a treatment and it doesn’t work it’s bad practice to blame the patient. It also has all sorts of undesirable consequences to keep telling someone that they should be able to get themselves better when there’s no good reason to suppose they can. So I don’t criticise the psychologisation or behaviouralisation of ME because I feel stigmatised by mental illness, I criticise it because it’s flawed, unsubstantiated and the treatments based upon it don’t work on me.

But then there’s no reason they should, actually, because these treatments aren’t designed for people as ill as me. No treatments are designed for people as ill as me. The UK medical establishment is simply not bothering with severe ME, and by this I mean the illest 25% of people, which runs to tens of thousands of patients. The studies that inform the NHS treatment guidelines don’t include anyone who isn’t already well enough to get to a clinic, and nor do most specialist services.  And that seems really weird, because if you want to understand a poorly-understood illness rather than just, say, bung at it the cheapest possible stopgap treatment you can pass off as effective, you probably shouldn’t be ignoring the people in whom its symptoms are most unambiguously present. It’s creating a vicious circle of inaccurate definitions leading to misleading research leading to inaccurate definitions. Progress in understanding ME is slowly being made, but it’s being made by doctors who started by believing their patients.  I don’t think that’s a coincidence; I think it’s vital.

And this is the final thing I want to say. I had wanted to explain how the workings of this disease have affected me specifically and in precisely what a precarious place it has left me, but this piece is already longer than I can read through in one go and it probably sounds disjointed and choppy, so thank you if you’ve stuck with it to the end.  The last thing I want to say is this. For me as an extremely badly affected ME patient, my quality of life is terrible and medical understanding is as yet inadequate, and these are misfortunes. But the lack of even the will to understand it among people whose job it is to do so is probably more traumatic and it is culpable. The gap in their knowledge has been filled with conclusions instead of questions, with ideology instead of evidence, and government seems OK with this because in the short term it’s cheaper. I much prefer the doctors – and there are plenty of them – who admit they don’t know what to do because nobody really does yet, but who want to understand and don’t start by presuming I’m an unreliable witness and don’t think that branding me delusional is preferable to admitting they don’t know. Nothing I’ve said here is new. It’s not distinctive, it’s not particularly insightful and it shouldn’t be controversial, it’s just bog-standard first-hand knowledge of an illness which I don’t fully understand and nobody else does either. I would like to be listened to, please, by people whose job it is to listen to me, and to be believed unless there’s good reason not to.

 

Notes
I’m including various references to substantiate my statements but please don’t feel you have to read on past here. I can’t read very much of anything any more so I’ve only got the gist of some of these articles myself, but most of the basic facts of ME that I’m talking about are pretty well established now. Again, I am trying not to make this post into a polemic but I do want to show that inactivity and fear of exercise are not the cause of ME because this misapprehension continues to do huge damage to patients and to the prospects of research and understanding.

1) http://www.ncbi.nlm.nih.gov/pubmed/23813081

The researchers who demonstrated this problem found a big decrease in aerobic function as measured by a cardiopulmonary exercise test (CPET), which is an objective measure, i.e. it doesn’t rely on a person saying they feel tired.  The clever bit is that they used a repeat test to allow for PEM’s delayed onset because it can take 24-48 hours to hit a person fully.  So they got people to do exercise on the first day, on which their results were normal or a bit unfit, and then tested them again on the second day once PEM had hit them and saw their results plummet. Apparently this is so unusual it sends exercise scientists scurrying off to check their equipment to ensure it’s working properly.  And apparently even people with seriously limiting fitness problems such as heart failure or COPD are roughly able to reproduce their previous day’s levels rather than nosediving the way ME patients do, and this is crucial because it demonstrates the difference between people with ME who can’t recover from effort and people who for whatever reason can’t put in the effort to begin with.  (Severely ill ME patients don’t have as much of a cushion and would probably get worse much quicker, but then severely ill patients are generally too ill to get anywhere to be studied. (But see note 7))

2)  I had to remove the reference to the famous twenty-times-lactic-acid in vitro result after further in vivo experiments didn’t replicate it.

www.meassociation.org.uk/2013/04/biological-breakthrough-offers-fresh-hope-for-me-sufferers-the-times-23-april-2013/

I’m not sure this is the study referred to in the ME Association’s article but you’re welcome to go through Dr. Newton’s publications and find the correct one for me if it isn’t. I know that conventional exercise burn is in fact thought to come from hydrogen ions in general, not lactic acid, but twenty times more lactic acid than normal suggests a problem with some process somewhere.   Many doctors think that CFS is a category rather than a single illness, and Dr. Newton has found different subtypes of abnormalities in the CFS patients she studied, including some which she thinks may actually respond to exercise. Here she is chatting about it with Dutch subtitles.  But I think she used one of the definitions of CFS in which exertion intolerance and PEM are optional, which illustrates the need for much more research into precisely what is going on in the biology of these conditions and the pressing need to disentangle the diagnostic clusterfuck of CFS and ME and ME/CFS and CFS/ME.

3) This later study confirms the findings in the (1) study and explains their implications in greater detail.

4) Dr. VanNess who is an author of the study in note 1 underlining the point that fear of exercise in ME is a rational protective response, not a phobia.

5) At 24:30 in this video Dr. VanNess from note 1 talks about a woman who believed she could fix her aerobic capacity with training and made it worse according to the cardiopulmonary exercise test. There isn’t a formal study of this sort of thing, presumably because it would be wrong to deliberately harm people to prove a point, but we do now have a lot of evidence verifying pwME’s perceptions that when we feel as though there is a problem with energy production it is because there is a problem with energy production. You might think this is depressing but mainly it’s a huge relief to have our experience heard, investigated and verified after decades of doctors setting out with the assumption that we’re all imagining, misunderstanding or overreacting to what is in fact a pretty unmistakable experience.

6) m.openheart.bmj.com/content/3/1/e000381

Another study by Dr. Newton, which finds physical pathology correlated with severity of fatigue but not length of disease, i.e. the findings are unlikely to be caused by inactivity.

7) A Dutch doctor with long-term severe ME who was formerly a highly trained amateur athlete wrote this rather charming if sprawling n=1 study on himself, analysing his muscle metabolism after exertion.  Unfortunately we don’t have data for before his illness, only his first-hand accounts:

“Overnight, from one day to the other, I lost 70 to 80 % of power in my legs and suddenly, walking 15-30 yards was very far, and after that my legs felt very heavy and needed 15 minutes to recover before I could walk 15-30 yards again.”

Not everyone gets this sudden onset – I didn’t –  but given how often ME does start suddenly in active people and produces symptoms which can be verified by blood tests it’s unhelpful that the current UK definition calls it chronic fatigue syndrome and requires the illness to have been present for six months before you can diagnose it.

8) Obviously I have had the seven things-which-are-probably-seizures, but they don’t happen when I’m particularly tired or stressed, they’re random.  My possibly-myoclonus tends to only happen when I’m feeling relaxed, bubbly and alone, so if it is psychogenic attention-seeking I don’t know why it doesn’t happen when anyone’s paying attention, unless that’s just too easy for the likes of me.  But I did get it on video once in the hope that somebody could tell me whether it is myoclonus or what, so if you want to see me making extravagant involuntary movements in my bra, you’re in luck

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