This is the last in a three-part series on the very severely ill in chronic fatigue syndrome (ME/CFS):
- What is Whitney Dafoe’s Extremely Severe ME/CFS Story Telling Us? – Whitney Dafoe describes in penetrating detail what it’s like to be extremely severely ill. (From the Health Care Journal Special edition on the severely and very severely affected.)
- We are Failing People with Very Severe ME/CFS – Amber Ella poses ways we can better support the very severely ill.
The last part of this series is an overview of an unusual journal publication, “Special Issue ‘ME/CFS – the Severely and Very Severely Affected'”, devoted to severely ill people with ME/CFS.
Devoting an entire edition to the severely ill with ME/CFS was shocking. Not only do the severely ill often not have access to doctors they hardly ever participate in research studies. If what we think we know about the prevalence of severe ME – as much as 25% of the ME/CFS population – is correct (see below) they make up a considerable portion of the ME/CFS population. As long COVID emerges the severely ill, in particular, present a cogent warning of how bad post-infectious diseases can get. This is a timely edition.
This landmark edition (edited by Kenneth Friedman, Lucinda Bateman, and Kenny De Meirleir) contains 21 papers, studies, and editorials which flesh out the experiences and biological aspects and needs of the severely ill in a way we’ve never seen before.
Severely Ill ME day seemed like a good time to provide an overview of the edition.
“Our intent is to redefine ME/CFS as the serious disease that it is”. Editors of the Special Issue on ME/CFS – the Severely and Very Severely Affected.
High Prevalence Found
The “Homebound versus Bedridden Status among Those with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome” study used a nice big sample size (n=2138) to conclude that the 25% figure for the severely ill is actually probably accurate (!). That suggested as many as 385,000 people in the U.S. may have a severe ME/CFS illness that leaves them home or bedbound.
Surely no other chronic illness comes close to matching those figures. We know that, relative to its needs, ME/CFS is easily the most underfunded disease at the NIH. It’s remarkable, indeed, that we’ve lacked good prevalence data for the severely ill. In fact, we lack the data needed to definitively determine a crucial epidemiological figure – disability-adjusted life years (DALYs) – which are used to determine if health care dollars are going where they are most needed. We’ve been banging on the CDC’s door for more dollars for a major epidemiological survey, and have gotten close, but we’re not there yet.
Studies like the above will hopefully open some eyes and get some more money flowing our way.
Snow to the Eskimo – Functionality to the ME/CFS Field
There’s an old report which points to the multiple terms Eskimos have for snow. They, apparently have distinctions for snow that we could never dream of. They just know snow. The ME/CFS community – from patients to doctors to researchers – just knows functionality. The ME/CFS field is creating terms and new ways of assessing functionality that the medical field hasn’t thought of in a hundred years.
In “Cerebral Blood Flow Is Reduced in Severe Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients During Mild Orthostatic Stress Testing: An Exploratory Study at 20 Degrees of Head-Up Tilt Testing”, the Van Campen, Visser and Rowe team, which has done so much important work recently, took their studies of functionality and cerebral blood flows to the severely ill.
Nobody wants to do a tilt table test. I, with my mild to moderate case of ME/CFS, did one years ago and was miserable throughout. It made sense that Van Campen et. al. asked if maybe a less severe tilt table test would suffice for the severely ill? There’s no need, after all, to torture someone confined to their home or bed with an aggressive tilt test.
Instead of a 30-minute 70-degree tilt table test, they tried a 15-minute 20-degree tilt and found that was indeed sufficient to send their severely ill ME/CFS patients’ heart rates soaring, their hearts’ stroke volume declining, their CO2 levels dropping, and their blood flows to the brains tanking.
Those precipitous declines (and the heart rate increases) even at that mild tilt led them to go even further. If even a mild tilt smacked the severely ill patients’ cardiovascular systems, what effect would something as innocuous as sitting up have?
This study found there was no need to do a regular, or even a mild, tilt table test to produce significant reductions in brain blood flows in many people with severe illness: all they had to have them do was sit up! Simply sitting up caused about a 27% reduction in blood flows to the brain in the severely ill.
The severely ill, like the less severely ill, however, are not a monolithic group. An interesting subset also appeared: severely ill ME/CFS patients who did not complain of orthostatic intolerance did not have reduced blood flows to the brain upon sitting. Something else was contributing to their severe illness.
The ME/CFS field is continuing to blast apart traditional measures of functionality. No one, to my knowledge, has felt the need to assess the effects of mild tilt table tests. Nor have other fields produced a measure like time spent with “feet on the floor”. Nor has anyone, apparently, attempted to determine if simply sitting up could mimic a tilt table test in the more severely ill.
Exercise Testing the Severely Ill
Van Campen, Visser, and Rowe then asked whether more severely ill ME/CFS patients did worse, as might be expected, on a two-day cardiopulmonary exercise test (CPET). They used the following classification:
- mild: approximately 50% reduction in activity;
- moderate: mostly housebound;
- severe: mostly bedbound;
- very severe: bedbound and dependent on help for physical functions.
They analyzed the CPET results of 82 patients, of which 31 were classified as severe. As expected, as patient severity got worse – so did their ability to generate energy, peak oxygen consumption, as well as oxygen consumption, peak workload, and workload – all at the ventilatory threshold. In fact, the severely ill ME/CFS patients posted the lowest CPET values yet reported in ME/CFS studies. This study suggested that energy production productions are, indeed, the distinguishing characteristic of ME/CFS.
Interestingly the drop in oxygen consumption (energy production) from day 1 to day 2 was not greater in the severely ill. All the groups had similar drops in energy production. This may suggest that exercise doesn’t mess up energy production more in this group but that they simply start from a lower baseline of energy production. The idea that they might not get whacked harder by a 2-day CPET test than moderately ill patients might good news for some more severely ill patients who are worried about tolerating these tests for a disability evaluation.
Amber Ella pointed in a recent blog on Health Rising “We are Failing People with Very Severe ME/CFS” the often-overlooked role that stomach issues and malnutrition present in the severely ill.
“The 5-case series reports in Helen Baxter’s paper ”The ‘Life-Threatening Malnutrition in Severe ME” validated Amber’s concern. Baxter et. al noted that all five ME/CFS patients became “seriously malnourished and dehydrated” before they were provided nutrition via tube feeding.
An inability to swallow on the part of some of them was believed to be psychological in origin and anorexia nervosa was often invoked – yet oddly enough treatments for severe anorexia nervosa were not provided. Several were threatened with being “sectioned” under the Mental Health Act if they did not start eating and drinking again.
Essentially the doctors had no idea, or interest, in the fact that severe malnutrition and dehydration can accompany severe ME/CFS. Instead, they focused on psychological issues but with a potentially deadly twist. Feeding tubes are provided to people with anorexia nervosa who need them but not for this group – at least not in a timely manner. Each of the five people developed life-threatening dehydration/malnutrition issues which were resolved only after they were given nutritional support. Getting dieticians involved was important in rescuing several of the ME/CFS patients.
The authors recommended that an ‘early warning system” be created so that if severely ill ME patients stop eating, they can be provided nutrition in another way before they become dangerously debilitated.
Loneliness and ME/CFS
“The Lonely, Isolating, and Alienating Implications of Myalgic Encephalomyelitis/Chronic Fatigue Syndrome” paper highlights the extra burden people with ME/CFS often carry. Noting the myriad of negative non-physical consequences that come with having a disease like ME/CFS, the authors report that the “adjustment period” to having a chronic illness – never an easy time – is, in the case of ME/CFS “filled with many new expected and unexpected vulnerabilities.”
The authors make a distinction between solitude and loneliness. One is a choice one makes and the other is a burden that causes “excruciating physical and mental suffering.” Loneliness, the authors assert, is an emotion that was created so that we will connect to others. As humans, “we need to feel connected to significant others”.
Loneliness doesn’t simply have emotional impacts – studies show it can have biological ones as well. Loneliness, in fact, is associated with a wide range of health problems and has been linked to an increased risk of dying, increased risk of cardiovascular disease, and reduced immune responses (including NK cells). They have as potent an effect on health as cigarette smoking, high blood pressure, obesity, and a sedentary lifestyle. In short, having social ties is helpful both emotionally and physically.
Most people with a chronic illness have to deal with some form of isolation. The authors acknowledge, though, the special problems ME/CFS poses in maintaining social relationships.
“It is quite clear that being riddled with such symptoms for a lengthy period of time would make socializing, interpersonal connection, and remaining connected to others problematic and would most often require a termination of those relationships.” Simply the newfound difficulty of keeping up a conversation poses its unique problems.
The question is what can one do about that? There are no easy answers. With the goal being to reduce the stress associated with isolation, there are no easy answers. The one takeaway – finding a way to reduce the isolation associated with severe ME (or ME for that matter) may help.
Joining support groups, interacting on social media, not giving energy to catastrophic thoughts, learning deep muscle relaxation are some suggestions. Another idea is that instead of choosing to see the social isolation produced by the disease as a burden and a loss, try to flip that viewpoint and see it as an opportunity to explore mindfulness/meditation and other spiritual practices in more depth than would otherwise be possible.
A Doctor’s View: “When Suffering is Multiplied”
“Chronic Fatigue Syndrome: When Suffering Is Multiplied“, by Anthony Komaroff is simply a lovely, heartfelt piece from a doctor and researcher who has thought about ME/CFS and its place in the medical field for many years.
Using Whitney Dafoe’s very severe ME/CFS story as a backdrop, Komaroff speculated why people with ME/CFS – in particular, people with severe ME/CFS – have had so much trouble with doctors. Why has the disease – even after thousands of studies – failed to attain legitimacy in many medical circles?
I still vividly remember Dr. Bateman’s comment after she and Ron Davis enrolled researchers, saying ME/CFS was a fascinating disease to study. Paraphrasing what she said, “Researchers weren’t hard – if you give them the facts, they get it about ME/CFS. Doctors, on the other hand, are the hardest to convince”.
But why? Komaroff notes that the symptomatic diagnosis does not help – but also notes that recognized diseases such as migraine are diagnosed on the basis of symptoms as well. It didn’t help that standard blood tests – which can reveal the presence of so many problems and conditions – are not totally illuminating in ME/CFS.
I still remember a jarring meeting with a doctor I’d looked forward to seeing after I found out he was a backpacking buff – my kind of guy, I thought! As he paged through my sheaves of unilluminating medical tests, I could see his anger build. His tone grew sharp. He was clearly emotionally disturbed by all these unrevealing tests. It wasn’t what he expected at all. Instead of seeing me as someone trying hard to find the answers, he saw me as a dead end, a problem, a kind of blight upon the medical system. By the end of our session, he was the one who needed psychological help – not me. (He sent me onto a psychologist – who promptly reported I was not depressed.)
At that, Komaroff says they have some pretty clear choices:
- They could entertain a new hypothesis and explore further;
- They could admit defeat and acknowledge that the medical profession just hasn’t figured this out yet;
- They could try treatments that might help the symptoms even if they don’t know what caused them;
- Even though they know that the “standard” tests they had ordered represented only a tiny fraction of all of the tests available to them”, they could decide “there’s nothing wrong with you”.
Komaroff noted how “efficient” a solution the last one was. It washed the doctor’s hands of the patient, leaving her/him to their own devices. That diagnosis oftentimes continued to insidiously do more harm by damaging the credibility of the patient in the eyes of her/his family, friends, work environment, etc., and in doing so, likely multiplied the patient’s suffering markedly.
How much easier it would have been for everyone – patients, family, even perhaps the doctor living with the niggling concern that he/she might have just betrayed their Hippocratic oath (and did harm, instead of good) – if he/she had just said: something is wrong, but I don’t know what.
Decades after dealing with all four categories of doctors, I get nervous telling a doctor I have ME/CFS/FM. I avoided telling a recent primary care doctor about it for a year or more into our relationship, at which point I said “there’s something I haven’t told you”. Luckily, he responded well. So did the next doctor I told.
Komaroff reported that if a doctor cares to look, he will find that the impact of new medical technologies on our understanding of ME/CFS has been massive. While we don’t know what causes it, it’s insane, at this point, to think that nothing is wrong.
For his part, Komaroff believes that all signs are point to the brain. The neuroinflammation, the autoimmune findings, the metabolic problems, the autonomic nervous system issues, and the gut problems all either emanate from the brain or affect it. The big question for him and this field is: where does it all start? What’s the core abnormality all these others feed off of?
In the end, Komaroff believes that ME/CFS is going to be a great teacher. Whitney Dafoe called it his greatest teacher, and Komaroff agrees. It’s not just that understanding what’s going on in ME/CFS is going to, as he and Ron Davis believe, help us understand what’s going on in “many other diseases”, but the teaching role he believes ME/CFS will play in the medical system as a whole. Never again, Komaroff, believes, will the medical system be so quick to believe that “nothing’s wrong”.
“if patients tell you they are suffering, your default assumption should be to believe them—even if you cannot find an answer with the diagnostic technology you first deploy. Above all, never succumb to the temptation to dismiss the patient’s symptoms because you cannot explain them. That may ease your anxiety, but it only multiplies the patient’s suffering.”
ME/CFS has been batted around by doctors, researchers, the NIH, the CDC, and other institutions for decades. It’s not that the NIH, CDC, and others are blasting ME/CFS. It’s more that they’re killing it with benign neglect; i.e. they’re saying all the right things, evoking sincere concern, providing (oral) support – while doing very little.
Telling the ME/CFS community that it’s responsible for raising itself by its bootstraps, for funding its own clinical trials and research studies, that it’s responsible for increasing researcher interest, may temporarily ease the NIH’s anxiety, but it does nothing to diminish their responsibility for creating a system which has allowed millions of very sick people to be essentially ignored for decades.
The response to the ME/CFS chapter constitutes a vast moral failure on the part of the NIH, the CDC, and the medical system overall. Hopefully, the long-COVID situation will shine a spotlight on that failure, and provide the NIH and the medical profession the opportunity to relieve their burden – and ours.
Perhaps it will give them the opportunity to acknowledge – if they have the guts – how badly they screwed up, and what the costs of that failure have been for many ill people. That would give them the opportunity to find ways to make up for that cost; to learn how they ended up acting so contrary to their missions, and to put in place procedures to ensure that they don’t happen in the future. We would all be happier for that.
To end this overview of some of the papers in the Special Edition, check out a series of videos on Severe ME from Natalie Bouton. Note also that all the papers in the Special Edition are free.
Severe & Very Severe ME / CFS – an Introduction / Symptoms and Management / Hospital Admission
Also, check out a powerful video Jonathan Avery’s 19-year-old daughter made for Severe ME day which juxtaposes her when she was healthy with her being ill. People with severe ME say expresses much of how they feel.
And do check out a poem “The Unsung Heros of Our Time” from Whitney Dafoe.
The Unsung Heroes of Our Time – A Poem by Whitney Dafoe for Severe ME Day
Whitney Dafoe provided the most complete illustration of a very severe ME Illness in his Extremely Severe ME/CFS—A Personal Account piece in the Special Edition. For Severe ME day, he provides an uplifting poem.
“ME/CFS patients are the unsung heroes of our time. We fight the suffering in this world silently and never get recognition from anyone. But still we fight. For freedom. For life. From the shadows we rise like pond lilies. Shining light. Silently, unbeknownst to the world we live in. Our courage soaked into the walls of time. And the rest of them. They ride the wave that silent heroes make. Alone. Generating the will to endure from someplace deep inside. And the rest of them. They live with that strength everyday. But they never know where it came from. No one does. It came from us. The ME/CFS heroes that cling to the edge of life for endless years, radiating that life outward to all. Because that’s what we do. We change the world. In silence. And without acknowledgement. But we don’t need it. That’s not why we fight. We do it for the light. That everyone else uses to see. And they never knew where it came from. It came from us. The silent ones. The forgotten ones. The ones who live our whole lives in darkness. We give that to the world. For free. And we don’t ask for gratitude. Because it’s what we do. We are ME/CFS patients. And we never stop giving off light. It flows from us endlessly. It weaves the fabric of the world. And the rest of them. They never knew where it came from. It came from us. The unsung heroes of our time.”
While several of the pieces in the Special Edition do not focus on the severely ill, most do, and they provide an introduction into the world of the severely ill for doctors, researchers, patients, and caregivers. If the goal was to “redefine ME/CFS as the serious disease that it is,” it was at least partly achieved.
The edition also showed a light on what’s missing with the severe ME/CFS population; a better understanding of prevalence, how they differ from the less severely ill (illness onset, duration, biological findings), what the risk factors for very severe illness are, how much medical attention they are getting, etc. Are they moderate ME/CFS cases that have simply worsened, or are they atypical in some way?
We should be getting a lot more biological findings with the publications coming out of the Open Medicine Foundation’s Severe ME/CFS study, as well as the (knock on wood) Center for Disease Control’s Multi-site study, which in its last iteration included a significant population of the severely ill.