Calling it chronic fatigue is a bit like calling a pie “a crust”
Anna, age 16, personal communication, 2018
Rose Silvester is a clinical psychologist based in Wellington, New Zealand. In the context of her son’s illness she has immersed herself in the literature available on ME/CFS and models of care for chronic illness. She is engaged with the global ME/CFS community of researchers, clinicians and advocates and has initiated a national carers support network (NZ carers of kids with ME/CFS and related illness - NZcare4ME) as well as a local carers support group.
This article was first published in the June 2018 edition of the Journal of the New Zealand College of Clinical Psychologists (NZCCP).
My son lives behind the closed door of a dark room. He has been there for three years, he is 18, he has myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS).
I cannot unlock the door.
Unlike most other people who have this problem, his onset was not abrupt and not obviously triggered by an assault to the immune system, such as by one of the many associated viruses, or by vaccination, or any event that created significant stress for the body. Toward the end of his primary years, I found myself frequently making excuses for him, for why he was very bright but struggled to be on task, why he went from being in the top 10 in the school cross country to being hundreds of metres behind the last kid, why at soccer following the half time sugar hit that revved the other kids up, he was listless and just watched as the ball rolled past. I blamed it on the insomnia, night sweats, and headaches because they were certainly there, and I thought to myself “kids are weird—they grow out of it.”
And he did grow out of it—for a time. He had periods of many months where we thought the difficulties had stopped. By the time he reached high school, however, a small cold would see him confined to bed long after the cold symptoms had gone. There was nothing specifically wrong, no fever, vomiting, rash, or cough. He denied that he was tired or fatigued. Nothing. His batteries were just flat. These episodes began to get more frequent, more severe, and his recovery in between, less complete. During one memorable episode, I took him to the GP. He could hardly walk and had to lean against the wall of the corridor to make it to the office. He slurred his words, he was drowsy—he was clearly a very sick kid. After much shoulder shrugging and head scratching we were off for blood tests. While these revealed minor abnormalities (hypoglycaemia, slightly off thyroid function), a blood test could not possibly reveal the problem. These minor things, however, threw everyone off track. And there we remained for over a year. It was during this year that the door began to slowly close.
Most of the fourth term of year 10 at high school was spent at home. We tried to keep him going. He would get up, get ready for school, but be stopped in his tracks by body aches, headaches, and weakness. He was dizzy and fainted half a dozen times. His heart rate while standing would soar to 140 bpm and stay there. We know now that these early symptoms were the hallmark orthostatic symptoms of postural orthostatic tachycardia that often precede full syndrome ME/CFS. He spent most of the term at home and did gradually recover—enough to have a good summer. Again, we thought “it” whatever “it” was, was going to leave him alone. He was excited to go to school with his mates on the first day of year 11. At Wellington High School, year 11 kids are allowed to go to town at lunch time and he took full advantage of the freedom. He came home happy. That night, sometime while he slept, the door slammed shut. He has not been able to return to school.
Myalgic encephalomyelitis (my*al*gic + en*ceph*a*lo*my*eli*tis) (ME), is commonly referred to as chronic fatigue syndrome (ME/CFS). We eventually achieved this diagnosis, but too late to be aware of the cumulatively damaging effects of the episodes of unwellness that we now know were post exertional malaise or “crashes.” The need for careful pacing was never discussed. Too late too, to take advantage of the potential that managing his orthostatic symptoms more effectively may have reduced some of the stress his body was under.
The Institute of Medicine (IOM) describes ME/CFS is a serious, chronic disease that affects an estimated 0.5%–1% of people (Institute of Medicine, 2015). That adds up to more than 20,000 in New Zealand. There is no known definitive aetiology or effective treatment. Diagnosing the disease remains a challenge, and patients often struggle with their illness for years before identification is made. Recovery of pre-illness functioning is rare. Many people with ME/CFS are more impaired in their functioning than those with other chronic and disabling illnesses, including congestive heart failure, depression, multiple sclerosis, and end-stage renal disease (Twisk, 2014). Women are more likely than men to be afflicted, and although peak onset is in adulthood, children also commonly get ME/CFS. It is estimated that as many as 90% of people (across all ranges of severity) are not diagnosed (Institute of Medicine 2015).
The cause remains unknown, but currently the most likely contender is that it is a systemic disease, and that in vulnerable (most likely genetically vulnerable) individuals, an assault to the immune system causes an abnormal immune response, which in turn causes an inflammatory reaction that affects the whole body right down to the level of mitochondrial energy production. In particular, it seems that the autonomic nervous system becomes unbalanced, leaving people stuck in a state of sympathetic overdrive—a constant state of fight or flight affecting heart rate and blood pressure (orthostatic regulation), temperature regulation, pupillary reaction, digestion, sensory filtering, emotional regulation, and so on. Pain in joints and/or the generalised ache of the first few days of the flu is usual. Cognitively, the effects can, for some, be devastating. Widespread endocrine abnormalities in many people suggest that the delicate balance of the hypothalamic-pituitary-adrenal axis has wobbled. Not surprisingly, sleep becomes inefficient and unrefreshing. Homeostasis is wrecked. It is exhausting. Post-exertional malaise is a hallmark symptom. It simply means that the window for tolerating any exertion, be it physical, cognitive, or emotional has narrowed. A marked worsening of symptoms occurs in the 12–72 hours following exertion. This worsening, sometimes called a crash, can persist for days, weeks, months, or years. Inter-episode recovery can decrease with each crash. For around 25% of people, the window narrows to a point whereby activities of independent living are not possible. They are confined to their houses. Many are bed bound.
The pathophysiology of ME/CFS is very, very complicated. While recent research has identified a range of potential biomarkers and clear metabolic abnormalities in people with ME, these tests remain tools of research. Diagnosis is by symptom identification and exclusion of other illnesses that may present in a similar way. Over the last few decades, diagnostic criteria for ME/CFS have been widely debated. In 2015, the National Academy of Medicine published new diagnostic criteria for ME/CFS requiring the presence of: substantial impairment in activity that lasts 6 months or more and is accompanied by fatigue, post-exertional malaise, unrefreshing sleep, and either cognitive impairment or orthostatic intolerance (Institute of Medicine 2015). These criteria are, on the face of it, a massive simplification of a very complex picture in which the likelihood of multiple disease processes exists. Agreement and consistency of definition is, however, welcome.
The label “ME/CFS” has been similarly debated. Clinicians, researchers, and people with ME/CFS have pushed back, particularly against the label of chronic fatigue, arguing that it minimises the breadth and savagery of symptoms. It also has the unfortunate history of the misunderstanding and minimisation that came with derogatory labels such as “yuppie flu.” While the term CFS is rapidly on its way out, ME (for now) remains (grudgingly), and the IOM label of systemic exertional intolerance disease may be on its way in (Institute of Medicine, 2015).
ME/CFS has not had an easy history, and it continues to sit uncomfortably. The diagnosis has fallen victim to the kind of stigma and marginalisation that is common when we are faced with things we cannot easily test or measure. The notion that ME/CFS can be formulated as a psychogenic disorder, such as functional neurological symptom disorder (conversion disorder), medically unexplained symptoms, or somatoform disorder has prevailed in both the medical and psychological communities.
Early hypotheses of psychological causation have long since been countered by growing research showing biological correlates of ME/CFS not found in depression or any other psychiatric disorder (Stein, 2005). The prevalence of known psychiatric disorders among patients with ME/CFS is similar to the rates in patients with other chronic, disabling medical conditions, such as rheumatoid arthritis; approximately 30%–40% (Thieme, Turk, & Flor, 2004). The World Health Organization classifies ME/CFS in the International Classification of Diseases, tenth revision as a neurological disorder (ICD 93.3). Little evidence exists for any disorder in this category conforming to a psychogenic formulation (Wilshire & Ward, 2016). Healthcare providers, however, remain sceptical about the physiological—rather than psychological—nature of the illness. “Once diagnosed, patients often complain of receiving hostility from their healthcare provider as well as being subjected to treatment strategies that exacerbate their symptoms” (Institute of Medicine, 2015). Within the ME/CFS community, anecdotes of gas- lighting and exclusion from treatment are the norm. I would suggest, where scepticism or doubt exists for health providers, that these notions are grossly outdated and perhaps “all in their heads.”
Psychology, and particularly cognitive behavioural therapy (CBT), has for some time been positioned in the centre of this discomfort. In recent decades, the officially recommended treatments for chronic fatigue syndrome (ME/CFS) have been graded exercise therapy (GET) and CBT. These treatments are formulated to satisfy the premise that ME/CFS symptoms are precipitated by factors such as trauma, prolonged stress, or personality factors, and are perpetuated by avoidance of activity and aberrant illness beliefs (Wilshire & Ward, 2016). The idea is that graded desensitisation (to the anxiety produced by activity) will help the individual to appreciate that pain and fatigue are not harmful. I recently Googled ME/CFS and CBT—the first entry that came up sums up this position:
...illness beliefs may lead to disability, as people obsess about their symptoms, entrench themselves in the conviction of organicity, and become disabled. Their marriages may break up; they may lose their jobs. The human consequences of these illness beliefs, in other words, may be considerable. Joining a sufferers’ support group that will irreversibly confer a label is really the last step on this pathway to disability. (Shorter, 2015)
Based on the assumption that psychological factors both precipitate and perpetuate the disorder, and, in an attempt to provide evidence of the efficacy of CBT and GET, White and colleagues undertook a large-scale randomised trial (White, Goldsmith, Johnson, & Potts, 2011). This was referred to as the PACE trial. Reports published in reputable journals (including The Lancet) concluded that the PACE graded exercise and CBT protocols were moderately effective treatments for ME/CFS, leading to “recovery” in over one-fifth of patients. The trial’s size and subsequent promotion of CBT and GET as a gold standard treatment became hugely influential in the development of treatment guidelines, including the National Institute for Health and Care Excellence (NICE, 2007) and Centre for Disease Control (CDC) guidelines.
Soon after publication, however, there began a clamour of dissent. People with ME/CFS were perplexed, stating that the premise was incorrect and that, in their experience GET and CBT were not only ineffective, but were harmful. In forcing people with ME outside of their safe window of energy consumption, they risked a crash. The result, for many, was catastrophic. There also emerged significant concerns about the methodology of the trial. Sitting at the tip of this rather large iceberg of concerns was that the outcomes and analyses reported did not follow the original published protocol (Wilshire et al., 2018). This was concerning given that the purpose of a trial protocol is to prevent post hoc changes to methodology that may favour the study hypotheses. Also, it was doubtful whether the trial’s conclusions about treatment efficacy were supported by the evidence. David Tuller, a critic and prolific writer on the failures of the PACE trials reported a “bizarre paradox” (Tuller, 2015). The scores that determined whether someone qualified as disabled enough to enter the trial could simultaneously qualify them as “recovered” (by the definition of the outcome measures). A full 13% had “recovered” before the trial started, based on the change in protocol that occurred months after data collection ended (Tuller, 2015).
It is in this context that within a few years, the clamour turned into a furore, and accusations as strong as scientific fraud were levelled against the PACE trial authors by the ME/CFS research community. In 2015, an open letter was written to Richard Horton, Editor of The Lancet, by eminent researchers in the field of ME/CFS, including Ron Davis, Professor of Biochemistry and Genetics at Stanford University (and a current leading light in the ME/CFS research community as well as father to Witney, a young man with very severe ME/CFS). The letter raised concerns about “serious ethical breaches in the study” and stated that the flaws inherent in the study “have no place in published research.” This was strongly worded criticism, and went on to say the shortcomings were “of particular concern because of its significant impact on government policy, public health practice, clinical care, and decisions about disability insurance and other social benefits.” The Lancet was urged to “seek an independent re-analysis of the individual-level PACE trial data, with appropriate sensitivity analyses, from highly respected reviewers with extensive expertise in statistics and study design” (Davis et al., 2015).
While voluminous discussion and reanalysis has subsequently been published, this reanalysis was hampered by the refusal of the original authors and PACE trial committee to release the original dataset for scrutiny. These data were, however, recently obtained as part of a Freedom of Information application, and made available to the public. In 2018, a new player walked onto this complex stage with the publication of a reanalysis of the PACE trial using this previously unavailable original data (Wilshire et al., 2018). Imagine my surprise when the author turned out to be Dr Carolyn Wilshire, from the School of Psychology at Victoria University. We talked about the big picture and her findings over coffee in Aro Street.
Carolyn Wilshire’s thorough and hopefully definitive reanalysis of the PACE trial concluded that, despite the power of the study afforded by the large sample size, the results were “modest, short-lived changes in self-report behaviour unaccompanied by objectively measurable changes.” She further commented that “it seems unlikely that further research based on these treatments will yield more favourable results” and “the time has come to look elsewhere for effective treatments” (Wilshire et al., 2018). Even the use of CBT for basic distress reduction has been called into question with the results of repeated patient surveys indicating that for many, CBT is harmful (Laws, 2017). Last year, in response to these contradictions, the CDC quietly changed its ME/CFS treatment guideline, removing any reference to GET and CBT, and clearly stating the likely harm (CDC, 2017). Similarly the NICE guidelines group announced a revision and specifically cited conflicts in the evidence for GET and CBT as a major basis for review (NICE, 2017). Unfortunately, the revision will not be released until late 2020 so for now the NICE guidelines remain available to view. Current National Institute of Health (the major US health research funder) research initiatives are focused on the pathophysiology of ME/CFS. The dream of the ME/CFS community is that these initiatives will play a key role in generating new treatment paradigms (Wilshire et al., 2018). A hopeful sign was that in April this year, the NIH hosted a collaborative research symposium with the aim of accelerating research and included workshops on promoting early career development of prospective researchers.
But the research initiatives are, in the scheme of things, in their infancy and comparatively poorly funded. Crowd funding and private donations for research remain common. For example, Ron Davis’ research has largely been funded by patients, and the Pineapple Fund, a fund provided by a private Bitcoin investor. Investigations into ME/CFS have lagged so far behind other disorders that some of the figures would be comical if they were not so tragic. Jorgen Jelstad (2016) quantified this inequity, and noted that although the measures of quality of life of people with ME fall below that of people with multiple sclerosis, more is spent on research into MS every year than has ever been spent on ME/CFS. Research into HIV/AIDS in the US attracts around 600 times the funding, although HIV affects similar numbers of people, with better outcomes. Male pattern baldness gets six times more federal funding than ME/CFS in the US (Jelstad, 2016). It is not so strange then, that it is taking time to find any credible answers to the ME/CFS enigma. Nancy Klimas, Professor of Microbiology and Immunology and a prominent ME/CFS specialist clinician (there are only a handful) was quoted in the New York times in 2009:
“My HIV patients for the most part are hale and hearty thanks to three decades of intense and excellent research and billions of dollars invested. Many of my ME/CFS patients, on the other hand, are terribly ill and unable to work or participate in the care of their families. I split my clinical time between the two illnesses, and I can tell you if I had to choose between the two illnesses I would rather have HIV”. (Klimas, 2009)
This lack of research is a barometer of a broader issue of the value that the health sector places on people with ME. Let me give you an example that is very close to home. A recent meeting for a very elderly relative who was moving to palliative care was attended by the geriatrician, rest home manager, charge nurse, dietician, social worker, and psychologist. While I am loath to sound petulant, my son, who is so very unwell, is seen by one physician around every 4 months. This physician has worked hard to consult and refer where possible. The waiting and time to get answers has however, been interminable. Even for some simple and commonly used specialist consultations it has been years. This is such a long time in a young person’s life at a critical developmental stage. Our health system is not designed to deal with complex problems involving multiple organ systems. If I had a magic wand I would conjure up specialist integrated teams of clinicians (physicians, cardiologists, endocrinologists, immunologists, neurologists, physiotherapists, occupational therapists, psychologists, and social workers) who would work together to optimise the management and quality of life for all people with ME.
So where does this leave psychology in supporting this population? Did you, like me, see the baby sailing through the air with the PACE trial bathwater? The downstream effect of the PACE study is that in all the fuss, the real value of psychology for people with ME has been neglected and remains ambiguous. For myself, I know that there is a lot to be gained from acceptance of what-ifs of the past, the grief of the present, and the uncertainty of the future. De-fusing from the guilt and fear demons that come flapping in the night. It has become important to be mindful of the moment and to connect with what is present, here and now, in all its limitations as well as possibilities—and to find value in this. And...to remain open to a new identity as it emerges: advocate, activist, educator, supporter, and friend to the community of people with ME and their carers. The support my son needs, if he were well enough, would be very similar and would also include finding the window of energy tolerance, establishing a routine around this and developing values and an identity within his limitations. You may notice that none of this involves an assumption that if he just pushed a little harder he would be ok, or a focus on aberrant illness beliefs. We went there. It failed.
Late 2017 the face of ME/CFS was finally revealed. Unrest, a documentary by Jennifer Brea was released and subsequently avalanched with awards. Jenn (yeah it feels like she’s been in my living room), a person with ME wrote and directed Unrest, largely from her bed. It is brave, informative and heart-breaking. She collaborated with families, researchers and clinicians— including Ron Davis and his family—and provided a focus for people with ME and their advocates around the world. Unrest has been shown at thousands of public education events and is now being used as part of the curriculum at universities such as Harvard Medical School. A clear direction for advocacy and education has now been illuminated. Please watch Unrest. It is on Netflix and iTunes.
So... what of my son? I wrote a paragraph on how he is now, then decided that he would not want you to know the details. He remains severely disabled. The door to the room that confines him is firmly shut and has a thousand locks on it. In our hands, there are a thousand keys. Every 4 months or so, when we meet with his very caring physician, we get to choose whether we keep wriggling the current key in the current lock, or try a different combination. In the vacuum of knowledge about the real mechanisms that drive ME/CFS there is little to guide our choice. And so, we wait.
Thanks to my friends (NZcare4ME) who are along for the ride, and helped with references.
Centre for Disease Control. (2017). Myalgic encephalomyelitis/chronic fatigue syndrome. Retrieved from https://www.cdc.gov/me-cfs/index.html
Davis, R., Jonathan, C. W., Jason, L., Levin, B., Racaniello, V. R., & Reingold, A. (2015). An open letter to Dr Richard Horton and The Lancet. Virology Blog. Retrieved from http://www.virology.ws/2015/11/13/an-open-letter-to-dr-richard-horton-and-the-lancet/
Institute of Medicine. (2015) Beyond myalgic encephalomyelitis/chronic fatigue syndrome: Redefining an illness. Washington, DC: The National Academies Press.
Jelstad, J. (2016). The male pattern baldness disease? Chronic fatigue syndrome’s chronic lack of research funding. Retrieved from https://www.healthrising.org/blog/2016/05/04/chronic-lack-funds-chronic-fatigue-syndrome-mecfs-research/
Klimas, N. (2009). A virus linked to chronic fatigue syndrome. New York Times Laws, K. R. (2017). Distress signals: Does cognitive behavioural therapy reduce or increase distress in chronic fatigue syndrome/myalgic encephalomyelitis? Journal of Health Psychology, 22(9), 1177–1180. National
Institute for Health and Clinical Excellence. (2007). Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy): Diagnosis and management of CFS/ME in adults and children. Retrieved from https://www.nice.org.uk/guidance/cg53/evidence/full-guideline-pdf-196524109
National Institute for Health and Clinical Excellence. (2017). Surveillance report. Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy): diagnosis and management (2007) NICE guideline CG53. Retrieved from https://www.nice.org.uk/guidance/cg53/resources/surveillance-report-2017-chronic-fatigue-syndromemyalgic-encephalomyelitis-or-encephalopathy-diagnosis-and-management-2007-nice- guideline-cg53-4602203537/chapter/how-we-made-the-decision#how-we-made-the-decision
Shorter, E. (2015) Chronic fatigue in the context of the history of medicine. Retrieved from https://www.psychologytoday.com/us/blog/how-everyone-became-depressed/201502/chronic-fatigue-in-the-context-the-history-medicine
Stein, E. (2005). Chronic fatigue syndrome. Psychiatric Treatment Guidelines. Retrieved from https://anzmes.org.nz/
Thieme, K., Turk, D. C., & Flor, H. (2004). Comorbid depression and anxiety in fibromyalgia syndrome: relationship to somatic and psychosocial variables. Psychosomatic Medicine, 66, 837–844.
Tuller, D. (2015). Trial by error: The troubling case of the PACE chronic fatigue syndrome study. Retrieved from http://www.virology.ws/2015/10/22/trial-by-error-ii/
White, P. D., Goldsmith, K. A., Johnson, A. L., & Potts, L. (2011). Comparison of adaptive pacing therapy, cognitive behaviour therapy, graded exercise therapy, and specialist medical care for chronic fatigue syndrome (PACE): a randomised trial. The Lancet. 377(9768), 823–36.
Wilshire, C. R., Kindlon, T., Courtney, R., Matthees, A., Tuller, D., Geraghty, K., & Levin, B. (2018). Re-thinking the treatment of chronic fatigue syndrome—a reanalysis and evaluation of findings from a recent major trial of graded exercise and CBT. BMC, 6, 6. Retrieved from https://bmcpsychology.biomedcentral.com/articles/10.1186/s40359-018-0218-3#Bib1
Wilshire, C. E., & Ward, T. (2016). Psychogenic explanations of physical illness: time to examine the evidence. Perspectives on Psychological Science, 11(5), 606.