This 3 minute film will give you an introduction to PEM (Post Exertional Malaise). Once you’ve understood what PEM is about, you’ll know a lot more about the debilitating chronic disease ME (Myalgic Encephalomyelitis). Published 2 April 2020
The film is made by the Norwegian ME Association (Rogaland County) with professional support from psychologist Ketil Jakobsen and paediatric neurologist Kristian Sommerfelt.
‘Fatigue is not the same as ME’
‘PEM is a long term worsening of symptoms that can be triggered by a relatively small exertion’
‘It is a common belief that everyone benefits from activity and effort, but this is not necessarily the case for patients with ME.’
‘The most important thing is to genuinely listen and believe what the patient is saying.’
ME/CFS patients more often get infected with COVID-19 than other people?
people with ME/CFS have other and/or more or more severe symptoms than others?
Internist Dr. Joris Vernooij and Dr. Rogier Louwen of Erasmus University, Rotterdam in the Netherlands, have developed an online scientific research project in which ME/CFS patients with and without COVID-19 and a control group of people around the patients are urged to participate.
The initiators of the project and all those involved in the research work are working completely pro bono. Therefore, no costs will be incurred by participants.
It will involve short weekly online surveys. You will need to register with Erasmus University and will need a mobile phone to receive a passcode.
In this paper we summarize the evidence regarding the role of autoimmunity in these four syndromes with respect to immunogenetics, autoimmune co-morbidities, alteration in immune cell subsets, production of autoantibodies and presentation in animal models.
These syndromes could be incorporated in a new concept of autoimmune neurosensory dysautonomia with the common denominators of autoantibodies against G-protein coupled receptors and small fiber neuropathy. Sjogren’s syndrome, which is a classical autoimmune disease, could serve as a disease model, illustrating the concept.
Development of this concept aims to identify an apparently autoimmune subgroup of the disputable disorders, addressed in the review, which may most benefit from the immunotherapy.
The etiology and pathogenesis of Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) are unknown, and autoimmunity is one of many proposed underlying mechanisms. Human Leukocyte Antigen (HLA) associations are hallmarks of autoimmune disease, and have not been thoroughly investigated in a large ME/CFS patient cohort.
We performed high resolution HLA -A, -B, -C, -DRB1, -DQB1 and -DPB1 genotyping by next generation sequencing in 426 adult, Norwegian ME/CFS patients, diagnosed according to the Canadian Consensus Criteria. HLA associations were assessed by comparing to 4511 healthy and ethnically matched controls. Clinical information was collected through questionnaires completed by patients or relatives.
We discovered two independent HLA associations, tagged by the alleles HLA-C*07:04 (OR 2.1 [95% CI 1.4–3.1]) and HLA-DQB1*03:03 (OR 1.5 [95% CI 1.1–2.0]). These alleles were carried by 7.7% and 12.7% of ME/CFS patients, respectively. The proportion of individuals carrying one or both of these alleles was 19.2% in the patient group and 12.2% in the control group (OR 1.7 [95% CI 1.3–2.2], pnc = 0.00003).
ME/CFS is a complex disease, potentially with a substantial heterogeneity. We report novel HLA associations pointing toward the involvement of the immune system in ME/CFS pathogenesis.
The Bateman Horne Center works for the medical advancement and treatment of Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome (ME/CFS)and Fibromyalgia in Salt Lake City, Utah, USA.
In addition to the resources for patients and doctors on ME/CFS they have produced some briefing papers on Covid-19 and ME/CFS, many of which are helpful people from anywhere in the world.
Dr Suzanne Vernon
Medical Director: Dr Lucinda Bateman
Researcher: Dr Suzanne Vernon
Medical Considerations Letter— In the event you become acutely ill, the Medical Care Considerations Letter serves as a guiding resource for outside medical care intervention. The intent of this letter is to provide care professionals with further information about your illness of ME/CFS and/or severe FM to assist them with their medical intervention decisions.
“you should assume they have a serious, chronic, multisystem illness that may negatively impact their prognosis”
“Based on current evidence the underlying pathology of ME/CFS involves energy metabolism, the nervous system, and the immune system.”
e.g. Are people with ME/CFS/FM at a higher risk than the general public of dying from COVID-19 if contracted? … The immune dysregulation of ME/CFS/FM may reduce ability to fight viral infections. Additionally, the presence of chronic inflammation, allergies, asthma, mast-cell activation may pose additional risks.
Advance Care Planning: The Advance Care Planning document provides guidance for establishing advanced-care-directives and POLST documents.
Personal Guidance and Decision Making
e.g. Advance Directives: Everyone, not just those with ME/CFS, should document end-of-life wishes on paper regarding aggressive medical care in a life-threatening situation. Discuss with family and medical professionals as needed.
Background:
Immune responses targeting microbes can contribute to chronic inflammation, particularly when the microbes are persistent such as herpes-family Cytomegalovirus (CMV) and EpsteinBarr virus (EBV). Such persistence of antigens can cause T cell exhaustion characterized by loss of appropriate effector functions, expression of inhibitory
receptors, as well as failure to return to homeostatic pre-inflammatory conditions. This results in immune senescence and dysregulation which may cause disrupted cell populations, homing and cytokine productions that mediate immunopathology and compromise anti-microbial defences.
Aims:
The aim of the study was to determine whether microbe specific particularly, interferon gamma immune responses measured in 2 disease states, where a herpes viral inflammatory aetiology and immune dysregulation are suggested, acute Myocardial Infarction (MI) with reperfusion and Chronic Fatigue Syndrome (CFS), are indicative of
disease presence and severity.
Patients: MI occurs due to blockage of the coronary artery, and treatment involves stent insertion, allowing reperfusion (R), which has associated immunopathology due to T cell homing. A total of 52 MI patients were studied. Blood samples were taken before and after reperfusion to investigate the dynamics of specific T cell homing to the heart, that may contribute to disease severity (reperfusion damage). For 50 CFS patients with measured disease levels, blood samples were taken to examine immune responses including those against microbes implicated in disease (CMV & EBV) compared to healthy controls.
Methods:
T cell immunity was measured by ELIspot and flow cytometry, and cytokine levels in cell culture supernatants were measured using multiplextechnology. Statistical analyses were carried out for normality in data sets. The Mann-Whitney test or unpaired t test was used to determine the difference between two unrelated groups. Differences between repeat or
paired measurements were determined by Wilcoxon signed rank tests or paired t tests. Associations between measurements were investigated using Spearman correlation.
Results and Discussion:
In MI patients, compared to before reperfusion, levels of the following cell populations fell significantly after reperfusion: terminally-differentiated CD8+ PD-1+ effector memory cells (p=0.016) and CMV-specific IFNg-secreting CD4+ T cells (p=0.002) the latter also being associated with injury. This suggests specific pathogenic T cell homing to the heart during reperfusion. In CFS patients, increased disease severity was associated with increased non-specific IFNy production (p=0.008), and reduced percentage of NK cells (p=0.0047). NK cell deficiency may reduce antiviral defences and allow detrimental viral
reactivation.
Conclusion:
T cell responses against CMV appeared to have greater involvement in MI + R than CFS. Immune responses involving IFN-gamma are demonstrated in both conditions as being associated with disease, and so this cytokine may be considered a disease biomarker and a therapeutic target for both.
Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS): significant negative impact of Quality of Life of both patients and family members, by Esme Brittain
During my 3rd year at Cardiff medical school, I conducted a 6-week research project looking at the impact of ME/CFS on both patients’ and their family members’ quality of life (QoL).
Background:
Our study was the first to look at the impact of ME/CFS on adult patients’ quality of life and that of their family members using validated questionnaires. Some studies have explored ME/CFS in paediatric patients and the impact on their mothers. They found that mothers experienced loss of monthly income and a marked impact on psychological health.
In another study siblings were asked to complete questionnaires and 9 siblings partook in a semi-structured interview. In the interviews, all siblings talked about restrictions on family life e.g. less holidays, don’t go out as a family as much. Some of the siblings spoke about the social stigma around ME/CF and how they had decided not to tell some of their friends about it because they may not understand. Emotional impact was also commonly talked about.
Methodology:
WAMES kindly posted information about our study on their website and social media pages. 39 patients/carers got in touch and wanted to get involved with the study, which was brilliant. We sent out a questionnaire to each patient (WHOQOL-BREF: World Health Organisation Quality of Life – Abbreviated version) and 4 questionnaires for their family members (FROM-16 – Family Reported Outcome Measures).
We had a 74% questionnaire response rate – this enabled us to achieve a robust and beneficial study.
Results:
Patients – WHOQOL-BREF
Patients scored very low in the physical health section, indicating this had a greater impact on their QoL.
There was a strong link between how the patients reported their QoL and how satisfied they were with their health i.e. the lower their health satisfaction, the poorer their QoL
Family Members – FROM-16
Every family member reported some degree of impact on their QoL. Our average score for ME/CFS was much higher (=poorer QoL) than studies looking at other diseases such as cancer
There was a strong link between the patients’ QoL and that of their family members i.e. the poorer the patients’ QoL, the greater the impact on their family members’ QoL
Conclusion: Our study has shown ME/CFS has a major impact on both patients’ and their family members’ quality of life. In fact studies of cancer patients and their families found their quality of life to be higher. Could this be because there is much more support from the NHS & charities for them? We hope clinicians will become more aware of this so that more appropriate support can be provided. Education and awareness are very important, but setting up support groups for family members could be a good start.
A larger-scale version of this study could be beneficial, as that could verify the findings and clarify the way forward for the NHS.
Miranda Fricker’s influential concept of epistemic injustice (Oxford University Press, Oxford, 2007) has recently seen application to many areas of interest, with an increasing body of healthcare research using the concept of epistemic injustice in order to develop both general frameworks and accounts of specific medical conditions and patient groups.
Testimonial injustice is unfairness related to trusting someone’s word. An injustice of this kind occurs when someone is ignored or not believed because they are a woman, because they are black, or broadly, because of their identity. Wikipedia
This paper illuminates tensions that arise between taking steps to protect against committing epistemic injustice in healthcare, and taking steps to understand the complexity of one’s predicament and treat it accordingly. Work on epistemic injustice is therefore at risk of obfuscating legitimate and potentially fruitful inquiry.
Hermeneutical injustice happens when someone’s experiences are not well understood — by themselves or by others — because these experiences do not fit any concepts known to them (or known to others), due to the historic exclusion of some groups of people from activities, such as scholarship and journalism, that shape which concepts become well known. Wikipedia
This paper uses Chronic Fatigue Syndrome/Myalgic Encephalomyelitis as a case study, but I suggest that the key problems identified could apply to other cases within healthcare, such as those classed as Medically Unexplained Illnesses, Functional Neurological Disorders and Psychiatric Disorders.
Future work on epistemic injustice in healthcare must recognise and attend to this tension to protect against unsatisfactory attempts to correct epistemic injustice.
Extract
Kidd and Carel argue that ill persons are especially vulnerable to testimonial injustice because there is often a presumptive attribution of certain characteristics to ill persons that negatively affects the perceived credibility of their testimony, such as cognitive unreliability and emotional instability. Accordingly, patient testimonies are often dismissed as irrelevant, confused, too emotional or time-consuming (pp. 529–30).
Where there is testimonial justice in healthcare, so they argue, patient testimonies would be recognised, actively sought out, and judged to be, at least in certain respects, epistemically authoritative (p. 532). The authors state that medical professionals have epistemic authority over some matters, but that the same applies to patients, yet the various structures of medical institutions are such that the epistemic authority of the patients is often not accommodated. Kidd and Carel acknowledge occasions whereby the medical professional would be right to exercise epistemic authority, but maintain that in such cases, the clinician can often be overly dismissive (p. 531).
They also argue that ill persons are especially vulnerable to hermeneutic injustice because experiences of illness are often difficult to understand and communicate due to inadequately developed or respected hermeneutic resources (p. 529). Where there is testimonial justice, the clinician will recognise that their failing to make sense of the patient’s experience is not due to any fault of the patient. An appropriately ‘just’ clinician, they suggest, might say ‘the fact that I don’t understand you isn’t your fault but mine … I am untrained in the kind of articulacy you are using, and this hermeneutical context does not provide me with those resources’ (p. 532).
Where there is hermeneutic injustice, then, testimony is not dismissed or disbelieved outright, but there exists a conceptual impoverishment. In which case, there is a lack of epistemic resources belonging to a particular institution, or a lack of epistemic resources being appropriately employed, which prevents the patient’s articulation of their illness-experience from being acknowledged and/or shaping clinical practice. Consequently, patient’s attempts to articulate their experience are often not adequately recognised by medical professionals. In this respect, the two injustices are closely linked and work to sustain one another.
Conclusion
I have highlighted two problems with how the concept of testimonial injustice might be applied to healthcare, using the example of CFS/ME. Although a useful concept which has already aptly shown how CFS/ME patients are vulnerable to suffering from epistemic harm, I have resisted claims that identify testimonial injustices where the epistemic problem can be just as plausibly explained by the medical professional exercising appropriate medical sensitivity within the context of a vast conceptual impoverishment. I have also argued that there is a problem with the idea of the CFS/ME patient as epistemically authoritative over their first-person lived experience, since their status as ‘knower’, of even their first-person lived experience, is highly complex.
I finished by suggesting that the process of gaining epistemic insight into CFS/ME ought to be deeply collaborative. Some researchers have suggested that, in psychiatry and medicine more broadly, there ought to be more collaboration between all ‘stakeholder groups’ (Fulford et al. 2014, p. 113). There are certainly promising avenues for further careful research into what the epistemic contributions of patients can reveal about CFS/ME. In the absence of a biomedical breakthrough, collaboration between patients, researchers, clinicians and medical professionals may prove to be the most effective way to enrichen the conceptual understanding of CFS/ME, empowering both patients and medical professionals.
Chronic fatigue syndrome (CFS) or myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a condition of unknown aetiology that commonly follows an infection. There are no known predictors for recovery or established treatments.
At the Royal Children’s Hospital (RCH) in Melbourne, Australia, the majority of young people with CFS are provided with symptom management and lifestyle guidance in an outpatient setting. However, for some, educational or social issues preclude progress and for those who request this assistance, since 2012, the Victorian Paediatric Rehabilitation Service has offered an Intensive Self-Management Program.
For this program, participants engage in both group and individual sessions, attending 3 days per week for 4 weeks in small groups of 3–4. Interdisciplinary input is from Occupational Therapy, Physiotherapy, Education and Psychology to assist with goal setting and strategies. Outcome measures are obtained at initial assessment, 6 weeks and 6 months post-program. Support is offered for 12 months post-program.
For both the outpatient program and the intensive program the outcomes and feedback from patient and family has influenced the approach and focus. This chapter outlines the current approach and how it has evolved over time.
Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a complex multisystem disease. Evidence for disturbed vascular regulation comes from various studies showing cerebral hypoperfusion and orthostatic intolerance. The peripheral endothelial dysfunction (ED) has not been sufficiently investigated in patients with ME/CFS. The aim of the present study was to examine peripheral endothelial function in patients with ME/CFS.
Methods and results:
Thirty‐five patients [median age 40 (range 18–70) years, mean body mass index 23.8 ± 4.2 kg/m2, 31% male] with ME/CFS were studied for peripheral endothelial function assessed by peripheral arterial tonometry (EndoPAT2000). Clinical diagnosis of ME/CFS was based on Canadian Criteria. Nine of these patients with elevated antibodies against β2‐adrenergic receptor underwent immunoadsorption and endothelial function was measured at baseline and 3, 6, and 12 months follow‐up.
ED was defined by reactive hyperaemia index ≤1.81. Twenty healthy subjects of similar age and body mass index were used as a control group. Peripheral ED was found in 18 of 35 patients (51%) with ME/CFS and in 4 healthy subjects (20%, P < 0.05). Patients with ED, in contrast to patients with normal endothelial function, reported more severe disease according to Bell score (31 ± 12 vs. 40 ± 16, P = 0.04), as well as more severe fatigue‐related symptoms (8.62 ± 0.87 vs. 7.75 ± 1.40, P = 0.04) including a higher demand for breaks [9.0 (interquartile range 7.0–10.0) vs. 7.5 (interquartile range 6.0–9.25), P = 0.04]. Peripheral ED showed correlations with more severe immune‐associated symptoms (r = −0.41, P = 0.026), such as sore throat (r = −0.38, P = 0.038) and painful lymph nodes (r = −0.37, P = 0.042), as well as more severe disease according to Bell score (r = 0.41, P = 0.008) and symptom score (r = −0.59, P = 0.005).
There were no differences between the patient group with ED and the patient group with normal endothelial function regarding demographic, metabolic, and laboratory parameters. Further, there was no difference in soluble vascular cell adhesion molecule and soluble intercellular adhesion molecule levels. At baseline, peripheral ED was observed in six patients who underwent immunoadsorption. After 12 months, endothelial function had improved in five of these six patients (reactive hyperaemia index 1.58 ± 0.15 vs. 2.02 ± 0.46, P = 0.06).
Conclusions:
Peripheral ED is frequent in patients with ME/CFS and associated with disease severity and severity of immune symptoms. As ED is a risk factor for cardiovascular disease, it is important to elucidate if peripheral ED is associated with increased cardiovascular morbidity and mortality in ME/CFS.
