Myalgic Encephalomyelitis / Chronic Fatigue Syndrome:
Clinical Working Case Definition,
Diagnostic and Treatment Protocols
A Consensus Document
David S. Bell, MD, FAAP
Past Chairman: Chronic Fatigue Syndrome Advisory Committee,
US Department of Health and Human Services
Myalgic Encephalomyelitis / Chronic Fatigue Syndrome is a complex illness that can cause life-long disability, yet has languished for years without clear recognition by the medical community. The paradox of the illness is that it can cause severe discomfort and markedly limit daily activity, yet persons with the illness may look, to the casual observer, relatively well. Because of this paradox, many in the medical profession have ignored the seriousness of ME/CFS.
In the past few years, science has made extraordinary strides in understanding the basic mechanisms of ME/CFS. Yet, because of its complexity, little of this science has reached medical practitioners to be used in relieving the suffering of patients affected with the illness. It is now possible to define abnormalities in the neurological, immune, autonomic, and neuroendocrine systems in a concise way that can paint a portrait of this disabling illness. The Canadian consensus definition of ME/CFS is a concise summary of these advances and permits a clear diagnosis for patients. The Canadian Consensus Document should be read and studied by every medical provider.
Charles W. Lapp, MD
Director: HUNTER-HOPKINS CENTER, Charlotte, North Carolina
Chronic Fatigue Syndrome Advisory Committee:
US Department of Health & Human Services
Board of Directors: International Association for Chronic Fatigue Syndrome
While the primary goal was to establish a clinical case definition for ME/CFS, the ME/CFS Consensus Document is a comprehensive overview of ME/CFS, including pathophysiology, symptoms, physical findings, and treatments. This Consensus Document is clearly the most comprehensive review of ME/CFS to date. It records the experience of many long time practitioners, which provides an insight into signs and symptoms that has never been recorded elsewhere.
Never before has there been a consensus on treatment. This paper considers not only pharmacotherapy, but also makes recommendations for patient education, energy conservation, pacing, stress reduction techniques, diet, and exercise. One of the most important aspects of the ME/CFS Consensus Document is that it indicates the level of proof for various recommendations.
This is THE MANUAL for diagnosing and treating ME/CFS. Perhaps every office that treats patients with ME/CFS should be using this document as the diagnosis and treatment blueprint.
Leonard A. Jason, Ph D
Director: Center for Community Research, DePaul University, Chicago IL
Board of Directors: International Association for Chronic Fatigue Syndrome
Our comparison study examined differences between patients meeting the Canadian clinical and the Fukuda et al. criteria for ME/CFS, with people who had chronically fatiguing illness explained by a psychiatric condition. The Canadian Clinical Criteria selected patients with more physical functional impairment, more fatigue/weakness, neurocognitive and neurological symptoms and had more variables that significantly differentiated them from the psychiatric comparison group than did the Fukuda et al. criteria. The findings do suggest that the Canadian criteria point to the potential utility in designating post-exertional malaise and fatigue, sleep dysfunction, pain, clinical neurocognitive, and clinical autonomic/ neuroimmunoendocrine symptoms as major criteria.
The selection of diagnostic signs and symptoms has major implications for which individuals are diagnosed with ME/CFS and how seriously the illness is viewed by health care providers, disability insurers, rehabilitation planners, and patients and their families and friends. I hope the results of this comparison study will encourage more physicians to use the Canadian clinical criteria.
Lynn Marshall, MD, FAAEM, FRSM
Medical Director: Environmental Health Clinic,
Sunnybrook & Women's College Health Sciences Centre
Member: Environmental Health Committee, Ontario College of Family Physicians
Lecturer: University of Toronto Department of Family and Community Medicine
Assistant Professor of Family Medicine: Northern Ontario School of Medicine
In my opinion, and in the opinions of the other doctors at the Environmental Health Clinic, the ME/CFS Consensus Document is extremely practical and useful. We have used it repeatedly in helping to develop comprehensive individual treatment plans in collaboration with patients. At the behest of the Ontario College of Family Physicians' (OCFP) Environmental Health Committee, and with approval of the publisher, the consensus diagnostic checklists were posted on the OCFP website. We also use the diagnostic criteria, checklists, and treatment suggestions as teaching tools in the OCFP's Environmental Health Day at their Annual Scientific Assembly.
Michael Barratt, MBBS, FRCPA
Medical Advisor: Alison Hunter Memorial Foundation
Australia
(Excerpt from a review of the Adelaide Forum, Australia, 2005)
The Canadian Clinical Case Definition has brilliantly rewritten the guidelines to capture, at last, what ME/CFS is really all about. It is not that patients are fatigued. Healthy people get fatigued. Rather the definition specifically selects patients who worsen with exercise. This takes the emphasis away from the subjective sensation of “fatigue” and forces one to clearly describe the connection between fatigue and activity. This also embraces mental fatigue (loss of cognitive function and alertness) as well as physical fatigue (lack of energy and strength, often felt in the muscles). The patient must become symptomatically ill after exercise and must also have evidence of neurocognitive, neuroendocrine, dysautonomic (e.g. orthostatic intolerance), and immune malfunction.
The Adelaide Forum agreed to unanimously embrace the Canadian Case Definition with a strong recommendation that it also be taken up by ME/CFS societies.
Richard Schloeffel, MBBS, FRACGP
General Practitioner
Sydney, NSW, Australia
The unanimous acceptance of the Canadian ME/CFS Guidelines will revolutionise the management of ME/CFS in the future, and its adoption by all Australian physicians is strongly recommended.
Terry Mitchell, MA, MD, FRCPath
Consultant Haematologist
Head: Norfolk and Suffolk ME/CFS Service
NHS Clinical Champion for ME/CFS in Norfolk, Suffolk and Cambridgeshire
Honorary Consultant: The National ME Centre
United Kingdom
Professor L J Findley TD KLJ MD FRCP FACP
Consultant Neurologist
Clinical Director: The National ME Centre
Clinical Director: Fatigue Service, Barking, Havering & Redbridge NHS Trust
United Kingdom
Myalgic Encephalomyelitis/Chronic Fatigue Syndrome is a common illness. Its impact on many sufferers can be profound with intrusive fatigue and multiple symptoms. The secondary burden of the condition is common to all chronic illnesses and includes impoverishment and a significant impact on personal and family life. We recommend and endorse the Canadian Consensus Document. We regard it as an extremely important contribution to understanding the physical basis of the condition. Future research should be directed to further defining the pathophysiology of the condition together with identifying the sub groups, which undoubtedly exist, within the illness complex currently termed ME/CFS.
Dr. Malcolm Hooper
Emeritus Professor of Medicinal Chemistry
Department of Life Sciences
University of Sunderland
United Kingdom
This is a VITAL DOCUMENT that gives a new focus and new direction to all involved with ME-CFS. It makes available the clinical experience and understanding of physicians who are pre-eminent in the field and encapsulates thousands of hours of clinical investigations that are important to sufferers from ME/CFS and all who are concerned for their care, support, and the understanding of this multi-faceted organic illness.
It offers-
HOPE - for patients whose multiple symptoms have so often been dismissed as psychiatric or of biopsychosocial origin with consequent loss of benefits and support when they are most needed.
CLARITY - for physicians by providing an abundance of clinical procedures and protocols that provide objective evidence of organic multi-system and multi-organ disorders associated with the neuroendocrine and immune systems. It is in agreement with the long established international classification of ME/CFS as a neurological disorder, ICD-10 G.93.3.
DIRECTION - for clinical treatments and research programmes especially the most recent ones concerning the need for sub-types in addressing ME/CFS and the deeper understanding of, changes in gene expression, mitochondrial dysfunction, and of pathological changes in the endothelium with concomitant vascular damage. Mitochondrial dysfunction offers an explanation of the debilitating fatigue that is one of the defining features of ME/CFS and is consistent with chronic heart failure recently described in a cohort of ME/CFS patients.
UNDERSTANDING - of the complexity and perplexity of ME/CFS as a multi-symptom, multi-organ and multi-system illness that is increasingly recognised as an archetype of other related illnesses such as Gulf War Syndrome, multiple chemical sensitivity (MCS), and Fibromyalgia syndrome (FMS).
Michael Barratt MBBS FRCPA
Medical Adviser, Alison Hunter Memorial Foundation
The Adelaide Forum, June 2005
Final Address (posted with permission from Dr. Barratt)
Where to now?
In the early years of ME/CFS societies in Australia our motto seems to have been, "We don't know where we/re going, but we are on our way".
After the Adelaide Forum, for the first time I believe, we are able to say, "Don’t give up now, big advances coming, finally!"
Various attempts to define Chronic Fatigue Syndrome have floundered on the vagueness of the word "fatigue". Everybody gets fatigue, physically and mentally, from time to time. It is found as well in "serious illnesses", including heart disease and major depression. Rather than making "fatigue" the main compulsory symptom, in a new attempt at a "case definition" of ME/CFS, the Canadian Clinical Case definition, 2003 has brilliantly rewritten the guidelines to capture, at last, what ME/CFS is really all about. It is not that patients are fatigued. Healthy people get fatigued. Rather the definition specifically selects patients who worsen with exercise. This takes the emphasis away from the subjective sensation of "fatigue" and forces one to clearly describe the connection between fatigue and activity. This also embraces mental fatigue (loss of cognitive function and alertness) as well as physical fatigue (lack of energy and strength, often felt in the muscles). The patient must become symptomatically ill after exercise and must also have evidence of neurocognitive, neuroendocrine, dysautonomic (e.g. orthostatic intolerance) and immune malfunction.
The Adelaide Forum agreed to unanimously embrace the Canadian case definition with a strong recommendation that it also be taken up by ME/CFS societies.
The obfuscation, deliberate I believe, of the previous case definitions resulted in the lumping together of numerous diseases that have little or nothing to do with each, apart from exhibiting "fatigue" to some degree. This has been corrected. The Canadian definition states that "patients become worse after exercise rather than better". There are pathologically slow recovery periods – usually 24 hours or longer.
Professor Kenny De Meirleir, who incidentally helped to frame the Canadian definition, gave a wonderful exposition summarizing the research enshrined in over 5000 scientific papers. His own immense contribution and modesty were inspirational. Briefly, he and others have elucidated a whole battery of biochemical tests that reflect the great complexity of CFS. Settling for the moment on a panel of five or six basic tests, mostly unavailable in Australia for the moment, he is able to separate ME/CFS patients into three separate groups, each with different causes, therapies and (sometimes) outcomes. His clinic in Brussels sees 800 CFS patients every three months.
Interestingly, given the biochemical results of these tests, without seeing the patient he is now able to predict into which group the patient falls, correctly in 95% of cases. This would be impossible if ME/CFS is a psychiatric illness. It is also highly significant that these tests can discriminate between ME/CFS and normal or non-CFS patients.
He told us of an encouraging story of a girl who was bed-bound from the age of 12 to 19 with ME/CFS. She is now running European marathons.
The take home message of the forum is, "do not give up, and hang on. The greyness is not that of evening before the dark night, it is the greyness of the dawn before a bright new day of hope for the sufferers of this hideous disease".
There was also discussion about encouraging laboratories of excellence to make readily available some or all of these tests so that the exciting work done in Belgium, Spain, Japan, Canada and the USA will soon be available in Australia.
Dr. Ellie Stein, MD FRCP(C)
Alberta, Canada
"I have now received all 6 copies that I ordered of the guidelines (ordered extra so I can educate my colleagues). I am very pleased with the paper, it really validates much of what I believe and have read and they avoided sliding down the CBT/GET slope. Very impressive. I have already quoted Appendix 12 (my personal favorite) in one letter for a patient. "
Angela Kennedy, RGN, MA
One Click Group, United Kindom
"The ME/CFS community in Britain found the Clinical Working Case Definition, Diagnostic and Treatment Potocols developed by your team in March 2003 to be a huge step towards excellence in care for ME/CFS sufferers, and have been endeavouring to promote these protocols in the UK, where ever possible. So firstly I would like to than you and your team for this outstanding work."
This is an exerpt from a letter sent to Dr. Carruthers posted with the pemission of Mrs. Kennedy. She is a caregiver for a teenager with ME, teaches sociology at a number of universities in Britain, is a researcher in the social sciences and is working towards a Ph D.
Maryann Spurgin, PH.D
ME Society of America, U.S.A
"This case definition is a vast improvement over the 1994 CDC Fukuda criteria. The Fukuda criteria makes ‘fatigue’ a compulsory symptom but downplays and makes optional post-exertional sickness and other cardinal symptoms. Because of this, the Fukuda criteria has led to tremendous misunderstanding in research, clinical care, and treatment protocols by fostering the selection of patients with chronic fatigue and fostering the view that exercise is an appropriate therapeutic approach.
“In sharp contrast, the new Canadian clinical case definition specifically selects patients who worsen with exercise. The clinical definition makes it very clear and compulsory that in order to meet the diagnostic criteria, the patient must become symptomatically ill after exercise (this is termed ‘post-exertional malaise’), and must also have neurological, neurocognitive, neuroendocrine, dysautonomic (for example, orthostatic intolerance), and immune manifestations. That is, worsening with exercise, and many symptoms other than fatigue, must be present for a patient to meet the diagnostic criteria. This case definition will go a long way to help distinguish ME/CFS patients from chronic fatigue patients, depressed patients, and patients with other illnesses who do improve with exercise. The Canadian definition specifically states that patients ‘become worse after exercise rather than better.’ This counters the view being put forward by persons such as Dedra Buchwald and Simon Wessely who hold that the disease is a behavioral problem leading to deconditioning. Unfortunately, such work does not even give patients the now-respectable status of a psychiatric illness, but rather refers to vague terms like ‘perception’ and ‘interpretation of symptoms’. The new case definition, much of which is backed by research, is a strong counter statement to the view held by Buchwald and Wessely.
“We encourage patients and patient groups to urge acceptance of this case definition in government documents so that appropriate research into the pathophysiology of the disease can be implemented and inappropriate, possibly harmful treatments will not be fostered. This case definition strengthens the ability to select and diagnose ME/CFS by sharpening the definition. We hope that patients will promote this definition by taking it to their primary care physicians, donating copies to medical libraries, and contacting their local media.
“We would like to thank Lydia Neilson and her group, The National ME/FM Action Network, the physicians from Canada and the USA, and the Journal of Chronic Fatigue Syndrome, all of whom worked together to bring this case definition to fruition."
The Myalgic Encephalomyelitis Association of Ontario
"Congratulations to the National ME/FM Action Network for achieving this remarkable consensus document published in the Journal of Chronic Fatigue Syndrome Volume 11 (1) by Haworth Press, New York. A special thanks to Marj van de Sande for the tremendous amount of work and energy involved for her to coordinate the project. We can all hope that this publication will increase having our voices heard on the severity of the symptoms of our illness, bring clarity and understanding of ME/CFS and stimulate more research. Thank you Lydia for having the fortitude to keep focused on achieving the completion of this project. Congratulations again and job well done!!"
Hugh Scher
Scher & De Angelis, Toronto
The clinical case definition, and diagnostic and treatment protocols reflect a culmination of modern research and clinical experience. It would be expected that application of the principles set out in the clinical case definition and treatment protocols will serve to enhance clinicians’ understanding of this illness, and will assist patients in their efforts to cope with the impact of the disorder and its symptomatology. The consensus document will also serve to provide benchmarks for disability benefits adjudicators and rehabilitation providers in a manner consistent with sound research, clinical experience and sound medical practice. In time, it would be expected that this enhanced understanding of ME/CFS and its functional effects will lead to an enlightened clinical approach to ME/CFS with a view to improving patient function, enhancing quality of life and addressing the underlying medical symptomatology and root causes of the disorder.
One Click Group - Jane Bryant, United Kingdom
Comments: French