Tuesday, February 10, 2015

New name, new approach?


Viewpoint | February 10, 2015

Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome:  An IOM
Report on Redefining an Illness

Ellen Wright Clayton, MD, JD1

1Center for Biomedical Ethics and Society, Vanderbilt University,
Nashville, Tennessee
JAMA. Published online February 10, 2015. doi:10.1001/jama.2015.1346

Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a
multisystem and often long-lasting disorder, with manifestations that
can cause substantial morbidity and can severely impair patients'
health and well-being. It is estimated that between 836 000 and 2.5
million individuals are affected in the United States.1,2 Patients
with ME/CFS are typically unable to perform their normal activities,
and as many as one-fourth are homebound or bedridden, sometimes for
extended periods.3 As a result, the personal and social effects and
ramifications of this disease are enormous.

However, ME/CFS is poorly accepted and poorly understood, and the
characteristics necessary to make the diagnosis are contested.
Patients' concerns are often met with dismay and skepticism, if not
outright dismissal. Clinicians, in turn, are confronted by competing
definitions, which were usually developed for research and are quite
complex and difficult to implement in a busy clinical practice.
Patients who are fortunate enough and persistent enough to receive a
correct diagnosis frequently report long delays before their disorder
was identified. It is almost certainly the case that the majority of
affected patients are never diagnosed. This is unfortunate because
effective symptom management is often available, whereas the wrong interventions can make symptoms worse.

Making the diagnosis is essential for providing appropriate care. To
that end, the Department of Health and Human Services (HHS), together
with the National Institutes of Health, US Social Security
Administration, US Food and Drug Administration, Centers for Disease
Control and Prevention, and Agency for Healthcare Research and
Quality, tasked the Institute of Medicine (IOM) to develop
"evidence-based clinical diagnostic criteria for ME/CFS for use by
clinicians, using a consensus-building methodology," with input from
patients and clinicians; to "recommend whether new terminology for
ME/CFS should be adopted"; and to create plans for disseminating these
conclusions to clinicians. To meet its charge, the IOM committee heard
testimony from patients, clinicians, and researchers, carefully
reviewed almost one thousand public comments, and conducted a
comprehensive literature review. Currently available evidence
permitted the committee, in its newly released report,4 to identify
symptoms and findings that collectively identify the disorder for
which a new name was proposed, as discussed below. These criteria are
set forth in the accompanying Box.

 IOM Diagnostic Criteria for Systemic Exertion Intolerance Disease

Diagnosis requires that the patient have the following 3 symptoms:

1. A substantial reduction or impairment in the ability to engage in
preillness levels of occupational, educational, social, or personal
activities that persists for more than 6 months and is accompanied by
fatigue, which is often profound, is of new or definite onset (not
lifelong), is not the result of ongoing excessive exertion, and is not
substantially alleviated by rest AND

2. Postexertional malaisea AND

3. Unrefreshing sleep

At least 1 of the 2 following manifestations is also required:

1. Cognitive impairmenta OR

2. Orthostatic intolerance

Frequency and severity of symptoms should be assessed. The diagnosis
of systemic exertion intolerance disease (myalgic
encephalomyelitis/chronic fatigue syndrome) should be questioned if
patients do not have these symptoms at least half of the time with
moderate, substantial, or severe intensity.

To assist with diagnosis, the committee developed an algorithm
incorporating these criteria as well as an extensive list of findings
on history, physical examination, and, when needed to clarify
symptoms, tests that support the diagnosis. Further details, including
recommended checklists, are contained in the committee's report.4 The
committee called on HHS to develop focused, evidence-based tools that
will help clinicians make this diagnosis efficiently. Patients with
ME/CFS, like many other patients, often have comorbid conditions that
clinicians also must be prepared to identify and treat.

The committee concluded that a new name—systemic exertion intolerance
disease—was warranted to capture the essence of this disorder until
causation and pathophysiology are better delineated by research.
Although patients differ in their triggers and manifestations, the
salient feature of this disorder is that any kind of
exertion—physical, cognitive, emotional—can adversely affect these
patients in many aspects of their biological function and in their
lives, frequently severely and often for a prolonged period. "Myalgic
encephalomyelitis," for which many people who provided input to the
IOM study through testimony and public comments advocated, simply does
not describe this disorder. "Chronic fatigue syndrome" fails to depict
the complexity of this disease and is also unacceptable to many
patients and their advocates, who reported that this term leads
clinicians and others to belittle or even dismiss their disease. The
new name, which should be accompanied by a new International
Statistical Classification of Diseases and Related Health Problems,
Tenth Revision (ICD-10) code, also distinguishes this definition from
previous ones. Thus, patients who meet these new criteria should be
diagnosed with systemic exertion intolerance disease even if they also
meet criteria for earlier variants of ME/CFS.

Patients will not be identified and treated if clinicians do not know
about the disorder and how to diagnose it. To that end, the IOM report
sets forth a variety of strategies that must be pursued to facilitate
dissemination of these recommendations to clinicians, patients, and
K-12 education professionals, such as school nurses and psychologists.
The IOM is creating educational materials for patients and clinicians.

Although the committee was able to make high-quality recommendations
based on the available evidence, the need for more research is clear.
Most of the research that has been conducted to date has compared
small numbers of affected patients with healthy controls, not with
patients with other complex fatiguing disorders. This fact, coupled
with years of limited funding, means that despite the dedicated
efforts of researchers in this field, many questions remain
unanswered. More needs to be learned about what causes this disorder,
what factors affect its course, and what therapies work for which
patients. New knowledge may make it possible to identify subsets of
systemic exertion intolerance disease and to provide the foundation
for better diagnosis and treatment.

The IOM's new report4 provides the foundation for greater action to diagnose and treat patients with systemic exertion intolerance disease and to learn more about the disease that affects them. This opportunity must be seized. These patients, who for too long have
received inadequate attention in research and clinical settings, deserve better.


Corresponding Author: Ellen Wright Clayton, MD, JD, Craig-Weaver
Professor of Pediatrics, Professor of Law, and Co-Founder, Center for
Biomedical Ethics and Society, Vanderbilt University, 2525 W End Ave,
Ste 400, Nashville, TN 37203 (ellen.clayton@vanderbilt.edu).

Published Online: February 10, 2015. doi:10.1001/jama.2015.1346.

Conflict of Interest Disclosures: The author has completed and
submitted the ICMJE Form for Disclosure of Potential Conflicts of
Interest. Dr Clayton reports receiving speakers' fees from Novartis
for a talk on research ethics in genomics.

Funding/Support: The IOM report described in this Viewpoint was
sponsored by the Office on Women's Health within the US Department of
Health and Human Services, National Institutes of Health, Centers for
Disease Control and Prevention, US Food and Drug Administration,
Agency for Healthcare Research and Quality, and US Social Security

Additional Contributions: Members of the IOM Committee on Diagnostic
Criteria for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome, which
wrote the report described in this Viewpoint, were Ellen Wright
Clayton (chair), Margarita Alegría, Lucinda Bateman, Lily Chu, Charles
S. Cleeland, Ronald Davis, Betty Diamond, Theodore Ganiats, Betsy
Keller, Nancy Klimas, A. Martin Lerner, Cynthia Mulrow, Benjamin
Natelson, Peter Rowe, and Michael Shelanski. Carmen C. Mundaca-Shah
was the study director.


1. Jason  LA, Torres-Harding  SR, Njoku  MGC. The face of CFS in the
U.S. CFIDS Chronicle. 2005-2006:16-21.
2. Jason  LA, Richman  JA, Rademaker  AW,  et al.  A community-based
study of chronic fatigue syndrome. Arch Intern Med.
3. Marshall  R, Paul  L, Wood  L.  The search for pain relief in
people with chronic fatigue syndrome: a descriptive study. Physiother
Theory Pract. 2011;27(5):373-383.
4. IOM (Institute of Medicine). Beyond Myalgic
Encephalomyelitis/Chronic Fatigue Syndrome: Redefining an Illness.
Washington, DC: The National Academies Press; 2015.

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