Positive Health Online

There are approximately 250,000 people diagnosed with Myalgic Encephalomyelitis (ME) in the UK. It is a disease that crosses the boundaries of all age groups, racial / ethnic groups, and socio-economic strata, and alarmingly 80% of those affected are women. It has been linked to fibromyalgia, and presents similar treatment challenges in that there is no known cure and no clear path to recovery. While experts around the globe agree that ME has a physical basis, when it comes to its name and the characterization of its symptoms, a lack of consensus that’s decades-long exists within the medical community at large.

A universally accepted name that accurately describes ME has unfortunately proven to be extremely difficult to nail down, which may be, in part, due to the lack of understanding of the root causes, and biological and physical mechanisms behind this disease. Virtually no other medical disorder has been referred to by so many different names, some of which include Chronic Fatigue Syndrome (CFS), Myalgic Encephalomyelitis, Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS), Chronic Fatigue and Immune Dysfunction Syndrome (CFIDS), Neurasthenia, Post Viral Fatigue Syndrome (PVFS), and most recently, Systemic Exertion Intolerance Disease (SEID). To further complicate matters, each name comes with a slightly different definition and criterion for diagnosis, adding to the frustration of both physicians and patients alike.

Chronic Fatigue Syndrome, or CFS, coined in 1988, is the most commonly used ‘label’ in the medical community in the United States, while ME is more commonly used in Europe and Canada. The ME community understandably shuns the label CFS - in my opinion, with good reason. The problem with the term CFS is that this name is non-specific, and the word “fatigue” trivializes the complex nature of the condition. It also fails to observe and acknowledge the wealth of research that points to this disease as one characterized by severe immunological and neurological dysfunction. For years, people suffering with ME/CFS have been battling a stigma - fighting claims that it is merely a manifestation of trauma or depression, or that it indicates a case of hypochondria. As a result, the burden of recovery more often than not falls back onto the patient and many find themselves battling years of misdiagnoses and unsuccessful treatments. The importance of getting the name and the diagnostic criterion right cannot be stressed enough. It will help physicians to make faster, more accurate diagnoses, and it will serve to empower patients in that they will no longer be stigmatized with a name that many say does almost as much damage as the symptoms themselves. Furthermore, with a universal name, definition, and set of diagnostic criteria, we may finally be able to address the severe underfunding that is currently crippling research efforts in the United States.

Last year in the US, the National Institutes of Health’s (NIH) Pathways To Prevention (P2P) program published a report titled Advancing the Research on Myalgic Encephalomyelitis/Chronic Fatigue Syndrome. Its goal was to take a close look at the state of current research, identify where the gaps were, and suggest future research needs. As part of the program, the Institute of Medicine (IOM), an independent, non-profit organization, responded to a request by The Department of Health and Human Services (HHS), the NIH, and several other agencies, and created a committee to examine the evidence base of ME/CFS. In February 2015, the IOM published a report, Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Redefining an Illness which proposed several positive changes, including a new name - Systemic Exertion Intolerance Disease (SEID) - and with that, a new set of diagnostic criteria.

One of the most important pieces of the IOM report was the critical acknowledgment that this is a complex, multi-system disease, and a medical rather than psychiatric condition, that presents devastating consequences for many of its victims. While we don’t know exactly what causes ME, the most recent evidence points to an underlying problem with the functioning of the immune system, as well as some degree of neurological disorder. The World Health Organization (WHO) regards this as a neurological disease, which in reality is neither fish nor fowl, because it impacts multiple body systems - neurological, cardiovascular, immunological, gastrointestinal, musculoskeletal, sleep/wake - meaning that it does not neatly fit into one singular system or specialty. The International Consensus Criteria (ICC) defines ME as an immunological mediated neuro-inflammatory disease with multi-system manifestations, supporting the idea that there is an inflammatory process in the brain, instigated and maintained by the immune system. This neuro-inflammation can account for the majority of symptoms of the disease, but there is also clearly an inflammatory process occurring throughout the body at the same time.

Because of concerns around the current stigma associated with the term “Chronic Fatigue Syndrome” and because of the subsequent failure by a large segment of the medical community to acknowledge that people suffering with this condition are dealing with a real disease, the IOM proposed a new name - Systemic Exertion Intolerance Disease (SEID). While a name change is necessary, SEID is still not appropriately descriptive in that it does not speak to the underlying complex pathophysiology of the disease, and remains descriptive only of what is now regarded as the hallmark symptom - post exertion malaise. “Systemic” acknowledges that this is a disease that affects multiple body systems and “exertion intolerance” names what the IOM is defining as the central feature of the disease. The IOM’s objection to using the name Myalgic Encephalomyelitis (ME) centered on the fact that not everyone suffers with muscle pain. However, while not ideal, the name ME does come closest to focusing on the pathophysiology of the condition, despite the fact that only about 60% of the patients will have significant muscle pain as part of their presentation. ME also has an international community of experts (WHO & ICC) who are involved in the research and treatment of this disease.

The IOM report stated that diagnosis requires the following symptoms:

• A substantial reduction or impairment in the ability to engage in pre-illness levels of occupational, educational, social or personal activities that persists for more than six 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;
• Post-exertional malaise; and
• Unrefreshing sleep;
• At least one of the two following manifestations is also required: cognitive impairment or orthostatic intolerance.

While the IOM successfully defined the criteria for diagnosis, one of my biggest concerns is the mandatory waiting period of 6 months before a diagnosis can be made. This delay subjects patients to unnecessary suffering, while also denying researchers access to study patients in the earliest stages of the disease. This is especially burdensome in the pediatric population where 6 months of an 11 y/o’s life can feel like an eternity for the child and parents. Insisting that a person suffering with this condition wait 6 months for a diagnosis, especially if they have no other infectious or disease process, shows an indifference to the patient’s need for urgent care. The IOM acknowledges that nearly a quarter of the approximately 1 million people in the US with this disease are housebound or bedridden; to delay their opportunity to seek specialty help is not only imposing unnecessary suffering, it’s negligent. My hope is that we will follow the ICC’s example and remove any limit on the length of time someone needs to be suffering with these symptoms before getting a diagnosis.

From my research and clinical experience I believe that the development of ME is a consequence of combined genetic susceptibility and multiple unresolved assaults on the body. This is a neuro-inflammatory disease, and must be treated as such. Once our immune system has been triggered by any number of infectious or environmental triggers [such as Epstein Barr virus, Enterovirus, physical assaults, exposure to pollutants (e.g. mould), and/or heavy metal toxicity] it causes inflammation in the brain and throughout the body.

Microglia, the innate immune cells in the brain and central nervous system, are the key to understanding this inflammatory process. They become active in response to any number of stresses on the body, secreting inflammatory chemicals that, when properly regulated, allow for harmful bacteria to be killed, and/or for healing to occur from an injury. Once the stress is resolved, the microglia typically return to a resting state until they are needed again. However, if too many stresses or assaults occur, the microglia can become hyper-reactive, which can trigger a system-wide inflammation that can be difficult to stop, often presenting symptoms such as fatigue, brain fog, sleep disorder, dysregulation of the cardiovascular system, muscular pain, and/or gastrointestinal disturbance. What’s worse, the very dysfunctions that can result from inflammation, such as sleep disturbances and gastrointestinal problems, can make the inflammation worse, presenting a vicious circle of chronic illness and misery.

In order to effectively treat this disease, the medical community must look for all potential sources of inflammation present in the body, such as toxins and infections. Once found, the next step is to eliminate them entirely, as it is only when we are able to fully calm the inflammatory process that we can nudge the microglia to down-regulate, and ultimately return to their normal resting state. Physicians also need to find a way to help quiet and reset the immune system itself because once it has been ‘turned on’ too many times, it can remain in this hyper-reactive state, continuing to produce inflammatory reactions even when the initial triggers are gone. A number of medications and supplements have been found to be quite effective in ‘quieting’ the immune system and the inflammation in the brain. A low-inflammatory diet is highly recommended. Meditation has also been proven to be highly beneficial in helping the brain to repair, as has exercise, though for some people suffering with ME, exercise can exacerbate their condition requiring their activity levels to be regulated carefully. I cannot emphasize enough, however, that treatment needs to be both individualized and comprehensive.

The reality is that while we have been able to help a number of people, there is still much we have to learn about this disease. Far too often the solutions are elusive, and the treatment evaluation cycles long and arduous. We desperately need the commitment of research dollars commensurate with the extent and severity of this disease to further its study and treatment. To give one a perspective on the current lack of research funding, in the United States, multiple sclerosis, a devastating disease that affects less than half the estimated number of people suffering from ME/CFS/SEID, will receive approximately $100 million in NIH research dollars this year, while ME/CFS/SEID will receive a mere $5 million. With the advent of new diagnostics and treatment approaches, a cure for ME is within reach IF we can commit to devoting the resources necessary to conduct research and do clinical studies on promising new treatments. The reports from the NIH P2P and the IOM have acknowledged the extent and severity of this disease, and this is indeed promising, but we need to see a drastic increase in research funds to assist in the creation of centers to study and treat this disease, and to further educate the next generation of physicians and researchers.