POST EXERTIONAL MALAISE (PEM)
PEM - THE DISTINGUISHING SYMPTOM OF ME/CFS
When symptoms resembling ME/CFS were first described in the late 1600s by Thomas Syndenham (muscular rheumatism) and documented as neurasthenia in the late 1800s, there was nothing in the literature to support a defining characteristic of the illness. However, in the mid-1950s, a doctor named Andrew “Melvin” Ramsey, an infectious disease specialist at the Royal Free Hospital in London, began studying outbreaks resembling polio that he and his colleagues called encephalomyelitis. As they continued to mull over the data and refine the characteristics in the late 1950s, they expanded the term to benign myalgic encephalomyelitis, and Ramsey discovered the characteristics of post-exertional malaise. He presented it as the distinguishing symptom of ME (after benign was dropped), and the research was included in the 1962 Diseases of the Nervous System 6th Edition and used by the World Health Organization to incorporate ME as a disease in 1969. The medical community largely ignored the WHO definition and Ramsey's work, deciding instead to listen to two psychiatrists who in 1970 stated ME was an illness of psychological origin. Understanding of ME and of post-exertional malaise was lost to history until the past 25 years when researchers finally started listening to the reports of patients and other tests have been conducted (please see HISTORY OF ME/CFS PAGE). A clearly distinguishing symptom post-exertional malaise (or PESE – post exertional symptom exacerbation) is the exacerbation of ME/CFS symptoms related to any kind of physical and/or mental activity that is undertaken by a person who exhibits signs of the illness. This is often described by the individual with ME/CFS as a “post-exertional crash”. The nature of PEM (PESE) has been documented as being quite variable, taking anywhere from 12 to 48 hours, the intensity varies with the intensity of the activities that precede it, the severity of the individual’s level of ME/CFS, the amount of time spent in that set of activities, and the threshold in which that individual exceeds their body's ability to produce energy. In the most severe form of PEM a person may be incapacitated, stuck in bed for several days or longer, struggling just to go to the toilet or feed themselves. Those who experience less severe PEM may still be able to accomplish at least some daily activities but struggle to maintain any previous semblance of normal functioning. Upon recovering, engaging in their previous range of normal activities will re-exacerbate the PEM via a push-crash cycle that will cause them to plunge back into PEM again. In other instances, the long-term PEM symptoms become the baseline. In the most severe cases the act of drinking water, turning over in bed, and the whispering of another person in the room can be enough to set off post-exertional malaise and maintain long-term PEM. Variabilities aside, sadly, the lack of education, understanding of what ME/CFS is, what it does to the body, and in particular the effects of the core symptom of PEM along with any guidance from clinicians may lead many people with ME/CFS to unknowingly engage in too much over-exertional activity on either a daily or cyclical basis, constantly putting themselves at the mercy of a push-crash cycle that compromises their already depleted and crippled functional baseline.
CAUSATION
It has been postulated that PEM is caused by a major disruption in the body’s ability to burn oxygen in the mitochondria to produce energy (known as aerobic respiration, oxidative phosphorylation, or aerobic energy production). Oxygen is the most efficient way for organisms to produce energy on earth and is the most vital component in the end stages of ATP production in the muscle cell. Without being able to properly use oxygen to burn glucose in the production of energy in the muscle cells, the muscles are forced to abandon aerobic energy processes and glean only what they can from the byproducts of glucose itself, known as anaerobic respiration. The point where the body shifts from this aerobic energy process to utilizing anaerobic energy is called the anaerobic threshold. Every organism on earth except for bacteria and viruses depends upon fully intact, efficient aerobic processes in order to function properly. In all of these organisms, there is a shift from aerobic to anaerobic energy production when the organism tires out and the products of energy production become used up. The organism then rests and replenishes those products so the body can go ahead and resume the activities and the aerobic process starts all over again when resuming those activities. The lower the anaerobic threshold, the lower the amount of aerobic energy the body can produce. Having an abnormally low anaerobic threshold is known as being aerobically impaired. This occurs with people who have severe metabolic conditions such as COPD, heart disease, or some other demonstrated physical process that interferes with the body’s ability to maintain proper energy for movement, usually due to improper oxygen delivery because the respiratory system or heart functioning is compromised. However, those with ME/CFS will be tested for these conditions and have none of them. They will also have been cleared of any and every other fatiguing condition that could possibly be diagnosed, including hormones, cancer, STDs, testosterone, vitamin deficiencies, rheumatological issues, Lyme’s disease, MS, MD, ALS, mental illness. Instead of a physical condition that can be detected by medical current medical testing, people with ME/CFS will experience a complete and total shutdown of normal aerobic processes within an unusual and abnormally quick time frame compared to all other individuals, even those with the above listed physical issues. They will then become stuck in anaerobic energy processes and be unable to replenish any vitally needed energy afterward due to the fact that the metabolic pathways that allow the chemical transport that uses oxygen in the mitochondria to complete the aerobic energy process is disrupted or nonexistent. The process of using aerobic respiration for energy production yields 34 to 36 ATP in the muscle in humans. Anaerobic respiration only yields 2 to 4 ATP. This is a thimbleful of the normal energy that the human body normally produces and requires. It is this not-so-well understood lack of normal energy production that leads to the incredible energy deficit experienced by people with ME/CFS and thus post exertional malaise. This inability of the body to not only produce but maintain proper energy for movement does not allow a person with ME/CFS to recover from even minimal physical and mental activities. This abnormally lowered aerobic energy threshold, that varies from individual to individual depending upon their illness level and is readily demonstrated by numerous two-day CPET (cardiopulmonary exercise stress testing) when compared to both healthy and even deconditioned controls is what leads to the extraordinary energy deficit. This deficit not only leads to an increase in profound exhaustion already existing, but also an increase in all the symptoms of ME/CFS as the body gets sicker due to not only the stress placed upon it by the demands of energy production it cannot possibly keep up with but also an explosion of other CNS and neuroinflammation processes that are associated with the condition. Thus, post exertional malaise (PEM) is also known as post exertional symptom exacerbation or PESE.
EFFECTS AND PACING
Due to the extraordinary demand it places on an already, ill, broken, and compromised system as - noted above - the effects of post-exertional malaise are destructive. The result of these metabolic disruptions and the subsequent impaired energy production, post-exertional malaise or PESE is what makes ME/CFS the devastating illness that it is. There is no other disease that is associated with this kind of hallmark symptom. It is completely distinguishable from every other kind of fatiguing illness or condition – cancer, autoimmune disease, heart disease and the like - because there is no other illness in which the body is unable to efficiently burn oxygen in the muscles. It is precisely this striking set of parameters that makes ME/CFS and the exertional intolerance that accompanies it, a devastating biological condition. In the most debilitating cases, the body has no ability to produce sustainable energy, causing that person to be bed-bound and living in darkness for 24 hours a day. In mild cases, a person may be able to recover from these post-exertional crashes in a daily fashion and may be capable of sustaining far more activity with considerably less symptoms and suffering. They may also be able to engage in a very light exercise program and even recover some prior stamina if they don't overdo it. This can fool researchers and even clinicians who have had those patients to believe that ME/CFS is a completely recoverable illness. However, this is not the experience of the typical person with ME/CFS. Most people with ME/CFS diagnosed or undiagnosed are, in the absence of other diseases, unable to carry out the normal tasks typical for your age and body type. Because of this profound inability of their bodies to produce and therefore recover normal energy, most people who have ME/CFS are not exhausted due to deconditioning and will not benefit from exercise. In fact, what is considered normally healthy cardiovascular exercise can plunge a person into long-term debilitating PEM from which they may never recover, due to the overwhelming intensity of the exertion damaging the body's already compromise energy processes. In fact, bouts of PEM from even non-cardiovascular or minimal exertional activity can damage the body system further by exacerbating the body's inability to efficiently produce aerobic energy. This can result in tremendous misunderstanding and hostility from treating clinicians, physical therapists, and medical establishments around the world who refuse to consider this paradigm. This is tragic because by using a technique called pacing, a form of energy management and conservation, borne out of the occupational therapy tradition, where the individual participates in activities as tolerated and does not regularly outperform their daily energy threshold, is extremely effective in minimizing PEM, reducing its duration, intensity, and severity, and therefore elevating quality of life for those with ME/CFS. This may take extra support from those who live with or near that person. In the most catastrophic cases, supportive and palliative care are necessary for the individual’s survival and well-being. In many other cases, proper pacing and energy conservation management can help a person with ME/CFS more readily maintain their own baseline functioning, rather than constantly fighting against and being submerged by a tide of out-of-control PEM.
The Workwell Foundation based in Ripon, California and the Bateman-Horne center in Salt Lake City, Utah have pioneered the use of the two-day CPET (cardiopulmonary exercise stress test) and has both measured and studied post-exertional malaise in ME/CFS patients who can tolerate the exertion, for disability purposes and scientific purposes over several years. They have published numerous papers and contributed to numerous studies of the phenomenon since 2007. This two-day CPET testing can be used to demonstrate post exertional malaise as a means of proving disability if the patient is confident they can tolerate it; However, very few medical providers are able to read the results of such a test with the analysis and understanding necessary to make the proper determinations. The exception would be a handful of the expert ME/CFS practitioners scattered across the far corners of the United States and the UK, including those at Workwell and Bateman-Horne. Also, insurance companies may not pay for such a procedure if it is indicated as a means of proving ME/CFS and so on top of the expense of a trip to a faraway expert, and a place to stay to rest during and after, the patient may very likely pay end up paying out of pocket for the actual procedure itself. Below you will find a series of videos and a comprehensive study from the Workwell Foundation that demonstrates the existence of PEM, what is behind it, how it distinguishes itself from tiredness or fatigue after exercise in other types of major medical illnesses, and the debilitating effects it has on people specifically with ME/CFS.
Very recent corroborated research from the very few ME/CFS-specific privately and publicly funded (NIH, NINDS) associated research centers in the world (Workwell, Bateman-Horne, Columbia, Stanford University, Jackson Laboratories, Cornell University) indicates that post-exertional malaise can reflect three different types of intensities (informational links below): immediate, short-term, and long-term. Each phase generally is associated with increased dysfunction.
IMMEDIATE: Tends to be a drain of energy and exaggeration of symptoms associated with or immediately after activity. In many cases this will occur as increased exhaustion, rapid heartbeat, labored breathing/shortness of breath, muscle pain, nausea, vertigo, dizziness, light sensitivity, decreased ability to process thinking (brain fog/cognitive impairment), orthostatic intolerance. It usually signals a person with ME/CFS has the need to stop the activities they are involving themselves and sit down or lay down. Once the activities cease, the post-exertional malaise tends to decrease and energy and symptom levels go back to baseline. If most people can learn to cut off the offending activities as soon as or shortly before the onset of immediate PEM, they can more consistently function at their own baseline illness level and will generally tend to keep the duration of any flare-ups of PEM to a minimum and achieve most efficient management of their broken energy production. This can allow a person to get ahead of the symptoms of their disease and positively manage their energy production so they can function in a manner that gives him the best quality of life given their condition and challenges and even allow them to do some extra things they may have found challenging to do before.
SHORT-TERM: If a person ignores the onset of immediate PEM and continues with activities well after the onset of post-exertional malaise, (or simply overexert themselves) this is what will tend to create the onset of the more intense and more functionally limiting type of post-exertional malaise that can last for two to five days. The onset of this post-exertional malaise or PEM does not usually tend to occur right after the over-exertional event(s). Onset usually begins 12 to 48 hours afterward. Much worse than immediate PEM, this is the intense PEM that can put people in bed. They will generally tend to experience heightened and persistent exhaustion, sleep disturbance, orthostatic intolerance, cognitive impairment, muscle pain, flu-like symptoms, and an increase in other associated ME/CFS symptoms such as vertigo, dizziness, light sensitivity, and insomnia. This is due to the poor energy recovery, as the body is unable replenish its energy levels and gets sicker with the domino effect of further restricted bodily function. The obvious effects of this depletion and lack of functioning puts an incredible collective stress on the body leading to the prolonged exacerbation of baseline ME/CFS symptoms that is well beyond what is experienced in the immediate PEM phase. Because of the body's compromised energy production, this PEM can take much longer to recover compared to a person’s normal compromised functioning with ME/CFS.
LONG-TERM: If a person continues to overexert themselves while experiencing short term PEM or participate in exercise or activity that is extremely exertional, they will find themselves most likely having their energy levels depleted so badly that they will enter into a long-term state of PEM that can last weeks to months and in most severe cases can be lifelong. In the least, long-term PEM tends to last 5 to 7 days or more and result from the continuing exacerbation of shorter-term PEM or a very intense exertional event. In this state of PEM, the effects and intensity of shorter-term PEM will be multiplied by episodes of further overexertion and further energy depletion, and the intensity over exertion will cause the PEM and the symptoms associated with it to continue to explode into an all-body shutdown that may be extremely recalcitrant and difficult for a person to retrieve themselves from as it may take numerous weeks to months of rest and complete inactivity to recover. The energy depletion is so complete that the immune system is compromised, digestion can be greatly affected, muscles are filled with the waste products of anaerobic production, inflammation rages in the brain in the form of cytokine storms, and the CNS is extremely stressed leading to severe insomnia, tinnitus, and light and hearing sensitivities. Because the body has become so energy depleted, being very deep into this level of PEM can be dangerous as the body systems have become so stressed that even the tiniest amount of activity can continue to maintain the state of long-term PEM that can lead to the most catastrophic cases of ME/CFS. Consistent bouts of overexertion can lead to disease progression over time.
It is almost universally recommended by expert clinicians, serious researchers, and community activists, that a person with ME/CFS and long COVID suffering from post-exertional malaise stay ahead of PEM by not exacerbating the symptoms of these conditions. This is done by rest, pacing, and the laying off of any and all activities that lead to PEM or PESE. Unfortunately, given the structure of the world, attitude of the medical community, and lack of understanding, validation and support for people who have ME/CFS from all levels of social institutions - this is often for many easier said than done.
WORKWELL FOUNDATION:
https://workwellfoundation.org/educational-videos/
INTENSITY AND DURATION OF POST-EXERTIONAL MALAISE IN PEOPLE WITH ME/CFS AFTER CPET VS HEALTHY CONTROLS
https://content.iospress.com/download/work/wor203168?id=work%2Fwor203168
Copyright 2024 Scott Schneider Productions - all text written and maintained by Scott Schneider.
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Instead of a physical condition that can be detected by medical current medical testing, people with ME/CFS will experience a complete and total shutdown of normal aerobic processes within an unusual and abnormally quick time frame compared to all other individuals, even those with the above listed physical issues. They will then become stuck in anaerobic energy processes and be unable to replenish any vitally needed energy afterward due to the fact that the metabolic pathways that allow the chemical transport that uses oxygen in the mitochondria to complete the aerobic energy process is disrupted or nonexistent. The process of using aerobic respiration for energy production yields 34 to 36 ATP in the muscle in humans. Anaerobic respiration only yields 2 to 4 ATP. This is a thimbleful of the normal energy that the human body produces and requires. It is this not-so-well understood lack of normal energy production that leads to the incredible energy deficit experienced by people with ME/CFS and thus post exertional malaise. This inability of the body to not only produce but maintain proper energy for movement does not allow a person with ME/CFS to normally recover from even minimal physical and mental activities. This abnormally lowered aerobic energy threshold, that varies from individual to individual depending upon their illness level and is readily demonstrated by numerous two-day CPET (cardiopulmonary exercise stress testing) when compared to both healthy and even deconditioned controls, is what leads to this extraordinary energy deficit. This deficit not only leads to an increase in the profound exhaustion that already exists, but also an increase in all the symptoms of ME/CFS as the body gets sicker due to not only the stress placed upon it by the demands of energy production it cannot possibly keep up with but also an explosion of other CNS and neuroinflammation processes that are associated with the condition. Thus, post exertional malaise (PEM) is also known as post exertional symptom exacerbation or PESE.
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