Explaining Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

Content Note: This post will be discussing, in depth, a very debilitating illness. I don’t recommend reading if you have health anxiety or are easily triggered reading about illness. As always, I am not a doctor and this is not medical advice.

July is Disability Pride Month and I wanted to kick it off by discussing a poorly-understood condition that I have been living with for years now, called Myalgic Encephalomyelitis.

What is Myalgic Encephalomyelitis (ME)?

Myalgic Encephalomyelitis (ME) is classified as a neuroinflammatory autoimmune disease.  It gets its name because of the following: Myalgic (pain) Encephalo (brain) myel (spinal cord) itis (inflammation).  Though the name ME was given to this disease first, it was later named Chronic Fatigue Syndrome.  This name has plagued those of us who have it because it makes it easier to confuse with the symptom of chronic fatigue, which is caused by many things, versus this multi-system condition.  Likewise, people hear the word “fatigue” and think we are simply sleepy, lazy, depressed, and deconditioned.  Though many people with ME may eventually become deconditioned or depressed, depression and deconditioning does not cause ME.

What triggers it?

Typically, a viral infection such as Epstein-Barr Virus, Mononucleosis, or Covid-19 (approx. 50% of all Long Covid cases meet the criteria for ME).  Lyme Disease that does not resolve with treatment may also meet the criteria, but because this has been mislabelled as “Chronic Lyme” (which is not an official diagnosis) it has been met with unfair scrutiny.  Trauma of any kind may also trigger ME.

In my case, I believe it was both a virus and trauma that contributed to my illness.  I was already chronically ill and was getting sick with severe viruses more frequently than most, including hospital visits.  My old family doctor used to see things in my blood work that would constantly indicate being at the tail end of a viral infection despite not exhibiting symptoms.  When I was in my twenties, I was also over-prescribed a neglectful and harmful amount of narcotics that caused a lot of damage.  These factors seem to have combined in a perfect storm in my body.

What mechanisms cause it?

ME has provable physiological changes and biomarkers in the body that can be seen with certain kinds of testing, and due to the systemic nature of this illness, researchers are finding more and more of these changes in the body all the time.  Some of the findings have shown:

• Micro clots and red blood cells that stick together
• Endothelial dysfunction
• Mitochondrial and ATP dysfunction (both power all the cells in the body)
• Metabolic dysfunction
• Brain inflammation, including brain stem abnormalities
• Spinal cord inflammation
• Lowered oxygen function and VO2 max
• Autonomic nervous system dysfunction (dysautonomia)

I’m probably forgetting some, due to the brain inflammation causing me my own memory issues, but this is a pretty good list of notable findings.

What makes it different from other chronic fatigue?

Chronic fatigue is a symptom of many things such as Fibromyalgia, Ehlers Danlos Syndrome, Lupus, Rheumatoid Arthritis, Postural Orthostatic Tachycardia Syndrome, Clinical Depression, and other chronic physical and mental health issues.  It can also be idiopathic, meaning of no known cause.  Chronic fatigue is just one of the many symptoms of ME, but a big difference is that many other conditions causing chronic fatigue can be treated in different ways and the fatigue will get better over time.  ME is not currently treatable, nor is the fatigue.  Differential diagnosis is always the first step because you need to rule out the possibility of a treatable illness first and foremost.

What is the diagnostic criteria?

The International Consensus Criteria is the most commonly used diagnostic criteria as it is the most thorough. I will leave a link to it, since it’s too difficult to fit it here.

What are differential diagnoses and comorbidities?

Differential diagnoses and comorbid conditions are usually the same, as it happens. Comorbidities are illnesses which are more likely to occur in conjunction with ME.  A partial list is as follows:

• Fibromyalgia
• Postural Orthostatic Tachycardia Syndrome (POTS)
• Lupus
• Mast Cell Activation Syndrome (MCAS)
• Lyme Disease
• Multiple Sclerosis
• Ehlers Danlos Syndrome
• Hypermobility Spectrum Disorder
• Narcolepsy or other sleep disorders
• Some vitamin deficiencies (such as B and D vitamins, or iron deficiency)

Many people with ME will likely have at least one other diagnosis from this list, but you can have a diagnosis from this list without having ME.  I had Fibromyalgia for years prior to developing or being diagnosed with ME, and I was also diagnosed with Hypermobility a few years after my ME diagnosis.  At this point in time, it isn’t fully clear if ME can cause these other issues to arise, or if ME just causes so many symptoms that it contains elements of many other illnesses.

What are the different stages of ME?

ME is categorized as Mild (stage one), Moderate (stage two), Severe (stage three), and Very Severe (stage four).  There are a lot of different scales measuring what these categories look like, and they’re all a bit different, so instead of relying on what doctors (who do not experience ME first hand) say, I prefer to listen to other people with ME.  There is more consensus within our community.  Generally, disability categories look something like this:

Mild (stage one):

• Operating between 50-75% of energy capacity.
• Able to go to work or school either full or part time.
• Able to leave the house for some social gatherings.
• Able to continue hobbies as usual.
• Able to get around without a mobility aid.
• Able to shower and use the toilet as usual.

Moderate (stage two):

• Operating at 30-50% capacity.
• May or may not be able to do remote work.
• Mostly housebound.
• Able to leave the house for medically necessary appointments.
• May only be able to socialize inside the house.
• No longer able to continue hobbies as frequently as before, or have switched to different ones.
• Dynamic disability requiring different kinds of mobility aids and adaptive measures around the house.
• Skips showers on worse days, uses shower chairs and other energy-saving equipment. Can still use the toilet without assistance.

Severe (stage three):

• Operating at 15-30%.
• Can no longer do remote work.
• Housebound.
• Mostly or fully bedbound (may depend on the day).
• Has to cancel most or all medical appointments.
• No ability to socialize even inside the house.
• Needs some amount of outside help.
• Requires a wheelchair for most or all things.
• Likely requires assistance in sponge bathing and using the bathroom.

Very Severe (stage four):

• 0-15% capacity.
• Fully housebound.
• Fully bedbound.
• Relies on support from caretakers.
• Spends days in a darkened room unable to tolerate any stimuli.
• May be on life assisting measures such as IV, feeding tube, and oxygen.
• Reliant on bedpan or diapers, and requires sponge bathing in bed.

Some of the disability scoring scales you might see for ME are the Bell Scale, created by Dr. David Bell and which uses a scale of 0-100 (0 being the worst and 100 being the best), or the Disability Rating Scale from Dr. Charles Shepherd of ME Association, which uses a scale of 0-100% (0 being the best and 100 being the worst).

The reason most people prefer to use the language of “stages” rather than to say “mild” is because even a “mild” case of ME can be debilitating, and can seem minimizing to call it so.  It feels more objective rather than subjective as, for example, I am nowhere near being “very severe” though I may feel very severe.  I am mostly considered moderate (stage two), with some severe (stage three) days. An example of a Very Severe figure in the ME community would be Whitney Dafoe, who spends all of his time in bed with round the clock care from his parents, who also help him advocate.

What is the hallmark symptom of ME?

The true hallmark symptom of ME is actually not fatigue but something known as Post-Exertional Malaise (PEM), sometimes referred to as Post-Exertional Neuroimmune Exhaustion (PENE).  It’s considered the hallmark feature because it’s a necessary diagnostic requirement by both the International Consensus Criteria as well as the Canadian Consensus Criteria.  ME cannot occur without PEM, and as far as current research shows, PEM only occurs in ME.

PEM is known as a worsening of all baseline symptoms, often accompanied by new ones, around 12-72 hours after exertion.  It also must last more than 24 hours in order to be considered PEM. If it gets better in less time, or by taking a quick rest, it would be a good idea to rule out other illnesses that cause exertion intolerance and symptom exacerbation.

Although many chronic illnesses can cause fatigue including symptom flares after exertion, so far PEM has been proven to be unique to ME as shown through 2-day Cardiopulmonary Exercise Testing.  The ME group was the only group to show significant oxygen usage deterioration on the second day which often took up to a few weeks to fully recover from, whereas other chronic illnesses (including Fibromyalgia, Lupus, Rheumatoid Arthritis, and others) and non-chronically ill sedentary controls did not demonstrate such effects.

The delay is one of the things that makes PEM so unique and so difficult to understand in terms of triggers, because while most symptom flares in other illnesses are more immediate, PEM reactions fluctuate and can take days to show up.  As someone who had Fibromyalgia for many years prior to receiving an ME diagnosis, I was always able to figure out what had caused me to feel worse because I would feel it the same day, and maybe the next day, and I could either push through it or rest and feel better fairly quickly.  PEM is harder to determine due to these delays and also has a delayed recovery time of several days, sometimes weeks, and for some people, even longer — and there is no pushing through.

PEM is thought to be the cumulation of all of the systems in the body malfunctioning.  It is caused by:

• Neuroinflammation, which is inflammation of the brain. 
• Mitochondria — the powerhouses to every cell in the body — have shown to be dysfunctional.
• Lowered immune function in the body, so cells that would normally activate to clear away “debris” that has accumulated during exertion, simply don’t respond in people with ME.
• Lack of proper oxygenation and blood flow in the areas of the body that need it most — both during the act of exertion, as well as in the post-exertion phase. 

Per this last point, an exercise physiologist in Germany, Dr. Perikles Simon, recently found that people with ME were not able to retain oxygenation in different muscles, and that they would essentially enter a kind of hypoxic state.  The oxygen and blood flow would pass too quickly from the needed area of the body — including the brain — or it would never receive the blood flow and oxygen at all.  This would push them into the PEM phase of the illness, and it’s also why shortness of breath is such a common symptom among people with ME who otherwise have normal lung and oxygen function.

PEM can be triggered by anything at all, depending on the severity of a person’s baseline illness.  Exertion is categorized as:

• Physical, such as any form of movement that pushes you outside your capacity. This looks different not only for every person, but can change from day to day. It could be taking a walk, sitting up for too long, or even brushing your teeth.
• Cognitive, which is anything that taxes the brain. Things such as reading, writing, or speaking/listening for long periods of time would fall under this category.
• Emotional, such as experiencing heightened anxiety or grief/loss. However, even intense positive emotions can be draining, especially if a lot of laughter is involved.
• Environmental, such as intense lights, sound, or exposure to smells or allergens.

PEM will look different for everyone, but these are some symptoms I have personally experienced and which are commonly experienced in the community:

• Extreme fatigue
• Flu-like symptoms
• Sore throat and/or foreign body sensation in throat
• Heavy limbs/heavy eyelids
• Feelings of weakness
• Feeling partially paralyzed
• Lightheadedness and faintness, with or without fainting
• Trembling and shaking
• Muscle spasms, tremors, and/or cramping
• Chills and/or hot flashes
• Nerve sensations throughout body (“electrical currents” are a commonly described one)
• Brain fog/cognitive dysfunction
• Slurred, slow, and/or weak speech
• Inability to find or say words
• Inability to retain information that is spoken to you
• Light sensitivity
• Noise sensitivity
• Smell sensitivity
• Shortness of breath with very minimal movement that would not ordinarily cause this symptom (such as sitting up on the toilet)
• Racing, pounding, and/or skipped heart beats
• Increase in heart rate with very little movement (such as rolling over in bed or lifting a cup to drink)
• High and/or low blood pressure
• Extreme thirst despite adequate levels of hydration
• Bladder and/or bowel dysfunction

Some of these PEM symptoms can mimic other, more serious conditions, so please don’t hesitate to reach out for medical care if you’re uncertain of what you’re experiencing.

In the most severe cases of ME, individuals may experience certain complications such as non-epileptic seizures, inability to swallow due to weakness of the throat muscles, or shallow breathing due to chest wall muscle weakness.  These individuals are in palliative care.  Severe and very severe ME patients have quality of life scores that are on par with or worse than individuals with aggressive HIV/AIDS and organ failure. To be perfectly clear, these are comparisons made by people experiencing them. It is not a competition. None of these things are nice.

PEM, in my opinion, is the hardest thing about having this condition because it’s so tough to avoid triggers (or even define them), and it’s so hard to be in that severe of a state where you feel like you are dying.  I do mean that non-hyperbolically, though I realize most people will think I am exaggerating.

It’s incredibly important to avoid PEM as much as possible as there has been some evidence to suggest that, over time and the more you enter this state, not only does the PEM get worse and longer to recover from, but the actual illness baseline can lower overall.  This means each time you exit PEM, the easier it can be to enter it again, and things that never used to trigger PEM will trigger it over time.  Speaking from my own experience, I would say this is likely true, as my PEM does seem to trigger more easily than it used to as of even just two years ago.

What are the treatments?

Unfortunately, there are no true preventions or treatments for ME or its hallmark symptom, PEM. The best treatment is pacing, which has people with ME doing only activities that stay within their energy capacity, and taking frequent breaks. Pacing can look like sitting down to do an activity instead of standing, or taking frequent breaks (as long or longer than the activity length), limiting stimuli by dimming lights and reducing noise, stopping an activity before feeling any symptoms of exhaustion, or delaying unnecessary activities for later days. However, pacing is not curative, nor is it even 100% preventative as many people with ME will have fluctuating energy capacities and enter PEM even when pacing as much as possible.

PEM recovery, for now, just takes time.  It means limiting all activity and stimuli as much as possible to allow the systems in the body to recover.  This means that people with ME require a lot of support during these times, more than while they are at their baseline, because some of us will not be able to move very much — others not at all.

There are some experimental, off-label uses for certain drugs and supplements that some people are finding helpful.  Experiences vary wildly, with some people doing better and some getting worse, so it’s up to the discretion of each individual and their provider (if they have one) on whether it is worth trying.  Some things people are trying include:

• Low and Very Low Dose Naltrexone (thought to reduce brain inflammation and help with immune response of Natural Killer cells)
• Low Dose Abilify (thought to reduce brain inflammation)
• Metformin (thought to perform well as an anti-viral in Long Covid and ME/CFS caused by latent viruses)
• Mestinon (thought to prevent chemical breakdown in muscle and nerve tissue and stimulate the vagus nerve)
• Benzodiazepines (acts as a nervous system depressant, which may lessen heightened nervous system activity and help sleep)
• Cannabis (typically CBD as THC is less tolerated; may help pain and promote sleep)
• Melatonin (may help sleep and mild night time anxiety)
• CoQ10 (may help convert available energy into ATP
• Magnesium (may help with IBS symptoms, muscle cramps, nerve pain, and sleep)

What isn’t recommended for ME?

The two forms of therapy that were (and sometimes still are) most commonly used to treat ME, which have been disavowed by all major medical and scientific affiliations, are Graded Exercise Therapy and Cognitive Behavioural Therapy.

Cognitive Behavioural Therapy is not recommended because this illness is not in the mind. CBT is a therapeutic modality which challenges behaviours in order to move past them, suggesting they are making you worse through irrational fears. In the case of CBT for ME, it was found that it would ask patients to challenge behaviours such as “if I do too much, I could have a setback” suggesting this was a matter of irrational thought patterns. The thing is, we know these thoughts are not irrational and are in fact true — moving outside energy capacity makes people with ME worse. So trying to train someone away from these thoughts forced people with ME to crash over and over.

Likewise, Graded Exercise Therapy is built upon the false idea that people with ME are simply deconditioned and need to build stamina. If ME was a problem of deconditioning, then doctors and scientists would see the same results in the 2 day Cardiopulmonary Exercise Test between people with ME and sedentary people without ME. That is not the case. All Graded Exercise Therapy does is push exertion levels higher and higher and also force people with ME into repeated crashes. I believe I myself did this as I was trying to rebuild my stamina over time between 2017 and 2021, and every time I would reach a point where my body said “no”, I would feel sick, and I would have to start again, not realizing this wasn’t helping.

The issue is that doctors will often diagnose patients but then not offer any guidance, so patients will try things that would ordinarily be helpful — like trying to slowly increase stamina — not realizing that this simply doesn’t work with an illness where that stamina does not exist. Without proper education of doctors in schools, patients have to become their own educators and advocates, leading to a systemic lack of care

In conclusion

ME is a serious condition that affects the entire body and the entire life of the person who has it.  While many conditions cause fatigue, ME is a multi-system disease with unique hallmark symptoms not shown in other fatigue-causing conditions.  ME is not the result of mental illness, deconditioning, or laziness.  We are still dealing with this stigma in 2023 despite Long Covid being around now and more and more people developing post-viral illnesses, including ME.  The best thing you can do to support your loved ones with this condition is to read or watch more about it, and ask what kind of support you can provide.  We are doing our best.

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