Post Exertion Neuroimmune Exhaustion
As early as the 1950s, Dr. Melvin Ramsey, the infectious disease physician who created the term “myalgic encephalomyelitis” after seeing multiple patients with the same unusual presentation, considered “as the sheet anchor of diagnosis”, “muscle fatigability, whereby, even after a minor degree of physical effort, three, four or five days, or longer, elapse before full muscle power is restored”. These parties believe the Fukuda definition’s emphasis on the ubiquitous symptom of fatigue as the principal and only mandatory symptom (none of the other eight auxiliary symptoms are required as long as any four symptoms are present) to be fallacious, resulting in both lack of an erroneous diagnoses by clinicians and inclusion of patients into studies who were not truly affected by ME.
Myalgic Encephalomyelitis M.E. is a distinctive neuromuscular disease, which was described in the medical literature between 1938 and 1993. Due to the introduction of chronic fatigue syndrome (CFS) and the misconception that ME and CFS as being ’similar disorders.’ ME was rarely considered until 2011, when a group of experts proposed using the International Consensus Criteria to define ME (ME-ICC) in order to separate a distinct patient group from the heterogeneous group of patients with CFS. This article reviews the similarities and differences between the original definition of ME and ME-ICC.
International Consensus Criteria for ME-ICC 2011
In view of more recent research and clinical experience that strongly point to widespread inflammation and multisystemic neuropathology, it is more appropriate and correct to use the term ‘myalgic encephalomyelitis’ (ME) because it indicates an underlying pathophysiology. It is also consistent with the neurological classification of ME in the World Health Organization’s International Classification of Diseases (ICD G93.3). Consequently, an International Consensus Panel consisting of clinicians, researchers, teaching faculty and an independent patient advocate was formed with the purpose of developing criteria based on current knowledge. Thirteen countries and a wide range of specialties were represented. Collectively, members have approximately 400 years of both clinical and teaching experience, authored hundreds of peer-reviewed publications, diagnosed or treated approximately 50,000 patients with ME, and several members coauthored previous criteria. The expertise and experience of the panel members as well as PubMed and other medical sources were utilized in a progression of suggestions/drafts/reviews/revisions. The authors, free of any sponsoring organization, achieved 100% consensus through a Delphi-type process. The scope of this paper is limited to criteria of ME and their application. Accordingly, the criteria reflect the complex symptomatology. Operational notes enhance clarity and specificity by providing guidance in the expression and interpretation of symptoms. Clinical and research application guidelines promote optimal recognition of ME by primary physicians and other healthcare providers, improve the consistency of diagnoses in adult and paediatric patients internationally and facilitate clearer identification of patients for research studies.
Post exertional neuroimmune exhaustion (PENE pen′-e) replaces the CFS term of
PEM ‘Post Exertional Malaise’. PEM is described as a vague feeling of discomfort or
fatigue. It is an inaccurate and inadequate word for the pathological
low-threshold fatigability and postexertional symptom flare.
Pain and fatigue are crucial bio-alarm signals that instruct patients to modify what they are doing in order to protect the body and prevent further damage. Post Exertional Neuroimmune Exhaustion is part of the body’s global protection response and is associated with dysfunction in the regulatory balance within and between the nervous, immune and endocrine systems, and cellular metabolism and ion transport. The normal activity/rest cycle, which involves performing an activity, becoming fatigued and taking a rest whereby energy is restored, becomes dysfunctional.
Numerous papers document abnormal biological responses to exertion, such as loss of the invigorating effects of exercise, decreased pain threshold, decreased cerebral oxygen and blood volume/flow, decreased maximum heart rate, impaired oxygen delivery to muscles, elevated levels of nitric oxide metabolites and worsening of other symptoms. Patients reach the anaerobic threshold and maximal exercise at a much lower oxygen consumption level. Reported prolonged effects of exertion include elevated sensory signaling to the brain that is interpreted as pain and fatigue, elevated cytokine activity, delay of 48 to 72 hrs in symptom activation and a recovery period of unknown length (could be hours, days, weeks, months or years.)
When an exercise test was given on two consecutive days, some patients experienced up to a 50% drop in their ability to produce energy on the second evaluation. Both sub maximal and self-paced physiologically limited exercise resulted in Post Exertional Neuroimmune Exhaustion..
Post-exertional Neuroimmune Exhaustion as per the ICC
This cardinal feature is a pathological inability to produce sufficient energy on demand with prominent symptoms primarily in the neuroimmune regions. Characteristics are:
Marked, rapid physical and/or cognitive fatigability in response to exertion, which may be minimal such as activities of daily living or simple mental tasks, can be debilitating and cause a relapse.
Post-exertional symptom exacerbation: e.g. pain and worsening of other symptoms.
Post-exertional exhaustion may occur immediately after activity or be delayed by for up to 48 to 72 hours.
Recovery period is prolonged, it can last hours, days, weeks, months or years.
Low threshold of physical and mental fatigability resulting in a substantial reduction in pre-illness activity level.
Depending on severity, physical triggers can range from, sitting up in bed, brushing hair, brushing teeth, showering, cooking, cleaning, taking a short walk, etc.
Mental activity - Things that require concentration and memory, such as reading, mental calculation, writing, using a computer, tablet or smart-phone.
Social activity - Interacting with people either in person, on the phone or online.
Emotions - Any activity that is likely to cause heightened emotion can be a potential trigger, for example, excitement, anger, frustration, fear, grief, guilt.
Sensory experience - Sensory sensitivities are regarded as a symptom and can range from intolerances to noise, light, touch, and even to changes in the weather or certain foods and medications.
Stress - The emotions that stress can generate as well as the hormonal changes it triggers in the body
PENE is an exacerbation of symptoms and can create new ones. Including: ‘Brain-fog’, Burning sensation on skin, Chronic pain (muscular or joint), Cognitive dysfunction, Disturbed sleep pattern (lack of sleep or need to sleep too much often incl. vivid dreams), Visual symptoms, Headaches and Migraines, Heaviness in the limbs or whole body, Heightened sensitivity to sensory stimuli (sound, light, smells), Loss of appetite, Loss of stamina/ functional capacity, Muscle fatigue and weakness to the point of paralysis, Nausea, Night sweats or chills, Orthostatic intolerance/dizziness, Problems regulating temperature (and cold/hot extremities ), Heart palpitations, Speech problems, Temporary paralysis, Short-term memory problems, Sore throat, Swollen and painful glands, Tinnitus (ringing in the ears), Trembling/ instability of muscle and/or limbs, Word Retrieval, etc…..
As a Cardinal Symptom for the ME-ICC, all patients must present: post-exertional exhaustion and symptom exacerbation after going beyond their energy limits.
Triggers in addition to physical/cognitive exertion can be were emotional events, creating sensory overload due to noise, light, smell.
Those who are bed bound or house bound find that basic activities of daily living lead to symptom exacerbation most or all of the time.
Symptom exacerbation 1-2 days but can be as long as 72 hrs after Exertional. There is no limit on how long PENE can last.
The most common symptom “Reduced stamina and/or functional capacity”, followed by “physical fatigue” and “cognitive exhaustion”.
Some patients experience ‘adrenaline surges’ during or after going beyond energy limits.
Many experience a “crash” that never resolved.
Pacing is basically modifying activity to meet personal restrictions of
Symptoms & Severity boundaries
Pacing is typically well accepted by people living with ME and is used primarily to manage symptom flare-ups and work within the available energy. The ‘Energy Envelope’ requires the patient to listen to their body and maintain expended energy levels consistent with available energy levels to reduce the frequency and severity of symptoms and avoid what we call a “CRASH”. A crash is when the patient ignores or tries to push through what their body is telling them and we collapse from exhaustion. For some it may only last an hour, but it can go on for days, weeks, months and years. Depending on each individual case of ME, the “crash’ can be delayed from a few or 24 to 72 hours post exertion.
The Energy Envelope To use the idea of the energy envelope, think of your situation as having three elements. The first is your available energy. This is the energy you have to accomplish things. It is limited and is replenished by rest and food. The second is your expended energy, the energy you lose through physical, mental and emotional exertion. The third is your symptoms, fatigue, brain fog, pain, and so on. In this view, if you expend more energy than you have available, you will exacerbate your symptoms. This is called living outside the energy envelope. An alternative is living inside the energy envelope.
A Bowl of Marbles & the Spoon Theory
If the idea of the energy envelope isn't appealing, you might consider other ways to think about your limitations. Some people, for example, like to visualize their limits. One version of this approach is to imagine available energy as a bowl of marbles. With this image, you see yourself as having a limited number of marbles to use each day. The number may vary from day to day. With each activity, you mentally take one or more marbles out of the bowl. Physical activity uses up your supply, but mental and emotional activity do as well. For example, if you feel frustrated about how few marbles you have, the frustration will take some marbles, too.
Another way to think about limits is called the spoon theory, which is explained in an article written by a Lupus patient. She describes how she once explained her limits to a friend by having the friend imagine that each task she did used up limited energy, symbolized by a fixed number of spoons. The fact that frustration uses marbles & spoons shows that the way we react to events can affect the amount of energy available to us. If we can respond in a relaxed manner to stressful situations, we can preserve energy that might otherwise be dissipated in tension and anxiety.
The key advice of the envelope theory is to stay within the envelope, meaning the limits ME imposes. Under the ‘Energy Envelope Theory’, treatment programs that recommend a steady increase in daily activity, such as Graded exercise therapy (GET), are discouraged because the person with ME may exceed the amount of energy available for that day, thereby initiating PENE or an increase in symptoms. By balancing energy expenditure and availability, patients can diminish the number of setbacks.
Pacing can result in social isolation. The energy envelope theory advises patients to only do as much as they can without triggering PENE and relapses. The phrase ‘staying within the envelope’ does not simply mean ‘don’t do too much’. Instead it refers to “a comfortable range of energy expenditure, in which an individual avoids both over-exertion and under-exertion, maintaining an optimal level of activity over time.”
Patients will experience fewer crashes and symptoms. This does not enlarge the envelope and expand the limits for people with ME but it will allow the patient to understand their limitations and use their energy levels more wisely.
References
Energy Conservation When you learn to conserve energy, you also reduce strain on your heart, fatigue, shortness of breath and stress related pain. Learning to conserve your energy is all about finding a good balance between activity, rest and leisure in order to decrease the amount of energy demand on your body.
Prioritize Decide: What needs to be done today and what can be done at a later date or time. For example, going to a doctor’s appointment would take priority over dusting the living room. When you have more than one thing to do, begin with the most important to make sure it gets done. Plan: Plan your activities first to avoid extra trips. Gather the supplies and equipment you need before doing the job. For example, get your garden supplies and tools ready before you start to plant flowers. Plan to alternate heavy and light tasks. Plan activities throughout the week to avoid doing too many activities in one day. Put a schedule on the refrigerator to remind you and others who is doing what. Plan to get a good rest each night. Use family and friends or pay for help to complete tasks you may struggle with or that require too much energy.
Pace: Never Rush: Maintain a slow and steady pace.
Rest often. Rest before you feel tired.
Avoid holding your breath. Practice breathing slow and steady. Use pursed lip breathing. Breathe in through your nose for a count of 2 and out from your mouth for a count of 4. This is like blowing out a candle on a cake.
Remember that you may have to ask for help to do some tasks and that is okay.
Listen to your body and know your limits.
Position Too much bending and reaching can cause fatigue and shortness of breath.
Use a reacher, sock aid, long handled shoe horn and/or elastic shoelaces. Avoid bending and reaching too much.
Always maintain a nice upright posture when sitting and standing. This helps you get more oxygen into your lungs and around your body to work better. This is a difficult suggestion for Moderate to Very Severe Patients with M.E.
Sit when you can. Sitting supports your body so you can focus on your breathing and activities while conserving your energy. Sitting reduces energy use by 25%.
Dressing and Hygiene Organize and lay out clothing the night before. Begin dressing your lower half first as this uses more energy. Avoid bending and reaching. Instead, use a reacher, sock aid or long handled shoe horn or lift your legs up onto the bed or chair.
Dry off with terry cloth robe. You use less energy than drying off with a towel. If you have a weaker limb or limbs, it is easier to dress the weaker limb first. It is easier to undress your strong limb first.
Wear clothes that are easy to put on and take off. For example, use clothes and shoes with velcro instead of small buttons, clasps or laces
Avoid using scented products such as hair products and lotions. These can irritate your lungs and cause shortness of breath for you and others around you. Many people are allergic to scents. These types of products are not allowed in the hospital.
Be cautious when bathing. Use warm, not hot water. This helps eliminate shortness of breath from a buildup of steam and condensation. Use the bathroom equipment suggested by your Occupational Therapist. For example using a bath bench, bath stool, grab bars or a raised toilet seat can make bathing and toileting easier and safer.
Shopping Bring a prepared list of things you need to buy
Organize your shopping list by aisle or section of the store.
Transport items in a buggy or shopping cart rather than carrying them in a basket.
Load and carry grocery bags that are only half full or shop with someone who can help pack and carry bags.
Avoid going out during rush hour when stores and streets are crowded.
Consider using a delivery service.
Housework Divide activities and do them throughout the week.
Balance light with heavy tasks.
Make one side of the bed at a time. Sit to change pillow cases and unfold linens
Avoid spray cleaners that may irritate your lungs.
Clean the bathtub by sitting or kneeling.
Clean one whole room at a time instead of going back and forth between rooms to do each job. Consider asking for help from family members or hiring a cleaning service or housekeeper.
After washing dishes, allow them to air dry.
Have work in front of you rather than at your side.
Slide rather than lift objects.
Use long handled dustpans and cleaning sponges to decrease the need for bending.
Make a weekly plan for major jobs such as laundry, cleaning and changing sheets on beds.
Do one job each day. Keep a trash can in every room to avoid too much walking. Buy more than one of each item you use around the house. For example, keep sink cleaner in the bathroom and kitchen. Keep a vacuum on each level of your home.
Cooking Cook and bake in steps to reduce energy use.
Gather all ingredients and utensils before starting.
Plan ahead with meal preparation. Make large meals and freeze in servings for later use.
Use lightweight cookware and dishes to conserve energy.
Use paper plates and cups to eliminate dish washing.
Use electric appliances such as can openers, blenders, food processors and dishwasher to conserve energy.
Consider buying easy to prepare or frozen meals, or using a meal delivery service.
Key Points: *Prioritize activities of the day. *Do heavier tasks when you have more energy. *Plan your days’ and weeks’ activities. *Set up your work area so you do not have to move around a lot looking for items to complete the task.
*Plan rest times. Pace yourself. Do not try to complete the whole task in one session. *Break it into smaller, easy to do steps. A good guide to follow is to take 10 minutes each hour to rest. Do not rush.
*Position and Posture are important. Sit to work when you can to use 25% less energy. Sit and stand as upright as you can. *Practice deep breathing exercises while you work to maintain your breathing rate and stay relaxed.
*Use assistive devices when recommended to save energy and make it more comfortable and easy taking care of yourself.
Remember… The most important energy conservation tip is to listen
to your body. Stop and rest BEFORE you get tired. Plan rest times. Rest often.