Myalgic Encephalomyelitis Primer for NDIS Decision Makers

Posted onAuthorJackie Stallard

Download as PDF

Introduction

Myalgic encephalomyelitis (ME) is a system-wide health condition that can affect the heart, lungs, muscles, metabolism, immune system, brain and cognition, causing severe disability. This document explains why people with severe ME should be accepted onto the NDIS; the functional disability caused by ME; and the supports that participants with ME commonly need.

The following sections explain that:

  • ME is a physical health condition. It is not psychological and is not caused by physical deconditioning.
  • ME can cause severe or extreme disability.
  • ME is a permanent condition; there is no known cure for ME
  • People with ME unfairly experience discrimination, disbelief and a denial of supports
  • People with severe ME need extensive support with daily activities and social and community participation

These facts are supported by the research papers listed at the end of the document.

ME is a Physical Health Condition

The World Health Organisation identifies ME as a neurological disorder1.

ME symptoms commonly begin after an acute viral, bacterial or parasitic infection2,3. There is a strong genetic predisposition2.

The UK’s National Health Service’s NICE guidelines4 describe ME as “a complex, chronic medical condition affecting multiple body systems”. The guidelines explain that people with severe ME are house- or bed-bound and unable to perform most daily tasks.

The US Institute of Medicine (IOM) has reported that ME is “a serious, chronic, complex, systemic disease that often can profoundly affect the lives of patients”5. The IOM suggested that ME be renamed “systemic exercise intolerance disorder”, because exercise intolerance is a defining feature of ME.

ME can affect the heart, lungs, immune system, nervous system, cognition, sensory system, digestion, muscles, sleep and metabolism. A quarter of people with ME are house- or bed-bound. Appendix A contains a list of commonly-experienced symptoms; Appendix B summarises some of the many physiological markers of ME.

Graded exercise therapy is harmful for people with severe ME4,8. Exercise intolerance is a key symptom of ME5. Consequently, exercise and cognitive exertion can cause long-lasting symptom flares. Gentle, carefully-designed exercise programmes may help to maintain function, providing that they are custom-designed for each ME patient by a health professional with deep knowledge of ME.

Cognitive behavioural therapy cannot cure ME because ME is a system-wide neurological condition4. ME is not a psychological disorder. However, cognitive behavioural therapy can help people with ME to manage their symptoms and reduce the distress of having a chronic illness.

ME Can Cause Severe or Extreme Disability

ME has the following functional impacts:

  • 98% of people with ME are unable to perform their usual activities 6
  • Only 7.6% of people with ME can maintain full-time employment (as opposed to 52% of the general population), despite being more highly educated 6; around 65% are unable to work at all 7.
  • A quarter of people with ME are house- or bedbound 8
  • 12% of people with ME are considered to be in a state worse than death 6
  • Quality of life is lower than that of people with multiple sclerosis, stroke, depression, heart disease, renal failure and most cancers 6
  • 97% of people with ME are in pain 6

ME Causes Permanent Disability

There is no known cure for ME/CFS 9 and only 5% of adults recover from ME/CFS 10. Children and teenagers have better recovery rates than adults of around 60% 10,18.

Discrimination Against People with ME

Because ME is not appropriately recognised and treated by government and medical systems, patients bear 94% of the cost of their health condition 7. Additionally 7 :

  • The average annual income loss for each person with ME is $45,000 – $56,000
  • The average annual out of pocket healthcare costs are $14,000 – $24,000
  • The average annual personal cost of having ME is ten times that of chronic obstructive pulmonary disease

The following social factors cause further distress to people with ME 8:

  • The misconception that ME is a psychological condition or caused by physical deconditioning
  • Undereducated health professionals
  • An absence of effective treatments
  • The downplaying of symptoms and suicidality by medical professionals
  • Social stigma when dealing with family, friends, employers, medical professionals and the general public
  • The burden of constantly needing to justify, explain and defend ME symptoms
  • Being blamed for their illness
  • Feeling like a burden to society
  • Social isolation and an inability to maintain relationships, due to functional limitations
  • A loss of trust in health and social services 4

Consequently:

  • People with ME are socially marginalised 8
  • ME causes a sevenfold increase in suicide rates 8

ME Supports

Health Interventions

There is no known cure for ME. However, it is important that medical and allied health professionals identify, treat and support the symptoms. Each patient will have a different symptom cluster.

People with ME should be diagnosed and treated by specialist multisdisciplinary medical teams with strong communication between team members 4.

As noted above, graded exercise therapy harms people with severe ME. However, carefully-designed, customised exercise may help some people to maintain function. Exercise programmes should only provided when actively sought by the participant. It is important to inform people with ME of the risks 4.

Cognitive behaviour therapy does not treat the physical disability caused by ME. However, it may help people with ME to cope with their disability.

Support with Daily Activities and Social and Community Participation

People with severe ME commonly need the following supports:

  • Cleaning and gardening
  • Meal preparation and/or prepared meals from a meal delivery company
  • Help with showering and daily care
  • Transport. Many people with ME are unable to drive or to use public transport
  • Help accessing public places such as shops and medical centres
  • Help with cognitively-demanding tasks
  • Disability aids such as recliners, laptop stands and tables, kitchen devices, shower chairs
  • Mobility aids such as wheelie walkers, wheel chairs and mobility scooters
  • Cognitive aids and supports, including NDIS support coordination, disability advocacy and help filling out forms and navigating government systems.
  • Allied health support, including:
    • physiotherapy
    • hydrotherapy
    • psychology
    • occupational therapy
    • massage therapy
    • dietician
  • In-home or tele-appointments
  • Pain supports such as heated blankets, compression gloves
  • Communication aids, such as speech to text software
  • Social support
  • Help with pacing activity
  • Sensory supports, such as noise cancelling headphones, dark glasses, Irlen’s lenses
  • Extensive support coordination

Appendix A

ME Symptoms

ME is a complex disorder that affects multiple body systems. The Australian definition of ME requires the following symptoms to have been present for at least 6 months, but not since birth:

  • Cognitive impairment
  • Physical and cognitive fatigue
  • Daily activity reduced by at least 50%

In addition, other countries include many of the following symptoms as essential or optional criteria for an ME diagnosis 11:

  • Fatigue:
  • Post exertional symptom exacerbation (physical exercise and mental exertion cause reduced aerobic capacity; increased pain and fatigue; and symptom flares)
  • Fatigue is not reduced by sleep or rest
  • Muscle weakness
  • Lower levels of metabolites
  • Neurocognitive symptoms:
    • Brain fog
    • Difficulty thinking/processing
    • Short-term memory loss
    • Difficulty focusing and paying attention
    • Depression
    • Anxiety
    • Anorexia
  • Sensitivities to:
    • Food
    • Odours
    • Medications
    • Photophobia (light sensitivity)
    • Chemicals
  • Immune system:
    • Mild fever
    • Feeling feverish
    • Tender lymph nodes
    • Flu-like symptoms
    • Sore throat
    • Susceptibility to viruses
    • Unusual patterns of inflammation-causing cytokines, depending on symptom severity
  • Pain:
    • Myalgia (pain, including joint and muscle pain)
    • Headache
    • Generalised hyperalgesia (increased pain sensitivity)
  • Autonomic dysfunction:
    • Sleep disorder/disturbance
    • Drowsiness
    • Orthostatic intolerance
    • Muscle disturbance
    • Double vision
    • Thermostatic instability
    • Tinnitus
    • Widespread neuroinflammation
    • Cardiovascular problems
    • Respiratory problems
    • Genito-urinary symptoms
    • Gastro-intestinal symptoms

Appendix B

Research Evidencing Physiological Changes in ME

Researchers have found the following physiological changes in ME.

Neurological changes

Neurological changes include:

  • Neuroimmune activation 2
  • Neuroinflammation, evidenced by increased glial cell activity 2,3
  • Blood-brain barrier permeability 2
  • Reduced biosynthesis of neurotransmitters such as glutamate, aspartate, and gamma aminobutyric acid (GABA) in the frontal, temporal, cingulate and occipital cortices and basal ganglia 2
  • Higher levels of neurotransmitters such as norepinephrine and epinephrine 2
  • Alterations to seratonin transporters, receptors and synthetic enzymes 2
  • Impaired cerebral blood flow 2,3
  • Reduced volume in the prefrontal cortex; reduced volume of the right prefrontal cortex correlates with fatigue severity and reduced function 2
  • Decreased connectivity in the brainstem, which controls autonomic activity such as sleep and breathing 3,12.
  • Increased myelination in the sensory cortices 12. This may explain the increased sensitivity of people with ME to noise and light.
  • Neuronal microstructural changes 13
  • Abnormal cortical volume 3,14
Autoimmunity

With autoimmunity, the immune system attacks the body’s own tissues. Many autoimmune changes have been associated with ME, including:

  • The presence of autoantibodies against neurotransmitters, neurotransmitter receptors, neuron membranes and neuron cell nuclei. 2,3
  • Antinuclear antibodies (ANA) 2
  • Antibodies against acetylcholine receptors on muscles 2,3
  • An increase in B cells that are associated with autoimmunity 2
  • Production of autoantibodies that are known to cause severe metabolic disturbances 3
Immune System

The following findings suggest that people with ME have decreased immunity:

  • Compromised natural killer cells and B leukocytes, leading to greater susceptibility to infections. 2,3
  • Chronically activated immune responses 3
  • Abnormal natural killer cell and T cell function, and T cell exhaustion 2,3
  • Fewer TRPM3 ion channels on the surface of natural killer cells, causing reduced calcium flow in and out of the cells 2. These changes are associated with certain genetic SNPs 2,3
  • Reduced calcium concentration within natural killer cells, leading to compromised natural killer cell function 2
  • Fewer peripheral blood lymphocytes 2
  • Intestinal permeability that allows passage of bacteria from the gut into the blood stream 2
  • Significant increase in pro-inflammatory cytokines in spinal fluid 2
Hormone disturbance

People with ME display abnormal hormone levels, including:

  • Low cortisol 2
  • Low levels of hypothalamic-pituitary-adrenal axis hormones 2,3
  • Increased catecholamines (that activate the fight-flight system) 2
Inflammation

People with ME show chronic low-grade inflammation that may be systemic 2. Studies show:

  • Increased levels of pro-inflammatory cytokines 2,3
  • Oxidative and nitrosative stress damage to membranes, proteins and DNA, causing serious tissue and organ damage 2,3
  • Lowered antioxidant levels 2
  • Gliotoxins, produced by certain species of bacteria, fungi and viruses, that can damage glial cells in the brain, causing inflammation 2
  • Significant differences in DNA methylation, mostly in genes relating to preservation of an inflammatory state 2
Energy Production

In ME, reduced energy is associated with the following:

  • Mitochondrial damage (compromised energy production) 2,3
  • Reduced ATP production (fewer energy units) 2
  • Mitochondrial damage and reduced ATP lead to autoimmunity 3
  • Fewer nicotinic acetylcholine alpha 3 receptors 2
  • A blockade of protein kinase R synthesis. This protein is important for the metabolism; compromised protein production has been associated with inflammation and neurodegeneration. 2
  • Presence and reactivation of human herpesvirus-6 (roseola), which fragments mitochondria16,17
  • Reduced oxygen uptake by muscle cells during exercise. 19,22,23
  • Reduced exercise capacity on the second day of two-day cardiopulmonary exercise tests, compared to the first day of testing, unlike sedentary controls or controls with other chronic health conditions. 19,20,21,22,23
Cardiovascular

In ME, cardiovascular changes include:

  • Abnormal blood pressure, hypertension and orthostatic intolerance 3
Genetic

People with ME have a strong genetic predisposition for many of the above changes 2.

Diagnostic Test

Esfandyarpour et al. (2019)15 have developed a test that can reliably discriminate between people with ME and healthy controls. When stressed with a high salt concentration, the peripheral blood mononuclear cells in the plasma and serum of people with ME respond with high electrical impedance. The test is in the early stages of development but shows promising results.

References

  1. World Health Organization. (2019). ICD-11: International classification of diseases (11th revision), chapter 8, 8E43.Z. Retrieved from https://icd.who.int/
  2. Cortes Rivera, M., Mastronardi, C., Silva-Aldana, C. T., Arcos-Burgos, M., & Lidbury, B. A. (2019). Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A Comprehensive Review. Diagnostics (Basel, Switzerland), 9(3), 91. https://doi.org/10.3390/diagnostics9030091
  3. Sukocheva, O. A., Maksoud, R., Beeraka, N. M. et al. (2021). Analysis of post COVID-19 condition and its overlap with myalgic encephalomyelitis/chronic fatigue syndrome, Journal of Advanced Research, https://doi.org/10.1016/j.jare.2021.11.013
  4. National Institute for Health and Care Excellence (2021). Myalgic encephalomyelitis (or encephalopathy)/chronic fatigue syndrome: Diagnosis and management. Royal College of Physicians Guidelines, downloaded from https://www.nice.org.uk/guidance/ng206/chapter/Recommendations#assessment-and-care-and-support-planning-by-an-mecfs-specialist-team, accessed 22 October, 2022
  5. Institute of Medicine (2015). Beyond myalgic encephalomyelitis/chronic fatigue syndrome: Redefining an illness. Military Medicine, 180(7), 721–723. https://doi.org/10.7205/MILMED-D-15-00085
  6. Hvidberg, M. F., Brinth, L. S., Olesen, A. V., Petersen, K. D., Ehlers, L. (2015). The health-related quality of life for patients with myalgic encephalomyelitis / chronic fatigue syndrome (ME/CFS). PLoS ONE, 10(7): e0132421. doi:10.1371/journal.pone.0132421
  7. Close S., Marshall-Gradisnik S., Byrnes J., Smith P., Nghiem S., & Staines Don (2020). The Economic Impacts of Myalgic Encephalomyelitis/Chronic Fatigue Syndrome in an Australian Cohort, Frontiers in Public Health, 8. https://doi.org/10.3389/fpubh.2020.00420
  8. Chu, L., Elliott, M., Stein, E., & Jason, L. A. (2021). Identifying and Managing Suicidality in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome. Healthcare, 9(6), 629. https://dx.doi.org/10.3390/healthcare9060629
  9. National Institute for Health and Care Excellence (2021). Myalgic encephalomyelitis (or encephalopathy) / chronic fatigue syndrome: diagnosis and management [G] Evidence reviews for the non pharmacological management of ME/CFS, NICE guideline NG206, downloaded from https://www.nice.org.uk/guidance/ng206/evidence/g-nonpharmacological-management-of-mecfs-pdf-9265183028, accessed 22 October, 2022
  10. Cairns, R., & Hotopf, M. (2005). A systematic review describing the prognosis of chronic fatigue syndrome. Occupational medicine (Oxford, England), 55(1), 20–31. https://doi.org/10.1093/occmed/kqi013
  11. Lim, E.-J., & Son, C.-G. (2020). Review of case definitions for myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). Journal of Translational Medicine, 18(1), 1–10. https://doi-org.rp.nla.gov.au/10.1186/s12967-020-02455-0
  12. Barnden, L. R., Shan, Z. Y., Staines, D. R., Marshall-Gradisnik, S., Finegan, K., Ireland, T., & Bhuta, S. (2018). Hyperintense sensorimotor T1 spin echo MRI is associated with brainstem abnormality in chronic fatigue syndrome. NeuroImage: Clinical, 20, 102–109. https://doi.org/10.1016/j.nicl.2018.07.011
  13. Thapaliya, K., Marshall-Gradisnik, S., Staines, D., & Barnden, L. (2021). Diffusion tensor imaging reveals neuronal microstructural changes in myalgic encephalomyelitis/chronic fatigue syndrome. European Journal of Neuroscience, 54( 6), 6214– 6228. https://doi.org/10.1111/ejn.15413
  14. Thapaliya K, Marshall-Gradisnik S, Staines D, Su J and Barnden L (2022) Alteration of Cortical Volume and Thickness in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome. Front. Neurosci. 16:848730. doi: 10.3389/fnins.2022.848730
  15. Esfandyarpour, R., Kashi, A., Nemat-Gorgani, M., Wilhelmy, J., & Davis, R. W. (2019). A nanoelectronics-blood-based diagnostic biomarker for myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). Proceedings of the National Academy of Sciences of the United States of America, 116(21), 10250–10257. https://doi.org/10.1073/pnas.1901274116
  16. Schreiner, P.,Harrer, T., Scheibenbogen, C., Lamer, S.,Schlosser, A., Naviaux, R. K., Prusty, B. K. (2020). Human herpesvirus-6 reactivation, mitochondrial fragmentation, and the coordination of antiviral and metabolic phenotypes in myalgic encephalomyelitis/chronic fatigue syndrome. Immunohorizons, 4(4), 201–215. https://doi.org/10.4049/immunohorizons.2000006
  17. Mozhgani, S. H., Rajabi, F., Qurbani, M., Erfani, Y., Yaslianifard, S., Moosavi, A., Pourrostami, K., Baradaran Bagheri, A., Soleimani, A., Behzadian, F., Safavi, M., & Rezaei, F. (2022). Human Herpesvirus 6 Infection and Risk of Chronic Fatigue Syndrome: A Systematic Review and Meta-Analysis. Intervirology, 65(1), 49–57. https://doi.org/10.1159/000517930
  18. Moore, Y., Serafimova, T., Anderson, N., King, H., Richards, A., Brigden, A., Sinai, P., Higgins, J., Ascough, C., Clery, P., & Crawley, E. M. (2021). Recovery from chronic fatigue syndrome: a systematic review-heterogeneity of definition limits study comparison. Archives of disease in childhood, 106(11), 1087–1094. https://doi.org/10.1136/archdischild-2020-320196
  19. van Campen, C. L. M., Rowe, P. C., & Visser, F. C. (2020). Two-day cardiopulmonary exercise testing in females with a severe grade of myalgic encephalomyelitis/chronic fatigue syndrome: Comparison with patients with mild and moderate disease. Healthcare, 8(3), 192. https://doi.org/10.3390/healthcare8030192
  20. Vanness J.M., Snell C.R., Stevens S.R. Diminished cardiopulmonary capacity during post-exertional malaise. J. Chronic Fatigue Syndrome, 2007(14), 77–85. doi: 10.1300/J092v14n02_07
  21. Hodges, L. D., Nielsen, T., & Baken, D. (2018). Physiological measures in participants with chronic fatigue syndrome, multiple sclerosis and healthy controls following repeated exercise: a pilot study. Clinical Physiology and Functional Imaging, 38(4), 639–644. https://doi.org/10.1111/cpf.12460
  22. Snell, C. R., Stevens, S. R., Davenport, T. E., & Van Ness, J. M. (2013). Discriminative validity of metabolic and workload measurements for identifying people with chronic fatigue syndrome. Physical therapy, 93(11), 1484–1492. https://doi.org/10.2522/ptj.20110368
  23. Vermeulen R.C.W., van Eck I.W.V. Decreased oxygen extraction during cardiopulmonary exercise test in patients with chronic fatigue syndrome. J. Transl. Med. 2014(12), 20. doi: 10.1186/1479-5876-12-20