Disbelief and Disregard in health and social care for people with Energy Limiting Conditions (ELC) Part 1

A research project into Energy Limiting Conditions (ELC) wanted to understand the problems people experience getting the support they need from health and social care services.

Project 1: Disbelief and Disregard: The Gendered Experiences of Energy Limiting Chronic Illnesses

This project looked at the experiences of health and social care for people who identify as women, trans men and non-binary people with ELCs.

According to estimates by the Department for Work and Pensions (DWP), 1 in 3 disabled people in the UK experienced impairments in stamina/breathing/fatigue.

These experiences are shared by people living with a range of conditions, including neurological, musculoskeletal, and auto-immune diseases, ME/CFS and fibromyalgia. The COVID-19 pandemic has significantly increased this population.

Watch this summary of experiences

They found that many face discrimination, often in the form of not being listened to or believed.

This is compounded by other forms of discrimination such as sexism, racism, transphobia, homophobia and fatphobia.

Disbelief and disregard in interactions with healthcare professionals has significant negative impact on all areas of people’s lives.

Disbelief and disregard: psychological harms

Disbelief and disregard project website

A letter to the world

This is a piece of prose I wrote in the early years of living with ME. I remember feeling immensely frustrated when people in my life told me I looked well and was really lucky to have fully recovered from ME!

The fluctuating and invisible nature of the illness just passed everyone by, leaving me feeling completely lost, alone and unseen…

Letter to those in a world untouched by ME

By Rachel Hazlewood

Your sleepless night; your tiredness; your headache or foggy brain.

All of your bodily aches and pains I understand. I am compassionate. You have my concern and support for all of these and so much more.

But more than this, you have my understanding – my empathy. For I know what these feel like. In your moment of ill health you are not suffering alone – for I am right there with you, walking by your side, feeling every step with you.

I do not have a monopoly on pain, on fatigue, on feeling ill from head to toe, or that inevitable accompanying feeling of frustration and incompetence. Yes, you are entitled to this and you will have my love and support and concern – without hesitation or question.

But know this: take that feeling and know that it will pass. It may take only minutes, it may take hours or even days. If you’re really unlucky you will feel ill for a few weeks, or even months. But it will pass. And when it does you will forget – oh how quickly you will forget what torment you lived through.

But know this… it will pass… how quickly you will forget

So, when you are at your lowest ebb; when you are in the deepest depths of your suffering, please find a small chink of humanity and compassion to capture that feeling and to hold onto it.

Think. Even just for the most fleeting of instants, what life is like for those of us patiently waiting – waiting for minutes, hours, days, weeks, months and years. So many minutes, so many days, weeks, months and years we can no longer keep count. Waiting for the ubiquitous symptoms to pass, hoping those symptoms will pass, yet knowing, in our hearts it is unlikely.

We go on. Of course we go on – we have no choice. Finding ways to keep going, adapting, adjusting – gleaning what pleasure we can.

Drifting through the world – invisible- living a ghostly half life – mere shadows of the people we once were. Our full, energy rich lives a distant memory.

I know you cannot give me energy, or bring a cure. You cannot stop the pain or blow the fog from my mind so my thoughts are clear again. And yet you can make a difference. You can take the time to look at me….. I mean really look at me and see beyond the body standing in front of you, going about her business: functioning. Look closer. Lean in.

you cannot give me energy, or bring a cure… and yet you can make a difference

See the tired eyes, the droopy shoulders and the shuffling feet. See the times I hold onto something to prevent myself from falling over, see the involuntary yawns and grimaces, the shaking muscles spasming uncontrollably….

See the slightly robotic gait or delay when I try to stand. See my lungs struggling to give me enough air to breathe freely. See how often I vigorously yet fruitlessly rub at my glasses trying to remove imaginary smudges, then realise it’s my poor tired eyes that prevent me from seeing the world in all its wonderful clarity.

Look……Look….. and see me – the exhausted, confused and frightened soul trapped in a failing and unpredictable body and mind. See what ME has stolen from me.

Look……Look….. and see me

If you can see all of this and can still bear to keep looking then you may also be wise enough to see deeper still. If you have compassion in your heart then you will see that there is still a glimmer – a small spark of life and hope.

But when you tell me I look well I feel silenced, shut down. My suffering invisible, unimportant, unacknowledged, insignificant. To acknowledge my suffering is to acknowledge me. My illness does not define me, but it is part of me, part of my every day.

To acknowledge my suffering is to acknowledge me

To tell me I look well you remove my voice. I can no longer express who I am. So next time you tell me I look well, I know you do not know me, you have not really looked – you have cast the most fleeting of glances in my direction and moved on in your world of wellness.

Every minute of every day is a challenge. But the essence of being determined is to keep fighting – determined to dig deep and somehow find the strength to get out of bed, to push through the extreme fatigue, the confusion and the pain to make the best of what each day has to offer: to hold down a job; to spend time with family and friends; to contribute to the world in whatever way I can – no matter how small and seemingly insignificant that contribution may appear. To me it is a triumph – a triumph of spirit. Each moment a mini victory.

If you see this, if you really see me, then I thank you. I thank you for pausing, for noticing, for caring and for helping to make the invisible visible.

I thank you for… helping to make the invisible visible

So next time you have a headache or feel tired, or are simply feeling unwell, celebrate its transience and think of all those trapped souls…… think of ME.

A brief screening scale for ME/CFS

Health professionals often use questionnaires to determine if a person has a particular illness. Prof Leonard Jason’s DePaul Symptom Questionnaire has 54 questions and takes much time and energy to get a full picture of a person’s experience of ME/CFS.

Jason and his team have now developed a short questionnaire with 4 key symptoms to screen for ME/CFS in line with the US IOM criteria. This accurately identified people with ME/CFS from healthy controls, but had more difficulty differentiating ME/CFS from other chronic illnesses like MS, Long COVID and PPS.

The team conclude that the DePaul Symptom Questionnaire-Brief (DSQ-Brief) is useful as a first step, but the longer questionnaire may be needed to reliably confirm ME/CFS.

Developing and validating a brief screening scale for ME/CFS, by Leonard A Jason, Sage Benner, Jacob Furst & Paul Cathey, in Fatigue: Biomedicine, Health & Behavior, 07 Sep 2023 [doi.org/10.1080/21641846.2023.2252613]

Research abstract:

Objective:
The purpose of the current study was to develop and evaluate a brief screening instrument for ME/CFS. The current study identified 4 symptom items that identify those positive for the IOM ME/CFS case definition.

Study Design:
A data set of over 2,000 patients with Myalgic encephalomyelitis/ chronic fatigue syndrome (ME/CFS) and over 350 controls were assessed for the 4-item DePaul Symptom Questionnaire-Brief (DSQ-Brief). All respondents also completed the longer 54-item DePaul Symptom Questionnaire (DSQ-1) as well as the 14-item DePaul Symptom Questionnaire-Short Form (DSQ-SF).

These data sets were collected from multiple countries. We also examined the DSQ-Brief, DSQ-1, and DSQ-SF with other chronic illness groups [Multiple Sclerosis (MS) and Post-Polio Syndrome (PPS)] and those with Long COVID. Random Forest comparisons were employed in these analyses.

Results:
When contrasting ME/CFS from controls, high levels of accuracy occurred using the DSQ-1, DSQ-SF, and DSQ-Brief. High accuracy again occurred for differentiating those with ME/CFS from MS, PPS, and Long COVID using the DSQ-1 and DSQ-SF, but accuracy was less for the DSQ-Brief.

Conclusions:
The DSQ-Brief had high sensitivity, meaning it could identify those with ME/CFS versus controls, whereas accuracy dropped with other chronic illnesses. However, it was possible to achieve better accuracy and identify those cases where misidentification occurred by administering the DSQ-SF or DSQ-1 following the DSQ-Brief. It is now possible to screen individuals for ME/CFS using the DSQ-Brief and in so doing, identify those who are most likely to have ME/CFS.

Image by Peggy und Marco Lachmann-Anke from Pixabay

WASF3 – disrupting cellular energy production in ME/CFS

Malfunctioning mitochondria have long been thought to play a role in ME/CFS but working out exactly what is wrong is taking time.

You may hear mitochondria called “the powerhouse of the cell.” Mitochondria are an energy factory. The job of mitochondria is to process oxygen and convert substances from the foods you eat into energy. Mitochondria exist in nearly every cell in the human body. Mitochondria produce 90% of the energy our bodies need to function. [Cleveland]

US researchers have now discovered unusually high levels of a protein called WASF3 in a woman with ME/CFS.  They tested it in cultured cells and mice and found the protein could disrupt mitochondrial function. They then compared healthy people with people with ME/CFS and found all people with ME/CFS had high protein levels.

Other experts warn this might only be part of the answer but the group is now looking at drugs that could reduce WASF3’s effects on mitochondria, with an eye toward designing a clinical study.

WASF3 disrupts mitochondrial respiration and may mediate exercise intolerance in myalgic encephalomyelitis/chronic fatigue syndrome by Ping-yuan Wang, Jin Ma, Young-Chae Kim and Paul M Hwang in PNAS Vol. 120, No. 34, Aug 2023 [doi.org/10.25444/nhlbi.23681013]

Significance: 

Chronic fatigue is a debilitating symptom that affects many individuals, but its mechanism remains poorly understood. This study shows that endoplamic reticulum (ER) stress–induced WASF3 protein localizes to mitochondria and disrupts respiratory supercomplex assembly, leading to decreased oxygen consumption and exercise endurance.

Alleviating ER stress decreases WASF3 and restores mitochondrial function, indicating that WASF3 can impair skeletal muscle bioenergetics and may be targetable for treating fatigue symptoms.

Research abstract:

Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is characterized by various disabling symptoms including exercise intolerance and is diagnosed in the absence of a specific cause, making its clinical management challenging.

A better understanding of the molecular mechanism underlying this apparent bioenergetic deficiency state may reveal insights for developing targeted treatment strategies.

We report that overexpression of Wiskott-Aldrich Syndrome Protein Family Member 3 (WASF3), here identified in a 38-y-old woman suffering from long-standing fatigue and exercise intolerance, can disrupt mitochondrial respiratory supercomplex formation and is associated with endoplasmic reticulum (ER) stress.

Increased expression of WASF3 in transgenic mice markedly decreased their treadmill running capacity with concomitantly impaired respiratory supercomplex assembly and reduced complex IV levels in skeletal muscle mitochondria. WASF3 induction by ER stress using endotoxin, well known to be associated with fatigue in humans, also decreased skeletal muscle complex IV levels in mice, while decreasing WASF3 levels by pharmacologic inhibition of ER stress improved mitochondrial function in the cells of the patient with chronic fatigue.

Expanding on our findings, skeletal muscle biopsy samples obtained from a cohort of patients with ME/CFS showed increased WASF3 protein levels and aberrant ER stress activation.

In addition to revealing a potential mechanism for the bioenergetic deficiency in ME/CFS, our study may also provide insights into other disorders associated with fatigue such as rheumatic diseases and long COVID.

Independent: A woman who survived breast cancer twice may have inadvertently furthered long Covid research

Read the story of how Amanda Twinam’s search for answers to her low energy inspired Dr Paul Hwang to study how the protein called WASF3 was plugging up her energy production process. Dr Hwang is now focused on curing ME/CFS, and his team plan to trial a new drug.

Science.org: A protein that disrupts cells’ energy centers may be a culprit in chronic fatigue syndrome

Psychiatrist: A Protein That Zaps Cellular Energy May Underlie Chronic Fatigue

Initial findings from the DecodeME questionnaire data published

The Study team report:

We’ve analysed questionnaire answers from the first 17,000 DecodeME participants and the results have been published by NIHR Open Research – read the paper HERE.

We are still recruiting participants and will continue to collect information as more join the study – take part HERE.

Main findings from our analysis:

Being female, older and ill for longer increase the chance of greater severity

Our findings support the assumption that ME/CFS is significantly more common in females as 83.5% of participants reported their sex assigned at birth as female. Furthermore, our data suggests that females are more likely to experience severe symptoms. This likelihood further increases with age and if they have had the condition for more than 10 years.

Additionally, a higher percentage of female participants reported other co-occurring health conditions.

Two-thirds (66.7 per cent) of women, and slightly more than half (52.7 per cent) of men, reported at least one active co-occurring condition. Similarly, 39.2 per cent of women and 28.6 per cent of men reported at least one inactive co-occurring condition.

The most common active co-occurring condition was irritable bowel syndrome (41.3 per cent), with clinical depression (32.4 per cent), fibromyalgia (29.5 per cent), anaemia (14.1 per cent) and hypothyroidism (12.8 per cent) also featuring prominently.

Women also reported, on average, more symptoms than men – 42 compared with 36.

The most common of these symptoms were brain fog – a term commonly used to describe the cognitive impairment experienced by participants – unrefreshing sleep, and muscle pain.

Experts say that gaining a better understanding of how ME/CFS affects people is the first step to developing effective treatment options.

To further improve our understanding, we are still recruiting participants whose questionnaire answers will help us understand even more about ME/CFS. Many of these participants will also be invited to provide a DNA sample to contribute to the DNA portion of the study, which aims to identify the biological causes of the illness.

Take part HERE.

Do you know someone with ME/CFS? Spread the word and help them take part in the world’s largest ME/CFS study. Go to our ways to share page to find out how.

See also:

Trial By Error video: Professor Chris Ponting Discusses on DecodeME’s First Results

Open Access Government: ME/CFS study reveals chronic fatigue syndrome affects women more than men

Guardian: ME/CFS Women with ME tend to have more symptoms than men, study suggests

Independent: Women with ME tend to have more symptoms than men, study suggests 

Sky News: Women ‘suffer more from ME’, according to largest ever study into the disease

The viral origin of ME/CFS

Experienced US researcher Prof Maureen Hanson explores the evidence for the viral connection to ME/CFS and finds no proof that multiple infections lead to ME/CFS. She sees enteroviruses as the most likely culprit and although long COVID and ME/CFS overlap more data is needed to determine if they are identical.

Excerpts from her article:

Can any infection lead to ME/CFS?

There is actually no proof that multiple different pathogens can cause ME/CFS. Yet, this hypothesis persists largely due to the overinterpretation of data from at least 2 studies. The limited evidence depends on how ME/CFS is defined.

Why is the enterovirus family the most likely culprit in ME/CFS?

History offers persuasive evidence to suspect the enterovirus (EV) family of causing ME/CFS. Both circumstantial and direct evidence exists to support such a conclusion.

What is the relationship between human herpesviruses (HHVs) and ME/CFS?

A striking number of ME/CFS patients mention an acute infection with EBV or some other human herpesvirus (HHV) as the start of their illness. Whether this is true or not is not known. If someone has a long course of mononucleosis, an additional virus that may or may not cause symptoms might be necessary for induction of ME/CFS.

Infections with HHVs are common and lifelong. Healthy people maintain viruses such as EBV in a latent state.

Reactivation of dormant HHVs as a result of onset of ME/CFS may have sometimes been mistaken for a new infection, resulting in patients believing that an HHV induced their chronic illness.

Should the post-SARS-CoV-2 infection syndromes be called “ME/CFS”?

… while it would be correct to say that someone has post-COVID illness with symptoms diagnostic of ME/CFS, referring to a post-COVID syndrome as actual ME/CFS will confuse the scientific literature and cloud clinical trials.

Recently, a study of 9,764 individuals experiencing symptoms following acute COVID-19 resulted in classification of the cases into 4 subgroups and the identification of 12 core symptoms among 44 that were considered. Unfortunately, one of the key ME/CFS symptoms—unrefreshing sleep—was not evaluated. The 12 core symptoms include ones that are not identified as core symptoms in any of the ME/CFS diagnostic criteria.

Nevertheless, the intriguing overlap in symptoms between some forms of post-COVID illness and ME/CFS suggests that disruptions in the same pathways may be occurring in both diseases, but to conclude the 2 syndromes are identical without more data, especially at the molecular level, is currently unwarranted.

Conclusion

Ignoring the abundant evidence for EV involvement in ME/CFS has slowed research into the possible dire but hidden consequences of EV infections, including persistence in virus reservoirs. Prior to the SARS-CoV-2 pandemic, the ability of RNA viruses to persist in tissues for long periods was largely ignored.

Further, recognizing that EVs are prime candidates for causing ME/CFS suggests how critical it is to pursue a relevant inquiry into this diverse virus family. Do hidden reservoirs harbor these viruses? Have they induced autoimmunity through molecular mimicry? Is it past or current infection that has resulted in the many findings of immune dysfunction in ME/CFS?

Read the full paper:

The viral origin of myalgic encephalomyelitis/chronic fatigue syndrome, by Maureen R Hanson in PLoS Pathog 19(8): e1011523. [doi.org/10.1371/journal.ppat.1011523]

Medical express: Chronic fatigue syndrome may have a post-viral infection origin

Home-based testing protocol to measure physiological responses to everyday activities in ME

Physios for ME and an exercise specialist have trialled a safe method of measuring the effect of everyday activities on people with ME/CFS.

CPET exercise testing in the lab has, in the past, shown abnormal reactions to exercise, but now a portable metabolic assessment system and a physiological stress monitor has shown people with ME/CFS also react abnormally to normal everyday activities such as:

• Lying for 5 min
• Sitting for 5 min
• Standing for up to 5 min
• Typical bathroom activities (e.g. washing, grooming, toileting) for up to 5 min
• Walking downstairs
• Typical kitchen activities (e.g. preparing breakfast, hot drinks) for up to 5 min
• Walking upstairs
• A formal cognitive activity (serial sevens test [31]) and other standardised mental arithmetic problems were performed while sitting for up to 5 min.

“All participants demonstrated some physiological abnormalities during testing, which supports preliminary success to further develop this home-based assessment approach.

However, there was no single activity that all participants could complete that caused roughly similar abnormal response(s) in all participants. Thus, future work to produce a standardised testing protocol that yields broadly informative findings on ME pathophysiology is likely to require additional study with larger samples.

Perhaps surprisingly, the activity that caused most participants to exceed their anaerobic threshold was the cognitive task, which all but one participant was able to complete.”

All participants were positive about the testing process and would recommend it to others. 11 showed signs of undiagnosed Orthostatic Intolerance, and the evidence of over doing it (i.e. exceeding anaerobic thresholds) suggested who might benefit from heart rate monitoring  as part of an energy management strategy.

Home-based testing protocol to measure physiological responses to everyday activities in ME: a feasibility study, by Nicola Clague-Baker, Sarah Tyson, Karen Leslie, Helen Dawes, Michelle Bull & Natalie Hilliard in Fatigue: Biomedicine, Health & Behavior (2023) [DOI: 10.1080/21641846.2023.2245584]

Research abstract

Background and objectives
Individuals with Myalgic Encephalomyelitis (ME) have shown altered physiological responses during maximum cardiopulmonary exercise testing. However, maximal testing is not representative of the everyday activities reported to cause or increase symptoms in ME, and is not accessible for those with severe or very severe illness. The aim of this study was to assess the feasibility and acceptability of a home-based testing protocol to measure physiological responses in ME to everyday activity.

Methods
Researchers attended participants’ homes to collect data and provide equipment for independent testing. Adults with ME who met the International Consensus Criteria wore a portable metabolic assessment system and a physiological stress monitor. Blood pressure, heart rate, oxygen saturation and lactic acid were assessed during a range of everyday positions and activities in their own homes.

Results
Online recruitment yielded 70 volunteers in 24 h. 17 eligible individuals reflecting a range of illness severities were enrolled. All participants found the procedures acceptable with 12 (70%) subjects completing every listed activity. Apparent physiological abnormalities were identified in all participants.

Conclusion
Physiological measurement during everyday activities was feasible for our participants who represented a range of ME severities. Activities must be adapted for different levels of severity to avoid significant symptom exacerbation. Further research is needed to develop home-based assessment protocols to advance the biobehavioral understanding of ME.