An open response from parents and carers of people with Severe ME

A group of parents and carers of people with severe ME have welcomed Prof Edwards’ paper on the ‘Management of Nutritional Failure in People with Severe ME/CFS’, but want to also highlight the need for health professionals to understand the underlying pathology of ME and the importance of maintaining appropriate hydration / fluid balance.

An open response from parents and carers of people with Severe Myalgic Encephalomyelitis.pdf – Google Drive

“Our group suggests several additional factors not included in the preprint, which we see as critical to the analysis of the issues highlighted. This includes the need to acknowledge the growing body of scientific and clinical evidence explaining the underlying pathology in patients with Severe ME. We believe this to be critical to countering the misinformation and bias that often influences clinical and managerial decision-making about patients with this condition.”

“As carers, we strongly believe that the onus should be on acknowledging what is now known and knowable. This alone would strongly refute the notion of a need for a middle ground and would instead focus attention on the need to generate solutions from both evidence-based practice and practice-based evidence for managing symptoms, not only in relation to nutritional failure, but also (but not limited to): orthostatic intolerance, dysautonomia, hypovolemia, autoimmunity, sleep disorders, fluid balance, gastroparesis, mast cell activation, and pain.”

“The paper focuses on nutritional management. However, there is also overwhelming evidence from the lived experience of patients with Severe ME that maintaining appropriate hydration / fluid balance constitutes an equally important challenge. Patients with ME have not only been denied appropriate nutritional management but have also been denied appropriate management of fluids.”

“We believe our group has a unique vantage point and first hand lived experience from which to view this phenomenon.It is our collective view that there is a dangerous lack of understanding and awareness of the underlying pathology, especially in relation to Severe ME in NHS hospitals, which leaves patients vulnerable to harm and serious misunderstandings.

People with ME and their carers have experienced decades of marginalisation, discrimination, neglect, and often even a lack of compassion and understanding from some of the people they turn to for help.

Once again, we very much welcome this contribution that spotlights managing nutritional failure in patients with ME and would ask that the barriers to this and fluid management are scrutinised more closely. We agree with Dr Hemsley that action is indeed required at the highest level to address the need for informed, patient-led and safe care for the loved ones we care for, many of whom currently live with a rational fear of hospital admissions.”

Read the full response

See also:

Management of nutritional failure in severe ME 

More research needed into the lack of recovery time in PEM

The journal Science Norway looks at the key characteristic in ME/CFS of PEM, which can also be found in some people with Long COVID and Fibromyalgia.

It highlights that a type of test called repeated CPET measurements can precisely show how a session of physical activity affects a person’s condition afterwards – they perform worse and the lactic acid in their muscles builds up faster, they have poorer pain regulation and changes in the immune system and interaction with intestinal flora.

One study showed that strenuous mental tasks led to changes in the brains of ME/CFS patients that were not found in healthy people. The changes could correspond with the patients’ experiences of having an impaired ability to think and concentrate after the activity.

No research has been done into whether the same mechanisms can trigger PEM following social or mental exertion as from physical activity. It is not known if the same causes underlie fatigue and symptoms in the muscles and general fatigue throughout the system.

Researchers do not know if this is due to the same mechanisms as exercise-induced PEM. A 2016 study suggested that these might be different phenomena.

Prof Nina Vøllestad believes that existing research may provide a distorted picture, because the patients in the studies had to be able to complete a physical training session.

This probably means that the participants who were selected either had a lighter disease burden or were in a recovery phase, according to Prof Vøllestad and colleague Prof Emerita Anne Marit Mengshoel.

“When we engage in demanding activity, we get tired and need to recover,” Vøllestad says.

Vøllestad sees interesting similarities between PEM in ME/CFS patients and the symptoms of athletes with overtraining syndrome.  Overtraining is where the athlete does not get enough recovery time between training sessions over a long period.

“They’re not back to baseline before they start again. So, they wear themselves out, and it can take half a year to recover. The symptoms are very similar to ME/CFS,” says Vøllestad.

The question is: Why don’t ME/CFS patients recover normally?

But here, there are few answers.

“We lack research on biological changes during the recovery phase,” she says.

Read the full article

Pride of Wales Dad of the Year 2024

Nation Radio holds Pride of Wales awards every year where people are nominated in 17 categories. The winner of  Dad of the year for 2024 is Rob Messenger, carer of a son with severe ME, advocate, fundraiser and friend of WAMES.

Rob’s son Cerith was diagnosed with M.E. at the age of 11 and is still confined to a dark room twenty years later. Striving to be a voice for his son, Rob rose to the challenge and is speaking out for those suffering with M.E. and long covid.

Watch the video

Read more

500 miles for ME: Carer Rob’s fundraising challenge!

500 miles for ME: Carer Rob raises £1,000 for WAMES!

Rob’s #500miles4ME awareness raising reflections

Rob’s #500miles4ME awareness raising with Dr Nina Muirhead

Rob’s #500miles4ME awareness raising with physio Karen Leslie

ME/CFS: the biology of a neglected disease

A group of South African and British researchers have reviewed the literature and described ME/CFS in terms of symptoms, severity and burden, diagnostic criteria, causes and triggers; followed by an overview of the complex pathophysiology and management of the condition. They conclude by listing research priorities for the future.

They emphasise the necessity of a holistic approach to diagnosing, researching, and treating ME/CFS, urging the scientific community to reconsider the disease’s complexity and the multifaceted approach required for its study and management.

ME/CFS symptoms

Patients display varying symptoms and several body systems are impacted: Immunological impairments; pain; gastrointestinal problems; autonomic dysfunction; respiratory issues. Although many of the symptoms overlap with other diseases, one feature that sets ME/CFS apart is a worsening of symptoms in response to relatively minor physical, cognitive, orthostatic or even emotional exertion. This phenomenon is known as post-exertional malaise (PEM) or PESE (post exertional symptom exacerbation).

The researchers find that research confirms our understanding of the range of severity of the disease and that despite its high prevalence, there are still no universally accepted clinical criteria to characterise ME/CFS, making early and accurate diagnosis difficult.

Probable causes of ME/CFS

The researchers conclude that “ME/CFS is likely to have a multifactorial origin, with multiple physiological processes implicated” in the way the disease develops. They discuss genetic presdisposition, viral triggers and reactivation, toxin and drug exposure.

The complex pathophysiology of ME/CFS

“As a consequence of infection and other stressors, such as poly-trauma for example, a state of acute inflammation and aberrant immune activation may occur. A compensatory anti-inflammatory mechanism then typically follows), causing an imbalance in immune responses combined with hypothesised autoimmunity. This may lead to subsequent physiological abnormalities including gut dysbiosis and systemic inflammation, eventually resulting in a pathological clotting system, chronic endothelialitis, vasoconstriction, and hypoxia, as found in similar conditions such as heat stroke. Additionally, dysfunctional energy metabolism along with oxidative stress are also hypothesised in the development of ME/CFS. It is hypothesised that these mechanisms occur in a spiralling, progressive way, toppling the host’s homeostatic equilibrium.” A detailed discussion is included.

Management of ME/CFS

No effective therapies and few established non-pharmacological treatments for ME/CFSwere found. Hence current advice is aimed at symptom management and lifestyle changes. The current available treatments/lifestyle modifications are summarised in the illustration.

The researchers conclude by making suggestions for future research and calls for increased support for the ME/CFS community. They also list some potential treatments for furhter investigation: Rintatolimod; steroids; mitochondrial nutraceutical; PPAR agonists; Activators of AMPK; Immunostimulative therapy; Antivirals; Antioxidants; Immunomodulators; B-cell depleting therapy; Immunoadsorption therapy.

Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: the biology of a neglected disease, by Hayley E Arron, Benjamin D Marsh, Douglas B. Kell, M Asad Khan, Beate R Jaeger, Etheresia Pretorius in Front Immunol. 2024; 15: 1386607 2024 Jun 3. [doi:10.3389/fimmu.2024.1386607]

See also:

ME Research UK: An overview of ME/CFS

Whilst not necessarily providing added information about ME/CFS, this review pools together information from multiple sources to provide a helpful overview of this complex disease.

Successful subcutaneous Immunoglobulin Therapy in patients with ME/CFS

Swedish researchers have found promising effects on symptoms in 17 people with infection associated ME/CFS from subcutaneous treatment (under the skin) with low-dose Immunoglobulin (IG) – Hizentra. Immunoglobulins are antibodies found in white blood cells that fight infection. Low levels suggest your immune system is not functioning well.

Patients were predominantly female (65%) with mild-to-moderate disease severity (82%) and with poor self-reported quality of life and working ability before treatment.

This is thought to be the first study of subcutaneous IG as previously studies tested intravenous or intramuscular IG treatment, which results in much higher drug doses.

The findings from this case series (i.e. not a Randomized control trial – RCT) merit further investigation through well-controlled trials on subcutaneous IG treatment of patients with ME/CFS selected for being potential responders to the treatment.

Successful subcutaneous Immunoglobulin Therapy in a case series of patients with ME/CFS by Per Sjogren, Bjorn Bragée, Sven Britton in Clinical Therapeutics June 22, 2024 [doi.org/10.1016/j.clinthera.2024.05.010]

Research abstract:

Purpose
Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) remains an enigma with no curable treatment options at hand. Although patients with ME/CFS are a heterogeneous group, a large proportion of patients present with an infection-driven symptomatology, making them potential responders to immunologic treatments, such as immunoglobulin (IG). Previous studies on IG treatment in patients with ME/CFS have not been consistent but have described beneficial effects in subgroups of patients.

Methods
Here we present data on a series of cases (n = 17) with infection-related ME/CFS (as defined by disease history and ongoing recurrent infections) treated with subcutaneous low-dose IG (0.06 g/kg/mo) over 5 weeks with continuous monitoring of symptoms.

Findings
Patients were predominantly female (65%) with mild-to-moderate disease severity (82%) and with poor self-reported quality of life (median, 25 on a 0–100 scale) and working ability (median, 5 on a 0–100 scale) before treatment. After 5 weeks of treatment with low-dose IG, significant improvements in symptoms, quality of life, and working ability were noted (all P < 0.05). Among the 7 patients who reported the highest benefit of the treatment, quality of life increased by 35 units (on a 0–100 scale), with 1 patient reporting complete elimination of ME/CFS symptoms. No serious side effects were detected with the treatment.

Implications
In this limited-sized case series, we found pronounced beneficial effects of low-dose IG in a large proportion of patients with infection-related ME/CFS. Further well-controlled studies are needed to verify the potential benefits of IG treatment in patients with ME/CFS with infection-driven symptomatology.

NB

Back in 2021 our medical adviser Dr Nigel Speight and colleague Helen Brownlie wroe a paper suggesting a role for IG Therapy in ME/CFS.

Back to the Future? Immunoglobulin therapy for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome, by  Helen Brownlie and Nigel Speight in Healthcare 2021, 9(11), 1546; [doi.org/10.3390/healthcare9111546]   12 Nov 2021 (This article belongs to the Special Issue ME/CFS – the Severely and Very Severely Affected)

Management of nutritional failure in people with severe ME/CFS: Review of the case for supplementing NICE Guideline NG206

Prof  Jonathan Edwards writes:

“In the United Kingdom, a small but steady stream of people diagnosed with ME/CFS have run into serious problems with nutrition because of difficulties with eating and drinking, and some have not survived.

The clinical problem is extremely difficult, with little or no formal evidence on which to base diagnosis-specific recommendations for care, or information about prognosis. In several cases, however, the problem has apparently been compounded by a lack of provision of adequate services, misunderstandings, and conflict between health care professionals over diagnosis and approach to management.

The following is a review of the clinical problem, including some suggestions for protocol content that might supplement NICE Guideline NG206.

The main conclusion is that there is an urgent need for a consensus amongst professionals that focuses on practice based reliable evidence rather than theory-laden diagnosis.”

All reasonable efforts should be made to avoid stimuli to which patients are intolerant, which can include light, sound, physical contact, and odours. Exposure to stimuli has in the past led to a breakdown in trust and care and failure of life support.

Content for a protocol

Prof Edwards’ suggestions are based on consultation with health care professionals with experience in the care of severe ME/CFS. They are in line with both NG206 and the section on nutritional support in the recent BACME Shared Clinical Practice Document on Severe and Very Severe ME/CFS (2024) and draws from A Physiotherapist’s Guide to Understanding and Managing ME/CFS (Clague-Baker et al., 2023).

The protocol should apply to any patient if:

1. They have persistent and severe clinical features of ME/CFS as defined by disabling fatigue or exhaustion, exertion intolerance with post-exertional malaise, and more generalised intolerance of environmental stimuli.
2. They are unable to maintain body weight and/or hydration through normal feeding.
3. There is no contraindication to specific feeding procedures (such as intestinal obstruction).

Recommendations:

• An outline of management is given in NICE Guideline NG206, including general recommendations on minimising environmental stimuli in hospital.
• Feeding support with nasogastric tube, gastrostomy, PICC, or other methods should be offered in a timely fashion but may require modification in the context of orthostatic intolerance without the use of a standard angle of head elevation during feeding periods.
• Standard nutritional guidelines should be used based on loss of weight and poor oral intake, in line with recommendations from the British Association for Parenteral and Enteral Nutrition (which uses the Malnutrition Universal Screening Tool, MUST (see https://www.bapen.org.uk/pdfs/must/must_full.pdf)
• Home-based services should be made use of wherever possible.

Read the full recommendations and review of evidence:

Management of Nutritional Failure in People with Severe ME/CFS: Review of the Case for Supplementing NICE Guideline NG206, by Jonathan Edwards in Qeios Jun 25 2024 [doi.org/10.32388/T9SXEU] Preprint

ME/CFS service development 

The development of health services for people with ME/CFS in Wales continues, although only one Health Board has fully launched their service.  Staff recruitment is a slow business and Health Boards continue to experience internal pressures due to staff shortages and financial constraints.

Sixteen months ago, in March 2023, the Health Minister announced funding to:

“widen access to Wales’ long-Covid services… to people with other long term conditions whose rehabilitation and recovery are similar – for example, people with myalgic encephalomyelitis/ chronic fatigue syndrome (ME/CFS), fibromyalgia and other post-viral associated conditions.”‘

WAMES has been working hard to explain to Health Boards how Post-Exertional Malaise (PEM) affects the lives of people with ME/CFS and how this impacts symptom management.

We are pleased that many Health Boards have been keen to hear about ME/CFS from those living with the condition in their area, and that some intend to consult further with patients. We hope this will translate into appropriate services for people with ME of all ages and severities.

This is what we know about the current state of development. Please let us know if you have additional information.

Aneurin Bevan University Health Board

ABUHB are well advanced with the development of education and symptom management sessions for adults with a range of conditions. These can be accessed by referral from a GP or other health professional. They are also are exploring extending services to include children with ME/CFS.

Betsi Cadwaladr UHB

BCUHB is accepting a few people onto revised, albeit reduced, ME/CFS services in Llanfairfechan near Conwy and Connah’s Quay in the east. The Health Board continues to develop a Community Complex Conditions Service for adults, which aims to include the housebound. There are still plans to consult with residents, but this has been delayed.

The ME/CFS service offers individual biopsychosocial assessments, which can be face to face or virtual, some individual follow-up sessions if appropriate, and group work based on Acceptance and Commitment (ACT) principles. The group focuses on better managing symptoms and includes: Pacing of activity/energy management; improving sleep; diet; and stress management.

Cardiff and Vale UHB

The development of a Long Term Conditions service in the Cardiff and Vale area is well under way, and a soft launch of some self-management support sessions has begun i.e they are accepting adult participants before all aspects of the service are up and running. They are also exploring the possibility of setting up a Complex illness clinic, which could cater for the severely affected. Their information about ME/CFS can be found on the optimistically named Keeping me well website. Continue reading →

Functional Capacity in ME/CFS: an assessment questionnaire

Researchers from Norway and Norwich have collaborated to improve the ability to  assess funcional capacity in ME/CFS, something they believe can inform all areas of life for affected people.

“The absence of effective tools to assess FC has significant consequences for timely diagnosis, clinical follow-up, assessments for patient disability benefits, and research studies.”

Using feedback from patients they have identified eight domains divided by activity types:

1. personal hygiene/basic functions
2. walking/movement
3. being upright
4. activities in the home
5. communication
6. activities outside the home
7. reactions to light and sound
8. concentration.

Two versions of the questionnaire were developed: a longer version comprising 55 questions (FUNCAP55), developed for improved diagnostic and disability benefit/insurance FC assessments; and a shorter version (FUNCAP27) for clinical patient follow-up and potential use in research.

Assessing Functional Capacity in Myalgic Encephalopathy/Chronic Fatigue Syndrome: A Patient-Informed Questionnaire, by Kristian Sommerfelt, Trude Schei, Katharine A Seton, and Simon R Carding in J. Clin. Med. 2024, 13(12), 3486; [doi.org/10.3390/jcm13123486] Published: 14 June 2024

Scottish medical students’ thoughts on learning about ME/CFS

In early 2024 medical students at Scottish medical schools were invited to take part in an essay competition, 500 words on the topic of ‘What is your most important learning point about myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)?’

In an article for the Journal of the Royal College of Physicians of Edinburgh Dr Nina Muirhead gives an overview to what they learnt about ME/CFS and how they reacted when writing for the competition, which was funded by the Scottish Government’s education project: Learn About ME.

‘Imagine my surprise to discover ME/CFS is definitely not rare, but inexplicably and infuriatingly unacknowledged’. (Fifth year medical student, Scotland)

Dr Muirhead writes “ME/CFS affects at least 280,000 people in the UK, including approximately 23,000 in Scotland. It is a neurological disease with multisystem symptoms, often triggered by a virus. People with ME/CFS may be left bedbound or housebound with a significantly reduced quality of life. Yet many healthcare professionals do not know how to diagnose or manage this devastating disease, nor do they know how, or what, to teach our next generation of doctors.”

‘Alas, the only time in the last four years I have encountered the term ME/CFS at medical school was as a differential diagnosis for fibromyalgia’ [medical student, Scotland]

ME/CFS also has a major impact on family members’ quality of life. Nearly a third of medical students responding commented on the impact on quality of life as being one of the most important things to learn about this disease.

One student described the experience and delay in diagnosis for two of their family members:

‘. . . these young women were discarded as victims of teenage laziness or anxiety. They had to fight to gain support from their GP  and their diagnosis took several second opinions and ultimately years’.

Another medical student displayed incredible insight in recognising the deficiency of the current system, which relies largely on patient self-help:

‘the burden being placed on the patient to improve their condition through mental work’.

Dr Muirhead sums up:

“It is vital that this topic features more prominently in the medical curriculum, and in our medical textbooks, to avoid patient harm due to delayed or mis-diagnosis and mismanagement.

There is a lot we can offer ME/CFS patients:

• an early and accurate diagnosis
• medication for symptom control
• practical support with disability applications and mobility aids

but above all, these medical students have reminded us that ME/CFS patients should be treated with kindness, compassion and belief.”

Read the full article: Medical students highlight the importance of medical education, kindness, compassion and belief when learning about patients with myalgic encephalomyelitis/chronic fatigue syndrome

Read the winning essay: Learning points about myalgic encephalitis/chronic fatigue syndrome: Bridging the gap between research, clinical practice and awareness, by Bhanu Wahi-Singh

“My most important learning point about ME/CFS, however, is how it really exemplifies that the best doctors are the ones that listen and form a relationship with patients. ME/CFS is often a truly debilitating chronic condition, and it is imperative for us as clinicians to recognise this and understand how it impacts the patient’s life, not just dismiss it because it is complex with an incomplete knowledge base…

ME/CFS is often a truly debilitating chronic condition, and it is imperative for us as clinicians to recognise this and understand how it impacts the patient’s life, not just dismiss it because it is complex with an incomplete knowledge base.”

An open letter from unpaid carers

…to the future UK Prime Minister 

Carers UK has organised an open letter on behalf of unpaid carers across the UK. They will deliver the final letter to 10 Downing Street following the General Election that is taking place on 4 July 2024.

Join the thousands of people who have already added their support!

The key message is a rquest to “to take significant steps over the next five years towards the UK being a society that recognises, values and supports unpaid carers:

1. Reform and significantly increase Carer’s Allowance and other social security payments for carers.
2. Deliver an ambitious and fully funded National Carers Strategy.
3. Invest in breaks, respite services, and introduce new legislation so all carers have the right to regular and meaningful breaks.
4. Make the NHS the most carer-friendly health service in the world.
5. Introduce a right to paid Carer’s Leave for working carers.
6. Recognise caring as the 10th protected characteristic under the Equality Act 2010.
7. Invest in social care services so no one has to care alone, and so Local Authorities are able to meet their existing duties to unpaid carers.
8. Provide more support to help carers manage their health and wellbeing.

Read the full letter and sign