Severe ME Week: How might healthcare be improved?

Russell Fleming, Content Manager, ME Association.

Today marks the beginning of Severe M.E. Week. It runs from Monday 3rd to Sunday 9th August, with Severe M.E. Day falling on Saturday 8th August – when we remember those from our community who have died as a result of M.E.

Can you think back to the times when your M.E. was at its worst? Maybe you are currently suffering from Severe M.E. Maybe the person you care for is currently in the grip of Severe or Very Severe M.E. and you must be their voice.

Whatever your experience, and however you can contribute, this week we are focusing on the worst that M.E. can bring.

Sharing our own stories, considering those things that have helped us cope, and thinking about what must change to bring more effective and timely healthcare and support to those who have the greatest need.

Please join our conversations on social media this week, in particular the ME Association Facebook page.

Look out for our blogs and conversations starters, add your comments, send us messages, or email us with your own stories and about any of the issues raised this week: feedback@meassociation.org.uk

Below you will find my thoughts about the NICE clinical guideline and healthcare provision. And then I have provided some background about Severe and Very Severe M.E. and listed support that is available.

When you need to talk

The reality of Severe and Very Severe M.E. and the problems with appropriate healthcare are important topics that need to be talked about.

But we do appreciate that some people may find what we discuss this week in particular quite distressing.

If you find yourself struggling with any of the issues we raise or simply would like to speak to someone who can help, then please consider contacting:

Health should always comes first, so please do take care of yourself.

Contents

1. What is our best chance to improve NHS healthcare?

2. The MEA Website Survey: Severe and Very Severe M.E.

3. What are the symptoms?

4. What do we mean by Severe and Very Severe M.E.?

5. Prognosis and Quality of Life

6. What does the current NICE clinical guideline recommend?

7. How many people are likely to experience Severe M.E.?

8. Further information and support.

1. What is our best chance to improve NHS healthcare?

In the last couple of years, I have written several blogs introducing Severe ME week.

Looking back, I think the only change of note that I want to highlight here has been the decision by NICE, following concerted attempts over many years by the ME Association, other charities, and patients, to fully review and update its clinical guideline on ME/CFS.

This influential document provides a framework of care to the NHS and I believe it to be our best chance of improving NHS healthcare for anyone affected by M.E. particularly for those who are in most need.

The NICE clinical guideline review

  • The National Institute of Health and Care Excellence (NICE) Clinical Guideline on ME/CFS: CG53 (2007). Online version. Pdf Download.

If NICE get the new guideline right, it could mean that we have the tools to really start bringing positive change to the NHS – especially for those Severely and Very Severely affected. This is assuming it can be decided what those changes should be of course.

  • What would you like to see NICE recommend for people with Severe and Very Severe M.E.? Join our discussions on social media this week, or send in your thoughts: feedback@meassociation.org.uk and we will pass them on to NICE if at all possible.

If NICE get the new guideline wrong, then as stakeholders we have a chance in November to review the draft document. We will be advocating for change against anything that we feel is not reflective of the patient experience, does not provide sufficient detail, or undermines our goals of recognition, respect and appropriate and timely care for people with M.E.

  • The ME Association believes that graded exercise therapy (GET) has no place in any clinical guideline, nor should it be recommended by the NHS to anyone with M.E. We have been saying so since before the current guideline was published, and in our recommendations following patient surveys since then, and will keep on saying so until NICE and the NHS remove it.

The new clinical guideline on ME/CFS will be published on 21 April 2021. And NICE should include any accepted amendments from the November 2020 stakeholder consultation.

Clinical guideline committee

We have had some excellent representation on the clinical guideline committee who I know have worked incredibly hard on this vital project.

The committee is chaired by Peter Barry, Consultant Paediatric Intensivist, and Baroness Ilora Finlay, Consultant in Palliative Medicine, Clinical Lead for Palliative Care for Wales.

Patients are represented by 5 lay members with experience of M.E. (more were appointed than to any other NICE guideline), medical experts, like Charles Shepherd, from the ME Association, and William Weir, Luis Nacul and Caroline Kingdon, from the M.E. Biobank, Tony Crouch, an expert social worker with experience of M.E.

And Gabrielle Murphy and other clinical experts representing several NHS ME/CFS specialist services, also a physiotherapist, and a dietitian.

Because all the committee discussions have been confidential, we haven’t been able to keep you updated with progress as much as we would have liked, but an enormous effort has been made to try and make this guideline better.

We can only hope that NICE takes on board all the contributions and the published document is a noticeable improvement on the current guideline and that it, in turn, leads to better healthcare.

Without an effective NICE guideline, it has been extremely hard making any headway with NHS service provision. This is particularly true for people who suffer from Severe and Very Severe M.E.

  • We have been advocating that NICE reinstate the Very Severe illness definition into the new guideline (see below). At present it only talks about Mild, Moderate and Severe and the definition of Severe is not really representative. But by excluding Very Severe, NICE has not provided any guidance to the NHS about how to provide appropriate care and support to this most vulnerable section of the community.

Unfortunately, even if we do achieve a supportive guideline from NICE, we will still need to wrestle with the ‘bean counters’ in the NHS and the Clinical Commissioning Groups (CCGs) (in England) to try and ensure they allocate sufficient funds to provide the necessary recognition, care and support.

NHS Healthcare

It is one thing for NICE to tell the NHS, ‘These are the guidelines we recommend you adopt’, it is quite another for the NHS to comply – especially when budget allocations and priorities are concerned.

We will need to consider in the months following publication exactly how we can raise the profile of M.E. to the extent that the NHS is prepared to spend more of its money.

And that, I think, will need national and local support. Advocating for additional spending from CCGs can best be achieved with the help of people in the local community.

We will all need to come together to help spread the word about the new guideline – or those parts we endorse at least – if we are to stand a chance of improving service provision from the NHS.

  • Medical education and awareness initiatives have increased noticeably in recent years. With Dr Nina Muirhead (an NHS surgeon with M.E.) taking the lead with the CMRC Medical Education Group and Carol Monaghan, MP, by raising concerns in Parliament. The ME Association and Dr Charles Shepherd have been involved in all these initiatives.
  • Examples include: The new CPD online training module for health professionals, panel discussions around the country with GPs, representation at the Royal College of GPs annual conferences, the launch of the new All-Party Parliamentary Group on M.E., ongoing Forward ME initiatives, and several notable debates in the House of Commons and Westminster Hall.

We all know how current NHS provision can vary dramatically across the UK. Healthcare for people with M.E. is led by primary care physicians. Your own GP will order the blood tests, determine an initial diagnosis, prescribe symptom medications, make any referrals to specialists and be the person who oversees any ongoing care.

  • In the case of someone with Severe or Very Severe M.E., NICE say that, “Your doctor should consult a specialist if they are unsure about the diagnosis, or if you have severe symptoms.”
  • Unfortunately, many of the ME/CFS specialist services in the NHS do not provide appropriate assistance to people this affected. NICE do have more to say about people severely affected, and I discuss these recommendations below.

But even this basic level of support varies greatly – it often seems that no two people with M.E. receive the same degree of understanding let alone care. And some of these encounters with GPs and other health professionals can be devastating for patients and their families when met with disbelief.

  • In other instances, contact with the NHS for a person with M.E. is more positive, but as a national charity we rarely hear these stories I am afraid. If you do have positive experiences to report then please let us know: feedback@meassociation.org.uk

NHS ME/CFS specialist services

We used to have a large network of NHS ME/CFS specialist services in England, funded initially by the Government and before the NICE guideline was originally published. But these have dwindled as other priorities for spending have occurred.

In Wales, Scotland and Northern Ireland, NHS provision is patchy at best and non-existent at worst (see MEA website for full list of UK specialist services).

This often means people with M.E. are left trying to obtain appropriate care and support from their GPs who often lack the necessary expertise even with the framework provided by NICE.

  • For an illness with such a large prevalence (see below) that dwarfs Multiple Sclerosis for example, I just don’t understand why the NHS and the CCGs do not see spending on M.E. to be a priority. We cost this country a huge amount of money in lost revenue by being unable to work and having to depend on benefits. It just doesn’t make sense.

Of course, NHS specialist provision has not always been welcomed. With NICE advocating graded exercise and cognitive behavioural therapy for most of those affected, feedback wasn’t always positive (to say the least).

And, while NICE does not advocate GET for people severely affected, the NHS did not always seem to take on board this distinction.

NICE talks about the importance of personalised or individualised care and joint decision-making between the NHS and patients, but many patients have reported being prescribed inappropriate GET and management advice which directly led to harm and a deterioration in their health.

  • If the goal of the NHS is to improve or at least stabilise a person’s health and make them more comfortable, they don’t seem willing to listen to their patients or adapt their approach.

Some specialist services managed to stick to the ethos of the guideline offering tailored and more suitable care, home visits for the severely affected, activity management or pacing, alongside dedicated medical specialists, occupational therapists, psychologists, and physiotherapists i.e. a multi-disciplinary approach.

Unfortunately, such services seem to be rare and are also subject to cost restraints or threats of closure – others have closed – which means even the better services cannot do all they want to provide necessary care and support.

And this extends to those albeit limited parts of the NICE clinical guideline that do show concern for people with Severe and Very Severe M.E.

For example (and see section below):

1.9.1.1 Management of Severe ME is difficult and complex and healthcare professionals should recognise that specialist expertise is needed when planning and providing care for people with severe ME.

1.9.1.3 People with Severe ME may need to use community services at times. These services may include nursing, occupational therapy, dietetics, respite care, psychology and physiotherapy (see the ‘National service framework for long-term conditions'[11]). The input of different professionals should be coordinated by a named professional.

Hobson’s Choice

For many people with M.E. NHS healthcare can seem a Hobson’s Choice:

Will I find a specialist service near me? Will I be believed? Will I get appropriate and timely care?

Will I receive useful support and guidance? Will I get a helpful prescription? Will my GP come and visit me at home?

Or will I just have to accept what is available or take nothing at all and try to manage on my own?

However, not interacting with the NHS and achieving positive outcomes can lead to further dilemmas when it comes to accessing necessary welfare benefits and social care.

  • Asserting your rights to clinical care, benefits, and social care – and getting accurate medical reports to support any claims – can be an awfully difficult thing to achieve especially when you are Severely affected or care for someone Very Severely affected. There has to be a fairer and easier way to access support when you are in most need.

Despite being ill and deserving of care and support when at your worst, having to battle with those who make the decisions can be like climbing Mount Everest without oxygen.

Engaging with the NHS when at your worst

People in most need used to be able to rely on home visits from their GP but even this can prove a bridge too far. I don’t know how people manage to engage with the NHS and others when they are at their worst.

I suspect that most people don’t unless they have family support, or the battle is fought when their health has stabilised somewhat, and they can tackle it over a period of time. But it shouldn’t be this hard.

  • Maybe there will be some lessons learned from the Covid-19 lockdown. These should already have been in place and part of normal routines by the NHS but perhaps those in most need will continue to have access to GPs and hospital specialists via phone or video conferencing in the future.
  • Maybe NICE can incorporate this kind of remote access into the guideline for those caring for or for people housebound and bedbound by M.E.? It doesn’t replace the need for ‘at your bedside’ care, but it could present a more accessible route to healthcare services.

Our lives are bad enough when battling M.E. at its worst. Harder still when snatched from relative improvement back into Severe M.E. And, I can only imagine, how devastatingly difficult it must be when suffering with Very Severe M.E.

For people in this situation they can’t even feel the benefits of being part of the M.E. community. But we can be their voice. We can reflect on those times when we too were at our worst. We can listen to what their carers are telling us.

We can determine what we needed then and now from NICE and from the NHS. And we can try to ensure our representatives working in the guideline committee are aware of the results.

What do you want from NICE and the NHS?

Please join our discussions this week or send in your thoughts. We hope to update and make more information available about Severe and Very Severe M.E. based on your feedback this week. And we will be feeding back to NICE the results of the website survey and anything else considered appropriate to their deliberations.

Thank you for reading.

2. The MEA Website Survey: Severe and Very Severe M.E.

We have provided a simple website survey to gather some responses relating to your experience of Severe and Very Severe M.E.

You can find the survey in the right-hand margin of this page. Please take a moment to respond and post any comments or further thoughts on our social media pages.

We will relate the results to NICE at a future meeting.

Have you experienced Severe or Very Severe ME? If so, for how long were you affected to this extent? Please visit the MEA blog for severity definitions etc.

  • Up to a year
  • 1-2 years
  • 3-5 years
  • 5-7 years
  • 7-10 years
  • 10 years +
  • Whenever I relapse I experience Severe ME
  • Whenever I relapse I experience Very Severe ME
  • I have never experienced Severe ME
  • I have never experienced Very Severe ME
  • Other – please leave a comment on MEA Facebook.

3. What are the symptoms?

Symptom intensity and illness severity

Not everyone with Severe and Very Severe M.E. has the same collection of symptoms. Nor do they have the same intensity of individual symptoms all the time.

For some people generally feeling weak and unwell with flu-like symptoms can be most disabling. For others it might be pain, cognitive dysfunction, extreme sensitivity to stimulation, nausea, dizziness, difficulties maintaining sleep or oversleeping, communicating; or more likely a combination of them all.

The pattern and nature of individual symptoms and their intensity, which can vary from mild and moderate right through to severe, can often change over the course of time.

In terms of illness severity and functional impairment, some people may never experience the most severe end of the spectrum, while for others this degree of impairment is all they have known.

People can go from experiencing severe functional limitations to moderate to mild and back again following relapses and post-exertional malaise. Individual symptoms and general feelings of being extremely unwell will also vary in intensity during these periods.

This unpredictable pattern can be the norm for many people struggling to cope with M.E. and it can occur across many years, even across a lifetime. The unpredictable nature of a fluctuating illness like M.E. can be extremely hard to manage emotionally as well as physically.

Relative recovery from a period of worse health to a previously attained level of improved function and symptom stability, can be difficult to achieve even with careful management and care.

For some it is not possible, and they and their families must learn to manage as best they can, ensure that the right support is in place and that the person with M.E. is kept as comfortable as possible.

Below we have grouped together the main symptoms that are consistent with severe and very severe M.E.

  • Activity-induced muscle fatigue – where even very minimal amounts of activity can produce what is called post-exertional malaise or postexertional symptom exacerbation.
  • Cognitive dysfunction – involving problems with short-term memory, concentration, attention span. This leads to difficulties with all kinds of normal mental functioning – computers, reading, writing and conversations.
  • Dietary limitations and food intolerance.
  • Gastrointestinal problems – including irritable bowel symptoms.
  • Headaches – which may have a migrainous quality.
  • Inability to stand or sit upright (orthostatic intolerance) – accompanied by light-headedness/dizziness and feeling faint or fainting. If such symptoms are accompanied by a rapid rise in pulse rate, then it would be worth asking to be tested for Postural Orthostatic Tachycardia Syndrome (PoTS).
  • Muscle twitching (fasciculations) and spasms.
  • Nausea that may be accompanied by dizziness or feelings of vertigo.
  • Ongoing Flu-like symptoms – including sore throats and enlarged glands, fever-like sweats, and lethargy which is a lack of energy or exhaustion.
  • Pain – which can affect muscles, joints, and nerves – where it is called neuropathic pain.
  • Sensitivity and intolerance to noise (hyperacusis), bright lights (photophobia), perfumes, odours, chemicals, alcohol, and some types of medication.
  • Sensory problems – including loss of sensation, abnormal sensations (paraesthesiae) and increased sensitivity to touch.
  • Sleep problems – sleeping too much, or too little, vivid dreams, night sweats, restless legs syndrome, and sometimes a more severe disturbance of normal sleep rhythm pattern – all leading to non-restorative sleep.
  • Temperature control – including increased sensitivity to temperature extremes (cold hands and feet), sweating, feeling feverish and a general worsening of other symptoms.
  • Visual, swallowing, and communication difficulties.
  • Vitamin deficiency, especially vitamin D.

Very Severe M.E.

As noted in the Chief Medical Officer’s report into CFS/ME (2002, section 4:2:1:2), in addition to the symptoms listed above, people who are very severely affected may experience serious neurological symptoms including double vision, blackouts, atypical convulsions/atypical seizures, loss of speech, and loss of swallowing ability.

Extreme sensitivity to noise and light, muscle wasting, and dramatic weight loss may also occur. Swallowing difficulties may necessitate nasogastric or percutaneous endoscopic gastrostomy (PEG) feeding.

With so many symptoms, it’s not always easy knowing when a new symptom, or a change in the severity or characteristic of an existing symptom, is due to M.E. or to something else.

But when a change is recognised, it is important that someone speaks with your doctor or specialist to make sure that it’s not due to another medical problem that can be treated.

In particular, low thyroid function (hypothyroidism) or type-2 diabetes – which have a number of symptoms that overlap with M.E. – noticeable weight-change, any of the neurological symptoms above, and any marked deterioration in communicative ability.

Equally, it’s important to note that a progressive deterioration of symptoms is not a normal finding in M.E. So, when this occurs, there should always be a thorough medical re-assessment.

4. What do we mean by Severe and Very Severe M.E.?

In the absence of definitive illness severity definitions, the ME Association recommends use of the ones created by Dr Diane Cox and Professor Leslie Findley which were published in 1998.

They sub-grouped people with M.E. into FOUR severity definitions (Mild, Moderate, Severe, Very Severe). While not wholly encompassing of the levels of disability and of extreme symptoms in these latter two groups, these are better than the current NICE definitions.

The Cox & Findley definitions were built upon by members of the CFS/ME Working Group which included Dr Charles Shepherd from the ME Association, and were published in an influential Report to the Chief Medical Officer in 2002.

This report directly influenced the Government’s decision to fund a network of NHS specialist services in England, and the subsequent creation of the NICE clinical guideline on ME/CFS in 2007.

Mild 
Are mobile and can care for themselves and can do light domestic tasks with difficulty. The majority will still be working. However, in order to remain in work, they will have stopped all leisure and social pursuits, often taking days off. Most will use the weekend to rest in order to cope with the week.

Moderate 
Have reduced mobility and are restricted in all activities of daily living, often having peaks and troughs of ability, dependent on the degree of symptoms. They have usually stopped work and require rest periods, often sleeping in the afternoon for one or two hours. Sleep quality at night is generally poor and disturbed. 

Severe 
Will be able to carry out minimal daily tasks only, face washing, cleaning teeth, have severe cognitive difficulties and be wheelchair dependent for mobility. These people are often unable to leave the house except on rare occasions with severe prolonged after-effect from effort. 

Very Severe
Will be unable to mobilise or carry out any daily tasks for themselves and are in bed for the majority of the time. These people are often unable to tolerate any noise and are generally extremely sensitive to light.

The NICE clinical guideline on ME/CFS

The current NICE clinical guideline (e.g. Appendix D) only defines THREE categories of illness severity: Mild, Moderate and Severe.

While they have clearly used the definitions from the 2002 Report to the Chief Medical Officer, they chose to amalgamate Severe and Very Severe into one category.

Mild 
People with mild CFS/ME are mobile, can care for themselves and can do light domestic tasks with difficulty. Most are still working or in education, but to do this they have probably stopped all leisure and social pursuits. They often take days off or use the weekend to cope with the rest of the week.

Moderate
People with moderate CFS/ME have reduced mobility and are restricted in all activities of daily living, although they may have peaks and troughs in their level of symptoms and ability to do activities. They have usually stopped work, school or college and need rest periods, often sleeping in the afternoon for 1 or 2 hours. Their sleep at night is generally poor quality and disturbed.

Severe
People with severe CFS/ME are unable to do any activity for themselves or can carry out minimal daily tasks only (such as face washing, cleaning teeth). They have severe cognitive difficulties and depend on a wheelchair for mobility. They are often unable to leave the house or have a severe and prolonged after-effect if they do so. They may also spend most of their time in bed and are often extremely sensitive to light and noise.

In effect this means that Very Severe is not recognised to the extent that we feel it should and this could be having a negative effect on healthcare provision and understanding from the medical profession.

For example, there are no inpatient facilities for people with M.E., home visits from GPs remain difficult to obtain, specialist nurses are almost non-existent, and personalised care needs are not well defined or resourced.

We think that this decision by NICE to exclude Very Severe was wrong and we will be seeking the reinstatement of the four severity definitions in the new guideline.

And, while we don’t believe that reinstatement will immediately lead to improved healthcare provision, we hope that this change and the resulting discussion might lead to a more encompassing and relevant framework.

  • What do you think? Join our discussions on social media this week, or send in your thoughts: feedback@meassociation.org.uk and we will pass them on to NICE if at all possible.

Disability Rating Scale

The ME Association since expanded these definitions to create a disability rating scale (below).

It can help you and your carer to better gauge your level of disability and progress, and might be used when speaking with, for example, the NHS, Department for Work and Pensions, and your local authority about Social Care.

VERY SEVERE
100% DISABLED
Severe symptoms – often on a continual basis. Cognitive function (i.e. short-term memory, concentration, attention span) is likely to be very poor. Bedridden and incapable of living independently. Requires a great deal of supervision and practical support – including disability aids such as a hoist or a stair lift – with all aspects of personal care (i.e. feeding, dressing, washing) on a 24-hour basis.

90% DISABLED
Severe symptoms – often including marked cognitive dysfunction, for much or all of the time. Bedridden and housebound for much or all of the time. Has considerable difficulties with all aspects of personal care. Unable to plan or prepare meals. Requires practical support and supervision on a 24-hour basis.

SEVERE
80% DISABLED
Moderate to severe symptoms for most or all of the time. Only able to carry out a very limited range of physical activities relating to personal care without help. Requires help with meal planning and preparation.
Frequently unable to leave the house and may be confined to a wheelchair when up or spends much of the day in bed. Unable to concentrate for more than short periods of time. Usually requires daytime and night-time supervision.

70% DISABLED
Moderate to severe symptoms for most or all of the time. Confined to the house for much or all of the time. Normally requires help with various aspects of personal care and meal planning and preparation, possibly on a 24- hour basis. Very limited mobility. May require wheelchair assistance.

MODERATE
60% DISABLED
Moderate symptoms for much or all of the time. Significant symptom exacerbation follows mental or physical exertion. Not usually confined to the house but has significant restrictions on mobility when outside and may require wheelchair assistance. Likely to require help with aspects of personal care and meal preparation – but not necessarily on a full-time basis. Requires regular rest periods during the day. Unable to resume any meaningful regular employment or education.

50% DISABLED
Moderate symptoms for much or all of the time. Symptom exacerbation follows mental or physical exertion. Not usually confined to the house but mobility restricted to walking up to a few hundred yards at best. May require help with some aspects of personal care. May require help with meal planning and preparation. Requires regular rest periods during the day. Able to carry out light activities (i.e. housework, desk work) linked to normal daily living for short periods but not able to resume regular employment or education.

40% DISABLED
Moderate symptoms for some or much of the time. Normally able to carry out most activities linked to personal care and normal daily living but may require assistance with meal preparation. May be able to cope with some work-related tasks for short periods – provided they are not mentally or physically strenuous – but not able to resume regular work or education.

30% DISABLED
Fluctuating level of mild to moderate symptoms. Normally able to carry out all aspects of personal care and to plan and prepare meals. Able to walk short distances on a regular basis. May be able to return to work on a flexible or part-time basis – provided adjustments are made to cope with physical activity or cognitive problems. May have to stop leisure or social pursuits to resume work or education.

MILD
20% DISABLED
Normally only mild symptoms at rest but exacerbation will follow activity. Able to carry out all aspects of personal care and to plan and prepare meals. Able to walk short to medium distances (i.e. up to half a mile) on a regular basis. Normally able to return to flexible or part-time work or education.

10% DISABLED
Generally well with only occasional mild symptoms. No problems with personal care or daily living. Mobility and cognitive functions may still be restricted but almost back to previous levels. May be able to return to full-time work or education.

0% DISABLED
Fit and well for at least the past three months. No symptoms at rest or after exertion. Capable of full-time employment or education.

5. Prognosis and Quality of Life

Research relating to prognosis is scant, but most people with M.E. would seem to fall into one of four broad groups:

1. Those who manage to return to normal health, or near normal health, even though this may take a considerable period of time.

2. The majority who make some degree of improvement but eventually stabilise. They then follow a fluctuating pattern with both good and bad periods of health. Relapses or exacerbations are often precipitated by:

  • Post-Exertional Malaise (PEM) – a characteristic symptom of M.E.,
  • Infections, operations, temperature extremes, or emotional events.

3. A significant minority of patients who remain severely or very severely affected and may require a great deal of practical and social support.

4. Those who show signs of deterioration. Continued deterioration is unusual. When this occurs, a detailed medical re-assessment is advisable to rule out other possible diagnoses.

For those who don’t experience a significant improvement in health, the condition can maintain a fluctuating course of severity over the course of days, weeks, months, years – even decades.

Symptoms will fluctuate as will the level of discomfort and disability they bring.

You might not experience severe symptoms or severe functional impairment to the same extent all the time, but you likely will when the illness is most acute such as in the initial pre- and post-period of diagnosis, during a relapse, because of post-exertional malaise or other triggers (see above).

For some, severity of symptoms and the reduction in functional ability appears progressive, and their health can get worse over time. We don’t know why this happens; it remains one of the many unanswered questions about M.E.

Severely affected

Those severely affected will likely be housebound and require long periods in bed because they are unable to do otherwise.

Employment and education will most likely have to be curtailed or significantly reduced in scope and complexity.

They are also likely to require a wheelchair or other aid to help them mobilise, even when inside because standing and walking will be an issue.

And they will require help and support to cope with household chores and other activities such as eating and with personal care such as bathing.

Home visits from healthcare professionals will be required and particular attention to diet and nutrition will be needed. Periods of sensitivity to light, noise, and touch will make coping, caring, and communication, a challenge.

Very severely affected

The very severely affected will be bedbound for all or most of the time and require full-time care and support.

They will be unable to mobilise or sit upright in bed, to work or take part in any formal education. It is very likely that all responsibilities will need to be fulfilled by a family member and it will be necessary to employ a professional to provide homecare i.e. social care.

Home visits from health professionals will be needed, special attention to diet and nutrition– tube-feeding may be necessary – and extra assistance with toileting and sanitary needs will be important.

Prolonged periods of sensitivity to light, noise, and touch will make coping, caring, and communication, a very real challenge.

Research examining prognosis

Recent research into prognosis is scant. But several studies have been published that provide some idea of the possible outcomes for people diagnosed with M.E.

Results from these studies indicate that M.E. often becomes a chronic and very disabling illness with complete recovery only occurring in a small minority of cases.

  • A systematic review of 14 studies (Cairns and Hotopf 2005) found a median full recovery rate during the follow-up periods of 5%, and the median proportion of patients who improved during follow-up was 39.5%.

The Chief Medical Officer’s report (CFS/ME Working Group 2002; in section 1.4.3) noted that:

Prognosis is extremely variable. Although many patients have a fluctuating course with some setbacks, most will improve to some degree. However, health and functioning rarely return completely to the individual’s previous healthy levels; most of those who feel recovered stabilise at a lower level of functioning than before the illness….

Overall, there is wide variation in the duration of illness with some people recovering in less than two years while others remain ill after several decades. Those who have been affected for several years seem less likely to recover; full recovery after symptoms persist for more than five years is rare.

Strassheim et al (2017) carried out a scoping review to assess what is known about Severe and Very severe M.E. The review highlighted the limited research focusing on the severely affected M.E. population.

The heterogeneity (diverse nature) of the condition contributed to the lack of consensus concerning definitive diagnostic criteria and functional measures to assess disability.

Strassheim concluded that focused research to understand the disease characteristics of the most severely ill will help to advance our understanding of possible phenotypes associated with distinct severity categories.

Quality of life

Again, research here is lacking particularly in regard to people severely and very severely affected because they are not ambulatory and cannot take part in research away from their homes.

However, studies that have examined functional status and quality of life measures confirm that the scale of impairment across a range of physical and mental activities can be just as great or greater than in many other chronic medical conditions.

One study, from Nacul et al (2011a) in the UK, reported that:

M.E. is as disabling and has a greater impact on functional status and well-being than other chronic diseases such as cancer.

The emotional burden of M.E. is felt by lay carers as well as by people with M.E.

A study (Pendergrast et al 2016), from a research group at Newcastle University, reported that people with M.E. who are housebound are significantly more impaired in relation to physical functioning, bodily pain, vitality, social functioning, fatigue, post-exertional malaise, sleep, pain and neurocognitive, autonomic, neuroendocrine (hormonal) and immune system functioning than people with M.E. who are not housebound.

The most recent study on quality of life (Kingdon et al 2018), using anonymised clinical data from people with M.E. and multiple sclerosis who donated blood samples to the ME Biobank; reported that people with M.E. were measurably more disabled than people with multiple sclerosis or healthy controls. They also worked fewer hours and had lower incomes compared to those in the other two groups.

The ME Biobank at the Royal Free Hospital, which the ME Association supports with grant funding, is collecting blood samples from people with M.E. in their homes. This will enable researchers to analyse blood samples and clinical details in future studies about Severe and Very Severe M.E.

The ME Association recently announced the funding of a feasibility study that aims to measure physiological changes in the daily activity of people with M.E. at home. It will be a study that includes people with Severe M.E.

Participants will wear a portable VO2 system during some activities, as well as a heart rate monitor and an accelerometer continuously throughout the day. In addition, lactic acid levels and blood pressure will be taken regularly throughout the study period.

It is the first time research of this type has been conducted in the UK and we hope it will quantify the disability felt by people with M.E. who are unable to leave their homes.

6. What does the current NICE clinical guideline recommend?

  • The National Institute of Health and Care Excellence (NICE) Clinical Guideline on ME/CFS: CG53 (2007). Online version. Pdf Download.

General principals applicable to everyone

From the guideline introduction

‘Chronic fatigue syndrome (CFS)/myalgic encephalomyelitis (or encephalopathy) (ME) is a relatively common illness. The physical symptoms can be as disabling as multiple sclerosis, systemic lupus erythematosus, rheumatoid arthritis, congestive heart failure and other chronic conditions. CFS/ME places a substantial burden on people with the condition, their families and carers, and hence on society…’

‘CFS/ME comprises a range of symptoms that includes fatigue, malaise, headaches, sleep disturbances, difficulties with concentration and muscle pain. A person’s symptoms may fluctuate in intensity and severity, and there is also great variability in the symptoms different people experience. CFS/ME is characterised by debilitating fatigue that is unlike everyday fatigue and can be triggered by minimal activity. This raises especially complex issues in adults and children with severe CFS/ME.’

‘CFS/ME, like other chronic conditions for which the causes and disease processes are not yet fully understood, poses significant problems for healthcare professionals. It can cause profound, prolonged illness and disability, which has a substantial impact on people with CFS/ME and their carers. Uncertainties about diagnosis and management, and a lack of clinical guidance for healthcare professionals, have exacerbated this impact.’

‘The recommendations in this guideline emphasise the importance of early symptom management, making an accurate diagnosis, ensuring that significant clinical features are investigated, and working in partnership with people with CFS/ME to manage the condition. Different combinations of approaches will be helpful for different people.’

1.9 Key principles of care for people with Severe ME

1.9.1 General principles of care

1.9.1.1 Management of severe CFS/ME is difficult and complex and healthcare professionals should recognise that specialist expertise is needed when planning and providing care for people with severe CFS/ME.

1.9.1.2 Diagnosis, investigations, management and follow-up care for people with severe CFS/ME should be supervised or supported by a specialist in CFS/ME.

1.9.1.3 People with severe CFS/ME may need to use community services at times. These services may include nursing, occupational therapy, dietetics, respite care, psychology and physiotherapy (see the ‘National service framework for long-term conditions'[11]). The input of different professionals should be coordinated by a named professional.

1.9.1.4 People with severe CFS/ME should be offered a summary record of every consultation because of their cognitive difficulties.

1.9.1.5 Most people with CFS/ME will not need hospital admission. However, there may be circumstances when a planned admission should be considered. The decision to admit should be made with the person with CFS/ME and their family, and be based on an informed consideration of the benefits and disadvantages. For example, a planned admission may be useful if assessment of a management plan and investigations would require frequent visits to the hospital.

1.9.2 Rest

1.9.2.1 When making decisions about prolonged bed rest, healthcare professionals should seek advice from a specialist experienced in the care of people with severe CFS/ME. The significant physical and psychological risks associated with prolonged bed rest should be taken into account.

1.9.2.2 Healthcare professionals working with people with severe CFS/ME who are in bed most (or all) of the time, should explain the associated risks (such as postural hypotension, deep venous thrombosis, osteoporosis, pressure sores and deconditioning) and monitor these.

1.9.3 Management approaches

1.9.3.1 People with severe CFS/ME should be offered an individually tailored activity management programme (see recommendation 1.6.2.22) as the core therapeutic strategy, which may:

– be delivered at home, or using telephone or email if appropriate

– incorporate the elements of recommendation 6.2.22 and draw on the principles of CBT and GET (see recommendations 1.6.2.1–21).

1.9.3.2 An activity management programme should be reviewed regularly and frequently.

Note: 4.1 Extrapolating use of intervention strategies

Are intervention strategies that have been shown to be effective in mildly to moderately affected adults also effective in children and in people (adults and children) with severe CFS/ME?

Why this is important
There is limited evidence for the use or effectiveness of strategies recommended in this guideline in these two patient groups.

Population data suggest that these groups constitute a significant percentage of the population with CFS/ME. Some patient experience suggests that some of these interventions may be harmful and/or not effective.

7. How many people are likely to experience Severe M.E.?

Prevalence

The ME Association believe that between 133,594 – 267,187 adults and children in the UK suffer from ME/CFS.

  • We have no conclusive research about severity, but it has long been estimated that around 25% (33,398 – 66,796) of people with ME/CFS at any one time have Severe M.E.
  • And of these perhaps 2% (2,672 – 5,344) will be Very Severely affected and in need of most care and support.

This means that ME/CFS is not uncommon, in fact it has a higher prevalence than Multiple Sclerosis, which makes it even harder to understand why there is not the same level of support from the government and NHS.

(Based on 0.2-0.4% ME/CFS prevalence: 66,796,800 total UK population as at mid-2019 (ONS estimate, June 2020))

Nomenclature: A disease of many names

Most people with the condition prefer the term myalgic encephalomyelitis or myalgic encephalopathy, abbreviated to M.E. Both indicate problems with muscle and brain function.

The term ‘encephalomyelitis’ is not a pathologically proven explanation for what may be happening within the nervous system but it is an historic term associated with the condition. 

Encephalomyelitis often causes dissent in the medical profession and some preferred to use ‘chronic fatigue syndrome’ – a term that the ME Association considers to be inappropriate, demeaning, and unrepresentative of the patient experience.

We therefore proposed an alternative term, ‘encephalopathy’, meaning a significant disorder of brain function, until such time as research determines an actual cause for the symptoms.

Myalgic Encephalopathy is arguably a more appropriate way of describing the muscle symptoms – aches, pains, weakness – (myalgia) and the various abnormalities affecting hypothalamic, autonomic, and cognitive function and in cerebral perfusion (encephalopathy); that have been reported in the research literature.

We still consider the disease to be largely neurological but other bodily systems will also play a role. This alternative term has been accepted by the National Institute of Health and Care Excellence (NICE).

The World Health Organisation features Post-Viral Fatigue Syndrome (PVFS) as the parent term under diseases of the nervous system (i.e. neurological) in the International Classification of Diseases (ICD).

PVFS is another historic term that is still used to describe prolonged impairment following an infection (one of the most common routes to a diagnosis of M.E.). M.E. and CFS are both included as alternative descriptions for PVFS in WHO ICD.

The SNOMED CT, a clinical classification used by the NHS in the UK, has adopted the same terms as the WHO ICD.

While we continue to try and change certain medical attitudes, and research tries to find more conclusive explanations for disease pathology, the abridged term ME/CFS has become an accepted compromise.

8. Further information and support.

Resources available from the ME Association

ME Connect Telephone Helpline

ME Connect is the ME Association’s flagship service offering support and information to anyone affected by ME/CFS.

It is available every day of the year and has managed more than 35,000 calls since its inception in 2003.

Each call is dealt with sensitively, professionally and, perhaps most importantly, in total confidence by members of our fully trained team of volunteers – most of whom have personal experience of ME/CFS.

You can phone ME Connect on 0344 576 5326 during the hours of 10am-12noon, 2pm-4pm and 7pm-9pm, any day of the week, 365 days a year.

Please refer to our website for further details. All communications are treated in the strictest of confidence.
Calls cost the same as other standard landline numbers (starting 01 or 02). If you have a call package for your landline or mobile phone then calls will normally come out of your inclusive minutes.

ME Association Information Resources

The ME Association produces a wide range of leaflets relevant to Myalgic Encephalomyelitis/Encephalopathy or M.E.

It is the most extensive range available on the internet and all leaflets are regularly reviewed and updated. We also aim to cover developments and topics that are newly relevant to the community, such as the free range of Covid-19/Coronavirus leaflets.

Leaflets are produced by Dr Charles Shepherd, Hon. Medical Adviser to the ME Association, other advisers and topic experts who provide their help voluntarily or who are employed by the charity.

We make a nominal charge for the leaflets to help support the charity, and to cover postage costs where applicable, while ensuring a fair number are available free of any charges.

You can access the whole range of literature via our website shop where your selections can be purchased and then downloaded. Once the Covid-19 restrictions are lifted we will again be happy to send orders to you in the post if you prefer.

The following is a selection of what we think are useful leaflets, booklets and blogs aimed at carers and for people with Severe and Very Severe M.E.

Free Factsheets

M.E. Factsheet – What you need to know about M.E.
M.E. Research Summary – Understanding the pathology of M.E.
The DecodeME Genetics Study – Register your interest now!

Leaflets, Guides, Booklets and Blogs

The Importance of an Early and Accurate Diagnosis

Guide to coping with Severe ME

What is the prognosis for someone severely affected by ME?

Caring for a person with ME
Energy Management and Pacing
Going into hospital if you have ME/CFS
How to obtain a specialist referral
Prognosis, Permanency and Obtaining an Ill-Health Pension

Benefits and tax credits – An introduction
Blue Badge Scheme – An introduction
Carers Allowance
Disabled facilities grant
Getting help from social services
Guide to Employment Support Allowance (ESA)
Guide to Personal Independence Payment (PIP)
Guide to Reconsiderations and Appeals (ESA & PIP)

Medical Management

We have a full range of leaflets dealing with medical management e.g. symptoms, commonly prescribed drugs, vitamins and supplements, with new leaflets being added all the time.

To Whom it May Concern Letters

We offer a range of letter templates for when you need to explain to others how M.E. can affect your ability to do things.
Blue Badge and Wheelchair Applications
Disability Aids, Adaptations and Services
Examinations – Making special arrangements
Immunisations as possible triggers

The MEA Clinical and Research Guide (‘The Purple Book’)

The authoritative and vital ‘ME/CFS/PVFS An Exploration of the Key Clinical Issues‘ by ME Association Hon. Medical Adviser, Dr Charles Shepherd, and consultant neurologist, Dr Abhijit Chaudhuri.

An invaluable and fully referenced resource for medical professionals, patients and carers.

Please note that due to Covid restrictions and limited staffing at head office we are not currently able to post hard copies of the booklet. However, it remains available as a Kindle version on Amazon priced at £9.00.

We will very shortly be publishing the 2020 edition of this guide, so you might want to hold-fire on any purchases for the next week or so.

ME Association Facebook

You might consider sending any questions you may have on aspects of care or life with M.E. to our Facebook page. We regularly feature such questions (anonymously if required) as it can prove very helpful to share experiences. Simply ‘like’ our page and use messenger to send us a question.

Covid-19 Infection, Lockdown and ME/PVFS

During the unprecedented Covid-19/Coronavirus pandemic, we have ensured that people with M.E. – and those who continue to experience symptoms following infection – are kept informed of the latest guidance and related information.

All the leaflets in this range are free to download. We periodically review them and update when new information emerges or the situation regarding the national lockdown alters.

Recommended External Resources

If you are looking for recommended guides to caring for someone with Severe or Very Severe M.E. written by people with first-hand experience then please consider:

Emily Collingridge

Severe ME/CFS: A Guide to Living

Patients with very severe M.E. have innumerable questions about how to cope with their situation and often have nowhere to turn for appropriate help. 

This guide sets out to answer as many of those questions as possible.  The advice is based on the collective experiences of patients, carers and professionals and covers a huge variety of topics including personal care, mobility, symptom and activity management, hospital admission, tube feeding, emotions and entertainment.

The guide is designed to be a definitive reference tool for anyone whose life is affected by severe ME, whether personally or professionally.  It covers numerous medications, types of equipment and other tools for helping patients with ME and those who care for them. 

Whatever the crisis, it is hoped that a patient of any age will find reassurance and coping strategies within the pages of this guide.

“Never before has such extensive, understanding, empowering and detailed help been available for those with severe ME.  With a wealth of information on almost every symptom and problem imaginable, it is a book that no severe ME sufferer should be without,” Frances Goodchild.

“Our son was devastated that family members could not understand what he was going through – had they been able to read this book, it could have been so different,” Mary.

Stonebird – Greg and Linda Crowhurst

Severe M.E. Notes for Carers

“One of Greg’s most important works …the information here isn’t dry or wooden, it lives and breathes,” Kara Jane Spencer.

“This book is a beautifully written work. It captures the very essence of what it means to care for a severely ill person. Thoughtfully, tenderly put together and very importantly, it gives room for the views of the poorly person too, ” Nicky Baker.

Visit the Stonebird website to view and to purchase more of Greg’s important contributions to our understanding of caring for people with Severe and Very Severe M.E.

The 25% M.E. Group

The 25% M.E. Group exists to support all who have the severe form of M.E. and those who care for them. This includes people who are housebound, bedbound and wheelchair users.

The 25% M.E. Group is a unique nationwide community based voluntary group. We have two paid members of staff and a number of volunteers – most of whom have M.E. We provide a range of services to people affected by severe M.E. (Myalgic Encephalomyelitis).

Because of the intensity of the symptoms and disabilities experienced by severe M.E. sufferers we seek to alleviate the isolation which having this illness can cause. 

The 25% ME Group encourages: communication between members; participation in the Group at a number of levels; assistance with articles and information for the newsletter etc. These are just some of the initiatives employed by the group.

The ME Association

Please support our vital work

We are a national charity working hard to make the UK a better place for people whose lives have been devastated by an often-misunderstood neurological disease.

If you would like to support our efforts and ensure we are able to inform, support, advocate and invest in biomedical research, then please donate today.

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Just click the image opposite or visit our JustGiving page for one-off donations or to establish a regular payment. You can even establish your own fundraising event.

Or why not join the ME Association as a member and be part of our growing community? For a monthly (or annual) subscription you will also receive ME Essential – quite simply the best M.E. magazine!


ME Association Registered Charity Number 801279


ME CONNECT HELPLINE

0344 576 5326

Available every day of the week between these times: 10am - 12noon, 2pm - 4pm and 7pm - 9pm.

Calls cost the same as other standard landline numbers (starting 01 or 02). If you have a call package for your landline or mobile phone then calls will normally come out of your inclusive minutes.