By Russell Fleming, Content Manager, ME Association.
Severe ME Day is generally regarded as a time of remembrance for those who had been members of our community but were taken from us before their time. It is also a time to raise awareness and consider how we might improve the care and support for this most vulnerable and neglected patient group.
Personally, I will once again be remembering Vanessa Li with whom I worked in the early days of the Microbe Discovery Project. She was so keen and passionate about the future for ME research – it was her initial idea that set the wheels in motion for a crowdfund project with Professors Ian Lipkin and Mady Hornig from Columbia. I can’t say I knew Vanessa well, but she left a positive impression on me and is someone I don’t think I will ever forget.
One of the key difficulties we face as a community is getting our message heard by those who influence our care and by others in wider society. Raising awareness and understanding within our community is relatively straightforward.
But for someone severely affected – and even more so for someone very severely affected – this disease renders their voices mute, even to the extent of not being able to communicate basic needs.
In their absence – or even with their occasional input – those of us more able must be their voice. But how can we improve things for those who are most neglected? And what changes should we strive to make? It is not a straightforward campaigning decision.
For many of us, the spectre of severe ME is ever present. By its very nature ME is a fluctuating condition and we can relapse at any time.
For others, it seems they are held in the grip of severe ME and are unable to move forward, or severe ME is the only experience of the illness they have had.
And for those who are most vulnerable, those with very severe ME, the future can appear very bleak indeed.
Prevalence: How many people might be severely affected by ME?
In truth, we don’t know. Research is still incredibly patchy, and it shouldn’t really matter as every single person is important, but people do like figures so we can make some educated guesses:
131,280 – 262,560 UK prevalence of ME in adults and children
25% severely affected by ME = 32,820 – 65,640 adults and children
2% very severely affected by ME = 2,625 – 5,251 adults and children?
I think we can now feel sufficiently confident about what comprises ME to more openly promote the clinical criteria – which may be of benefit to those languishing without a diagnosis.
Recognising the full implications of a diagnosis can lead to more appropriate care and support, and to treatments that might be tolerated by, and be of benefit to, people with severe ME.
(0.2-0.4% ME/CFS prevalence. 65.64million UK population as at 2016 (World Bank estimate))
What can we do together to bring about positive change?
If you have been severely affected by ME, or are currently severely affected, or are a carer or family member of someone severely or very severely affected, what changes would you like to see to the care that you (or the person you care for) receive from the NHS?
Perhaps a campaign aimed at:
- improving understanding and public awareness that ME can be so devastating as to confine someone to their bed, or to being wholly reliant on personal care, or tube-feeding, would be a step in the right direction? If so, then how might we achieve this?
- NICE (National Institute for Care and Clinical Excellence) and the NHS, pointing out that GPs and specialist services are not meeting even the minimum recommendations laid down in the clinical guideline for people severely affected (McDermott et al. 2014)?
- better ensuring the NICE principles of personally tailored care and shared decision making, and the patients’ right to refuse or to withdraw from any aspect of their care and not have it affect their ongoing relationship with the NHS, are recognised and respected?
- ensuring everyone receives a personally tailored and mutually agreed care plan?
- making home visits from NHS ME/CFS specialists a reality for all people severely affected?
- more specific changes to domiciliary care and support that you think should be offered by the NHS for people severely and very severely affected by ME?
Or perhaps you or the person you care for doesn’t receive any care from the NHS, in which case what might we do to change that situation and what kind of care and support do you think is most appropriate?
If you can think of ways in which we can work together to bring the seriousness of ME and the plight of those severely and very severely affected to the attention of those in charge of healthcare, and bring about positive change – then please share your thoughts in the comments section below or on the ME Association Facebook page.
It need not be a campaign, or major offensive. It could be something on a smaller scale – which might stand a better chance of meeting with success.
In future years, on Severe ME Day, we might then look back and see that things for the most neglected members of our community have at the very least improved.
Menu to other sections:
Part One: What do we mean by severe and very severe ME?
Part Two: Resources available from the ME Association
Part Three: Other resources
Part Four: Severe ME research in the UK
Part Five: The NICE Guideline on ME/CFS
Part Six: Report to the Chief Medical Officer
What do we mean by severe and very severe ME?
As with prevalence, there are no agreed upon definitions for illness severity, and the degree to which symptoms can affect an individual do vary, as does the level of disability. There have been some attempts to quantify severity and some of these are shown below – but it can be very unique to the individual:
2007 NICE guideline ME/CFS
‘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.'
(Appendix D: definitions)
2002 Report to the Chief Medical Officer on ME/CFS
The ME Association most closely identifies with the definitions of illness severity outlined in the CMO report:
‘The term ‘severely affected’ has been widely applied to patients whose physical disability is most severe, leading to serious restrictions in mobility and functioning. In many, these restrictions are accompanied by other markers of severity, such as cognitive impairment or prolonged course.'
‘This degree of physical restriction, especially if prolonged, has profound effects on personal and social functioning, which in turn substantially affects the patient’s ability to access health and social services, and has an impact on the patient’s carers.'
‘A recent description* has suggested four categories of severity in CFS/ME (Mild, Moderate, Severe, and Very Severe), although care must be taken not to diminish inadvertently the experience of any patient by descriptors of severity.’ (3.4.3 Severity)
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.
(*Cox DL, Findley LJ. Management of chronic fatigue syndrome in an inpatient setting: presentation of an approach and perceived outcome. 1998)
ME Association Disability Rating Scale
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.
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.
Resources available from the ME Association
ME Connect 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 contact ME Connect by phoning 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 at the link above for further details and be advised that call charges to 0844 numbers are determined by your telephone provider. Check your contract for charging details. It is likely to be much cheaper calling ME Connect from a landline than a mobile phone.
ME Association Information Resources
You can access the whole range of literature via our online shop where your selections can be purchased and downloaded, or by phoning head office on 01280 818964 between 9.30am and 3.00pm.
The following are a selection of what we think are useful leaflets for carers and people severely affected:
What is the prognosis for someone severely affected by ME?
Benefits and tax credits
Blue Badge Scheme
Disabled facilities grant
Getting help from social services
Guide to Employment Support Allowance (ESA)
Guide to Personal Independence Payment (PIP)
Disability Aids, Adaptations and Services
A ‘To whom it may concern’ letter advising on supporting evidence for the provision of disability aids such as wheelchairs and adaptations within homes for people with ME.
ME/CFS/PVFS An Exploration of the Key Clinical Issues by ME Association Medical Adviser, Dr Charles Shepherd, and consultant neurologist, Dr Abhijit Chaudhuri. An invaluable, fully referenced and up-to-date, resource for medical professionals, patients and carers.
And 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.
ME Association Facebook
You might also consider sending any questions you may have on aspects of care or life with ME 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.
The 25% Group
Severe ME Day 2017: ‘Your illness does not define you. Your strength and courage does.’
Severe ME/CFS, A Guide to Living.
Severe ME, Notes for Carers.
Natalie Boulton and Josh Biggs
‘Voices from the Shadows’ an award-winning film about severe ME, now available to watch for free.
Severe ME research in the UK
ME Association and Professor Elisa Oltra in Spain
Biomarkers and markers of disease activity in people with severe ME/CFS.
ME Association and UK ME/CFS Biobank
The Biobank has obtained blood samples and clinical data from severely affected people by visiting them at home for use in its own research projects and to make available to other researchers who might wish to use them.
ME Association and Professor Derek Pheby
Risk Factors for Severe ME (2009), ‘Early management of the illness appeared the most important determinant of severity. Having a mother with ME/CFS was also important. Smoking and personality were not risk factors, neurotic traits being more frequent among the less severely ill. Conscientiousness overall was not related to severity.'
ME Research UK and Newcastle
Professor Newton’s team has initiated an exploratory study of the severely affected.
The NICE guideline on ME/CFS
The NICE guideline sets out a series of recommendations for healthcare professionals with regard to the care of people with ME/CFS.
It can be a useful reference when speaking with your GP or healthcare professional particularly in relation to tailored care packages, your right to refuse or withdraw from care or treatment, and shared decision making – as well as for other matters.
However, overall the ME Association considers the guideline to be ‘not fit for purpose' especially in relation to the recommendations for illness management and with regards to those who are severely affected.
The 2017 ME Association petition against the current NICE guideline and the recommendations set out in its review, together with our submission to the ongoing consultation, were featured in a recent blog.
From the introduction to the NICE guideline on ME/CFS:
‘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 CFS/ME
1.9.1 General principles of care
18.104.22.168 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.
22.214.171.124 Diagnosis, investigations, management and follow-up care for people with severe CFS/ME should be supervised or supported by a specialist in CFS/ME.
126.96.36.199 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'). The input of different professionals should be coordinated by a named professional.
188.8.131.52 People with severe CFS/ME should be offered a summary record of every consultation because of their cognitive difficulties.
184.108.40.206 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.
220.127.116.11 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.
18.104.22.168 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
22.214.171.124 People with severe CFS/ME should be offered an individually tailored activity management programme (see recommendation 126.96.36.199) 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 188.8.131.52–21).
184.108.40.206 An activity management programme should be reviewed regularly and frequently.
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.
In 2002 a report to the Chief Medical Officer from an Independent Working Group on ME/CFS – that was partly used in the production of the NICE guideline – contained a more detailed and empathetic review of severe ME and also highlighted some of the problems faced by carers, for example:
‘Having a loved one affected by CFS/ME has a profound effect on every part of life. Many carers reported that their world felt it had been “turned upside down” and they had feelings of “despair”.'
‘The difficulties experienced by patients around the widespread disbelief and misunderstanding compound problems for carers. One important aspect for carers – considered by some to be the most difficult to come to terms with – is the uncertainty about restoration of the previous level of functioning, and restructuring of both the patient’s and carer’s lives.'
‘Increased access to respite care would have a substantial positive impact on carers. Otherwise, for carers, particularly for those looking after severely affected patients, there is no rest.'
‘Some carers were clearly distressed about being ignored by GPs, and some reported unpleasant behaviour. Some reported that treatment regimes were occasionally advocated by clinicians against “expressed” wishes leading to loss of trust.'
‘In contrast, some reported supportive GPs who admitted their limited knowledge and “feeling out of depth” with the illness, and treated the family with respect. Carers respect honesty and prefer not to be given an over-optimistic prognosis.'
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