Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME-CFS): good practice statement

5. People who are more severely affected

The Report of the 2002 Chief Medical Officer (England) CFS/ME Working Group noted that not enough was known about severe forms of the illness: "that are reported to affect up to 25% of patients". A search of the scientific literature, has not been able to validate this estimate as no definitive studies have been carried out yet in the UK to determine the prevalence of severe disease. This also applies for less severe forms of ME-CFS. Recognising the heterogeneity and complexity of this area, research studies are therefore urgently required to inform our understanding of the prevalence, prognosis, impact and optimal management of individuals with severe disease (see also Section 6).

During preparation of this guidance many individuals kindly contributed by providing a number of comments on a previous draft version. Many of these comments came from individuals who have lived with severe ME-CFS over a number of years or who have cared for a relative/loved one with severe illness. A number of respondents also generously and confidentially shared specific aspects of their personal history of ME-CFS.

It is clear at some stage in their illness that many patients may fall into the Category of severe ME-CFS, with specific care needs.

It is recognised that the diagnosis and management of severe ME-CFS is an area which needs to be developed in a specific Good Practice Statement. However, the following is intended to provide summary guidance in the interim:

5.1 Severity scale of illness

The quality of life of ME-CFS patients shows marked diminution. However, there is a spectrum of severity. Those who are severely affected have severe restrictions in their mobility and ability to carry out essential daily tasks and attend to personal care. Patients may suffer severe debility or become bedbound from the intensity of one prominent symptom or from a cluster of symptoms such as sore throat, swollen glands and flu-like symptoms.

At its most extreme, patients may be totally bedbound and report constant pain, inability to tolerate movement, light or noise and certain scents or chemicals (including prescribed drugs). They will often spend periods of time bedbound, housebound or wheelchair bound. Some may have ME-CFS for several years, some for many years and some may never recover.

These latter cases account for a minority however, and a cautiously optimistic outlook is justified for the majority of patients.

NICE 2021 Update

The NICE 2021 update has provided a severity scale for adults. It is detailed as follows:

• Mild ME-CFS – care for themselves and do some light domestic tasks (sometimes needing support) but may have difficulties with mobility. Most are still working or in education, but to do this they have probably stopped all leisure and social pursuits. They often have reduced hours, take days off and use the weekend to cope with the rest of the week.
• Moderate ME-CFS – 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 or education, and need rest periods, often resting in the afternoon for 1 or 2 hours. Their sleep at night is generally poor quality and disturbed.
• Severe ME-CFS – are unable to do any activity for themselves or can carry out minimal daily tasks only (such as face washing or cleaning teeth). They have severe cognitive difficulties and may 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 sound.
• Very severe ME-CFS – are in bed all day and dependent on care. They need help with personal hygiene and eating, and are very sensitive to sensory stimuli. Some people may not be able to swallow and may need to be tube fed.

As for adults,^[38] there are no consistently used definitions for severe ME-CFS in children and young people. The Royal College of Paediatrics and Child Health (RCPCH) Guideline has helpfully proposed that severity is primarily defined in terms of the effect on the patient, which will be a combination of degree and duration of functional impairment^[39] For children and young persons in the context of its guidance, the RCPCH Guideline proposes the following definitions.

• Severe – any patient who is so affected as to be effectively housebound for a prolonged period of time (3 months or more) must be considered severely ill.
• Very severe – any patient who is so affected to be bedridden for a prolonged period of time (3 months or more) must be considered to be very severely ill.

As for other aspects of diagnostic criteria, definitions of degrees of severity of ME-CFS remain a work in progress, requiring further research and development.

Principles of care – individualised approach

The care and support of such severely affected patients requires a very individualised approach, with care being delivered in the patient's own home, as much as possible. It is vital for clinicians to be aware that there is very little research evidence on management of such patients and that simple extrapolation from other patient groups is usually inappropriate. Such patients can only manage physical or cognitive tasks for very short periods, if at all, and this will need to be reflected in consultations.

It is important to check for intercurrent illness such as chest infection and other potential co-morbidities, which may further diminish quality of life.

Expectations must be realistic and discussed with the patient to focus their very limited energy on things that are meaningful for them. For some severely affected patients, normal functions of daily living may be very challenging: eating, drinking, dressing, toileting. Severe effects may prevail for many years before any improvement or stabilisation is seen.

There should be understanding and agreement between clinicians and patients as to what are an individual's most important goals. It may be an agreement to try to achieve modest increases in self-care or activities of daily living.

The primary care team is crucial for supporting severely affected patients. Assessment by appropriately trained community nursing, community OT and community physiotherapy staff may also be valuable, depending on the specific needs of the patient.

A concern has been expressed that some GPs may not be aware of the clinical conditions of the most severely affected patients, making it difficult for their needs to be properly addressed. As with all conditions with complex needs, it is important that care is regularly reviewed for those patients who are severely affected with ME-CFS. Where appropriate, the out-of-hours services should be proactively notified of specific circumstances and needs of individual patients via the electronic care summary (ECS) to assist effective continuity of care. Where care is being delivered by the input of a number of different professionals, this should be coordinated by a named lead professional.

Medicines management

As for less severe forms of ME-CFS, medication can be useful to help with symptoms such as pain, sleep and secondary anxiety, muscle spasms and cramps, and nausea. Many experts have advised that very low doses of medication be used initially (liquid formulations can help with this) – see also Section 4.

Referral

Where provision exists, severely affected patients should be referred urgently to a specialist, experienced in severe ME-CFS. Where there is no specific provision, it would be appropriate to refer to the consultant physician most capable of dealing with the patient's particular clinical needs. The NICE Guideline has recommended that diagnosis, investigations, management and follow-up care for people with severe ME-CFS should be supervised or supported by a specialist in ME-CFS. The Scottish Public Network's Health Care Needs Assessment for people living with ME-CFS has also recommended the development of specialist services for ME-CFS and multidisciplinary teams with the requisite skill-mix.

Other care aspects

Diet – dietary intake may be compromised in these patients and in a few, very severe cases of ME-CFS, use of enteral feeding has been reported. Such interventions must only be instigated following full specialist assessment/investigation.

NICE 2021 Update

Advice may include eating little and often, fortifying foods, using softer textures and the use of eating aids.

Hospitalisation – most people with ME-CFS will not need hospital admission.

However, a planned admission may be useful in the event of deterioration or for further assessment and investigations which would otherwise require frequent or multiple visits to hospital. The decision to admit should be made with the patient with ME-CFS and their family/carers and be based on informed consideration of the benefits and disadvantages.

NICE 2021 Update

Advise minimising stimuli whilst in hospital with aiming to provide single inpatient room. Lowering sounds, keeping lights dimmed and reducing the number of duplicate assessments.

Respite care – may be helpful if desired, but ideally should be in settings that have experience with people with severe ME-CFS, and that are adapted to their particular needs.

Caring for the carers – Carers are especially important in supporting patients, and their own needs must be considered, as part of the ongoing management plans of people with severe ME-CFS.

Caring Together – the Carers Strategy for Scotland 2010-15, jointly produced by COSLA and Scottish Government, has recently committed to ten headline actions, including a Carers' Rights Charter.^{[40], [41]}

Timely support – Many people with ME-CFS, especially those who live alone, will lose the means to manage their home circumstances, including finances, and effectively seek help and support during their time of incapacity. Anecdotal evidence suggests that the longer it takes to receive medical and practical assistance, including social care support, where appropriate, the longer the patient's recovery time and the greater the likelihood of chronicity.

Long term conditions planning

It is hoped that aligning the care of those people severely affected with ME-CFS, to the long-term conditions planning work in NHS Boards – particularly their current work on neurological services improvement – will allow a full assessment of their care needs and allow appropriate management and support plans to be developed.

In turn, this should be facilitated by the National Action Plan for Improving Health and Wellbeing of People with Long Term Conditions in Scotland (2009) and the Healthcare Quality Strategy for NHS Scotland (2010).^[42]

Further information and assistance

Further sources of support and information are provided in Section 8 and Appendix 1.