An important update to guidance on the topic of chronic fatigue syndrome (CFS) and myalgic encephalomyelitis (ME) has been published by the National Institute for Health and Care Excellence (NICE, 2021a) to replace the existing guidelines relating to the diagnosis and management of these conditions, which was published in 2007. In that considerable length of time between publications, misunderstanding, confusion and even disbelief were observed among clinicians regarding these illnesses. This led to variations in care and outcomes, and therefore NICE (2021a) published the new guidance as a way to share what an expert committee have advised on multiple areas relating to these diagnoses, and to provide clarity and rectify misunderstanding, so that CFS and ME can be treated appropriately, the patient can receive the best care, a non-judgmental view can be fostered, and outcomes for the patient and NHS finances can be improved.
Lack of clear definition
The recommendations noted that both ME and CFS have not been clearly defined. They highlighted a lack of pathological evidence of brain inflammation, meaning the use of ‘encephalitis’ is problematic. ME is classed as a disease of the nervous system currently, while the term CFS is considered very broad, simplistic and judgmental by some patients, according to NICE (2021a).
Data from UK Biobank shows there are over a quarter of a million people living with ME/CFS in England and Wales, with approximately 2.4 times as many women affected as men. Both conditions are noted by NICE (2021a) to be complex, multi-system and chronic medical conditions, with significant impact on personal, social and economic welfare. Quality of life is reduced notably, with psychological impacts. Life for those living with, or caring for the person with, ME or CFS also becomes difficult and unpredictable. Many people with the conditions are unemployed, and are even housebound or bedbound – affecting about 25% of sufferers. The condition is noted by the new update to reduce the quality of life so much that the person living with ME or CFS experienced a lower quality of life than a cancer sufferer or someone with multiple sclerosis. It is clear, therefore, that it is of the most integral importance that the condition is taken seriously.
Difficulties with diagnosis
However, what may have led clinicians to be dubious about cases in the past, is that it still remains unclear as to the cause of the condition. However, it is obvious the condition has a huge impact on the person and this is what needs treating with an individualised care plan. In many cases, NICE (2021a) states that symptoms are thought to have been initially caused by an infection; however, they note it is not a simple post-illness fatigue, as it lasts longer and even minimal mental or physical activity can worsen symptoms.
The committee notes the importance of appropriate energy management to aid people in staying within their energy limits
Complicating things further is that there is no diagnostic test for ME or CFS. There is also no universally accepted definition of the illness. There are often delays in diagnosis, with many health professionals not being equipped with the knowledge and confidence to enable them to recognise, diagnose and manage the condition. Also, some practitioners may diagnose ME or CFS when the fatigue comes from another chronic disease. On the other hand, practitioners can also be reluctant to diagnose the conditions when no other causes can be identified.
People living with ME and CFS notably experience a sense of disbelief from professionals regarding their conditions and symptoms, which often results in them disengaging from services (NICE, 2021a). For younger people, school attendance can significantly reduce, leading to social care difficulties for families.
Inequity in ME/CFS
The previous guideline in 2007 made recommendations on cognitive behavioural therapy and graded exercise therapy. However, these treatments have been proven to be controversial for ME and CFS, with disagreements and uncertainty about their effectiveness among people with these conditions and clinicians. The evidence for the effects of other commonly prescribed therapies has also been questioned by the panel who formed the new updated guidance.
Significant to this work was the observation of inequity throughout England and Wales in specialist service access. In some areas, there is very limited access, thus causing inequity in treatment and outcomes.
A strong theme pervading the work of the reviewing committee observed that for someone experiencing a lack of understanding and prejudice about their condition, can lead to a loss of trust in health and social care services. Therefore, it is integral that health and social care professionals take into consideration how a lack of belief may impact on a patient with ME/CFS and their families. The committee stated this to be especially relevant to children and young people and therefore created additional recommendations highlighting communication with children. A late diagnosis and lack of monitoring was also seen to be a significant factor acting as a barrier to good patient care and management of ME and CFS.
There is a section in the guidance dedicated to those with severe or very severe ME/CFS, as this patient group is perceived to often have been neglected, with the severity of their symptoms largely misunderstood, therefore additional recommendations were required for this group. Recommendations generally concluded that home visits should be made available to people with severe or very severe ME/CFS so an assessment could be carried out. This recommendation can provide equity of access to this group as they are often housebound. Significantly, those with severe or very severe ME/CFS have little contact and support from health and social care services, therefore highlighting the need for flexible and specific support for those groups.
Improving patient care
Overall, the new recommendations should improve patient care and encourage best practice of a largely misunderstood illness. In addition, the committee agreed that the Institute of Medicine's 2015 criteria had the best balance of inclusion and exclusion of all the reviewed criteria, but it required adaptation in order for its use to be optimal. The 6-month delay should also be reduced in order for management to begin earlier, with fatigue and post-exertional malaise being defined clearly so that it can be easier to understand the new recommendations, the committee stated (NICE, 2021a).
The committee decided that a diagnosis of ME/CFS should be suspected if people have all four key symptoms. These key four symptoms are:
- Debilitating fatigue
- Post-exertional malaise
- Unrefreshing sleep or sleep disturbance (or both)
- Cognitive difficulties.
Symptoms last for at least 6 weeks in adults and 4 weeks in children and young people. A viral illness would not usually last this long, therefore clinicians should note the length of time the symptoms persist, for early identification of ME/CFS.
Other symptoms are not essential to aid diagnosis, however they should still be seen as important so that ME/CFS can be understood and symptoms can be managed. It was also considered important that research takes place to identify diagnostic tests for the condition as currently diagnosis remains difficult without access to more specific diagnostic tests or tools. Earlier diagnosis and thus earlier symptom management are important to prevent deterioration. Where the patient is a child, their place of education should be contacted to advise about flexible adjustments or adaptations, NICE (2021a) states.
The committee noted there will be no change to the current practice of diagnosing ME/CFS based on clinical assessment and history, as well as performing tests for differential diagnoses as appropriate. Awareness of these existing methods, however, is important to aid earlier diagnosis and management, and research should be ongoing to update existing methods in the future.
Managing symptoms early
The committee's review of qualitative evidence combined with the committee's experience suggested that managing symptoms early may prevent them from deteriorating. To reflect this, the committee made a recommendation to give people advice on symptom management drawn from their own knowledge and experience.
The qualitative evidence observed that deterioration can cause anxiety among people living with these conditions, therefore it was important for people to know who they can contact if their symptoms change. The recommendations note that this advice may cause fewer people to deteriorate, thus meaning such recommendations could be very cost effective.
As stated, for better overall management, the recommendations say the aim should be to reduce diagnosis time from 6 months to 3 months, which may also improve longer term outcomes and costs. Due to the lack of confidence by non-specialists in diagnosis CFS or ME, the committee has also recommended to refer people with ME/CFS to an ME/CFS specialist team at 3 months to confirm their diagnosis and develop a care and support plan.
A collaborative personalised care and support plan approach is needed for best outcomes, it was concluded. This can be based on a holistic assessment as soon as the person has a confirmed diagnosis. A medical assessment would be required as part of this assessment. A copy of the care and support plan should also be shared with primary care and a copy held by the person themselves, so that it can be used as a reference point in circumstances whereby the patient may require an admission to hospital.
Key areas of CFS/ME to manage and support all depend on the individual factors identified as the patient needs in their holistic assessment. A care and support plan can be created with a multi-disciplinary team of all those involved in the patient's care, including themselves, which may look at social care needs assessments, energy management, physical functioning and mobility, cognitive behavioural therapy (CBT) and dietary management. This is largely in place, but referral to be able to have such an assessment is in some places limited, and therefore the recommendations – by promoting a better understanding of the illness – can improve outcomes by helping professionals and non-specialists to signpost the patient care and management to the right providers.
Extra staff time or other healthcare resource use may be required resulting from the new guidance, flexible appointments and home visits may be needed, and adjustments during inpatient stays and access to aids and adaptations may be required. It is important that access to all services, however, is equitable. A single point of contact was also highlighted as a pull factor to keep the patient engaged with the multidisciplinary service.
Overall, the committee stated that evidence for non-pharmacological and pharmacological interventions for ME/CFS was found to be inconclusive, with limited evidence for any single intervention. The committee noted evidence was difficult to attain, due to the inconsistency in outcomes and concerns regarding validity of outcomes measures used for the conditions. This would make comparison and combination of results difficult. Cost effectiveness of interventions was therefore difficult to conclude. This makes it hard to make recommendations about the exact management styles for best outcomes.
Energy management
A key symptom of CFS and ME is of course fatigue/energy levels. The committee notes the importance of appropriate energy management to aid people in staying within their energy limits. This aims to prevent deterioration, while also supporting a possible increase in activity. The committee stated that any programme using fixed incremental increases in physical activity or exercise (for example, graded exercise therapy), or physical activity or exercise programmes that are based on deconditioning and exercise avoidance theories, should not be offered to people with ME/CFS. The committee wanted to reinforce the significance that there is no therapy based on physical activity or exercise that is effective as a cure for ME/CFS (NICE, 2021a). Unstructured or inappropriate physical activity or exercise programmes should be prevented by following the new NICE (2021a) recommendations, as they may do more harm than good. A specialist occupational therapist or physiotherapist, however, may be useful for the patient and despite the increase in costs, this may decrease the financial burden on the NHS due to improving outcomes. Significantly, it was determined that planning rest and activity was an important part of managing the conditions in an appropriate way, following advice from specialists involved.
Interventions for ME/CFS
Pain is a significant symptom associated with ME/CFS. Those with severe or very severe ME/CFS may also experience a very severe level of intolerable pain. The lack of evidence meant no interventions could be made by the committee, but reference was made to the NICE guidelines on neuropathic pain (NICE, 2020) and headaches (NICE, 2021b). It was recognised that medication is important and useful for some people in managing their pain; however, those with ME/CFS may also be more intolerant of drug treatment. The advice, based on the perceived increased risk of harm, was to take a cautious approach when prescribing, starting medications at lower dosages than would usually be used in clinical practice, while observing how the symptoms respond before then changing the dose. In the case for children, it is important to discern that medication should be managed with advice from a paediatrician due to further increased risk in this patient group.
Use of the ‘Lightning Process’ in practice was disputed by the committee, therefore the team agreed the Lightning Process – or therapies based on it – should not be offered to people with ME/CFS.
The qualitative evidence reviewed for this new guidance showed that people with ME/CFS have found cognitive behavioural therapy (CBT) useful when delivered by a therapist who understands ME/CFS, but crucially that there is the potential for harm when it is inappropriately delivered. It is important to use CBT for improving lifestyle and emotional factors, while it should not be seen as a ‘cure’.
Relapse prevention should also be in place. A relapse prevention plan is very useful in case of a sudden deterioration, so this can be best managed.
The need for training
A significant theme in the new recommendations is the acknowledgement that many staff lack knowledge, understanding and up-to-date training regarding ME/CFS. Therefore, NICE (2021a) recommends that all health and social care staff who deliver care to people with ME/CFS should be trained so they are able to provide the care in this guideline. This may improve staff attitudes and patient engagement as a result. Currently, training is not often given, and therefore there will be a financial impact. However, ultimately it is likely to be cost effective as it should lead to better outcomes.
Conclusion
Overall, it is pleasing to see that a much needed recommendation has been compiled by a specialist committee for NICE, so that practitioners of all specialities have a reference point for guidance, care and treatment can be optimal, and the patient can be better understood, improving engagement, and improving equity in access to services.