Background
Severe ME Day – 8th August – is a day dedicated to raising awareness for those suffering from severe myalgic encephalomyelitis (ME). This day also serves as a remembrance of those who have lost their lives to this debilitating condition – one such individual being Sophia Mirza, a British artist, who passed away aged 32. Her death brought significant attention to the disease, hence her birthday – 8th August – was chosen to mark Severe ME Day.
A more recent case that has garnered wide news coverage involves Maeve Boothby O’Neill. Almost 3 years after her death, a full Coroner’s Inquiry opened examining the circumstances surrounding the death of the 27-year-old as a result of severe ME. The two-week Inquiry looked at her care at the Royal Devon and Exeter Hospital, delays in palliative care, and the general lack of understanding in the medical community about the disease. What can never be lost sight of is that the Inquiry centred on the events surrounding a “premature and wholly preventable” death of a young woman described as “exceptional” and “intelligent” on 3 October 2021.
Prevalence
Estimates suggest that between 10% and 25% of individuals with ME suffer from the severe form of the illness. The impact of severe ME on an individual’s life is profound, leaving many confined to their homes, bedbound, or even immobile. The severity of the illness, coupled with the lack of understanding, can lead to sufferers being excluded from healthcare systems, and relying heavily on the support of a dedicated caregiver.
Symptoms and challenges
The challenges faced by those with severe ME extend far beyond debilitating fatigue and post-exertional malaise (PEM). According to the 25% ME group, “It is usual for people with severe M.E. to have between 50 and 100 separate symptoms. The impact of these combined is overwhelming and hard to communicate.”
The NICE 2021 guidelines describe the symptoms that those with severe forms of the illness may face:
- “Severe and constant pain, which can have muscular, arthralgic or neuropathic features
- Hypersensitivity to light, sound, touch, movement, temperature extremes and smells
- Extreme weakness, with severely reduced movement
- Reduced ability or inability to speak or swallow
- Cognitive difficulties that limit the person’s ability to communicate and take in written or verbal communication
- Sleep disturbance such as unrefreshing sleep, hypersomnia and altered sleep pattern
- Gastrointestinal difficulties such as nausea, incontinence, constipation and bloating
- Neurological symptoms such as double vision and other visual disorders, dizziness
- Orthostatic intolerance and autonomic dysfunction, such as postural orthostatic tachycardia syndrome (POTS) and postural hypotension.”
“People with 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). … People with 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.”
Yet those with severe and very severe ME not only endure a relentless burden of symptoms but also face significant stigma from both the medical community and society at large. This stigma may lead to various forms of neglect, compounding their challenges.
The unique challenges of living with the severe forms of the illness are highlighted as part of ‘Dialogues for a Neglected Illness’ – a moving and meticulously researched project supported by the Wellcome Public Engagement Fund (2018–21) and produced by Natalie Boulton with cameraman and editor Josh Biggs.
Prognosis
The prognosis for ME/CFS is generally poor, with low rates of both recovery and significant improvement. The impact of age on prognosis has produced mixed results from studies, however diagnostic delay is consistently associated with worse outcomes. Early diagnosis and management are crucial, but heterogeneous symptoms and lack of awareness among health professionals may lead to significant delays, exacerbating the condition. Moreover, our knowledge is limited about the biomedical basis of severe illness or its long-term consequences as biomedical research into severe ME is very limited.
Whilst no specific treatments are available, supportive care can be provided adapted to the unique needs of each individual. According to NICE, examples include adaptations/aids to assist mobility for daily activities and low-stimulus environments.
Conclusion
Severe ME Day serves as a crucial reminder of the profound impact of this illness on individuals with severe ME and their loved ones. Raising awareness and fostering understanding are essential steps toward improving care, reducing stigma, and ultimately finding effective treatments for severe ME.
