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Image by Greg Crowhurst
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'It is important to note that the full reality of a person's disability and illness experience can be missed if the moment they manage to communicate is considered the norm, when it is in reality rare to impossible.'
Severe Myalgic Encephalomyelitis (ME) includes people with very severe and profound ME.
In order for family carers, agency carers, doctors and other healthcare staff to manage Severe ME they would be required to know about Severe ME, in particular they would need to know details of the the complexities that separate Severe ME from less disabling severities in ME; they would need to recognise the distinctions between the illness as suffered by someone with mild or moderate ME and know that those with severe ME suffer a completely different condition, i.e., an horrendous life limiting condition that sees some patients permanently confined to bed, tube fed, suffering various hyper sensitivities, and post exertion crisis in response to the smallest movement, or to someone being near them trying to support them.
Those who describe themselves as having mild or moderate ME and some people in the severe ME category, suffer from fluctuating symptom severity, and can also suffer from very severe symptoms and profoundly disabling features at times.
Before we discuss management of Severe ME, it is important that we include some important information particular to Severe ME:
- the clinical features and symptoms;
- impact of symptoms on quality of life;
- important considerations for severe ME;
- problems and challenges for the person with Severe ME;
- challenges facing the person with Severe ME caused by others;
- the reasons why people with Severe ME may have to attend hospital or ED or other care setting;
- Important notes about the complexities of managing severe ME cases
Clinical Features of Severe and Very Severe ME
Cardinal Feature
Post Exertion Response/Crisis or Post Exertional Neuro
Immune Exhaustion (PENE), which in simple terms means lowered functional
capacity due to dysfunction in aerobic energy metabolism, is the cardinal feature in Severe ME. Simple movement in bed, a carer walking into the patient's bedroom, cognitive tasks,
such as thinking and speaking, all use oxidative energy metabolism. Exposure to
light and sounds are like exercise for people with very severe ME, sometimes
causing permanent crashes and further loss of function.
Other features
ME can cause dysfunction in all the major systems - neurological, immune, endocrine, cardiovascular, musculoskeletal, gastrointestinal, respiratory, and genito-urinary, which is why people with Myalgic Encephalomyelitis have so many symptoms. Below are some of the symptoms in Severe ME repeatedly reported in feedback.
- Minimal energy levels, resulting in the patient being mostly housebound or permanently confined to bed;
- Paralysis - full body or partial;
- Severe generalised continuous agonising pain;
- Severe continuous headache;
- Cardiac Issues;
- Hyperaesthesia/extreme sensitivity to touch;
- Abdominal pain, worse after food—this may be so severe as to interfere with nutrition;
- Sleep disturbance, possible hypersomnolence or difficulty sleeping because of pain and headache;
- Major problems with cognition, concentration and short-term memory;
- Heightened sensory sensitivities, including to light, sound, touch, taste and smell - smells set the person back and they can feel so unwell just from someone’s perfume;
- Multiple chemical sensitivities;
- Severe reactions to medications;
- No possible movement;
- Problems with eating and drinking - e.g., swallowing issues which can be due to either general weakness or actual dysphagia, or severe reactions to food, and these may necessitate tube feeding;
- Aphonia (mechanism unclear);
- Myoclonic jerks;
- Incontinence;
- Temperature dysregulation;
- Increased inflammation;
- Numbness, pins and needles;
- Extreme discomfort;
- Severe hyperacusis;
- Fainting (POTS/other);
- Orthostatic intolerance;
- Autonomic dysfunction;
- Unbearable muscle tremor;
- Spasms/seizures;
- Multiple sensitivities including sensitivities to fabric, to movement as well as seeing movement;
- Zero energy to function;
- Sore throat;
- Tender glands;
- And many other possible symptoms and features.
The danger is that the long list of symptoms can unintentionally, underplay their severity and seriousness, the totally disabling nature and the individual intensity of each one, which together add up to a ‘physical nightmare of indescribable proportion’.
Impact of Symptoms on Quality of Life
The symptoms experienced by someone with Very Severe/Profound ME can be unimaginable - the level of highly tormenting hypersensitivities to ordinary things are both distressing and attacking, adding to the difficulty of getting safe, supportive, and experienced care.
The sheer severity of suffering experienced by patients with the above symptoms can actually be worse than that suffered by patients with other chronic conditions such as multiple sclerosis and cancer.
The abdominal pain may be so severe as to interfere with nutrition, an issue which has been responsible for some fatalities.
The severity of the photosensitivity can be a trigger to disbelief, as the doctor may find it difficult to accept that the patient not only has to lie in a darkened room but has to wear eye protection in addition.
Considerations for Severe ME
- Severe ME includes people with severe, very severe and profound ME. Some patients with milder or more moderate ME may experience severity fluctuations in some symptoms, going from from mild to profoundly severe, so someone with mild or moderate ME may suffer from severely disabling symptoms.
- Severe ME constitutes a major challenge, not only for the patient and the family, but also for the doctor confronted with it, often for the first time. It requires from the doctor commitment, calmness, courage and compassion, indeed many of the qualities of the ideal doctor.
- For very severely affected ME sufferers there is virtually no ‘safe’ level of physical or mental activity, orthostatic stress or sensory input; no level which does not produce a worsening of symptoms.
- Even the most basic actions - speaking a few words, being exposed to moderate light or noise for a few minutes, turning over in bed, having hair or body washed in bed by a carer or chewing and swallowing food - can cause severe and extended symptom exacerbations in severe patients.
- It is not uncommon to hear of very severely affected sufferers who are unable to bathe themselves (or even be bathed by a carer) more often than once a week, or even once every few weeks, or even less.
- Some sufferers cannot chew or swallow food any longer and need food to be blended to a soup or at worst to be tube fed. Many patients with severe ME are no longer able to toilet themselves. Either sufferers are just too ill to do these things at all, or they cannot tolerate the very long and severe relapses that come after such activities.
- For people with severe ME even the smallest movement, thought, touch, light, noise or time being upright can make their already very severe symptoms far worse. Few illnesses demand such isolation and loss of quality of life as severe ME.
- Very often people with very severe ME can barely communicate, or even tolerate the presence of another person. This is what makes ME such a cruel disease and such an isolating disease.
- The illness can cause an unrelenting level of disability, suffering and isolation that is just unimaginable to anyone not familiar with very severe ME.
Problems and Challenges for the Patient with Severe ME
Patients with ME have often suffered from disbelief on the
part of their doctors or other healthcare staff, and even those severely affected
are not protected from this disbelief.
This disbelief can operate at several
different levels:
The first level is when the doctor disbelieves that ME even exists as an organic illness in the first place. This can lead to inappropriate referral to psychiatrists, and then to harmful management plans.
The next level is for the doctor to accept that ME exists, but to disbelieve that a particular patient has it.
A further level is to have an overoptimistic view on the efficacy of certain forms of treatment, usually Graded Exercise Therapy (GET) and/or Cognitive Behavioural Therapy (CBT). This leads to the patient having the diagnosis of ME withdrawn when they fail to respond to these management strategies, or as so often happens when the patient’s condition worsens.
GET is especially likely to lead to such deterioration. Rather than drawing the conclusion that these therapies are misguided, the doctor often changes the diagnosis to one involving a psychological causation.
The final level is when the doctor diagnoses ME at a stage when it is still mild or moderate, and then the patient’s condition worsens to the extent that it becomes severe. The doctor can then lose confidence in the original diagnosis, and change this to a psychological diagnosis such as Pervasive Refusal Syndrome, MUS or FND.
For the patient with severe ME to be subjected to such
disbelief is of course adding so much stress and harm to a very ill and
disabled person.
The worst scenario is when the paediatrician, as a result of
some of the above varieties of disbelief, makes a provisional diagnosis of
Fabricated and Induced Illness (FII), and refers the patient to Social Services
as a case needing safeguarding.
(the above information is based on notes by Dr Nigel Speight from 2020
paper entitled
Challenges Facing the Person with Severe ME Caused by Others
People with Very Severe ME can become isolated. As families, friends, and doctors slip away due to lack of knowledge, doubt and denial, Severe ME patients have to survive without care precisely when they need help the most. In the absence of assistance, those who are very severely ill continue to decline until they reach a state of medical crisis and land in the emergency department of a hospital.
The first challenge when a person with ME ends up in hospital is the enormous hyper sensitivites that are suffered by them as a result of lights, noise, heat and dealing with many staff and others in the hospital.
The second challenge is absence of concise guidelines from health agency sources that address the very severe end of the disease spectrum. There are many informal sources on ME, but we need buy-in from health agencies and busy ME-unaware hospital practitioners. The absence of guidelines make it more challenging for the patientfamilies and carers to advocate for my friend and undermined his case.
Reasons People with ME including Severe ME attend ED
Dysautonomia/POTS flares, severe dehydration, extreme pain, extreme headaches, malnutrition, clotting issues (DVT, elevated D-dimer, etc), infection, cardiovascular autonomic/low blood volume issues including heart palpitations, PVCs, chest pain, fast hr, microvascular angina coronary microvascular dysfunction (CMD) and microvascular ischaemia; severe aura migraine that affects speech and vision; hemiplegic migraine which affects speech; new onset migraines, abdominal migraines, deficiencies-related issues e.g., very low level of B12; non-epileptic seizures or tremors or spasms with/without features such as slurred speech and aphasia including oesophageal spasm; paralysis (whole body or partial), very low/very high bp, respiratory issues such as severe breathlessness, air hunger and low oxygen levels; bradycardia, tachycardia, loss of consciousness, weakness in legs - not able to stand; vomiting and nausea; when medications are not helping extreme symptoms, undiagnosed MCAS symptoms, UTIs, visual disturbances.
Important Notes about the Complexities of Managing Severe ME Cases
Doctor and other healthcare staff must know about the unique medical needs of people with very severe and profound ME, focusing on gastrointestinal manifestations and extreme hyper sensitivities.
Very severe ME can be a very frightening disease, not only for the family and carers of the severely ill patient, but also for doctors who are unlikely to have any previous experience of these most severe presentations. Patients can die from very severe ME and it’s complications, and this is more likely to happen when their condition is badly managed by ill-informed doctors. It is important that doctors listen to the patient, or if the patient wishes, to their carers.
Over time, very severe patients have less and less access to primary medical care simply because they become too sick to manage medical appointments or to have home visits from their doctor. Many GPs don’t want to take on a highly complex severe ME patient or know how to manage one. These patients end up falling through the cracks, have no meaningful healthcare, and continue to waste away in isolation, some end up dying due to neglect and/or malnutrition.
