About Severe and Very Severe Myalgic Encephalomyelitis (ME)

 

Myalgic Encephalomyelitis (ME) is a complex neurological disease involving profound dysregulation of the central nervous system & immune system, dysfunction of cellular energy metabolism & ion transport & cardiovascular abnormalities, as per the International Consensus Criteria, ICC 2011.

ME is classified as a neurological illness since 1969 by the World Health Organisation (WHO)- ICD G93.3

Myalgic Encephalomyelitis (ME) is one of the most disabling diseases there is. The level of disability can exceed that in Polio, MS, advanced HIV, and other serious illnesses.

It is hard for anyone to understand the horrors of living with Myalgic Encephalomyelitis (ME). Finding a way to enable the ME - unaware to get a glimpse of the conversation they need to enter into to learn about Severe ME is vital. It is very important to raise awareness amongst both health professionals and society about this group who just don’t exist in the consciousness & experience of those they need knowledgeable, guided & appropriate care from.

 

 

'An M.E. patient feels effectively the same every day as an AIDS patient feels two weeks before death.'

 

Severe Myalgic Encephalomyelitis (ME)

 

Severe ME

People with Severe Myalgic Encephalomyelitis require unique care due to the tendency for the illness to be exacerbated by everyday stimuli such as light, noise and movement.

They are often too ill to use a wheelchair or can only do so to a very limited degree. Many need to spend their time lying flat in silence and darkness to avoid deterioration.  Some are tube-fed, incontinent, unable to communicate, allergic to medications, and unable to move.  

For people with Very Severe ME 'resting' implies 'complete incapacitation’ which means that inactivity is the only option. Those severely ill are often incapacitated because it is physically impossible for them to do anything else.  Lying down in a dark room in silence and with absolute zero sensory input, with absolute zero physical movement or cognitive activity. Clothing and bed linen must be comfortable but sometimes no clothing is preferred and particular materials for bed linen essential. The room must have a very moderate temperature; not too hot or too cold.

 

“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, and perhaps also contribute to disease progression. 

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 – cause severe and extended symptom exacerbations in such 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 to be tube fed. 

Many patients with severe ME are no longer able to toilet themselves, and so on. 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 period upright can make their already very severe symptoms far worse. Thus few illnesses demand such isolation and loss of quality of life as severe ME.

Very often people with very severe M.E. 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.”

Severe ME/Very Severe ME equals severe complex illness and multi-system dysfunction.

Those with Very Severe Myalgic Encephalomyelitis (ME) experience profound levels of suffering and extreme symptoms, which can be life-threatening. Life may be reduced to basic survival, struggling moment by moment while enduring extreme pain.

For some there is no let up to the high intensity of their symptoms and their needs are diverse and complex.

Common Symptoms and Features of Severe ME include:

*PENE - Post Exertional Neuro-immune Exhaustion/Post Exertion Crisis

Pain (muscle, nerve, joint)

Weakness

Paralysis (partial or full body)

Spasms (muscles inc throat)

Severe Digestive problems

Dehydration

Minimal to zero energy levels, resulting in the patient being housebound or bedridden

Severe generalised continuous throbbing, burning & stabbing pain

Severe continuous headache

Hyperaesthesia/extreme sensitivity to touch

Abdominal pain, worse after food, this may be so severe as to interfere with nutrition

Problems with eating and drinking, chewing and swallowing, this can be due to either general weakness or actual dysphagia, and this may necessitate tube feeding

Vomiting

Food Allergies

Sleep disturbance, possible hypersomnolence or difficulty sleeping on account of pain and headache

Major problems with cognition, concentration and short-term memory

Extreme sensitivity to light and sound

Multiple chemical sensitivities

Persistent sore throats

Aphonia

Myoclonic jerks

Incontinence

Cardiac problems

Compromised immune system

Fainting

Dysautonomia

Hyper Sensitivities - intolerant to light/sound/smell/movement/touch/chemicals – profoundly hypersensitive to everything

Cognitive problems

Dystonia

No energy - Ability to make energy at cell level is lost

Pins and needles

Numbness

Feeling ill

Extreme discomfort

Temperature Dysregulation

Immobile - No Possible movement

Absolute pain

Profoundly hypersensitive even to fabrics, e.g. clothes and bed linen

Helpless

Blank mind

Look asleep but totally aware and conscious

Everything is out of reach

Completely unable to communicate

Cannot sit up/stand/move or walk due to orthostatic intolerance

Dizziness & balance problems

Struggle to breath

Light hurts and noise damages

Stroke-like experiences

Smells set the person back and they can feel so unwell just from someone’s perfume

Zero energy to function

A fog of inability

Impaired consciousness

Blackouts and coma-like states

Malnutrition

And the list goes on, it is limitless. 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.

The danger is that the long list of symptoms above can still, 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’.

The symptoms experienced by someone with Severe/Very Severe 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.

Guidelines from NICE [NG206] 2021

From theNICE 2021 Myalgic Encephalomyelitis Guidelines NG206

1.17.1 Be aware that people with severe or very severe ME may experience the

following symptoms that significantly affect their lives, including their mobility,

emotional wellbeing and ability to interact with others and care for themselves:

• 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, [insomnia], 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.

1.17.2 Recognise that symptoms of severe or very severe ME may mean that:

• people need a low-stimulus environment, for example a dark quiet room with

interaction at a level of their choice (this may be little or no social interaction)

• are housebound or bedbound and may need support with all activities of daily living,

including aids and adaptations to assist mobility and independence in activities of daily

living (e.g. hoist, electric wheelchair)

• need careful physical contact when supported with activities of daily living, taking

into account possible sensitivity to touch

• cannot communicate without support and may need to choose someone to be their

advocate and communicate for them

• are unable to eat & digest food easily and may need support with hydration & nutrition

• have problems accessing information, for example because of difficulty with screens,

sound and light sensitivity, headaches affecting their ability to read, or brain fog

affecting their concentration.

NICE 2021 Recommendations

Challenges for the person with Severe ME when faced with ME/Severe ME-unaware Doctors

Despite the relatively high percentage of people with Severe ME there is very little understanding of what people with Severe and Very Severe ME go through and little knowledge of how to safely look after them at home and elsewhere.

Most medics, ME- unaware medics do not know how to look after people with Severe/Very Severe ME, and the lack of awareness and education in a hospital or other healthcare setting can be difficult and dangerous for a person with Severe/Very Severe ME.

Most medics don’t know anything about ME because it doesn’t feature on medical school curricula, it’s not taught to professionals and there’s no directive calling for adoption and use of important gold standard guidelines like the International Consensus Criteria (ICC) 2011. There’s no national policy on ME.

Lack of knowledge can lead the doctor to adopting a strategy of avoidance, refusing to accept that they have a duty of care to the patient, and regrettably many patients with Severe ME suffer near total neglect by their doctors.

The extreme severity of the person with Severe ME 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. Abdominal pain may be so severe as to interfere with nutrition, an issue which has been responsible for some fatalities.

From the NICE 2021 Myalgic Encephalomyelitis Guidelines NG206 

 

 1.17.7 When planning hospital care for people with severe or very severe ME:

• discuss with the person (and their family or carers, as appropriate) what to expect

when they come into hospital

• aim to minimise discomfort and post-exertional malaise during transfer to hospital,

for example by planning the route in advance, avoiding noisy areas and admitting them

straight to the ward on arrival

• discuss the person's care and support plan with them, including information on

comorbidities, intolerances and sensitivities, to plan any reasonable adjustments that

are needed

• aim to provide a single room if possible

• keep stimuli to a minimum, for example by:

• seeing them one-to-one
• using calm movements and gestures
• not duplicating assessments
• being cautious about the pressure of touch
• keeping lights dimmed
• reducing sound
• keeping a stable temperature
• minimising smells.

‘I cannot convey how much the slightest wrong thing can cause unimaginable suffering and unnecessary endurance of intolerable levels of pain and other symptom deterioration, due to my profound levels of sensitivity to sound, light, movement, motion, perfume/chemical sensitivity.

The slightest wrong movement, sudden exposure to perfume, light, coughing, talking, clicking, banging or drilling sound, can trigger hours to days of unbearable paralysis, weakened muscles, shaking muscle spasms, intensified pain, massive headache, a completely blank mind, with every thought lost, just like that.

What is worse is knowing that a little extra awareness or care might make the difference between hours of paralysis or not: it is the unnecessary impact of small things that causes great dismay.’
~ Greg Crowhurst ~

Post Exertional Neuro-Immune Exhaustion 

Considerations for those with Severe and Very Severe ME

Myalgic Encephalomyelitis (ME) is an acquired neurological disease with complex global dysfunctions. Pathological dysregulation of the nervous, immune and endocrine systems, with impaired cellular energy metabolism and ion transport are prominent features. Also all over body pain.

Overload phenomena is part of the complex pathophysiology of Severe ME as the body reacts to sensory inputs which it does not have the energy to manage. Those unfamiliar with the pathophysiology of ME mistakenly interpret this as ‘anxiety’, which it is not. 

Minimising the need to ‘react’ is essential as reaction causes a physiological response which affects the already out of balance central and autonomic nervous systems. Being forced to react results in an exacerbation of symptoms.

It is important to realise that at the severe end of the spectrum people with Severe ME don’t need to exert themselves for the phenomenon PENE to operate, there can be a critical response to the slightest movement by the patient or by the movement of a visitor to the patient's bedroom. Even simple brain activity can lead to worsening of illness in ME, e.g., hearing noise from outside the bedroom.

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, and perhaps also contribute to disease progression.

Due to the severity of illness in people with Severe and Very Severe ME, who would most likely be permanently confined to bed, care and treatment would be planned and catered to ensure that management of illness, dispensing of treatments and provision of meals and bathing is done in the most non-invasive way possible. As some Very Severe/Profound ME patients may be tube fed and administered meds intravenously there would be a very special care given to them.

The post exertion response crisis that occurs following any exertion, mental and physical, in Severe ME results in horrendous features such as paralysis, seizures, fits, unconciousness. Patients are often too ill to use a wheelchair or can only do so to a very limited degree. Many need to spend their time lying flat in silence and darkness to avoid deterioration.  Some are tube-fed, incontinent, unable to communicate, allergic to medications, and unable to move.  

More about PENE here.

Issues of Noise, Light,Touch, Chemical & Movement Sensitivity in Severe/Very Severe ME 

Carers and others need to be aware of issues of noise, light, touch, chemical & movement sensitivity in Severe/ Very Severe ME, see more in Notes from Greg Crowhurst

 

How do people with Myalgic Encephalomyelitis become so extremely ill?

Some people with Severe Myalgic Encephalomyelitis (ME) may have been severely affected from the first day of the illness. In other cases a 'milder' form of Myalgic Encephalomyelitis (ME) may worsen and become severe. In some people the severity increases over time. The progress of the disease is unpredictable, and few recover fully.

Some people in the mild or moderate categories have been treated so badly by medics and mismanaged to the point that they deteriorate and drop to a very severe level of ME.

 

 

 

‘The main lesson I learnt is that one needs to be proactively protective of one’s patient, especially against other professionals, and disbelieving friends and relatives.

For professionals affected by “Furor Therapeuticus”, i.e., the feeling that one has to do something, it is better to remember that there is no proven curative treatment, and that they should perhaps concentrate on the “Do No Harm” element in the Hippocratic Oath.’

~ Dr Nigel Speight ~
 

 

 

Films About Severe ME

 

 

Two films from Dialogues for a Neglected Illness (Natalie Boulton & Josh Biggs)

‘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.  Specialist help should be available for these very severe cases.’

The first film is an introduction to the more severe end of the spectrum of ME and the second film gives a more detailed look at very severe ME

Please see the films here

 

 

 

‘Voices from the Shadows’, a very informative and educational piece, is a film about Severe ME that is available online and on dvd.

https://vimeo.com/ondemand/22513/108797012

The film shows the brave and sometimes heartrending stories of five ME patients and their carers, along with input from Dr Nigel Speight, Prof Leonard Jason and Prof Malcolm Hooper.

‘Voices From the Shadows’ was filmed and edited between 2009 and 2011 by the brother and mother of a person with ME in the UK. The film shows the devastating consequences that occur when people with ME are disbelieved and the illness is misunderstood.

Severe and lasting relapse occurs when patients are given inappropriate psychological or behavioural management: management that ignores the reality of this physical illness and the severe relapse or exacerbation of symptoms that can be caused by increased physical or mental activity, over exposure to stimuli and by further infections.

An incorrect attitude and very mistaken belief in behavioural and psychological causes, particularly when ME becomes very severe and chronic, following mismanagement, is still taught to medical students and healthcare professionals in the UK.

As a consequence, situations very similar to those shown in the film are taking place right now in Ireland, in the UK and in other countries.

The original 1 hour  film is available to view free of charge on Vimeo at this link

(enter the promo code VOICES at the $3 button)

See other details and a shorter 30 minute version of the film here

 

 'Wasteland' is a film by Greg Crowhurst which visually documents his wife Linda's Severe ME. (Please note that you may need to mute the volume because there are very loud adverts shown ahead of the film)

'Hope for a better Moment' is another film by Greg Crowhurst who produced and published this public visual document about Severe ME in 2007, showing his wife Linda speaking of her condition and showing features of her neurological symptoms. Linda is barely able to move and unable to feed herself. (Please note that you may need to mute the volume because there are very loud adverts shown ahead of the film)

 

​​​'Why Severe ME Relapses are Horrific' a video by Jodi Bassett of the Hummingbird Foundation for ME, describing Severe ME relapse

Online Support

The 25% Severe ME Group 

The 25% Severe ME Group (UK) was set up to support all who have the severe form of ME and those who care for them. This includes people who are housebound, bedbound and dependent on help for physical functions - people who may be tube fed, who suffer from great pain and multi-sensitivities along with other horrific disabling symptoms. 

25% Severe ME Group website https://25megroup.org/

25% Severe ME Group on Facebook

 

Stonebird Website

Stonebird: The Experience of Severe ME by Greg Crowhurst here

The Stonebird Definition of Severe ME here

The Moment Approach here

Paralysis in Severe ME here

Very Severe ME – Underlying Principles of Care here

The Care Needs of People with Severe ME here

Books about Severe ME

Greg Crowhurst has written books focusing on caring for Severe ME patients. These can be found on Amazon, Lulu, etc. These include:

• Severe ME: Notes for Carers (paperback)  here

• Severe ME: More Notes for Carers here

Statistics

We don’t have official data on how many people have ME in Ireland or data on how many with Severe ME. We could make an estimate that there are 100s of people with Severe ME given the estimated prevalence rates* and taking into account the approx 25% with Severe ME...which could be somewhere between 2500 and 4750 possible patients

That’s a lot given that there are no official guidelines for ME or Severe ME nor HSE notes for Carers of people with ME or Severe ME.

 

* International Prevalence rates suggest that there are somewhere between 10,000 - 20,000 people with ME in Ireland

 

People with Severe ME make up approximately 25% of our ME community. People with Very Severe ME are approximately 2% of our ME community.

 

“I am a ghost in the land of the living – forgotten, ignored and drifting on the edges of life, whispering my message in the ears of the lucky ones who can participate in life...I have Myalgic Encephalomyelitis. I call it paralysis, muscle and cardiac failure, brain injury, a living plague that kills only slowly, but does kill.”

 ~ Aylwin (Jennifer) Catchpole~ 

Person with Severe ME patient

Died in August 2010

Caring for Someone with Severe 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 here

See further information about caring for someone with Severe ME here, here & here 

Care Plan 

Care Plan Template

My Needs Statement

My Needs Statement Template

More Resources

Measuring and Tracking Symptoms in Severe ME 

The De Paul Symptom and Severity Questionnaire, and the De Paul PEM Questionnaire are very useful tools for recording symptoms and severites in Severe ME and for tracking them, i.e., noting anyt new symptom or changes in severities.

See our information re measuring and tracking symptoms in ME here

Adrenaline Issues 

People with M.E. can sometimes operate significantly above their actual illness level for certain periods of time because of surges of adrenaline - albeit at the cost of severe and prolonged worsening of the illness afterward. More here 

Paralysis 

Paralysis is a terrifying symptom that has often been reported in feedback from people with Severe ME, yet it is rarely referred to and has not been researched. Paralysis in ME means the patient cannot feel and cannot move and has no choice. Paralysis in ME can take over part of the body or the whole body, i.e., partial, or full body paralysis. More here

Oesophageal Spasms

Muscle spasms have been found to occur in people with ME; they include oesophageal spasms.  Muscle spasms are unintentional, involuntary, and painful muscle contractions. The pain from spasms can be very sharp and intense.

Oesophageal Spasms can be felt as extreme pain in the centre of the chest that sometimes radiates to the chest or mid-back & difficulty swallowing or an inability to swallow. More here

Excessive Thirst

Many ME patients suffer from polydipsia – a condition that involves unquenchable thirst, dilute urine, a worsening of thirst during a post-exertional response phase (PENE) and, at least in some patients, the development of hyponatraemia (low blood sodium). Patrick Ussher, an Irish ME patient, used to suffer from this symptom at its most extreme to the point that he developed life-threatening hyponatraemia and was hospitalised.  More here

Visual Disturbances

Visual issues in ME are frequently discussed and may have a significant impact on the quality of everyday life for the ME patient. Some people have to stop driving or find reading or watching television to be a problem. The De Paul Symptom Questionnaire, the DSQ-2 which is based on research includes questions based on visual issues in ME; e.g., eye pain, sensitivity to bright lights, inability to focus vision, loss of depth perception, blurred vision or tunnel vision after standing, aching of the eyes or behind the eyes. More here

Malnutrition

One often overlooked but crucial issue that significantly impacts the lives of individuals with severe Myalgic Encephalomyelitis (ME) is malnutrition. Patients with very severe ME can experience difficulty maintaining their nutrition and hydration. In the most severe cases it is not uncommon. The most common reason for malnutrition in a severe ME patient is ME associated debility. There are a variety of other reasons which we will discuss below. More here

Reasonable Adjustments while in Hospital

Supporting Individuals with Severe ME in a Hospital or Other Healthcare Setting Information Pack here  (the pack which includes signs for the door/over the door and link to signs to support an individual with communication issues during a hospital stay)

Much thanks to Linda Crowhurst and Greg Crowhurst for much of our content re Severe ME

Disclaimer: The information in this post is for general information purposes only. While we endeavour to keep the information up to date and correct, we make no representations or warranties of any kind, express or implied, about the completeness, accuracy, reliability, suitability or availability with respect to the post or the information, products, services, etc contained in the post for any purpose. Any reliance you place on such information is therefore strictly at your own risk.The suitability of any solution is totally dependent on the individual. It is strongly recommended to seek professional advice and assistance.