Tuesday, 10 May 2022

About the Potential Symptoms of Myalgic Encephalomyelitis (ME)

Image behind our script by Jennifer Walker, Texas - her art is a medium through which she can convey what it's like to physically & emotionally cope with chronic pain

Myalgic Encephalomyelitis (ME)

Myalgic Encephalomyelitis is a chronic, fluctuating, multi-systemic, neurological disease with many organ and bodily systems affected, producing a myriad of symptoms. It is characterized by neurological, immune, cardiac, energy production and ion transport dysfunction. The main feature is exertion intolerance where there is severe worsening of symptoms following any exertion.

There are several levels of severity in ME, ranging from mild to profound ME and which include mild, moderate, severe, very severe and profound ME. Within each range of severity are other levels which means that within the mild range of ME there may be significant impact on an individual’s health. Severe to very severe ME sufferers are profoundly affected. They can be completely immobile, lying still in darkened rooms, requiring help with their basic needs.

People with ME can experience debilitating pain, inability to be active, lack of energy (ME is an inability to properly generate energy which prevents sufferers from being active mentally or physically), cognitive difficulties, multiple sensitivities, a range of other symptoms associated with post-exertional neuroimmune exhaustion (PENE) - the body and brain’s inability to recover after using even small amounts of energy, and marked fluctuation of symptom severity from day to day or hour to hour.

The physical symptoms of ME can be as disabling as multiple sclerosis, systemic lupus, rheumatoid arthritis, congestive heart failure and other chronic conditions. Research shows that people with ME score lower overall on health-related quality of life tests than most other chronic conditions.

Very few people make good progress or recover, many can remain ill for a number of years and may not get better. Feedback shows that those who experience recovery and remission may relapse so it may always be necessary to manage the illness to avoid relapse. Feedback shows us that people who experience remission don’t go back to complete full health the way they felt before they became ill, but they do recover sufficiently to lead a fairly fulfilling life.

A few important points about ME before we look at the symptoms in ME.

Myalgic Encephalomyelitis (ME) is a severely debilitating disease affecting millions of people around the world. Prevalence: 0.4 – 1%. ME affects an estimated 10,000 -19,000* people in Ireland; 250,000 people in the UK.

ME is a physiological illness that is defined by the World Health Organisation (G93.3) as neurological. (Ireland’s NASS use the WHO code G93.3)

There is no formal collation of numbers diagnosed with ME so we don't have official figures.

Despite the high numbers suffering from ME in Ireland there are no HSE ME Clinical Guidelines and there’s no information on the HSE website which means that healthcare for those with ME can be non existant or inappropriate.

A lack of understanding and awareness about ME means patients can experience disbelief, discrimination, and gaslighting from friends and family, and from health and community services.

There is much discussion about the underlying cause of ME, about treatments and what does/doesn’t work. There is currently no single diagnostic tool nor drug treatment directed specifically at ME, treatment of some of a person's individual symptoms is usually how ME is managed as well as pacing and resting. Because of the complexity of ME, its fluctuating nature and the wide range of symptoms, different things work for different people. For those with severe ME complete rest and stillness is necessary, pacing is not a management option and currently available treatments of little benefit. Multiple sensitivities in severe ME may cause harm and it may not be always possible to prevent certain sensitivities causing harm.

Prognosis: Currently there is no known cure. Early intervention and appropriate treatment strategies may lessen severity of symptoms. Restoration to full pre-morbid health and function is rare. Prognosis for an individual cannot be predicted with certainty. Paediatric: Children can be very severely afflicted. Children with less severe symptoms are more likely to go into remission than adults.

ME includes different severities i.e., mild/moderate/severe/very severe/profound ME.

* Figures are based on International prevalence rates: These figures are extrapolations based on a comparison of prevalence rates worldwide as there is no official collation of data on prevalence here in Ireland, and caution is urged with these figures as the diagnosis of ME is not always made using the best clinical evidence available, i.e. the International Consensus Criteria - ME 2011. The criteria for a diagnosis of Chronic Fatigue Syndrome (CFS) for example is less strict and there are concerns that the numbers of true ME patients may be over-estimated as a result.

Symptoms in Myalgic Encephalomyelitis (ME)

Research shows, due to the severity of symptoms, 75% of people with ME are unable to work and 25-29% are unable to leave their home or bedroom due to profound symptoms. ME is more debilitating than many chronic illnesses including multiple sclerosis, chronic renal failure, lung cancer, stroke, and type 2 diabetes.

Symptoms can fluctuate and vary in severity. Individual symptoms vary widely in severity and people may have some symptoms more severely than others. ME can be unpredictable - symptoms may come and go - some symptoms may disappear and new symptoms may come along, or there may be changes in how they improve or how bad they are over time.

A person's experience with ME and symptoms associated with ME can vary enormously. Not everyone will experience the same symptoms so it's important not to compare yourself or someone who has ME to another person who has ME.

Symptom management should not be delayed until a diagnosis is established. Management should be tailored to the specific symptoms the person has and be aimed at minimising the impact of daily life and activities on symptoms and exertional response, i.e., PENE.

NB: The presence or absence of ‘fatigue’ is largely irrelevant in determining an ME diagnosis.

List of Potential Symptoms/Features in ME

Here is a list of potential symptoms that can occur in ME; it includes symptoms as described by those who responded to a question posed by ME patient advocate, carer and writer Greg Crowhurst, and advocate, artist and severe ME patient Linda Crowhurst, when they looked for more information and recognition of the true symptoms of ME and people’s actual experience of the illness.

Post-Exertional Neuro Immune Exhaustion (PENE)

Post exertional neuro immune exhaustion is the cardinal feature of ME as per the International Consensus Criteria (ICC). PENE is sometimes referred to by others as ‘payback’ / ‘crash’ / ‘relapse’/’PEM’.

Energy issue

Lack of energy most of the time which feels different from ordinary fatigue. Ability to make energy at cell level is lost.

Pain

Persistent pain

Chronic nerve pain

Crushing pain

Global wide-spread pain

Muscle pain

Joint pain

Jolts of pain

Increased sensitivity to pain

Painful feet

Glandular pain

Sore throat (recurrent)

Hoarseness

Swollen glands

TMJ

Trigeminal neuralgia

Occipital neuralgia

Chest pain

Back pain

Twitching muscles or cramps

Abdominal pain (stomach or bowel problems)

Costochondritis (inflamed chest wall)

Skin crawling sensations

Burning itching

Stabbing pain

Aching

Skin pain

Eye pain, back of eyes pain

Vein inflammation pain

Severe menstrual pain

Swelling

Inflammation

Cramps

Migraine

Generalised head pain

Neck pain

Cognitive dysfunction

Problems concentrating, e.g. an inability to follow conversation

Lack of ability to process information

Loss of ability to plan

Loss of ability to think

Loss of memory

Poor short term memory

General forgetfulness

Loss of speech

Slowed Speech

Slurring

Forgetting names

Not recognising faces

Word loss

Inability to understand

Inability to identify left from right

Switching letters round in words

Difficulty retaining information

Mental Confusion

Difficulty concentrating

Unable to hold two-way conversation

Disorientation

Not fully conscious

Inability to read (without a learning issue present)

Inability to write (without a learning issue present)

Problems with thinking

Cognitive overload

Reduced attention span

Word-finding difficulties

Inability to or organise thoughts and/or speech

Cardiac issues

Palpitations

Cardiomyopathy

Bradycardia

Tachycardia

Orthostatic intolerance

POTS

Insufficient increase in blood pressure on exertion

Reduced blood flow

Arterial elasticity dysfunction

Reduced heart rate variability during sleep suggests a pervasive state of nocturnal sympathetic hyper-vigilance and may contribute to poor sleep quality

Vascular abnormalities suggest there is insufficient circulating blood volume in the brain when in an upright position, and blood may pool in the extremities.

Respiratory issues

Respiratory depression

Air hunger

Chest pain

Laboured breathing

Breathing difficulty

Pins and needles

Oxygen depletion

Shortness of breath

Multiple sensitivities

(intolerance to light/sound/smell/movement/touch/food/materials/other)

Hyperacusis (noise sensitivity)

Photophobia (light sensitivity)

Hypersensitivity to smells

Hyperesthesia (touch sensitivity)

Motion sensitivity

Chemical sensitivity

Drug sensitivity

Food sensitivity

Alcohol sensitivity/intolerance

Materials sensitivity/intolerance

Vibration intolerance

Visual disturbances

Staring

Inability to focus

Impaired depth perception

Poor spatial recognition

Fuzzy/ blurred vision

Double letter vision

Tunnel vision

Eye pain

Dry eyes (do not hold tear film)

Itching eyes

Burning eyes

Flashing eyes (open or shut)

Seeing pinpoints of light in the dark

Paralysis

Total paralysis

Partial paralysis

Channelopathy

Elevated oxidative stress markers, ncreased with exertion

Gastrointestinal issues

Loss of appetite

Indigestion

Excessive wind/bloating

Stomach Cramps

Alternating diarrhoea and constipation

Incontinence

Nausea

Vomiting

Food Allergies (multiple)

Gastric issues

Swallowing difficulties

Choking

Acid reflux

IBS

Gut and bowel issues

Food sensitivity

Malabsorption issues

Weight loss

Hypoglycaemia

(Note: some of the above symptoms may lead to life threatening malnutrition in severe ME cases)

Genitourinary issues

Urinary urgency

Urinary frequency

Excessive urination at night

Dental issues

Mercury intolerance (silver fillings)

Temperature control problems/ dysregulation & loss of thermostatic stability

Sweating profusely

Feeling feverish

Feeling extreme cold, cold extremities

Abnormal body temperature

Temperature fluctuations

Heat intolerance

Cold Intolerance

Poor Circulation

Sleep difficulties

Hypersomnia

Lucid dreaming

Insomnia

Sleep disruption - frequent awakening

Unrefreshing sleep

Sleep apnea

Nightmares

Inability to stay awake

Difficulty getting to sleep

Waking for long periods

Restless sleep

Sleep reversal (e.g. sleeping from 4am till noon)

Flu-like symptoms

Sore throat

Tender Lymph nodes

Susceptible to infection

Susceptible to repeated infections

Slow recovery

More symptoms/features

Excessive Thirst

Tingling

Peripheral neuropathy

Tremor

Muscle spasms (shaking) including in the throat)

Twitching

Violent, uncontrollable ‘tics’ in limbs

Restless legs

Muscle wasting

Lack of strength

Heavy limbs

Muscle weakness

Hyperacusis (sensitivity to noise sometimes alternating with deafness or normal hearing)

Tinnitus (constant sound in the ears)

Feeling of toxicity

Loss of sensation

Paraesthesiae: numbness or tingling in the extremeties

Pins & needles

Loss of proprioception

Sinus problems

Extreme excess mucous

Severe anxiety

Hypnogogic jerks

Hypoglycaemia leading to fainting attacks

Dizziness

Vertigo

Black outs

Light-headedness

Low and/or high blood pressure

Dysautonomia

Dystonia

Loss of equilibrium

Loss of balance

Inability to stand

Inability to walk

Inability to sit

Poor/loss of co-ordination

Clumsiness

Raynaud’s phenomenon (poor circulation in fingers and toes)

B12 deficiency is common

Vitamin D deficiency is common

Endocrine dysfunction

Seizures

Unconsciousness

Inability to hold things

Inability to lift things

Sensory overload

Severe sensory issues

Loss of touch

Loss of taste

No energy

No strength

Lack of stamina

Disabling fatigue* (please see more re fatigue below)

Emotional lability

Compromised immune system

Dehydration

Excessive saliva

Mouth sores

Kidney infections

Crippling Exhaustion

Chronic infections

Slow recovery from colds/flu

Cysts

Skin rashes

Fevers

Loose joints

Easily dislocated joints

Hair loss

Weight loss

Weight gain

Dehydration

Compromised immune system

Fainting

Discomfort all over

Feeling generally unwell (malaise)

Feeling generally exhausted, exhaustion reached more rapidly

Comments:

Within each symptom there may be a wide spectrum of severity, from mild to moderate to severe to very severe and profound. There may be marked fluctuation of symptom severity from day to day or hour to hour.

You or a person with ME may only experience some of the symptoms listed above, and not all at the same time. Everyone with ME has a different range of symptoms and severities.

We are not medics but our advice is to always get new or worsening symptoms checked by your doctor so that they can assess whether the symptoms are part of your ME or are caused by something different.

As before, treat individual symptoms as far as possible, looking at the worst symptoms first (eg. pain, sleep issues, restless legs orthostatic intolerance, irritable bowel syndrome, migraine, headaches, etc).

Because of the complexity of ME - its fluctuating nature and the broad range of symptoms, different management strategies work for different people. Not all prescription drugs/over the counter medicines/supplements work in the same way for everyone so it may be a case of trial and error getting the drug/medicine/supplement and dosage right for an individual. Some people may be intolerant to drugs/medicines/supplements and may have to look into alternative therapies.

For the millions of patients suffering from the intense symptoms of ME additional research is needed to understand the disease process, identify biomarkers for diagnostic criteria and determine appropriate treatment for care.

How the person with Severe/Very Severe/Profound ME Feels

(as well as suffering from the above debilitating symptoms/features) by Greg & Linda Crowhurst:

• Totally dependent

• Extreme discomfort

• Trapped inside a numb, empty shell of a body

• No possible movement

• Total paralysis

• 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

• Shaking

• Cannot sit up/stand/move or walk

• Extremely dizzy

• Struggles to breath

• Eating is difficult so tube fed

• Cannot comprehend information

• Light hurts and noise damages

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

• Zero energy to function

• Lives in a fog of inability.

* The Problem with ‘Fatigue’

People in the ME community have different severities of ME- Mild, Moderate, Severe, Very Severe, Profound. Within each symptom suffered there is a fluctuating range of severity where symptoms can be either mild, or moderate or severe, or very severe or profound. No matter what the degree of severity people with ME do not describe themselves as being fatigued and they do not relate to ‘fatigue’ as their primary symptom. The presence or absence of ‘fatigue’ is largely irrelevant in determining an ME diagnosis.

There is a concern that the real symptoms of ME (mild to profound) are being covered up under the term fatigue, or ignored.

According to the International Consensus Criteria (ICC) 2011:

"Using ‘fatigue’ as a name of a disease gives it exclusive emphasis and has been the most confusing and misused criterion. No other fatiguing disease has ‘chronic fatigue’ attached to its name – e.g., cancer/chronic fatigue, multiple sclerosis/chronic fatigue – except ME. Fatigue in other conditions is usually proportional to effort or duration with a quick recovery and will recur to the same extent with the same effort or duration that same or next day."

Diagnosis

A doctor needs to ask about the symptoms suffered by the person with ME to learn how often they occur, how bad they are, and how long they have lasted. It is also important for doctors to talk with patients about how the symptoms affect their lives.

As well as a symptom checklist, a thorough clinical evaluation involving history taking, physical examination and investigations should be carried out to exclude other medical conditions that can cause ME-like symptoms.

Doctors need to ask questions such as:

- What are you able to do now?
- How does it compare to what you were able to do before?
- How long have you felt this way?

- Can you remember when your symptoms began? Was it a sudden/gradual onset?
- What makes you feel worse? What helps you feel better?
- What happens when you try to do activities that are now difficult for you?

- What symptoms keep you from doing what you want to do?
- What symptoms keep you from doing what you need to do, e.g., your job?

For a diagnosis of Myalgic Encephalomyelitis (ME), symptoms and symptom severity must result in a significant reduction of a patient’s premorbid activity level.

Adult Diagnosis & Management

International Consensus Primer (ICP):

The ICC and ICP encompass symptoms that capture the unique characteristics of ME. Tthe ICP is an effective tool to help medical practitioners diagnose and manage ME. It also offers thorough screening guidance to help identify other treatable conditions. Operational notes following criterial categories clarify how symptoms may be expressed and interpreted. Grouping symptoms by regions of pathogenesis provides focus.

For Diagnosis see the 2012 page 7, 8 ICP

For Clinical Assessment and Diagnostic Worksheet see ICP pages 10-12 ICP

For Personalised Management and Treatment see ICP pages 13 - 18 ICP

Symptom and Severity Questionnaires

The De Paul Symptom & Severity, and PEM Questionnaires are useful tools to use alongside the International Consensus Primer (ICP) to evidence and track your symptoms and symptom severities, and to show your doctors or anyone who you feel should see your symptom profiles.

These tools can be printed, then completed and handed to the doctor at an appointment, or emailed, you could ask that they are kept in your medical file.

See links to those questionnaires below.

De Paul DSQ-2 Symptom & Severity Questionnaire: DSQ-2

De Paul Post Exertional Questionnaire (short form): DSQ-PEM

De Paul Post Exertional Questionnaire (long form): DPEMQ

‘The danger is that the lengthy list of symptoms 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.’
~Greg Crowhurst~

Paediatric Considerations

Symptoms may progress more slowly in children than in teenagers or adults. In addition to post exertional neuroimmune exhaustion, the most prominent symptoms tend to be neurological: headaches, cognitive impairments, and sleep disturbances.

1. Headaches: Severe or chronic headaches are often debilitating. Migraine may be accompanied by a rapid drop in temperature, shaking, vomiting, diarrhoea, and severe weakness.

2. Neurocognitive impairments: Difficulty focusing eyes and reading are common. Children may become dyslexic, which may only be evident when fatigued. Slow processing of information makes it difficult to follow auditory instructions or take notes. All cognitive impairments worsen with physical or mental exertion. Young people will not be able to maintain a full school programme.

3. Pain may seem erratic and migrate quickly. Joint hypermobility is common.

Pediatric Management

International Consensus Primer:

Prompt treatment can lessen the impact of ME in some cases. Monitor the child’s health on an ongoing basis. Management is similar to adults. Great caution is required in prescribing any medications - use low dosage. Involvement of family members is essential. They monitor the child’s health and are the primary care givers.

For management and treatment see the International Consensus Primer (ICP) 2012 page 9

For paediatric personalized treatment see page 19 ICP

Paediatric Questionnaires:

The De Paul Symptom & Severity Paediatric Questionnaires are useful tools to use alongside the International Consensus Primer (ICP) to evidence and track your child's symptoms and symptom severities, and to show doctors or anyone who you feel should see your child's symptom profiles. These tools can be printed, then completed and handed to the doctor at an appointment, or emailed, you could ask that they are kept in your child's medical file.

DePaul Symptom Questionnaire – Pediatric Screening Questionnaire (DSQ-PSQ):

DSQ-Ped: Parent Form
DSQ-Ped: Child Form

‘Each child (all young people) will have his/her own unique combination of the ME criteria symptoms. The onset of ME in children often occurs around twelve years of age but it has been diagnosed in a child who was two years old. More than one member of a family may have ME or other neurological diseases.’

About Myalgic Encephalomyelitis (ME)