Myalgic Encephalomyelitis (ME) & Severe ME: Diagnosis & Management Information for Doctors & Healthcare Providers

 

Myalgic Encephalomyelitis

(ME)

 

Diagnosis & Management

 

Patients are our best experts, learn from ME

What people with ME & Severe ME want doctors 

& other healthcare staff to know 

Patients, especially those with Severe ME want to be listened to and believed. 

They want to hear the words 'I believe you'.

They do not want to be harmed, they already suffer from an horrendous debilitating illness. 

They want doctors and other healthcare staff to address impaired function and provide support as needed.

They want doctors to know they have many symptoms, and that each symptom that can be treated will improve their quality of life.

 Patients need a doctor who will listen and try to help, or at the very least, one who will admit they do not know about ME but will do their best to learn about ME and support the patient.

Myalgic Encephalomyelitis (ME) 

WHO Classification ICD 10 G93.3 classified as a Neurological disorder

WHO Classification ICD 11 8E49 classified as a Neurological disorder

SNOMED Classification SCTID: 118940003 Disorder of the nervous system (Disorder)

NASS (HRB) G93.3

Myalgic encephalomyelitis 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. Although signs and symptoms are dynamically interactive and causally connected, the criteria are grouped by regions of pathophysiology to provide general focus. How is ME Diagnosed The symptoms of ME are numerous and can include but are not limited to the following:

A patient will meet the criteria for postexertional neuroimmune exhaustion (A), at least one symptom from three neurological impairment categories (B), at least one symptom from three immune/gastro-intestinal/genitourinary impairment categories (C), and at least one symptom from energy metabolism/transport impairments (D).

A. Postexertional neuroimmune exhaustion (PENE pen’-e): Compulsory

This cardinal feature is a pathological inability to produce sufficient energy on demand with prominent symptoms primarily in the neuroimmune regions. Characteristics are as follows:

1. Marked, rapid physical and/or cognitive fatigability in response to exertion, which may be minimal such as activities of daily living or simple mental tasks, can be debilitating and cause a relapse.

2. Postexertional symptom exacerbation:e.g.acute flu-like symptoms, pain and worsening of other symptoms.

3. Postexertional exhaustion may occur immediately after activity or be delayed by hours or days.

4. Recovery period is prolonged, usually taking 24h or longer. A relapse can last days, weeks or longer.

5. Low threshold of physical and mental fatigability (lack of stamina) results in a substantial reduction in pre-illness activity level.

B. Neurological impairments

At least one symptom from three of the following four symptom categories

1. Neurocognitive impairments

a. Difficulty processing information: slowed thought, impaired concentration e.g. confusion, disorientation, cognitive overload, difficulty with making decisions, slowed speech, acquired or exertional dyslexia

b. Short-term memory loss:e.g. difficulty remembering what one wanted to say, what one was saying, retrieving words, recalling information, poor working memory

2. Pain

a. Headaches:e.g. chronic, generalized headaches often involve aching of the eyes, behind the eyes or back of the head that may be associated with cervical muscle tension; migraine; tension headaches

b. Significant pain can be experienced in muscles, muscle-tendon junctions, joints, abdomen or chest. It is noninflammatory in nature and often migrates. e.g. generalized hyperalgesia, widespread pain (may meet fibromyalgia criteria), myofascial or radiating pain

3. Sleep disturbance

a. Disturbed sleep patterns:e.g. insomnia, prolonged sleep including naps, sleeping most of the day and being awake most of the night, frequent awakenings, awaking much earlier than before illness onset, vivid dreams/nightmares

b. Unrefreshed sleep:e.g. awaken feeling exhausted regardless of duration of sleep, day-time sleepiness

4. Neurosensory, perceptual and motor disturbances

a. Neurosensory and perceptual:e.g. inability to focus vision, sensitivity to light, noise, vibration, odour, taste and touch; impaired depth perception

b. Motor:e.g. muscle weakness, twitching, poor coordination, feeling unsteady on feet, ataxia

C. Immune, gastro-intestinal and genitourinary Impairments

At least one symptom from three of the following five symptom categories

1. Flu-like symptoms may be recurrent or chronic and typically activate or worsen with exertion.e.g. sore throat, sinusitis, cervical and/or axillary lymph nodes may enlarge or be tender on palpitation

2. Susceptibility to viral infections with prolonged recovery periods

3. Gastro-intestinal tract:e.g. nausea, abdominal pain, bloating, irritable bowel syndrome

4. Genitourinary: e.g. urinary urgency or frequency, nocturia

5. Sensitivities to food, medications, odours or chemicals

D. Energy production/transportation impairments: At least one symptom

1. Cardiovascular:e.g. inability to tolerate an upright position - orthostatic intolerance, neurally mediated hypotension, postural orthostatic tachycardia syndrome, palpitations with or without cardiac arrhythmias, light-headedness/dizziness

2. Respiratory:e.g. air hunger, laboured breathing, fatigue of chest wall muscles

3. Loss of thermostatic stability:e.g. subnormal body temperature, marked diurnal fluctuations; sweating episodes, recurrent feelings of feverishness with or without low grade fever, cold extremities

4. Intolerance of extremes of temperature

Paediatric considerations

Symptoms may progress more slowly in children than in teenagers or adults. In addition to postexertional 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.

Notes: Fluctuation and severity hierarchy of numerous prominent symptoms tend to vary more rapidly and dramatically than in adults. [Severe ME considerations Severe ME, which includes those with severe, very severe and profound ME, sees patients suffering from a horrendous, disabling form of Myalgic Encephalomyelitis. These patients are isolated/confined to bed due to the severity of their symptoms and disabilities, and are often unable to leave their home even to seek medical care.]

Myalgic Encephalomyelitis:  International Consensus Criteria 2011

The most appropriate diagnostic criteria for Myalgic Encephalomyelitis are the International Consensus Criteria  and the Primer, supported by research. Scientific advances have revealed a complex, multisystem disease involving neurological, immunological, autonomic, energy and ion transport impairments. 

ME has previously been undermined by lax criteria that has contributed to misdiagnoses and contributed to ongoing stigma and discrimination. 

The Oxford, Fukuda and NICE 2007 criteria should not be used, and research studies using those criteria do not apply to ME.

Myalgic Encephalomyelitis (ME) is a common multi-system, multi organ physiological disease often with devastating consequences.

ME affects millions people globally, thousands in Ireland. 

Classified by the World Health Organization ICD and SNOMED CT as a neurological condition, ME causes severe disability.  

ME most often manefests post virally. 

It can affect people of any age or demographic, including children.

The ability of the body to create energy is flawed

Mitochondrial tests show that the  ATP production is poor:

Low whole-cell ATP

Low mt-ATP and very poor provision of ‘new’ mt-ATP

Very rapid depletion of ATP on increased energy demand

When there is no energy, systems in the body shut down – this is what happens in a crash, where extreme paralysis can occur, particularly in those with severe ME.

Points to Consider About Myalgic Encephalomyelitis (ME)

• Exertion Intolerance Response is the distinguishing feature of ME, referred to as Post Exertional Neuro-immune Exhaustion (PENE) by the International Consensus Criteria and Primer (also referred to as PEM by others). 

Further distinguishing features of PENE include:

• Immediate onset: symptom exacerbation may occur especially for people with Severe ME
• Delayed onset: symptom exacerbation may occur 24 to 48 hours after exertion 
• Variable and often prolonged period of recovery to baseline illness severity and symptoms. 

Patients may use language such as 'crash', 'setback', 'relapse', 'payback' to describe exertion intolerance, i.e., PENE. The DePaul PEM questionnaire may be helpful to establish if a patient is experiencing an exertion intolerance response, i.e., PENE. 

• People with ME must reduce and adapt their activities to conserve energy. This includes all types of activity including physical, cognitive, social, emotional and stress activity, and takes into account overall level of activity. Management consists of teaching patients to stay within their energy limits to avoid triggering a post exertional response, i.e., PENE. Pacing activity and resting throughout the day can help manage symptoms and prevent deterioration. 

However, people with Severe ME spend days struggling to cope from moment to moment in the most awful suffering. For those pacing and resting may not be an option to manage ME because the body can be in such turmoil that patients can't pace or rest enough to impact disease processes, for example, someone who has severe/very severe or profound ME who lies completely still because any movement at all can cause awful debilitating symptoms. For those patients no amount of pacing can help if digesting food requires more energy than the body can produce; no amount of pacing can help when trying to keep warm; no amount of pacing can help when the person has multiple sensitivities and is already responding negatively to light or sound or movement.

 

• The patient's ability to engage in occupational, educational, social or personal activities is significantly reduced from pre-illness levels. 

• Biological abnormalities are observable in research contexts with the two day cardio-pulmonary exercise tests, and in metabolic and other findings.

• Illness severities include mild, moderate, 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, i.e., someone with mild or moderate ME may suffer from severely disabling symptoms.

• ME can be profoundly disabling. Most patients are disabled and cannot work, and about 25% are confined to bed and cannot care for themselves, some may require fulltime care, i.e., 24hrs a day.

• ME can affect different aspects of the life for people with ME and their families and carers, including activities of daily living, family life, social life, emotional well-being, work, education and finances. 

• Early diagnosis enables appropriate management that may reduce the risk of the patient's condition deteriorating and reduce further disability. Early diagnosis enables referral for practical supports as well as vital support services, and provides answers for the patient. There is no requirement that the individual have symptoms for a specified period of time for diagnosis. For pediatric and adult cases a diagnosis should be made immediately; there is no need to wait up to 6 months, as per the International Consensus Criteria.

• Diagnosis and management is possible in a general practice setting, alongside a multi-disciplinary team, but likely not  suitable for those with severe and profound ME. 

• A thorough medical workup is required for a diagnosis of ME, which includes medical history, physical examination, appropriate tests and differential diagnosis. 

• Diagnosing ME may take several visits. Discuss with your patient how best to balance multiple medical visits with their disabilities and energy limitations.

  

• Keep in mind some patients may not appear unwell during clinic visits, will likely need rest before and after an appointment, and recovery time may be required, those with more severe ME may suffer from relapse (PENE) because the exertion required to even get dressed for a medical appointment may be too much.

 

• More severe patients, those who are very severe and profoundly severe may not be able to leave the house for medical appointments. Important or urgent hospital visits are extremely difficult and can cause immediate relapse which may last for days, months or years. 

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

• The belief that ME has no diagnostic marker and is a diagnosis of exclusion is a misrepresentation. Exclusion is critical to avoid misdiagnosis of other physical diseases as ME, it is important to rule out other potential causes for symptoms and to look for comorbidities. As in all diagnoses, exclusion of alternate explanatory diagnoses is achieved by the patient’s history, physical examination, and laboratory/biomarker testing as indicated. It is possible to have more than one disease but it is important that each one is identified and treated. Exertion intolerance response characterised by Post Exertional Neuroimmune Exhaustion (PENE), also referred to as PEM, is a cardinal symptom of ME. 

• ME is not psychosomatic, and psychological therapies will not cure the disease. 

• Comorbid Entities include: Fibromyalgia, myofascial pain syndrome, temporomandibular joint syndrome, irritable bowel syndrome, interstitial cystitis, Raynaud’s phenomenon, prolapsed mitral valve, migraines, allergies, multiple chemical sensitivities, Hashimoto's thyroiditis, Migraine and irritable bowel syndrome may precede ME but then become associated with it.

• It is important to distinguish between Myalgic Encephalomyelitis and Chronic Fatigue Syndrome to improve understanding of the disease, treatment and patients’ lives, as using incorrect criteria can lead to incorrect treatment. (it must be acknowledged that some patients have been diagnosed with the CFS label or use the CFS label to refer to a set of symptoms and the cardinal feature PENE as per ME.

• There is a critical need for an informed and educated approach to management of the disease and accompanying comorbidities.

Compassionate Care

While there is currently no cure for ME, compassionate care can make a vast difference to the patient experience. ME patients have traditionally been treated with disbelief, disdain, neglect, and abuse, including gaslighting.

Many patients have found healthcare encounters so traumatic that they avoid it altogether, to the detriment of their health.

ME Advocates Ireland (MEAI) recommend a patient centred approach. A doctor who is willing to work with the patient to manage and treat their symptoms is invaluable.

Compassionate care includes a multidisciplinary approach where each person involved in the patient's care, including other healthcare staff and the patient's carer(s), coordinates among the various doctors, specialists and healthcare staff involved with a patient, and especially with a complex case of severe ME. 

Doctors should:

• Listen to their patients

• Believe their patients

• Learn about ME - patients want a doctor who is willing to support, and if that means taking the time to learn about a particular aspect of their illness, they will be happy to wait

• Learn about Severe/Very Severe/Profound ME, paying particular attention to (1) the possibility of paralysis, (2) to heightened sensory sensitivities including to light, sound, touch, taste, smell, movement (patient's movement and motion by other individuals or things), food, chemicals, prescription medicines, fabric, temperatures; (3) to horrendous widespread pain 

• Diagnose ME - patients deserve a concrete diagnosis and to be validated 

• Treat symptoms where possible

• Advise patients re management strategies such as pacing, resting, etc where possible

• Provide support in applications for welfare and community services

• Refer patients to specialist care, preferably where there exists ME-aware practitioners

• But be prepared to look after those who are unable to access a specialist, who do not wish to be referred to a specialist, or do not feel that their experience attending a specialist, has been helpful

• Review individual cases regularly e.g., investigate new symptoms or change in symptoms to determine if they are due to ME or another condition 

• Protect patients, especially children, from wrongful safeguarding and misinformed practitioners and other healthcare staff

• Protect patients from inappropriate psych referrals, and psychological or psychiatric labels

• Be accessible - provide alternative measures for those with severe ME in other ways e.g., home visits, telephone or virtual appointments (zoom/other); nutritional support where there is malnutrition due to symptoms associated with severe ME

• Refer patients early, especially severe ME patients, for nutritional assessment and support to prevent deterioration from avoidable malnutrition

• More recognition of other interventions such as IV fluids, peg feeding, etc for people with very severe/profound ME is necessary

• Lack of help and guidelines from the HSE and the Dept. of Health means that patients have had to help themselves and learn from their experiences and other patients' experiences, please keep an open mind that patients are the experts and doctors can learn from patients who will be able to provide doctors and other healthcare staff with relevant information and resources.

 

Resources for Diagnosing ME

• International Consensus Criteria  - research and clinical definition

• International Consensus Primer - (based on the International Consensus Criteria) how to understand, diagnose and manage the symptoms of ME.

• Questionnaires for Diagnosing or confirming a suspected diagnosis in ME

• Patient Doctor Check List Based on ME- ICC from GAME - ICC 

• Alternative Simple Tool to determine if the patient meets the ICC Criteria

• Important Patient Documents To Consider

• Useful Information Sheets by ME International (US)

• Films about ME 

International Consensus Criteria for Adult and Paediatric ME 

The International Consensus Criteria (2011), a medical case definition that can accurately diagnose Myalgic Encephalomyelitis (ME), can identify the unique and distinctive characteristic patterns of symptom clusters of ME. The broad spectrum of symptoms alerts medical practitioners to areas of pathology and may identify critical symptoms more accurately. Operational notes following each criterion provide guidance in symptom expression and contextual interpretation. This will assist the primary clinician in identifying and treating patients with ME in the primary care setting.

International Consensus Primer for Adult and Paediatric ME for Medical Practitioners 

The International Consensus Primer for Medical Practitioners (2012) was written about ME to aid clinicians in diagnosis and management. It allows one to make a positive diagnosis based on symptom constellation, without having to wait six months. This is important as it allows timely diagnosis and management. Diagnostic delay and lack of crucial medical advice in the early part of the illness frequently results in significant harm and increased severity of illness. The Primer is a valuable tool for working with people with suspected ME to get a diagnosis and treatment without delay. 

Content: 

Pathophysiology

Post-Exertional Neuroimmune Exhaustion (cardinal feature), Neurological Abnormalities,  Immune Impairments,  Energy Production and Ion Transport Impairments ...... pages 2-6

Clinical Assessment and Diagnosis 

Post-Exertional Neuroimmune Exhaustion (PENE pen׳-e) (compulsory), Neurological Impairments, Immune, Gastro-intestinal & Genitourinary Impairments, Energy Metabolism/Ion Transportation Impairments, Clinical & Physical assessments  .......pages 6-11

Lab Tests

Laboratory/Investigative Protocol: Diagnose by criteria. Confirm by laboratory and other investigations ......pages 11-12

Paediatric Considerations

Each child (all young people) will have his/her own unique combination of the ME criterial 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 ......pages 9 & 19

 

Questionnaires for Diagnosing or confirming a suspected diagnosis in ME & for Assessing Symptom Severity & Impact

Along with the Primer doctors could use several questionnaires, which are based on research, to aid a diagnosis. These include the De Paul Symptom & Severity, PEM and Paedriatric questionnaires and the Bells Disability Scale. All questionnaires can also be used for management purposes following diagnosis to track impact of symptoms and severity, to identify new symptoms and keep a record.

• Symptom and Severity DSQ-2 Questionnaire

• Post Exertional Intolerance DSQ-PEM Questionnaire

• Paediatric Questionnaires: 

Parent Form DSQ-Ped here

Child Form DSQ-Ped here

• Bells Disability & Severity Scale 

Patient Doctor Check List Based on the ICC

Patient Doctor Check List Based on ME- International Consensus Criteria (ICC) from GAME - ICC, thanks to Wendy Boutillier here

Alternative Simple Tool to determine if the patient meets the ICC Criteria

An online questionnaire was developed by Schweizerische Gesellschaft für ME, a patient association in Switzerland, to help determine whether the diagnosis of ME according to the International Consensus Criteria (ICC) is applicable to a patient. This tool could be used as an easier/shorter alternative to the De Paul symptom and severity questionnaires by the doctor as well as the patient: ICC Questionnaire

Important Patient Documents To Consider

Seldom do two ME patients present with the same set of symptoms and severities, a person with ME has a unique set of symptoms and fluctuating severities, as well as their own unique response to exertion, and response to medications, With that in mind it is important to know that each patient a doctor or other healthcare staff meets will have their own individual needs.

Included below are two documents which have been completed by the patient to outline their individual needs. They are carried by some patients who attend in a healthcare setting such as a hospital or in ED and include in-patient information and discharge planning notes, i.e., Hospital Transfer/Hospital Stay Notes, Personal Care Plan and Needs Statement

  

 

Hospital Transfer/Hospital Stay Notes Template 

 

These are short handy notes with all the information about you that anyone transferring you to hospital or managing you in hospital can read quickly. The notes tell a lot about your illness and your needs in a short snappy way for a busy hospital/other care setting environment. They are a useful alternative to the more detailed Care Plan which you could also include in your medical file or bring with you when you are going into hospital or another care setting.

Please see Hospital Transfer/Hospital Stay Notes Template here 

 

 

Personal Care Plan Sample

 

• would outline the severe ME patient’s illness and needs
• would outline the difficulties the patient has being in the unfamiliar environment of a hospital where there are many potential ‘harms’ to their health,
• would warn medics of other difficulties that might come about,
• would be an individual file on each patient put together by the patient and/or carer, together with information added from the patient’s primary carer and the specialist consultant(s) the patient attends,
• would include strict protocols to make arrival, admission, stay, and discharge, as well as medical assessments and procedures as easy as possible on the person with Severe ME,
• would go some way to help prevent relapse, paralysis, and other horrific symptoms, 
• would be different and personal to the individual patient's needs.

 

It is important to remember that a person with ME may carry their care plan file for any hospital admission and to the ED. Please check with the patient or carer.

Please see sample Personal Care Plan

Needs Statement Sample

Based on the HSE’s 'National Guidelines on Accessible Health & Social Care Services' (2014), the patient's 'My Needs Statement' can be used during a hospital admission etc when the patient is faced with the problem of how to explain ME & their individual needs to healthcare staff.

 Please see sample My Needs Statement

Useful Information Sheets 

• Ruling Out Other Conditions  (by ME International US)

Part of the process of confirming an ME diagnosis is to rule out other diseases. Too often patients discover after getting an ME label that a treatable illness had been overlooked. ME International's Conditions to Rule Out handout has useful patient information regarding how to help diagnose ME.  

• Testing and Treating information based on the Primer for busy doctors by North Carolina/Ohio ME & FM Support Group: Information Sheet Re Testing & Treating ME

Films about various aspects of ME including Severe ME, from Dialogues for ME, by Natalie Bolton & Josh Biggs, produced with a Wellcome Public Engagement Fund Award 

• Introduction to ME entitled Introduction to ME 

• Reduced function and the range of symptoms in ME: Patients Accounts - Symptoms  

• Exertional intolerance, the cardinal feature of ME: Understanding Post Exertional Malaise - a Hallmark Feature 

• A Brief Guide to Post Exertional Intolerance