Friday, 12 May 2023

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.

  • 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.  





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 

 




 



Managing and Treating ME

There is no cure for ME. In the Dialogues of a Forgotten Illness series on severe and very severe ME, Dr Nigel Speight discusses the need for a particular type of GP to manage people with ME - people with severe ME need GPs/doctors who can make house calls and coordinate among the various specialists involved with a complex patient. They also require a GP who understands the emotional experience of severely ill patients and how isolation, financial difficulty, neglect, and medical trauma such as abuse and gaslighting take their toll. 

There are many strategies healthcare professionals can use to support people with ME including: 
    • exercise intolerance avoidance management strategies such as pacing* and resting* 
    • symptom management to reduce symptom burden & increase QOL 
    • providing validation and reassurance that the patient's symptoms are due to a biological issue across various body systems 
    • help accessing supports such as Community Services & Welfare payments 


* NB: Re Pacing and Resting - People with Severe ME spend days struggling to cope from moment to moment in the most awful suffering. For those, and others with ME, pacing and indeed resting may not be an option to manage ME because the body can be in such turmoil that patients can't pace 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 above listed strategies are all covered within and across the information below in the various resources with information for managing and treating ME.




Resources for Managing & Treating ME
  • International Consensus Primer  
  • Information about Managing Myalgic Encephalomyelitis (ME) compiled by ME Advocates Ireland (MEAI)
  • Questionnaires for Continual Assessment and Tracking Symptoms, Severities & Impact - De Paul Questionnaires
  • Film about ME 
  • Patient Documents To Consider such as Personal Care Plan and Needs Statement
  • Creating an ME Accessible Healthcare Setting - Information Sheet by MEAI 
  • Information Sheets based on the Primer by ME International
  • More Notes on Testing compiled by ME Advocates Ireland (MEAI)







International Consensus Primer 
The pathophysiology of ME and laboratory findings must be reflected in all treatment/management programs. Adverse reaction to exertion accompanied by a prolonged recovery period must be respected and accommodated. 
All health-care personnel can become  sufficiently knowledgeable to manage ME following diagnosis by adopting and using the Primer for Medical Practitioners, ......see pages 13-19






Information about Managing Myalgic Encephalomyelitis (ME) compiled by ME Advocates Ireland (MEAI), which includes notes on Energy and Step by Step Symptom Management as well as notes on Early Diagnosis, Pacing (activity management) & Resting, Energy Envelope Technique, Medical Treatments, other treatments and management strategies e.g., managing sleep & diet; using a Heart Rate Monitor, and managing relapses and adrenaline surges in Myalgic Encephalomyelitis (ME).

We briefly refer to three important management techniques here: - 


Early Diagnosis 
 
Early Diagnosis is essential in ME.
The aim should be to provide people with an early and accurate diagnosis which will allow patients to receive appropriate treatments in a timely fashion, which may lessen the severity and impact.
Early diagnosis should enable a comprehensive plan of management to be agreed between the patient and doctor which should cover symptom management and symptom relief, which may lessen the severity and impact.
Developing an agreed plan of appropriate management should prevent inappropriate or harmful approaches taking place – e.g. attempting to ‘work through illness’ – and should therefore reduce the risk of a more prolonged and severe illness occurring.

 

NB: The 6-month waiting period before diagnosis is not required as per the ICC 2011.

 




Energy Management   
 

Energy management strategies of pacing and resting for those with mild, and moderate ME, and some people within the severe category, are crucial self-management strategies for ME as they minimise harm caused when patients undertake activity beyond their capacity and trigger a post-exertional response i.e., PENE, also referred to as PEM by others. 

The aim of pacing is to minimise or avoid triggering PENE, by keeping physical and cognitive activity within the available energy reserves, often referred to as the energy envelope.  
 


Step by Step Symptom Management  
 
ME patients typically present with a multitude of symptoms indicating a multi-system disorder. And individuals have different clusters of symptoms. This can be overwhelming for both the clinician and patient to work through. However, by taking a structured, step by step approach, health professionals can work with the patient to rank their symptoms and address them one at a time, starting with the most debilitating/disruptive. This can be done using the De Paul Symptom and Severity questionnaire or the doctor and patient can discuss the patients most debilitating and severe symptoms.
Although there are no universally approved treatments for the underlying cause of ME, medication may help alleviate some symptoms (e.g. pain, orthostatic intolerance, insomnia). 
ME patients are typically sensitive to pharmaceuticals, so it is advisable to start on low doses and titrate up slowly. 
More on these three important management strategies and all other strategies previously listed, via link below: 

 









Questionnaires for Continual Assessment and Tracking Symptoms, Severities & Impact

Doctors, as well as patients could use several questionnaires, which are based on research, to track symptoms and severities following diagnosis. These include the same questionnaires suggested for diagnosis, i.e., the De Paul Symptom & Severity, PEM and Paedriatric questionnaires (parent and child) and the Bells Disability Scale. 

  • Paediatric Questionnaires: 
Parent Form DSQ-Ped here
Child Form DSQ-Ped here

  • Bells Disability & Severity Scale 








Film about ME from Dialogues for ME, by Natalie Bolton & Josh Biggs, produced with a Wellcome Public Engagement Fund Award 

    • Activity and Energy Management - Pacing








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 that a doctor or other healthcare staff sees will have their own individual needs.
Included below are two sample documents which may 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 important in-patient information and discharge planning notes, i.e., Personal Care Plan and Needs Statement
  

 


Personal Care Plan
 
    • 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 consultants 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 the ME patient may carry their care plan file for any hospital admission including ED, or their plan may be in their medical file held by a doctor. Please check with the patient, or carer, or doctor.

 

Please see sample Personal Care Plan




Needs Statement

Based on the HSE’s 'National Guidelines on Accessible Health & Social Care Services' (2014), the patient's 'Needs Statement' can be used during a hospital admission or other care setting, 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










Creating an ME-Accessible Healthcare Setting  - Information Sheet by ME Advocates Ireland (MEAI) 

People with Myalgic Encephalomyelitis (ME) have a limited amount of energy and suffer from a post exertional response/crisis, i.e., PENE. Helping them conserve their energy and providing an ME- friendly environment will reduce the impact of attending essential medical appointments. See MEAI's useful information sheet with tips to aid a person with ME attend their appointment: here

 





Information Sheets 
    •  Emergency Room Information Sheet  here
    •  Anaesthesia Info for ME Patients here






More Notes on Testing compiled by ME Advocates Ireland (MEAI)

Further to the tests to be done, as suggested in the ICP, there is further information re testing in post by ME Advocates Ireland (MEAI) here






ME Specialists in Ireland

There are two private consultants in Ireland with an interest in ME who treat people with the illness, one is Dublin based and the other is in Co Roscommon. The Roscommon specialist may see people in his catchment area via HSE. Please contact ME Advocates Ireland (MEAI) for further information at info@meadvocatesireland.com










Severe Myalgic Encephalomyelitis (ME)

Clinical Features of Severe and Very Severe ME

Cardinal Feature

  • Exertion Intolerance resulting in Post Exertion Crisis called Post Exertional Neuro Immune Exhaustion (PENE), in the International Consensus Criteria and Primer, also referred to as PEM by others.

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 repeatedly reported in feedback.

  • Minimal to zero energy levels, resulting in the patient being mostly housebound or permanently confined to bed;
  • Paralysis;
  • Severe generalised continuous agonising pain;
  • Severe continuous headache;
  • Cardiac Problems;
  • 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;
  • No possible movement;
  • Malnutrition & problems with eating and drinking - e.g., swallowing issues - these can be due to either general weakness or actual dysphagia, and this 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 by others;
  • 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 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.

  • 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 mild to profoundly severe, so someone with mild or moderate ME may suffer from severely disabling symptoms.

  • 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 (based on notes by Dr Nigel Speight from 2020 paper entitled Severe ME in Children).



The Value of Home Visiting by the Doctor
People with more severe ME may find it very difficult to attend at the doctor's surgery or  hospital and will require ME-friendly healthcare practice. The magnitude of the challenge posed by a case of severe ME can produce an avoidance reaction on the part of some doctors. Many patients are virtually abandoned by both their family doctors and local and regional specialists. The practice of doing home visits seems to have gone into decline, both with general practitioners and hospital consultants. This is regrettable, especially in the case of severe ME. The cases mentioned in the paper by Dr Nigel Speight entitled Severe ME in Children all seemed to benefit from the ongoing home visits they received, both for moral support and symptomatic treatment. Sadly, many cases of severe ME lie at home without having seen a doctor for many years.




Create an ME Accessible Care Setting
Tips on how to support the severe ME patient when they attend for a medical appointment in a doctor's surgery or hospital setting: Create an ME Accessible Care Setting





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.



Gurney Transportation for patients who must remain lying down
Some patient's may only be able to be brought to hospital by ambulance because they need to be lying down at all times. The level of disability that requires gurney transportation for someone with Severe or Very Severe ME and/or structural issues usually comes with extreme suffering. The inability to sit up accompanies myriad symptoms ranging from brain inflammation to unrelenting pain throughout the body.
Orthostatic Intolerance: Some people with ME might have symptoms of orthostatic intolerance that are triggered or made worse by standing and/or sitting upright. Those with severe ME can suffer from this symptom and find sitting up or standing next to impossible. These symptoms can include dizziness and light headedness; changes in vision; weakness;  patient feeling like their heart is beatin too fast or too hard or skipping a beat.
Finding non-urgent transportation on a gurney (or stretcher) for a bed-bound person is not just challenging, sometimes it seems impossible.  
Gurney Guide by Galen Warden here

 



Malnutrition in Severe ME
Some of the most severely affected ME patients experience serious difficulties in maintaining adequate nutrition and hydration and some will require feeding enterally.
Why: The reasons can be an inability to swallow, severe gastrointestinal problems tolerating food or the patient being too debilitated to eat and drink, and to digest food. 

How to Support: Some patients with very severe ME will require tube feeding, either enterally or parenterally. 
Case Report: Life-Threatening Malnutrition in Very Severe ME by Helen Baxter, Nigel Speight and William Weir.

How to Support Patients with Malnutrition Problems: please see 12 minute video from the 25% ME Group entitled Preventing Avoidable Malnutrition in Severe ME.
 




Paralysis in ME
Paralysis is a terrifying feature that has often been reported in feedback from people with Severe ME.
Paralysis in ME means the patient cannot feel and cannot move and has no choice. It can take over part of the body or the whole body, i.e., partial, or full body paralysis.  

The Canadian Consensus Document on Myalgic Encephalomyelitis 2003 defines a crash as: 

 

“a temporary period of immobilizing physical and/or cognitive exhaustion”.

 


Important notes in the event that a patient enters a paralysis/crash phase 
 
The patient may become paralysed and unable to inform the HCP of the extent of their pain or other symptoms.
Paralysis may be limited to limbs only or may affect the whole body.

 

Sample Vital signs of an ME patient in paralysis/crash/PENE state: 
 
BP can vary hugely, for example, in the 130-160/85-95
• Going as high as 224/164 or
• In a crash state BP will drop to <120/<80 (e.g. 96/64) which results in the patient feeling drained & unwell.
• Temp basal temp usually around 35.5, can increase in warmer weather
When exhausted or experiencing post exertional response (PENE/PEM) temperature can drop as low as 33.8.
• Legs/arms will be very cold & head very hot with raised temp, in this case cover limbs and cool head, fan/cool cloth
OR
• Whole body temp lowers & all body requires warming, especially extremities.
HR Resting HR varies 40-50 depending on current baseline.
• Heart rate varies hugely depending on current baseline and ‘activity’
• ‘Activity’/effort can increase heart rate to 130+, going to bathroom, eating etc
• During a crash or when exhausted HR can drop to 30, this is part of the ME ‘dauer’ state which will resolve without intervention. 
 
 
Do Not
• Attempt to elicit a “pain response” – this will hurt the patient
• Raise one’s voice to “get through” to the patient – noise hurts
• Tell the patient to open their eyes – the patient may not be able to do so for several hours

 

Do
• Take hold of patient's fingers and frame questions to require a “Yes” answer
• Do this quietly and slowly
• Give the patient time:
to hear
to process the words
to move a finger to indicate “yes” – this is an extreme effort
• Reassure the Patient that you know they are paralysed and you will check on them

 

 

More information re crash/paralysis management pages 6, 8 and 9 here 
(the above sample vital signs and advice is a portion of the full document)

 


Expert information about Paralysis from severe ME patient Linda Crowhurst and her husband, carer Greg Crowhurst here 

 

Research by Leonard Jason et al refers to paralysis in the paper entitled,  ''Assessment of Post-Exertional Malaise (PEM) in Patients with Myalgic Encephalomyelitis (ME): A Patient-Driven Survey'





Resources for Managing & Treating Severe ME



As well as the above information about diagnosis, testing, treating and management in ME, included below are additional items specific to severe ME.

  • Resources for Managing Severe ME 

  • Films about Severe ME 

  • Books about Severe ME: Carers Notes

  • 'Create a Severe ME Accessible Healthcare Setting' Information Sheet by ME Advocates Ireland (MEAI) 

 


Resources for Managing Severe ME 
    • Personal Care Plan which gives an overview of ME in a person with Severe ME, including treatments and essential care in healthcare settings: Considerations for Severe ME








    • Information from a UK charity that helps children and young people with ME and their families, Tymes Trust (currently there is no charity in Ireland that deals specifically with paediatric ME.




 Films About Severe ME
    • An introduction to the more severe end of the spectrum of ME from Dialogues for ME, by Natalie Bolton & Josh Biggs, produced with a Wellcome Public Engagement Fund Award : Severe & very Severe ME


    • 'Voices From the Shadows' -  About the trailer/30 min film and 1hr film here, available on Vimeo, free using the code "VOICES"

    • Preventing Avoidable Malnutrition in Severe ME by Helen Baxter here


 Books about Severe ME: Carers Notes
THe ME communities worldwide are grateful for the work of husband and carer of severe ME patient Linda Crowhurst, Greg Crowhurst. Books by Greg and information provided by Linda focus on caring for Severe ME patients and provide a close focus and concise overview of severe ME.  
Greg's books include:
      • Severe ME: Notes for Carers here
      • Severe ME: More Notes for Carers here 
      • Issues of Noise, Light, Touch, Chemical & Movement Sensitivity in Severe/Very Severe ME that Carers Need to be Aware Of Free PDF 
      • Very Severe ME - Underlying Principles of Care Free PDF 
      • The Care Needs of People with Severe ME Free PDF 






'Create a Severe ME Accessible Healthcare Setting' Information Sheet by MEAI 

People with Myalgic Encephalomyelitis (ME) have a limited amount of energy and suffer from a post exertional response, i.e., PENE. Helping them conserve their energy and providing an ME- friendly environment will reduce the impact of attending essential medical appointments. 
 
People with very severe/Profound ME are most often too ill to attend 
doctors surgeries, hospitals, outpatient departments. These healthcare settings are not generally organised to accommodate multiple chemical sensitivities, hyper sensitivities such as noise sensitivity, light sensitivity, and tthe need for bed rest, especially in the case of a patient experiencing a crash or paralysis. Being exposed to the major physical trauma of dressing to go out, of travel and then being exposed to so many healthcare staff and other people, and so many sources of noise, bright lights and chemicals will be too much for the person with Very Severe/Profound ME.
 
 
People with Very Severe/Profound ME are physically profoundly ill and often cannot take the risk of trying to access support, from a consultant or doctor unknown to them, when any interaction and effort may lead to profound consequences of health deterioration, temporary or permanent, or very long term.
 
 
People with Very Severe/Profound ME cannot endure two-way communication at times, and even if possible will be limited and potentially harmful, leading to severe post-exertional crisis. It is essential that anyone trying to communicate with a person with very severe/profound ME understands the way the person communicates, what they can tolerate and how little energy they have and not ignore or forget the persons reality.
 

People with Very Severe/Profound ME cannot access anything required to negotiate with doctors surgeries and hospital settings, treatments, follow up appointments, Physiotherapy, OT, medical assessments, surgeries, etc. 

 


People with Very Severe/Profound ME do not have enough energy to explain their chronic condition and symptoms or express their concerns about their health, even if the person can temporarily access a doctor or healthcare provider. The effort of speaking, if the person is able to speak, is too much and can lead to a post exertional crisis which goes beyond PENE as we know it among those less severe.


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.

 

People with Severe/Profound ME cannot concentrate and deal with complex interactions with other people because of extreme pain and physical discomfort, the interaction itself will most likely increase the pain even more. 

 

People with Severe/Profound ME cannot function during a period of paralysis. Paralysis makes any interaction impossible, the patient cannot function when they are completely paralysed either mentally or physically; they cannot even speak or open their eyes. Transient paralysis can hit any muscles at any moment.

 

See MEAI info sheet for doctors and healthcare staff which includes tips to aid a person with Severe ME attend their appointment here.



Notes on Crash/Paralysis 
 
Please see notes on Crash/Paralysis pages 6, 8 and 9 here. 


 

See Greg and Linda Crowhurst's guideline entitled 55 Reasons why it is difficult to treat a person with Very Severe ME.


 







Further Considerations for Severe ME


Notes about Prescription Medicines 
While there are no treatments approved specifically for ME, a number of different pharmacological and non-pharmacological treatments can help reduce the severity of symptoms. 
Different drugs have different effects on symptoms and they also differ in their side effects from one patient to another. If a drug is ineffective or cannot be tolerated, it may be worth systematically trying others. 
People with ME often have a limited tolerance to drugs, so starting lower doses than usual may be needed, then slowly increasing if appropriate. Finding the 'sweet spot' may involve trial and error. Particular medications might not suit all. It's a case of trial and error with treatments. Some patients react badly to medicine, be extremely cautious.
NB: A person with Very Severe ME is likely to be extremely drug sensitive; they require an awareness of anaesthetic sensitivity from the medical practitioner or other healthcare provider. 



Some examples of commonly used medications for ME
Commonly used medications in management of symptoms in ME include low-dose naltrexone (LDN), duloxetine, gabapentin, cyclimorph, oxynorm or  pregabalin for pain; fludrocortisone, midodrine, olanzapine, pharma GABA, amitriptyline, pyridostigmine, low dose beta-blockers and/or intravenous hydration for orthostatic intolerance; low dose rivotril (Klonopin) (2.5 mg) to get a handle on overactive central nervous system’s adrenaline rushes, mirtazepine at night to help with sleep along, occasional use of modafinil, methylphenidate, or dexedrine for cognitive or fatigue issues, molipaxinm melatonin, prednisone, adderall, corlanor, valium; in addition to these basic treatments, some disease experts are evaluating the selective use of antivirals and immune modulators in ME and have seen evidence of efficacy in some patients; some extremely ill people with ME have had success with low dose abilify (antipsychotic) - used off label to treat neuroinflammation but its use comes with serious warnings; iv fluids and iv zofran for dehydration, iv saline for dysautonomia 
Note: Despite the availability of medicines for treating various symptoms in ME some people cannot tolerate medicines even at low doses. 
Note: For people with Severe/Profound ME prescribing new medications is a very high risk strategy without knowing if the person will be able to tolerate new medications and without understanding possible reactions. Prescribing new medications should never be undertaken without due consideration of risk and possible side effects. People with very severe/profound ME are desperate for support and so open to mistreatment by who don't understand the risks. Patients are at massive risk of deterioration and harm being inadvertently or carelessly done to them; for this reason a proper Risk Assessment should always be performed.




Notes about Pacing and Resting 
People with Severe ME spend days struggling to cope from moment to moment in the most awful suffering. For those, and others with ME, pacing or resting may not be an option to manage ME because the body can be in such turmoil that patients can't pace 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 or resting 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.
Recognition of other interventions such as IV fluids, peg feeding, etc may be necessary in some cases.






Notes about making a referral

Referral to other services may also be required for additional assessment, symptom control or further support and advice. This could include referral to a specialist consultant, physiotherapist, occupational therapist, endocrinologist or rheumatologist. Unfortunately most won't be familiar with ME.

There are two private consultants in Ireland with an interest in ME who treat people with the illness, one is Dublin based and the other is in Co Roscommon. Please contact ME Advocates Ireland (MEAI) for further information at info@meadvocatesireland.com




Notes about inappropriate referrals and inappropriate treatments !!! 


IMPORTANT WARNINGS


GRADED EXERCISE THERAPY (GET) or any programme that uses fixed incremental increases in physical activity or exercise CAN CAUSE HARM

Graded Exercise Therapy is NOT the same as Pacing. GET requires the patient to gradually increase activity over a period, potentially pushing them to repeatedly trigger the PENE response described further above. GET has been reported by many patients, scientists & some orgs to be unhelpful and harmful and has left some patients significantly worse than they were before they started. 

Given that PENE is the hallmark symptom of ME, GET is not an appropriate treatment for people with ME.
 



Graded Exercise Therapy (GET) warnings explaining the dangers of GET from the 25% ME Group entitled '25% ME Group stance on Exercise' here

 

Understanding the Dangers of Graded Exercise Therapy Video from Dialogues for ME here 

 

More from Dialogues for ME about the dangers of GET here 

 




Further Warnings


Do Not Offer the Lightning Process, or therapies based on it to people with ME.





Talking therapies such as CBT can at best provide psychological support, and at worst contribute to harm by using precious energy, or misinforming patients. CBT does not cure ME and must not assume people have abnormal illness beliefs and behaviours as an underlying cause of their ME.




Current default referrals to psychiatry where ME has been diagnosed or is suspected can be inappropriate and harmful because the referral can delay diagnosis and management for the person with ME which not only adds to a feeling of being dismissed and denied care but can often lead to deterioration and worsening severity.






Notes about inappropriate labels 

People with ME are frequently misdiagnosed or given various psychiatric/psychological labels. Please do not use inappropriate labels for ME which include:
  • Functional Neurological Disorder (FND)
  • Fabricated or Induced Illness (FII)
  • Medically Unexplained Symptoms (MUS)
  • Perplexing Physical Symptoms 
  • Pervasive Refusal Syndrome
  • Deconditioning
  • Psychiatric
  • Psychological
  • Bodily Stress Disorder
  • In the case of malnutrition as a result of complexities in severe ME, do not refer to 'eating disorder'
  • In the case of young people with ME where there is reduced or no attendance, do not refer to 'School Avoidance'



Supporting Caregivers 

Informal caregivers often provide significant levels of support. Caregivers need to be supported and linked with appropriate services. ME Advocates Ireland (MEAI) has information dedicated to carers in their notes entitled Carers in the ME Community - A one stop post with information for & about Carers of People with ME

Carer Greg Crowhurst's guide about people with very severe or profound ME for medics Very Severe ME : Underlying Principles of Care



"All the time that ME is treated only as a fatigue illness the health needs of the very severely affected, who feel unimaginably ill in every moment, who are not generally part of research and whose experience is poorly if at all understood, are massively at risk."
~ Greg Crowhurst ~








ME in Paediatrics


About ME in children and adolescents


ME is a complex disease that affects children & adolescents as well as adults. While many paediatricians and other health-care providers are aware of ME, they often lack essential knowledge that is necessary for diagnosis and treatment. Many young patients experience symptoms for years before receiving a diagnosis.
 
While some young people with ME can attend school, on a full or part-time basis, many others are wheelchair dependent, housebound, or bedbound. Prevalence estimates for paediatric ME vary from 0.1 to 0.5%.
 
Because there is no diagnostic test for ME, diagnosis is purely clinical, based on the history and the exclusion of other illnesses by physical examination & medical testing.
 
Co-existing medical conditions including orthostatic intolerance (OI) are common. Successful management is based on determining the optimum balance of rest and activity to help prevent post-exertional symptom worsening.
 
 

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.
 
Notes: Fluctuation and severity hierarchy of numerous prominent symptoms tend to vary more rapidly and dramatically than in adults. - ICC 2011

4. Sleep problems in young children may be mistaken for as a lack of their usual energy. In adolescents with ME, sleep problems may be hard to detect, as sleep cycles change during puberty. The demands of school, homework, after-school activities, and social activities also affect sleep.

Common sleep complaints in children and adolescents with ME include:
·       Difficulty falling or staying asleep
·       Daytime sleepiness
·       Intense and vivid dreaming
 
Unlike adults with ME, children, and adolescents with ME may not usually have muscle and joint pain.
However, headaches and stomach pain may be more common in this age group.
 
Note: The difficulties with younger children are that they may not be able to describe the pain well.
 





 
Diagnosis in children & adolescents

As in adults, symptoms of Myalgic Encephalomyelitis (ME) in children and adolescents may appear like many other illnesses, and there is no single diagnostic test to confirm ME, making ME difficult to diagnose.

The illness can be unpredictable. Symptoms may come and go, or there may be changes in how bad they are over time.
 
Children and adolescents should be seen by doctors and get support as soon as they become ill. In other words, a child with some or all of the typical symptoms of paediatric ME should not have to wait for months to see a doctor. Early diagnosis is essential to prevent a worsening of ME. There is no requirement that the individual have symptoms for a specified period of time for diagnosis, as per the International Consensus Criteria.

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.

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

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 program.

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.
 
Diagnostic assessment of possible ME in children is very similar to that in adults. The child’s/adolescent’s doctor may undertake the following:
 
·       Ask about child’s/adolescent’s and family’s medical history, including a review of any medications and recent illnesses

·       Do a thorough physical examination

·       Order blood, urine, or other tests

·       The doctor may ask many questions and depending on the age of the child/adolescent, the questions might be asked of the patient, and/or parent/guardian.
 
Questions the doctor may ask might include:
How long has the child/adolescent been ill?
How did the illness start? Was it after an illness/ What was that illness?
What is the child /adolescent able to do now? How does it compare to what the child /adolescent was able to do before?
Does the child feel any improvements after sleeping or resting?
What makes the child/adolescent feel worse? What helps the child/adolescent feel better?
What symptoms keep the child/adolescent from doing what he/she wants to do?
What symptoms prevent the child/adolescent from being able to attend school/out of school activities/carrying out hobbies/socialising? 
What happens when the child/adolescent tries to do activities that they used to do without problems?
Does the child/adolescent seem to have trouble remembering or focusing on tasks?
 
Primary Carers/Medics might refer children/adolescent with ME to see a specialist, like a neurologist, rheumatologist, or a sleep specialist, consultant paediatrician to check for other conditions and to help manage the complexities of their symptoms of ME.

 

 

 
 
Resources for Managing & Treating Paediatric ME

  • International Consensus Primer  
  • Paediatric questionnaires
  • Frontiers A Primer
  • Films about ME in children & adolescents
  • Importance of Early Diagnosis  
  • Learn About the Patient's Perspective

  • Notes about making a referral

  • Feedback from a parent of a child with ME in Ireland




International Consensus Primer
The Primer (2012) is a guideline written about and for Myalgic Encephalomyelitis (ME) in Adults & Children. 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 ......See paediatric considerations pages 9 & 19 of the International Consensus Primer for Adults and Children 

 







Paediatric questionnaires (De Paul)
Along with the recommendations as per the International Consensus Primer an ME diagnosis can be confirmed using the DSQ- PED questionnaires from De Paul consists of a parent form and a child form. Parents can fill in a symptom questionnaire to present to the child’s medic. Medics can collect data from both children and their parents to obtain a thorough understanding of the child's ME. Children under the age of 12, or those with reading or comprehension difficulties, complete this questionnaire with the assistance of a parent or guardian. These forms are based on research.

DSQ-Ped Parent Form 

DSQ-Ped Child Form 








Frontiers Primer for Diagnosis and Management of ME in Young People

The Frontiers Paediatric Primer (2017) for Diagnosis and Management in Young People has advice re schools, education plans as well as general paediatric advice re management. The writers are all very experienced ME Paediatricians worldwide. This primer has been written to provide the information necessary to understand, diagnose, and manage the symptoms of ME in children and adolescents: Frontiers Paediatric Primer 


 





Films about ME in Children & Adolescents

An overview of Paediatric ME across a few videos from an expert in the UK, Dr Nigel Speight Paediatric Consultant, who talks about ME in children, what can be done to help them & their families & the issues around management:
 
  • ME & Children Discussion by Dr Speight Part 1
 
  • ME & Children Discussion by Dr Speight Part 2
 







Importance of Early Diagnosis and Management in Young People
Unlike with adults, children have a much better chance of recovery if treated correctly, most recover within 3 years. However, children who receive an incorrect or delayed diagnosis continue, on the advice of their doctors with sports activities and to attend school full time, resulting in worsening severity and prolonged illness.





Notes about making a referral
Referral to other services may also be required for additional assessment, symptom control or further support and advice. This could include referral to a specialist consultant, physiotherapist, occupational therapist, endocrinologist or rheumatologist. Unfortunately most won't be familiar with ME.
Currently there is no consultant in Paediatric ME or a Paediatric Consultant in Ireland who is fully knowledgeable about ME. We understand that Dr Nigel Speight tends to see patients in Ireland sometimes. Please get in touch via email if you need more information about referrals to consultants/specialists to info@meadvocatesireland.com












Further Resources for Doctors and other Healthcare Staff
Some of the information below is available via HSE, Citizens Information, etc, the information ME Advocates Ireland (MEAI) provide is specific to those in the ME community with advice and tips included based on feedback from the ME community in Ireland.

  • Welfare Payments  - Information re accessing Welfare Payments for those with ME: here

  • Community Services - Information re accessing Public & Community Services for those with ME: here

  • Carers - Information for Carers: here
  • Essential Reading for staff unfamiliar with ME: 
    • Myalgic Encephalomyelitis – Adult and Paediatric: International Consensus Primer  Pages 3-12

  • Research & Films about ME ME Research Extracts and Films About ME: here






Learn About the ME Patient's Perspective

  • The Health-Related Quality of Life for Patients with Myalgic Encephalomyelitis 2015 Danish Research Article 





'CFS' and 'ME/CFS' Label Issues

ME is sometimes referred to by the problematic term 'chronic fatigue syndrome' or CFS for short, or the acronym ME/CFS. The inappropriate, misapplied and stigmatising focus of the label chronic fatigue syndrome lies in the over emphasis of fatigue.

The World Health Organisation does not use combined terms ‘Myalgic Encephalomyelitis/chronic fatigue syndrome’ in ICD-10 or ICD-11, for which the terms are retained as separate terms.

The double-barrelled acronym ME/CFS is used by others, such as researchers and other government health agencies internationally, to bridge beyond terminological and definitional failures, and the original disease name Myalgic Encephalomyelitis (ME). Many professionals and people in the ME community are more comfortable with the term ME because it is the name of their illness as per the ME-ICC case definition.


'CFS/ME' is a term used by BPS cabal in the UK to put emphasis on the inappropriate concept that ME is only about fatigue which as they see it can be treated, and as a psychologic disorder, in their attempts to frame ME patients as malingering.

ME is a biological illness, not a psychologic disorder. People with ME are neither malingering nor seeking secondary gain. (BPS papers were ranked 87% very low quality, 13% low quality in UK NICE assessment). Being explicit that ME is biophysical, seriously debillitating, multisystemic and not due to malingering, the CDC has removed dangerously irrelevant biopsychosocial guidance. This is further reflected in the US Institute of Medicine, now called National Academy of Medicine (NAM), review of thousands of scientific papers and articles.

The Holmes (US),  Oxford (UK), Fukuda (US) and NICE 2007 (UK) criteria, where there lies over emphasis on fatigue and absence of important criteria re ME, should not be used to diagnose ME, and research studies on ME should not include those criteria nor people diagnosed with them. 


ME Advocates Ireland (MEAI advocate for the adoption and use of the International Consensus Criteria for research and diagnosis, and the Primer for diagnosis and management of ME.



More on CFS and ME/CFS
The International Consensus Criteria and Primer for Myalgic Encephalomyelitis in Adults and Paediatrics is very clear on the topic of names.
Problem: The label ‘chronic fatigue syndrome’ (CFS), coined in the 1980s, has persisted due to lack of knowledge of its etiologic agents and pathophysiology. Misperceptions have arisen because the name ‘CFS’ and its hybrids ME/CFS, CFS/ME and CFS/CF have been used for widely diverse conditions. Patient sets can include those who are seriously ill with ME, many bedridden and unable to care for themselves, to those who have general fatigue or, under the Reeves criteria, patients are not required to have any physical symptoms. There is a poignant need to untangle the web of confusion caused by mixing diverse and often overly inclusive patient populations in one heterogeneous, multi-rubric pot called ‘chronic fatigue syndrome’. We believe this is the foremost cause of diluted and inconsistent research findings, which hinders progress, fosters scepticism, and wastes limited research monies.
Solution: The rationale for the development of the ICC was to utilize current research knowledge to identify objective, measurable and reproducible abnormalities that directly reflect the interactive, regulatory components of the underlying pathophysiology of ME. Specifically, the ICC select patients who exhibit explicit multi-systemic neuropathology, and have a pathological low threshold of physical and mental fatigability in response to exertion. Cardiopulmonary exercise test- retest studies have confirmed many post-exertional abnormalities. Criterial symptoms are compulsory and identify patients who have greater physical, cognitive and functional impairments. The ICC advance the successful strategy of the Canadian Consensus Criteria (CCC) of grouping coordinated patterns of symptom clusters that identify areas of pathology. The criteria are designed for both clinical and research settings.








What Mental Health Practitioners and Psychiatrists Need To Know

Mental Health practitioners and psychiatrists need to be familiar with the guidelines as per the International Consensus Primer and would learn a lot about ME diagnosis and management from all of the above information provided for doctors and other healthcare staff; they need to become familiar with and knowledgeable about ME for times when patients are referred to them especially  when a doctor did not pick up on Myalgic Encephalomyelitis. 
Unfortunately referring people with ME to psychiatry has happened too often because of previous inappropriate guidelines. The delay in diagnosis and appropriate management has significantly been attributed to worsening of severity and disability.


ME has a post exertional response, i.e., cardinal feature PENE, also referred to as PEM by others. In depression and anxiety, most feel better after exercise. 

It must not be assumed that people have abnormal illness beliefs and behaviours as an underlying cause of their ME.

ME patients have a wish list of activities they would like to be able to do  but cannot because of post or immediate exertion response, i.e., PENE

Immune manifestations including tender lymph nodes, sore throat, chemical and food sensitivities common in ME. Not found in Depression or Anxiety.

Loss of body thermostatic stability, intolerance to extremes of temperature common in ME. Not reported in Depression or Anxiety

Orthostatic intolerance, tachycardia and other autonomic dysfunctions are common in ME. Not found in Depression. 



Please note that rates of mental health issues in ME are similar to rates in other chronic medical conditions. As in the general population depression and anxiety are the most common symptoms.

All mental health practitioners must also have a solid understanding of the principles of psychological support for ME and understand that ME is a biological illness characterised only by pathophysiological features. Mental health practitioners have to understand the physiological implications and the sheer physical vulnerability of the people they aim to support and aid. 



Types of 'Depression' in ME
1. Reactive grief due to loss of health, social connections, family support, financial capability, career and uncertainty regarding all of these. 
2. Biological change in mood/cognition as part of the disorder of ME (similar to mood change in MS or Parkinson’s disease and as reported in epidemic ME). 
3. Comorbid depressive disorder. 
4. Mood change due to medication or food or withdrawal from either

 

 
Types of 'Anxiety' in ME
1. Anxiety about health e.g. prognosis, cause of symptoms or unpredictability of symptoms. 
2. Anxiety as a result of the impact of having ME. 
3. Anxiety about being denied disability payments is common. 
4. Biological anxiety as part of the physical disorder of ME. 
5. Anxiety in reaction to a drug.

 
Do Psychiatrists Need to Know How to Diagnose ME?


Yes, psychiatrists have an important role to play in the diagnosis, and other mental health practitioners should recognise their role too by:

• Validating diagnosis is therapeutic and does NOT reinforce 'illness behavior' any more than in any other chronic illness.
• Offering to meet with family members to explain the illness and help patient with support.  
 
• Ruling out psychiatric or psychological causes of symptoms; 
 
• Recognising other causes of symptoms;
• Detecting and treating secondary anxiety or depression caused by the patient having a chronic illness;
• Recognising when symptoms are not caused by mental illness or psychological factors;
• Referring for further investigation when no psychiatric cause is found or when a psychiatric diagnosis does not explain all symptoms;
• Making a positive diagnosis of ME where relevant;
• Giving crucial early advice re management as soon as ME is suspected. Advise patients to rest and pace as soon as the diagnosis is suspected, use International Consensus Primer guidelines and provide other information on how to do this.
 


Psychiatrists need to keep in mind that: 

    • ME is a physiological/biological/pathophysiological illness;
    • ME is not a psychological or mental health illness;
    • standard scoring systems for depression or anxiety or emotional symptoms are non-specific and may mislead clinicians into misdiagnosing ME as psychiatric if they fail to take a thorough medical history;
    • someone who suffers from depression can acquire ME;
    • those with suspected or confirmed ME can suffer from secondary depression as a result of isolation, grief and loss - loss of health, loss of friends, relationship and financial difficulties;
    • talking therapies such as CBT can at best provide psychological support, and at worst contribute to harm by using precious energy, or misinforming patients. CBT does not cure ME and must not assume people have abnormal illness beliefs and behaviours as an underlying cause of their ME.
    • prescribing certain medicines may be harmful to those with ME, whether they are depressed or not, low doses work better then titering/altering to find the 'sweet spot'; 
    • not all medicines suit everyone with ME, in fact some patients cannot tolerate some medicines, so it is important to prescribe alternative medicines when one does not suit;
    • some patients cannot tolerate any medicine so alternative treatments may be required;
    • Given that PENE is the hallmark symptom of ME exercise can be very harmful
Do not offer people with ME:
 - Any therapy based on physical activity or exercise as a cure for ME 
 - Generalised physical activity or exercise programme
 - Any programme that uses fixed incremental increases in physical activity or exercise, for example Graded Exercise Therapy (GET) 
 - Physical activity programmes that are based on deconditioning and exercise avoidance theories as perpetuating ME
NICE’s comprehensive review of all studies of Graded Exercise Therapy (GET) and CBT found them to be universally of very low or low quality. 


 

Learn More About ME
  • Information and Resources for the Diagnosis & Management of ME for doctors and other healthcare staff further above


Learn About the Patient's Perspective

  • The Health-Related Quality of Life for Patients with Myalgic Encephalomyelitis 2015 Danish Research Article 







"People become traumatised by debilitating ME 
and then become traumatized again by the reaction to them 
by people who don't understand"

~ Professor Leonard Jason, Director of Centre for Community Research ~





" We need to build a future where every surgery, hospital, agency, insurance provider and employer is enabled with accurate information that supports their patients and clients"
~ ME Advocates Ireland (MEAI) ~






Special thanks to 
The Hummingbirds Foundation for ME,
Leonard Jason et al at De Paul University, 
 Greg Crowhurst,
 Linda Crowhurst
 Dialogues For ME: Natalie Boulton & Josh Biggs;
 the 25% ME Group,
 ME International (US),
 Galen Warden,
 Dr Nigel Speight,
 Helen Baxter,
Dr Nina Muirhead,
 Wendy Boutillier,
 Sheffield ME Group,
 writers of the ICC & ICP - BM Carruthers et al;
 Frontiers Paediatric Primer by writers including Peter Rowe et al;
 Dr William Weir,
 Dr David Bell,
Michael falk Hvidberg et al,
 Voices from The Shadows Team,
 The Irish ME Community & ME communities elsewhere








PDF version of Myalgic Encephalomyelitis (ME) Diagnosis & Management for Doctors and Other Healthcare Providers by ME Advocates Ireland (MEAI) here





Written & Compiled for Doctors & Healthcare Staff by ME Advocates Ireland (MEAI) May 2023








 





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 in some instances. 
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