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Diagnosis Part 1
Introduction - what is Myalgic Encephalomyelitis (ME)
Myalgic Encephalomyelitis (ME) is a severely debilitating disease. It is a complex, chronic, multi-system physical disease which causes unusually severe disability and can affect people of any age or demographic, including children.
Myalgic Encephalomyelitis, a name that originated in the 1950s, is the most accurate and appropriate name
because it reflects the underlying multi-system pathophysiology of the disease.
We strongly recommend that
only the name ‘Myalgic Encephalomyelitis’ be used to identify patients meeting the International Consensus Criteria (ICC).
Why?
Because a distinctive disease
entity should have only one name. Patients diagnosed using broader or other criteria for CFS or its hybrids (Oxford, Reeves,
London, Fukuda, CCC, etc.) should be reassessed with the ICC. Those who fulfill the criteria have ME; those who do not fulfill the criteria would remain in the more encompassing CFS classification.
The purpose of diagnosis is to provide clarity.
The criterial symptoms, such as the distinctive abnormal responses to exertion can differentiate ME patients from
those who have other fatiguing conditions.
Not only is it common sense to extricate ME patients
from the assortment of conditions assembled under the CFS umbrella, it is compliant with the WHO classification rule
that a disease cannot be classified under more than one rubric.
The ICC are a refinement of patient stratification. As other identifiable patient sets are
identified and supported by research, they would then be removed from the broad CFS/CF category.
Facts about ME
- Myalgic Encephalomyelitis is classified as a neurological disease by the World Health Organisation (WHO) ICD G93.3 and SNOWMED-CT.
- It is characterized by immune, neurological and cardiac dysfunction with severe worsening of symptoms following any exertion. The disease most often manifests post-virally. Myalgic Encephalomyelitis has been associated with antecedent infection, often viral, in up to 80% of cases.
- The cardinal symptom is a pathological low threshold of fatigability that is characterized by an inability to produce sufficient energy on demand. There are measurable, objective, adverse responses to normal exertion, resulting in exhaustion, extreme weakness, exacerbation of symptoms, and a prolonged recovery period.
- It has been reported that patients with ME have impaired oxygen extraction on cardiopulmonary exercise testing in proportion to the severity of their symptoms, with both oxygen extraction and workload being decreased on the second day of 2-day cardiopulmonary exercise testing. This may in part explain the characteristic delay between exertion and post exertion response (PENE) onset in ME, and distinguishes patients from deconditioned and fatigued controls. However, we do not recommend routine exercise testing for patients with ME, given its propensity to generate PENE (also referred to as PEM) and to decrease functional capacity.
- Myalgic Encephalomyelitis (ME) can affect all aspects of life of the individual with ME, as well as their families and carers. It has the worst quality of life score than many other serious illnesses including MS, cancer, stroke, congestive heart failure and Rheumatoid Arthritis.
- Myalgic Encephalomyelitis (ME) is not 'functional' or 'psychiatric'; it is not anxiety or depression; it is not Medically Unexplained Symptoms, Functional Neurological Disorder, an eating disorder or any other psychiatric or psychological labels.
- Myalgic Encephalomyelitis (ME) is a distinct, recognisable disease entity that is not difficult to diagnose and can in fact be diagnosed relatively early in the course of the disease, providing that the doctor has some experience with ME. Diagnosis of ME is based on positive signs and symptoms and is therefore not a diagnosis of exclusion.
- Myalgic Encephalomyelitis (ME) is a serious, biophysical, multisystemic disease; it is not due to malingering. Individuals with ME are neither malingering nor seeking secondary gain. This is reflected in reviews of 1000s of biological scientific papers and articles.
More about Myalgic Encephalomyelitis here
Diagnosis
As with a wide variety of illnesses; lupus and multiple sclerosis (MS), for example, there is as yet no single test which can diagnose ME. Therefore, ME must instead be diagnosed by:
- taking a detailed medical history to rule out other possible causes of symptoms;
- noting the type and severity of symptoms and other characteristics of the illness;
- noting the type of onset of the symptoms; acute or sudden onset of symptoms is seen in ME though feedback tells us that some people experience gradual onset;
- using one of the best set of international guidelines, e.g., the International Consensus Criteria and International Consensus Primer;
- checking for the cardinal feature, the post exertional response to activity, whether physical, mental or emotional, i.e., Post Exertional Neuro-Immune Exhaustion (PENE).
It is estimated that a very high percentage of people with ME are undiagnosed, possibly up to 90%, and 29% of those who have been diagnosed waited 5 years or more to receive an accurate diagnosis. This may be due to:
- the lack of a reliable diagnostic biomarker,
- heterogeneity in clinical presentation across patients that may mimic the presentation of other diseases, and
- suboptimal clinician awareness of the condition.
ME benefits from diagnostic criteria that are clear and straightforward. Misdiagnosis of other physical diseases or a mental health condition as ME must be avoided. Exertion intolerance characterised by Post Exertional Neuroimmune Exhaustion (PENE) is a cardinal symptom.
Abnormalities in ME are observable in controlled research contexts, for example, with the two day cardio-pulmonary exercise tests and in metabolic and other findings. (a risky test for diagnosis for many with ME, especially those with Severe ME)
ME can be diagnosed within a few consultations, if the doctor has some experience with ME. An accurate diagnosis or confirmation of a suspected diagnosis can be made by a doctor using diagnostic criteria specifically written for and about ME, such as the International Consensus Criteria and Primer.
ME isn’t difficult to diagnose, unfortunately we have a healthcare system in Ireland which doesn’t know about ME and healthcare professionals who are not taught about ME, so it can take a long time to get a diagnosis.
Getting an accurate ME diagnosis requires proper understanding of ME and is based on a detailed clinical history, physical examination, and some laboratory investigations.
A common story is that once a diagnosis happens here, there are no HSE guidelines/policies for management and treatment of ME which means a GP/consultant may give you a diagnosis, or confirm a suspected diagnosis, however, they will more than likely not have the information to follow up and advise you about management and treatments.
There is practical advice available about management and treatments within the expert patient community, as well as from patient organisations/charities and advocacy support groups. More formal information about diagnosing, testing, and managing ME is available in the International Consensus Criteria (ICC) and the International Consensus Primer (ICP).
The presence or absence of ‘fatigue’ is largely irrelevant in determining an ME diagnosis.
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 manner, which may lessen the severity and impact.
Early diagnosis should enable a comprehensive plan of individual management to be agreed upon, between the patient and doctor. The management plan should include detaiils about symptom management and symptom relief, which may lessen the severity and impact of the individual's symptoms.
Developing an agreed plan of appropriate management - appropriate to the individual's needs - should prevent harmful approaches occurring, e.g. attempting to ‘work through illness’.
Unfortunately most GPs and doctors are not aware of the importance of this treatment practice in ME. Early and appropriate management helps reduce the risk of a more prolonged and more severe form of the illness occurring.
NB: The 3/6-month waiting period before diagnosis is not required as per the ICC 2011.
Resources for Supporting a Diagnosis of ME
Guidelines, Questionnaires, Tools & More Resources to Assess Symptoms in ME & Severity of Symptoms
While symptoms vary from patient to patient there are a few resources available to aid a proper understanding of ME, and to confirm a diagnosis, documents that can be used to highlight individual symptoms and disability. There are many general self-report tools available online but we feel that the ones we suggest below are some of the best tools available. These include:
· One of the best international criteria documents currently available, i.e., the Myalgic Encephalomyelitis International Consensus Criteria and Primer (ICC & ICP)
· Symptom & Severity, PEM & Paediatric questionnaires based on research by DePaul University
· Disability and Functional Ability Scales
Guidelines - International Criteria
For diagnosis and management we suggest that you use the Myalgic Encephalomyelitis International Consensus Criteria (ICC 2011) & the Myalgic Encephalomyelitis Adult & Paediatric International Consensus Primer for Medical Practitioners (ICP 2012), both specifically written about and for ME, designed to assess the unique combination of symptoms and features found in ME.
The International Consensus Primer is written with doctors and other healthcare providers in mind, to provide clinicians with a one-stop user friendly reference for ME.
The Frontiers Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Diagnosis and Management in Young People: A Primer (2017), Peter C Rowe et al, is another very useful paediatric primer.
The Myalgic Encephalomyelitis (ME) International Consensus Criteria (ICC)
The ICC is a medical case definition that can accurately diagnose Myalgic Encephalomyelitis (ME). As per the ICC, for pediatric and adult cases a diagnosis can be made immediately; there is no need to wait up to 6 months.
The International Consensus Criteria (ICC) 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.
| 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 |
See the patient checklist from the ICC here
The Myalgic Encephalomyelitis (ME) International Consensus Primer (ICP)
The ICP is a physician guide that includes immune dysfunction, neurological issues, and cardiac abnormalities that are often overlooked in other information. The ICC alone is not enough to make an ME diagnosis, the next step is to confirm the ME diagnosis. The International Consensus Primer guides doctors to diagnose, treat and rule out other diseases. It is a booklet prepared by ME experts to guide doctors in treating those who fit the ICC.
A personalised clinical assessment and diagnostic worksheet for ME begins on page 10 of the ICP and includes how to confirm issues recognized in ME, and testing recommendations to rule out other diseases.
Note: The ME International Consensus Primer states:
“Patients diagnosed using broader or other criteria for CFS or its hybrids (Oxford, Reeves, London, Fukuda, etc.) should be reassessed with the ICC. Those who fulfill the criteria have ME; those who do not would remain in the more encompassing CFS classification.“
Note: Part of the process of confirming an ME diagnosis is to rule out other diseases. Too often patients discover after getting an ME diagnosis that a treatable illness had been overlooked.
Other Guidelines
Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Diagnosis and Management in Young People: A Primer
This primer, written by the International Writing Group for Pediatric ME/CFS, provides information necessary to understand, diagnose, and manage the symptoms of ME in children and adolescents. ME is characterized by a substantial loss of physical and mental stamina.
Please see the Frontiers Paediatric Primer here
Questionnaires
Symptom Questionnaire (DePaul Symptom Questionnaire DSQ-2)
The DePaul Symptom Questionnaire (DSQ-2) is a self-report assessment created by Leonard Jason at DePaul University, US. With 54 questions in total, the DSQ-2 Symptom Questionnaire assesses key symptoms of ME such as the post-exertional response in ME, sleep, pain, neurological/cognitive impairments and autonomic, neuroendocrine and immune symptoms. At each item, participants have to rate the frequency and severity of the symptom on a scale from 0 to 4.
Patients can put a score for how frequently they have each symptom and how severe it is. The questionnaire is based on research so it is useful to print out to complete and to show to a GP/Consultant/other. It is also very useful for tracking symptoms.
The DSQ-2 questionnaire can support you to educate your GP/others about ME.
PEM Questionnaire (De Paul PEM Questionnaire - DPEMQ)
Post Exertional Neuroimmune Exhaustion (PENE) is a key symptom of Myalgic Encephalomyelitis (ME). PENE is sometimes referred to as PEM. The PEM questionnaire by De Paul, the DPEMQ, is a questionnaire about the post exertional response in ME, the essential criterion for an ME diagnosis.
By answering the questions in the DPEMQ questionnaire, you get an idea of how ‘activity’, i.e., how anything you do physically, cognitively, emotionally, affects you, and what your individual post exertional response is, i.e., what symptoms occur and worsen.
Every person with ME is different. The post exertional response for a lot of people might not occur straight away; it can be delayed 24 hours to 48 hours after activity.
The questionnaire includes key indicators that show within answering a set of questions that your illness sounds like ME.
Paediatric Questionnaires
The DSQ-PED questionnaires from De Paul consist of a parent form and a child form. Parents can fill in a symptom questionnaire to evidence their child's symptoms and severities and to present to the child’s doctors. From a completed questionnaire medics will receive data about the child with ME from the child and the parent 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 by De Paul University.
Full access to all the De Paul University adult & paediatric ME questionnaires in different languages here
Other Tools To Support Diagnosis
Bells Disability Scale
We tend to refer to the different severities of ME as Mild, Moderate, Severe, Very Severe, Profound - we refer to those as general categories which really have ranges within themselves ie Mild has its own range, as does Moderate and so on.
A good scale that could be used along with these categories to determine near exact range is the Bells Disability Scale. Different people suffer in different ways but this scale gives an idea of the level of disability.
• It can be used by both Patient and Doctor to monitor progress/relapses of ME over time.
• Tick which descriptor best describes you today.
Bells Disability Scale
% Description
100 No symptoms at rest; no symptoms with exercise; normal overall activity level; able to work fulltime without difficulty.
90 No symptoms at rest; mild symptoms with activity; normal overall activity level; able to work full-time without difficulty.
80 Mild symptoms at rest; symptoms worsened by exertion; minimal activity restriction noted for activities requiring exertion only; able to work full-time with difficulty in jobs requiring exertion.
70 Mild symptoms at rest; some daily activity limitation clearly noted; overall functioning close to 90% of expected except for activities requiring exertion; able to work full-time with difficulty.
60 Mild to moderate symptoms at rest; daily activity limitation clearly noted; overall functioning 70% - 90%; unable to work full-time in jobs requiring physical labour, but able to work full-time in light activities if hours flexible.
50 Moderate symptoms at rest; moderate to severe symptoms with exercise or activity; overall activity level reduced to 70% of expected; unable to perform strenuous duties, but able to perform light duty or desk work 4-5 hours a day, but requires rest periods.
40 Moderate symptoms at rest; moderate to severe symptoms with exercise or activity; overall level reduced to 50% - 70% of expected; not confined to house; unable to perform strenuous duties; able to perform light duty or desk work 3-4 hours a day but requires rest periods.
30 Moderate to severe symptoms at rest; severe symptoms with any exercise; overall activity level reduced to 50% of expected; usually confined to house; unable to perform strenuous tasks; able to perform desk work 2-3 hours a day, but requires rest periods.
20 Moderate to severe symptoms at rest; severe symptoms with any exercise; overall activity level reduced to 30% - 50% of expected; unable to leave house except rarely; confined to bed most of day; unable to concentrate for more than 1 hour a day.
10 Severe symptoms at rest; bedridden the majority of the time; no travel outside of the house; marked cognitive symptoms preventing concentration.
0 Severe symptoms on a continuous basis; bedridden constantly; unable to care for self
Bell's Disability Scale here
More about Bell's Disability Scale here
Functional Ability
A functional ability scale is an important tool to help you work out where you are with your illness severity, we include the one from Action for ME (UK) here.
Grip Test
Research has
shown that grip strength is associated with a number of health indicators,
including mobility. While grip strength isn't necessarily used when you're
walking, it's associated with mobility. People with physical limitations are
more likely to have decreased grip strength.
A grip test is used as an
objective measure of disease status and severity in ME and correlates with
fatigue and pain severity and with physical and mental functioning.
'Hand grip
strength (HGS) is a reliable measurement of localized muscle strength and
reflects the force derived from the combined contraction of extrinsic hand
muscles.
Originally developed for hand surgery to determine capacity after
trauma or surgery, hand grip strength correlates well with other muscle
function tests such as knee extension strength. Moreover, reduced HGS has been
associated with morbidity and mortality, with low values associated with falls,
disability, impaired health-related quality of life and prolonged length of
stay in hospital. It has also shown to be strongly correlated with
post-operative complications and has been reported as a predictor of loss of
functional status and short-term survival in hospitalized patients.'
How to Measure Grip Strength:Grip strength is measured using an instrument called a dynamometer. Measure your grip strength with a dynamometer using the following steps:
- Hold your arm with your elbow bent at a 90-degree angle.
- Squeeze the dynamometer as hard as possible.
- Apply grip force in a smooth motion. Avoid jerking.
- Repeat twice more for a total of three times.
- Your grip strength is the average of the three readings.
Overall, patients with ME have significant lower hand grip strength (HGS) values than healthy controls. MS is the most common immune-mediated inflammatory demyelinating disease of the central nervous system. One of the most prominent features of MS is motor weakness. Therefore, it is expected people with MS to display lower HGS. However, ME patients also have significantly lower HGS values compared to healthy controls, even after controlling for age, sex, and BMI, with even mild cases showing lower HGS.
People with ME have preserved muscle tonus, suggesting that other than local factors related to the integrity of upper limb must be involved.
It has been suggested that these might relate to ongoing inflammation or disruption of signaling mechanisms between central nervous system and periphery, and it may also represent an overall measurement of “physical health and functioning.”
More here
More Useful Resources for Diagnosis
Patient Doctor Check List Based on the ICC
Patient Doctor Check List Based on the ME- International Consensus Criteria (ICC) from GAME - ICC, thanks to Wendy Boutillier here.
Simple Questionnaire 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 by the patient: ICC Questionnaire.
Differential Diagnoses - Conditions to Rule Out Information Sheet (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. When indicated on an individual basis, rule out other diseases that could plausibly simulate the
widespread, complex, symptom pathophysiology defining ME, e.g; infectious disorders: TB, AIDS, Lyme, chronic
hepatitis, endocrine gland infections; Neurological: MS, myasthenia gravis, B12; Autoimmune disorders:
polymyositis & polymyalgia rheumatica, rheumatoid arthritis; Endocrine: Addison’s, hypo & hyper thyroidism,
Cushing’s Syndrome; cancers; anemias: iron deficiency, B12 [megaloblastic]; diabetes mellitus; poisons.
ME International's Conditions to Rule Out handout has useful patient information.
Films About ME To Educate & Support a Diagnosis
Films about ME & Severe ME, from Dialogues for ME, by Natalie Bolton & Josh Biggs
- Dialogues for ME introduction to ME, link here: Introduction to ME
- Reduced function and the range of symptoms in ME: Patients Accounts - Symptoms
- Post exertional intolerance and the post exertional response, i.e. PENE, also referred to as PEM, the cardinal feature of ME: Understanding Post Exertional Malaise - a Hallmark Feature
- A Brief Guide to Post Exertional Intolerance
More About Diagnosis
Diagnosis in ME using Cardiopulmonary Exercise Testing (CPET)
Cardiopulmonary exercise test-retest studies have confirmed many post-exertional abnormalities. What can be helpful in relation to diagnosis of ME is having a two-day cardiopulmonary exercise test which measures oxygen uptake and use.
Cardiopulmonary Exercise Testing (CPET) testing is not easy to organise as it is a hospital-based investigation; neither is it suitable for most people with ME.
The two-day cardiopulmonary exercise test (CPET) is a diagnostic test which involves testing an ME patient exercising on an exercise machine, while monitoring their respiration, especially oxygen consumption. This test is repeated the following day in order to confirm the patient's inability to replicate the first-day performance. This test is thought to be the most objective way to detect the atypical post-exertional response in ME, i.e. post exertional neuroimmune exhaustion (PENE).
CPET is a two consecutive day comprehensive 8-12 minute cardiopulmonary exercise stress test (measuring heart, lung, and
metabolic function) only suitable for some people with mild-moderate ME (with caution).
Testing is performed on a stationary bicycle with resistance added incrementally. The results of studies show that only ME patients have significantly worse scores the second day and abnormal recovery from exertion.
CPET is an exercise tolerance test with expired gas exchange - (2 consecutive days) - measures cardiovascular, pulmonary & metabolic responses at rest & during exercise: peak oxygen consumption VO2 or VO2 at anaerobic threshold (AT) -
decline of 8% or greater on test 2 indicates metabolic dysfunction; post-exercise blood analysis - increase in
sensory, adrenergic and immune genes - increase in metabolite receptors unique to ME.
More about CPET here
Diagnosis of Orthostatic Intolerance in ME & the NASA Lean Test
Orthostatic intolerance is common among individuals with ME. Some studies have employed tilt table tests lasting 2–5 min to diagnose one common form of orthostatic intolerance, i.e., postural tachycardia syndrome (POTS).
During tilt testing measurements show that cerebral blood flow is reduced in ME patients. A tilt table test is used to help diagnose forms of dysautonomia such as postural orthostatic tachycardia syndrome (POTS) or neurally mediated hypotension (NMH).
Symptoms caused by dysautonomia include dizziness, lightheadedness, or fainting (syncope). Vital signs, especially heart rate and blood pressure are monitored as the table slowly raises the patient from lying to nearly standing position.
A tilt table test is generally safe, and complications are rare, but it would only suit some people with mild ME. As with any medical procedure, it carries some risk. Potential complications include: nausea and vomiting, fainting, weakness that can last several hours, and prolonged low blood pressure after the test.
An alternative to Tilt Table Testing is the NASA Lean TestNote! Most people with ME especially those with more severe ME would not be able to do Tilt Table Testing. An alternative test for those who can get out of bed without difficulties, and who are able to sit up for 10 minutes and then also stand for at least 10 minutes is the NASA Lean Test.
For those with more severe ME, doctors can monitor pulse and blood pressure while the patient is standing, and again while lying down. This may need to be repeated several times.
It is known as the ‘poor man’s tilt table test, or more modernly as the NASA Lean Test.
Step By Step Procedure in the NASA Lean Test
Ask the patient to lie down and rest quietly for 15 - 20 minutes.
Then take the first blood pressure and pulse readings and make a note of them.
Ask the patient to sit up for 10 minutes, or as long as they can manage without severe problems, and then take another set of readings and make a note.
Then direct the patient to stand up for 10 minutes, leaning against a wall, but without fidgeting or moving or talking which can affect the result. Then take another set of readings and make a note.
After another 10 minutes of standing and leaning, take the readings again and make a note.
Keep leaning. Do not flex any muscles or talk.
After another ten minutes, take the readings again.
Important Notes for the doctor and patient with regards to the NASA Lean Test!
If at any time the patient starts to feel sweaty or hot or nauseous, the doctor/practitioner needs to take the patient's bp and pulse readings right away and get the patient lying down as soon as possible.
Many patients will not be able to tolerate this much time upright and will need to stop the test partway through. If this happens, take another reading (if possible) and then the patients lies down again.
Failure to stop the tests when the patient becomes severely ill can lead to a loss of consciousness, or severe relapse lasting days, weeks or even months in the very severely affected. Someone should always be standing near the patient to catch them if they fall and serious requests to stop the test must be acted on in a timely manner.
Assessing the readings taken:
For Neurally Mediated Hypotension* (NMH), the patient has to have a 20-25 mm drop in systolic blood pressure (the higher number).
If the patient's pulse suddenly rises at least 30 bpm (beats per minute), then it is likely they have Postural Orthostatic Tachycardia Syndrome* (POTS).
Dr. Rowe believes that they are both really the same thing - with either, if the patient doesn't lie down, they're going to pass out. And the treatment for both is the same.
*Neurally mediated hypotension is also known by the following names: the fainting reflex, neurocardiogenic syncope, vasodepressor syncope, the vaso-vagal reflex, and autonomic dysfunction. Hypotension is the formal medical term for low blood pressure, and syncope is the term for fainting. Neurally mediated hypotension occurs when there is an abnormal reflex interaction between the heart and the brain, both of which usually are structurally normal.
*Postural orthostatic tachycardia syndrome (or POTS) is a condition of orthostatic intolerance in which a change from the supine position to an upright position causes an abnormally large increase in heart rate, often, but not always accompanied by a fall in blood pressure. Patients with POTS also have problems in maintaining homeostasis when changing position ie moving from one chair to another or reaching above their heads.
Symptoms include an abnormally large increase in heart rate upon standing, lightheadedness, extreme fatigue, nausea, headache, chest pain, exercise intolerance and impaired concentration. Patients may exhibit mild hypotension while standing, but most do not experience fainting. POTS patients are usually significantly debilitated by their symptoms.
More Information on the NASA Lean Test
'A Simple Way to Assess Orthostatic Intolerance', an information document by Lucinda Bateman, includes images and pdf copies of instructions for medical providers and patients about how to do the NASA Lean Test, see here.
Common Comorbidities in Myalgic Encephalomyelitis
The International Consensus Primer for Myalgic Encephalomyelitis lists the following comorbidities: myofascial pain syndrome, temporomandibular joint syndrome (TMJ), interstitial cystitis, Raynaud's phenomenon, prolapsed mitral valve, irritable bladder syndrome, Hashimoto's thyroiditis, sicca syndrome (Sjögren's syndrome), secondary depression, allergies, and multiple chemical sensitivities.
Further Reading
- Diagnosis of Myalgic Encephalomyelitis (ME) Part 2 here
- Diagnosis of Myalgic Encephalomyelitis (ME) Part 3 here
- Potential Symptoms in ME here
- Diagnostic Overshadowing here
- Communicating with Doctors & other healthcare providers here
- Supporting Individuals with Severe ME in a Hospital or Other Healthcare Setting Information Pack for doctors and other healthcare providers here
A Comment on the Quality of Life in ME
Danish QOL Paper
ME needs to be acknowledged as a serious disease causing significant impact on health and QoL, not only of the individual but also of their family. Education for healthcare practitioners must reflect this.The medical encounter can be vastly improved by acknowledging the impact on family members and providing practical advice and support to both people with ME and their family members.
A Danish research paper, 'The Health-Related Quality of Life for Patients with Myalgic Encephalomyelitis', Michael Hvidberg et al, 2015 discusses the very low quality of life in ME. Please see paper here.
The analysis in the study confirmed that poor HRQoL of ME is distinctly different from and other conditions. All members of the Danish ME Patient Association in 2013 (n=319) were asked to fill out a questionnaire including the EQ-5D-3L. The EQ-5D-3L-based health related quality of life (HRQoL) of ME is significantly lower than the population mean and the lowest of all the compared conditions.
Important Note About Labels
Labels used to Refer to ME
ME is often referred to by the problematic term “chronic fatigue syndrome” or ME/CFS. The incomplete and stigmatising nature of the term chronic fatigue syndrome is frequently compared to previously misapplied hysterical paralysis terminology used for multiple sclerosis patients.
Diagnosis has previously been undermined by lax criteria that has resulted in misdiagnoses and contributed to ongoing stigma and discrimination; the Oxford, Fukuda and NICE 2007 criteria should not be used for diagnosis. Research studies using those criteria do not apply to ME.
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