US ME/CFS Clinician Coalition: Guideline - Diagnosing and Treating ME/CFS, 2019, and new website 2020

The content is not bad but I wonder who it is intended for?

I am afraid that to me it has the air of the 'proclamation by clinical experts' that belongs to the 1970s. The statements about diagnosis do not seem to have any particular justification and I am not sure they could. The list of treatments looks unproven and muddled.

I think PWME want something better than this - something genuinely evidence based and something emphasising just how little we know. I am tempted to say that if a physician wants to know what ME is they would do better to chat to 1000 people with the illness than read this.

 

Seems very much an IOM SEID guide and that was supposed to do away with ME and CFS so is this Beyond Myalgic Encephalomyelitis part deux

 

i agree that the treatment list is unproven and muddled, and interestingly each of these physicians (or their office partners) are known to prescribe well beyond these recommendations, may it be antivirals, immune modulators, plasmapheresis, Ampligen etc.

While there is a need for basic care documents for physicians, we have guidelines already. We do need clinical research really badly. Drug trials to show effectiveness and safety even if it is for something as trivial as low-dose naltrexone. Teasing out sub groups would be in my view a priority.

Research is what’s desperately needed.

 

There are a lot of long guideline documents. I'm not sure there are that many short ones, apart from some ones from some patient organisations that might be treated with some scepticism by many medical professionals.

Agree about the need for more research. For that, we need more public and private money to be available.

 

This definitely does not solve the problem of how skewed the scale is when the "mild" form of this disease is a 50% reduction in ability to function, something anyone would consider to be ruinous.

But to properly educate on this would basically require completely changing the perception of the entire medical profession about things it absolutely and aggressively does not want to see differently. We have seen plenty of examples in other diseases how fatigue is trivialized even in serious diseases. To properly educate on what PEM is, a unique concept that runs counter to almost everything the medical profession believes and teaches its own, is a seriously daunting task.

The simple truth is that the basic facts about how broken everything is about this disease sound hyperbolic. It's a bit like overturning slavery when the common perception of the enslaved is that they are basically animals without any soul or inherent value and so why would anyone even care one bit about them? We can only accomplish so much from our current position.

It's stupid but stupidly broken is where we're at. I don't really know a better way than working by steps. This effort could be better but quite frankly medicine as a whole simply isn't ready and willing to be told just how massively and thoroughly it has failed us in the worst possible way.

 

The document says that symptoms must be "moderate to severe". Most of us have a distorted view of ME. Our mild ME has moderate to severe symptoms. It is just that severe is very severe for us.

 

I think you have to take into account what the document is and what it isn’t. The document is an extremely brief diagnostic assist for clinicians. It isn’t a new definition of ME/CFS. I imagine they would HAVE to comply with one of the definitions that has reached a consensus. In that regard I think they got it right, though your suggested wording could be a beneficial tweak.

Such a strong emphasis on PEM. An actual tool to help clinicians identify PEM. Awesome! A GP’s takeaway is going to be PEM and how to identify it. In my mind that’s stepping away from a fatigue definition.

I would be curious about how many of us who develop the full raft of ME symptoms use the words “fatigue”, “no energy” or “tired” that first time we roll up to the dr’s office without a clue about what’s wrong with us. And in those first months or years some of us might not discover that rest actually makes a dent in fatigue. It’s the type of wording that a pwME would use in the first year of confusion that is the most important to capture. Though I recognise many go undiagnosed for years and maybe evolve how they describe their symptoms differently than those diagnosed early and given literature on ME, but in that case a pattern of PEM will hopefully be uncovered by the questions this document suggests. If the vast majority of ME patients initially use words like fatigue (and I still do) then it continues to be valuable to have in a diagnostic criteria. Even now I struggle to put any other name to the sensation which I don’t believe is described by any word in the English language. In 10 years I hope a word for this sensation enters the vernacular.

Having a foggy day so I hope I have got my thoughts across without coming across as argumentative.

 

Not perfect but I do think it's positive to have a short document that may actually be read and also places heavy emphasis on PEM. But yes, the obsession with fatigue is getting rather tedious.

The description of PEM could be clearer. The quote below (my bolding) leaves plenty of room for confusion between PEM and post-exertional fatigue; the latter is common enough in other conditions, i.e. a known quantity for doctors and thus the lens through which most of them will try to understand PEM - and end up misunderstanding.

That raises issues for me, too. In addition to the above I worry that very mild - and therefore undiagnosed - cases unnecessarily end up exerting themselves into a more severe state. Plenty of anecdotal evidence for that occurring though there is probably no ethical way of ever studying this in a controlled manner.

In my own experience, back when I was chasing a diagnosis I asked a doctor if ME was a possibility. His answer was that the thought had occurred to him but that I wasn't bad enough to have ME. So I continued my attempts to regain my previous fitness and no prizes for guessing how that turned out...

In hindsight I can see I clearly had PEM. So by the reasoning that if you have PEM you have ME, I had ME, but by the reasoning that you have to have moderate-severe symptoms at least half the time I didn't have ME. Very Schrodinger.

At the very least we need a concept of pre-ME, similar to prediabetes. You don't quite meet all the criteria for ME, or diabetes, but you're at high risk of doing so in the future if you don't start managing your condition now.

 

I don't think they have the mild, moderate, severe classifications of ME/CFS in the US, it's only used in the UK. And what is called 'mild' in the UK is generally called 'moderate' in the US, which all just adds to the confusion.

(I appreciate they are using moderate/severe in relation to symptoms in this instance).

Something re severity scales in UK (NICE) that needs clarification is; are the classifications in relation to severity of 'symptoms', or are they being used in relation to the disease itself
eg "patient A has a mild form of ME" . (phrases like this often seen and of course are unhelpful as you either have it or you don't).

 

https://twitter.com/user/status/1168569273956143104


Hillary Johnson’s ( Osler’s Web) and others’ tweets about this. I am not able to read this morning, only scan, but others might be interested.

Eta: sp

 

I think the diagnosis would be missed in more people when they are milder or at an earlier stage with the international consensus criteria than with the IoM criteria.

https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2796.2011.02428.x

Table 1. Myalgic encephalomyelitis: international consensus criteria


Adult and paediatric ● clinical and research

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.

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 24 h 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.

Operational notes: For a diagnosis of ME, symptom severity must result in a significant reduction of a patient’s premorbid activity level. Mild (an approximate 50% reduction in pre‐illness activity level), moderate (mostly housebound), severe (mostly bedridden) or very severe (totally bedridden and need help with basic functions). There may be marked fluctuation of symptom severity and hierarchy from day to day or hour to hour. Consider activity, context and interactive effects. Recovery time : e.g. Regardless of a patient’s recovery time from reading for ½ hour, it will take much longer to recover from grocery shopping for ½ hour and even longer if repeated the next day – if able. Those who rest before an activity or have adjusted their activity level to their limited energy may have shorter recovery periods than those who do not pace their activities adequately. Impact : e.g. An outstanding athlete could have a 50% reduction in his/her pre‐illness activity level and is still more active than a sedentary person.

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
Notes: Neurocognitive impairments , reported or observed, become more pronounced with fatigue. Overload phenomena may be evident when two tasks are performed simultaneously . Abnormal accommodation responses of the pupils are common. Sleep disturbances are typically expressed by prolonged sleep, sometimes extreme, in the acute phase and often evolve into marked sleep reversal in the chronic stage. Motor disturbances may not be evident in mild or moderate cases but abnormal tandem gait and positive Romberg test may be observed in severe cases.

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

Notes: Sore throat, tender lymph nodes, and flu‐like symptoms obviously are not specific to ME but their activation in reaction to exertion is abnormal. The throat may feel sore, dry and scratchy. Faucial injection and crimson crescents may be seen in the tonsillar fossae, which are an indication of immune activation.

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

Notes: Orthostatic intolerance may be delayed by several minutes. Patients who have orthostatic intolerance may exhibit mottling of extremities, extreme pallor or Raynaud’s Phenomenon. In the chronic phase, moons of finger nails may recede.

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.
Classification
——— Myalgic encephalomyelitis
——— Atypical myalgic encephalomyelitis: meets criteria for postexertional neuroimmune exhaustion but has a limit of two less than required of the remaining criterial symptoms. Pain or sleep disturbance may be absent in rare cases.
Exclusions: 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. Primary psychiatric disorders, somatoform disorder and substance abuse are excluded. Paediatric:‘primary’ school phobia.
Comorbid entities: 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, Sicca syndrome, reactive depression. Migraine and irritable bowel syndrome may precede ME but then become associated with it. Fibromyalgia overlaps.

 

I’m not sure , seeings these are based on SEID criteria, why it was felt needed to release these when the IOM NAM guidance was to help diagnosis and management?
I would take these over nice criteria dominating perhaps GPs in the UK and think they’re reasonable, like SEID the emphasis is on PEM which is welcome. They do acknowledge also the other common accompanying serious symptoms which aren’t universal.

“Other ME/CFS symptoms include widespread pain; additional forms of sleep disturbance; light, noise, and chemical sensitivity; sore throat; tender lymph nodes; headaches; gastrointestinal problems; and genitourinary issues. The NAM Clinician’s Guide provides further explanation and guidance on how to identify both core symptoms and additional symptoms (see references at the end)..”

 

I've worked with the US ME/CFS Clinician Coalition on this document and can provide feedback to them if you wish. In the meantime, I wanted to give my own perspective on the discussion so far. I'm speaking for myself and not on behalf of the clinicians.

1. Regarding comments on the wording of the criteria.... This is directed to US clinicians and is based on the IOM criteria, which has been adopted by the CDC and some of the key medical education providers. The IOM includes core criteria as follows but also lists a number of other criteria (pain, immune, infection, GI, etc) which can be found in the IOM Clinician Guide. The IOM criteria state the frequency and severity requirements based on Jason's work which showed that misdiagnosis was more likely if people were just reporting mild and infrequent symptoms (The Clinician's Guide gives some examples of Jason's analyses)


As pointed out in a recent article by Lapp, experts may use an instrument like the Canadian Criteria to help confirm a diagnosis

2. Regarding why this was needed since the IOM already discussed diagnosis and treatment - The IOM report provided a review of the scientific literature and also proposed diagnostic criteria. But IOM did not cover differential diagnosis, comorbidities, or treatments. On a CFSAC workgroup on medical education I was on, we heard that attitude and disbelief was a problem with some doctors and others were harming patients with recommendations for CBT and GET because they had been told that was appropriate. But we also heard that some docs believed the patients but were turning them away because they didn't think there was anything they could do to help them. We need to change the misinformation while also helping doctors understand what they can do instead

3. Regarding the treatments recommended - this is intended as a basic and brief guide for the medical providers such as GPs who are likely to come in contact with people with ME. As such, while it states that experts may use treatments such as antivirals, those are not included in the basic list of treatments. GPs may not be willing/feel qualified to use such drugs. But as above, there are still things that GPs can do to help patients. In addition to specific drugs, pacing is a big one as is gaining accommodations and disability support. I dont know how it is elsewhere but in the US, you can't get disability without a physician's documentation.

4. Regarding levels of severity - While this does suggest levels of severity with the statement about some patients not being able to do basic activities of daily living, I agree this does not explicitly define the different levels of severity. At least in the US but I think elsewhere, that's is a gap that needs to be addressed and explicitly defined for both clinical care and in research. One way to define severity is by the overall functioning of the patient rather than by the severity of a given symptom. The specifics of the severity framework are a topic for another thread (and I think already are) but in a very general sense, I've seen some define this as those who can still work are mild, those who are bedbound are very severe, those who are housebound are severe and those who can leave the house some but not work are moderate.

5. Regarding the need for treatment trials - I completely agree. That's a huge issue. But we know that that will take time and money. And we are in a catch-22 when there are so few clinicians who can properly identify the patients that trial recruitment is an issue. In the meantime, GPs and others who care for ME patients could use already approved drugs and non-pharmacological approaches to treat symptoms and/or comorbidities which could help reduce the burden of the disease. This provides the basic approaches these doctors have found helpful. Many/most of these drugs are also listed in the 2014 IACFS/ME primer and the 2017 Pediatric Primer published in Frontiers. (I can imagine different countries have different rules about off-label use of drugs but it's allowed in the US

6. I think I saw a tweet about no specific lab tests being recommended for diagnosis. This may again be US specific but the challenge is that tests that experts use may not be available to mainstream clinicians through the lab testing services they use - or if they are available, the results are not reliable. For instance, for NK-cell function, those that use that test process the samples very quickly after draw but the lab testing services can take too long to process the samples and give a misleading result on function.

7. And a question for you on the document focusing too much on fatigue...
Could you give me feedback on where and how it does that? Fatigue is mentioned is in the first criteria and is part of the name that's used here still unfortunately. But beyond that, I think fatigue is only mentioned in differentiating from "chronic fatigue" and from the historical "medically unexplained fatigue" used by Fukuda (which the US has been using for clinical care for decades).

Let me know if I missed any key themes, didn't address the concerns being raised, etc. I'd really appreciate any additional perspective you have.

 

Thank you for the background information.Your work and that of the clinicians is appreciated.

Some more feedback.

There's a lot that's good about this document, especially its brevity and its focus on PEM, but I think the document suffers from the authors knowing too much and being too deeply involved in the topic. That may sound paradoxical but it is their in-depth knowledge that frequently leads experts to being unable to envisage how their output will be interpreted by non-experts. The linguistic equivalent of Murphy's Law applies: if it can be misunderstood it will be misunderstood. The expert authors know what they mean. We, the expert patients, know what they mean. But does your average family doctor? Or the newly-diagnosed patient?

It's highly likely many in the latter two groups will misunderstand parts of the document and that's because it's human nature to try to make sense of difficult new stuff by linking it to stuff we already know and are comfortable with. This applies to doctors, too, and three things most doctors do know about are TATT (tired all the time), the "fact" that CFS is all about fatigue, and post-exertional fatigue (because it occurs in a number of other conditions).

Those three things will therefore form the frame of reference into which doctors will try to fit what they read about ME.

In addition, when old and new knowledge aren't very clearly separated in our brains we often don't remember the new knowledge long-term but revert back to the old. Cue another variant of Murphy's law: if it can be misremembered it will be misremembered.

Because of the above the word fatigue should only ever appear once in the document, in the title, for example: "Myalgic Encephalomyelitis (also known as Chronic Fatigue Syndrome or ME/CFS)" - and thereafter be banned. Every mention of the f****** word just further reinforces the preconceived notion that ME is about tiredness. When talking about the symptom of f****** it could be replaced by "extreme exhaustion" for example.

"The fatigue is profound, not lifelong, not the result of ongoing exertion, and not alleviated by rest." The underlined parts are problematic. They directly contradict the concept of PEM: exertion increases symptoms including fatigue/exhaustion, conversely rest and pacing alleviate symptoms including fatigue/exhaustion.

Yes, there are many cases where it may appear as though rest does not help. There are the milder cases who keep trying to lead a normal life and who never rest enough to get themselves out of permanent PEM, and there are the very severe who simply cannot rest enough to escape permanent PEM. Those in the middle, however, who have learned to pace, will often report substantial reductions in fatigue/exhaustion during non-PEM periods

How about changing the above sentence to "The exhaustion is profound, not lifelong, not the result of excessive exertion, and not fully alleviated by normal amounts of rest"? ("Excessive exertion" here refers to what a healthy person would view as excessive; there's probably a clearer way to express the point but it's eluding me right now. ETA: I finally found what I was trying to express: "out of proportion to the triggering exertion" instead of "excessive exertion").

As I explained in my previous post, the explanation given for PEM leaves room for it to be misinterpreted as the sort of post-exertional fatigue doctors already know from other conditions. My neighbour gets disproportionately tired after minor exertion and takes a long time to recover. That would fit the definition of PEM in the document. Does my neighbour have ME? No, he has a heart condition.

This part of the definition is the most problematic: "patients can experience an exacerbation of one or all of their symptoms" because if the only symptom that gets exacerbated happens to be fatigue, especially if it happens without any delay ("the crash, which may be immediate but more often delayed"), that wouldn't be very specific to ME.

How about changing to "patients typically experience an exacerbation of many or all of their symptoms, and symptoms may have no obvious link to the triggering activity, for example a taking a shower may cause flu-like symptoms the following day"? After all, who is so lucky as to get PEM with only a single symptom getting worse?!

 

What @Ravn said in the post above, at this moment I think it covers all points, and more, that I would want to make.

 

I think Ravn makes an important point here.

Around 1990 one of my academic colleagues in Rheumatology wrote a paper entitled something like 'Do Rheumatologists Do What They Think They Do?' The point of the paper was to show that the opinions given by rheumatologists about how to practice rheumatology often deviated significantly from the way they actually practiced it.

The message was that as human beings we are often not very good at describing our own practices or motivations. In medicine this has been a big problem over the years. The American textbooks of the 1990s got very close to a rigorous way of presenting material that minimised this. In comparison UK textbooks had been didactic up until then. That was improving but sometime around 2000 all textbooks became international in authorship and things started to get sloppy generally. My impression is that now things have slipped back to the didactic talk of the 1970s. And so very often this includes statements that are pretty guaranteed to be misunderstood, as Ravn says.

My feeling is that the international ME clinician community has to move on from a few 'experts' making pronouncements for the benefit of ignorant primary care physicians. It equally has to move on from bureaucratic organisations generating guidelines based on poor quality trials. Quite how we get there I do not know, but maybe with more patient involvement and more involvement from clinicians outside the field with wider experience of drafting advice things will get there.

 

this should feature somewhere as it is one feature that is possibly unique and one that cannot be 'explained away' by those supporting the 'deconditioning/fear avoidance' model.

 

In Lily Chu's paper she found that inflammatory symptoms were common after exertion in people with ME but absent in healthy people. This covers chills as well as sore glands and throats.

It is such a unique category of symptom type that it should be emphasised.