Solving the ME/CFS criteria and name conundrum: the aftermath of IOM, 2020, Jason & Johnson

[Dolphin]

https://www.tandfonline.com/doi/full/10.1080/21641846.2020.1757809

Journal
Fatigue: Biomedicine, Health & Behavior
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Articles
Solving the ME/CFS criteria and name conundrum: the aftermath of IOM
Leonard A. Jason &Madeline Johnson
Received 18 Mar 2020, Accepted 15 Apr 2020, Published online: 28 Apr 2020

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• https://doi.org/10.1080/21641846.2020.1757809
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• Case definition, systemic exertion intolerance disease, chronic fatigue syndrome, myalgic encephalomyelitis

ABSTRACT

In 2015, the Institute of Medicine (IOM) proposed a new name and set of clinical criteria for what had previously been referred to as Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS).

This committee recommended the adoption of the term systemic exertion intolerance disease (SEID) and clinical criteria that required specific symptoms such as post-exertional malaise and unrefreshing sleep.

This article reviews efforts to evaluate the revised criteria as well as reactions to the new criteria and name.

Since these recommendations have been made, the proposed name change has not been widely adopted by the scientific or patient community.

Even though the IOM’s proposed criteria were intended to be a clinical rather than a research case definition, over the past few years, an increasing number of studies have employed these criteria for research purposes.

One unwitting consequence of the IOM criteria, which excludes few other illnesses, is the broadening of the number of individuals who are diagnosed and included in research studies.

There is still a need to implement the IOM’s recommendation to form a multidisciplinary committee to review research and policy changes following the release of the new criteria.

We conclude by presenting a possible roadmap for overcoming barriers in order to make progress on developing a consensus for a name and criteria.

KEYWORDS: Case definition, systemic exertion intolerance disease, chronic fatigue syndrome, myalgic encephalomyelitis

 

[Andy]

Open access, https://sci-hub.se/10.1080/21641846.2020.1757809

 

[Hoopoe]

One unwitting consequence of the IOM criteria, which excludes few other illnesses, is the broadening of the number of individuals who are diagnosed and included in research studies.

In my opinion Jasons's argument is flawed because he operationalized PEM incorrectly when assessing the IOM criteria, and then found that there are problems. He claims this is due to lack of exclusionary conditions. I think it's because he misunderstands PEM.

He also claims that the core symptoms in the IOM criteria are found in many other illnesses, which implies that many other illnesses have PEM as symptom. I doubt we can say that. They might have "being tired after activities" as symptom, which is how Jason defined PEM in one of his studies but that is not what PEM is. PEM is a delayed relapse and symptom exacerbation after exertion and the IOM report is very clear on this.

It might be the case that other illnesses do have PEM but if that is the case, my understanding is that IOM criteria are meant to be applied only when the other illnesses of the patient are insufficient to explain the symptoms, and the symptoms are consistent with IOM criteria. If multiple sclerosis were found to cause PEM then this wouldn't lead a double diagnosis of ME and ME/CFS but just MS because the MS would be sufficient to explain the presence of PEM.

It also should be said that more people being diagnosed with ME/CFS and participating in research studies is not necessarily a bad thing. It's only a bad thing if they were misdiagnosed. If they were correctly diagnosed then it would be a very good thing. The real issue seems to be our lack of understanding of the underlying biology and the lack of an objective diagnostic test. This opinion-based debate about criteria would end pretty quickly if this problem was overcome.

 

[Snow Leopard]

I think the solution is to form another 20 committees, each to propose their own name, leading to much confusion in academia, until they have the bright idea to just let us name it!

 

[Hoopoe]

@Snow Leopard what name would you choose?

 

[Hoopoe]

Moving on to the consensus part of the article:

As an example of what might occur, the term Myalgic Encephalomyelitis (endorsed by the majority of patients) could be reserved for those patients who meet the research criteria, and it would identify a smaller more homogenous group of patients with more functional impairment (using either the CCC, the ME-ICC, the IOM with more exclusions, or a new set of empiric criteria). Another group of patients would be those who meet the IOM clinical criteria and their illness could be called ME/CFS (or a different name). Differentiating ME from ME/CFS would appeal to many within the patient community. Those that do not meet the criteria for the above groups could be classified as having chronic fatigue, which represents those with six or more months of fatigue. Such an approach would differentiate those patients with solely fatigue from those with a multitude of other symptoms such as post-exertional malaise.

 

[rvallee]

There's no reason for a name change until we find the core mechanism. Any such change would be temporary and would only add to confusion. We are already at 99% confusion so let's keep our meager chances where they can land safely somewhere.

The name CFS is an absurdly bad name promoted by incompetent people who still do not know the first thing about what's going on. It should never have been used and is itself highly misleading, a change for the worst. The name ME has remained in the form of ME/CFS and as such has continuity. It is not ideal but neither is 20M sick people being left to fend for themselves while being subjected to contempt and mockery by the medical profession so let's not make too much of what is ideal and what isn't.

Criteria matter a lot. The name, we're pretty much stuck with it. Past errors carry over for a long time, let's not do repeat this one again.

 

[Barry]

He also claims that the core symptoms in the IOM criteria are found in many other illnesses, which implies that many other illnesses have PEM as symptom. I doubt we can say that. They might have "being tired after activities" as symptom, which is how Jason defined PEM in one of his studies but that is not what PEM is. PEM is a delayed relapse and symptom exacerbation after exertion and the IOM report is very clear on this.

Yes. Maybe the symptoms of PEM warrants a study in itself, because it is so misunderstood by people convinced they understand. I think at its most general, the description I sometimes see, that PEM represents an abnormal response to exercise, is a good starting point, though of course it is only a starting point. Because pwME do respond abnormally to exercise, and pretty much everything else follows on from there. PEM is pretty much the cardinal symptom of that.

 

[ME/CFS Skeptic]

Made a twitter thread on this for ME Awareness hour:


1) Leonard Jason and Madeline Johnson have written an interesting paper where they reflect on diagnostic criteria for ME/CFS and the 2015 recommendations by the Institute of Medicine (IOM) report.

2) On the new proposed name, Systemic Exertion Intolerance Disease (SEID), they write: “it was probably naïve to try to impose a new name like SEID on a diverse patient community, especially with little input from major constituent groups.”

3) When it comes to diagnostic criteria proposed by the IOM report, Jason & Johnson argue that these have their merits such as a focus on core symptoms but that for research there is a major problem that exclusion of other illness were not clearly specified.

4) “At the present time”, Jason & Johnson write, “there are multiple names and criteria that are used by scientists and patients”. And that’s a problem because it makes research more difficult to compare.

5) Jason & Johnson propose to come together with all stakeholders (i.e. patients, scientists, clinicians, and government officials) to work out diagnostic criteria.

6) Working groups could develop detailed recommendations for example on how to define systematic exclusions. Once working papers are finalized, a conference could bring the different working groups together for adoption of a research case definition and name.

7) As an example of what might occur, Jason & Johnson suggest that the term ME could be preserved for patients who meet those strict research criteria, while the IOM clinical criteria could select a broader group termed ME/CFS.

8) The authors conclude: “the criteria and name conundrum can be solved, but it will take a concerted commitment to a process and collaboration from government officials, patient organizations, and the scientific community.”

 

[cassava7]

Thank you for the summary Michiel.

5) Jason & Johnson propose to come together with all stakeholders (i.e. patients, scientists, clinicians, and government officials) to work out diagnostic criteria.

6) Working groups could develop detailed recommendations for example on how to define systematic exclusions. Once working papers are finalized, a conference could bring the different working groups together for adoption of a research case definition and name.

OK, that is a sensible idea. Needs funding though, and that draws away from biomedical research funding which is already very scarce.

7) As an example of what might occur, Jason & Johnson suggest that the term ME could be preserved for patients who meet those strict research criteria, while the IOM clinical criteria could select a broader group termed ME/CFS.

Many patients from countries where CFS is the commonly used denomination don't know about the term ME and get confused: "what's the difference between CFS and ME?". So this problem won't be solved. This terminology would also make any kind of communication to the public, including advocacy, a nightmare -- people will still end up mixing terms with this proposal.

Granted, it's just a suggestion.

 

[ME/CFS Skeptic]

that draws away from biomedical research funding

I don't think it works that way.

If I understand correctly there isn't a pot of ME/CFS money that gets distributed, it's more that funding agencies like the NIH evaluative each project/study separately whether it is worth investing in (whether it's high quality impactful etc.). So if a biomedical research project is worth funding and this project to work out a new name and case definition is also worth an investment, they shouldn't hinder each other in getting funding.

The goal is to create as many of those high-quality research proposals so that overall funding for ME/CFS increases.

 

[Forbin]

He also claims that the core symptoms in the IOM criteria are found in many other illnesses, which implies that many other illnesses have PEM as symptom. I doubt we can say that. They might have "being tired after activities" as symptom, which is how Jason defined PEM in one of his studies but that is not what PEM is. PEM is a delayed relapse and symptom exacerbation after exertion and the IOM report is very clear on this. [bolding mine]

Yes. I suspect that distinguishing PEM from delayed PEM may be very important. Dr. Komaroff has said that delayed PEM is something that he's never seen in any patients other than those with chronic fatigue syndrome.

Delayed PEM may not be noticable in all ME/CFS patients at all times, however.

I didn't notice it in the first few years because it was being swamped by the constantly high "noise level" of other symptoms. It was only after I'd seen some improvement in other symptoms that delayed PEM emerged from the "background" as a feeling of being "completely beat up the next day," to paraphrase Dr. Komaroff.

So, I'd say that the absence of delayed PEM does not rule out ME/CFS, but I suspect that those who have delayed PEM are probably pretty "high confidence" cases. In other words, it's a better research criterion than a clinical criterion.

It might be helpful if they would use the terms "PEM" and "dPEM" (with "d" for "delayed").

 

[Cinders66]

There's no reason for a name change until we find the core mechanism. Any such change would be temporary and would only add to confusion. We are already at 99% confusion so let's keep our meager chances where they can land safely somewhere.

The name CFS is an absurdly bad name promoted by incompetent people who still do not know the first thing about what's going on. It should never have been used and is itself highly misleading, a change for the worst. The name ME has remained in the form of ME/CFS and as such has continuity. It is not ideal but neither is 20M sick people being left to fend for themselves while being subjected to contempt and mockery by the medical profession so let's not make too much of what is ideal and what isn't.

Criteria matter a lot. The name, we're pretty much stuck with it. Past errors carry over for a long time, let's not do repeat this one again.

In the U.K. the dominant name in official establishment nhs circles is more Chronic Fatigue Syndrome alone I feel. Certainly I’ve never met a dr who uses MECFS or the more common CFSme in the uk. My GPs usually call my illness Chronic Fatigue Syndrome or even chronic fatigue. I imagine medical schools who teach it are only using Chronic Fatigue Syndrome and We do have to factor in how much this advantages the MUS lot and consider how tarnished = deterring in countries where the bps narrative dominated, “CFS” is. More so than in America, I’ve always wondered how Ron Davis could bear to use the name when it’ seems so inappropriate but in his circles Chronic Fatigue Syndrome is less belittling.

if the choice was in the UK , this status quo or a move to systemic exertion intolerance disease, I’d take SEID. I don’t think there Is much appetite for more discussion on name and criteria though after it all being bungled for so long.

 

[Barry]

Yes. I suspect that distinguishing PEM from delayed PEM may be very important. Dr. Komaroff has said that delayed PEM is something that he's never seen in any patients other than those with chronic fatigue syndrome.

Delayed PEM may not be noticable in all ME/CFS patients at all times, however.

I didn't notice it in the first few years because it was being swamped by the constantly high "noise level" of other symptoms. It was only after I'd seen some improvement in other symptoms that delayed PEM emerged from the "background" as a feeling of being "completely beat up the next day," to paraphrase Dr. Komaroff.

So, I'd say that the absence of delayed PEM does not rule out ME/CFS, but I suspect that those who have delayed PEM are probably pretty "high confidence" cases. In other words, it's a better research criterion than a clinical criterion.

It might be helpful if they would use the terms "PEM" and "dPEM" (with "d" for "delayed").

I think the notion of delay - or not - needs very careful consideration. My wife is mild/moderate, and on good days can do fairly low power activities for quite a long'ish period of time, until she then eventually has to stop. It may not then be too long after that that she starts to feel bad. But even though it can happen soon after she finishes, who is to say it is not a delayed consequence of what she was doing when she started?

 

[Ravn]

core IOM symptoms are common in other illnesses such as cancer, Hashimoto, lupus,chronic heart failure, and multiple sclerosis

Arrgh! If even people who should know better appear to suggest that PEM (an IOM core symptom) is common in other illnesses, what hope do we have?

No point in discussing better diagnostic research criteria if we define PEM so loosely as to make it meaningless.

 

[Sean]

Very annoyed and concerned at PEM being so blatantly misinterpreted and misappropriated. This is not good, and could well set us back a long way.

(Not suggesting Jason & Johnson are doing it, but it is clearly starting to happen more generally.)

 

[Andy]

Very annoyed and concerned at PEM being so blatantly misinterpreted and misappropriated. This is not good, and could well set us back a long way.

(Not suggesting Jason & Johnson are doing it, but it is clearly starting to happen more generally.)

From what I can see, Jason is doing exactly that.

From Jason et al's paper, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4556426/ (my bolding),

Systemic Exertion Intolerance Disease [12]
Our group developed operationalized the SEID criteria [12] in the following way: a patient needed to have 6 or more months of illness (For how long have you had problems with fatigue or energy?) To meet the requirement regarding substantial reduction from previous levels of functioning, a patient needed to meet 2 of the following 3 criteria: role physical < 50, social functioning < 62.5, or vitality < 35. To meet the post-exertional malaise criteria, a patient would need to have a frequency and severity >=2 on at least one of five post-exertional malaise items.

Their PEM "items" are

Post-exertional malaise
 Dead, heavy feeling after starting to exercise
 Next-day soreness after non-strenuous activities
 Mentally tired after slightest effort
 Minimum exercise makes you tired
 Physically drained / sick after mild activity

I appreciate that itemising PEM is difficult, but they've made an awful attempt with that list, in my opinion, and subsequently it's no surprise that they think that SEID is as bad as Fukuda - I think they are wrong, and setting the bar so low seems very strange to me.

 

[Invisible Woman]

core IOM symptoms are common in other illnesses such as cancer, Hashimoto, lupus,chronic heart failure, and multiple sclerosis

Bolding mine.

I was diagnosed with Hashimoto's many years before developing ME. As a sufferer of both I don't believe the two feel the same, to me at any rate.

I think this is where the experienced patient counts - my own personal view. In the beginning I fought against ME and waged constant small wars to try to push boundaries. I could hear my body complain but refused to listen closely to what it was saying. I didn't want to "focus on the negatives" as the BPSers might say. It's only when I understood this wasn't going away and I had to be a lot smarter that I started to pay proper attention.

I can understand why people think Hashimoto's when undertreated or untreated feels the same. It's not though. Purely from my own personal experience -

Some Similarities between Hashimotos and PEM

• Feeling cold - but in Hashimoto's it cold nearly all the time.
• Achiness - in Hashimoto's it's a more general, background ache. You get this in PEM but in Hashimoto's I didn't het the excruciating muscle specific pain.
• Tiredness - it's true and profound exhaustion in Hashimoto's - I'll fall asleep anywhere. PEM feels more like you have been poisoned.
• Forgetfulness - both
• Cognitive issues - in PEM there's sometimes a blank space or invisible wall or a fog between you and the thought or process you're trying to access. In Hashimoto's your just too darn tired.

Some differences between the two for me

• Sore throat - this is more likely to happen with PEM for me.
• Voice starts sounding gravelly - Hashimoto
• Skin becomes very dry (even though bathing products and routines haven't changed) Hashimoto's.
• Weakness - especially the sudden, unexpected kind - PEM
• Loss of coordination and fine control - walking into things, knocking things over- PEM
• Swollen ankles - I get this with Hshimoto's but not PEM. OI can cause it too.
• Low mood - Hashimoto's.
• P***ed off - PEM

I could probably bore you with a similar list for anaemia.

I also note that people I know who have ME & who have also experienced cancer & cancer treatment will tell you that PEM, or an ME relapse, is much worse. In fact one person who survived rectal cancer told me she was so scared of an ME relapse and the effect treatment would have on her ME she considered not having treatment. Once out the other side, she reckoned the hardest thing to cope with was how it affected her ME, not the treatment itself.

There can be quite a big difference between similar and the same.

 

[Invisible Woman]

Post-exertional malaise
 Dead, heavy feeling after starting to exercise
 Next-day soreness after non-strenuous activities
 Mentally tired after slightest effort
 Minimum exercise makes you tired
 Physically drained / sick after mild activity

Might as well have just said "tired and a bit sore" and been done. Awful. This is a huge step back.

 

[Mij]

I appreciate that itemising PEM is difficult, but they've made an awful attempt with that list, in my opinion, and subsequently it's no surprise that they think that SEID is as bad as Fukuda - I think they are wrong, and setting the bar so low seems very strange to me.

The thing is that Jason claims to have had CFS post EBV in 1990. This describes HIS experience. This is not the PEM that I experience, not even close.