Updates on status of ICD-11 and changes to other classification and terminology systems

"Next steps for countries

"The adoption of ICD-11 in May 2019 marked the start of an implementation phase by WHO member states. In 2022, reporting of mortality data according to ICD 11-MMS will commence, with a transition period of at least five years. The nature, timing, and complexity of implementation for morbidity coding will vary from place to place. In general, the transition will be most complex in places in which ICD is used widely, in multiple systems, and underlies processes such as activity-based funding.

"The countries that are first to adopt ICD-11-MMS may well be some that do not have substantial legacy systems that will require alteration to accommodate the revision."​

 

There has been relatively little debate about what seems an important issue. So here's my go at it.

I have been able to look at this relatively briefly but it looks like the new proposal would improve things. I think it would be clearer to healthcare professionals that the appropriate code for what US agencies such as the CDC and NAM call "ME/CFS" is 93.32. The info that these agencies have published about "ME/CFS" is relatively ok so this could be something to work with.

I don't quite understand the comments that reject it and prefer the status quo. I have a hard time seeing the benefits of the current situation where Myalgic Encephalomyelitis is placed only as a synonym under the same code (G93.3) as "post-viral fatigue syndrome".

Postviral fatigue syndrome isn't a well-defined term. I don't know any diagnostic criteria for it and almost never see it used in scientific research. There was this study on insurance data that used this code (G93.3) and from the age structure, we could see it is very unlikely that this group captures ME patients accurately. The peak age of incidence was 80-89 suggesting that doctors use this code for old people who have problems recovering from a viral infection. See: https://www.s4me.info/threads/estim...aldez-proskauer-et-al.7279/page-2#post-134989

I don't understand why one could oppose having CFS added when the current G93.3 seems much broader and less clearly defined. In the proposal, ME/CFS would get its own subcode 93.32 while currently ME is only mentioned under postviral fatigue syndrome which is a bit absurd because a viral onset is not a requirement in most ME case definitions.

Apologies in advance if I misunderstood something.

@Colleen Steckel

 

@Michiel Tack I do hope you've read my feedback submission:

Feedback on proposals for Postviral and Related Fatigue Syndromes, Suzy Chapman:

https://dxrevisionwatch.files.wordp...n-postviral-and-related-fatigue-syndromes.pdf


Please note that although I reject both sets of proposals, I am not one of the commenters who would prefer the status quo.

I would prefer to see the following structure (and with no SEID term added to the Index).

Three separate sub-codes for PVFS; ME; and CFS had been proposed by NCHS in 2018:


G93.3 Postviral and related fatigue syndromes (though I am not happy about the inclusion of the words "fatigue" and "syndrome" in the name of this proposed revision to the G93.3 class)

G93.31 Postviral fatigue syndrome​

G93.32 Myalgic encephalomyelitis [ME]​

G93.33 Chronic fatigue syndrome [CFS]

Chronic fatigue syndrome NOS*​

G93.39 Other post infection and related fatigue syndromes (though I am not happy about the inclusion of the words "fatigue" and "syndrome" in the name of this proposed sub-code)

*NOS = “Not otherwise specified” This abbreviation is the equivalent of unspecified.


My feedback submission sets out why I don't consider either set of proposals is fit for purpose.

 

Yes I did, thanks but many arguments were about that the proposal might not fit with ICD conventions. If I understand correctly, that might lead to problems for the proposal to be adopted but doesn't say much about whether the proposal, if adopted, would be good or bad. It's the latter that I'm more interested in discussing.

That seems reasonable as well but it's hard to find agreement in the ME/CFS community and the proposal on the table is supported by major ME/CFS organizations and looks like a significant improvement from the current situation.

 

Please note also that in ICD,

"Inclusion terms

Within the three- and four-character rubrics,1 a number of other diagnostic terms are usually listed. These are known as 'inclusion terms' and are given, in addition to the title, as examples of the diagnostic statements to be classified to that rubric. They may refer to different conditions or be synonyms. They are not a subclassification of the rubric.

Inclusion terms are listed primarily as a guide to the content of the rubrics. Many of the items listed relate to important or common terms belonging to the rubric. Others are borderline conditions or sites listed to distinguish the boundary between one subcategory and another. The lists of inclusion terms are by no means exhaustive and alternative names of diagnostic entities are included in the Alphabetical index, which should be referred to first when coding a given diagnostic statement."

 

If a proposal doesn't meet taxonomic principles for ICD; would result in problems for data analysis; would result in problems with potential loss of legacy data going forwards and problems for those already coded with R53.82, then whether it would be "good or bad" is immaterial.

The fact that 7 US organisations have supported the first of the two sets of proposals does not necessarily make it a valid or good proposal.

I don't think you have actually said which of the two proposals you support, ie the 7 org proposal or the revised proposal by NCHS, as set out in the agenda Topic Packet. The 7 orgs are asking for support for their own proposal.

The orgs' proposal is in any case incomplete since it omits to make recommendations for the existing R53.82 code or discuss what would happen to patients with historical diagnoses of R53.82.

(The orgs have not requested that R53.82 is deleted. Nor have they proposed that CFS, or CFS and CFS NOS should be added under a proposed new sub-code under a revised G93.3 class.)

There are two different proposals on the table. Which of the two sets of proposals are you supportive of?

 

For clarification:


The 7 organisations' proposal:

Org Option 1 proposes revising the existing code title: G93.3 Postviral fatigue syndrome to G93.3 Postviral and related fatigue syndromes and to create separate sub-codes for Postviral fatigue syndrome and Myalgic encephalomyelitis, with Myalgic encephalomyelitis/chronic fatigue syndrome and ME/CFS as inclusions under new sub-code G93.3x Myalgic encephalomyelitis [2]:

G93.3 Postviral and related fatigue syndromes

G93.3x Postviral fatigue syndrome

G93.3x Myalgic encephalomyelitis
Myalgic encephalomyelitis/chronic fatigue syndrome
ME/CFS

‘[Org Option 1] did not make recommendations regarding the term “chronic fatigue syndrome” because of the lack of consensus on earlier proposals on how this should be addressed.’

Nor does Org Option 1 make recommendations for code R53.82 Chronic fatigue syndrome NOS.


The NCHS alternative proposal, as set out in the agenda Topic Packet:

NCHS Option 2 proposes revising the existing code title: G93.3 Postviral fatigue syndrome to G93.3 Postviral and related fatigue syndromes and to create separate sub-codes for Postviral fatigue syndrome and new code title, “Myalgic encephalomyelitis/chronic fatigue syndrome”.

Chronic fatigue syndrome; Myalgic encephalomyelitis; and conjoined acronym, ME/CFS are proposed to be listed as inclusions under the new G93.32 title code (see also Fig 3):

G93.3 Postviral and related fatigue syndromes

G93.31 Postviral fatigue syndrome

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome
Chronic fatigue syndrome
ME/CFS
Myalgic encephalomyelitis

Systemic exertion intolerance disease [SEID] is proposed to be indexed to the G93.32 code title.

The existing inclusion term: Chronic fatigue syndrome NOS under R53.82 Chronic fatigue, unspecified is proposed to be deleted from the Tabular List.

 

Both seem like an improvement over the current situation.

 

You are supportive then of:

the (unadopted) term, SEID being added to the US CM index;

adding a composite term (which does not exist in any edition of the WHO's ICD) formed from two existing discrete ICD entities as a new title code;

adding all these terms under that proposed new G93.3 2 sub-code title:

Myalgic encephalomyelitis/chronic fatigue syndrome
Chronic fatigue syndrome
ME/CFS
Myalgic encephalomyelitis​

with Systemic exertion intolerance disease [SEID] proposed to be indexed to the same G93.32 code title;​

and removing "Chronic fatigue syndrome NOS" from under R52.82.

 

Since neither the 7 orgs nor the NCHS has discussed how patients already assigned the code R53.82 would be accounted for going forward in the context of their respective proposal sets, I don't see that either set can be considered fit for purpose, as stakeholders have insufficient information on which to base their feedback.

Which is why I have said at the end of my feedback:

Queries relating to anomalies and ICD conventions which were not identified or discussed following the presentations but have significance for the safety and utility of both sets of proposals require clarification, in addition to questions around unintended consequences.

These as yet unaddressed concerns may present obstacles to constructive stakeholder feedback. My recommendation would be to consider these issues and public comment and revise and re-present proposals at the March 2022 meeting.

In the meantime, revision of the term, “Benign myalgic encephalomyelitis” to “Myalgic encephalomyelitis” (approved by URC in September 2016) should be implemented for the next release of ICD-10-CM, to bring the US modification in line with the final update in the life of the WHO’s International edition of ICD-10, and in line with ICD-11.​

 

Note that IACFS/ME and the six orgs that have supported the org proposal did not put their proposal out for public consultation before submitting it to NCHS/CDC, last December, for consideration for adding to the final agenda for presentation and discussion at the September C & M Committee meeting.

SOLVE M.E. held a webinar and listening session in early September to discuss the proposal "and hear any concerns and answer questions" - at which point, the proposal had already been submitted. As far as I know, nothing has been reported publicly by SOLVE M.E. about this webinar and what concerns and questions had been raised.

It wasn't clear until the full September 14-15 agenda was published, shortly before the NCHS/CDC C & M Committee meeting, that NCHS would be presenting a second, alternative proposal set* at the September meeting.


*Screenshot 1 NCHS proposal for changes to Tabular List: https://dxrevisionwatch.files.wordpress.com/2021/09/cm75.png

Screenshot 2 NCHS proposal for Index Modifications: https://dxrevisionwatch.files.wordpress.com/2021/09/cm752.png

 

I think Dx Revision watch has covered a lot of what I would have said.

Key points

1. You are right there has been very little debate on this as this was not made public until right before the meeting. Repeated requests over the years to deal with the ICD codes has too often left out views of various groups of patients. We as a community really need to adhere to our own motto of "nothing about us without us." I certainly feel left out of this and it affects me directly as someone who has G93.3 as my diagnosis and have been able to receive testing covered by my insurance not otherwise available (brain SPECT scan) using that code.

2. What was proposed and what the NCHS put together are not the same thing. (See Dx Rev Watch explanation)

3. CFS in the US refers back to the Fukuda definition that does NOT require PEM.

4. By lumping all patient groups, CFS, ME/CFS, & ME under one code we need to discuss unintended consequences. Many of us can use our own experience to understand how CFS in the US is viewed and how doctors have been trained to treat it. That is a status quo I do not want continued.

5. And in that same context those who have been given the CFS code need to be recognized as well. As noted in Dx Revision Watch info, that was not addressed. This really comes down to education of medical professionals on how to distinguish between CFS (no PEM required) and those who have PEM as well as immune, neurological & cardiac issues. That is the purpose of the ME-ICC & the ME-IC Primer.

I am sure we would all agree that an unintended consequence of lumping CFS in with ME leading to all patients under this new code being viewed as not requiring PEM (aka can exercise safely) would be a disastrous result.

We tried to explain our concerns here (If you read it and it didn't answer your questions, please let me know. We have a few more days to clarify and submit additional info.): https://www.me-international.org/uploads/1/2/7/6/127602984/mei_icd-10-cm_response_211105.pdf

NOTE: ME International is all volunteer and most of us are quite sick so this was a herculean endeavor on short notice.

We can & should work as a community to make sure all voices are heard and concerns raised addressed. Jumping into something as important as changing ICD codes should not be taken lightly.

 

I find all this very confusing and I don't have any strong opinion on how to classify.
What I would say is that I do not see any place for the term CFS unless it is used for ME. People who do not have PEM, in simple terms, do not have a 'syndrome'. They may have chronic fatigue of unknown cause which might be classified as CF (NOS) but there is no other syndrome that deserves a category.

So the system that makes sense to me is the one the puts ME, CFS and ME/CFS under one code.

 

In the WHO's International ICD-10 edition both ME and CFS are coded to one code and that has been the case since ICD-10 was published in 1992.

So this is an issue exclusive to the US clinical modification for morbidity use (an adaptation of which is also used in Spain).

The US is the only member state that has located CFS (as Chronic fatigue syndrome NOS) under the R codes, in the Symptoms, signs chapter. As the draft of ICD-10-CM had stood in 2001, the intention had been to follow the WHO's coding structure and code both CFS and BME to G93.3 Postviral fatigue syndrome. This intention was changed in 2004 by Dr Bill Reeves.

Each ICD entity (an entity may be a three character category title, a code title, an inclusion term, or a synonym or index term) is a unique word or phrase.

ICD does not take existing ICD entities and conjoin them to form hybrid terms.

Additionally, retiring a code altogether from an ICD edition presents problems for data aggregation, disaggregation and for loss of legacy data going forward, and ICD's primary purpose is data collection, analysis and comparability of statistics on causes of mortality and morbidity between places and over time.

See: Feedback on proposals for Postviral and Related Fatigue Syndromes, Suzy Chapman:
https://dxrevisionwatch.files.wordp...n-postviral-and-related-fatigue-syndromes.pdf

[Edited for clarity]

 

This is very confusing. I concur that CFS when it is synonymous with ME would make sense for them to have the same code. But, in the US the CFS-Fukuda definition diverted the US medical community AWAY from ME giving us a distinct different description that is not ME. That led to CFS & ME having completely different coding.

The CDC's toolkit was based on the CFS-Fukuda definition and pushed GET & CBT. There is no official recognition of ME and education of Drs about ME has fallen to the patients & charities. As of 2015 most charities switched from educating about ME/CFS-CCC to educating about ME-IOM. That criteria leaves out a lot of what is specific to ME. NICE followed suit and used the IOM as a guide. Hence what so many have now recognized as a "watered down" description of ME.

I think this is why there is so much consternation in the community over how "CFS" & "ME" are viewed. When using labels we really need to know what criteria they are connected to.

The labels that go to which criteria can be seen on the OMF website here: https://www.omf.ngo/history-mecfs/

In essence:
CFS has two meanings - Holmes & Fukuda
ME/CFS has two meanings - Canadian Consensus Criteria (CCC) & IOM Report (aka SEID)
ME is the International Consensus Criteria (ICC)

There isn't any clarification as to whether the "ME/CFS" refers to the CCC or the IOM.

I do not think ME is synonymous with CFS or ME/CFS-IOM.

 

I can see that but it makes no sense. There is no other syndrome as far as I am aware. People with chronic fatigue of unknown cause that do not have ME have no particular reason to be put in a syndrome.

I think medical categories should be based on what makes sense rather than what has been fashionable with physicians for a while.

 

But perhaps one could also argue that ME described by Ramsay or the later London criteria are not the same as ME defined by the ICC. And CFS defined by Holmes et al. is very different from that of Sharpe et al. The differences between case definitions within a label, whether CFS or ME, can sometimes be larger than those between them.

 

In the US's ICD-10-CM, "People with chronic fatigue of unknown cause" can be diagnosed with R53.82 Chronic fatigue, unspecified.

One of the problems with the US's ICD-10-CM is that "Chronic fatigue syndrome" is currently an inclusion under R53.82 Chronic fatigue, unspecified, so it takes the same code.

SNOMED CT International Edition and the UK Edition have "Chronic fatigue syndrome" as the lead Concept term, with BME and ME under the CFS Synonyms list. But all terms under this Concept code are cross-mapped to ICD-10's G93.3. (It is SNOMED CT that is mandatory for use in primary care in NHS England.)

In the US extension of SNOMED CT, CFS is cross-mapped to a choice of ICD-10-CM codes, to G93.3 or to R53.82, reflecting the variation in the coding of these terms for the US's ICD-10 clinical modification compared with how the terms are coded by the WHO.


In March 2017, a joint proposal submitted by me and Mary Dimmock called for separate codes for ME and CFS, and to also separate ME from PVFS. The WHO would not accept this change to the coding structure. For ICD-11, both ME and CFS are specified as inclusions under 8E49 Postviral fatigue syndrome. (Note that ICD inclusion terms may or may not be synonyms to the ICD title to which they are coded.)

As I've said in previous posts, retiring an existing term entirely from ICD presents problems for legacy data and for comparability with data reported using various editions of ICD.

Some low resource countries, and also Italy, are still using the WHO's ICD-9 edition for reporting of data.

From January 01, 2022, the WHO will start collecting data from member states that has been recorded using the new ICD-11 codes, the International edition of ICD-10 (which may be Version: 2019, Version: 2016 or earlier releases, and in some countries, ICD-9) and also from the various clinical modifications including, ICD-10-CM (US), ICD-10-CA (Canada), ICD-10-GM (Germany), ICD-10-AM (Australia). All these editions have variances in their content and codes but the data they generate has to be collated for comparison.

So terms that are being retired or deprecated need to be retained somewhere in the classification in order that legacy data can be carried forward for analysis or they need to be mapped (or cross-walked) to an existing code or to a newly created code, otherwise legacy data is lost.

Neither the orgs' proposal or the alternative proposal presented by NCHS has set out how the existing "Chronic fatigue syndrome NOS" term would be mapped in future, if the term were to be deleted from ICD-10-CM or whether patients with an existing diagnosis of R53.82 would be assigned a new code by their providers.

Nor was the potential for unintended consequences discussed at the meeting and how that might be mitigated. I believe that if CDC's Donna Pickett had been presenting, these issues would have been acknowledged and clarified at the meeting; but the presentation was overseen, on this occasion, by NCHS's Traci Ramirez who has not been involved in previous submissions (in 2011, 2012, and 2018).

These issues, the inconsistencies I have identified in the NCHS proposal and the lack of adherence to ICD conventions need public discussion at a future meeting before any decision is made by the NCHS Director.

At the moment, neither proposal is fit for purpose, irrespective of the terms, per se, and the changes being proposed to the terms, and no decisions should be made on either proposal, as they currently stand.

[Edited for clarity]

 

@Colleen Steckel , as we have discussed before, here in the US, essentially no one was being diagnosed with ME before the creation of the diagnosis of CFS in the late 1980’s. The various definitions, rather than delineating different diseases represent a variety of efforts to diagnose what Dr Bell has called “the disease of 1000 names”. None of the definitions are sufficiently scientifically sound. (I know that you feel that ME-ICC is, but many people disagree.)

We need better criteria that are scientifically based. Many of us have called for years for NIH to fund a colloquium of experienced clinicians and researchers to come up with better criteria.

In the meanwhile, we have an ever increasing number of people coming under the ME/CFS umbrella who are not being coded and counted. Primary care physicians need a straightforward way to code the patients that they see. We need better statistics indicating how many of us there are and what our disease progression looks like.

Additionally, as an African American, I know that the physicians who treat our community - excellent but overextended medical professionals - need a straightforward and logical way to code their patients. Those patients need an inclusive community where they can learn about their disease. Coding will be a big step forward in this effort. As you know, in the US, community-based studies have shown that African Americans are at least as likely as the larger community to have this disease. My guess would be that you can’t name 20. This is a problem. Straightforward coding will help in bringing members of underserved communities out of the shadows to be correctly counted.

We need a serious effort to straighten out the issues around definitions. In my opinion, any definition that does not focus on PEM is useless. But those problems should not stand in the way of coding and counting those who live with our disease. This is a step forward that is badly needed.

 

ICD-10-CM provides no definition texts or instruction notes for clinicians/coders within the classification and neither does the WHO's ICD-10.*

NCHS proposes to code all these terms under a new sub-code term:

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome

with these inclusion terms:

Chronic fatigue syndrome
**ME/CFS
Myalgic encephalomyelitis

and with Systemic exertion intolerance disease [SEID] also indexed to the proposed new G93.32 sub-code title.

(The existing inclusion term, Chronic fatigue syndrome NOS under R53.82 Chronic fatigue, unspecified is proposed to be deleted from the Tabular List but no cross-walk to an alternative code for historical R53.82 diagnoses has been discussed.)


**Acronyms listed as inclusion terms don't meet the ICD-10-CM convention for handling of acronyms. An acronym should appear in square brackets at the end of the fully specified term, eg as "SEID" does, above.

So the proposal should have read:

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome [ME/CFS]

Chronic fatigue syndrome
Myalgic encephalomyelitis​

Systemic exertion intolerance disease [SEID] indexed to the proposed new G93.32 sub-code title.


But ICD cannot code for conjoined entities. So both proposals have requested for changes that don't conform to WHO ICD conventions, which is why you don't see "Myalgic encephalomyelitis/chronic fatigue syndrome" in ICD-10, ICD-11 or in SNOMED CT, because each ICD entity or SNOMED CT Concept term is a unique, machine readable word or phrase.


*ICD-11 does have the capacity for inclusion of disorder and disease description texts for all ICD-11 MMS terms and in some cases, criteria are included for diseases for which there exist international consensus criteria.

ICD-11 could have included description texts for all three of the ICD-10 legacy terms but has elected not to. WHO/ICD-11 has said: "...There is no agreement on a reliable diagnostic symptom pattern. The etiology is still being discussed and there is no uniform treatment approach with reliable outcomes." So the 8E49 Description field remains unpopulated.

[Expanded for clarity]