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

I am also surprised that a submission to the NCHS/CDC ICD-10-CM C & M Committee supported by 7 US organisations was not put out first for public review and consultation, and that #MEAction has signed up to this submission.

If the submission (in whatever form) is accepted for inclusion on the agenda for discussion at the September meeting of the C & M Committee there will be a public comment period:

"October 15, 2021 Deadline for receipt of public comments on proposed new codes discussed at the September 14-15, 2021 ICD-10 Coordination and Maintenance Committee Meeting being considered for implementation on April 1, 2022."



The "Tentative Agenda" is already posted: https://www.cdc.gov/nchs/data/icd/Tentative-Agenda-September-2021.pdf

This proposed discussion topic has not been included in the Tentative Agenda, however:

"This updated list of tentative agenda of topics is not final. The final topics material will be available electronically from the NCHS and CMS web sites prior to the meeting..."

The final agenda (called the "Diagnosis Codes Topic Packet") is usually posted one or two days prior to Meeting Day One.

 

Just to update that today I had cause to check the NHS Digital SNOMED CT request archive and noted that the request for addition of the term "Systemic exertion intolerance disease" under SNOMED CT Concept: 52702003 Chronic fatigue syndrome, which was processed by SNOMED International and rejected, has now been marked as "Closed - Declined" in the NHS Digital requests archive:

https://isd.hscic.gov.uk/rsp-snomed/user/guest/request/view.jsf?request_id=30104


Request 30104

Type
Add description

Status
Closed - Declined

Concept identifier
52702003

 

The last time the US's coding structure for G93.3 and R53.82 was discussed at an NCHS/CDC ICD-10-CM Coordination and Maintenance Committee Meeting was at the public meeting held on 11th and 12th September 2018.

At this meeting, two proposal options had been presented by Donna Pickett (CDC), following submission of a proposal by Lily Chu, on behalf of the IACFS/ME. Dr Chu participated in discussion of these proposals via phone link.

 

A recap of ICD guidelines and conventions for inclusion terms and the use of acronyms in ICD-10; ICD-10-CM; and ICD-11:


https://icd.who.int/browse10/Content/statichtml/ICD10Volume2_en_2019.pdf

ICD-10 Volume 2 Instruction manual Fifth edition 2016

(...)

Page 13

A statistical classification of diseases must be confined to a limited number of mutually exclusive categories that are able to encompass the whole range of morbid conditions. The categories have to be chosen to facilitate the statistical study of disease phenomena. A specific disease entity that is of particular public health importance, or that occurs frequently, should have its own category. Otherwise, categories will be assigned to groups of separate but related conditions. Every disease or morbid condition must have a well-defined place in the list of categories. Consequently, throughout the classification, there will be residual categories for other and miscellaneous conditions that cannot be allocated to the more specific categories.

(...)

Page 27

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.

-----------------------------------------------


https://ftp.cdc.gov/pub/Health_Stat...idelines-FY2022-7-2022-7-15-21-update-508.pdf

ICD-10-CM Official Guidelines for Coding and Reporting FY 2022 (October 1, 2021 - September 30, 2022)

Page 9

Inclusion terms

List of terms is included under some codes. These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.

-----------------------------------------------

ICD-11 Content Model 2011

https://dxrevisionwatch.files.wordp...20model20reference20guide20january2020111.doc

Page 59

Use of acronyms

An acronym is an abbreviation formed from the first letters of other words and pronounced as a word (e.g. NASA). Acronyms may never be used for titles of categories. They should be added as synonyms to the appropriate spelt out disease entity thus facilitating identification of the relevant cases and categories.

Page 59

Retired Entities

If for any reason an ICD entity is retired, this will be indicated accordingly and the term will be included in the inclusion terms and indexes as appropriate, allowing uniform assignment of cases that bear the label of the retired entity.

More extracts from ICD-11 Reference Guide concerning conventions for Inclusions in quote box:

How are Acronyms handled in ICD-11?

For ICD-11, the legacy entities, "myalgic encephalomyelitis" and "chronic fatigue syndrome" are both specified as the Inclusion terms under 8E49 Postviral fatigue syndrome in the Mortality and Morbidity Statistics (ICD-11 MMS) linearization (which is ICD-11's equivalent to the Tabular List).

8E49 Postviral fatigue syndrome:

https://icd.who.int/browse11/l-m/en#/http://id.who.int/icd/entity/569175314


For ICD-11, the following 11 terms, including a number of historical terms, have been retained under the All Index Terms list:

Postviral fatigue syndrome
Benign myalgic encephalomyelitis
akureyri
akureyri disease
epidemic neuromyasthenia
myalgic encephalomyelitis
myalgic encephalomyelitis syndrome
chronic fatigue syndrome
neuromyasthenia
Iceland disease
Icelandic disease


Also included under All Index Terms for code 8E49 are three acronyms, with the disease entity term spelt out in full within square brackets. Note this is a standard format for listing acronyms in ICD-11, for example: "COPD - [chronic obstructive pulmonary disease]" and "SIDS - [sudden infant death syndrome]".

https://icd.who.int/dev11/f/en#/http://id.who.int/icd/entity/133207228
https://icd.who.int/dev11/l-m/en#/http://id.who.int/icd/entity/569175314

CFS - [chronic fatigue syndrome]
ME - [myalgic encephalomyelitis]
PVFS - [postviral fatigue syndrome]



NB: The conjoined terms: "myalgic encephalomyelitis/chronic fatigue syndrome" and the conjoined acronyms: "ME/CFS" being proposed by SOLVE M.E.* to be added to the ICD-10-CM Tabular List are not included in the WHO's International version of ICD-10 (Version: 2019), either as specified Inclusion terms or listed under Synonyms/Index Terms.

For the WHO's forthcoming ICD-11, the terms "myalgic encephalomyelitis" and "chronic fatigue syndrome" continue to be listed as separate entities.

*with the support of IACFS/ME, #MEAction, Open Medicine Foundation, Massachusetts ME/CFS & FM Association, the Minnesota ME/CFS Alliance, and Pandora Org.

 

Responding to the point made about the request to add ME/CFS to the ICD-10-CM

The term ME/CFS, as used in the Canadian Consensus Criteria, the IOM criteria, and the IOM report is not just a joining of two existing discrete ICD entity terms. Both of these criteria require PEM, not required by Oxford or Fukuda CFS criteria. I would be surprised if WHO ICD-10's taxonomic principles and conventions would disallow the definition and establishment of a term that is clearly not just the conjoining of separate and discrete entities.

This term ME/CFS has been adopted by the federal government, by numerous clinical guidance providers and by the US ME/CFS Clinician Coalition. When the term is used by US doctors in medical records, NCHS has said they need to provide a way to code it.

NICE's draft guidance has also adopted the term ME/CFS. If that is ultimately adopted, I'd expect this could come up in the UK as well.

 

The previous NICE guideline (Clinical guideline [CG53] Published: 22 August 2007) used the term "Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy)" and the acronym "CFS/ME".

The conjoining of these two terms for the NICE guideline caused no difficulties with coding using ICD-10 codes, since either term, "chronic fatigue syndrome" or "myalgic encephalomyelitis" is coded to G93.3 in the WHO ICD-10 International version - as you know.

Additionally, WHO is silent on how any of the terms: Postviral fatigue syndrome; [Benign] myalgic encephalomyelitis; and Chronic fatigue syndrome are defined for ICD-10, or how they are differentiated.

The WHO's ICD-10 (Version: 2019), which was released in January 2020, is the final update in the life of ICD-10.

There will be no further updating of ICD-10 other than addition of new viruses or correction of errors. Otherwise, the content of the International editions of ICD-10 (ICD-10: Version: 2016, which is currently mandated for use in NHS England) and the final update (ICD-10 Version: 2019) are frozen.

As for ICD-11, as you know, that has inherited the ICD-10 convention of mutually exclusive terms and ICD-11 would not be able to accommodate, for example:

8E49 Postviral fatigue syndrome

Inclusions
chronic fatigue syndrome
myalgic encephalomyelitis
myalgic encephalomyelitis/chronic fatigue syndrome

All Index Terms

• Postviral fatigue syndrome
• Benign myalgic encephalomyelitis
• akureyri
• akureyri disease
• CFS - [chronic fatigue syndrome]
• epidemic neuromyasthenia
• myalgic encephalomyelitis
• myalgic encephalomyelitis syndrome
• chronic fatigue syndrome
• ME - [myalgic encephalomyelitis]
• ME/CFS [myalgic encephalomyelitis/chronic fatigue syndrome]
• PVFS - [postviral fatigue syndrome]
• neuromyasthenia
• Iceland disease
• Icelandic disease

or

8E49 Postviral fatigue syndrome

Inclusions
myalgic encephalomyelitis
myalgic encephalomyelitis/chronic fatigue syndrome


All Index Terms

• Postviral fatigue syndrome
• Benign myalgic encephalomyelitis
• akureyri
• akureyri disease
• CFS - [chronic fatigue syndrome]
• epidemic neuromyasthenia
• myalgic encephalomyelitis
• myalgic encephalomyelitis syndrome
• chronic fatigue syndrome
• ME - [myalgic encephalomyelitis]
• ME/CFS [myalgic encephalomyelitis/chronic fatigue syndrome]
• PVFS - [postviral fatigue syndrome]
• neuromyasthenia
• Iceland disease
• Icelandic disease

In NHS England, SNOMED CT UK Edition is the mandatory terminology system for use in primary care settings, at the point of care. SNOMED CT is in the process of being implemented across all NHS England secondary care settings, including mental health and dentistry. SNOMED CT also uses the convention of unique disorder terms.

 

ICD-10 may be frozen but ICD-10-CM, used in the US, is not frozen. Changes are still being made to it.

Regarding the term ME/CFS - yes, WHO does not provide criteria for ME or CFS. But as used in the Canadian Consensus criteria and the IOM criteria, the term ME/CFS is clearly not simply a conjoining of unique diseases - CFS and ME. And NICE 2020 is also not using ME/CFS as a simple conjoining of all CFS and all ME cases

 

I am fully aware of that. My response specifically addressed your comment:

"When the term is used by US doctors in medical records, NCHS has said they need to provide a way to code it. NICE's draft guidance has also adopted the term ME/CFS. If that is ultimately adopted, I'd expect this could come up in the UK as well."

and my response quite clearly refers to the WHO ICD-10 International version and the final update in the life of ICD-10 - not to the US's ICD-10-CM clinical modification, for which annual changes and updating are the responsibility of NCHS/CDC.

I have not said nor implied conjoining of "unique diseases".

In the context of ICD coding and conventions, what I am referring to is the conjoining of hitherto mutually exclusive terms.


The WHO does not give a stuff about the various ME, CFS criteria.

"...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...The Medical and Scientific Advisory Committee and the Classification and Statistics Advisory committee supported this decision. Team 2 WHO 2019-Mar-12 - 07:44 UTC"

"The purpose of the ICD is to permit systematic recording, analysis, interpretation and comparison of mortality and morbidity data collected in different countries or areas and at different times. ICD is used to translate diagnoses of diseases and other health problems from words into an alphanumeric code, which permits easy storage, retrieval and analysis of the data." [1]

What WHO is concerned about is data collection, data aggregation, disaggregation and the potential for loss of legacy data going forward when changes are proposed.


I note that although the submission by SOLVE ME/CFS and six other US orgs is said to have been submitted to the ICD-10-CM Coordination and Maintenance Committee last December, discussion of SOLVE ME/CFS's proposals for the restructuring of G93.3 Postviral fatigue syndrome and Benign myalgic encephalomyelitis [which will inevitably require discussion of the handling of the existing ICD-10-CM R53.82 term: Chronic fatigue syndrome NOS, on which SOLVE ME/CFS is silent] has not been included in the Tentative Agenda for the upcoming September 14-15 meeting.

It is to be hoped that if Donna Pickett (CDC) does decide to add this proposal to the full agenda (usually released one day before meeting Day One), that it is included in the Topic Packet along with the proposal's rationale and any alternative proposals presented by Ms Pickett, for ease of public scrutiny, and not shoehorned into the agenda at the 11th hour, as happened at the September 2018 meeting [2].


1. International statistical classification of diseases and related health problems. 10th revision, Fifth edition, 2016. Volume 2: Instruction manual

2. Pages 11-12, ICD-10 Coordination and Maintenance Committee Meeting September 11-12, 2018 Diagnosis Agenda Part 2: https://www.cdc.gov/nchs/data/icd/Topic_packet_Sept_2018_part2.pdf

 

My bolding added to the above


As WHO notes above, ICD codes are codes for diseases (and other health problems). The terms in the ICD are the names of those diseases, not just meaningless terms with no relation to a disease. So WHO must take account what diseases these terms refer to.

While WHO may not care about different criteria and may not have different codes for different criteria of the same disease, how these diseases are described in a general sense is highly relevant to what terms are added and what gets coded in the ICD. Otherwise, ICD would not be able to fill the role described above.

In the case of ME/CFS, that term has been used to describe a disease that is clearly not just a simple conjoining of the diseases labelled with the terms ME and CFS

 

The next meeting of the NCHS/CDC ICD-10-CM Coordination and Maintenance Committee takes place on Tuesday and Wednesday this coming week (14-15 September):

https://www.cdc.gov/nchs/icd/icd10cm_maintenance.htm

The Final Agenda (Diagnosis Codes Topic Packet) is now available:
https://www.cdc.gov/nchs/icd/Sept2021-TopicPacket.pdf


NB: Proposals for changes to the US's ICD-10-CM Tabular List and Index were submitted by SOLVE ME, supported by six US organisations, including IACFS/ME and #MEAction.

The proposals, as set out in the Final Agenda, have been modified by NCHS and are not the same as the proposals submitted last December by SOLVE ME et al.

These proposals relate only to the US's ICD-10-CM, which was developed by NCHS/CDC, and is managed, revised and updated by NCHS/CDC - not by the WHO.

These proposals do not relate to the WHO's International edition of ICD-10; to ICD-11 or to any of the various national modifications of ICD-10.

The proposals are scheduled for discussion at the NCHS/CDC C & M public meeting, this coming week (Tuesday 14 and Wednesday 15 September), which will be held virtually. CDC may potentially present alternative proposals at the meeting which have not been included in the Final Agenda (Topic Packet).

No decisions on proposals are made at the meeting, itself. Following the meeting, there will be a public review and feedback period during which comment, objections etc can be submitted to NCHS/CDC.

The Final Agenda (Diagnosis Codes Topic Packet) is here:
https://www.cdc.gov/nchs/icd/Sept2021-TopicPacket.pdf


The agenda topic:

Postviral and Related Fatigue Syndromes
Traci Ramirez (Ed: CDC)
Mary Dimmock

is listed on Page 169 of what looks to be a very full agenda. This will be a virtual meeting and presentation and discussion of the diagnosis code proposals may run on into Day Two.


Extract Page 169: https://www.cdc.gov/nchs/icd/Sept2021-TopicPacket.pdf

Rationale for proposal submitted by SOLVE ME:

Postviral and Related Fatigue Syndromes

In 2015, the Institute of Medicine (IOM), now called the National Academy of Medicine, published an extensive evidence review of “myalgic encephalomyelitis/chronic fatigue syndrome” (ME/CFS) and recommended new diagnostic criteria. The CDC, disease experts, and many clinical guidance and medical education providers have already adopted the IOM criteria along with the term “myalgic encephalomyelitis/chronic fatigue syndrome.” Postviral Fatigue Syndrome proposals were presented at the September 2011 and September 2018 C&M meetings; the comments did not support the proposals. An updated proposal is included. This new proposal was submitted by The International Association for Chronic Fatigue Syndrome/Myalgic Encephalomyelitis, #MEAction, Open Medicine Foundation, Solve M.E., Massachusetts ME/CFS & FM Association, the Minnesota ME/CFS Alliance, and Pandora Org.

Currently, the term does not exist in the ICD-10-CM and the ICD-10-CM code most often used is the one for “chronic fatigue syndrome,” which is the same code as the symptom of “chronic fatigue”, (R53.82). As a result, it is difficult to accurately track ME/CFS separate from the symptom of chronic fatigue. This could also have secondary effects on healthcare resource planning, fiscal support for clinical care, use of medical records in future research, provisioning of workplace/school accommodations, and determination of disability benefits.

In its 2015 report, the Institute of Medicine concluded that ME/CFS is “a serious, chronic, complex, and multisystem disease,” characterized by the hallmark symptom of post-exertional malaise (PEM) in which even small amounts of cognitive and physical exertion can exacerbate symptoms that can last for days, weeks or sometimes months. ME/CFS is debilitating with an estimated 25% of patients homebound or bedbound and as many as 75% unable to work or go to school. ME/CFS is known to often follow a viral infection

The rationale for including ME/CFS as a synonym to ME is that the ME and ME/CFS criteria, including the IOM criteria, all require the hallmark symptoms of post-exertional malaise, unrefreshing sleep, profound fatigue, and significant impairment in function. These criteria also include other symptoms such as cognitive impairment, orthostatic intolerance, pain, and sensory sensitivity. The US ME/CFS Clinician Coalition recommends the IOM criteria for diagnosis in the US, but also acknowledges the similarities across these definitions and notes that some experts use the 2003 Canadian Consensus Criteria (labeled as ME/CFS) and the 2011 ME International Consensus Criteria (labeled as ME) to validate an IOM-criteria clinical diagnosis of ME/CFS.

The IOM report established new diagnostic criteria that include PEM and substantial impairment in functioning, profound fatigue, unrefreshing sleep, and either cognitive issues or orthostatic intolerance. Other common but non-required symptoms include joint and muscle pain, headaches, and sensitivity to noise and light. Published peer-reviewed studies have demonstrated neurological, immunological, autonomic, and energy metabolism impairment associated with these symptoms.

In addition to the new clinical diagnostic criteria, the IOM also recommended a new name, “Systemic exertion intolerance disease (SEID).” Federal agencies including CDC and NIH, disease experts, and medical education and clinical guidance providers have adopted the term “ME/CFS,” instead of SEID, along with the new clinical diagnostic criteria recommended by the IOM in the 2015 report.

The IOM noted that not all persons previously diagnosed with CFS using the Fukuda CFS definition, used in the US prior to adoption of the IOM criteria, would meet the new IOM criteria for ME/CFS. The IOM did not intend the term ME/CFS to be a replacement for the term chronic fatigue syndrome or an amalgamation of all ME and CFS diagnoses.

Consideration was given as a potential alternative is to have coders separately apply the two codes for the terms, myalgic encephalomyelitis and chronic fatigue syndrome when the doctor diagnoses ME/CFS. However, this does not capture the name of the disease as specified by the doctor or in clinical guidance and medical education. Additionally, this currently could not be done due to existing instructional (Excludes1) notes.

The code title for ICD-10-CM code, G93.3, is currently, postviral fatigue syndrome (PVFS) and ME is an inclusion term. Because the G93.3 title specifies postviral illness, some doctors have declined to diagnose ME when viral illness is not proven. However, according to practice and established criteria, ME can be triggered by both viral and non-viral precipitants, including nonviral infections and non-infectious causes. This is also true for the definitions that use the ME/CFS label, including the IOM criteria.

It is also being recommended the code title G93.3, Postviral fatigue syndrome, be revised to Postviral and related fatigue syndromes to include other precipitants and still maintain the code title’s original wording. By its name, the term postviral fatigue syndrome is intended only for post-viral illness.

But as noted above, the terms ME and ME/CFS include both viral and nonviral precipitants. The ME and ME/CFS definitions further specify that symptoms should persist for 6 months while PVFS is used even if patients have not been sick for six months.

References

1. Institute of Medicine. Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Redefining an Illness. The National Academies Press; May 2015. https://www.nap.edu/catalog/19012/b...chronic-fatiguesyndrome-redefining-an-illness

2. In addition to the US ME/CFS Clinician Coalition, examples of medical education and clinical guidance providers that use the ME/CFS term and the IOM criteria include
• CDC ME/CFS website – https://www.cdc.gov/me-cfs/index.html
• Medscape 2019 ME/CFS CME - https://www.medscape.org/viewarticle/907632?src=acdmpart_cdc
• Kaiser Permanente. ME/CFS medical information. https://healthy.kaiserpermanente.or...halomyelitis-chronic-fatigue-syndrome.hw32907 Content provided by Healthwise which provides content to a number of other sites as well
• Cleveland Clinic - https://my.clevelandclinic.org/heal...ncephalomyelitischronic-fatiguesyndrome-mecfs

3. US ME/CFS Clinician Coalition letter to medical providers. October 30, 2020

Code:

https://drive.google.com/file/d/15Z1pPMsTvxKe_eJtNG3XyXNxx9gB2xxU/view

4. US ME/CFS Clinician Coalition Handout. July 2020.

Code:

https://drive.google.com/file/d/1SG7hlJTCSDrDHqvioPMq-cX-rgRKXjfk/view

5. Bateman L, et al. Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Essentials of Diagnosis and Management Mayo Clinic Proceedings. August 2021. https://www.mayoclinicproceedings.org/article/S0025-6196(21)00513-9/fulltext

6. ICD-10-CM Official Guidelines for Coding and Reporting. FY2020 https://www.cdc.gov/nchs/data/icd/10cmguidelines-FY2020_final.pdf

7. Institute of Medicine 2015 report. Page 210.

8. Institute of Medicine 2015 report. Page 1. Also see the 2003 Canadian Consensus Criteria and the 2011 ME International Criteria which use the terms ME/CFS and ME respectively.
• Carruthers B, Jain A, De Meirleir K, Peterson D, Klimas N, et al. Myalgic encephalomyelitis/chronic fatigue syndrome: Clinical working case definition, diagnostic and treatment protocols (Canadian case definition). Journal of Chronic Fatigue Syndrome. 2003 11(1):7-115.
• Carruthers B. van de Sande M, De Meirleir K, Klimas N, Broderick G, et al. Myalgic encephalomyelitis: International consensus criteria. Journal of Internal Medicine 2011. 270(4):327-338.

9. World Health Organization ICD-10: Version 2019. https://icd.who.int/browse10/2019/en#/F48.0

10. International Association of CFS/ME Chronic fatigue syndrome/myalgic encephalomyelitis Primer for Clinical Practitioners Primer. 2014. https://growthzonesitesprod.azureed.../1869/2020/10/Primer_Post_2014_conference.pdf

11. Institute of Medicine 2015 report. Page 5

12. Institute of Medicine 2015 report. Page 8, 77

13. See the evidence review in the Institute of Medicine report. Also see Komaroff AL. Advances in understanding the pathophysiology of chronic fatigue syndrome. JAMA. 2019;322(6):499.






[Proposal text ends]


Note that the proposal for changes to the Alphabetical Index includes the proposal to add the term

Systemic exertion intolerance disease [SEID] G93.32

to the Index, indexed to proposed new sub-code: G93.32, which is the proposed new sub-code for proposed new ICD-10-CM term:

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome

with the following Inclusion terms proposed under

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome:

Chronic fatigue syndrome
ME/CFS
Myalgic encephalomyelitis

 

Note:

The NCHS proposal (as set out in the Final Agenda) calls for the existing ICD-10-CM Inclusion term: R53.82 Chronic fatigue syndrome NOS in the Symptoms, signs chapter to be Deleted.

If a term is deleted from ICD altogether, there may be an issue with loss of legacy data going forward for that code.

I assume, but it's not implicit in the Rationale text, that in order that legacy data collected in different countries or areas and at different times for aggregation and disaggregation of data over time is not lost going forward, historic data coded with R53.82 Chronic fatigue syndrome NOS would be mapped to one of the proposed new sub-code's Inclusion terms.

The proposed elimination of R53.82 Chronic fatigue syndrome NOS, the potential for loss of data going forward and how code R53.82 Chronic fatigue syndrome NOS would need to be handled in the context of these proposals, is an issue I anticipate Donna Pickett or a CDC colleague will likely be addressing during the presentation.*


The proposal calls for Adding Chronic fatigue syndrome as an Inclusion term under proposed new sub-code

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome

with the following proposed Inclusion terms under G93.32:

Chronic fatigue syndrome
ME/CFS
Myalgic encephalomyelitis

[The issue of the use of conjoined terms, acronyms and conjoined acronyms as Inclusion terms in ICD are issues I anticipate CDC will be addressing during the presentation.]

The proposal also calls for Adding new term:

Systemic exertion intolerance disease [SEID] G93.32

to the Index and indexing Systemic exertion intolerance disease [SEID] to the proposed new G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome sub-code.

The addition of Systemic exertion intolerance disease [SEID] to the Index did not appear in the proposals, as circulated a few weeks ago by SOLVE ME and appears to be one of several modifications to the original proposal that has been recommended by NCHS.

For those not familiar with the required format for setting out proposed modifications for Additions, Deletions etc I will set out more simply, in the next post, how the ICD-10-CM Tabular List would be re-structured, if these proposals were to be accepted by CDC and approved by the Director of NCHS.


*Dr Wanda Jones: "If code R53.82 were eliminated it would not be possible to disaggregate cases that are now distinguishable through the use of two codes." Source: Extract: ICD-related questions from CFSAC, May 2011 meeting

Dr Wanda Jones: "It should be noted that including chronic fatigue syndrome NOS at code G93.3 would make it difficult to disaggregate cases that are now distinguishable through the use of two separate codes."

 

ICD-10-CM Tabular List structure as it would look if approved and implemented (NCHS alternative Option, as included in Topic Packet):

ICD-10-CM Tabular List, Chapter 6: Diseases of the nervous system

G93 Other disorders of brain [existing Category code]

G93.3 Postviral and related fatigue syndromes [new Concept Title term]
Excludes1: chronic fatigue, unspecified (R53.82) [new Excludes1]
neurasthenia (F48.8) [new Excludes1]

G93.31 Postviral fatigue syndrome [new sub-code]

G93.32 Myalgic encephalomyelitis/chronic fatigue syndrome [new sub-code, new term]
Chronic fatigue syndrome [new Tabular List Inclusion under new sub-code term]
ME/CFS [add acronym as Inclusion under new sub-code term; NB: this acronym is not included in ICD-10, ICD-11 or in any national modification of the WHO's ICD-10]
Myalgic encephalomyelitis [existing Inclusion term minus "Benign", now Inclusion under new sub-code term]

G93.39 Other post infection and related fatigue syndromes [new sub-code]
​

ICD-10-CM Tabular List, Chapter 18: Symptoms, signs chapter

Currently:

R53 Malaise and fatigue
R53.8 Other malaise and fatigue

R53.82 Chronic fatigue, unspecified
Chronic fatigue syndrome NOS
Excludes1: postviral fatigue syndrome (G93.3)


Proposed to be revised to:

R53 Malaise and fatigue
R53.8 Other malaise and fatigue

R53.82 Chronic fatigue, unspecified
Excludes1: chronic fatigue syndrome (G93.32) [new Excludes1]
myalgic encephalomyelitis (G93.32) [new Excludes1]
post infection and related fatigue syndromes (G93.39) [new Excludes1]
postviral fatigue syndrome (G93.31) [Revised code]​

Proposed modifications to Index terms:

Systemic exertion intolerance disease [SEID] G93.32 [Added] [new Index term indexed to new G93.32 code]

Akureyri's disease G93.3 .39 [sic] [Revised]

Disease, diseased - see also Syndrome
- Iceland G93.39 [Revised]
Encephalomyelitis G04.90 - see also Encephalitis
- myalgic, (benign) G93.32 [Revised term and code]
Neuromyasthenia (epidemic) (postinfectious) G93.39 [Revised]


Syndrome - see also Disease
- postbacterial fatigue G93.39 [Added]
- postinfectious fatigue G93.39 [Added]
- postviral NEC G93.31 [Revised]
- - fatigue G93.31 [Revised]​

-------------------------------------------------------------------

*Excludes1 A type 1 Excludes note is a pure excludes. It means 'NOT CODED HERE!' An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

Excludes2 A type 2 excludes note represents 'Not included here'. An excludes2 note indicates that the condition excluded is not part of the condition it is excluded from but a patient may have both conditions at the same time. When an Excludes2 note appears under a code it is acceptable to use both the code and the excluded code together.

Source: ICD-10-CM FY 2022 Tabular List



I hope the above setting out will make these proposed changes easier to visualise than the format that is required for the submission process.

NB: All enquiries about these proposals will need to be directed to SOLVE ME or the presenters: Traci Ramirez and Mary Dimmock, as I have no involvement in this submission.

 

From the Diagnosis Codes Topic Packet:

September 14-15, 2021 The September 2021 ICD-10 Coordination and Maintenance Committee Meeting is fully virtual by zoom and dial-in.

September 2021 Recordings and slide presentations of the September 14-15, 2021 ICD-10 Coordination and Maintenance Committee Meeting will be posted on the following web pages: Diagnosis code portion of the recording and related materials– https://www.cdc.gov/nchs/icd/icd10cm_maintenance.htm

October 15, 2021 Deadline for receipt of public comments on proposed new codes discussed at the September 14-15, 2021 ICD-10 Coordination and Maintenance Committee Meeting being considered for implementation on April 1, 2022.

[Ed: that's a comment deadline of 4 weeks and 2 days.]

November 15, 2021 Deadline for receipt of public comments on proposed new codes and revisions discussed at the September 14-15, 2021 ICD-10 Coordination and Maintenance Committee Meeting being considered for implementation on October 1, 2022.

[Ed: that's a comment deadline of around 8.5 weeks.]

 

I'm afraid you've missed my point about classification system "terms" (as opposed to "diseases"). My point was this:

"Myalgic encephalomyelitis" is a unique, machine readable ICD term (or entity).

"Chronic fatigue syndrome" is a unique machine readable ICD term (or entity).

"Myalgic encephalomyelitis/chronic fatigue syndrome" would not be a unique term, since the term amalgamates existing, unique ICD terms which are intended to remain within the Diseases of the nervous system chapter of ICD-10-CM (in the case of "Myalgic encephalomyelitis") and be Added to the Diseases of the nervous system chapter (in the case of "chronic fatigue syndrome").

ICD-10 does not specify what the WHO understands by the terms: Postviral fatigue syndrome and Myalgic encephalomyelitis or how it views the relationship between these two terms. Nor has the WHO described either of these two terms for ICD-11, despite the fact that ICD-11 diseases and disorders all have the capacity for including substantial Description texts. The WHO has had the opportunity but has chosen not to populate the Description parameter for 8E49.

In indexing Chronic fatigue syndrome to G93.3, the WHO's ICD-10 does not specify whether it views the term as a synonym, sub-entity or "best coding guess" to Postviral fatigue syndrome.

In specifying Chronic fatigue syndrome in ICD-11 as an Inclusion under 8E48 Postviral fatigue syndrome, again, ICD-11 does not specify how it views the relationship between Chronic fatigue syndrome and Postviral fatigue syndrome; or how it views the relationship between Chronic fatigue syndrome and Myalgic encephalomyelitis.

From the ICD-11 Reference Guide:

"2.2.2 Inclusions
Within the coded categories there are typically other optional diagnostic terms. 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 category. They may refer to different conditions or be synonyms. They are not a sub-classification of the category.

Inclusion terms are listed primarily as a guide to the content of the category, in addition to the descriptions. Many of the items listed relate to important or common terms belonging to the category. 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."

​

After 13 years working on the revision of the Diseases of the nervous system chapter (which included reviewing the IOM Report, other recent US documents, undertaking a literature review, reviewing materials provided by ourselves, and a detailed proposal rationale), we are no better able to say what the WHO understands by any of the three legacy terms than we were 13 years ago.

Six years after publication of the IOM Report, the WHO has made no statement on what it understands by the term "Myalgic encephalomyelitis/chronic fatigue syndrome" (nor for that matter has it made any statement regarding "SEID"); and I suspect if one were to request the WHO's opinions and understandings of what the term "Myalgic encephalomyelitis/chronic fatigue syndrome" describes and how this differs from "the diseases labelled with the terms ME and CFS" that no meaningful response would be forthcoming, either.

The bottom line is, the WHO is silent on what any of the three unique ICD-10 and ICD-11 terms describe and provides no guidance to the coder or other professional end users on how to differentiate between them.

 

Extract from SOLVE ME, IACFS/ME, #MEAction et al's proposal:

It's 6 years on since the IOM panel published its report, in early 2015.

The report should have contained a far stronger caveat that its Recommendations were just that - recommendations for the sponsor agencies to review and give consideration to.

Within days of the report's release, one of the peer reviewers for the report, who is also an editor for the CFS pages of the medical information website "UpToDate", inserted the term "Systemic exertion intolerance disease (SEID)" into the site's CFS, ME pages as though it were a given that the term would be adopted and implemented by the sponsor agencies. We have also seen incontinent use of the "SEID" term in media articles and by some patient organisations, including by some orgs outside the US whose countries do not use ICD-10-CM.

The term "Systemic exertion intolerance disease (SEID)" was a recommendation only and the term hasn't been adopted.

It was said by Donna Pickett, at a C & M meeting, that because some clinicians had started to use the SEID term, the term needed to be incorporated into ICD-10-CM in order that it could be coded for.

This is a specious argument. Clinicians should not be applying a diagnostic term that was the recommendation only of an ad hoc panel - a term that has not been adopted and implemented by the agencies which sponsored the panel's report.

I see no rationale for requesting that the (unadopted) term, "Systemic exertion intolerance disease (SEID)" should be added as an Index term to ICD-10-CM, and indexed to a proposed new sub-code:

G93.32 Myalgic encephalomyelitis/Chronic fatigue syndrome

under which the following terms

Chronic fatigue syndrome
ME/CFS [a conjoined acronym not currently included in ICD-10, ICD-11 or in any national modification of the WHO's ICD-10]
Myalgic encephalomyelitis

would sit as inclusions.


Nor do I accept arguments that it is "too late to stuff the cat back into the bag because clinicians are already using the term."

 

DX Revision watch - From earlier comment"...The request was referred for consideration to SNOMED International's terminology team who agreed that since the term has not been added to the U.S.'s ICD-10-CM by NCHS/CDC or adopted by any of the sponsor agencies of the IOM Panel's Report, it would be premature to add the term to SNOMED CT's International Edition [2].....

If the proposal to add SEID to the index terms goes through in the US, would this be likely to add SEID to SNOMED?

 

There is a classification map between SNOMED CT Concept codes and the WHO's ICD-10 codes.

SNOMED CT's SCTID: 52702003 Chronic fatigue syndrome (disorder) and all terms listed under Synonyms are mapped across to ICD-10's G93.3 code.

For the US's ICD-10-CM the map between SNOMED CT US Edition and ICD-10-CM gives the coder or other end user a choice. They can map to ICD-10-CM's G93.3 or to ICD-10-CM's R53.82 code.

If addition of the SEID term to the Index and the other proposed changes were to be approved and implemented for ICD-10-CM, the SNOMED CT to ICD-10-CM map would need to be revised.

So yes, if the SEID term were to be approved for addition to the ICD-10-CM Index, it is likely that one of the federal agencies or someone who works within the coding industry might submit to SNOMED CT International or to the managers of the SNOMED CT US Edition for addition of the term under the Synonyms list for 52702003 Chronic fatigue syndrome (disorder); or alternatively for creation of a unique SNOMED CT Concept code for Systemic exertion intolerance disease [SEID].

If addition of the SEID term were requested and approved for the International Edition it would subsequently be absorbed by all the various national editions (as has happened this year, in the case of Somatic symptom disorder, which was requested by UK IAPT leads*).


*Although IAPT was unsuccessful in obtaining creation of a unique Concept code for SSD for SNOMED CT, the term was approved for adding under Synonyms under Bodily distress disorder (bringing SNOMED CT in line with ICD-11). So IAPT now has a SNOMED CT Concept code available for use in its new Data Set v2.0, as Somatic symptom disorder takes the SCTID code assigned to SNOMED CT's Bodily distress disorder.

 

https://www.icd10monitor.com/coordination-and-maintenance-meeting-starts-today

Coordination and Maintenance Meeting Starts Today
By Laurie M. Johnson, MS, RHIA, FAHIMA AHIMA Approved ICD-10-CM/PCS Trainer

Original story posted on: September 13, 2021

Coordination and Maintenance committee meeting remains virtual.

The Coordination and Maintenance Committee meeting is scheduled for today, Sept. 14, and continues through Sept. 15. As in the past, the two-day event will be held virtually.

Comments may be emailed to ICDProcedureCodeRequest@cms.hhs.gov for the procedure codes and nchsicd10cm@cdc.gov for the diagnosis codes.

The meeting will begin with the procedure topics under the guidance of the Centers for Medicare & Medicaid Services (CMS).

 

I did not know POTS also did not have an ICD code either. Not surprising really but wow is Long Covid exposing major gaps in basic areas of medicine, which somehow only we and a few others understood. What a mess, decades of useful information unusable because the recording machine was turned off on purpose.

https://twitter.com/user/status/1437862264393064448