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ICD-11 status reports: G93.3 legacy terms: PVFS, BME, CFS

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New proposal added which seems to support the one submitted by Suzy and Mary

Patient organisations can help by registering and "agree" with proposals being made


Diseases of the nervous system
Detailed Explanation of the Proposal
As the Board of the largest international organization of professionals dedicated to the care and research of people affected by myalgic encephlomyelitis/ chronic fatigue syndrome (ME/CFS), we would like to submit the following comments regarding ICD-11 classification of these conditions:

We recommend the following:

1) The continued classification of these three terms in the neurological chapter, as was done in ICD-10, until such time that research provides the evidence for a more appropriate classification.

2) The retention of the term "postviral fatigue syndrome" (PVFS) as a concept title along with the elevation of the terms "chronic fatigue syndrome" (CFS) and "myalgic encephalomyelitis" (ME) to concept titles at the same level, with each of the three terms given a unique code.

3) Modify the ICD-10 term "benign myalgic encephalomyelitis" to "myalgic encephalomyelitis" as the disease is not benign.

4) Add back the reciprocal exclusions between these terms and the word “fatigue” (as was done in ICD-10) and also between these terms and bodily distress disorder.

5) We oppose the classification and/or dual parenting of these terms in either the symptoms chapter or the mental health chapter in the ICD 11 Handbook.

Thank you for your attention,

Board of the International Association for Chronic Fatigue Syndrome/ Myalgic Encephalomyelitis

www.iacfsme.org


Fred Friedberg, PhD
Stony Brook University
Stony Brook, NY, USA

Lily Chu, MD, MSHS
Burlingame, CA, USA

Steve Krafchick, MPH, JD
Krafchick Law Firm PLLC
Legal Services for Injured and Disabled People
Seattle, WA, USA

Sonya Marshall-Gradisnik, PhD

National Centre for Neuroimmunology and Emerging Diseases, Griffith University
Melbourne, Australia

Jon D. Kaiser, MD
UCSF Medical School
San Francisco, CA, USA

IACFS/ME ICD-10/11 Committee Member:

Alison Bested, MD, FRCP

Toronto, Canada





Rationale
Rationale corresponding to each point in the proposal. Names in parentheses correspond to references.



[1] and [2]:

ME, CFS, and PVFS may differ from each other in terms of their symptoms, features, potential etiologies,

prognoses, and treatment. Consequently, the three entities should be treated separately as they have

have been in past editions of the ICD codes. We support the decision to have a separate code for each of the different conditions.



[3]



When the first outbreaks of ME occurred, during the 1930s and 1950s, when polio outbreaks were common, ME was labelled "benign" because, comparatively, it did not frequently cause paralysis or death. However, since that time, much evidence has accumulated that the condition is far from mild. A systematic review of prognosis revealed that only a mean 5% of patients ever recovered fully (Cairns) and a 25-yr. follow-up study (Brown) showed than many subject continued to be severely affected. At least 25% of patients are bed- or housebound (Institute of Medicine, Key Facts) and their function/ quality of life is frequently worse than that of people affected by stroke, breast cancer, multiple sclerosis, and rheumatoid arthritis (Falk). There is also some suggestion that patients may die earlier of suicide, heart disease, or cancer (McManimen,Jason).



[4] and [5]:



Although fatigue is a common and severe symptom of ME, PVFS, CFS, all the current case definitions for these conditions include many more symptoms than symptoms than fatigue. (Institute of Medicine) In 2015, the United State Institute of Medicine completed a review of over 9,000 research articles and concluded that ME and CFS were not the same as fatigue, not a mental health condition, and not a "figment of the patient's imagination." (Institute of Medicine, Report Brief) There are thousands of articles demonstrating physiological abnormalities in these conditions; some are summarized in these materials from our 2014 and 2016 conference. (Komaroff, Vallings) Subjects feel unwell because of these abnormalities, not because they are oversensitive to or exaggerating normal bodily sensations.



The classification of these conditions as psychiatric or psychological conditions is also detrimental in that they contribute to decreasing investment into medical interventions which actually might be more effective in helping patients. IACFS/ME has always recognized that co-morbid psychological and psychiatric conditions should be treated and that certain counseling treatments may help patients cope better with their illness. (Goudsmit) However, claims that psychological or psychiatric treatment substantially help subjects with their symptoms associated with these disorders are unwarranted and the latest, multi-million dollar trial claiming this came with many flaws. (Coyne, Sense About Science) The preponderance of evidence suggests that these conditions do not originate from psychiatric or psychological causes but from physiological ones.

References
Brown MM, Bell DS, Jason LA, Christos C, Bell DE. Understanding Long-Term Outcomes of Chronic Fatigue Syndrome. Journal of clinical psychology. 2012;68(9):1028-1035. doi:10.1002/jclp.21880. [ISBN: 3940158]
Coyne J. Uninterpretable: Fatal flaws in PACE Chronic Fatigue Syndrome follow-up study. PLOS One Blogs. October 2015.
Falk Hvidberg M, Brinth LS, Olesen AV, Petersen KD, Ehlers L (2015) The Health-Related Quality of Life for Patients with Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME/CFS). PLoS ONE 10(7): e0132421. doi:10.1371/journal.pone.0132421
Fatigue. 2016;4(4):195-207. doi: 10.1080/21641846.2016.1236588. Epub 2016 Oct 12. Mortality in Patients with Myalgic Encephalomyelitis and Chronic Fatigue Syndrome. McManimen SL1, Devendorf AR, Brown AA, Moore BC, Moore JH, Jason LA. [ISSN: 28070451]
Goudsmit, G. Cognitive-behavioral therapy.
Jason LA, Corradi K, Gress S, Williams S, Torres-Harding S.Causes of death among patients with chronic fatigue syndrome. Health Care Women Int. 2006 Aug;27(7):615-26. [ISSN: 16844674]
Komaroff A. Highlights from the 2014 IACFS/ME Conference. [ISBN: 0]
Occup Med (Lond). 2005 Jan;55(1):20-31. A systematic review describing the prognosis of chronic fatigue syndrome. Cairns R, Hotopf M. [ISSN: 15699087]
Sense About Science, USA. PACE: The research that sparked a patient rebellion and challenged medicine. 3/21/2016.
United States Institute of Medicine Committee on Diagnostic Criteria for ME/CFS. Beyond ME/CFS: Redefining an Illness. Key Facts. 2015.
United States Institute of Medicine Committee on Diagnostic Criteria for ME/CFS. Beyond ME/CFS: Redefining an Illness. 2015. [ISBN: 190121]
United States Institute of Medicine Committee on Diagnostic Criteria for ME/CFS. Beyond ME/CFS: Redefining an Illness. Report Brief, 2015.
Vallings R. Summary of the 12th Annual (2016) IACFS/ME Conference.
 

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Suzy Chapman Owner of Dx Revision Watch
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I shall continue very tied up with ICD-11 related tasks until after the weekend.

I won't have much time for monitoring this thread or my PR Inbox. So please direct any enquiries via the Contact Form on my website.

While I am here, there are a couple of posts to post:

1 Our proposal, which was prepared prior to the restoration of the terms to the draft, recommended that exclusions are inserted under Fatigue for the three terms and that Fatigue is also inserted as a reciprocal exclusion.

On March 26, when the three terms were restored to the Beta draft, "Team WHO" also approved and Implemented my December 2014 proposal for exclusions for BME and CFS under Fatigue.

The Implementation of the December 2014 proposal for an exclusion for PVFS under Fatigue remains outstanding. It is unclear whether this is an oversight or if there is another reason for this not having been approved yet. (Where there is a concept title and inclusion(s), it would usually be the concept title that is specified as the exclusion term. So this is an anomaly which needs an explanation.)

Before the deadline ended, last night, I also put in stand alone proposals for exclusions for all three terms under Bodily distress disorder (and reciprocating exclusions) as that proposal had been Rejected in November 2016, since the terms did not exist in the draft to be mapped to. I also submitted a stand alone proposal for reciprocal exclusions for Fatigue (since these had not formed part of my original December 2014 proposals).

I also submitted stand alone proposals, before the deadline was reached, for deprecation of the designation "benign"; and for the deletion of the term "chronic fatigue, unspecified" under the Synonyms list for PVFS, as the list currently stands in the Beta draft.

This is a Symptoms, signs chapter category (R53.82) that is unique to the U.S. specific, ICD-10-CM.

It is the "concept title" code under which the inclusion term, "chronic fatigue syndrome NOS" sits under in ICD-10-CM. (ICD-10-CM does not have a "chronic fatigue syndrome" under G93.3. It only has a "chronic fatigue syndrome NOS" under the R codes, with PVFS and BME at G93.3).

I have proposed its deletion. In 2009-2013, the list had also included the U.S. specific "chronic fatigue syndrome NOS." But that was not included in the list of synonym terms, as restored on March 26. If it does appear, I will propose its removal.*

I will let you know if any of these stand alone proposals are approved and Implemented.

*Proposals can continue to be submitted. However, proposals received after March 30, will be carried forward for consideration for inclusion in the first update and revision process, in 2019.


2 I shall be contacting Mark Berry, today, to ask whether Phoenix Rising, as a registered organization, would like to review our proposal document.

3 For those registered with the Beta drafting platform, certain other functions are available via the "Linearizations" tab. For example, the Coding Tool, Specialty Linearizations and Print Versions.

The Print Versions include PDFs and Word documents for the "Top Level" category list (that includes concept titles only); for All Chapters; for Individual Chapters and for the Print Version of the Index.

These PDFs are electronically generated from the Foundation and MMS electronic platform and are updated every three or four days to reflect the daily revisions to the platform. (So they are a snapshot of the draft as it stands for the date in the footer of the PDFs.)


Although, "Team WHO" restored the three terms to the Beta platform on March 26, the Print Versions and Index were not updated until March 27.

I can confirm that the current PDFs do contain the three terms. This screenshot shows the entry in Chapter 08 Diseases of the nervous system and underneath that, the three entries compiled from the Index.

In a day or two, if not already, the codes will have changed as the codes in the Beta are unstable as new terms are added and category blocks continue to be reorganized.*




*Since last night, the code has changed to 8C69.
 
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Suzy Chapman Owner of Dx Revision Watch
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I have already posted Pages 1 - 4 from our Proposal and Rationale document in Post #137.

The full Proposal and Rationale can be downloaded here, or there is a PDF attached to Post #137.

These are the section headers included in our proposal and following that, another couple of sections:

Page 1
Proposal for the ICD-10 G93.3 legacy terms for ICD-11
How the G93.3 terms are currently represented in ICD-10:
(CFS is included in the Index, only)
[Image]
Proposal for ICD-11: (CFS and ME assigned to separate codes; PVFS under Synonyms to ME)
[Image]


Page 2 Detailed Explanation of the Proposal

Page 3 Proposed hierarchical restructure

Page 4
Notes:
Note 1: Inclusion of chronic fatigue syndrome in Foundation and MMS Linearization

Page 5 Note 2: Deprecation of "benign"

Page 6
Note 3: Deprecation of postviral fatigue syndrome
Note 4: Exclusions

Page 7
4.1 Exclusions under Fatigue
4.2 Classificatory revisions to SNOMED CT and Clinical Terms version 3 (CTV3)
4.3 Exclusions under Bodily distress disorder

Page 8 4.4 Potential for conflation of bodily distress disorder and bodily distress syndrome

Page 9
Rationale
1. Scientific evidence that exists for neurological dsyfunction

Page 10
2. Recent federal agency reviews and scientific evidence
2.1 2014 AHRQ Evidence Review
2.2 Highlights of Recent Research

Page 11
3. Options and considerations
3.1 Classification of multisystem diseases within ICD-11
3.2 ICD-11 Joint Task Force position on precedence

Page 12
3.3 Consideration of creating a sub class under: Other disorders of the nervous system
3.4 Assigning unique codes for chronic fatigue syndrome (CFS) and myalgic encephalomyeltis (ME)
3.5 Consideration of the IOM Report's recommendation for a new case definition and change of nomenclature

Page 13 4. Definitions

Page 14 Submitting comments on Proposals

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

Page 5

Note 2: Deprecation of "benign": The term "benign myalgic encephalomyelitis" was first introduced in 1956, in a Lancet editorial later attributed to the former Chief Medical Officer, Sir Donald Acheson [1]. The designation "benign" had originally been suggested because no fatalities had been recorded at the time from myalgic encephalomyelitis.

WHO included "benign myalgic encephalomyelitis" as an index entity within ICD-9 (published 1975). For ICD-10 (published 1992), WHO created a new category G93, Other disorders of brain, in Chapter VI: Diseases of the nervous system, and created a new code G93.3, Postviral fatigue syndrome, an entity previously in the symptom chapter of ICD-9. WHO moved benign myalgic encephalomyelitis under the new G93.3 code [2].

ICD-10 has continued to include the designation "benign" within its classification.

In 1978, an international symposium was held at the Royal Society of Medicine to discuss the disease and future research directions. A British Medical Journal editorial reported there had been clear agreement that myalgic encephalomyelitis was a distinct, organic, nosological entity and that dismissal of the disease as psychological was no longer tenable. Attendees discussed the variety of physical findings, in both epidemic and sporadic cases, and described the characteristic feature of post effort exhaustion and fatigability. There was agreement that future outbreaks should be studied by a collaborative team of neurologists, epidemiologists, virologists, and immunologists and that findings would also be important for other neurological disorders, including multiple sclerosis.

Attendees considered other terms used to describe the disease but rejected these as unsatisfactory. They agreed on "myalgic encephalomyelitis," omitting the prefix "benign" in response to the high burden of morbidity associated with the disease and because the term "benign" was misleading from the patient's perspective, "since the illness may be devastating" [3][4].

For at least the last 14 years, researchers have published studies reporting an increased risk of death due to cancer, cardiac disease, and suicide. While preliminary, these studies have reported similar findings which can be explained by what is known about the pathology of the disease and the stigma patients experience [5][6].

On July 11, 2009, autopsy evidence was presented at a Royal Society of Medicine meeting by Dr Abhijit Chaudhuri, Essex Centre for Neurosciences, where slides of inflammation of dorsal root ganglia in three patients with myalgic encephalomyelitis were discussed [7]. A disease specific post-mortem brain and tissue bank for the study of myalgic encephalomyelitis and chronic fatigue syndrome is proposed for the UK [8].

Given the high burden of morbidity and recorded mortalities (the earliest known recorded cause of death due to ME, CFS in the UK was 2003), the authors see no justification for retaining the designation "benign" for ICD-11.

Recommendation: Replace entity, "Benign myalgic encephalomyelitis" with "Myalgic encephalomyelitis." For continuity and comparability with ICD-10 and with SNOMED CT International Edition and National Extensions, the authors propose listing "myalgic encephalomyelitis (benign)" under Synonyms to concept title: Myalgic encephalomyelitis.

References:

1 A new clinical entity? Editorial; Lancet 1956 (May 26);789-790. http://www.sciencedirect.com/science/journal/01406736/267/6926

2 A Summary of Chronic Fatigue Syndrome and its Classification in the International Classification of Diseases, Centers for Disease Control and Prevention, National Center for Health Statistics, March 2001. http://www.institutferran.org/documentos/icd_code.pdf

3 Epidemic myalgic encephalomyelitis. British Medical Journal. 1978 Jun 3; 1(6125): 1436–1437. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1604957/

4 Postgraduate Medical Journal November 1978: 54(637): 709-774. http://pmj.bmj.com/content/54/637.toc#Articles

5 ME/CFS: A Primer for Clinical Practitioners 2014. (p. 26). http://iacfsme.org/portals/0/pdf/Primer_Post_2014_conference.pdf

6 Dimmock ME, Mirin AA, Jason LA (2016). Estimating the disease burden of ME/CFS in the United States and its relation to research funding. J Med Therap 1: doi:10.15761/JMT.1000102. https://oatext.com/Estimating-the-d...ates-and-its-relation-to-research-funding.php

7 O’Donovan D, Harrower T, Cader S, Findley L, Shepherd C, Chaudhuri A. Pathology of Chronic Fatigue Syndrome: Pilot Study of four autopsy cases. International Science Symposium 3-4 – Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) Queensland, Australia: Population Health and Neuroimmunology Unit, Bond University; 2010.

8 Nacul L, O’Donovan DG, Lacerda EM, et al. Considerations in establishing a post-mortem brain and tissue bank for the study of myalgic encephalomyelitis/chronic fatigue syndrome: a proposed protocol. BMC Research Notes. 2014;7:370. http://bmcresnotes.biomedcentral.com/articles/10.1186/1756-0500-7-370
_______________________________________________________________________________

Page 6

Note 3: Deprecation of postviral fatigue syndrome: The term "postviral fatigue syndrome" appears to be an ill-defined term that is not found extensively in the literature.

A search via PubMed on March 12, 2017 returned 55 papers referencing "postviral fatigue syndrome" (1985 - 2005) and 39 papers referencing "post viral fatigue syndrome" (1985 - 2014). Whereas a search for "myalgic encephalomyelitis" returned 629 papers (1956 - 2017) and a search for "chronic fatigue syndrome" returned 6020 papers (1985 - 2017) [1].

The extent to which the term is used by practitioners in primary care and specialty clinical settings and the frequency of recording in electronic medical record systems has not been determined.

Other than the Oxford Criteria for Post-infectious Fatigue Syndrome (PIFS), which is defined as "a subtype of CFS which either follows an infection or is associated with a current infection," the authors can find no working case definition for postviral fatigue syndrome [2]. The U.S. National Institutes of Health (NIH) has declared the Oxford Criteria severely flawed and recommended its retirement [3].

Postviral fatigue syndrome is an inappropriate concept title for myalgic encephalomyelitis, as not all cases of myalgic encephalomyelitis are preceded by a viral infection [4].

The authors consider there is insufficient justification for retaining postviral fatigue syndrome as a concept title for ICD-11.

Recommendation: For continuity and comparability with ICD-10, the recommendation is to include postviral fatigue syndrome as a synonym term under new concept title: Myalgic encephalomyelitis.

3.1 Synonyms: Synonym terms for Myalgic encephalomyelitis have been suggested with reference to those listed in SNOMED CT International Edition and SNOMED CT National Extensions [5].

References:

1 PubMed, US National Library of Medicine National Institutes of Health. https://www.ncbi.nlm.nih.gov/pubmed

2 Sharpe MC, Archard LC, Banatvala JE, et al. A report - chronic fatigue syndrome: guidelines for research. Journal of the Royal Society of Medicine. 1991;84(2):118-121. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1293107/

3 National Institutes of Health Pathways to Prevention Workshop: Advancing the Research on Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome, Final Report. December 9–10, 2014 (p. 16). https://prevention.nih.gov/docs/programs/mecfs/ODP-P2P-MECFS-FinalReport.pdf

4 "The cause of ME/CFS remains unknown, although in many cases, symptoms may have been triggered by an infection or other prodromal event, such as "immunization, anesthetics, physical trauma, exposure to environmental pollutants, chemicals and heavy metals, and rarely blood transfusions" (Carruthers and van de Sande, 2005, p. 1)." National Academy of Medicine. Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Redefining an Illness. The National Academies Press. May 2015. https://www.nap.edu/catalog/19012/b...hronic-fatigue-syndrome-redefining-an-illness

5 SNOMED International SNOMED CT Browser. http://browser.ihtsdotools.org/ [Accessed: March 16, 2017]

 
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Suzy Chapman Owner of Dx Revision Watch
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wow, that is fantastic work Suzy and Mary, thank you both

I do hope many patient organisations will register to leave comments or at least support the proposals made by you
Thank you. And I would like to publicly acknowledge Mary's contribution. Her collaboration in this initiative has been crucial.
 

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http://apps.who.int/classifications...lGroupId=e3426560-b6e4-4c94-b0c8-ba25fabe66fa

Content Enhancement Proposal

Fatigue


While the optimal place in the classification is still being identified, the entity has been put back to its original place in ICD.

Team WHO 2017-Mar-26 - 12:46 UTC


Comment


I note that on March 26, after a four year long absence from the draft, ICD Revision restored the ICD-10 legacy concept: Postviral fatigue syndrome under Other disorders of the nervous system, with Benign myalgic encephalomyelitis and Chronic fatigue syndrome specified as inclusion terms.

I further note that my proposals of December 30, 2014 to insert an exclusion under Fatigue for (Benign) myalgic encephalomyelitis and for Chronic fatigue syndrome have been Implemented.

The additional proposal of December 30, 3014 to insert an exclusion under Fatigue for Postviral fatigue syndrome remains unapproved. This appears to be an anomaly.

Would Team WHO please clarify whether this is an oversight and if not, what is the rationale for inserting exclusions for Postviral fatigue syndrome's two inclusion terms, but not Postviral fatigue syndrome, per se, which is currently listed as the ICD concept title?

Thank you.

Suzy Chapman 2017-Mar-31 - 12:57 UTC
 

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Suzy Chapman Owner of Dx Revision Watch
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Pages 6 to 9

The full Proposal and Rationale can be downloaded here, or there is a PDF attached to Post #137.

Page 6

Note 4: Exclusions: One of the authors submitted proposals for adding exclusions for the G93.3 legacy entities under Fatigue and under Bodily distress disorder in December 2014 [1][2]. The ICD-11 principle is that exclusion terms must exist in the classification as entities to enable linking. It follows that until the G93.3 legacy entities are restored to the Beta draft, exclusions for these terms cannot be inserted.

It is the authors' understanding that the number, type and application of exclusions for ICD-11 have been under review since publication of the Content Model Reference Guide 2011, and that the principles for exclusions may be in the process of being updated [3][4].


Page 7

On the basis that exclusion type: "elsewhere classified, not to be coded here" (with a link to where the entity should be coded) is being retained, the authors recommend that this type of exclusion should be inserted under Fatigue and also under Bodily distress disorder for concept title Chronic fatigue syndrome and concept title Myalgic encephalomyeltis to mitigate the risk of miscoding under Fatigue or misdiagnosis with, or misapplication of, an additional diagnosis of Bodily distress disorder.


4.1 Exclusions under Fatigue: In ICD-10: Tabular List, Chapter XVIII: Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified, there is an Excl. for "fatigue syndrome: • postviral (G93.3)" under R53 Malaise and fatigue.

For ICD-11, the legacy concept title, Malaise and fatigue, is replaced with new concept title, Fatigue, which is primary parented under General symptoms, and secondary parented to proposed grouping, "Symptoms or signs involving motivation or energy." It is noted that Bodily distress disorder is listed under exclusions to Fatigue.

Fatigue is a common complaint. Many disease processes cause symptoms of fatigue or prolonged fatigue which can be explained by the illness and which in some cases may be treatable. Fatigue, chronic fatigue, idiopathic fatigue and idiopathic chronic fatigue do not meet the specific criteria for chronic fatigue syndrome and myalgic encephalomyeltis [5].

The Canadian Institute for Health Information (CIHI) clinical modification, ICD-10-CA, and the German Institute of Medical Documentation and Information (DIMDI) clinical modification, ICD-10-GM, rightly retained an Excl. for "fatigue syndrome: • postviral (G93.3)" under R53 Malaise and fatigue.

Recommendation: Retain exclusions under Fatigue for concept title Chronic fatigue syndrome and concept title Myalgic encephalomyeltis to mitigate the risk of miscoding these defined diagnostic categories. Insert exclusions for Fatigue under concept title Chronic fatigue syndrome and concept title Myalgic encephalomyeltis.


4.2 Classificatory revisions to SNOMED CT and Clinical Terms version 3 (CTV3):
Prior to July 2015, SNOMED CT International Edition and its National Extensions had Chronic fatigue syndrome (with Myalgic encephalomyelitis and several related and historical terms as Synonyms terms) classified under two parent disorder classes: Mental disorder; and Multisystem disorder.

In March 2015, Health and Social Care Information Centre (now NHS Digital) confirmed that the owners of SNOMED CT (IHTSDO) had reviewed the relationship between the entry for SCTID: 52702003 Chronic fatigue syndrome and its Mental disorder parent and had retired the Mental disorder parent [6].

Since the July 2015 release of SNOMED CT International Edition, Chronic fatigue syndrome is classified under the single parent, SCTID: 281867008 Multisystem disorder.This revision was incorporated into all subsequent releases of the various SNOMED CT National Extensions.

The Clinical Terms version 3 (CTV3) primary care clinical terminology product (aka: the UK Read Codes v3)* also had Chronic fatigue syndrome classified under two parent disorder classes: as a sub class of Neurasthenia, under Mental health disorders; and as a sub class of Neurological disorders. The April 2016 release for CTV3 also retired the Mental health disorder parent for Chronic fatigue syndrome to reflect the revised classification in the SNOMED CT International Edition and its Extensions [7].

*Ed: The UK CTV3 (Read Codes) that are used in primary care are scheduled for retirement in 2018. SNOMED CT will be be implemented in primary care settings. SNOMED CT is scheduled for adoption across all NHS clinical settings by 2020.


4.3 Exclusions under Bodily distress disorder:
For ICD-11, the Somatic Distress and Dissociative Disorders Working Group (S3DWG) proposes to replace F48.0 Neurasthenia and all the F45.0 - F45.9 somatoform disorder categories (with the exception of Hypochondriasis) with a new and much simplified, single diagnostic category, for which the current suggested name is "bodily distress disorder" [8][9].

As defined for ICD-11, the proposed bodily distress disorder diagnostic construct has strong conceptual, characterization and criteria alignment with DSM-5's somatic symptom disorder [10]. In the ICD-11 Beta draft, somatic symptom disorder is listed under Synonyms to bodily distress disorder.

For both bodily distress disorder (as defined for ICD-11) and DSM-5's somatic symptom disorder, the distinction between medically explained and medically unexplained somatic complaints is abolished. There is no longer the requirement for symptoms to be medically unexplained in order to meet the criteria, and the symptoms may or may not be associated with another medical condition: "If a medical condition is causing or contributing to the symptoms, the degree of attention is clearly excessive in relation to its nature and progression." [9


Page 8


Thresholds to meet the criteria for bodily distress disorder and for somatic symptom disorder are substantially lower than those of the somatoform disorders which they replace: the presence of bodily symptoms that are distressing to the individual and excessive attention directed toward the symptoms; or a single symptom, for example, pain or fatigue, in association with the psychobehavioural features that define the disorder, will be sufficient to meet the diagnosis.

Frances and Chapman (2013) argue that the somatic symptom disorder criteria - far looser than those of the ICD-10 and DSM-IV somatoform disorders, with low sensitivity and specificity and highly subjective, difficult to measure cognitions - present significant potential for misdiagnosis with, or misapplication of, an inappropriate psychiatric diagnosis [11].

Patients with chronic, troubling somatic complaints associated with any disease are potential candidates for an additional diagnosis of bodily distress disorder - if the clinician considers the criteria are otherwise met.

In a BMJ commentary, Frances (2013), highlights the particular vulnerabilities of some disease groups: patients with multisystem diseases like multiple sclerosis (MS), Behçet disease or systemic lupus erythematosus (SLE), where it may take several years before a diagnosis is arrived at; patients with one of the so-called, "functional somatic syndromes" (under which umbrella term chronic fatigue syndrome and myalgic encephalomyeltis have often been bundled); patients with rare diseases; patients presenting with single or multiple chronic somatic complaints of uncertain aetiology whose symptoms were, in hindsight, prodromal [12].

Patients diagnosed with chronic fatigue syndrome or myalgic encephalomyeltis, or awaiting a diagnosis, may be particularly vulnerable to misdiagnosis with, or misapplication of, an additional diagnosis of bodily distress disorder. The authors are not persuaded that the S3DWG sub working group has incorporated adequate safeguards for these patient groups within its proposed criteria [9][13].

Recommendation: Insert exclusions under Bodily distress disorder for concept title Chronic fatigue syndrome and concept title Myalgic encephalomyeltis to mitigate the risk of misdiagnosis with, or misapplication of, an additional diagnosis of bodily distress disorder. Insert exclusions for Bodily distress disorder under concept title Chronic fatigue syndrome and concept title Myalgic encephalomyeltis.


4.4 Potential for conflation of bodily distress disorder and bodily distress syndrome:
One of the authors has demonstrated how the S3DWG's suggested name for the proposed replacement for the ICD-10 somatoform disorders has a history of usage in the literature and in the field interchangeably with that of the already operationalized, divergent Fink et al. (2010) disorder construct, and has raised the alarm for the diagnostic and statistical implications for confusion and conflation between the two, and the difficulties for maintaining construct integrity, within and beyond ICD-11 [14].

The Fink et al. (2010) "bodily distress syndrome" disorder construct, already in use in Denmark and several other EU countries, in research and clinical settings, is differently conceptualized, has a very different criteria set and is intended to capture a different patient population.

Fink et al. (2010) consider their "bodily distress syndrome" disorder construct has the ability to capture the ICD-10 somatoform disorders, neurasthenia, "functional symptoms", noncardiac chest pain, chronic pain disorder, MCS and some others, but to also subsume chronic fatigue syndrome, myalgic encephalomyeltis, fibromyalgia and irritable bowel syndrome (well described categories that are discretely classified in other chapters within ICD-10, ICD-11, and SNOMED CT), under a single, unifying "BDS" diagnosis [15][16].

Since researchers, clinicians and commissioners of services already do not differentiate between these two terms (and in some cases, one observes the conflations, "bodily distress syndrome or disorder" and "bodily distress syndrome/disorder"), these patient groups will be especially vulnerable to being misclassified.

References:

1 Proposal: Bodily distress disorder, ICD-11 Beta drafting platform Proposal Mechanism, December 30, 2014. http://bit.ly/2n86iNN

2 Proposal: Fatigue, ICD-11 Beta drafting platform Proposal Mechanism, December 30, 2014. http://bit.ly/2nrg1Sw

3 Content Model Reference Guide ICD-11 Revision, World Health Organization Geneva, 2011 (p. 23, 4.3 Exclusion Terms). http://www.who.int/classifications/icd/revision/Content_Model_Reference_Guide.January_2011.pdf

4 Summary Report, Third Meeting of the JLMMS Task Force Cologne, Germany, 11-14 April 2016 (p. 12, 10.5 Exclusion Types). http://www.who.int/entity/classific...016.04.11-14_iSummaryMeetingReportCologne.pdf


Page 9

5 ME/CFS: A Primer for Clinical Practitioners 2014. (p. 13). http://iacfsme.org/portals/0/pdf/Primer_Post_2014_conference.pdf

6 Correspondence: Health and Social Care Information Centre (now NHS Digital), FORWARD-ME March 20, 2015. http://www.forward-me.org.uk/Reports/SNOMED.pdf [Accessed: March 16, 2017]

7 Correspondence: UK Terminology Centre (UKTC) (now NHS Digital), Suzy Chapman, November 20, 2015.

8 Creed F, Gureje O. Emerging themes in the revision of the classification of somatoform disorders. Int Rev Psychiatry. 2012 Dec;24(6):556-67. doi: 10.3109/09540261.2012.741063. [PMID: 23244611]

9 Gureje O, Reed GM. Bodily distress disorder in ICD-11: problems and prospects. World Psychiatry. 2016 Oct;15(3):291-292. doi: 10.1002/wps.20353. [PMID: 27717252]

10 American Psychiatric Association. (2013). Somatic Symptom and Related Disorders. In Diagnostic and statistical manual of mental disorders (5th ed.). Washington, DC: Author.

11 Frances A, Chapman S. DSM-5 somatic symptom disorder mislabels medical illness as mental disorder. Aust N Z J Psychiatry. 2013 May;47(5):483-4. [PMID: 23653063]

12 Frances A. The new somatic symptom disorder in DSM-5 risks mislabeling many people as mentally ill. BMJ. 2013 Mar 18;346:f1580. [PMID: 23511949]

13 Frances A. DSM-5 Somatic Symptom Disorder. J Nerv Ment Dis. 2013 Jun;201(6):530-1. [PMID: 23719325]

14 Proposal: Bodily distress disorder, ICD-11 Beta drafting platform Proposal Mechanism, March 2, 2017. http://bit.ly/2nWnyKd

15 Fink P, Toft T, Hansen MS, Ornbol E, Olesen F. Symptoms and syndromes of bodily distress: an exploratory study of 978 internal medical, neurological, and primary care patients. Psychosom Med. 2007 Jan;69(1):30-9. [PMID: 17244846]

16 Fink P, Schröder A. One single diagnosis, bodily distress syndrome, succeeded to capture 10 diagnostic categories of functional somatic syndromes and somatoform disorders. J Psychosom Res. 2010 May;68(5):415-26. [PMID: 20403500]


[This completes the "Detailed Explanation of Proposal" section. I will post the Rationale section later.]
 
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Suzy Chapman Owner of Dx Revision Watch
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A note about Tuesday's anticipated Frozen Release:

On Tuesday April 4, ICD Revision is anticipated to release a frozen version of the Beta draft for field testing by centres that have signed up to participate in testing the utility of ICD-11.

For these purposes, a stable version of the Beta (which changes daily, as new terms are added and category groups are shunted around) is required.

ICD Revision periodically releases frozen versions for testing purposes. This is not the same as the field trials on selected individual categories that were carried out in small numbers of centres between 2011 and 2016.

For those registered for increased interaction with the Beta platform, there is a page in the draft where Frozen Versions are posted:

Log in (or Register, if you don't already have access).

Hit Info tab (far right) > Select Frozen Releases (last in the dropdown menu). You should arrive here.

http://apps.who.int/classifications/icd11/browse/frozenreleases

Frozen Releases
See what's changed since the last frozen release (2015-05-31)


Since nothing to the contrary has been announced by ICD Revision, I am assuming that the April 4, 2017 Frozen Release will be posted on the above page.

While field testing is being undertaken over the next couple of months, the Beta draft will continue to be accessible and will continue to be updated on a daily basis. TAG group chairs, TAG managing editors and stakeholders can continue to submit proposals via the Proposal Mechanism, make comments on outstanding proposals or comment on existing listings in the Beta Foundation and MMS Linearizations.


The opaque responses given to inform the Minister's Written Response to the two Parliamentary Written Questions submitted by the Countess of Mar, in February, appear in part to refer to my own proposals of December 2014 for exclusions under Fatigue.

There was a deadline of December 30, 2016 for proposals for consideration for the frozen version March 2017.

Logically (though I see little evidence of logic out of Geneva this last few years), there are these options:

1 TAG Neurology missed the December 30 deadline for inclusion in the April 4 Frozen Version and so the terms won't be included in the Frozen Version.

2 The Frozen Version will contain the terms as they were restored to the draft on March 26 with, one assumes, my proposals for inclusions under Fatigue (or the two out of three that have been approved so far) because they have been sitting in that location in the non public version of the draft since between early 2013 and March 26.

3 The Frozen Version may contain different proposals to the locations and hierarchy as restored on March 26 (which would mean that the Beta as it stands today, will be at odds with the Frozen Version).

That would also call into question the point of the caveat posted on March 26:

“While the optimal place in the classification is still being identified, the entity has been put back to its original place in ICD.”

Team WHO 2017-Mar-26 – 12:46 UTC
My guess is, the Frozen Version will most likely contain the terms as restored on March 26.

I will check the Beta platform on Tuesday and review any new Frozen Version that has been posted overnight and update the thread.
 
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Now that the terms have been restored to the Beta draft (albeit with a caveat) I will pass on what Dr Christopher Chute told me, on February 22; that is, on the last day of the face to face Joint Task Force meeting in Cologne (February 20-22).

Bear in mind that Dr Jakob and the Joint Task Force co-chairs have been asked to confirm whether this information was correct, still stood and to which of the three terms it applied and that neither have been willing or bothered to confirm, deny, clarify or elaborate.

On February 22, I was advised by Dr Chris Chute (who I imagine had not been deputed as a spokesperson for the Joint Task Force but possibly considered that I ought to be informed. Dr Chute is chair of Revision Steering Group and lives in the U.S. He did not clarify whether he had attended the three day meeting in Cologne, in person, but he did say in a second email that this condition is not within his personal area of expertise or responsibility, so he could not give me informed answers to my specific questions):

a) that this had been discussed [on February 22] in Köln.

b) that "evidently, there are plans to include these terms as index entries."

c) that his advice would be to create a Beta draft proposal entry for these terms and/or

d) identify an existing draft proposal and add evidence.*

Now if this information had been correct, possibly they had been considering, at that point, to retain CFS as lead term but place BME and PVFS in the index.

Or retain CFS as lead term, with BME as inclusion and PVFS in the index.

Which might potentially explain why they have not approved my proposal for inserting PVFS as an exclusion under Fatigue (but have approved CFS and BME) because under either of the above hierarchies, CFS would be the lead (or concept title) term - not PVFS.

*That might also explain why Dr Jakob has seemingly been so focused on my proposals for exclusions under Fatigue.
 
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Suzy Chapman Owner of Dx Revision Watch
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Dr Chute went on to say:

That he appreciate my thoughtful and informed letter. However, he would urge me to work through the proposal system; that it may not surprise me that the “letter” campaign was neither appreciated nor effective; that he would suggest that for many, it may be counterproductive. That it is unlikely to be a way to foster transparency.* That as I may discover, the proposal system has status information and commentary. That he admitted the [Joint Task Force] are overwhelmed and handling of proposals after January 1 (Ed: ie that are submitted after the deadline for the Frozen Version) was going to be slow, but this issue (Ed: the issue of the absence of proposals for the G93.3 terms for four years) is on [the Joint Task Force's] radar. That they consider the proposal system, with its public rendering of comments and status, as relatively transparent and certainly more so than any historical ICD revision.

That he considered the most persuasive component of a proposal is scientific evidence around etiology. That he realized that this is somewhat thin for this common syndrome, which must be even more exasperating for those who suffer from the condition and their advocates. Nevertheless, this is what they will really weigh heavily.

That I/we also have to think about the long game.That what is produced for example in 2018 will continue to evolve, that ICD-11 is designed for graceful evolution. That it may differ in substantial ways by the time the first country implements it, say 5-6 years from now from what is put forth in 2018. That he thought I should "not panic." That this condition is not within his personal area of expertise or responsibility, so he could not give me informed answers to my specific questions.

(Though he did not say that as chair of Revision Steeing Group, whose members included all the TAG chairs, that he would endeavor to ensure that I did receive timely responses to my questions and requests for clarifications.)

*I practically choked on that bit - after four years of no transparency and continued obfuscation from WHO/ICD Revision.
 
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Suzy Chapman Owner of Dx Revision Watch
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Ironically, one of the professional bodies that was unhappy with recommendations for the dementia categories had also launched a "letter campaign" which involved contacting their international constituents to ask them to email WHO's Dr Saxena and also copy to those members of the working groups who lived in their own countries.

I wonder if these professionals were similarly stonewalled by Dr Saxena and the co-chairs of the JTF and told that their requests to open a dialogue with WHO/ICD "was neither appreciated nor effective and... would suggest that for many, it may be counterproductive."


ICD Revision must get fed up with all these pesky letter campaigns....

http://www.wpanet.org/detail.php?section_id=25&content_id=1921

World Psychiatric Association

(...)

4. You may know that there is a proposal to shift diagnosis of Dementia from Mental Health and Behavioural Disorders to Neurology section of ICD-11. I know that many organisations have written to Mental Health Division in the WHO. Please find attached herewith a letter to Dr Shekhar Saxena. If your organisation has not already written protesting this change please do so. I would suggest that please write to Dr. Saxena directly and copy the WPA in. It will strengthen the case if you were to contact your WHO country expert as well as your representatives on ICD-11 working groups.

5. I am also attaching a paper from Professor Graham Thornicroft and colleagues for your information and action.
 
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Suzy Chapman Owner of Dx Revision Watch
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All you ever wanted to know about the forthcoming field testing:

https://hscic.kahootz.com/gf2.ti/af/762498/122441/PDF/-/ICD11_FT_Information_and_ToE.pdf

ICD-11 Field Trials
Information and Terms of Engagement


17 March 2017


Also summary at:

https://hscic.kahootz.com/connect.ti/t_c_home/view?objectId=297939

"...The World Health Organization (WHO) is currently developing the 11th revision of ICD. Once endorsed by the World Health Assembly (WHA), WHO Nomenclature regulations stipulate that Member States must use the most current revision for mortality and morbidity purposes. For this reason and to allow member countries to adopt the new revision when they are ready, WHO will brief the WHA on ICD-11 in May 2018 but will not seek endorsement at this time."
 

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Hopefully the growing awareness of the PACE trial scandal might make them feel more nervous about all this? As you've been going at them for years, surely they don't want to ignore all you've done just as people in the medical research community wake up to the fact that ME activists conducting unappreciated campaigns had been right all along.
 

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Here are the remaining pages of our proposal and rationale:


Pages 9 to 14

The full Proposal and Rationale can be downloaded here, or there is a PDF attached to Post #137.

Page 9


Rationale

In July 2015, the authors provided Dr Robert Jakob (WHO, ICD-11 MMS Joint Task Force) and Ms Anneke Schmider (ICD-11 Revision Project Manager) with background materials and literature sources on myalgic encephalomyelitis and chronic fatigue syndrome for the consideration of the Topic Advisory Group for Neurology and to inform the revision process. This section includes selected of those materials plus new materials that have been published since then.


1. Scientific evidence that exists for neurological dsyfunction: The document Evidence Supporting a Classification of ME and CFS in the Neurological Chapter [1] summarizes many of the neurological and neurocognitive studies in this disease going back to the early 1990s.

PDF: Evidence Supporting a Classification of ME and CFS in the Neurological Chapter 2017

http://bit.ly/2nPj8Vv

In addition to a list of relevant neurological studies, this document includes a 2011 review that summarized evidence of reduction in gray matter volume, reduction in blood flow in the brain, increases in brain lactate levels, changes on MRI and EEG, evidence of autonomic dysfunction, neuroendocrine dysfunction, the presence of abnormal proteins in the spinal fluid, and neurocognitive changes that include deficits in attention, memory and reaction time [2].

This document also includes a 2016 presentation by Professor AnthonyKomaroff, Harvard Medical School, in which he discussed more recent studies that demonstrated brain inflammation that varies with disease severity and also studies showing impaired functional connectivity in the brain [3]. In that presentation, Komaroff concluded with the hypothesis that "low grade brain inflammation causes the symptoms of ME/CFS and this involves a connection between the brain, the immune system and possibly in some people the gut." He went on to state that according to this hypothesis, "there are different factors that can trigger ME/CFS but the hypothesis goes they all end up causing low grade inflammation in the brain and that low grade inflammation causes the symptoms of the illness."


Page 10

References:

1 Evidence Supporting a Classification of ME and CFS in the Neurological Chapter 2017. http://bit.ly/2mwLvYE

2 Komaroff AL, Cho TA. Role of infection and neurologic dysfunction in chronic fatigue syndrome. Semin Neurol July 2011. http://dx.doi.org/10.1055/s-0031-1287654

3 Komaroff A. Presentation for U.S. disease organization Solve ME/CFS Initiative. November 2016.


Minute 14:23, 48:46.

Presentation slides:
http://solvecfs.org/wp-content/uploads/2016/11/SolveME-CFS-Webinar-Dr-Komaroff.pdf


2. Recent federal agency reviews and scientific evidence:
The Report of the U.S. Institute of Medicine (IOM), recently renamed, "The National Academy of Medicine," contains one of the most comprehensive literature reviews to date of the biomedical evidence for the disease [1]. The report concluded, as have numerous researchers, that the disease is a "serious, chronic, complex, and multisystem disease" that causes neurological, cognitive, immunological, autonomic, and energy metabolism impairment.

The Report further noted that the hallmark of the disease is post exertional malaise (PEM), where even very limited physical or mental exertion causes an exacerbation of all symptoms that can last for days, weeks or even months. As a result, the IOM concluded that "some patients diagnosed by other criteria, such as the Fukuda [CFS] definition, will not fulfill" the IOM's recommended diagnostic criteria. Noting the differences in inclusion and exclusion criteria between CFS and ME definitions, the IOM stated "a diagnosis of CFS is not equivalent to a diagnosis of ME."

The IOM Report firmly dismissed the idea that this is a psychogenic disease. The Report also noted that the disease can result in prolonged and significant disability with impairment in ability to engage in educational, occupational, social and personal activities; that there is a considerable economic burden associated with these conditions, with many patients unable to continue in employment or in mainstream education; and that 25% of patients are bed- or house-bound during their illness and will require high levels of personal care and assistance from family or social caregivers.


2.1 2014 AHRQ Evidence Review: An evidence review was carried out by the U.S. Department of Health and Human Services' Agency for Healthcare Research and Quality (AHRQ) in parallel with the IOM evidence review [2]. The 2014 AHRQ review included studies using the Oxford CFS Criteria, studies often based on the biopsychosocial theory that patients' symptoms are caused by fear-avoidant decreased activity and resultant deconditioning.

The AHRQ evidence review on the Diagnosis and Treatment of Myalgic Encephalomyelitis/Chronic Fatigue Syndrome was published in December 2014 and used as input by the 2015 National Institutes of Health Pathways to Prevention (P2P) Workshop on Advancing the Research on Myalgic Encephalomyelitis/Chronic Fatigue Syndrome [3].

The 2014 AHRQ review called for the Oxford CFS criteria to be retired because "it is at high risk of including patients who may have an alternate fatiguing illness, or whose illness resolves spontaneously with time." The NIH P2P report concurred.

In 2016, AHRQ reanalyzed the evidence for Oxford CFS Criteria studies separately from the remaining studies and as a result, issued an addendum that downgraded previous findings once Oxford studies were excluded.


2.2 Highlights of Recent Research:
In October 2016, the International Association for CFS/ME held its biannual conference which highlighted numerous scientific findings. As documented by Professor Anthony Komaroff of Harvard Medical School [4], presentations included studies on brain and nervous system, immunology, the microbiome, epigenetics, energy metabolism and metabolomics. Komaroff summarized that there was robust evidence of an underlying biological process involving a) the brain and autonomic nervous system, b) the immune system, c) energy metabolism, and oxidative and nitrosative stress. Given the scope of biological evidence, he concluded, "The illness is not simply the expression of physical symptoms by people with a primary psychological disorder."


Page 10


References:

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


Page 11

2 U.S. Agency for Healthcare Quality and Research. Research Review. Diagnosis and Treatment of Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome. Evidence Report/Technology Assessment Number 219. U.S. Agency for Healthcare Quality and Research. December 9, 2014. Addendum July, 2016. AHRQ Publication No. 15-E001-EF. https://effectivehealthcare.ahrq.gov/ehc/products/586/2004/chronic-fatigue-report-160728.pdf

3 Green CR, Cowan P, Elk R, O'Neil KM, Rasmussen AL. National Institutes of Health Pathways to Prevention Workshop: Advancing the Research on Myalgic Encephalomyelitis/Chronic Fatigue Syndrome. Ann Intern Med. 2015. http://annals.org/aim/article/23228...-advancing-research-myalgic-encephalomyelitis

4 Komaroff A. 12th International IACFS/ME Conference Emerging Science & Clinical Care. October 2016. http://iacfsme.org/Conferences/2016...6-Summary-Slides/IACFSME-Komaroff-Slides.aspx


3. Options and considerations: As noted above, the IOM expert panel concluded that a comprehensive review of the evidence demonstrates a serious, chronic, systemic disease with dysfunction in neurological, immunological, autonomic and energy metabolism systems.

In SNOMED CT, chronic fatigue syndrome, myalgic encephalomyelitis and other associated terms are already classified under the Parent: Multisystem disorder.


3.1 Classification of multisystem diseases within ICD-11: Previous editions of ICD have organized diseases into chapters based on aetiology or affected organ or body system. The architecture of ICD-10 does not facilitate scientific and taxonomical representation of diseases with varied manifestations that belong to or affect multiple body systems, for example, systemic lupus erythematosus (SLE) and Behçet disease, or where no single organ system predominates to guide chapter placement.

In 2010, the Revision Steering Group posted a discussion paper on the potential for incorporating within ICD-11 a new chapter for Multisystem diseases [1]. This proposal was subsequently rejected in preference to the consideration of generating a separate linearization from the Foundation, as a "virtual" multisystem chapter [2].

It is currently unclear whether ICD Revision intends to implement a multisystem linearization or whether diseases that would have been candidates for assigning to a "virtual" multisystem chapter will be represented solely through multiple parentage and the explicit listing in the content model of all body systems involved for that entity.

Regardless of how multisystem diseases will be handled in general in ICD-11, it is premature to suggest multiple chapter parentage at this time.

Noting the need for increased biological research, the director of the National Institutes of Health said in a recent article, "ME/CFS is such a complex condition, affecting so many body systems, that we do not know where the answers will come from." [3].

However, given what we do now know as documented above, the authors see no justification for any proposal for secondary parenting under the Symptoms, signs or clinical findings, not elsewhere classified chapter or Mental, behavioural or neurodevelopmental disorders chapter.

References:

1 Aymé, Chalmers, Chute, Jakob (2010). ICD Revision: Discussion paper: Multisystem Disorders. http://bit.ly/2nnilKW

2 WHO ICD Revision Information Note 19: Multisystem Diseases Chapter in ICD, January 29, 2013.

3 Koroshetz W, Collins F. Moving Toward Answers in ME/CFS. NIH Director's Blog. March 2017.
https://directorsblog.nih.gov/2017/03/21/moving-toward-answers-in-mecfs/



3.2 ICD-11 Joint Task Force position on precedence:
General considerations for potential chapter relocations were discussed at a meeting of the Joint Task Force, in July 2016 [1]. According to the meeting Summary Report (5.2 Key discussion), a general principle was reiterated that:

"...in the absence of compelling evidence mandating a change, legacy should trump with regard to the question of moving certain conditions to new chapters...JTF members confirmed that continuity over time is desirable.


Page 12


Where there is a rationale for change, the changes can be accommodated for, but there was a question about how to justify the effort required to make the changes in data reporting systems in the absence of compelling information indicating that the change makes things better or more accurate."


Although currently a disease of incompletely understood aetiopathogenesis, active research will inform decisions about potential chapter relocation or whether to assign multiple parents. For now, current scientific evidence of neurological impairment and WHO/ICD Revision's position on precedence supports retention of chronic fatigue syndrome and myalgic encephalomyeltis in Diseases of the nervous system.

References:

1 Fourth Meeting of the JLMMS Task Force, Queensland, Australia, 11-14 July 2016
http://www.who.int/entity/classific....07.11-14_iSummaryMeetingReportQueensland.pdf


3.3 Consideration of creating a sub class under: Other disorders of the nervous system:
Until such time that further research supports the creation of, or assignment to a more specific parent class, either within Diseases of the nervous system, or under another chapter, or assignment to multiple parents, the recommendation is to retain chronic fatigue syndrome and myalgic encephalomyeltis as siblings under Other disorders of the nervous system.


3.4 Assigning unique codes for chronic fatigue syndrome (CFS) and myalgic encephalomyeltis (ME): In ICD-10, all three terms are coded or indexed to the same code. The IOM Report, the AHRQ Evidence Report, the IACFS/ME Primer for Clinical Practitioners [1] and studies on exercise testing [2] all highlight the importance of post exertional malaise (PEM) as a hallmark symptom, both clinically and in research.

As a result, the IOM specified a clinical criteria that requires PEM, a feature not required for CFS criteria. In addition, researchers internationally are increasingly using the Canadian Consensus Criteria (CCC) [3] or the ME International Consensus Criteria (ME-ICC) [4] - both of which require PEM - to progress research into disease pathology, subtypes, diagnostics, and treatments.

Notably, the IOM Report compared the CFS definitions with the CCC and the ME-ICC and as noted previously, concluded that a CFS diagnosis is not equivalent to an ME diagnosis.

Therefore, the recommendation is to place both chronic fatigue syndrome and myalgic encephalomyeltis in the same location under parent class: Other disorders of the nervous system, and to assign a unique code to each.

The authors would not support precedence being given to chronic fatigue syndrome as the new concept title, with myalgic encephalomyeltis as the inclusion term, synonym term, or consigned to the Index.

References:

1 ME/CFS: A Primer for Clinical Practitioners 2014.
http://iacfsme.org/portals/0/pdf/Primer_Post_2014_conference.pdf

2 Keller B, Pryor J, Giloteaux, L. Inability of myalgic encephalomyelitis/chronic fatigue syndrome patients to reproduce VO2peak indicates functional impairment. Journal of Translational Medicine April 2014, 12:104. PMID: 24755065. http://dx.doi.org/10.1186/1479-5876-12-104

3 Carruthers BM, Jain AK, De Meirleir KL, Peterson DL, Klimas NG, Lerner AM, Bested AC, Flor-Henry P, Joshi P, Powles et al. Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Clinical Working Case Definition, Diagnostic and Treatment Protocols. Journal of Chronic Fatigue Syndrome 2003; 11(1): 7-117.

4 Carruthers BM, van de Sande MI, De Meirleir KL, Klimas NG, Broderick G, Mitchell T, Staines D, Powles ACP, Speight N, Vallings R et al. Myalgic Encephalomyelitis: International Consensus Criteria. Journal of Internal Medicine October 2011; 270(4): 327–338. [PMID: 21777306]


3.5 Consideration of the IOM Report's recommendation for a new case definition and change of nomenclature:
The IOM expert panel members were charged with developing clinical diagnostic criteria and making recommendations for a new disease name. The IOM recommended that the term, "chronic fatigue syndrome" should not be used for those patients who meet the IOM's diagnostic criteria and instead, proposed that the term be replaced with "Systemic Exertion Intolerance Disease (SEID)."

Experts and patients have raised concerns over the suggested new name [1].


Page 13

No proposals have been submitted to the U.S. Coordination and Maintenance Committee to add "Systemic Exertion Intolerance Disease (SEID)" to the U.S. specific ICD-10-CM. Instead, the CDC has begun to use the hybrid term "ME/CFS" [2] and intends to use that term as it implements the IOM clinical diagnostic criteria. Other agencies within the U.S. Department of Health and Human Services, including the National Institutes of Health, are already using this term and not "SEID" [3].

Therefore, the authors consider there is currently insufficient evidence or support for incorporating the proposed "Systemic Exertion Intolerance Disease (SEID)" definition into ICD-11, either as a replacement for one or more of the G93.3 legacy terms or for insertion as an inclusion term, synonym term or index term.

References:

1 Jason LA, Sunnquist M, Brown A, McManimen S, & Furst J. (2015). Reflections on the Institute of Medicine’s systemic exertion intolerance disease.Polskie Archiwum Medycyny Wewnetrznej. 125(7-8), 576–581. [PMID: 26176405]

2 Unger E, Lin J, Brimmer D, Lapp C, Komaroff A, Nath A, Laird S, Iskander J. CDC Grand Rounds: Chronic Fatigue Syndrome — Advancing Research and Clinical Education. MMWR Morb Mortal Wkly Rep 2016. http://dx.doi.org/10.15585/mmwr.mm655051a4

3 A Report from the Federal Partners Meeting of the National Institutes of Health Pathways to Prevention Workshop: Advancing the Research on Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS). May 2016. https://prevention.nih.gov/docs/programs/p2p/mecfs-federal-partners-report.pdf


4. Definitions: There are 20 or more CFS and ME definitions, which have been conflated into a single evidence base, with evidence from one definition being assumed to apply to patients meeting any definition [1][2].

PDF: Overview of key definitions used for ME and CFS 2017

http://bit.ly/2niiKfu

Yet, as noted in published research and by U.S. government reports published in 2015-2016, these definitions have significant differences in inclusion and exclusion criteria [2][3][4].

The CFS definitions all require medically unexplained chronic fatigue lasting 6 or more months and either do not require other symptoms or else require one additional or any combination of 4 of 8 common symptoms. As a result, the CFS definitions do not require symptoms recognized by the IOM Report as hallmark symptoms for this disease while allowing the inclusion of primary psychological illness.

The 1991 Oxford definition has most often been used in studies based on a psychogenic disease model. The 1994 Fukuda definition has more often been used for biomedical research. As noted above,U.S. government reports stated that the Oxford CFS definition included patients with other fatiguing illnesses and noted that not all Fukuda CFS patients will meet the IOM’s new clinical diagnostic criteria.

The ME definitions (the 2003 Canadian Consensus Criteria (CCC) uses the hybrid term ME/CFS) and the 2011 ME International Consensus Criteria (ME-ICC) both require hallmark symptoms of the disease such as PEM, sleep dysfunction and neurological/cognitive manifestations and also highlight immunological impairment [3].

Expert clinicians use the CCC in clinical practice; researchers have increasingly used the CCC or the ME-ICC in published research, and the U.S. National Institutes of Health is using the CCC in its current intramural study.

The Institute of Medicine (now the "The National Academy of Medicine") has published new clinical diagnostic criteria, which are based on the Canadian Consensus Review and also require hallmark symptoms such as PEM. The IOM clinical diagnostic criteria have not been officially implemented in U.S. clinical practice and are not in use in other countries.

At this time, there is no single CFS or ME definition that is used worldwide. However, it is possible to describe the core features specified in the CFS and ME definitions.

Definitions

CFS and ME have been historically treated as synonymous but the case definitions encompass different populations.

CFS Definition Features: According to commonly used CFS definitions, CFS is medically unexplained chronic fatigue that is disabling and lasts six months or more for adults, (3-4 months for pediatric). No other symptoms are specifically required, although some CFS definitions do require an optional combination of other common symptoms. CFS is most often described as fatigue that does not get better with bedrest.

ME Definition Features: According to commonly used ME definitions, ME is a serious, chronic, complex, and multisystem disease that causes neurological, cognitive, immunological, autonomic, and energy metabolism impairment. It is characterized by the hallmark post-exertional malaise in which even small amounts of physical and mental exertion causes an often-delayed exacerbation of all symptoms that can last for days, weeks or even months. Onset can follow an infectious trigger, which is often but not always viral.

References:

1 Brurberg K, Fønhus A, Larun L, Flottorp S, Malterud K. Case definitions for chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME): a systematic review. BMJ Open. February 7, 2014; 4(2): e003973. http://dx.doi.org/10.1136/bmjopen-2013-003973

2 Jason LA, So S, Evans M, Brown A, Sunnquist M, Im Y, Schafer C. An overview of operationalizing criteria for ME, ME/CFS, and CFS case definitions. Prev Interv Community. 2015;43(1):1-4. [PMID: 25584523]

3 National Academy of Medicine. Beyond Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Redefining an Illness. The National Academies Press; May 2015. https://www.nap.edu/catalog/19012/beyond-myalgic- encephalomyelitischronic-fatigue-syndrome-redefining-an-illness

4 U.S. Agency for Healthcare Quality and Research. Research Review. Diagnosis and Treatment of Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome. Evidence Report/Technology Assessment Number 219. U.S. Agency for Healthcare Quality and Research. December 9, 2014. Addendum July, 2016. AHRQ Publication No. 15-E001-EF. https://effectivehealthcare.ahrq.gov/ehc/products/586/2004/chronic-fatigue-report-160728.pdf

5 Overview of more commonly used myalgic encephalomyelitis and chronic fatigue syndrome definitions. http://bit.ly/2niiKfu

[Ends]


[List of 45 links for aggregated references]

[Rationale field]

The Rationale for this Proposal has been appended to the Detailed Explanation for improved accessibility for a stakeholder patient group, some of whom may experience cognitive impairment and visual problems when viewing unformatted plain text manuscripts on computer screens.

A copy of this Proposal and Rationale can be accessed here in PDF format: http://bit.ly/2nLdvHy

[Ends]
 
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Dx Revision Watch

Suzy Chapman Owner of Dx Revision Watch
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Two Summary posts on my Dx Revision Watch site:

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A proposal for the ICD-10 G93.3 legacy terms for ICD-11: Part Two
http://wp.me/pKrrB-4eH
http://wp.me/pKrrB-4eH

and for background to the G93.3 and ICD-11 issue:

A proposal for the ICD-10 G93.3 legacy terms for ICD-11: Part One http://wp.me/pKrrB-4dV
http://wp.me/pKrrB-4dV

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Dx Revision Watch

Suzy Chapman Owner of Dx Revision Watch
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Frozen Release?

Nada. Not here anyway:
http://apps.who.int/classifications/icd11/browse/frozenreleases


Dx Revision Watch‏@dxrevisionwatch 2m2 minutes ago

.@WHOICD11 At what URL can stakeholders access the April 4, 2017 Frozen Release, thank you?


Just to recap on what we have been told about an April Frozen Release:

"(...) The WHO has also confirmed that the proposal [unspecified, but likely refers to my proposals for exclusions under Fatigue for PVFS, BME, CFS] submitted for chronic fatigue [sic] is currently with the relevant groups of the organisation to consider the scientifically-based placement of this condition in the classification. This will be included in the next version of ICD-11 to be released on 4 April 2017 for field testing."

February 27, 2017, Parliamentary Written Answer, Department of Health
HL5683


"The World Health Organization (WHO) has reaffirmed with NHS Digital that the proposal [unspecified] submitted on the ICD-11 platform for chronic fatigue [sic] is currently with the relevant groups of the organisation to consider the scientifically-based placement in the classification. We understand that the WHO are still reviewing this matter and expect that the next iteration of ICD-11, expected in April 2017, will reflect the WHO’s conclusions."

March 28, 2017, Parliamentary Written Answer, Department of Health
HL6136


Dr Robert Jakob to Suzy Chapman, personal correspondence, March 1, 2017:

"Further to our earlier correspondence and phone calls, including contacts with other parties linked to chronic fatigue [sic], I reiterate that WHO appreciates the work that you and others have put in to chronic fatigue [sic].

"So far, you submitted 6 proposals linked to 3 entities on the proposal platform. All were submitted in time to be addressed for the field testing version that will be released on 4 of April."​

Will update if any frozen release package is posted on the Frozen Release page today.

http://apps.who.int/classifications/icd11/browse/frozenreleases
 
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Joh

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Thank you so much for everything, Suzy! (My English is lacking this morning, please insert witty jokes about villains with frozen hearts and frozen releases and our princess Suzy fighting and shouting "let it go" and a happy ending here. And excuse me if this is too childish.)