Clark Ellis brings us Part 2 of an interview with Dr. Lucinda Bateman, where she answered questions posed by the patient community …
The Institute of Medicine recently published its report into myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). One of the committee members, Dr. Lucinda Bateman, graciously agreed to answer questions submitted by members of the patient community.
Questions were submitted on the Phoenix Rising forum and they can all be viewed here.
Questions have been arranged roughly by topic and have been published in two parts. Part 1 can be found here. Part 1 covered questions on the committee and IOM process, and the IOM’s diagnostic definition.
This second part covers the clinicians’ guide, the new disease name “systemic exertion intolerance disease” or SEID, that the IOM has proposed, international classification of diseases (ICD) coding, and miscellaneous topics.
It’s known that the public and the medical community take illnesses with a more “medical-sounding” name more seriously. ME/CFS has suffered from this phenomenon. Why didn’t the IOM committee go with a more “medical-sounding” name?
Historically, some illness names are/were eponyms — meaning the illness was named after a person or place. Examples of this are naming the illness after the person who first published a description of the illness, or a patient (Lou Gehrig’s disease), or a place the illness was identified (Lyme disease).
It has always been considered bad form within scientific and medical communities to name an illness after oneself.
In the mid 1970s an article in the Lancet discouraged the use of eponyms, and the idea was gradually accepted, although not without controversy; it is still a problem and the World Health Organization just this month urged more care in the naming of diseases. I was taught in my residency training (1987-91) to stop using the eponyms I learned in medical school and replace them with descriptive names.
So, the modern trend in medicine has been to name conditions in a way that either describes the cause or the primary signs of illness. The biggest risk of naming an illness with a description of the cause is that it will be proven incorrect. These factors all played a role in the decision of what to re-name CFS.
The words “systemic” and “disease” are well-established medical terms. The words “exertion intolerance” describe not only a main presenting feature of the illness, and perhaps the aspect of illness most often overlooked or minimized, but also comes straight from the term “post-exertional malaise” found in all of the previous case definitions.
There are a number of reasons the IOM committee chose a new name over using the term ME, but in my opinion, the most important reason is that the SEID diagnostic criteria, drawn from the published data, are not identical to the description of ME.
Calling it ME would have created even more confusion, giving the same name to an illness complex described in two different ways. Either way, the intent of the IOM report is to have more physicians diagnose the illness and take care of patients, with a goal of reviewing the criteria as research unfolds. The identification of bio-marker defined subsets might lead to even more names in the future.
Although post-exertional malaise (PEM) is the distinguishing symptom of this illness, many patients feel very ill and disabled even if they have no exertion. Doesn’t the suggested name lead to the misconception that resting patients don’t still feel terribly sick? What must be done to avoid this misconception taking hold?
This misconception might occur from rushing to a judgement about what “exertion” means, rather than understanding its use and meaning in the diagnostic criteria and the entire document. Exertion is described many times, and even within the first few pages: (IOM report page 11)… “exertion of any sort—physical, cognitive, emotional—can adversely affect these patients in many organ systems …”
So, if exertion is viewed as intended, it’s not possible for a patient to be “feeling very ill and disabled even if they have no exertion.” The only way to avoid exertion completely is to stop thinking and feeling, no longer cognate or experience an emotion. Every patient knows that cognitive and emotional stress (i.e., exertion) can trigger PEM. We all know that PEM is an important component of this illness.
I will also note that no disease description can possibly span the range of illness severity. For example, there is a spectrum of illness in patients with MS ranging from complete remission, the appearance of normal while quite symptomatic, to being visibly crippled and wheelchair bound. Do they not all deserve the term “multiple sclerosis” to describe their disease?
One large concern that patients are focused on is how the name will play among the general public (‘I can’t tolerate exertion either!’). And how will clinicians interpret the name?
Certainly there have been examples of people in public media joking about the new name, but I doubt those uninformed opinions will have any lasting significance. Obviously the people who have had the most problem with the new name are those who are/were loyal to the term ME, especially since it has been widely used outside the U.S.
The term ME has played an important role in the U.S. advocacy effort to develop more respect and recognition for the illness, and was perhaps seen as the most realistic solution for abandoning the stereotyped and derogatory term Chronic Fatigue Syndrome.
In general, the medical community is fairly neutral about the name, never paid much attention to CFS anyway, and tends to focus more on evidence-based diagnostic criteria than a name. I think it won’t take long for the new name and criteria to be routinely used and taught, if an effort is made to disseminate the report.
The most important next step is to teach physicians to recognize and diagnose the illness based on the rich information in the evidence-informed report, not based on hearsay or opinions. It would be a shame if the entire effort is stalled or even destroyed by the very people it was intended to help.
The longer we go on without movement toward aggressive dissemination, the more likely it is the entire effort will fade back into oblivion.
We should ride the wave of publicity provided by the report release, not wait for everyone to forget about it and go on ignoring patients with this tragic and disabling illness. We should hold DHHS to the task of following through with the plan of dissemination, not allow them to duck the responsibility while we are all squabbling over the name.
In your comment on the IOM for the M.E. Global Chronicle, you stated:
“I don’t recall anything in the IOM report that states the term Myalgic Encephalomyelitis, or ME, can not be used to describe someone who meets published ME criteria. The recommendation is stop using ME/CFS.”
Can you clarify what you meant by that statement?
It’s not complicated — but apparently seems to be problematic for some. It’s not what I “meant” but what is stated in the report. There isn’t anything in the report that tells anyone they cannot use ME criteria or use the term ME. The IOM committee was charged to review the literature (and that includes the ME and CFS literature), use the highest quality data to design evidence-based diagnostic criteria for use by clinicians, and recommend whether new terminology for the term ME/CFS should be adopted.
Remember, within the U.S., the term “ME” was gradually linked to the term “CFS”, by advocates, by CFS researchers, by the IACFS, and eventually the federal agencies, to create the hybrid term ME/CFS. The term SEID will describe illness defined by the SEID diagnostic criteria and hopefully the term CFS will become history.
All criteria in existence define slightly different groups and subgroups with overlapping symptoms. None of the criteria are perfect. We can argue all we want about what to call an illness or subgroups of illness, but no one will be able to definitively prove their argument until we have objective tests. Even with objective tests it can be difficult to diagnose an illness correctly.
Think about rheumatology (lupus or not?) and neurology (MS or not?) as examples. The important thing is to recognize illness, care for patients, and get better at doing research in order to move the field forward.
The name ‘CFS’ has been a terrible burden upon patients. We are the ones who must carry the consequences of any new name. Did the IOM committee have the option of conducting a wide consultation exercise with patients? [original wording and context]
No they did not. It simply was not what the IOM was asked to do. But honestly, I think the IOM committee did everything possible to gather opinions from the public/patient community. They collected and studied hundreds of pages of references, personal experiences and descriptions of the illness, and compiled an exhaustive list of suggested names (70+). The report also does a great job of describing how the name CFS has been a terrible burden upon patients.
The Clinicians’ Guide
a) Do you think the emphasis on EBV while not naming any other infections will affect Social Security Agency (SSA) benefits when SSA had included human herpesvirus 6 and other infections on the list of test results which could be used to support a diagnosis of CFS? [original wording and context]
b) I worry that the emphasis on EBV might trigger old stereotypes and lead to doctors missing reactivation of other very important viruses. Are the other viruses our doctors commonly test for (HHV-6, Coxsackie, CMV, etc.) not supported in the evidence enough to be included in the clinician’s guide?
No. The report doesn’t say that EBV is the only infection that causes ME/CFS or SEID. It says that the existing evidence base is the strongest for EBV. Those are different concepts.
Do you agree with my view that the clinician’s guide seems to leave out the experiences of severely ill patients? How will it be communicated to physicians that some patients may have great difficulty even getting to their offices and that there is a population of patients trapped at home, perhaps bedbound, who have more severe symptoms, and perhaps a different presentation than the disease described in the clinician’s guide? [original wording and context]
I anticipate that as awareness and acceptance of ME/CFS or SEID grows in the general medical and scientific community, we will have more funding, studies and publications. More emphasis on the severely ill will be important.
Why was the delay in peak of PEM not more emphasized in the clinicians’ guide? Doesn’t a lack of emphasis of the “post” in post-exertional malaise risk confusing the PEM of SEID with other forms of exertion intolerance that may be more immediate? [original wording and context]
This is beyond the scope of the current evidence base and the IOM task. Many more studies are needed. As a clinician, I will say that most other illnesses characterized by exertion intolerance are readily diagnosable.
If I understand the report correctly, it says that any physician should be able to diagnose ME/CFS/SEID patients. But don’t ME/CFS patients in fact need biomedical specialist care?
Statements from patients and primary physicians alike make it clear that ME/CFS does not belong to primary care. A specialist is needed, especially for treatment. For example, at the Invest in ME Conference in London 2013, Dr. Clare Gerada, head of Royal College of General Practitioners stated:
“General practitioners, as generalists, ‘cannot know a lot about everything’ and when faced with a ‘chronic disease with such levels of disability’ can only refer their patients to a specialist. GPs simply cannot provide the care that ME patients need, she explained, ‘That takes skills, and resources, and where are the experts? How, if there are no specialists’ can GPs provide for the needs of ME patients?”
Isn’t it of utmost importance that ME/CFS patients get access to biomedical specialist care?
This question is complicated by the different use of language across continental divides. First of all, I am a general internal medicine specialist, board certified and trained to manage the basics of severe chronic illnesses across the fields of cardiology, endocrinology, gastroenterology, pulmonary, nephrology, geriatrics, adolescent medicine, and to manage quite a bit of neurology, psychiatry, pain medicine, etc . I am considered a primary care provider in the U.S.
I think general internal medicine specialists are probably better trained and prepared than a family practice provider, for example, for dealing with complex chronic illness. So, I’m not quite sure what constitutes a “GP” outside of the U.S. I can’t speak for systems outside the U.S. because I am not familiar enough with them.
That said, in the U.S. system there is no medical specialty that trains physicians and scientists to diagnose and treat ME/CFS or SEID. Even fibromyalgia, an illness with 3 FDA-approved drugs and a broad literature, has no “medical home” within a specialty. Rheumatologists are divided about whether they will even care for a patient with FM, and yet this is the field from which most of the research emerged.
So, while this illness is complex and challenging, it must be initially diagnosed and probably managed in the realm of primary care, with referral to appropriate specialists where possible. So, it’s fine to say that patients should be cared for by specialists … but there aren’t any, exactly.
ME/CFS “specialists” in the U.S. are people from various fields of medicine and science that developed an interest and become “self-taught”. Leading specialists in the U.S. are infectious disease specialists (with no proven infection to treat), general internal medicine specialists, pediatricians, immunology/infectious disease, and a rare neurologist. There are a few very good rheumatologists and psychiatrists who specialise in FM, but many more in their fields are not prepared to offer high quality care.
One of the goals of “Centers of Excellence” in the U.S. is to provide a place for physician training, along with clinical care and research. But until such centers can receive substantial funding and support, it is unlikely they will significantly impact the deficit of well-trained specialists.
This is another argument for the support of the new diagnostic criteria. While the IOM report is aimed at primary care providers for initial diagnosis, it is likely that there will emerge an increasingly aware group of specialists who are asked to evaluate orthostatic intolerance, cognitive impairment, sleep dysregulation, neuroendocrine and neuroimmune symptoms, and the physiologic causes of reduced function, activity intolerance and post-exertional malaise. We might not need “a specialist” but rather a team of specialists, until we can get to the root of the illness and develop early interventions.
What does the committee see as the difference between SEID and fibromyalgia?
The committee didn’t review the literature on fibromyalgia, except where it is referenced or included in the ME and CFS literature, nor did it make a statement about differences between FM and SEID. This comes back to my discussion above about how syndrome criteria create various overlapping groups rather than delineate completely separate illnesses.
Fibromyalgia is a syndrome defined by the presence of chronic widespread pain and tenderness, specifically, hyperalgesia (pain amplification) and allodynia (nonpainful stimuli amplified to pain), and is not considered to be a diagnosis of exclusion, but rather a condition commonly present with a number of illnesses, including ME and CFS. The literature demonstrates that there are people who meet FM criteria, people who meet CFS criteria, and people who meet both FM and CFS criteria — and the latter group is the most severely impaired group.
In my opinion, most who meet ME criteria would also meet FM criteria (severe central sensitivity, sensory amplification, etc.), depending on how one interprets the criteria. So … back to the problem of overlapping symptom-defined criteria.
The SEID criteria are particularly less focused on the pain symptoms, but that doesn’t mean having pain excludes one from the diagnosis of SEID. It is also possible and useful to clearly state that a patient meets both SEID and FM criteria, for example. Think of the criteria as describing aspects of the illness presentation in different individuals. Some have severe multiple chemical sensitivities (MCS) and some do not, etc.
Under its Comparison of Existing Diagnostic Criteria, the IOM report states, “While all of the criteria make clear that they are describing the same illness, some vary in the terminology used to refer to the illness or to specific symptoms.”
The AHRQ Evidence Report, however, finds to the contrary:
“The case definitions overlap but vary greatly in their symptom set, leading to concern that they do not all represent the same disease or identify the same cohort of patients.”
The IOM Report acknowledges that patients who fulfil the International Consensus Criteria have “more severe functional impairment and more physical, mental, and cognitive problems than those that fulfil the Fukuda definition.” But it fails to report that the International Consensus Panel of experts recommends:
“Individuals meeting the ICC have myalgic encephalomyelitis and should be removed from the Reeves empirical criteria and the National Institute for Clinical Excellence (NICE) criteria for chronic fatigue syndrome.”
ME and CFS are also classified as mutually exclusive ICD-10-CM diagnoses.
Why did the IOM not separate ME from CFS in a similar way to the ICC? [original wording and context]
Apologies to the authors, but all of the questions above, in my opinion, are just confusing. As I have said above, arguing about these subjectively-based case definitions is an intellectual exercise but not very practical. By definition, different sets of criteria will describe slightly different groups of people. Broad nonspecific symptoms, like fatigue, pain, cognitive changes, sleep disturbances, dizziness, etc., are not related to just one physiologic process or disease, so until we have specific biomarkers, we’re going to have to live with some overlap.
We should think about why we care about the criteria. The end purpose is to identify sick patients, offer the best possible care, and accomplish progress with research. This can be done in a number of ways. The reasons we have made slow progress in the field are complex, but some of the problem has been the case definitions and a particularly thorny presentation of illness that eludes our current abilities to measure and image.
International Classification of Diseases (ICD) Coding
(a) Could the references within the report to WHO ICD, Tenth Revision please be amended to reflect the fact that the U.S. will be implementing an adaptation of ICD-10 which is known as ICD-10-CM, which is the responsibility of NCHS?
(b) Could the link given in the report (on page 27) to a commercial data scraping website please be amended to the official current release of ICD-10-CM (FY 2015 release) that is available for download from the CDC’s website at: https://www.cdc.gov/nchs/icd/icd10cm.htm
(c) The report states, “In the World Health Organization’s International Classification of Diseases, Tenth Revision … the clinical descriptions of ME and CFS are identical …” However, there are no clinical descriptions or definitions of ME, CFS or PVFS within either the WHO’s ICD-10 or within the U.S. ICD-10-CM (FY 2015 release). Could this be amended please?
(d) Which agency would be taking the lead in drawing up and submitting a request for consideration of a new code (or new inclusion term) to the ICD-10-CM Coordination and Maintenance Committee that oversees the revision and update process?
e) If obtaining an ICD code for SEID will take time (at least one year or longer) won’t this cause providers in the meantime to use the IOM proposed clinical diagnostic criteria to identify patients with this disease, but then force them to continue coding it as CFS or ME? [original wording and context]
f) In order for a new code to be added to a medical dictionary such as ICD, SEID needs to be placed within a particular chapter – e.g. neurological, immunological, etc. Did the IOM panel have a recommendation on where it should go?
g) Did the IOM committee receive instructions or recommendations from a sponsor to address/consider whether the ICD code for this illness be changed? If yes, was this instruction/recommendation applicable only if a name change to the illness was placed? [original wording and context]
I am going to defer the questions about coding because I am not qualified to answer. The short answer is that to my knowledge the IOM committee was not asked to address coding, nor did it, other than to recognize that at some point coding would need to be addressed. It is my impression that our committee, as a U.S.-based non-profit contracted by DHHS, was generally referring to ICD-10-CM.
I do not contest the excellent and detailed questions and remarks about coding that have circulated after the release of the report. These comments should be directed to the appropriate coding bodies. This was a one-time contracted academic project, so I don’t know of a mechanism to “amend” the report. I was simply an invited expert to the committee. I’m not a member of the IOM.
As a clinician in private practice that personally codes each billing request, I will say that I have been successfully “creative” with coding for the last 25 years regarding this illness complex. We can all continue to manage while codes evolve. The science moving forward cannot be delayed by coding discussions.
The IOM’s report contained much that was encouraging to the patient community. What positive practical changes to the treatment patients receive does the committee hope will come out of it in both the short and long term?
Of course, there is nothing in the report about treatment. The report is limited to diagnostic criteria, so I will express my personal opinion to answer the question. Diagnosis is the first step toward treatment, and much of the suffering by patients comes from lack of proper diagnosis and routine supportive care. There are many ordinary things doctors can be doing to help patients. In addition, research can’t move forward very well until there are clear diagnostic criteria. Better treatment hinges on better research. So, it’s not just practical changes, but maybe pivotal changes regarding treatment that will come from the diagnostic criteria, if they are properly understood and applied.
The report states that the committee was struck by the “relative paucity of research,” and how “remarkably little research funding has been made available to study the etiology, pathophysiology, and effective treatment of this disease” and concluded that “More research is essential.”
Since the lack of research funding is clearly a major obstacle to tackling this disease, is there some specific advice you can give, please, to patients and patient groups on how they can best channel their efforts into ensuring that the desperately needed research is in fact carried out? What avenues or methods of advocacy would you recommend as those with the best chance of being productive of public funding? [original wording and context]
I’m sounding like a broken record, but my answer is that we can’t make much more progress than we’ve made in the last 20 years until we disseminate and adopt the new diagnostic criteria. The IOM report is the type of change needed to influence and change academic institutions, pharmaceutical companies and federal agencies.
Public pressure should be applied to DHHS to act and act definitively on the report, which includes a detailed dissemination plan. Once the information is trickling down effectively, the next step is public pressure on elected officials who are responsible for the budgets and direction of the DHHS agencies.
What is the next step? Does the new definition need to be approved by the sponsors / validated / operationalized? [original wording and context]
I don’t know what the next step should be for sure. The report was given to the sponsors and seems to have fallen into a black hole. I encourage anyone interested to read Chapter 8 of the IOM report, “Dissemination Strategy”, pages 231-246. A relatively detailed plan is laid out for DHHS.
If what they are lacking is funding, then pressure should be placed on our government leaders to appropriate the funds. If the report moves forward, validation and other studies will follow.
There hasn’t been public comment by any of the relevant governmental agencies that the recommendations made by the IOM will be implemented. Has there been any internal communication between the IOM and the relevant agencies that any of these agencies are willing to and going to make the suggested changes?
See answer above. I do not know of any, but the IOM committee no longer exists, and I am not in the leadership of either the IOM or the DHHS.
I think the patient community did a great job of coming up with questions. But perhaps I will be allowed to ask one of my own?
I read your helpful clarification about the IOM process and what the committee were tasked to do (and what you were not tasked to do). I think the patient community were unsure what to expect, and some may have failed to understand the purpose and limitations of the various project deliverables. I believe this was largely due to a lack of interaction and communication with the patients by the IOM’s sponsors.
While understanding that the IOM process is not a transparent, publicly interactive, or open process, do you think the IOM or its sponsors could have done a better job of communicating to patients on what to expect (and what not to expect) upfront?
I don’t think it was the IOM that needed to do a better job, but rather the sponsors. The IOM was almost an innocent bystander. They had no idea what they were getting into with this project until it exploded in controversy.
As the contract was announced, the sponsors were attacked by a tidal wave of opposition based on years of disappointment and distrust, and the IOM got swept up into fray. In defence of the sponsors, once the frenzy started, I’m not sure there was anything they could have said to calm the storm. So … I say let’s just move on and hope things are much better in the future!
A big thank you to Dr. Lucinda Bateman for her answers and willingness to engage with patients.
Thank you also to members of the patient community for taking part and submitting questions. Please note that it was not possible to include every question in the interview, but we included as many as we could.
Some of the questions were shortened or reworded to improve readability of the article and to ensure that we could cover as many questions as possible. In doing so, I tried to maintain the spirit of each question. In such cases, a link to the specific original question has also been provided for full context.
Please also note that if your question did not appear in the interview then that may have been because Dr. Bateman has already provided an answer in her earlier response to an article by Dr. Leonard Jason, or the answer to the question may be evident from the IOM report itself.
Photo by geralt/pixabay