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Otherwise publishing, removing this copyright notice, or in any way revising this document's contents is forbidden without written consent from the owner.] Advocacy, Information, Research, and Encouragement for the CFIDS Community November 1993 THE CFIDS CHRONICLE Journal of The Chronic Fatigue and Immune Dysfunction Syndrome Association of America Meetings Held at CDC to Review CFS Case Definition: Recommendations Made by Government Officials Have Potential to Define the Disease Out of Existence Officials at the Centers for Disease Control (CDC) in Atlanta hosted a public meeting on September 27 to review data from population and clinical studies related to the use of the chronic fatigue syndrome (CFS) case definition published by Holmes, et al. in the Annals of Internal Medicine. [Editor's Note: A reprint of the Annals article is available from the Association. The criteria that comprise the current case definition are outlined on page 4.] Since 1988, this case definition has been used to guide CFIDS research, diagnose patients, determine issues of disability, direct patient care, and calculate the prevalence of the syndrome. The efforts of an 11-member panel (see box on page 2) that gathered in Atlanta to consider revisions to the case definition would have the unparalleled ability to affect the life of every person with CFIDS or anyone suspected of having it. Setting the Scene Panelists heard scientific presentations from 14 CFIDS researchers and clinicians in the morning and from eight representatives of support and advocacy organizations and five members of the public in the afternoon. The sessions were chaired by Dr. Brian W.J. Mahy, director of the CDC's Division of Viral and Rickettsial Diseases, and Dr. William C. Reeves, chief of the CDC's Viral Exanthems and Herpesvirus Branch. Dr. Gary Holmes opened the meeting with a history of the process by which the 1988 case definition was established, explaining that it was designed to stimulate research, standardize patient selection criteria, and improve comparability of research. He acknowledged the inherent weaknesses of the case definition due largely to the limited body of available scientific knowledge about the syndrome in 1987, but noted it had become one of the most heavily referenced articles in medical literature according to the Institute for Scientific Information. He charged the panel with the task of improving upon the '88 definition stating, "If we are to consider ourselves successful in this endeavor, the imperfections within the new definition must be fewer and must be offset by improvements in clarity, reproducibility, and objectivity. We must undertake this effort carefully and with as little bias as possible because the outcome of our efforts will affect the way we conduct research and care for our patients and will certainly impact directly upon thousands of patients who have CFS symptoms for many years to come." In the first of his two presentations, medical epidemiologist Dr. Keiji Fukuda stated the CDC's CFS research goal, "to find the cause of CFS so it will be treated more effectively and ultimately prevented," and discussed obstacles to conducting research on the syndrome and providing proper care for patients. "Most importantly, most fundamentally, the care given CFS patients is compromised by the lack of general social and medical support. ... The lack of fact and the resulting plethora of speculation has contributed to the image of CFS as a controversial illness. ... If CFS patients are to receive the kind of social and medical care routinely given to other patients of other illnesses then CFS must shed this controversy." He asserted that only through scientifically- based research will CFS become credible and that the proper identification of CFS cases is the major obstacle facing those conducting research on the illness. Dr. Fukuda suggested that the failure of the case definition to identify a homogeneous group of patients has likely contributed to the conflicting research results obtained by various research groups. In his second presentation Dr. Fukuda made recommendations for revisions to the case definition, setting the focus for the rest of the day's remarks. He was the first to advocate for a case definition free of symptom criteria, thereby expanding the definition to capture all persons with chronic, unexplained fatigue. Subsequent speakers also called for specific changes, although the extent and form of the requested amendments varied greatly. Dr. Mahy's mid-morning announcement that Dr. Peter Manu (an outspoken proponent of the "CFS equals depression" theory) would be unable to personally present his data due to a scheduling conflict elicited both applause and forced sighs of disappointment from observers. Drs. Simon Wessely and Ian Hickie displayed their wit and nationalism with jokes about their respective countries (England and Australia), while Dr. Byron Hyde of the Canadian Nightingale Foundation was the most critical speaker of all, attacking the composition of the panel and the views of certain panelists. The most stirring presentation was made by Ms. Wilhelmina Jenkins, a person with CFIDS (PWC) and woman of color whose daughter also has the illness. She criticized the CDC's efforts to document the basic epidemiology of the disease and its reports that the disease affects only middle-aged white women. "This is as insulting and erroneous as labelling AIDS a gay disease. Such misper-ceptions lead to misdiagnosis and misunderstanding of those who do not meet the typical CFS profile. ... In whatever definition is agreed upon, the debilitating nature of this mysterious illness must be empha-sized to the primary care and family physicians who are first to see people with this disease D in particular, those in patient populations of primarily poor or rural or African American, Asian American, Native American or Latino patients. These doctors, often burdened by other health crises, may underestimate the severity of illness which has been known by the misleading name 'chronic fatigue syndrome.' The CFIDS community must let these doctors know that CFIDS is far too serious an illness to be taken lightly. It is the dream of every person with CFIDS to return to a full and productive life. With your help, we will be able to realize this dream." The Morning Session Few Points of Agreement Complete agreement was reached on only two points; the need to eliminate both the specificity of the 50 percent reduction in activity (from the first of two "major criteria") and the three physical criteria (part of the "minor criteria"). There was consensus among the speakers that the second major criteria, which requires laboratory tests to exclude any condition which may produce similar symptoms, was too costly for patients and impossible for practicing clinicians to follow strictly. From these three points of accord, the views of those heard by the panel diverged. Controversial Symptom Criteria The greatest points of contention centered on the 11 symptom criteria, of which eight are necessary for inclusion under the present case definition. Among those who addressed this issue, suggestions ranged from dropping the symptom criteria altogether (Fukuda, Straus, and Vercoulen) to changing the number required for inclusion (Hickie, Lane, Manu, and Wessely) to expanding the listed criteria (Rest, Schmaling, and Tirelli). Dr. Anthony Komaroff recognized the value of using the symptom criteria to distinguish CFS patients from healthy controls but found that only a few were helpful in discerning CFS patients from those with depression or multiple sclerosis (MS). The scientists pushing to drop the minor criteria asserted that, based on their independent research studies, these symptoms were arbitrary and ineffective in distinguishing CFS patients from patients with other fatiguing illnesses. Dr. Stephen Straus of the NIH was perhaps the most vocal of these critics. His argument was based on the belief that the symptom criteria were too numerous, too vague, and did not delineate a discrete syndrome or entity. He found that the use of these symptoms to define the syndrome excluded large numbers of patients who were as equally impaired as those who met the criteria. Finally, he maintained that any list of criteria would prove to be just as arbitrary. While three presenters agreed that the number of symptoms required for inclusion should be changed, they differed in opinion on what the new requirement should be. Dr. Ian Hickie, an Australian researcher now living in the U.S., reported that 30 percent of 565 CFS cases identified in four international sites actually suffered from somatiform disorder, based on the presence of a diverse and over-inclusive range of somatic and psychological symptoms, deviant illness behavior, and concurrent severe psychological morbidity. He concluded that the criteria set needed to be adjusted to exclude these patients, who were more homogeneous as a group than were those in the other 70 percent. Dr. Simon Wessely, a British psychiatrist, presented data from a London study which showed that as the number of symptoms experienced by patients increased, so did the rates of psychiatric disorder as evidenced by a Diagnostic Interview Schedule (DIS)-like inventory. Like Dr. Hickie, he recommended setting a maximum for the number of symptoms and excluding those who reported more than this number in an attempt to rule out persons with psychiatric illnesses. Due to Dr. Manu's absence, his associate Dr. Thomas Lane was placed in the awkward position of first presenting Dr. Manu's data and then his own. Even though the doctors practice together in a Connecticut chronic fatigue clinic, they reached different conclusions about how best to revise the case definition. Dr. Manu's data suggested reducing the necessary minor criteria to one D fever present at onset. Dr. Lane then presented his own data, recommending that the 11 criteria be decreased to four (fever or chills, sore throat, adenopathy in the neck or axilla, and sudden onset of the main symptom complex) and that 2 of 4 of these symptoms (in addition to the two major criteria) be required for inclusion. This group's focus on fever drew strong criticism from clinicians who reported that fever was rarely present, and in fact, patients often had sub-normal temperatures. Drs. Schmaling, Rest, and Tirelli recommended additions to the current list of 11 symptoms. All three suggested that cognitive dysfunction be listed as a separate criterion, a recommendation repeated by many of the patient advocates and clinicians who spoke in the afternoon. Dr. Karen Schmaling, of the University of Washington noted that information processing inefficiency was useful as an objective measure of impairment and that her group's studies had confirmed a study published by DeLuca, et al. Berkeley clinician Jonathan Rest reported that his patients often have difficulty concentrating and describe themselves as "feeling in a fog." Dr. Umberto Tirelli, a researcher from Italy, recommended further that cognitive dysfunction be elevated to the major criteria required for inclusion. Drs. Schmaling and Rest listed other symptoms which should be considered for addition: awake in the morning unrefreshed, weight gain, shortness of breath, and nausea (Schmaling); night sweats, dizziness/light-headedness, new or worsening allergies, and gastrointestinal troubles (Rest). Depression as an Exclusionary Criteria Among the few presenters that specifically addressed the issue of whether depression should continue to be listed as an exclusionary criterion, the general consensus was that it should not, consistent with the recommendations made by Schluederberg, et al. following a March 1991 NIH-sponsored workshop on the Definition and Medical Outcome Assessment of CFS in Research. [Editor's Note: See the Fall '91 Chronicle, page 27, for a reprint of the NIH report.] The NIH report recommended that those with "non-psychotic depression or a prior, remote, or history of a depressive episode be stratified in studies." Dr. Fukuda qualified the CDC's recommendation for inclusion, stating that patients with a concurrent psychiatric condition should be excluded if specific therapy was available or if a CFS diagnosis could potentially prevent appropriate treatment. He based this qualification on concern for patient safety and treatment. Dr. Rest voiced a strong objection to excluding patients by virtue of concurrent psychiatric illness. "It is true that a very high percentage of CFS patients evidence some psychiatric symptoms during the course of their illness. This probably is a result of dealing with chronic fatigue and may also represent a symptom secondary to the pathogenesis of the illness." In his abstract he attributed the depression experienced by some patients to their isolation and profound lifestyle limitations, as well as the possibility that the disease may have a direct impact on the brain. Other Sub-Groups Considered Other stratifications of patients for research purposes were proposed by various speakers. Dr. Fukuda was the first to propose such sub-classes with his group's recommendation that cases be categorized by specific criteria. Dr. JHMM Vercoulen of the Netherlands recommended identifying different subgroups among the apparently heterogeneous patient group to better assess disease severity and the consequences of various complaints. In the last presentation of the morning, Dr. Paul Levine of the NIH's National Cancer Institute recommended the use of a scoring system, in which "points" would be given for the presence of important criteria, and classification of patients as having "classic", "probable", and "possible" disease states. Those not meeting the criteria would be classified as "inconsistent." He stated in his abstract that this process of classification, which is comparable to that used in the case definition for adult T-cell leukemia/lymphoma (ATL), would be "more applicable to clinical diagnosis in a non-research setting and will assist patients and physicians in obtaining medical and disability compensation." Surprisingly, the panel expressed little interest in this novel proposal. He also addressed patients who exhibit more severe neurological symptoms. "We would make a plea for taking people who you think might have idiopathic neuromyopathy, or something else, but otherwise look like chronic fatigue syndrome and putting them into a subset. You may be tossing out some typical patients who have atypical features. If you look at them all together, these patients may give you a clue as to what is going on with this illness." Further, he suggested classifying patients according to acute versus gradual onset. In contrast to Dr. Levine's recommendation, Dr. Dedra Buchwald of Harborview Medical Center in Seattle reported data which showed that there were no discernable differences between patients with acute versus gradual onset or between those with and without the symptoms of fibromyalgia. A Diagnosis of Inclusion Drs. Komaroff and Rest articulated the need to move to inclusionary criteria, rather than relying so heavily on the exclusion of all other conditions that might cause similar symptoms. Dr. Komaroff supported his statement with evidence from over 20,000 laboratory studies performed over the last seven years in collaboration with Dr. Buchwald. "When you compare the CFS patients to age, gender and socioeconomic status-matched healthy control subjects from the same clinical setting there are substantially higher frequencies of circulating immune complexes (an odds ratio of nearly 28:1), elevated levels of immunoglobulin G, atypical lymphocyte counts (on average above two percent), and elevated alkaline phosphatase (with very high odds ratios and clearly significant discriminators). Now this is comparing cases with healthy controls. The next obvious question, which we are now investigating, is whether the disease comparison groups are distinguished by any of these laboratory parameters." Dr. Rest writes in his abstract, "History indicates that syndromes such as CFS/CFIDS can be diagnosed by identifying footprints of symptom clusters and patient history, and although exclusion of other conditions is always sound diagnostic procedure, it should not be the primary diagnostic route, but rather secondary or helpful in conjunction with the overall picture." He also expressed his anticipation of meaningful laboratory criteria in the near future. Need for a Clinical Definition Several speakers addressed the need for a separate clinical definition for use by physicians to diagnose the disease and by government and private institutions to settle medical-legal issues such as disability. While CDC officials denied responsibility for establishing such a clinical definition prior to and during the course of the meeting, a number of speakers acknowledged the fact that in the absence of such a definition, the "research" case definition has been and will continue to be used for such non- research purposes. Dr. Hickie condemned the "one size fits all" use of the present case definition at the conclusion of his presentation. "There are different needs in criteria sets. One is a clinical one. It is inherently patient-focused. It's about disability. It's about issues of duration and it's tied up in the issues related to the healthcare system. ... Quite separately, one needs to have research criteria that allow specific questions to actually be asked that match hypotheses and criteria. So we would strongly recommend that you can't simply come up with one size fits all. You've got to have some separation that allows the research to continue dealing with fundamental issues and similarly allows the patients to get on with their treatment and not feel that they have to fit the research criteria in order to receive care and disability." This opinion was expressed frequently by patients, advocates and clinicians who addressed the panel during the afternoon session. Pediatric Criteria Proposed Dr. David Bell, a clinician among the first to characterize CFIDS during an outbreak in a small farming community in upstate New York, discussed the difficulties of applying the CDC case definition to children and proposed an alternative case def-inition for children ages 12 and under. He related his experience trying to apply the case definition to children in private practice and hospital settings and reported that children more frequently experience a gradual onset of symptoms, lack established activity levels (by which to judge the 50 percent reduction), and more frequently experience infections of the upper respiratory tract, thus making the minor physical criteria less reliable. Given these differences between adult and pediatric patients, he proposed the following criteria for a pediatric case definition: 1. Activity limitation causing disruption of normal lifestyle for at least six months; 2. No obvious physical or emotional cause of fatigue; and 3. At least 8 of 12 symptom criteria: a. Fatigue b. Headache c. Abdominal pain d. Lymph node pain e. Recurrent sore throat f. Muscle pain g. Joint pain h. Sleep disturbance i. Eye pain and/or photophobia j. Depression k. Neurologic symptoms: light-headedness, balance disorder, paraesthesia l. Cognitive difficulties (short-term memory loss, attention deficit) Dr. Bell presented data from a study of 56 children he and his collaborators had seen and suggested that the proposed criteria more effectively captured CFS cases than did the CDC's criteria. Perhaps because the present case definition ignores children, Dr. Bell's presentation was among the most compelling and sparked the greatest number of questions from the panel. The Afternoon Session While scientific presentations dominated the morning session, after lunch the discussion took on a more personal tone. Representatives of national, state, and local support groups were given the opportunity to speak for 10 minutes, but were asked by Dr. Reeves to confine their comments to the case definition. Most speakers obliged this request, with the noted exception of Dr. John Renner, representing Kansas City's National CFS Association. Dr. Renner chose to use his time at the podium instead to review the affiliations of each of the morning's speakers and to lecture about the need for more mobile medical records, better patient diaries, and more frequent physician inquiries into the CFIDS patient's knowledge base about the disease. Among those last to speak were Drs. Dan Peterson, Charles Lapp, William Crook, Byron Hyde, and Nancy Klimas D individuals who, despite their substantial individual and collective understanding of this illness were acknowledged only as members of the "public." Drs. Peterson and Lapp spoke of their clinical experience with the disease and pointed out some of the medical/legal realities associated with the use of the case definition by those outside the research community. Dr. Crook urged doctors to listen to their patients and keep an open mind. Dr. Hyde condemned the composition of the panel and elicited applause from the audience when he pointed out the lack of clinical expertise among the panelists. This group of speakers generally agreed with the consensus formed during the morning session: dropping the physical criteria, dropping depression as an exclusionary criterion, and eliminating the specificity of a 50 percent activity reduction. In contrast to those of the morning's speakers, the recommendations made by the afternoon speakers were more similar than different. What's In A Name? A common theme articulated by the afternoon speakers was the urgent need to change the name, although a few lamented that they had no reasonable alternative to propose. Kim Kenney (Executive Director of The CFIDS Association of America), Marya Grambs (Director of The CFIDS Foundation), and Dr. Nancy Klimas (a noted CFIDS immunologist at the University of Miami) supported a formal change to "chronic fatigue and immune dysfunction syndrome" in recognition of the various immune abnormalities documented by private and public-sector researchers. They requested that this change be identified as an interim step between CFS and a name that would more accurately reflect the etiology or pathogenesis of the illness. Dr. Klimas presented Dr. Reeves with a notebook filled with medical articles on the immune abnormalities found in CFIDS in defense of this recommendation. [Editor's Note: See Ms. Kenney's presentation in its entirety on page 13 of this Bulletin.] Other patient advocates including Tom Hennessy of Repeal Existing Stereotypes against Chronic Immunologic and Neurologic Diseases (RESCIND), Meghan Shannon, a PWC from San Diego, and Dr. Hyde rejected keeping the word "fatigue" as part of the name, arguing that its inclusion trivializes the disease and demeans those who suffer from it. Ms. Shannon offered two alternatives: Acquired Immunoneurological Syndrome (AINS) or AIDS - HIV negative. Dr. Hyde introduced the concept of naming the syndrome after a person and suggested Guilliam's Disease after Dr. (Sandy) A.G. Guilliam, the Assistant Surgeon General who described this disease in 1938. Mr. Robert Landau of the New Jersey CFS Association asked that if an eponym was adopted for non-scientific reference to the disease, that it not be known as "Cher Syndrome." The Private Case Definition Meeting According to reports from observers of Tuesday's private meeting, there was little agreement among panelists and groups representing three positions were formed. There were those intent on broadening the definition to chronic fatigue with no symptom criteria at all; Drs. Holmes and Straus formed this first group. Drs. Komaroff, Natelson, and Gantz recommended various stratifications of the existing case definition while Drs. Abbey, Jolson, and Jones were uncommitted to either position. Following this private session, Dr. Komaroff reported that few conclusions were reached and that the group would try to reconvene in approximately six weeks. He stated that they had agreed not to change the name of the disease at this time, but committed to acknowledging in any article published as a result of the efforts that the name is a poor one and does not adequately reflect the severity of the illness. Dr. Komaroff suggested that the patient community agree on an eponym to be used simultaneously with chronic fatigue syndrome. He suggested naming the disease after a famous PWC or an early physician who studied it. Dr. Komaroff cautioned that permission for the use of the person's name should be first obtained from that person, if living, or the family, if deceased. Research is underway to investigate the possibilities for such historic figures. Marc Iverson Responds The very composition of the panel, based on the tremendous power it wields, has launched leaders of The CFIDS Association of America into action. A letter was sent by Marc Iverson, president of the Association, to Dr. Reeves with a number of requests. First, he asked that the panel, which is a subset of the Physician Review Committee of the CDC's Surveillance Study, be expanded to include the three clinicians currently on this committee who were omitted from the panel D Drs. David Bell, Nancy Klimas, and Susan Levine. Second, Mr. Iverson asked that 2 of 5 Association-recommended physicians be invited to serve on the panel to further expand its clinical expertise. Third, Dr. Reeves was requested to add Dr. Paul Levine, who serves as president of the American Association for Chronic Fatigue Syndrome (a professional association) and also conducts CFIDS research at the NIH's National Cancer Institute. Fourth, a request was made to include a private sector researcher with expertise in CFIDS-specific prevalence studies; Dr. Leonard Jason of DePaul University was recommended to fill this void. Finally, Mr. Iverson requested that two or more patient advocates be added to the panel and recommended Ms. Kenney and Ms. Grambs, given their proven abilities to represent patients' needs in their daily work as professionals leading two of the largest and most active organizations dedicated to conquering CFIDS. In a letter dated October 22, 1993, Dr. Reeves declined Mr. Iverson's requests to expand the case review panel. "I have limited our working group to this number for reasons of efficiency. Since our intent is to prepare a research case definition for publication in a peer reviewed medical journal, I have limited the group to individuals with academic credentials and not included patient advocates." Dr. Reeves informed Mr. Iverson that he had invited U.S. clinicians (Drs. Dan Peterson, Jonathan Rest, David Klonoff, and Dedra Buchwald) and representatives from major foreign CFS research groups (Drs. Brigitta Evengard -Sweden, Umberto Terelli - Italy, Ian Hickie - Australia, Simon Wessley - UK, and J. Vercoulen - Netherlands) to provide their input, although none of these consultants would be listed among the authors in any publication that resulted from the group's efforts. Mr. Iverson finds Dr. Reeves' justifications to lack merit and is in the process of appealing his decision. He will also urge Dr. Reeves to include Dr. Jay Levy in the process of revising the case definition. Dr. Levy participated in the private case definition meeting, but is not currently a member of the formal working group. Help! If the CFS case definition is revised to require only the presence of chronic fatigue, from a public health standpoint the distinct entity now known as CFS/CFIDS will cease to exist D and so will the credibility built over the last nine years. If researchers are to have a chance at finding a marker, a cause, efficacious treatments, and ultimately a cure for this disease, the new case definition must differentiate between the symptom of chronic fatigue and the distinct clinical entity known as chronic fatigue syndrome. Association staff will continue to monitor D and participate in (to the greatest extent possible) D further deliberations by the panel and will keep members informed of the panel's progress through the Chronicle and the CFIDS Information Line's monthly update (900/896-2343). Your support of these efforts is critical and it is imperative that you make your voice heard. If the government's position on the case definition revision or its efforts to keep expert CFIDS clinicians off the review panel disturb you, send your comments to: Dr. William Reeves, Viral Exanthems Branch, Division of Viral and Rickettsial Diseases, CDC, Mail Stop G-18, 1600 Clifton Rd., NE, Atlanta, GA 30333 (Fax: 404/639-3163). Send your check earmarked for advocacy to The CFIDS Association of America so we can continue our focussed efforts too. But whatever you do, don't let the government write off this disease D and those who suffer from it. Kim Kenney of The CFIDS Association of America Speaks Out CDC Case Definition Review Meeting September 27, 1993 Thank you for the opportunity to address this audience of researchers, clinicians, persons with CFIDS, patient advocates, and interested others. I believe that this meeting represents a milestone in the history of the CFIDS movement and marks another positive shift in the relationship between CFIDS advocates and officials at the Centers for Disease Control. My name is Kim Kenney. I am Executive Director of The CFIDS Association of America and feel extremely fortunate to be able to say that I do not have CFIDS, nor does any member of my family; this is strictly a professional endeavor for me. The CFIDS Association of America is the world's largest and most active non-profit organization dedicated to conquering CFIDS. Since 1987 we have funded more than $1.5 million in private sector research. Over the last two years we have led a national grassroots movement to advance a public policy agenda whose singular purpose is to effect an aggressive and comprehensive public health response to this disease. It is relevant to note that our legislative efforts have resulted in a 100 percent increase in the CDC's budget for CFS research over the last two years. While our public policy agenda outlines an action plan for each of the public health service agencies inclusive of research, prevention, care and education, I will confine my remarks this afternoon to the Centers for Disease Control and its responsibility for the case definition now used to describe chronic fatigue syndrome. Since 1988 when the case definition for chronic fatigue syndrome was published by Holmes et al., many published scientific discoveries have led to an improved understanding of this illness. The Holmes paper served many purposes for its time. It helped legitimize this syndrome, put an end to the use of the term "chronic Epstein-Barr virus," and it provided a consensus opinion on the salient characteristics of the syndrome as the basis for further research. However, it did not provide a description of the syndrome sufficient for practicing physicians to readily use in making a diagnosis. Nor has it sufficiently aided research studies by identifying a homogenous group of patients. For five years clinicians, researchers, and patients alike have struggled with this case definition. While the description of this syndrome may, in fact, turn out to be a process and not an event, it is crystal clear that the case definition must now be altered in ways that will benefit all three groups. The CFIDS Association of America has taken this opportunity to build a consensus opinion on recommendations for changes to the current case definition. We have consulted members of our Scientific Advisory Committee, Board of Medical Advisors, and Public Policy Advisory Committee. We propose 10 changes to the current definition based on published research and the extensive experience of our advisors. Specific Recommendations Our first recommendation is that the name used to define the syndrome be again amended, as it was with the 1988 definition, from chronic fatigue syndrome to chronic fatigue and immune dysfunction syndrome. This change would recognize numerous published studies conducted by private and public sector scientists which document defects of the immune system in patients with this syndrome. We request that this name change be made and recognized as an interim step until a definitive marker or an etiologic agent is found, at which time a more accurately descriptive term should be used for the syndrome. The name CFS has serious problems in and of itself D most serious of all is that the disease and those who suffer from it are trivialized and not taken seriously by the medical community, the public, and the media. By changing the name to incorporate the immune defects that have been found by Drs. Stephen Straus, Nancy Klimas, Jay Levy, Michael Caliguiri and others, the name begins to reflect the seriousness and nature of this condition. This name has been widely used by patient organizations and their members and has been used by growing numbers of clinicians, researchers, and members of the public and media. In fact, this year the Senate Appropriations Committee used the term CFIDS in its fiscal year '94 funding bill. If the public health community would adopt this term as well, the stature of the disease, its study, and those who suffer from it would be raised immediately. This change would also help to distinguish the illness from chronic fatigue, a symptom of many, many medical conditions and one with which the disease is frequently confused. Chronic fatigue does not accurately or adequately describe this symptom complex and we strongly urge you to adopt a name that identifies it as a distinct clinical entity and eliminate a name that implies that seriously ill patients are malingering or are just tired at the end of the day. Number Two: In preparing a revised case definition, it must be acknowledged that in the absence of a clinical definition for this disease, the case definition has been and will continue to be used by physicians to diagnose the disease and the Social Security Administration and private insurers to determine disability. The medical and legal ramifications of this case definition for physicians and their patients are far-reaching. Every attempt should be made by those gathered here to revise the current case definition in ways that take these users' needs into consideration. More specific recommendations follow. Number Three: In March of 1991, Dr. Ann Schluederberg of the National Institutes of Allergy and Infectious Diseases convened a workshop on the definition and medical outcome assessment of chronic fatigue syndrome in research. As a result of this workshop recommendations for revisions to the case definition were made. We advocate that all of the recommendations for the case definition and confounding diagnoses be adopted by this panel. These changes are listed in the NIAID report as "Table I." [Editor's Note: See the Fall '91 Chronicle for a reprinting of the NIAID report.] Number Four: In reviewing the 1988 case definition, the only acknowledged hallmark of the illness is fatigue. The first of two major criteria states "a new onset of persistent or relapsing, debilitating fatigue or easy fatiguability ... that is severe enough to reduce or impair average daily activity below 50 percent of the patient's premorbid activity level for a period of at least six months." While this criterion clearly attempts to distinguish the fatigue experienced by those suffering from this syndrome from the fatigue that even healthy people may experience at the end of the day, this wording and the specific requirement of a 50 percent reduction in activity is not meaningful or usable by the clinician or researcher. We offer instead a modification of the description for fatigue used in the Australian case definition: "chronic persisting or relapsing fatigue of a generalized nature exacerbated by physical or mental exertion that is serious enough to require significant alterations in life- or workstyle in order to accommodate the illness." Number Five: The current definition calls for the illness to be present for a period of six months before the patient can be considered a "case." We recommend that this period not be extended beyond six months. Our physician advisors felt strongly that six months was an adequate period of time to distinguish this syndrome from self-limited and acute syndromes like mononucleosis or persisting influenzas. Number Six: While we understand the usefulness of stratifying those patients with a history of non-psychotic depression in research studies, consistent with the recommendations of Schluederberg, et al., we recommend that any history of non-psychotic depression cease to be used as an exclusionary criterion. Number Eight: The current definition has a cumbersome structure using major criteria and minor symptom and physical criteria. Based on physician experience, we recommend that the physical criteria be eliminated thus simplifying the case definition. It should be noted that each of the three physical criteria are already listed among the symptom criteria. Number Nine: Along those lines, based on physician report and the results of the CDC's own surveillance study, there are a number of potential cases who fail to meet the case definition by one or two criteria. We recommend that the panel consider changing the requirement to either fewer than eight symptoms for inclusion or expanding the list of symptom criterion to include other symptoms common to persons with CFIDS. Physicians who regularly apply the case definition have also asked for clarification of each symptom criterion. For instance, it is often difficult to measure muscle weakness, despite the patient's convincing report of the sensation of muscle weakness. The same may be true for fever. Patients also describe headaches and sore throats of new types and varieties not experienced in their premorbid state. There is also a need to emphasize the chronically waxing and waning (relapsing and remitting) features of the illness over time and space as the disease changes from its acute clinical presentation to its chronic picture. Number Ten: We strongly suggest that the feature of cognitive dysfunction be listed as a separate "symptom criterion" instead of including it as one of many neuropsychologic symptoms. It has become as prominent a hallmark of the disease as the fatigue itself. For many patients, this symptom is perhaps the most disabling of all. Finally, we recommend that vertigo and balance problems be included with the other listed neuropsychologic symptoms, as these symptoms are extremely common among and problematic for those with the disease. Broad Dissemination Requested These changes serve to describe CFIDS not as a disease characterized almost entirely by fatigue, but as a multi-systemic disorder which affects the immune, endocrine, rheumatologic, neurologic and metabolic systems of the body. To describe this disease as such recognizes the complexity and genuine severity of the illness and rightfully elevates its stature as a disease deserving the medical community's attention. And for these reasons, we urge the CDC and the panel gathered today to publish the results of this meeting and the revised case definition in Morbidity and Mortality Weekly Report and in the Journal of the American Medical Association, with editorial comment, or the New England Journal of Medicine. Without the broad dissemination of the results of this effort, the changes in the understanding of this disease that have occurred over the past five years will go unnoticed by the vast majority of practicing physicians and bench researchers. The CDC is charged with implementing public health strategies to combat disease, illness, accidents, and health risks. We feel strongly that in order to fulfill its mission, the CDC must bring these advances to the medical community and the public at large. Solve the Paradox The 1988 case definition has created an interesting and problematic paradox. The name chronic fatigue syndrome is too broad and too easily confused with the symptom chronic fatigue, while the case definition for chronic fatigue syndrome is too restrictive and doesn't identify a large enough percentage of actual cases. Every day we have the opportunity to hear from thousands and thousands of people who have this syndrome and people who think they have this syndrome. The name creates an illusion that this disease is just fatigue. That it's just being tired. It's dangerous to have people out there diagnosing themselves with this disease because the name sounds like how they feel. What's even worse is when physicians try to apply this case definition to make a diagnosis and find very few cases because of its inherent restrictiveness, leaving them with the feeling that this disorder simply doesn't exist. If we are to determine the true prevalence of this disease and combat the overly- generous sense of medical skepticism that continues to cloud clinical and research issues related to this disease, this condition must be rectified. Dozens of basic questions remain unanswered. Children and adults suffering from CFIDS demand and deserve answers. As a fundamental step toward finding these answers through the process of scientific inquiry, we eagerly await the published results of this effort and a revised case definition which incorporates the 10 recommendations we have put forth today. And we look forward to strengthening our partnership with the Centers for Disease Control and other federal agencies so that we can use the considerable force of our grassroots movement to advance our collaborative efforts. Editor's Note: Throughout this Bulletin we have used the name "chronic fatigue syndrome" (CFS) when it was used by the speaker, although CFIDS continues to be the name preferred by the Association. The Dialogue Continues: Day Two in Atlanta When Dr. Walter Gunn (then principal investigator for CFS studies at the Centers for Disease Control (CDC)) created the Chronic Fatigue Syndrome Interagency Coordinating Committee (CFSICC) in 1989, he sought to join resources across the agencies of the Public Health Service (PHS) to provide a more effective and unified response to chronic fatigue syndrome. In late 1991 Dr. Gunn retired from CDC and the committee lost the leadership it needed to be productive. Through the congressional appropriations process, patient advocates sought to reinstate this committee as a function of the Assistant Secretary for Health's office to provide oversight of federal CFS research programs. In 1992, these efforts paid off with a $1 million earmark to operate the committee and a directive that the Assistant Secretary for Health would serve as its chair. Confidence in the potential for this committee to act as a watchdog over federal programs soared when long-time CFIDS advocate Dr. Philip Lee was sworn in as Assistant Secretary this summer. Despite his personal commitment to make the committee a valuable asset to government officials and advocates alike, Dr. Lee had to delay taking command of this committee because of the all-consuming nature of his role in preparing healthcare reform legislation. Dr. Lee's first meeting as chair was scheduled for September 28, 1993 in Atlanta following the CDC case definition review meeting. Unfortunately, at the last minute Vice President Gore requested Dr. Lee's assistance in California to help "sell" President Clinton's healthcare reform package. With influence from Tom Sheridan (the Association's lobbyist), the decision was made to proceed with the September 28 meeting, but to make it a planning session for the first meeting under Dr. Lee's leadership, which must be held before December 15, 1993 as required by congressional mandate. The Planning Begins Dr. Elaine Esber served as acting chair for the meeting which involved other members of the committee representing the Centers for Disease Control (CDC), National Institutes of Health (NIH), Food and Drug Administration (FDA), and Social Security Administration (SSA) and members of the public. Drs. William Reeves and Ann Schluederberg offered broad overviews of their respective agencies' CFS-related activities, reporting no new data or study results. Dr. Dianne Murphy and Mr. Barry Eigen focused their remarks on efforts to follow CFS research more closely and to initiate programs within each agency that would enable them to act quickly in response to new research findings. So each speaker could finish his or her prepared remarks without interruption, the audience was asked to reserve questions until a specified question and answer period scheduled for the end of the morning session. This provision allowed observers ample time to contemplate points made by the agency spokespersons. Dr. Esber asked that questions be written on forms passed through the meeting room, but advocates resisted this mechanism (which is often used to avoid or discourage probing inquiries). At the end of the scheduled presentations, Ms. Kim Kenney of The CFIDS Association of America approached 1 of the 2 floor microphones to begin a series of questions, a move that others quickly followed. Speakers were clearly unprepared for the number and specificity of questions posed which ranged from challenges to a draft booklet distributed by CDC, "The Facts About Chronic Fatigue Syndrome," [Editor's Note: See the ADVOCACY ALERT on page 31] to criticism of reported prevalence rates. Some of the answers to these questions demonstrated the speakers' lack of enthusiasm for and familiarity with CFS-related activities at their own agencies. Following a continuation of the morning's question and answer period after lunch, members of the public were given five minutes each to comment on the government's response to CFS. Most of the persons who had asked to speak represented national, state, or local support groups, although individuals who simply wished to speak from personal experience also spoke. By the time the afternoon session began, tempers were flaring over the committee members' unresponsiveness and trivializing statements in the CDC's booklet; patient advocates held back little of their frustration. Committee members, seated together at a long table along the side of the room, seemed unaffected by the statements made and often appeared to be lost in their own thoughts. The amount of information exchanged and the level of interaction between committee members and observers is evidenced by the CDC- prepared transcript of the day's meeting, which is 160 pages in length. In consideration of space and brevity, a synopsis of the government agency speakers' presentations, notable quotes from the question and answer periods and afternoon session, and transcripts of presentations made by advocates Ms. Kim Kenney and Ms. Marya Grambs follow. CDC Activities Dr. Reeves began his presentation by stating the four goals of the CDC's investigation of CFS: to identify its prevalence and incidence in the United States; to document its clinical course; to identify risk factors for the disease; and to determine methods of intervention. He then outlined four types of studies now underway at CDC to meet these goals. The four-year old surveillance study was the first he reviewed. Sentinel physicians in the four study sites (Atlanta, Grand Rapids MI, Reno NV, and Wichita KS) have referred 2,232 patients to the study, 491 of which have met all the criteria required to be considered actual cases, resulting in an overall prevalence estimate of 5.2 per 100,000. The median income for women identified through the study was $50,000/year. Dr. Reeves also described two population surveys of chronic unexplained fatigue to be carried out in San Francisco and Wichita. Case/control studies being conducted at CDC will attempt to define immunologic and virologic differences between samples taken from CFS cases and appropriately matched controls. These studies will also seek to uncover risks related to environmental exposures, animal/insect contact, and other factors such as diet, travel, occupation, and prior health. Finally, he discussed two cluster outbreaks under investigation by CDC in Pigeon, MI and Sacramento, CA. He indicated a moderate response rate (58 percent) to questionnaires sent to residents of Pigeon and surrounding communities which resulted in the identification of 18 cases of fatigue persisting longer than one month. Sixty-one percent of these cases were female. In Sacramento, the investigation of an office building cluster generated a better response to the initial questionnaire (85 percent). Nineteen percent of those responding were found to have fatigue which persisted for more than one month; 69 percent of those with such fatigue were female. Dr. Reeves explained that an additional responsibility of the CDC is to provide informational services to the medical community and public at large. He indicated that the CDC seeks to publish its study results in peer-reviewed literature and the CDC's own publication, Morbidity and Mortality Weekly Report, and to present its findings at scientific meetings. In an effort to stay abreast of current private sector research activities, Dr. Reeves acts as the CDC liaison representative to the American Association for Chronic Fatigue Syndrome (AACFS) and provides information to patient groups about the CDC's activities. Finally, through the CDC's voice information system and its written materials, CDC seeks to inform interested persons and members of the public about CFS. The previously mentioned draft booklet, "The Facts About CFS," which he had circulated for comment, generated a great deal of controversy during the question and answer period. [Editor's Note: See ADVOCACY ALERT on page 31.] The NIH Response Standing in for Dr. John LaMontagne, Dr. Ann Schluederberg reported on CFS-related research at the largest government-sponsored agency dedicated to conducting and funding basic biomedical research -- the National Institutes of Health. Since research on CFS had begun at the National Institutes of Allergy and Infectious Diseases (NIAID) in the 1980s and continues to be focused there, Dr. Schluederberg's report dealt mainly with that institute's programs. She did, however, state that related intramural research is now taking place in 9 of the other 17 NIH institutes. The most active programs are housed at the National Institute of Child Health and Human Development (NICHHD), the National Institute of Mental Health (NIMH), National Institute of Neurologic Disorders and Stroke (NINDS), and the National Cancer Institute (NCI). Intramural support for CFS-related research during fiscal year 1993 totalled $1.4 million. NIH funding for extramural research is directed through NIAID's department of microbiology and administrated by Dr. Schluederberg. Additional support for pilot studies has come from the National Center for Research Resources. Extramural funding for CFS research totalled $3.8 million in FY '93, representing 83 percent of NIH's total CFS budget. Dr. Schluederberg described the process by which extramural grant applications are reviewed at NIH, stating that scientific merit, programmatic need, availability of funds, and congressional interest govern which types of research proposals are awarded funding. She attributed the growth in extramural funds available for CFS research from $425,000 in the late 1980's to the current level of $3.8 million to successful patient advocacy efforts. In discussing the extramural funding program, she maintained that NIH has traditionally not received many applications for CFS research, averaging only seven per quarter last year. Due to the small number and broad scope of applications received in the past, a number of different study sections had reviewed these proposals, some of which had no working knowledge of CFS. This year the Division of Research Grants established a Special Emphasis Panel (SEP) to evaluate all applications for CFS research to ensure that each application is reviewed by a multi-disciplinary panel with the expertise necessary to evaluate the proposal. [Editor's Note: The decision to establish an SEP for CFS research was likely due, at least in part, to pending legislation contained in the NIH Revitalization Act requiring such a body. The CFIDS Association of America lobbied intensely for such legislation.] In an effort to stimulate CFS research, Dr. Schluederberg noted that the NIH has organized four workshops, issued two program announcements, and published one request for applications. Three types of awards have been made for extramural CFS research: the cooperative agreement, research project grants, and first awards. At the core of the extramural effort are the three Cooperative Research Centers (CRCs) located in Boston, MA (Dr. Anthony Komaroff, principal investigator (PI)), East Orange, NJ (Dr. Benjamin Natelson, PI), and Denver, CO (Dr. James Jones, PI). There are currently 10 projects underway within the Research Centers program. The CFS Research Working Group, comprised of intramural researchers, meets every two weeks to discuss progress, problems, etc. Extramural research is addressed quarterly by the NIH CFS Coordinating Committee. Members of this committee include representatives from each NIH institute sponsoring extramural CFS- related research, although meetings are open to all members of the NIH community. Dr. Schluederberg emphasized her participation as a liaison representative of the AACFS and as a member of the CFS Interagency Coordinating Committee. According to Dr. Schluederberg, the NIH's research program is directed at answering the following questions: What is CFS? Who gets it? What causes it? How can it be treated? Can it be prevented? Dr. Schluederberg indicated that several approaches are being employed to find answers to these questions. A long-term study of CFS which began at NIH continues with the CRCs. A database holding information on clinical and laboratory parameters is being reviewed for diagnostic and prognostic markers to help define what CFS is. Patient demographics and physiologic characteristics are being evaluated to help understand who gets it. Epidemiologic and clinical studies will help reveal what causes it and the development of animal and other disease models will lead to a better understanding of its patho- genesis and will aid in the search for treatments and preventative strategies. Dr. Schluederberg discussed various obstacles to effective research including the absence of a biologic marker, inability to obtain suitable control data, use of medications by cases which can confound data, and cyclical factors such as time of year and time in the menstrual cycle (for female cases). Dr. Schluederberg described the NIH Coordinating Committee's attempts to seek comment on the NIH's research program goals from patient groups. She reviewed her perception of the suggestions received from Kim Kenney of The CFIDS Association of America, Orvalene Prewitt of the National CFS Association, Dr. Walter Gunn of the AACFS, Marya Grambs of The CFIDS Foundation, and Judy Basso of the CFS Association of Minnesota. [Editor's Note: Please see the June '93 and Summer '93 issues of the Chronicle for full reports on these meetings.] She expressed a desire to expand the dialogue with the patient community in the future. Dr. Schluederberg concluded her remarks by reiterating statements made in NIAID's report to the director of NIH. A recommendation was made to continue funding all of the ongoing CFS research programs and to give particular attention to the following areas of research: to uncover biologic and epidemiologic explanations for observed increases in frequency associated with gender, age, race, and socioeconomic status; to investigate risk factors including genetics, behavior, and environmental factors; to develop methods to evaluate the clinical impact of viral reactivation; to expand research on gender-specific interaction between the immune system and the hypothalamic pituitary adrenal network; and to develop normative data for the interpretation of brain scans. FDA: Regulatory Agency with a Poorly Understood Role Dr. Dianne Murphy began her brief presentation by defining the FDA's function for members of the audience in response to previous conversations with PWCs about the FDA's role: to assess and assure the safety and effectiveness of therapies. Contrary to popular belief, its role is not to research chemicals, produce drugs, or provide therapies. She asserted that limited research, primarily on regulatory issues to determine optimal therapy usage, is conducted at FDA. Its main function is to provide guidance on issues related to clinical trial design. "False hope [is] a study that is not ... properly designed to answer the questions that it seeks to address." FDA's current CFS research is primarily related to the search for meaningful endpoints. Measures of physical activity, quality of life, and potential memory studies are being evaluated. Dr. Murphy also indicated a continuing commitment to work with HEM Pharmaceuticals in its pursuit of licensure for Ampligen. Dr. Murphy reported that the FDA's Antiviral Drugs Advisory Committee has begun to familiarize itself with issues related to the evaluation of therapies for CFS. In September 1992, the committee held a closed meeting to begin this discussion and in February 1993 invited Drs. Reeves and Fukuda of the CDC, Dr. Schluederberg of the NIH, and Dr. Anthony Komaroff of Brigham and Women's Hospital to comment on CFS treatment study design issues. [Editor's Note: The Association was represented at this meeting by PPAC co-chair Jerry Crum and his wife, Coco, and by Medical Advisor Dr. Paul Cheney.] A closed follow-up meeting was held in August 1993. To demonstrate the FDA's commitment to CFS, Dr. Murphy pointed to the fact that it is conducting CFS-related activities even though it receives no specific funds for such projects. A pilot project to evaluate the use of actimeters to measure activity may prove useful in the search for meaningful study endpoints. Dr. Murphy did not provide an expected date for the conclusion of this study. Getting Through SSA's System Mr. Barry Eigen gave a basic presentation on the benefits available through SSA and the process by which benefit determinations are made. He provided a definition for disability and stated the requirements for entitlement to Social Security Disability Income (SSDI) benefits. A person who is disabled must prove that he/she is unable to engage in "substantial gainful activity," which is demonstrated by an inability to work continuously for at least one year. He stated that a "medically determinable impairment is shown by anatomical, physiological or psychological abnormalities demonstrable by medically accepted clinical and laboratory diagnostic techniques. In other words, objective clinical and laboratory findings and not symptoms." He emphasized this point, declaring that an individual's diagnosis is not the central issue; objective proof of impairment is. Mr. Eigen stated that since 1988, Congress has made an annual request of the SSA to follow CFS research to ensure that cases are being handled appropriately. He claimed that they have, indeed, kept abreast of research developments. He expressed confidence that as objective markers for the disease are found, the approval rate for SSDI benefits will increase significantly. Finally, he noted that reviews of applications for disability for CFS revealed no correlation between those who meet the CDC case definition for CFS and those who are found to be disabled by SSA standards. Memorable Questions; No Real Answers Written Question: "How and when will CFS be given a clinical diagnosis; the medical diagnosis books I have seen do not include CFS." Dr. William Reeves: "This is a difficult question to address and I'll give a partial cop-out answer. The CDC, the NIH and the FDA are charged with public health and public health research. We are not charged with determining standards of care and standards for establishing clinical diagnoses. We are trying to come up, in this particular case, with a research case definition which for all practical purposes will probably be used in a clinical setting." Written Question: "What can you say is the actual prevalence of CFS in the US population?" Dr. Reeves: " ...We are trying to get away from just counting up the number of people who may have this. I don't think that's the major impact; I think the major impact is why people get it, what it does to them, what the clinical course is ... I can't give you a number." Ms. Kim Kenney: "Dr. Schluederberg can you tell us, of the provisions in the NIH Revitalization Act that are specific to CFIDS, what the current status of enactment of these provisions is and what programs are in place to make sure that law is carried out?" Dr. Ann Schluederberg: "[We've discussed the study section.] Would you prompt me on some of the other actual provisions that are in the law? ..." Ms. Kenney: "... the appointment of private sector scientists and patient advocates to appropriate advisory committees across the NIH." Dr. Schluederberg: "People have been nominated for inclusion on committees ... We cannot make the nominations public ... It is not appropriate to say who is nominated ... We certainly have opened the meetings to the participation [of CFIDS clinicians and patient advocates]." Ms. Kenney: "Dr. Reeves, this year the Association spent considerable time, money, and effort getting the appropriations committee of the Senate to appropriate $2 million in earmarked funds for CFS research at the CDC. Can you tell me how that money might be used and what the research priorities are for that new money coming to your agency?" Dr. Reeves: "We don't have it yet. ... But, we will continue in the areas we are working in. What you saw in my presentation was how we're using the money we have and until we actually have the money I can't deal in that sort of speculation." Ms. Kenney: "[Dr. Reeves] can you tell me what the process is for reporting cluster outbreaks to the CDC?" Dr. Reeves: "We receive reports of cluster outbreaks from physicians, state health departments, etc. ... The reports should be addressed directly to me. ... Let me just clarify one thing with regard to cluster outbreaks; we are a federal organization; in order to investigate an outbreak, we must be invited by the state governments to do so. That's the way we've investigated the two that have been done." Ms. Marya Grambs: "Can you tell us how many people in the country are currently seeking Social Security benefits for chronic fatigue syndrome?" Mr. Barry Eigen: "Unfortunately not. We don't have a listing for chronic fatigue syndrome and so, because of the way our computer system is set up, we can't track cases. However, in August we created a special computer program just for people with chronic fatigue syndrome and we are hoping that in the next few months we will be able to tell you about new cases." Ms. Kenney: "The manufacturer of Ampligen, a potentially promising drug for the treatment of CFIDS, has decided not to continue the pursuit of licensure for Ampligen at this time due to financial difficulties. Is there some way FDA could facilitate the production of Ampligen?" Dr. Dianne Murphy: "I was trying to relay that the FDA's mandate is to assure the efficacy and safety of products. ... We cannot provide product and we cannot produce product. We can only encourage companies to look at certain things ... but we can't make them produce a product they don't want to produce." Mr. Robert Landau: "We've found in New Jersey that there has been tremendous variation between SSA offices. It seems very consistent and almost haphazard. What would appear to be milder cases seem to go right through the process and get approved on the first application. Others, who have more severe and profound problems, sometimes go on for 2 or 3 years before they get benefits." Mr. Eigen: "Would you be willing to send me information on this occurrence? It's something we're always interested in because it happens not just with these cases but for all kinds of disabilities." Notable Quotes: Patient Advocates Join to Voice Their Displeasure "With all due respect ... even the make-up of yesterday's panel ... shows that you still do not fully understand how to even look for answers to this tragic illness." "There was an Oscar-nominated movie last year called 'A Few Good Men.' In the climactic courtroom scene, the hot-shot young attorney turns to the grizzly base commander and demands, 'We want the truth!' The commander shoots back across the stunned courtroom, 'You can't handle the truth!' Ladies and gentlemen, up to this moment, a better script could not have been written to describe the abysmal response of the United States government and the U.S. medical community to this vicious epidemic." "... all I can say is calling this terrible illness chronic fatigue syndrome is akin to calling General Sherman's march through Atlanta the story of a boy with a match." "If a woman has ovarian cancer and then she gets depression, no one says, 'Oh, now you've got depression; you can't have ovarian cancer; that's negated.'" "... [There is] a movie on HBO called 'And the Band Played On.' Please, I beg of you, change the name and do not replicate that movie, so that 10 years from now the story of CFIDS will be 'And the Band Played On #2.'" Thomas Hennessy, Jr. R.E.S.C.I.N.D. "I find it abhorrent that you have a person on your committee who publicly, on several occasions, to large numbers of people, has ridiculed people with chronic fatigue syndrome." "The term[s] 'fatigue' and 'chronic fatigue' never existed in this entity until it was put into [the name] in 1988 ... the whole concept of fatigue has warped our understanding of this illness." Dr. Byron Hyde The Nightingale Foundation "... I'm wondering if one of the priorities of the agency's committees is to take a real point position and educate the primary care physicians in the care of chronic fatigue syndrome patients." Dr. Nancy Klimas University of Miami "I believe there is a big [CFS] problem in the healthcare profession ... if there are no front-line healthcare workers to take care of you, we have a problem." Meghan Shannon CFIDS Support Group of Northern San Diego "I hear once again the '97 percent white' statistic came up. This is a number that comes out of a study, not a number that comes out of reality. ... In a city like Atlanta, where there are many black professionals in the teaching field, where we all know CFS is rampant in the healthcare field and in the airline industry, there are a great number of black people suffering from chronic fatigue syndrome who have not been identified. ... Those of you from the east and the west coast are aware that there must be many Asians and Hispanics who are not being reached ... With a serious outreach effort, I think the number and distribution of the population of CFS will become much clearer. But, it does take an effort. Please make the effort to reach out a little further." Wilhelmina Jenkins Northeast Atlanta CFIDS Support Group In contrast to Monday's meeting, which was restricted to case definition issues, the meeting on Tuesday encompassed all aspects of the government's response to chronic fatigue and immune dysfunction syndrome. In preparation for the meeting with Dr. Lee, government officials were given the opportunity to provide the CFIDS community with a status report on their agencies' activities to solve the chronic fatigue syndrome crisis. Patient advocates, in turn, were allowed time to voice their concerns and recommendations for further action. This meeting, momentous because it was the first CFSICC meeting open to the public, provided the necessary medium for a healthy exchange of information and opinions. One thing became clear in Atlanta: Dr. Lee's leadership will be the most critical factor in generating a committed and tenacious government effort to solve the puzzle of chronic fatigue and immune dysfunction syndrome. We are working diligently to preserve his commitment to effect the changes needed to justly serve PWCs. Leading Advocates Kim Kenney & Marya Grambs Challenge the CFSICC September 28, 1993 Good afternoon. I am Kim Kenney, Executive Director of The CFIDS Association of America, the world's largest and most active non- profit organization dedicated to conquering CFIDS. Since 1987, our association has grown to represent more than 23,000 members. We have funded over $1,500,000 in research grants, responded to over 200,000 inquiries by sending out information packets free-of-charge, and published and distributed hundreds of thousands of copies of our journal, The CFIDS Chronicle. Our primary purposes and goals are to fund private research, broadly disseminate current information, and influence government to aggressively pursue all aspects of a comprehensive public health response, including research, prevention, care, and education. We work to accomplish these goals by collaborating with more than 400 state and local support groups nationwide. Because the association was founded by a group of patients working with thousands of other patients and a handful of clinicians exploring CFIDS, from the beginning we have known that CFIDS in not just fatigue, not depression D that it is a distinct clinical entity, a real disease, that it can be a serious disease, and that it is spreading. As an organization, we have focused our research efforts on finding the cause or causes of and markers for CFIDS; establishing the basic epidemiology of the disease and identifying effective treatments for it. Because finding the cure for CFIDS is the ultimate goal, we feel that these three avenues of investigation hold the most promise. In addition to our research initiatives, through the focused efforts of our Public Policy Advisory Committee and our professional Washington, D.C. representative, Tom Sheridan, our Association leads a national grassroots advocacy effort. Our accomplishments in funding promising research provide an important and critical foundation for furthering the understanding of this disease. But in order to conquer CFIDS, we know that the government must take the lead on the vigorous investigation of this disease. And we are committed to making that happen. Through our legislative initiatives, we have enrolled the considerable assistance of the Congress to help us secure adequate funding and oversight language to make sure that federal agencies are responsive to the needs and demands of PWCs. The very existence of and funding for this Committee has been legislated by Congress as a direct result of our efforts. We anxiously await its first meeting with Dr. Phil Lee as chair, which we understand will take place in October or November. At the next meeting we look forward to CFIDS- specific presentations that describe program priorities, seek appropriate budget levels, and provide oversight for purposes of accountability. I would like to take this opportunity to review with the members of this committee the public policy initiatives that we have established for each of the federal agencies represented here today. We have outlined these initiatives in a letter that was delivered to a mem-ber of then President-Elect Clinton's transition team on December 9 of last year. A copy of this memo is included in your pack-age. I will follow the order of this morn-ing's pre-sentations. Centers for Disease Control We feel that yesterday's meeting addressed one of our top priorities for action at CDC. As I stated yesterday, the current case definition has become outdated in the five years since it was published. We are hopeful that a satisfactorily revised definition will incorporate first and foremost a new name for the syndrome D chronic fatigue and immune dysfunction syndrome. In the interest of time, I will not reiterate the other nine changes we recommended to the panel yesterday, but will summarize by saying that the case definition should be amended to more accurately describe the symptom complex as one which is a multi-systemic disorder of the entire body D not just chronic fatigue. We also request that the revised case definition be published in the MMWR and JAMA or the New England Journal of Medicine in order to continue the efforts to better educate the medical community about this illness. The Surveillance Study which has been underway since 1989 has served its original purpose as a prototype study designed to determine a point in time minimum prevalence of CFS in four U.S. cities. It should be noted that this study severely underrepresents the prevalence of this disease in people of color and among lower socioeconomic groups. This is evidenced by the CDC's own data. Dr. Reeves indicated this morning that the study has found that 50 percent of the women identified make more than $50,000 per year. This study must be replaced with a community based study to more accurately determine the nationwide prevalence of CFIDS under a revised case definition. The U.S. Senate Appropriations Committee has recently passed legislation that will require this. We expect this mandate to be passed into law within a very few days. Further, we request a full debriefing of current investigations of possible cluster outbreaks. We also need to learn how these outbreaks are to be repor-ted to the CDC and what procedures are in place for following up on such reports. It is imperative that this information be disseminated to state and local departments of health across the country as well so that CDC will be informed of every sus-pected clustered occurrence of this disease. As part of its responsibility to document the basic epidemiology of CFIDS, the CDC must work diligently to understand how this disease is transmitted and must prepare to provide appropriate education programs for the medical community and the public at large about CFIDS and any health risks associated with it. It is absolutely imperative that we stop the continued spread of this disease. National Institutes of Health The NIH should be the lead agency for the critical biomedical investigations into the causal agent(s) for this disease. Through a collaborative effort of the many NIH institutes and extramural researchers we feel there is an excellent chance that definitive marker(s) and ultimately a causal agent or agents will be identified. We were encouraged by Dr. Stephen Straus' publication earlier this year and believe that focused investigations in virology, immunology, rheumatology, and autoimmune and infectious disease specialties will prove to be enlightening. Through the implementation of the CFIDS provisions of the NIH Revitalization Act, we look forward to more focused research efforts and better representation of private sector research and patient advocate needs and expectations. We look forward to working with Dr. Schluederberg and others to accomplish the intentions of our President and Congress in passing this important legislation. We understand that present funding levels remain far below those needed to pursue comprehensive investigations. We are committed to working through Congress to increase these resources provided our partnership remains strong and our participation in the activities at NIH is welcomed and appreciated. Food and Drug Administration FDA has reviewed only one application for a drug to be used to treat persons with CFIDS and this would, of course, be Ampligen. The present situation with HEM Pharmaceuticals and its decision to discontinue studies in this population for financial reasons has drawn attention to the urgent need for the FDA to develop a formal policy for the compassionate care of patients who need to remain on, return to, or be placed on Ampligen. And, for the sake of those patients who have seen dramatic improvement on Ampligen, we ask that a policy be developed to allow CFIDS patients access to Ampligen or any potentially promising drug at the earliest opportunity after safety is established. There are nine patients who have just been withdrawn from Ampligen. The lack of either a compassionate care policy or a policy to provide access once safety is established may put their very lives in danger. We are encouraged by recent activities within the Division for Antiviral Drugs at FDA to become familiar with CFIDS and by efforts there to identify endpoints for future drug studies and we ask that patient advocates, researchers, and clinicians be formally notified of and invited to each meeting. We request that this work continue. We also ask that a streamlined process through which potentially promising drugs that can treat CFIDS be investigated and approved without delay, given the lack of treatment options available to clinicians and patients alike. Social Security Administration Many persons with CFIDS are disabled by the physical and cognitive deficits they suffer as a result of this illness. Those who are disabled are incapable of employment and require considerable medical treatment. The Social Security benefits to which these persons are entitled are frequently denied or delayed based on the inability of SSA employees to efficiently process CFIDS claims. We receive sporadic reports from areas in which claims are processed quickly and benefits awarded before the claim reaches the administrative court review level. This situation seems to occur most frequently in the areas in which CFIDS support groups have taken the initiative to educate local SSA employees about the disease. We are confident that more consistent handling of these claims can be accomplished with a few minor, yet significant action steps on the part of SSA officials. We strongly recommend that an SSA CFIDS Advisory Committee be established. This committee would regularly review current medical standards and investigate the needs of regional SSA offices with regard to training and information resource needs. We also urge Mr. Eigen to continue his participation in this committee and to stay informed of the latest in CFIDS research. To summarize, the message I would like to leave with this committee as it prepares for its first meeting with Dr. Lee as chair is that The CFIDS Association of America is committed to achieving a unified and dedicated response to CFIDS from our federal public health agencies. We wish to make use of the political resources we have to expand the resources each of you has so that together we can beat CFIDS. We look forward to forming and maintaining a productive partnership characterized by mutual respect and reciprocity. Thank you Marya Grambs The subject of my presentation to you was initially going to be "the San Francisco model." I had intended to tell you about the unique partnership we've developed in San Francisco D about how for several years we've worked very closely with the San Francisco Department of Public Health and the California Department of Health Services, as well as with Dr. Philip R. Lee, who was chair of our Public Health Commission and director of the Institute for Health Policy Studies. I was going to talk to you about the process of working with the DPH and the California Health Department to develop a videotaped grand rounds on the diagnosis and treatment of CFIDS, and to write an algorithm for Fatigue and Treatment and Laboratory Protocols for CFIDS that were published in the San Francisco Epidemiological Bulletin and the California Morbidity report. And I was going to tell you about our outreach program to clinics serving ethnic communities so they can become aware of how to identify and treat CFIDS and about our work with the CDC on both a cluster outbreak study and a population-based epidemiological study in diverse communities. But I'm feeling a bit discouraged these past two days and I feel I have to be a bit more direct. What I'd like to talk to you about today is democracy D the democratization of public health. What this means, specifically, is that clinicians and medical researchers can no longer work in isolation, but must work in collaboration with patients and their advocates. It means that we are now in an era of teamwork, in which all sides of the equation D patients, researchers, and clinicians D must come together to work on the problems of disease and public health. And if that happens, if we as patient advocates are invited at the outset we will come to you cooperatively and collaboratively. But, if we are invited after the fact, we will come with anger. Yesterday a few people came up to me and said, "This meeting about the case definition is a sham. It was put on to placate the patients. They are going to go back behind closed doors and do whatever they want." I said that was ridiculous. That it was paranoid. And I didn't believe it. However, when I saw the composition of the case definition panel and learned that indeed it was meeting today behind closed doors, I began to wonder if I had spoken too hastily. The other patient advocates and I are extremely concerned about the inadequate representation of clinicians deeply experienced with CFIDS. In order to do accurate and relevant research that enables us to learn more about CFIDS, any case definition must be grounded in the broadest, deepest, and most precise clinical knowledge possible about the illness; and this information resides in large part in those physicians who have seen the greatest numbers of patients, over the greatest number of years, and who have earned the highest confidence in patients and their advocates. Unfortunately, these physicians are not represented on your panel. Therefore, we will be presenting you with a list of clinicians that we recommend for inclusion, and request that you accept several. With regard to this Interagency Coordinating Committee, you know that Congress mandated consumer representation on this Committee. Why are there no consumers behind that blue-skirted table today? We firmly expect that when the Committee officially meets before December 15 there will be adequate consumer representation. And we would like to be informed of the process for that selection. We have been informed that on November 18 and 19 there will be a clinical workshop sponsored by NIH/NIAID on the clinical management of CFIDS. We have also been informed that for two days clinicians will gather to talk about how they manage CFIDS patients, but there will not be any opportunity for patients to talk to the clinicians. As we told Dr. Schluederberg yesterday, that is unacceptable. We insist that a forum be provided at that workshop for patients to present to and react to the presentations made by the physicians, much in the way it is done at AIDS conferences, and was done at the first National Medical Conference on CFIDS held in San Francisco several years ago D a conference which Dr. Lee was the keynote speaker for. We also request that pediatrics be included and that the importance of support groups in the overall management of the illness be addressed. [Editor's Note: Kim Kenney has since been invited to speak on the benefit of support groups; Dr. James Jones will address pediatrics; and a panel of patients will offer personal information about what it is like to live with CFIDS.] We understand from Dr. Schluederberg that some consumer representatives have been nominated for the NIAID Advisory Committee and perhaps other advisory committees at NIH. We would like to know the process for nomination and we expect there will be west coast representation. A few other points: We are disturbed by the trend we noticed yesterday at the case definition meeting that would open up the case definition essentially to anyone suffering severe fatigue for a prolonged period of time. While that would certainly increase the prevalence rates, it would create such a giant black hole as to be meaningless for research or clinical purposes; and would ultimately have the effect of further reducing believability in CFIDS as a distinct clinical entity. Dr. Reeves, it is irresponsible for you to publish, as you have in this draft CDC pamphlet on CFS, that the prevalence of CFIDS as determined by the CDC is 2 to 7 per 100,000 or 3000 to 10,000. You know that if you combined all the CDC-identified CFIDS patients in current and recent study protocols they would amount to more than 3000. And your study only captured white women with incomes averaging $50,000. The only way we feel it would be acceptable to publish that number is if you qualify it clearly and strongly with the descriptors that this is a serious underestimation (which were your words yesterday); and if you show as comparison the rates other investigations report. One final comment: believe it or not, those of us presenting today are the polite ones and we are the reasonable ones. There are a great many other CFIDS patients out there who are not so polite or reasonable. And believe me, we can get them here. So we sincerely hope you are amenable to working with us on these issues and resolving them satisfactorily.