Draft Recommendations of the CFS Name Change Workgroup

The following message from the DHHS CFS Coordinating Committee's (CFSCC) Name Change Working Group (http://www4.od.nih.gov/cfs/default.html) was released on the CFSCC announcement list on Wednesday.

The Name Change Working Group was established by the federal CFS Coordinating Committee of the Department of Health and Human Services. Its purpose has been to study name change issues and make recommendations for a new name for "chronic fatigue syndrome." Towards that goal, the workgroup has been meeting every two weeks for the past 18 months.

The document below is an interim draft of the workgroup's recommendations. It is being distributed to update interested parties. Also enclosed is an informal questionnaire to obtain feedback.

We would appreciate it if you would read the attached Name Change Proposal and then provide the Name Change Workgroup with your feedback by completing the questionnaire and returning it to either:

1) to cfs@od.nih.gov <> and put "Name Change" in the subject line, or

2) by mail: Name Change Workgroup, c/o Ms. Janice C. Ramsden, National Institutes of Health, 1 Center Drive, MSC 0159, Building 1, Room 333, Bethesda, MD 20892-0159.

(A) Draft Recommendations of the Name Change Workgroup

I. Introduction

The illness known as chronic fatigue syndrome (CFS), has over the years been referred to by a variety of names. Because the names for this illness are widely believed to be inadequate, the CFS Coordinating Committee established the Name Change Workgroup (NCW). Its charge was to investigate name change issues and present name change recommendations. The NCW reviewed the published CFS/ME literature, communicated with researchers, patients, and physicians, and conducted a survey to further gauge opinions of various stakeholders. Based on these communications, the NCW has established that there are several different groups of stakeholders with strong feelings about changing the name. To assess all the data, the NCW held regular discussions for 13 months and debated the relative merits of stakeholder concerns, related issues and a variety of potential names. Based on our discussions, the NCW concluded the following:

1. Many patients and physicians believe that the current name, CFS, too narrowly focuses upon a single, poorly defined symptom (fatigue) and profoundly promotes misunderstanding of the illness.

2. Patients feel the name CFS has substantially contributed to the disparaging manner in which they are perceived and treated by physicians, family, and the public in general. They also believe that this misunderstanding has directly and negatively impacted the quality of medical care and support they are able to obtain. Research by Dr. Leonard Jason validates the adverse influence of name impact (1).

3. No one name is the obvious choice based on the current state of the science, nor can a single name fulfill all of the demands of all interested parties. Therefore, we recommend that the new name serve as an umbrella term. Under that term, subgroups of patients can be more accurately stratified according to variations in illness presentation, pathophysiology, results of diagnostic testing, or other factors.

4. This condition is a serious illness, which like several other significant and recognized conditions, is best categorized as a syndrome. This syndrome is a collection of signs and symptoms that when taken as a whole under the appropriate conditions, defines this illness. Utilization of this approach in this condition is analogous to the medical community's traditional approach to other serious, organic syndromes such as Organic Brain Syndrome, Sjogren's Syndrome, and Multiple Sclerosis.

II. Factors and Formulation of a New Name

Formulation of a new name was guided by at least four important principles. First, the new name must not imply that the etiology of this syndrome or its pathogenesis is understood by the biomedical community. Second, the name must reflect the common symptoms reported by most patients with this condition without overemphasizing any one system. Third, data which has been published in peer-reviewed literature must lend support to the new name. Fourth, the name must include language that reflects the fact that the illness is chronic.

The number of symptoms reported by patients with this syndrome is very large (2). However, most of the commonly reported symptoms are associated with or may be indicative of an aberration or dysfunction in one or more of these systems neurologic, neuroendocrine, and the immunologic systems. The following selected scientific publications provide a sound basis for a new name that is based on common patient symptoms associated with these systems. The articles were selected because they have withstood scientific scrutiny and represent critical findings. There are other publications available, but the chosen articles are widely respected, cited, and felt to be representative of the current understanding of the science. For purposes of this document, the articles have been categorized into their relevant subsections pertaining to each of the systems.

A. Neurological

Autonomic nervous system (including orthostatic intolerance)

Several authors have published findings demonstrating that some of the symptoms seen with this syndrome are associated with autonomic nervous system dysfunction.

Bou-Holaigah I, Rowe PC, Kan J, Calkins H. The relationship between neurally mediated hypotension and the chronic fatigue syndrome. JAMA 1995; 274:961-967.

Schondorf R, Freeman R. The importance of orthostatic intolerance in the chronic fatigue syndrome. 1999 Am J Med Sci 1999;317(2):117-123.

Freeman R, Komaroff A. Does the chronic fatigue syndrome involve the autonomic nervous system? Am J Med 1997;102:357-364.

Neuroendocrine system The best studied evidence of neuroendocrine dysfunction involves the hypothalamic-pituitary-adrenal axis.

Demitrak MA, Dale JK, Strauss SE, et al. Evidence for impaired activation of the hypothalamic-pituitary-adrenal axis in patients with chronic fatigue syndrome. J Clin Endocrinol Metab 1991;73:1223-1234.

Scott LV, Medbak S, Dinan TG. Blunted adrenocorticotropin and cortisol responses to cortocotropic-releasing hormone stimulation in chronic fatigue syndrome. Acta Psychiatr Scand 1998;97:450-457.

Neurocognitive

Neurocognitive symptoms are reported with relatively high frequency in this syndrome. Many meritorious articles have been published, but at least one seems to be scientifically robust and has not been substantially challenged by other publications.

DeLuca J, Johnson SK, Ellis SP, Natelson BH. Cognitive functioning is impaired in patients with chronic fatigue syndrome devoid of psychiatric disease. J Neurol Neurosurg Psychiatry 1997;62:151-155.

Sleep studies

Complaints of sleep disturbances are common in this patient group. Two independent research teams have published separate studies supporting the high-frequency of sleep dysregulation. These studies have also found the sleep dysregulations are not related to psychiatric disorders, and there are differences between patients diagnosed with this syndrome versus multiple sclerosis or normal controls.

Buchwald D, Pascualy R, Bombardier C, Kith P. Sleep disorders in patients with chronic fatigue. Clin Infect Dis 1994;18(suppl. 1):S68-72

Krupp LB, Jandorf L, Coyle PK, Mendelson WB. Sleep disturbance in chronic fatigue syndrome. J Psychosom Res 1993;37:335-331.

B. The Immunologic System

Several articles had been published investigating the relationship between the immunologic system and chronic fatigue syndrome. The best validated work and most consistent findings demonstrate decreased function of natural killer cells and reduced responses of T cells to mitogens and other specific antigens. The literature also supports evidence of chronic immune activation in CFS, with increasing emphasis on cytokine dysregulation.

Caligiuri M, Murry C, Buchwald D, et al. Phenotypic and functional deficiency of natural killer cells in patients with chronic fatigue syndrome. J Immunol 1987;139:3306-3313.

Hanson, S.J., Gause, W., & Natelson, B. (2001). Detection of immunologically signficant factors for chronic fatigue syndrome using neural-network classifiers. Clinical and Diagnostic Laboratory Immunology, 8, 658-662.

Klimas NG, Salvato FR, Morgan R, Fletcher MA. Immunologic abnormalities in chronic fatigue syndrome. J Clin Microbio 1990;28:1403-1410.

Patarca R, Klimas N, Sandler D, Garcia MV, Fletcher MA. Interindividual immune status variation patterns in patients with chronic fatigue syndrome: association with gender and tumor necrosis factor system. J of CFS 2(1):7-41, 1996.

Cannon JG, Angel JB, Abad LW, Vannier E, Mileno MD, Fagioli L, Wolff SM, Komaroff AL. Interleukin-1 beta, interleukin-1 receptor antagonist, and soluble interleukin-1 receptor type II secretion in chronic fatigue syndrome. Journal of Clinical Immunology 17(3):253-61, 1997.

Sudaholnik RJ, Peterson DL, O'Brien K, Cheney PR et al. Biochemical evidence for a novel low molecular weight 2-5A-dependent Rnase L in chronic fatigue syndrome. J of Interferon&Cytokine research. 17(7):377-85, 1997

III. A Change in Name

The NCW recommends that the name of the syndrome be changed to chronic neuroendocrineimmune dysfunction syndrome, or CNDS. This is recommendation is based on 1) the profile and frequency of the commonly reported symptoms of patients with chronic fatigue syndrome, 2) the chronicity of the illness and the lack of understanding of its cause(s) and, 3) the published evidence supporting an aberration or dysfunction of the neurological and immunologicsystems. The name is in accordance with the principles outlined in SectionII., above. Changing the name to CNDS does and should not imply that the etiology or pathophysiology is understood. This name is broad enough to encompass the most commonly reported symptoms. It is quite reasonable to conclude that the commonly reported symptoms are associated with or referable to the neurologic, neuroendocrine, and immunologic systems. Finally the name explicitly states that the disorder is chronic.

IV. Utilization of CNDS

Advances in biomedical research may ultimately discover the pathophysiology or cause(s) of CNDS. Until the etiology is known, the name CNDS should be used for the reasons outlined above. The NCW anticipates that the biomedical community may find that subgroups or subtypes of CNDS may provide useful nosology (e.g., CNDS--orthostatic intolerant-predominant). Thus, the use of the name CNDS in conjunction with subgroup stratification offers flexibility and adaptability to the inevitable advances based on scientific research. This approach also promotes more accurate understanding of the illness when compared with the current name, chronic fatigue syndrome.

In the past there have been many efforts to categorize the syndrome based on a variety of criteria. Some of the more prominent of these potential subgroups have been used by scientists and patients, and will be reviewed below. The NCW does this in an effort to provide a conceptual framework for the name CNDS, and to better define the status of other names in use vis-à-vis our recommendations.

1) CFS: CFS is a term first introduced in 1988 in conjunction with the research case definition (Holmes, et al, 1988). It was maintained in the revision of the 1994 case definition (Fukuda et al., 1994). The 1994 definition is being used by researchers internationally and is in the process of being revised by an international working group. A research case definition is designed to specifically define a research study population that excludes those potential study candidates who do not meet the criteria. The research case definition attempts to identify and categorize a homogeneous group of patients. However, some of the criteria are so restrictive that some patients who really do have the syndrome fail to meet the research case definition. Though the term CFS should refer to the research case definition, it has been used for all practical purposes to define all individuals with this condition. A research definition by its very nature should be used for research purposes only, not for clinical or diagnostic use in general practice. Scientists may continue to use the research case definition to identify homogenous groups of patients in order to compare the participants across different settings.

2) ME/CFS: A consensus panel in Canada has recently proposed a clinical case definition. The proposed criteria differ from and are broader than the Fukuda criteria for CFS. These criteria were developed specifically to be used in clinical practice.

3) ME: Myalgic encephalomyelitis (ME) is a condition first mentioned in the literature in the 1950s by Dr. Melvin Ramsey. It describes a condition similar to CFS. Myalgic means muscle pain and encephalomyelitis means an acute inflammation of the brain and spinal cord. Some patient groups have endorsed the term myalgic encephalopathy, because the term encephalopathy does not necessarily require an inflammation in the central nervous system. To be classified with ME according to the London criteria (3), patients are required to report the occurrence of post-exertional malaise, impairment of memory and concentration for a period of 6 months or longer, and a fluctuation or cycling in the severity of symptoms. Other groups subscribe to a description provided by Ramsey (4, 5).

4) Post-infectious fatigue syndrome (6) follows an infection or is associated with a current infection. According to the definition, individuals with this subtype should also fulfill the following additional criteria: definite evidence of infection at onset or presentation, presence of the syndrome for at least six months after onset of infection, and corroboration of the infection by laboratory evidence.

V. Conclusion

The NCW urges the CFS Coordinating Committee to adopt the name CNDS. In conjunction with potential subgroup stratification, we believe the new name meets the current need for a more accurate label for the illness while allowing room for sub-grouping as biomedical advances take place.

References

(1) Jason, L.A., Taylor, R.R., Stepanek, Z., & Plioplys, S. (2001). Attitudes regarding chronic fatigue syndrome: The importance of a name. Journal of Health Psychology, 6, 61-71.

(2) Komaroff AL, Buchwald D. Symptoms and signs of chronic fatigue syndrome. Rev Infect Dis1991;13(Suppl 1):S8-11

(3) Dowsett, E. G., Ramsay, A. M., McCartney, R. A., & Bell E. J. (1990). Myalgic Encephalomyelitis - a persistent enteroviral infection?. Postgraduate Medical Journal, 66, 526-530.

(4) Leading article. A new clinical entity? Lancet, May 26, 1956, pp. 789-90.

(5) Ramsay, M. (1988). Myalgic encephalomyelitis and postviral fatigue states: The sage of Royal Free disease. 2nd edition. Gower Medical Publishing, London.

(6) Sharpe, M.C., Archard, L.C., Banatvala, J.E., Borysiewicz, L.K., Clare, A.W., David, A., Edwards, R.H.T., Hawton, K.E.H., Lambert, H.P., Lane, R.J.M., McDonald, E.M., Mowbray, J.F., Pearson. D.J., Peto, T.E.A., Preedy, V.R., Smith, A.P., Smith, D.G., Taylor,D.J., Tyrrell, D.A.J., Wessely, S., White, P.D., Behan, P.O., Rose, F.C., Peters, T.J., Wallace, P.G., Warrell, D.A., & Wright, D.J.M. (1991). A report-chronic fatigue syndrome: guidelines for research. Journal of the Royal Society of Medicine, 84, 118-121.draft_recommendations.htm