EDS
Fatigue Is a Frequent and Clinically Relevant Problem in Ehlers-Danlos Syndrome

https://doi.org/10.1016/j.semarthrit.2009.08.003Get rights and content

Objectives

Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Fatigue and musculoskeletal pain are associated features but have never been studied systematically. We used a multidimensional assessment method to measure fatigue, its clinical relevance, and possible determinants.

Methods

A questionnaire study was performed among 273 EDS patients. The following dimensions were assessed: fatigue severity, functional impairment in daily life, physical activity, psychological distress, sleep disturbances, concentration problems, social functioning, self-efficacy concerning fatigue, causal attribution of fatigue, pain, and disease-related factors.

Results

More than three-quarters of EDS patients suffer from severe fatigue. Patients who are severely fatigued are more impaired than nonseverely fatigued patients and report a higher level of psychological distress. The 5 possible determinants involved in fatigue are sleep disturbances, concentration problems, social functioning, self-efficacy concerning fatigue, and pain severity.

Conclusions

This is the first study of fatigue and its possible determinants in EDS and shows that fatigue is a frequent and clinically significant problem in EDS. The 5 possible determinants of fatigue could form a starting point for the development of an effective cognitive behavioral intervention for fatigue in EDS.

Section snippets

Patients

A cross-sectional design was used to assess fatigue in patients with EDS. We asked 500 members of the Dutch patient organization of EDS (VED: www.ved.nl) to participate, and 19 additional patients were recruited from the outpatient departments of the Radboud University Nijmegen Medical Centre; these latter patients were also included in a clinical study on neuromuscular features in EDS (14). Three hundred twenty-seven questionnaires were returned (63% response rate). The intention of the study

Results

Excluded were patients who were younger than 16 years (n = 16), patients in whom EDS had not been diagnosed (n = 30), and patients who had incompletely filled out the CIS fatigue severity scale (n = 8). Hence, 273 patients were included; in 53 of them the specific type of EDS was not (yet) known, although EDS was diagnosed by a medical specialist. In all other patients the EDS type was classified according to the diagnostic criteria (1). Demographic characteristics of the patients are

Discussion

This is the first in-depth questionnaire study of fatigue in EDS and shows that fatigue is a frequent and clinically relevant problem in EDS: 77% of EDS patients suffer from severe fatigue; and patients who are severely fatigued are more impaired and report a higher level of psychological distress. Patients with the hypermobility type EDS are most often severely fatigued. Furthermore, severe fatigue in EDS is related to sleep disturbances, concentration problems, social functioning,

Acknowledgments

We are grateful to R. Janse and C. Kruip for assistance in storage and analysis of the data, and to the members of the Dutch patient organization of EDS for filling out the questionnaires.

References (33)

  • J. Schalkwijk et al.

    A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency

    N Engl J Med

    (2001)
  • G.H. Burch et al.

    Tenascin-X deficiency is associated with Ehlers-Danlos syndrome

    Nat Genet

    (1997)
  • B. Steinmann et al.

    The Ehlers-Danlos syndromes

  • F. Gawthrop et al.

    Ehlers-Danlos syndrome

    BMJ

    (2007)
  • J.S. Kalkman et al.

    Experienced fatigue in facioscapulohumeral dystrophy, myotonic dystrophy, and HMSN-I

    J Neurol Neurosurg Psychiatry

    (2005)
  • S.R. Schwid et al.

    Fatigue in multiple sclerosis: current understanding and future directions

    J Rehabil Res Dev

    (2002)
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    N.C. Voermans was supported by a research grant of the NWO (Netherlands Organization for Scientific Research). The NWO had no influence on the design or outcome of the study; hence, N.C. Voermans was independent of this sponsor. None of the authors have any conflicts of interest to disclose. All authors of the article had access to the original data, have fulfilled the criteria for authorship, and have read and approved the final version of the article.

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