Background Ehlers-Danlos Syndromes (EDS) are a group of hereditable disorders of connective tissue characterized by joint hypermobility, skin hyperextensibility and tissues fragility. Patients with EDS often show symptoms that affect of significant form their quality of life, but that are not totally recognized by the health professionals.
Objectives The purpose of this study is to describe and to analyze the epidemiological characteristics and the health related quality of life (HRQOL) in patients with EDS.
Methods We have realized a descriptive transversal study. We sent the Questionnaire of Health (MOS) SF-36 to investigate the health related quality of life, the Brief Pain Inventory for the Evaluation of the Pain (BPI-CBD), the scale of Intensity of the Fatigue (FSS) and a demographic questionnaire to 211 potential participants. In all the cases, the surveys were auto-administered, and the confidentiality of the information was kept to the maximum. The included variables evaluate demographic date, mobility, personal care, usual activity, pain, fatigue and distress / depression. The information was analyzed using SPSS 15.0 for Windows. To measure the reliability of the results we used Cronbach’s alpha coefficient.
Results We evaluated 126 questionnaires, which means a response of 59 %. 67,3 % was women and 32,7 %, males, with an average ages of 28,8 ± 10,9 years. 11,5 % presented a very impaired HRQOL, 46,2 % moderately impaired and 42,3 % lightly impaired. Only 4,8 % of the survey respondents indicated that EDS does not affect their activities of the daily life. Practically in all aspects, the HRQOL in patients with EDS was significantly worse than is considered to be normal in the general population.
Conclusions: Conclusions In our study it is demonstrated that the physical functioning, the pain and the emotional role are the fields with a more significant impact, therefore we can conclude that the injuries, the pain, the fatigue and the anxiety / depression are possible factors that contribute to the limited quality of life in the patients with EDS. The health professionals should use a multidisciplinary approach to treat with efficiency the symptoms that appear associated to EDS, and in this way to help to improve the quality of life of patients with EDS.
Acknowledgements We thank the asedh patients for their cooperation partnership.
Disclosure of Interest None Declared
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