• Larissa Kalisch, Claude Hamonet, Caroline Bourdon, Lucile Montalescot, Cécile de Cazotte, and Carolina Baeza-Velasco.
• Department of Psychology, Anglia Ruskin University, Cambridge, UK.
• Disabil Rehabil. 2020 Dec 1; 42 (25): 3679-3686.

AbstractAim: Among the Ehlers-Danlos syndromes, the hypermobile subtype (hEDS) is the most common. The variety, accumulation and duration of the painful symptoms make hEDS a chronic and highly disabling condition. Identifying drivers of pain and mobility disability in hEDS is necessary to provide adapted prevention and intervention programs.Methods: A cross-sectional sample of 75 adults with hEDS according to Villefranche criteria (67 women; 36 years, SD = 11.2) responded to self-assessment questionnaires regarding psychological (negative thoughts and emotions), health (pain, fatigue, diagnostic delay, body mass index, and mobility disability) and socio-demographic variables.Results: Forty-three percent of the participants suffer from severe pain, and two thirds suffer from a high level of mobility disability. Multivariate analyses show that delay of diagnosis, being professionally active and thoughts of helplessness are variables that increase the likelihood of severe pain, whereas age, fatigue, and body mass index predict mobility disability.Conclusion: These results confirm previous research concerning the high disease burden observed in hEDS patients, and highlight the importance of multidisciplinary management that includes nutritional and psychological advice to address this pathology. Increasing awareness of the clinical aspects of hEDS among physicians is necessary to reduce diagnostic delay and minimize negatives outcomes.Implications for RehabilitationA high proportion of patients with hypermobile Ehlers-Danlos syndrome suffer from severe pain (>42%) and a high level of mobility disability (>65%).Different psychosocial and health variables predict pain and mobility disability in patients with hypermobile Ehlers-Danlos syndrome.Awareness concerning the clinical picture of hypermobile Ehlers-Danlos syndrome among health care professionals is necessary to reduce diagnosis delay and the burden of the disease.

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