Living with Ehlers-Danlos syndrome

University dissertation from Stockholm : Karolinska Institutet, Neurobiology, Care Sciences and Society

Abstract: This thesis focuses on how individuals with the Ehlers-Danlos syndrome (EDS), an inherited connective-tissue disorder, experience and describe their daily lives with focus on physical and psychosocial problems. The syndrome primarily affects the skin, ligaments, joints and blood vessels and the symptoms in EDS vary from mild to more severe forms. Individuals with EDS were recruited via the Swedish National EDS Association. Paper (I). The aim was to explore how individuals with EDS perceive their daily life. Eleven individuals were interviewed. The collection and analysis of-data were guided by the basic principles of the constant comparative method. The identified main theme, "Living a restricted life," seemed to explain the way in which fears, pain, stigmatisation and experiences of non-affirmation in health-care limited the interviewees possibilities of self-actualisation in daily living and social life. Paper (II). The aims were to describe symptoms reported by individuals with EDS, to investigate the impact on functional health-status by means of the Sickness Impact Profile (SIP) and to compare these results with those from a Swedish reference group and from females with rheumatoid arthritis (RA) and fibromyalgia (FS). The mailed questionnaires were completed by 77 individuals. The most frequent symptoms were related to activity (75%), pain (71%) and skin/tissue (52%). Pain was reported by 37 individuals (48%) as their most severe symptom. The functional health in the EDS group was in comparison with a reference group significantly affected, and the females with EDS reported higher SIP score than the females with RA. hi comparison with females with FS, the EDS females rated their functional health as worse on the SIP physical dimension scale and on the SIP subscale home management and as better on the SIP subscale work. Paper (III). The aims were to investigate the amount and severity of podiatric problems as well as their consequences on daily life activities, and to compare these results with data from a randomly chosen comparison group from the general population. A foot-specific questionnaire was answered by 136 individuals with EDS and by 292 individuals in the comparison group. The results showed that daily life activities in the EDS group were strongly restricted by their foot pain and related disability. Paper (IV). The aims were to describe the degree to which individuals with EDS accept and cope with perceived limitations related to the syndrome and to determine the relationships between acceptance of disability, sense of coherence, functional health-status and background data. Seventy-seven individuals with EDS answered two questionnaires (the ADM and the SOC scales). The results showed that the level of acceptance of disability (ADM) and sense of coherence (SOC) in the EDS group is similar to other patient groups, e.g. individuals with ostomy and diabetes. Further, a greater ADM was associated with a higher level of SOC and a better functional health-status. In conclusion, these studies indicate substantial impact of EDS on everyday life. Hypermobile joints, fragile tissues, foot deformities and chronic pain limit the EDS individuals' participation in daily life activities. For health promotion in nursing practice, it is important to have insight and knowledge into the problems that may influence the overall life situation of individuals with EDS.

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