Psychosocial Adjustment in Genetic Aortic Disorders (Marfan, Loeys-Dietz and Thoracic Aortic Aneurysm and Dissection): A Qualitative Insight

Abstract

Maladaptive psychological factors may inhibit coping ability and adherence to medical recommendations in individuals with a Genetic Aortic (GA) disorder, contributing to potentially fatal risks. The current study aimed to explore how people adjust to living with a GA disorder and cope with different aspects of their illness. Twenty-one individuals affected by a GA Disorder, of different ages (M = 38.8 SD = 11.9), varying stages of disease, treatment, and coping abilities were recruited. Semi-structured interviews were conducted and psychosocial measures administered to characterise the sample (DASS & COPE). A number of main themes were identified following grounded theory analysis; Destabilisation, Trauma, Grief & Loss, Reaching a Resolution, Getting on with it, Support, Unmet Needs and Having Children. Psychological distress and maladaptive coping strategies were identified as most likely to occur during Destabilisation and Trauma stages. Connection with peers was identified as the main unmet need. Fifteen (71%) participants scored within the normal range on all aspects of the DASS. The remaining six participants, scores varied from moderate to very severe across depression, anxiety and stress. The most common coping strategies identified by participants on the COPE were acceptance, positive reinterpretation, planning and active coping. One participant reported substance use as the main coping strategy. This is the first study to explore adjustment in an Australian sample, and to investigate adjustment across a spectrum of GA disorders. These findings will be used to inform the development and timing of resources/interventions aimed at improving psychosocial adjustment of individuals with a GA Disorder.