Adjustment and Coping Mechanisms for Individuals with Genetic Aortic Disorders

Emily Connors; Richmond W Jeremy; Alana Fisher; Louise Sharpe; Ilona Juraskova

doi:10.1016/j.hlc.2015.05.003

Adjustment and Coping Mechanisms for Individuals with Genetic Aortic Disorders

Heart Lung Circ. 2015 Dec;24(12):1193-202. doi: 10.1016/j.hlc.2015.05.003. Epub 2015 Jun 6.

Authors

Emily Connors¹, Richmond W Jeremy², Alana Fisher¹, Louise Sharpe¹, Ilona Juraskova³

Affiliations

¹ School of Psychology, The University of Sydney, Sydney, NSW, Australia.
² Sydney Medical School, The University of Sydney, Sydney, NSW, Australia. Electronic address: richmond.jeremy@sydney.edu.au.
³ School of Psychology, The University of Sydney, Sydney, NSW, Australia; Centre for Medical Psychology and Evidence-based Decision making (CeMPED), The University of Sydney, Sydney, NSW, Australia.

PMID: 26105988
DOI: 10.1016/j.hlc.2015.05.003

Abstract

Background: Advances in diagnosis and management of Genetic Aortic (GA) Disorders have improved prognosis for affected individuals, yet many do not adhere to key management recommendations, and some may experience clinically significant levels of psychological distress. These issues are often not communicated to treating clinicians. Poor adjustment and coping may adversely impact on prognosis, but little is known about the processes contributing to negative outcomes. This study investigated adjustment to GA disorders to determine which processes facilitated or hindered good adherence and psychological outcomes.

Methods: Semi-structured interviews involving 21 individuals (12M, 9 F; age 19-62 years) with a GA Disorder and psychosocial measures of depression/stress/anxiety (DASS), coping (COPE) and involvement in treatment (CPS) were used. Qualitative data were analysed using grounded theory and a model of adjustment was developed.

Results: Although most participants adhered to physician management recommendations and experienced minimal emotional distress, a subset reported poor adherence and/or sub/clinical levels of depression/anxiety/stress (29%). Dysfunctional coping mechanisms were infrequent, however 22% participants reported 'little or no' acceptance and 43% avoided life planning in response to a diagnosis of GA disorder. Interviews revealed an overarching theme: Negotiating perception of self and GA disorder, supported by five sub-themes: Restrictions upon Lifestyle, Destabilisation, Future, Support, and Unmet Needs. Accepting restrictions and having support were conducive to better adherence, whilst destabilisation and loss of control had a negative impact. A model of adjustment is proposed to explain how patients reached one of four outcomes relating to psychological distress and adherence to physician recommendations. The central tenet of the model is founded on how realistically patients appraise their vulnerability to GA threat and whether they are able to integrate their perceptions of illness with their sense of self-identity.

Conclusions: This study indicates that individuals with GA are at risk of experiencing psychosocial distress and coping difficulties, even years after diagnosis. Key factors likely to be associated with impaired coping among GA patients include inability to integrate the illness into one's identity/life, or to follow physician recommendations. Potential unmet needs were identified, including the provision of more relevant information and opportunities for peer support. These findings may also be applicable to other inherited cardiac disorders.

Keywords: Aorta; Genetics; Marfan syndrome; Patient adherence; Psychological adjustment..

MeSH terms

Adaptation, Psychological*
Adult
Anxiety* / psychology
Anxiety* / therapy
Aortic Diseases* / psychology
Aortic Diseases* / therapy
Depression* / psychology
Depression* / therapy
Female
Genetic Diseases, Inborn* / psychology
Genetic Diseases, Inborn* / therapy
Humans
Male
Middle Aged
Stress, Psychological* / psychology
Stress, Psychological* / therapy