Physical exercise for people with hereditable thoracic aortic disease. A study of patient perspectives

Abstract

Purpose

To improve the knowledge about physical exercise in patients with Hereditable Thoracic Aortic Disease, insight to the patient perspectives is necessary. The aim of this study was to explore aspects related to physical exercise as highlighted by the patients themselves.

Methods

Focus group interviews with 36 people with Marfan syndrome, Loeys-Dietz syndrome and vascular Ehlers Danlos syndrome were conducted. Inductive systematic condensation analysis was performed.

Results

Four themes related to physical exercise were elucidated by the participants: (1) Being diagnosed. (2) Considerations of physical exercise. (3) Body image and function. (4) Future perspectives. The four themes are mutually interrelated in terms of barriers, facilitators and strategies for dealing with physical exercise. Our findings indicate that the participants experience exercise as a consistent dilemma between what is healthy and what is risky. Inconsistent professional advice, non-engaging activities, unpredictable health conditions and a fear of exercising were factors that may contribute to inactivity and a sedentary lifestyle.

Conclusions

The complexity and existential internal conflict related to physical exercise seemed to be a huge dilemma among persons with Hereditable Thoracic Aortic Disease. The balance between safe and healthy activities should be a research priority in these groups.

Implications for rehabilitation

• Physical activity and exercise pose a difficult dilemma for patients with Hereditable Thoracic Aortic Disease, in terms of what is healthy and what is dangerous.

• People with Hereditable Thoracic Aortic Disease need help to minimize concern, stress and anxiety associated with exercise.

• Individualized adapted programs including physical, psychological and social rehabilitation goals are most likely to be successful in encouraging exercise in these patient groups.

Keywords:

• Genetic aorta disease
• physical exercise
• comply
• patient experiences
• focus groups

Acknowledgement

We thank the participants, the Norwegian Marfan foundation and the Ehlers-Danlos syndrome foundation who have made this study possible. Thanks to colleagues at TRS National Resource Centre for Rare Disorders, and especially to Nina Riise (MD) and Trine Bathen (OT) for important professional contributions.

Disclosure statement

No potential conflict of interest was reported by the authors.