Self-care behaviors and their individual-level determinants in Italian adults with Marfan syndrome: A single-center cross-sectional study

Aim: This study aimed to describe the levels of self-care behaviors and self-care self-efficacy in patients with Marfan syndrome and to identify the individual-level determinants of self-care behaviors.

Background: The behaviors aimed at maintaining health stability (self-care maintenance), monitoring signs and symptoms (self-care monitoring), and taking action when signs and symptoms occur (self-care management) are key aspects of the care for addressing the complexity of care of patients with Marfan syndrome. However, the description of self-care behaviors and their determinants in patients with Marfan syndrome are highly under-described.

Methods: The adopted design was descriptive observational with a cross-sectional data collection on 111 patients with MFS in a single Italian specialized and reference center for this disease between 2020 and 2021.

Results: Performing healthy activities and managing illness, therapies, and follow-ups to maintain health over time (self-care maintenance) was almost adequate (mean score = 67.87 ± 13.17), as well as the ability to recognize signs and symptoms promptly (self-care monitoring, mean score = 67.95 ± 26.70). The ability to respond to symptoms when they occur (self-care management, mean score = 54.17 ± 19.94) was sub-optimal. The stronger positive predictor of each self-care behavior was self-care self-efficacy.

Conclusions: This study suggested prioritizing educational activities focused on enhancing self-care management in patients with Marfan syndrome and strengthening their self-care self-efficacy. Researchers should develop and validate evidence-based educational approaches to enhance self-care in patients with Marfan syndrome, and clinical nurses should strengthen their focused educational activities to improve the self-care management of these patients.