Path analysis of illness uncertainty and psychosocial adaptation of patients with Marfan syndrome.

Aims: Marfan syndrome (MFS) is a genetic disorder that causes sudden or chronic cardiovascular problems, which can be fatal. Since MFS patients require regular close medical observation, it is important to understand the factors and pathways associated with psychosocial adaptation to the disease. This study aimed to identify the relationships among illness uncertainty, uncertainty appraisal, and psychosocial adaptation in MFS patients using path analysis.

Method and results: This descriptive cross-sectional survey study was conducted from October 2020 to March 2021, in compliance with STROBE guidelines. Using data from 179 participants aged older than 18 years, we constructed a hypothetical path model to identify determinants of illness uncertainty, uncertainty appraisal, and psychosocial adaptation. In the path analysis, disease severity, illness uncertainty, anxiety, and social support were significant factors influencing MFS patients' psychosocial adaptation. Disease severity and illness uncertainty exerted direct effects, while anxiety and social support exerted both direct and indirect (through illness uncertainty) effects. Finally, anxiety showed the greatest total effect.

Conclusion: These findings are useful for enhancing MFS patients' psychosocial adaptation. Medical professionals should focus on managing disease severity, decreasing anxiety, and increasing social support.