Patient-Centric Approach to Improve the Quality of Life and Healing Efficacy for Patients With Fabry Disease: A Grounded Theory-Based Analysis

以患者为中心的改善法布里病患者生活质量和疗效的方法:基于扎根理论的分析

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Abstract

BACKGROUND: Fabry disease (FD) poses multifaceted challenges for patients in Republic of Korea (South Korea), but an in-depth qualitative understanding of their experiences and support needs is still lacking. This study aimed to qualitatively explore the experiences and coping strategies of Korean patients with FD who reported dissatisfaction with their well-being and faced social, occupational, or personal difficulties, to inform patient-centered care and healthcare policy. METHODS: We conducted a qualitative study using a grounded theory approach to explore the experiences of individuals with FD in Korea. Eligible participants were aged 19-70 years, had been diagnosed for more than three years or treated for at least one year, and reported dissatisfaction with their well-being alongside social or personal disadvantages. Each participant completed three in-depth interviews. Data were collected iteratively and analyzed through open and axial coding until theoretical saturation was reached. RESULTS: Theoretical saturation was achieved after conducting three in-depth interviews with each of the 10 participants (5 males, 5 females; age range: 34-66 years). A total of 273 initial concepts were generated and refined into 21 categories across six conceptual domains: causal conditions, contextual conditions, central phenomenon, intervening conditions, actions/interactions, and consequences. Patients reported persistent physical and emotional burdens, financial strain, limited systemic support, and stigma. However, they developed adaptive coping strategies, including lifestyle modifications, medical engagement, and meaning-making. The coping process followed five progressive stages: diagnostic odyssey, treatment entry, life reconstruction, symptom management, and hope, highlighting dynamic psychological adaptation. Three distinct coping styles emerged: religious will, optimistic acceptance, and altruistic solidarity. CONCLUSION: Fabry patients experience physical pain, emotional and socioeconomic burdens, and limited systemic and social support, yet they develop adaptive coping strategies and resilience. These findings highlight the importance of developing patient-centered, context-sensitive care approaches in Korea.

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