Motivation in Rare Disease Self-Care: A Phenomenological Study of Pediatric Patients With Spinal Muscular Atrophy and Their Caregivers.

Abstract

Background: Spinal muscular atrophy can cause progressive physical disability and difficulties with self-care. Self-care motivation can enhance patient persistence in self-care behavior and maintain health.

Purpose: This study was designed to explore and describe motivations for self-care among school-aged children and adolescents with spinal muscular atrophy and the perspectives of their primary caregivers.

Methods: Husserl's phenomenological research method was adopted, and data were collected using in-depth, face-to-face, open-ended interviews with patients and their primary caregivers aged 8-18 years with spinal muscular atrophy. A total of eight patient-caregiver dyads were recruited via purposeful sampling from a hospital clinic and through the Muscular Dystrophy Association of Taiwan. The interviews were conducted in the participants' homes and transcribed verbatim. Data were then thematically analyzed using the Giorgi analysis method.

Results: Six themes were identified from the patients' perspective: (a) adapting to dietary restrictions, (b) proactive measures for physical safety, c3) pursuing social connection and peer support, (d) navigating emotional well-being, (e) seeking independence, and (f) achieving success motivation. From the caregivers' perspective, the motivations for patient self-care were identified as follows: (a) ensuring safe and enjoyable eating for patients, (b) facilitating patient autonomy in health risk management, (c) facilitating patient engagement with peers, (d) balancing support with encouraging independence, (e) assessing and balancing independence and safety in patient caregiving, and (f) admiring patients' resilience and self-care innovation.

Conclusions/implications for practice: This study highlights the vital roles of caregivers in adapting to the dynamic self-care motivations of children and adolescents and of healthcare professionals in facilitating communication and care strategies. Personalized approaches are essential for enhancing autonomy and well-being in pediatric patients.