Understanding the burden of pulmonary arterial hypertension among patients and caregivers through narrative medicine: data from Italian multicentre observational INSPECTIO study.

Abstract

Introduction: A key challenge in pulmonary arterial hypertension (PAH) research is assessing the specific impact of this condition on health-related quality of life (HRQoL), also considering the patient's subjective experience and caregiving burden. The prospective, observational, multicentre Non-INterventional Study on Pulmonary arterial hypertension patients treated with macitentan and/or selexipag: ExperienCe from an ITalIan cOhort (INSPECTIO) study integrated narratives with patient-reported outcomes (PROs) and clinical data to gain deeper insight into patients' experiences of PAH, also involving caregivers' perspectives.

Methods: The study was conducted across 29 Italian hospital-based centres and enrolled adult patients with PAH already on treatment with macitentan and/or selexipag as part of combination therapy. A dedicated narrative collection was carried out within the larger study. Patient and caregiver narratives were collected at enrolment (visit 1; V1) and at 12 months (visit 3; V3), then analysed through MAXQDA software and correlated with emPHasis-10 questionnaire scores and clinical data.

Results: Out of the 186 patients enrolled in the overall INSPECTIO study, 96/186 (52%) completed the narrative at V1 and 58/186 (31%) at V3; 29/54 (54%) caregivers completed the narrative at V1 and 16/54 (30%) at V3. The analysis revealed an alignment between patient narratives and emPHasis-10 scores. Specifically, at V3, emPHasis-10 scores were higher for patients who did not mention their domestic life in narratives, reported feelings of shame or isolation, limitations or breathlessness also in daily activities and reported ongoing treatment-related issues. Nonetheless, at V3, 36/58 (62%) of patients described living with PAH as manageable. The care pathway showed a positive impact on caregivers: their narratives indicated a shift from V1 to V3, with improved perceptions of the care relationship and PAH therapies.

Conclusion: INSPECTIO is the first study to integrate narratives, PROs and clinical data in PAH research. Findings suggested that appropriate support and therapeutic management are crucial to help patients cope with the condition. Narratives have proven to be a valuable tool for understanding the impact of PAH and improving its management, providing insights that can inform a more patient-centred approach.

Trial registration number: NCT04567602.