Abstract
INTRODUCTION: Idiopathic Pulmonary Fibrosis (IPF) is a rare disease that causes shortness of breath, dry cough, and tiredness. While there is no cure for IPF, current therapeutic treatments aim to slow lung degeneration while managing side effects. There is little known about patient experience and attitude with regards to their disease and medication. PURPOSE: To understand the perceptions, behaviors and drivers of treatment decision-making among patients, caregivers and pulmonologists in IPF. PATIENTS AND METHODS: Online surveys to patients with IPF, caregivers and pulmonologists were developed and administered in Belgium, Finland, France, Greece (pulmonologists only), the Netherlands, Ireland and the United Kingdom between November 2021 and January 2022. RESULTS: A total of 111 patients, 22 caregivers and 140 pulmonologists participated. Half (47%) of patients rated their disease as "severe", while pulmonologists reported that a quarter of their patients had a low Forced Vital Capacity (FVC) (below 50% of the predicted value). Between 21% and 42% of the patients do not take an IPF medication (patients' perception) or antifibrotic (physicians' perception). Pulmonologists reported that a total of 58% of their patients were receiving antifibrotic medication, any IPF medication, while around 53%, 55%, 35% and 73% of the patients limited their exposure (sometimes or often) to the sun due to IPF, considered taking medication against diarrhea, nausea/vomiting and heartburn, respectively. Treatment adherence was relatively high (81%), in line with the caregivers' view and the pulmonologists' expectations. Overall, cultural, clinical or socio-demographic factors impacted patients' perceptions or behaviors. CONCLUSION: This study shows there is a significant proportion of IPF patients who remain untreated, a misalignment of disease severity between patients and their physicians and patient background impacts behavior. Overall, more in-depth patient-physician communication is needed to improve treatment experience.