Addressing advance care planning for idiopathic pulmonary fibrosis: a call to action

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by relentless parenchymal scarring, deteriorating pulmonary function, and unpredictable exacerbations. The prognosis remains poor, despite antifibrotic and other therapies. Patients with IPF experience a high symptom burden, frequent hospitalizations, and uncertainty regarding disease progression. Advance care planning (ACP), the process of defining patients' preferences for medical care, is inconsistently implemented in IPF care. This often leads to end-of-life care unaligned with the patient's wishes and increased distress amongst patients and their care partners.

Areas covered: In this perspective, we argue for the early and routine integration of ACP into IPF management. We review the multifaceted patient-, provider-, and system-level barriers to ACP and propose actionable strategies to normalize, document, and operationalize patient-centered ACP across the IPF disease trajectory. We highlight the critical need for an interdisciplinary team to best address ACP.

Expert opinion: While IPF research has resulted in huge progress in the understanding of disease pathobiology and the expansion of treatment options, perhaps the most patient-centered portion of care remains under-studied. We must prioritize research to better understand an interdisciplinary system of iterative ACP that gives IPF patients a clear voice until the last moment of life.

Keywords: Idiopathic pulmonary fibrosis (IPF); advance care planning (ACP); intensive care unit (ICU); interstitial lung disease (ILD); medical orders for life sustaining treatment (MOLST).