The Role of Palliative Care in Reducing Symptoms and Improving Quality of Life for Patients with Idiopathic Pulmonary Fibrosis: A Review

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a median survival of 3-4 years from time of initial diagnosis, similar to the time course of many malignancies. A hallmark of IPF is its unpredictable disease course, ranging from long periods of clinical stability to acute exacerbations with rapid decompensation. As the disease progresses, patients with chronic cough and progressive exertional dyspnea become oxygen dependent. They may experience significant distress due to concurrent depression, anxiety, and fatigue, which often lead to increased symptom burden and decreased quality of life. Despite these complications, palliative care is an underutilized, and often underappreciated, resource before end-of-life care in this population. While there is growing recognition about early palliative care in IPF, current data suggest referral patterns vary widely based on institutional practices. In addition to focusing on symptom management, there is emphasis on supplemental oxygen use, pulmonary rehabilitation, quality of life, and end-of-life care. Importantly, increased use of support groups and national foundation forums have served as venues for further disease education, communication, and advanced care planning outside of the hospital settings. The purpose of this review article is to discuss the clinical features of IPF, the role of palliative care in chronic disease management, current data supporting benefits of palliative care in IPF, its role in symptom management, and practices to help patients and their caregivers achieve their best quality of life.

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“…Current clinical management of IPF patients includes anti-fibrotic drugs and nonpharmacological support (Richeldi et al, 2017). For patients with advanced disease, reducing symptoms and improving quality of life are required (Zou et al, 2020). Long-term oxygen therapy, with high flow and high concentration of oxygen, is often used to decrease dyspnea and improve exercise tolerance (Koyauchi et al, 2018;Faverio et al, 2019).…”

Care programs and their components for patients with idiopathic pulmonary fibrosis: a systematic review

“…Recent reviews highlighted the importance of pharmacological management of IPF, palliative care, the potential use of PROs and the multidimensional supportive needs of patients and caregivers [ 11 , 51 , 53 – 55 ]. Our review adds to this by evaluating care models and components thereof from the perspective of chronic integrated care.…”

A near-infrared fluorescent probe for evaluating glutamyl transpeptidase fluctuation in idiopathic pulmonary fibrosis cell and mice models

“…Idiopathic pulmonary fibrosis (IPF), acknowledged as a chronic, non-reversible, progressive, and ultimately lethal disease, is a most common type of the idiopathic interstitial pneumonia [1]. The median survival time of IPF is between 3-4 years from diagnosis and it largely occurs in older male adults.…”

“…The median survival time of IPF is between 3-4 years from diagnosis and it largely occurs in older male adults. The incidence increases with age, from 1.1 new cases per 100,000 person-years in adults ages 18-34 to 19.3 new cases per 100,000 person-years in adults ages 55-64 [1]. Early diagnosis of IPF is vital to enable patients to get treatments that slow disease progression.…”