Marked Deterioration in the Quality of Life of Patients With Idiopathic Pulmonary Fibrosis During the Last Two Years of Life

K. Rajala; J. T. Lehto; E. Sutinen; H. Kautiainen; M. Myllärniemi; T. Saarto


BMC Pulm Med. 2018;18(172) 

In This Article


Idiopathic pulmonary fibrosis (IPF) is a chronic disease with high morbidity and poor survival.[1–4] It occurs mainly in older adults, but the etiology of this progressive disease is still unknown.[1] Although the disease trajectory of IPF is variable, for many patients with IPF, survival is worse than many common malignancies. This necessitates early integration of palliative care to improve patients' quality of life (QOL) and to relieve symptoms in addition to disease-specific pharmacological treatment and lung transplant assessment.[5–9]

Existing studies have shown low health-related quality of life (HRQOL) in IPF patients. However, only few of them were prospective longitudinal studies, and most were relatively small in terms of sample size or were concentrated on pharmacological treatment.[10–12] IPF patients have been shown to suffer from lower HRQOL in real-life studies than in clinical studies.[12,13]

IPF patients suffer from many difficult symptoms, of which breathlessness and cough are the most common ones.[8,10,14–20] In addition, a substantial proportion of patients report anxiety, depression and pain.[10,21–26]

Dyspnea is a major contributor to HRQOL, and decreased HRQOL is associated with higher mortality.[27,28] In a prospective Australian longitudinal registry study, impaired HRQOL was related to frequent respiratory hospitalizations and higher mortality.[27] However, to our knowledge, no previous studies have reported changes in HRQOL and symptom burden in connection with forthcoming death.

This study aimed to investigate IPF patients' HRQOL and symptom burden during the last two years of life in a prospective longitudinal follow-up study to recognise needs for palliative care and end-of-life care planning and to characterise their symptom burden in a unique follow-up setting.