Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a high symptom burden and poor survival that influences patients' health-related quality of life (HRQOL). We aimed to evaluate IPF patients' symptoms and HRQOL in a well-documented clinical cohort during their last two years of life.

Idiopathic Pulmonary Fibrosis

(IPF) is a chronic disease with high morbidity and reduced survival. It occurs mainly in older adults, but the etiology of this progressive disease is still unknown. 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.

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

IPF patients suffer from many difficult symptoms, of which breathlessness and cough are the most common ones. Also, a substantial proportion of patients report anxiety, depression, and pain. Dyspnea is a major contributor to HRQOL, and decreased HRQOL is associated with higher mortality In a prospective Australian longitudinal registry study, impaired HRQOL was related to frequent respiratory hospitalizations and higher mortality. However, to our knowledge, no previous studies have reported changes in HRQOL and symptom burden in connection with inevitable death.

In April 2015, we sent the Modified Medical Research Council Dyspnea Scale (MMRC), the modified Edmonton Symptom Assessment Scale (ESAS) and a self-rating HRQOL questionnaire (RAND-36) to 300 IPF patients, of which 247 (82%) responded. After that, follow-up questionnaires were sent every six months for two years. Ninety-two patients died by August 2017. Among these patients, HRQOL was found to be considerably low already two years before death.

The most noticeable declines in HRQOL occurred in physical function, vitality, emotional role and social functioning (p < 0.001). The proportion of patients with MMRC scores ≥three increased near death. Breathlessness and fatigue were the most severe symptoms. Symptom severity for the following symptoms increased significantly and reached the highest mean scores during the last six months of life (numeric rating scale/standard deviation): breathlessness (7.1/2.8), tiredness (7.0/2.3), dry mouth (6.0/3.0), cough (5.8/2.9), and pain with movement (5.0/3.5).

To our knowledge, this is the first study demonstrating, that IPF patients experience remarkably low HRQOL already two years before death, especially regarding the physical role. Besides, they suffer from severe breathlessness and fatigue. Furthermore, physical, social and emotional wellbeing deteriorate, and symptom burden increases near death. Regular symptom and HRQOL measurements are essential to assess palliative care needs in patients with IPF.