Summary
Background: 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.
Methods: 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. Thereafter, follow-up questionnaires were sent every six months for two years.
Results: Ninety-two patients died by August 2017. Among these patients, HRQOL was found to be considerably low already two years before death. The most prominent declines in HRQOL occurred in physical function, vitality, emotional role and social functioning (p < 0.001). The proportion of patients with MMRC scores ≥3 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).
Conclusions: To our knowledge this is the first study demonstrating, that IPF patients experience remarkably low HRQOL already two years before death, especially regarding physical role. In addition, 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.

Relevance
The study focuses on the end stage of life of people with idiopathic pulmonary fibrosis (IPF). The participants reported their quality of life as well as their physical activity level every six months during two years. The baseline physical activity level was evaluated as the self-reported frequency of leisure time physical exercise that causes breathlessness and sweating for a minimum 30 min per time, the frequency of commuting walking or cycling, and how physically strenuous is their work, if employed. The RAND questionnaire includes the health concept “physical functioning” with ten questions regarding the ability to take care of personal hygiene and the ability to move and exercise, and the concept “physical role” which describes the role limitations due to physical health. The MMRC measures the degree of disability that breathlessness causes during day-to-day activities or exercise.
The results show that the proportion of the patients who could not perform continuous moderate intensity physical exercise for at least 30 min during the previous six months increased from 34% 18–24 months before death to 62% during the last six months of life. There was a steep deterioration of physical function during the last year of life. The level of physical role was low already two years before death.
The authors conclude: ”In addition to dyspnea, other activity-limiting symptoms such as fatigue and pain in movement were among the most severe symptoms in our study. These activity-limiting symptoms can lead to physical inactivity and functional impairment, triggering a vicious circle with worse HRQOL.”… ”Our results suggest that the relief of activity-limiting symptoms together with psychosocial support may improve HRQOL in advanced IPF.”