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


In this study, we demonstrate a rapidly increasing impairment in HRQOL and escalating symptom burden in IPF patients approaching death. Low HRQOL together with severe breathlessness and fatigue were detected as early as two years before death. In addition, several dimensions of HRQOL declined further and the severity of many symptoms other than dyspnea increased during the last two years of life.

In the present study, HRQOL was considerably impaired two years prior to death in IPF patients. Physical role, i.e., role limitations due to physical health, was exceptionally low, but physical functioning, vitality and general health appeared to be below the general population level as well. Similar to our findings, an Australian registry study of 516 IPF patients reported HRQOL impairments in all domains, with the lowest score in activity, i.e., activities that cause or are limited by breathlessness.[27] The importance of decreased HRQOL was further highlighted in a recent study by Furukawa et al. that demonstrated that low HRQOL was actually an independent prognostic factor.[28]

Although previous studies have demonstrated low HRQOL in IPF patients,[10–12] none of them focused on the HRQOL from the aspect of approaching death in a follow-up setting. The uniqueness of this study stems from the continuing follow-up until death, and the subsequent finding of a salient decline in HRQOL during the last two years of life that was intensified near death. Deterioration was identified in all domains except physical role, which was already remarkably low two years before death. The most integral impairment was in physical, social and emotional functioning and vitality. In the Australian registry study, approximately one-third (38%) of the IPF patients experienced clinically important differences in the decline of HRQOL during 12 months.[27] However, in that study, no HRQOL data was available from the period preceding death.[27]

In lung cancer, functional concerns relating to physical movement or functioning predominate patients' symptom burden throughout the disease course and have a negative impact on HRQOL.[42] In addition, the severity of symptoms escalates, and the number of severe symptoms increases during the last three months of life.[42–44] A steep decline in HRQOL at the end-of-life is typical in patients dying of cancer compared with other terminally ill patients.[45,46] In COPD patients, HRQOL gradually declines over time without a steeper decline at the end-of-life.[47] Our data imply that patients with IPF experience gradual impairments in HRQOL comparable to COPD patients but suffer from a pronounced, rapid deterioration in HRQOL during the last year of life, more closely resembling the disease trajectory of cancer.

Our results corroborate earlier findings on dyspnea and cough as the most severe symptoms in IPF patients regardless of the disease phase.[10,21,27,48] The intensity of dyspnea increased during the follow-up, being one of the most severe symptoms before death. In a previous IPF registry study, dyspnea yielded the strongest association with impaired HRQOL accounting for 71% of the variation in HRQOL.[27] In addition, exertion dyspnoea measured by the MMRC has been shown to correlate to HRQOL and symptom burden.[21,49]

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. The intensity of depression and anxiety were, however, relatively mild two years before death, although these symptoms increased thereafter. In a recent study, depression was an independent predictor for HRQOL impairment, although it only accounted for 3.5% of the variation, whereas dyspnea accounted for 71%.[27] Our results suggest that the relief of activity-limiting symptoms together with psychosocial support may improve HRQOL in advanced IPF.

As discussed above, patients with advanced IPF, COPD and lung cancer suffer from a heavy symptom burden and deteriorating HRQOL. This calls for comprehensive symptom management and integrated palliative care concomitant with disease-modifying therapies.[21,27,47,50–52] Early integrated palliative care for patients with lung cancer has shown substantial benefits, such as lower depression scores, higher HRQOL, better communication of end-of-life care preferences, less aggressive care at the end-of-life, and longer overall survival.[51,53] Similarly, a randomised trial demonstrated better control of dyspnea and a survival benefit with integrated palliative care in patients with COPD and interstitial lung disease.[54] In addition to cancer patients, early integrated palliative care may reduce end-of-life acute care utilisation, and allow patients with IPF to die in their preferred locations.[55–58] Integrated palliative care in IPF patients seems to lower respiratory-related emergency room visits and hospitalisations and may allow more patients to die at home.[55] In this study, 67% of patients died in hospital and 11% in intensive care, which is in line with earlier findings, implying the necessity of improvements in advanced care planning and palliative care of patients with IPF.[55,59] Our results provide insight into the most important needs of end-stage IPF patients and support the use of early-integrated palliative care, which should include symptom control beyond treatment for dyspnea and psychosocial support.

The relatively small study population limits our study, as did not having systematic follow-up data on lung function, which is at least partially due to the poor conditions of many of our patients. Our study is to our knowledge first to present follow-up data of HRQOL and symptoms for over two years before death. The strength is its real-life longitudinal design, with a unique cohort of IPF patients approaching death with an outstanding response rate, particularly considering the fact that some of the patients were probably too weak to respond during their final days or weeks of life. To our knowledge, this is the first study describing comprehensive patient-reported data on the HRQOL and symptom burden of IPF patients from the perspective of approaching death.