Health-related quality of life among patients with idiopathic pulmonary fibrosis.

The hallmark of idiopathic pulmonary fibrosis (IPF) is relentless and progressive breathlessness culminating in respiratory failure and death. Clinicians and investigators are increasingly aware that many patients with chronic diseases, like IPF, value the quality of their lives as much or more than their length of survival. Despite this growing awareness, little research has focused on quality of life (QOL) in IPF patients. Nevertheless, the few studies that have been performed uniformly show the negative impacts of IPF on QOL, particularly in the areas of physical health, energy, and symptoms. To fill important knowledge gaps, more research is needed. For example, future studies in well-defined IPF patient populations should rigorously assess the psychometric properties of different measures of QOL. Currently, there is no disease-specific instrument for use in patients with IPF. A carefully developed, IPF-specific instrument that includes items most relevant to IPF patients should be more sensitive to change than existing generic or non-IPF respiratory disease-specific instruments. Longitudinal assessments are needed to map the trajectory of QOL in relation to disease progression and to reveal whether different aspects of QOL become impaired over time. Addressing these research opportunities will markedly improve our knowledge of this outcome, which is highly valued by patients. These endeavors will also help clinicians who care for patients with IPF to develop a better understanding of its profound negative impact, and it will help future IPF clinical investigators to select the most valid, reliable, and appropriate QOL instrument to fill the roles their studies require.