Chest
Volume 127, Issue 1, January 2005, Pages 284-294
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Health-Related Quality of Life Among Patients With Idiopathic Pulmonary Fibrosis

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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.

Section snippets

QOL and HRQL

QOL refers to a person's “holistic” evaluation of satisfaction with his own life in areas (domains) that he considers to be important.5678 Accordingly, one aspect of an individual's QOL may seem to others to be significantly impaired, but if that aspect is unimportant to the individual, his perceived impairment could be minimal or nil.

According to the World Health Organization (WHO), health not only affects other life domains, it encompasses many of them. Health includes aspects of well-being

Domains Impacted

Only a handful of studies have measured HRQL (or QOL) in patients with IPF (Table 1). Collectively, the investigators that performed these studies used five different instruments to measure HRQL, including two disease-specific HRQL instruments developed for patients with obstructive lung disease, two generic HRQL instruments, and one generic QOL instrument (Table 2). While the studies vary in their case definitions of IPF, inclusion criteria, and reporting of instrument scores, the common

Gaps in Our Understanding of HRQL and QOL in Patients With IPF

While the handful of studies assessing HRQL and QOL have shed light on some of the difficulties encountered by IPF patients, important questions remain. For example, what is the impact of supplemental oxygen use and drug therapy on HRQL and QOL? Are the existing HRQL instruments really appropriate for assessing patients with IPF? Are there important features of this disease, as it affects aspects of HRQL, that are not well-assessed by the existing instruments? What are the strongest predictors

Research Opportunities

Whether for an existing instrument or for a newly developed IPF-specific HRQL instrument, the need to determine floor and ceiling effect levels, to perform assessments of reliability, to evaluate sensitivity to change in HRQL (also called responsiveness), and to establish predictive and concurrent validity create opportunities for research. To date, the studies designed to validate HRQL (or QOL) instruments in patients with IPF have used only the approach of establishing concurrent validity.

Conclusion

HRQL is impaired in many facets in patients with IPF, but domains concerning physical health and physical well-being are the most profoundly impacted, insofar as HRQL is appropriately measured by the currently available instruments. However, our ability to draw confident conclusions is impaired by the fact that only a handful of studies has examined HRQL in IPF patients. Their small sample sizes, variable case inclusion criteria, and other issues related to study design and instrument

Appendix: Definitions of Some Key Terms

Construct: some intangible thing (eg, HRQL) intended to be measured.

Item: a statement or question devised to assess a particular construct.

Instrument: a collection of related items intended to measure the same construct.

Domain: a small group of closely related items (within the larger instrument) that assess a specific attribute or dimension (eg, physical activity) of the broader construct (eg, HRQL) being measured by the instrument.

Subscale: in the context of an instrument's scores, a subscale

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    Dr. Swigris received support from a National Institutes of Health training grant (T32 HL07948–01A1). Dr. Gould received an Advanced Research Career Development Award from the Veterans Affairs Health Services Research and Development Service. The views expressed in this article are those of the authors and not necessarily the views of the Department of Veterans Affairs.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: [email protected]).

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