Chest
ReviewsHealth-Related Quality of Life Among Patients With Idiopathic Pulmonary Fibrosis
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
References (40)
- et al.
Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment
Am J Med
(1990) - et al.
Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival
Am J Med
(2001) - et al.
Assessing health status and quality of life in idiopathic pulmonary fibrosis: which measure should be used?
Respir Med
(2000) - et al.
The St. George's Respiratory Questionnaire
Respir Med
(1991) - et al.
Evaluation of the short-form 36-item questionnaire to measure health-related quality of life in patients with idiopathic pulmonary fibrosis
Chest
(2000) - et al.
Assessment of health-related quality of life in patients with interstitial lung disease
Chest
(1999) - et al.
Colchicine versus prednisone as treatment of usual interstitial pneumonia
Mayo Clin Proc
(1997) - et al.
The epidemiology of interstitial lung disease
Am J Respir Crit Care Med
(1994) Idiopathic pulmonary fibrosis: diagnosis and treatment; International consensus statement—American Thoracic Society (ATS), and the European Respiratory Society (ERS)
Am J Respir Crit Care Med
(2000)- et al.
Patient-assessed health outcomes in chronic lung disease: what are they, how do they help us, and where do we go from here?
Am J Respir Crit Care Med
(1997)
A research approach to improving our quality of life
Am Psychol
Subjectively perceived quality of life
Image (IN)
Quality of life resource
Constitution of the World Health Organization: Chronicle of the World Health Organization 1
Current concepts: assessment of quality-of-life outcomes
N Engl J Med
Stamp collecting versus science
Am Psychol
Health measurement scales: a practical guide to their development and use
Health-related quality of life among patients with chronic obstructive pulmonary disease
Thorax
Quality of life of idiopathic pulmonary fibrosis patients
Eur Respir J
The world health organization quality of life assessment (WHOQOL): development and general psychometric properties
Soc Sci Med
Cited by (72)
Idiopathic pulmonary fibrosis: Diagnosis, biomarkers and newer treatment protocols
2023, Disease-a-MonthCitation Excerpt :But the number of patients with an increase of more than 20 points at the end of a year (minimal important difference for UCSD-SOBQ= 8 points for IPF) was lesser in the treatment arm (24.0%) than the placebo arm (31.4%).383 The UCSD-SOBQ is not a measure specifically designed to assess health-related QoL, but it assesses SOB, which in itself is a determinant of health-related QoL in IPF patients.384 Thus, if pirfenidone impedes the decrease in SOB, which is apparent herein by a lesser increase in UCSD-SOBQ score, it may be deduced that pirfenidone improves health-related QoL.
Comprehensive Care of Interstitial Lung Disease
2021, Encyclopedia of Respiratory Medicine, Second EditionEvaluation of permanent disability levels in occupational pulmonary diseases
2019, Revue des Maladies RespiratoiresSildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: A Phase IIb, randomised, double-blind, placebo-controlled study – Rationale and study design
2018, Respiratory MedicineCitation Excerpt :Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive and often rapidly fatal fibrosing interstitial lung disease with a 5-year survival rate between 20% and 40% [1,2], which is lower than that reported for many common cancers [2,3]. IPF profoundly affects patients' quality of life (QoL), with dyspnoea reducing physical functioning, leading to limited independence and depression [4,5]. Pirfenidone is an oral antifibrotic agent that slows IPF disease progression and, along with nintedanib, is one of only two antifibrotic therapies that were conditionally recommended for the treatment of IPF in the 2015 update to the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society IPF treatment guidelines [6].
The earlier, the better: Impact of early diagnosis on clinical outcome in idiopathic pulmonary fibrosis
2017, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :The identification and prompt treatment of comorbidities can have a significant impact on overall outcome [41]. IPF alone and in combination with comorbidities significantly impairs QoL and imposes a substantial burden on health care resources [42]. The diagnosis of IPF is often made late in the disease course for a number of reasons — patients may present to their physicians having had ‘non-specific’ symptoms, such as non-productive cough, exertional dyspnoea for months/years previously or they may have been diagnosed with other more common respiratory diseases such as asthma or COPD before IPF is finally diagnosed [43].
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]).