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.



      CRQ (chronic respiratory questionnaire), Dlco (diffusing capacity of the lung for carbon monoxide), HRQL (health-related quality of life), ILD (interstitial lung disease), IPF (idiopathic pulmonary fibrosis), NSIP (nonspecific interstitial pneumonia), QOL (quality of life), QWB (quality of well-being), SF-36 (Medical Outcomes Study 36-item short form), SGRQ (St. George Respiratory Questionnaire), TLC (total lung capacity), WHO (World Health Organization), WHOQOL-100 (World Health Organization quality of life assessment 100-item instrument)
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        • Coultas D
        • Zumwalt R
        • Black W
        • et al.
        The epidemiology of interstitial lung disease.
        Am J Respir Crit Care Med. 1994; 150: 967-972
      1. 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; 161 (American Thoracic Society and the European Respiratory Society): 646-664
        • Panos RJ
        • Mortenson RL
        • Niccoli SA
        • et al.
        Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment.
        Am J Med. 1990; 88: 396-404
        • Flaherty KR
        • Toews GB
        • Lynch III, JP
        • et al.
        Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival.
        Am J Med. 2001; 110: 278-282
        • Curtis J
        • Martin D
        • Martin T
        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; 156: 1032-1039
        • Flanagan J
        A research approach to improving our quality of life.
        Am Psychol. 1978; 33: 138-147
        • Oleson M
        Subjectively perceived quality of life.
        Image (IN). 1990; 22: 187-190
      2. Quality of life resource.
        (Accessed March 1 2004 American Thoracic Society Available at)
        • Organization WH
        Constitution of the World Health Organization: Chronicle of the World Health Organization 1. 1947; (World Health Organization. Geneva, Switzerland)
        • Testa M
        • Simonson D
        Current concepts: assessment of quality-of-life outcomes.
        N Engl J Med. 1996; 334: 835-840
        • Landy F
        Stamp collecting versus science.
        Am Psychol. 1986; 41: 1183-1192
        • Streiner D
        • Norman G
        Health measurement scales: a practical guide to their development and use. 1995; (Oxford University Press New York)
        • Curtis J
        • Deyo R
        • Hudson L
        Health-related quality of life among patients with chronic obstructive pulmonary disease.
        Thorax. 1994; 49: 162-170
        • De Vries J
        • Seebregts A
        • Drent M
        Assessing health status and quality of life in idiopathic pulmonary fibrosis: which measure should be used?.
        Respir Med. 2000; 94: 273-278
        • Jones P
        • Quirk F
        • Baveystock C
        The St. George's Respiratory Questionnaire.
        Respir Med. 1991; 85: 25-31
        • De Vries J
        • Kessels B
        • Drent M
        Quality of life of idiopathic pulmonary fibrosis patients.
        Eur Respir J. 2001; 17: 954-961
        • Group TW
        The world health organization quality of life assessment (WHOQOL): development and general psychometric properties.
        Soc Sci Med. 1998; 46: 1569-1585
        • Martinez T
        • Pereira C
        • dos Santos M
        • 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; 117: 1627-1632
        • Ware Jr, JE
        • Sherbourne C
        The MOS 36-item short-form health survey (SF-36): I. Conceptual framework and item selection.
        Med Care. 1992; 30: 473-483
        • Ware Jr, JE
        SF-36 health survey update.
        Spine. 2000; 25: 3130-3139
        • Mc Horney C
        • Ware Jr, JE
        • Lu J
        • et al.
        The MOS 36-item Short Form Health Survey (SF-36): III. Tests of data quality, scaling assumptions, and reliability across diverse patient groups.
        Med Care. 1994; 32: 40-66
        • Bousquet J
        • Knani J
        • Dhivert H
        • et al.
        Quality of life in asthma: I. Internal consistency and validity of the SF-36 questionnaire.
        Am J Respir Crit Care Med. 1994; 149: 371-375
        • Mc Horney C
        • Ware Jr, JE
        • Raczek A
        The MOS 36-item short-form health survey (SF-36): II. Psychometric and clinical tests of validity in measuring physical and mental health constructs.
        Med Care. 1993; 31: 247-263
        • Martinez J
        • Martinez T
        • Galhardo F
        • et al.
        Dyspnea scales as a measure of health-related quality of life in patients with idiopathic pulmonary fibrosis.
        Med Sci Monit. 2002; 8: CR405-CR410
        • Chang J
        • Curtis R
        • Patrick D
        • et al.
        Assessment of health-related quality of life in patients with interstitial lung disease.
        Chest. 1999; 116: 1175-1182
        • Spencer S
        • Calverley P
        • Burge P
        • et al.
        Health status deterioration in patients with chronic obstructive pulmonary disease.
        Am J Respir Crit Care Med. 2001; 163: 122-128
        • Burge P
        • Calverley P
        • Jones P
        • et al.
        Randomized, double-blind, placebo controlled study of fluticasone proprionate in patients with moderate to severe chronic obstructive pulmonary disease: the ISOLDE trial.
        BMJ. 2000; 320: 1297-1303
        • Guyatt G
        • Berman L
        • Townsend M
        • et al.
        A measure of quality of life for clinical trials of chronic lung disease.
        Thorax. 1987; 42: 773-778
        • Kaplan R
        • Bush J
        • Berry C
        Health status: types of validity and the index of well-being.
        Health Serv Res. 1976; 11: 478-507
        • Kaplan R
        • Bush J
        Health-related quality of life measurement for evaluation research and policy analysis.
        Health Psychol. 1982; 1: 61-81
        • Clark M
        • Cooper B
        • Singh S
        • et al.
        A survey of nocturnal hypoxaemia and health related quality of life in patients with cryptogenic fibrosing alveolitis.
        Thorax. 2001; 56: 482-486
        • Johnston ID
        • Gomm S
        • Kalra A
        The management of cryptogenic fibrosing alveolitis in three regions of the United Kingdom.
        Eur Respir J. 1993; 6: 891-893
        • Douglas WW
        • Ryu JH
        • Schroeder DR
        Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival.
        Am J Respir Crit Care Med. 2000; 161: 1172-1178
        • Douglas WW
        • Ryu JH
        • Bjoraker JA
        • et al.
        Colchicine versus prednisone as treatment of usual interstitial pneumonia.
        Mayo Clin Proc. 1997; 72: 201-209
        • Raghu G
        • Brown KK
        • Bradford WZ
        • et al.
        A placebo-controlled trial of interferonγ-1b in patients with idiopathic pulmonary fibrosis.
        N Engl J Med. 2004; 350: 125-133
        • Mc Horney C
        • Tarlov A
        Individual-patient monitoring in clinical practice: are available health status surveys adequate?.
        Qual Life Res. 1995; 4: 293-307
        • Howard G
        • Dailey P
        Response shift bias: a source of contamination of self-report measures.
        J Appl Psychol. 1979; 64: 144-150
        • Golembiewski R
        • Billingsley K
        • Yeager S
        Measuring change and persistence in human affairs: types of change generated by old designs.
        J Appl Behav Sci. 1976; 12: 133-157
      3. Measurement excellence initiative (MEI).
        (Accessed March 1 2004 Veterans Administration Health Services Research and Development Service Available at)
        • Guyatt G
        • Feeny D
        • Patrick D
        Measuring health-related quality of life.
        Ann Intern Med. 1993; 118: 622-629