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BMC Pulmonary Medicine

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Open Access 01-12-2018 | Research article

Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

Authors: K. Rajala, J. T. Lehto, E. Sutinen, H. Kautiainen, M. Myllärniemi, T. Saarto

Published in: BMC Pulmonary Medicine | Issue 1/2018

Abstract

Background

Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a high symptom burden and poor survival that influences patients’ health-related quality of life (HRQOL). We aimed to evaluate IPF patients’ symptoms and HRQOL in a well-documented clinical cohort during their last two years of life.

Methods

In April 2015, we sent the Modified Medical Research Council Dyspnea Scale (MMRC), the modified Edmonton Symptom Assessment Scale (ESAS) and a self-rating HRQOL questionnaire (RAND-36) to 300 IPF patients, of which 247 (82%) responded. Thereafter, follow-up questionnaires were sent every six months for two years.

Results

Ninety-two patients died by August 2017. Among these patients, HRQOL was found to be considerably low already two years before death. The most prominent declines in HRQOL occurred in physical function, vitality, emotional role and social functioning (p < 0.001). The proportion of patients with MMRC scores ≥3 increased near death. Breathlessness and fatigue were the most severe symptoms. Symptom severity for the following symptoms increased significantly and reached the highest mean scores during the last six months of life (numeric rating scale/standard deviation): breathlessness (7.1/2.8), tiredness (7.0/2.3), dry mouth (6.0/3.0), cough (5.8/2.9), and pain with movement (5.0/3.5).

Conclusions

To our knowledge this is the first study demonstrating, that IPF patients experience remarkably low HRQOL already two years before death, especially regarding physical role. In addition, they suffer from severe breathlessness and fatigue. Furthermore, physical, social and emotional wellbeing deteriorate, and symptom burden increases near death. Regular symptom and HRQOL measurements are essential to assess palliative care needs in patients with IPF.

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Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar;183(6):788–824.CrossRef

Kaunisto J, Kelloniemi K, Sutinen E, Hodgson U, Piilonen A, Kaarteenaho R, et al. Re-evaluation of diagnostic parameters is crucial for obtaining accurate data on idiopathic pulmonary fibrosis. BMC Pulm Med [Internet]. 2015;15:92 Available from: https://doi.org/10.1186/s12890-015-0074-3.CrossRef

Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of Nintedanib in idiopathic pulmonary fibrosis. N Engl J Med [Internet]. 2014;370(22):2071–82 Available from: https://www.nejm.org/doi/abs/10.1056/NEJMoa1402584.CrossRef

King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of Pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med [Internet]. 2014;370(22):2083–92 Available from: http://www.nejm.org/doi/10.1056/NEJMoa1402582.CrossRef

Behr J, Richeldi L. Recommendations on treatment for IPF. Respir Res [Internet]. 2013;14(Suppl. 1):S6 Available from: http://respiratory-research.com/content/14/S1/S6.

Raghu G, Rochwerg B, Zhang Y, Garcia CAC, Azuma A, Behr J, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis: an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19.CrossRef

Kalluri M, Richman-Eisenstat J. Early and integrated palliative care to achieve a home death in idiopathic pulmonary fibrosis. J Pain Symptom Manage [Internet]. 2017;53(6):1111–5 Available from: https://doi.org/10.1016/j.jpainsymman.2016.12.344.CrossRef

Raghu G, Richeldi L. Current approaches to the management of idiopathic pulmonary fibrosis. Respir Med [Internet]. 2017;129:24–30 Available from: https://doi.org/10.1016/j.rmed.2017.05.017.CrossRef

Caminati A, Cassandro R, Torre O, Harari S. Severe idiopathic pulmonary fibrosis: What can be done? Eur Respir Rev [Internet]. 2017;26(145) Available from: https://doi.org/10.1183/16000617.0047-2017.CrossRef

10.

Ahmadi Z, Wysham NG, Lundström S, Janson C, Currow DC, Ekström M. End-of-life care in oxygen-dependent ILD compared with lung cancer: a national population-based study. Thorax [Internet]. 2016;71(6):510–6 Available from: http://thorax.bmj.com/lookup/doi/10.1136/thoraxjnl-2015-207439.CrossRef

11.

Tomioka H, Imanaka K, Hashimoto K, Iwasaki H. Health-related quality of life in patients with idiopathic pulmonary fibrosis -cross-sectional and longitudinal study. Intern Med [Internet] 2007;46(18):1533–1542. Available from: http://joi.jlc.jst.go.jp/JST.JSTAGE/internalmedicine/46.6218?from=CrossRef%5Cn. http://www.ncbi.nlm.nih.gov/pubmed/17878639.CrossRef

12.

Richeldi L, Cottin V, du Bois RM, Selman M, Kimura T, Bailes Z, et al. Nintedanib in patients with idiopathic pulmonary fibrosis: combined evidence from the TOMORROW and INPULSIS?? trials. Respir Med. 2015;113:74–9.CrossRef

13.

Kreuter M, Swigris J, Pittrow D, Geier S, Klotsche J, Prasse A, et al. Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry. Respir Res [Internet]. 2017;18(1):139 Available from: http://respiratory-research.biomedcentral.com/articles/10.1186/s12931-017-0621-y.CrossRef

14.

Hubbard RB, Smith C, Le Jeune I, Gribbin J, Fogarty AW. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med. 2008;178(12):1257–61.CrossRef

15.

Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, et al. Impact of comorbidities on mortality in patients with idiopathic pulmonary fibrosis. PLoS One. 2016;11(3):1–18.CrossRef

16.

Hyldgaard C, Hilberg O, Bendstrup E. How does comorbidity influence survival in idiopathic pulmonary fibrosis? Respir Med [Internet]. 2014;108(4):647–53 Available from: https://doi.org/10.1016/j.rmed.2014.01.008.CrossRef

17.

Nathan SD, Basavaraj A, Reichner C, Shlobin OA, Ahmad S, Kiernan J, et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med [Internet]. 2010;104(7):1035–41 Available from: https://doi.org/10.1016/j.rmed.2010.02.008.CrossRef

18.

King CS, Nathan SD. Idiopathic pulmonary fibrosis: effects and optimal management of comorbidities. Lancet Respir Med. 2017;5(1):72–84.CrossRef

19.

Buendía-Roldán I, Mejía M, Navarro C, Selman M. Idiopathic pulmonary fibrosis: clinical behavior and aging associated comorbidities. Respir Med. 2017;129:46–52.CrossRef

20.

Bajwah S, Higginson IJ, Ross JR, Wells AU, Birring SS, Riley J, et al. The palliative care needs for fibrotic interstitial lung disease: a qualitative study of patients, informal caregivers and health professionals. Palliat Med [Internet]. 2013;27(9):869–76 Available from: http://www.ncbi.nlm.nih.gov/pubmed/23885010.CrossRef

21.

Yount SE, Beaumont JL, Chen S-Y, Kaiser K, Wortman K, Van Brunt DL, et al. Health-related quality of life in patients with idiopathic pulmonary fibrosis. Lung [Internet]. 2016;194(2):227–34 Available from: http://link.springer.com/10.1007/s00408-016-9850-y.CrossRef

22.

Ryerson CJ, Berkeley J, Carrieri-Kohlman VL, Pantilat SZ, Landefeld CS, Collard HR. Depression and functional status are strongly associated with dyspnea in interstitial lung disease. Chest. 2011;139(3):609–16.CrossRef

23.

Akhtar AA, Ali MA. Smith RP. Chron Respir Dis: Depression in patients with idiopathic pulmonary fibrosis; 2013.

24.

Matsuda T, Taniguchi H, Ando M, Kondoh Y, Kimura T, Kataoka K, et al. Depression is significantly associated with the health status in patients with idiopathic pulmonary fibrosis. Intern Med [Internet]. 2017;56(13):1637–44 Available from: http://www.ncbi.nlm.nih.gov/pubmed/27274328.CrossRef

25.

van Manen MJG, Geelhoed JJM, Tak NC, Wijsenbeek MS. Optimizing quality of life in patients with idiopathic pulmonary fibrosis. Ther Adv Respir Dis [Internet]. 2017;11(3):157–69 Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5933652/.

26.

Lee YJ, Choi SM, Lee YJ, Cho Y-J, Il YH, Lee J-H, et al. Clinical impact of depression and anxiety in patients with idiopathic pulmonary fibrosis. PLoS One [Internet]. 2017;12(9):e0184300 Available from: http://dx.plos.org/10.1371/journal.pone.0184300.CrossRef

27.

Glaspole IN, Chapman SA, Cooper WA, Ellis SJ, Goh NS, Hopkins PM, et al. Health-related quality of life in idiopathic pulmonary fibrosis: data from the Australian IPF registry. Respirology. 2017;22(5):950–6.CrossRef

28.

Furukawa T, Taniguchi H, Ando M, Kondoh Y, Kataoka K, Nishiyama O, et al. The St. George’s Respiratory Questionnaire as a prognostic factor in IPF. Respir Res [Internet]. 2017;18(1):18 Available from: http://respiratory-research.biomedcentral.com/articles/10.1186/s12931-017-0503-3.CrossRef

29.

Hays RD, Morales LS. The RAND-36 measure of health-related quality of life, Ann Med [Internet]. 2001;33(5):350–7 Available from: http://www.ncbi.nlm.nih.gov/pubmed/11491194.CrossRef

30.

Aalto AM, Aro AR, Teperi J. RAND-36 terveyteen liittyvan elamanlaadun mittarina. Mittarin luotettavuus ja suomalaiset vaestoarvot. Stakes, Sos ja terveysalan tutkimus- ja Kehitt tutkimuksia. 1999;101:78.

31.

Swigris JJ, Olson AL, Brown KK. Understanding and optimizing health-related quality of life and physical functional capacity in idiopathic pulmonary fibrosis. Patient Relat Outcome Meas [Internet]. 2016;7:29 Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5519463/.

32.

Swigris JJ, Brown KK, Behr J, du Bois RM, King TE, Raghu G, et al. The SF-36 and SGRQ: validity and first look at minimum important differences in IPF. Respir Med [Internet]. 2010;104(2):296–304 Available from: https://doi.org/10.1016/j.rmed.2009.09.006.CrossRef

33.

Mahler DA, Wells CK. Evaluation of clinical for rating dyspnea. Chest. 1988;93:580–6.CrossRef

34.

Bestall JC, Paul EA, Garrod R, Garnham R, Jones PW, Wedzicha JA. Usefulness of the Medical Research Council (MRC) dyspnoea scale as a measure of disability in patients with chronic obstructive pulmonary disease. Thorax [Internet]. 1999;54(7):581–6 Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1745516&tool=pmcentrez&rendertype=abstract.CrossRef

35.

Bruera E, Kuehn N, Miller MJ, Selmser P, Macmillan K. The Edmonton symptom assessment system (ESAS): a simple method for the assessment of palliative care patients. J Palliat Care. 1991;7(2):6–9.PubMed

36.

Hui D, Bruera E. The Edmonton symptom assessment system 25 years later: past, present, and future developments. J Pain Symptom Manage [Internet]. 2017;53(3):630–43 Available from: https://doi.org/10.1016/j.jpainsymman.2016.10.370.CrossRef

37.

Chang VT, Hwang SS, Feuerman M. Validation of the Edmonton symptom assessment scale. Cancer. 2000;88(9):2164–71.CrossRef

38.

Hannon B, Dyck M, Pope A, Swami N, Banerjee S, Mak E, et al. Modified Edmonton symptom assessment system including constipation and sleep: validation in outpatients with cancer. J Pain Symptom Manage [Internet]. 2015;49(5):945–52 Available from: https://doi.org/10.1016/j.jpainsymman.2014.10.013.CrossRef

39.

Oldenmenger WH, De Raaf PJ, De Klerk C, Van Der Rijt CCD. Cut points on 0-10 numeric rating scales for symptoms included in the Edmonton symptom assessment scale in cancer patients: a systematic review. J Pain Symptom Manage [Internet]. 2013;45(6):1083–93 Available from: https://doi.org/10.1016/j.jpainsymman.2012.06.007.CrossRef

40.

Harrell F. Regression modeling strategies with applications to linear models, logistic regression, and survival analysis. New York: Springer Series in Statistics; 2001.

41.

StataCorp LP; Collage Station, Texas, USA; 2017.

42.

LeBlanc TW, Nickolich M, Rushing CN, Samsa GP, Locke SC, Abernethy AP. What bothers lung cancer patients the most? A prospective, longitudinal electronic patient-reported outcomes study in advanced non-small cell lung cancer. Support Care Cancer. 2015;23(12):3455–63.CrossRef

43.

Iyer S, Taylor-Stokes G, Roughley A. Symptom burden and quality of life in advanced non-small cell lung cancer patients in France and Germany. Lung Cancer [Internet]. 2013;81(2):288–93 Available from: https://doi.org/10.1016/j.lungcan.2013.03.008.CrossRef

44.

Brown S, Thorpe H, Napp V, Brown J. Closeness to death and quality of life in advanced lung Cancer patients. Clin Oncol. 2007;19(5):341–8.CrossRef

45.

ManLois Downey MA, Ruth A, Engelberg RA. Quality-of-Life Trajectories at the End of Life: Assessments Over Time by Patients with and without Cancer. J Am Geriatr Soc. 2011;58(3):472–9.CrossRef

46.

Teno JM, Weitzen S, Fennell ML, Mor V. Dying trajectory in the last year of life: does Cancer trajectory fit other diseases? J Palliat Med [Internet]. 2001;4(4):457–64 Available from: https://www.ncbi.nlm.nih.gov/pubmed/?term=teno+2001+Dying+trajectory+in+the+last+year+of+life%3A+does+Cancer+trajectory+fit+other+diseases.CrossRef

47.

Habraken JM, van der Wal WM, ter Riet G, Weersink EJM, Toben F, Bindels PJE. Health-related quality of life and functional status in end-stage COPD: a longitudinal study. Eur Respir J [Internet]. 2011;37(2):280–8 Available from: http://erj.ersjournals.com/cgi/doi/10.1183/09031936.00149309.CrossRef

48.

Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Kosche D, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J [Internet]. 2015;46(1):186–96 Available from: https://doi.org/10.1183/09031936.00217614.CrossRef

49.

Rajala K, Lehto JT, Sutinen E, Kautiainen H, Myllärniemi M, Saarto T. mMRC dyspnoea scale indicates impaired quality of life and increased pain in patients with idiopathic pulmonary fibrosis. ERJ Open Res [Internet]. 2017;3(4):00084–2017 Available from: http://openres.ersjournals.com/lookup/doi/10.1183/23120541.00084-2017.

50.

Mohan A, Singh P, Singh S, Goyal A, Pathak A, Mohan C, et al. Quality of life in lung cancer patients: impact of baseline clinical profile and respiratory status: original article. Eur J Cancer Care (Engl). 2007;16(3):268–76.CrossRef

51.

Temel JS, Greer JA, Muzikansky A, Gallagher ER, Admane S, Jackson VA, et al. Early palliative Care for Patients with metastatic non–small-cell lung Cancer. N Engl J Med [Internet]. 2010;363(8):733–42 Available from: http://www.nejm.org/doi/abs/10.1056/NEJMoa1000678.CrossRef

52.

Rajala K, Lehto JT, Saarinen M, Sutinen E, Saarto T, Myllärniemi M. End-of-life care of patients with idiopathic pulmonary fibrosis. BMC Palliat Care [Internet]. 2016;15(1):85 Available from: http://www.ncbi.nlm.nih.gov/pubmed/27729035.CrossRef

53.

Temel JS, Greer JA, El-Jawahri A, Pirl WF, Park ER, Jackson VA, et al. Effects of early integrated palliative care in patients with lung and gi cancer: a randomized clinical trial. J Clin Oncol. 2017;35(8):834–41.CrossRef

54.

Higginson IJ, Bausewein C, Reilly CC, Gao W, Gysels M, Dzingina M, et al. An integrated palliative and respiratory care service for patients with advanced disease and refractory breathlessness: a randomised controlled trial. Lancet Respir Med [Internet]. 2014;2(12):979–87 Available from: https://doi.org/10.1016/S2213-2600(14)70226-7.CrossRef

55.

Kalluri M, Claveria F, Ainsley E, Haggag M, Armijo-Olivo S, Richman-Eisenstat J. Beyond Idiopathic Pulmonary Fibrosis diagnosis: Multidisciplinary care with an early integrated palliative approach is associated with a decrease in acute care utilization and hospital deaths. J Pain Symptom Manage [Internet]. 2017; Available from: http://www.ncbi.nlm.nih.gov/pubmed/29101086.

56.

Bakitas MA, Tosteson TD, Li Z, Lyons KD, Hull JG, Li Z, et al. Early versus delayed initiation of concurrent palliative oncology care: patient outcomes in the ENABLE III randomized controlled trial. J Clin Oncol. 2015;33(13):1438–45.CrossRef

57.

Marie B, Balan S, Brokaw FC, Seville J, Jay G. The project ENABLE II randomized controlled trial to improve palliative Care for Patients with advanced Cancer. JAMA. 2009;302(7):741–9.CrossRef

58.

Zimmermann C, Swami N, Krzyzanowska M, Hannon B, Leighl N, Oza A, et al. Early palliative care for patients with advanced cancer: a cluster-randomised controlled trial. Lancet. 2014;383(9930):1721–30.CrossRef

59.

Lindell KO, Nouraie M, Klesen MJ, Klein S, Gibson KF, Kass DJ, et al. Randomised clinical trial of an early palliative care intervention (SUPPORT) for patients with idiopathic pulmonary fibrosis (IPF) and their caregivers: protocol and key design considerations. BMJ Open Respir Res [Internet]. 2018;5(1):e000272 Available from: http://bmjopenrespres.bmj.com/lookup/doi/10.1136/bmjresp-2017-000272.CrossRef

Title: Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life
Authors: K. Rajala
J. T. Lehto
E. Sutinen
H. Kautiainen
M. Myllärniemi
T. Saarto
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: BMC Pulmonary Medicine / Issue 1/2018
Electronic ISSN: 1471-2466
DOI: https://doi.org/10.1186/s12890-018-0738-x

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Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

Abstract

Background

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Results

Conclusions

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Abstract

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