Abstract
Purpose
People living with cystic fibrosis (CF) experience impaired quality of life, but the extent to which pulmonary function is associated with quality of life in CF remains unclear
Methods
Using baseline data from a trial of specialist palliative care in adults with CF, we examined the association between pulmonary obstruction and quality of life (measured with the Functional Assessment of Chronic Illness Therapy Total Score).
Results
Among 262 participants, median age was 33, and 78% were on modulator therapy. The median quality of life score was higher in those with mild obstruction (135, IQR 110–156) compared to moderate (125, IQR 109–146) and severe obstruction (120, IQR 106–136). In an unadjusted model, we observed a non-significant trend toward lower quality of life with increased obstruction—compared to participants with mild obstruction, those with moderate obstruction had quality of life score 7.46 points lower (95% CI -15.03 to 0.10) and those with severe obstruction had a score 9.98 points lower (95% CI -21.76 to 1.80). However, this association was no longer statistically significant in the adjusted model, which may reflect confounding due to sex, age, BMI, and modulator therapy. Comorbidities (depression and anxiety) and social determinants of health (financial insecurity and education) were also associated with quality of life.
Conclusion
Advancing our understanding of patient-centered markers of quality of life, rather than focusing on pulmonary function alone, may help identify novel interventions to improve quality of life in this patient population.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References:
Sawicki GS, Sellers DE, Robinson WM (2008) Self-reported physical and psychological symptom burden in adults with cystic fibrosis. J Pain Symptom Manage 35(4):372–380. https://doi.org/10.1016/j.jpainsymman.2007.06.005
Chen E, Killeen KM, Peterson SJ, Saulitis AK, Balk RA (2017) Evaluation of pain, dyspnea, and goals of care among adults with cystic fibrosis: a comprehensive palliative care survey. Am J Hosp Palliat Care 34(4):347–352. https://doi.org/10.1177/1049909116629135
Trandel ET, Pilewski JM, Dellon EP et al (2019) Symptom burden and unmet existential needs in adults with cystic fibrosis. West J Nurs Res 41(10):1448–1464. https://doi.org/10.1177/0193945919852585
Balfour-Lynn IM, King JA (2022) CFTR modulator therapies - effect on life expectancy in people with cystic fibrosis. Paediatr Respir Rev 42:3–8. https://doi.org/10.1016/j.prrv.2020.05.002
Middleton PG, Mall MA, Dřevínek P et al (2019) Elexacaftor–tezacaftor–ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med 381(19):1809–1819. https://doi.org/10.1056/NEJMoa1908639
Regard L, Martin C, Chassagnon G, Burgel PR (2019) Acute and chronic non-pulmonary complications in adults with cystic fibrosis. Expert Rev Respir Med 13(1):23–38. https://doi.org/10.1080/17476348.2019.1552832
Trandel ET, Pilewski JM, Dellon EP et al (2020) Prevalence of unmet palliative care needs in adults with cystic fibrosis. J Cyst Fibros 19(3):394–401. https://doi.org/10.1016/j.jcf.2019.11.010
de Jong W, Kaptein AA, van der Schans CP et al (1997) Quality of life in patients with cystic fibrosis. Pediatr Pulmonol 23(2):95–100. https://doi.org/10.1002/(sici)1099-0496(199702)23:2%3c95::aid-ppul4%3e3.0.co;2-n
Bradley J, McAlister O, Elborn S (2001) Pulmonary function, inflammation, exercise capacity and quality of life in cystic fibrosis. Eur Respir J 17(4):712–715. https://doi.org/10.1183/09031936.01.17407120
Gold LS, Patrick DL, Hansen RN, Goss CH, Kessler L (2019) Correspondence between lung function and symptom measures from the cystic fibrosis respiratory symptom diary-chronic respiratory infection symptom score (CFRSD-CRISS). J Cyst Fibros 18(6):886–893. https://doi.org/10.1016/j.jcf.2019.05.009
Solem CT, Vera-Llonch M, Liu S, Botteman M, Castiglione B (2016) Impact of pulmonary exacerbations and lung function on generic health-related quality of life in patients with cystic fibrosis. Health Qual Life Outcomes. 14:63. https://doi.org/10.1186/s12955-016-0465-z
Lowers J, Dellon EP, Stephenson A et al (2022) Integrating specialist palliative care to improve care and reduce suffering: cystic fibrosis (InSPIRe:CF) - study protocol for a multicentre randomised clinical trial. BMJ Open Respir Res. https://doi.org/10.1136/bmjresp-2022-001381
Lyons KD, Bakitas M, Hegel MT, Hanscom B, Hull J, Ahles TA (2009) Reliability and validity of the functional assessment of chronic illness therapy-palliative care (FACIT-Pal) scale. J Pain Symptom Manage 37(1):23–32. https://doi.org/10.1016/j.jpainsymman.2007.12.015
Arrington-Sanders R, Yi MS, Tsevat J, Wilmott RW, Mrus JM, Britto MT (2006) Gender differences in health-related quality of life of adolescents with cystic fibrosis. Health Qual Life Outcomes. 4:5. https://doi.org/10.1186/1477-7525-4-5
Nagy R, Gede N, Ocskay K et al (2022) Association of body mass index with clinical outcomes in patients with cystic fibrosis: a systematic review and meta-analysis. JAMA Netw Open 5(3):e220740. https://doi.org/10.1001/jamanetworkopen.2022.0740
Luo N, Johnson JA, Shaw JW, Feeny D, Coons SJ (2005) Self-reported health status of the general adult U.S. population as assessed by the EQ-5D and health utilities index. Med Care 43(11):1078–1086. https://doi.org/10.1097/01.mlr.0000182493.57090.c1
Szczesniak R, Heltshe SL, Stanojevic S, Mayer-Hamblett N (2017) Use of FEV(1) in cystic fibrosis epidemiologic studies and clinical trials: a statistical perspective for the clinical researcher. J Cyst Fibros 16(3):318–326. https://doi.org/10.1016/j.jcf.2017.01.002
Miaskowski C, Dodd M, Lee K (2004) Symptom clusters: the new frontier in symptom management research. J Natl Cancer Inst Monogr 32:17–21. https://doi.org/10.1093/jncimonographs/lgh023
Funding
This article funded by the National Heart, Lung, and Blood Institute, Cystic Fibrosis Foundation (Grant No.: 5T32HL116271, KAVAL20QI0
Author information
Authors and Affiliations
Contributions
N.S. contributed to Conceptualization, Formal analysis, and Writing of the Original Draft. J.L. contributed to Investigation and Writing, Reviewing, & Editing of the manuscript. M. J. M contributed to Formal analysis, Statistical expertise, and Writing, Reviewing, & Editing of the manuscript. S. C. A contributed to Formal analysis and Writing, Reviewing, & Editing of the manuscript. W. R. Ht contributed to Validation and Writing, Reviewing, & Editing of the manuscript. A. F contributed to Validation and Writing, Reviewing, & Editing of the manuscript. V. L contributed to Writing, Reviewing, & Editing of the manuscript. D. K contributed to Conceptualization, Investigation, Methodology, Validation, Investigation, Writing, Reviewing, & Editing of the manuscript, and Supervision
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing interests.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Smirnova, N., Lowers, J., Magee, M.J. et al. Pulmonary Function and Quality of Life in Adults with Cystic Fibrosis. Lung 201, 635–639 (2023). https://doi.org/10.1007/s00408-023-00658-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00408-023-00658-y