Skip to main content
Log in

The Correlation of Health-Related Quality of Life with Cystic Fibrosis Severity Markers in Chest CT Scan and 6-Minute Walk Test: A Cross-Sectional Study

  • Original Article
  • Published:
Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Objective

To investigate the correlation between severity of lung disease determined by chest computed tomography (CT) and 6-min walk test (6MWT) with health-related quality of life (HRQoL) score in cystic fibrosis (CF) patients.

Methods

This cross-sectional study evaluated 76 CF patients referred to CF Clinic, aged 7–14 y. Subjects were asked to complete Pediatric quality of life (PedsQL4.0) forms, during their outpatient visits to determine their HRQoL score. Patients’ lung disease severity was quantified by Bhalla score determined by the child’s chest CT and their 6MWT. These three variables were then analyzed to determine whether there is correlation between HRQoL with severity of lung disease.

Results

The mean distance of patients 6MWT score was 447.4 ± 81.4 m. There was a positive correlation between distance and HRQoL score in total, social, school and emotional function (p < 0.05). However, in physical function the correlation lacked significance (p = 0.07). Patients with a Bhalla score of less than 15 were older than patients with a Bhalla score of more than 15 (p < 0.001). Physical, emotional, social, school, and total function scores were significantly lower in patients with Bhalla score less than 15, compared to those with Bhalla score greater than 15 (p < 0.05).

Conclusions

The correlation among Bhalla score on CT scan, 6-min walk test, and HRQoL indicates that pulmonary disease has a clear impact on the quality of life of CF patients. HRQoL can be used in the care program of children with CF.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245:1073–80.

  2. Ratjen F, Döring G. Cystic fibrosis. Lancet. 2003;361:681–9.

  3. Barrio Gómez de Agüero MI, García Hernández G, Gartner S, Grupo de Trabajo de Fibrosis Quística. Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística [protocol for the diagnosis and follow up of patients with cystic fibrosis]. An Pediatr (Barc). 2009;71:250–64.

    Article  Google Scholar 

  4. Brody AS, Kosorok MR, Li Z, et al. Reproducibility of a scoring system for computed tomography scanning in cystic fibrosis. J Thoracic Imaging. 2006;21:14–21.

    Article  Google Scholar 

  5. Davis SD, Fordham LA, Brody AS, et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med. 2007;175:943–50.

    Article  Google Scholar 

  6. Gulmans V, Van Veldhoven N, De Meer K, Helders P. The six-minute walking test in children with cystic fibrosis: reliability and validity. Pediatr Pulmonol. 1996;22:85–9.

    Article  CAS  Google Scholar 

  7. Ratjen F. Recent advances in cystic fibrosis. Paediatr Respir Rev. 2008;9:144–8.

    Article  CAS  Google Scholar 

  8. Orenstein DM, Rosenstein BJ, Stern RC. Cystic fibrosis medical care. USA: Lippincott Williams & Wilkins; 2000.

  9. Spieth LE, Harris CV. Assessment of health-related quality of life in children and adolescents: an integrative review. J Pediatr Psychol. 1996;21:175–93.

    Article  CAS  Google Scholar 

  10. Abbott J, Webb K, Dodd M. Quality of life in cystic fibrosis. J R Soc Med. 1997;90 :37–42.

  11. Gee L, Abbott J, Hart A, Conway SP, Etherington C, Webb AK. Associations between clinical variables and quality of life in adults with cystic fibrosis. J Cyst Fibro. 2005;4:59–66.

    Article  Google Scholar 

  12. Riekert KA, Bartlett SJ, Boyle MP, Krishnan JA, Rand CS. The association between depression, lung function, and health-related quality of life among adults with cystic fibrosis. Chest. 2007;132:231–7.

    Article  Google Scholar 

  13. McCoy KS, Quittner AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB. Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care Med. 2008;178:921–8.

    Article  CAS  Google Scholar 

  14. Abbott J, Hart A. Measuring and reporting quality of life outcomes in clinical trials in cystic fibrosis: a critical review. Health Quality Life Otcomes. 2005;3:19.

  15. Bradley J, McAlister O, Elborn S. Pulmonary function, inflammation, exercise capacity and quality of life in cystic fibrosis. Eur Respir J. 2001;17:712–5.

    Article  CAS  Google Scholar 

  16. Staab D, Wenninger K, Gebert N, et al. Quality of life in patients with cystic fibrosis and their parents: what is important besides disease severity? Thorax. 1998;53:727–31.

    Article  CAS  Google Scholar 

  17. Farrell PM, Rosenstein BJ, White TB, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008;153:S4–S14.

  18. Rabin HR, Butler SM, Wohl MEB, et al. Pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol. 2004;37:400–6.

    Article  Google Scholar 

  19. van Beek EJ, Hill C, Woodhouse N, et al. Assessment of lung disease in children with cystic fibrosis using hyperpolarized 3-helium MRI: comparison with Shwachman score, Chrispin-Norman score and spirometry. Eur Radiol. 2007;17:1018–24.

    Article  Google Scholar 

  20. ATS committee on proficiency standards for clinical pulmonary function laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002;166:111–7.

    Article  Google Scholar 

  21. Eiser C, Morse R. Quality-of-life measures in chronic diseases of childhood. Health Technol Assess. 2001;5:1–157.

    Article  CAS  Google Scholar 

  22. Varni JW, Burwinkle TM, Rapoff MA, Kamps JL, Olson N. The PedsQL™ in pediatric asthma: reliability and validity of the pediatric quality of life inventory™ generic core scales and asthma module. J Behav Med. 2004;27:297–318.

    Article  Google Scholar 

  23. Varni JW, Limbers CA, Burwinkle TM. How young can children reliably and validly self-report their health-related quality of life?: an analysis of 8,591 children across age subgroups with the PedsQL™ 4.0 generic core scales. Health Qual Life Outcomes. 2007;5:1.

  24. Bhatia R, Lesser DJ, Woo MS, Keens TG. Six-minute walk test and health-related quality of life: objective tools to assess improvement in cystic fibrosis patients hospitalized for pulmonary exacerbation. Pediatr Allergy Immunol Pulmonol. 2012;25:86–91.

    Article  Google Scholar 

  25. Troosters T, Langer D, Vrijsen B, et al. Skeletal muscle weakness, exercise tolerance and physical activity in adults with cystic fibrosis. Eur Respir J. 2009;33:99–106.

    Article  CAS  Google Scholar 

  26. Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326:1187–91.

    Article  CAS  Google Scholar 

  27. Kilcoyne A, Lavelle LP, McCarthy CJ, et al. Chest CT abnormalities and quality of life: relationship in adult cystic fibrosis. Ann Transl Med. 2016;4:87.

  28. Tepper LA, Utens EM, Caudri D, et al. Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis. Eur Respir J. 2013;42:371–9.

    Article  Google Scholar 

Download references

Code Availability

Not applicable.

Author information

Authors and Affiliations

Authors

Contributions

MM: Final approval of the version to be submitted; RS: The conception and design of the study, acquisition of data; ZR: Drafting the article, revising it critically for important intellectual content. MM will act as guarantor for this paper.

Corresponding author

Correspondence to Rohola Shirzadi.

Ethics declarations

Conflict of Interest

None.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Modaresi, M., Roshanzamir, Z. & Shirzadi, R. The Correlation of Health-Related Quality of Life with Cystic Fibrosis Severity Markers in Chest CT Scan and 6-Minute Walk Test: A Cross-Sectional Study. Indian J Pediatr 89, 113–117 (2022). https://doi.org/10.1007/s12098-021-03784-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12098-021-03784-8

Keywords

Navigation