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Comparative Study
. 2011 Jul;140(1):170-177.
doi: 10.1378/chest.10-1504. Epub 2010 Nov 24.

Relationships among health-related quality of life, pulmonary health, and newborn screening for cystic fibrosis

Audrey Tluczek  1 , Tara Becker  2 , Anita Laxova  3 , Adam Grieve  3 , Caroline N Racine Gilles  3 , Michael J Rock  3 , William M Gershan  4 , Christopher G Green  3 , Philip M Farrell  3
Affiliations
Comparative Study

Relationships among health-related quality of life, pulmonary health, and newborn screening for cystic fibrosis

Audrey Tluczek et al. Chest. 2011 Jul.

Abstract

Background: The objective of this study was to examine relationships between pulmonary health and health-related quality of life (HRQOL) in patients with cystic fibrosis (CF) evaluated longitudinally in the Wisconsin Newborn Screening Project.

Methods: Patients aged 8 to 18 years (mean ± SD, 13.5 ± 2.8) in early diagnosis (n = 45) and control (n = 50) groups completed Cystic Fibrosis Questionnaires (CFQs) to measure HRQOL at three data points over a 2-year period. Pulmonary health was evaluated concurrently by the Wisconsin chest x-ray scoring system (WCXR) and pulmonary function tests (PFTs).

Results: WCXR showed significant group differences (P ≤ .023), with the early diagnosis group showing more-severe lung disease. When adjusted for group differences in mucoid Pseudomonas aeruginosa status and pancreatic status, however, WCXR differences and PFT data were not significant. Most patients (74%) had FEV(1) values ≥ 80% predicted (within normal range). For patients aged < 14 years, as WCXR scores worsened CFQ respiratory and physical domain scores decreased (both P ≤ .007). FEV(1)/FVC showed a positive relationship with the respiratory and physical domains (both P ≤ .006). WCXR scores for patients aged ≥ 14 years were associated with CFQ weight, respiratory, and health domains (all P ≤ .011). FEV(1) was associated with CFQ weight, respiratory, health, and physical domains (all P ≤ .003). Changes in pulmonary health were not associated with changes in CFQ over time. Significant group differences on the CFQ-Child social functioning domain favored the control group.

Conclusions: To our knowledge, this study is the first to compare pulmonary outcomes with HRQOL indicators assessed by serial, standardized, patient-reported outcome measures for patients with CF identified either through newborn screening or diagnosed by use of traditional methods. This study found no benefits of newborn screening for pulmonary health or HRQOL after controlling for risk factors. Using WCXR and PFT data collectively helped to identify associations between pulmonary health and HRQOL.

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Figures

Figure 1.
Figure 1.
Enrollment and data collection time frames for the original RCT and current HRQOL study. HRQOL = health-related quality of life; RCT = randomized clinical trial.
Figure 2.
Figure 2.
Group comparison of objective measures of pulmonary health. PFT = pulmonary function test; WCXR = Wisconsin chest x-ray scoring system.
Figure 3.
Figure 3.
Group differences on CFQ-Child and CFQ-Adolescent/Adult scores. CFQ = Cystic Fibrosis Questionnaire.
See this image and copyright information in PMC

References

    1. Cystic Fibrosis Foundation Web site. http://www.cff.org. Accessed November 4, 2009.
    1. Farrell PM. Wisconsin Cystic Fibrosis Neonatal Screening Study Group Improving the health of patients with cystic fibrosis through newborn screening. Adv Pediatr. 2000;47:79–115. - PubMed
    1. Farrell PM, Kosorok MR, Laxova A, et al. Wisconsin Cystic Fibrosis Neonatal Screening Study Group Nutritional benefits of neonatal screening for cystic fibrosis. N Engl J Med. 1997;337(14):963–969. - PubMed
    1. Farrell PM, Kosorok MR, Rock MJ, et al. Wisconsin Cystic Fibrosis Neonatal Screening Study Group Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Pediatrics. 2001;107(1):1–13. - PubMed
    1. Caine N, Sharples LD, Smyth R, et al. Survival and quality of life of cystic fibrosis patients before and after heart-lung transplantation. Transplant Proc. 1991;23(1 pt 2):1203–1204. - PubMed

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