OBJECTIVE: To review health-related quality of life (QOL) and associated issues and to describe a study investigating "Child Health Questionnaire" (CHQ) scores in relationship to newborn screening (NBS) for cystic fibrosis (CF) and markers of disease severity. METHODS: A total of 36 patients from 10-15.5 years old who were enrolled in the screened or control group of the Wisconsin CF Neonatal Screening Project completed the CHQ. Scale scores comprised the dependent variables. Independent variables included study group and measures of disease severity. Analyses included Fisher's exact, 2-sample Wilcoxon, and t tests. RESULTS: QOL did not differ significantly between the screened and control groups for any of the scales. None of the comparisons of CHQ scale scores across measures of disease severity were significant in this small sample, but the CHQ and power were limiting. CONCLUSIONS: Our results did not demonstrate a benefit of CF NBS on QOL; however, the CHQ may not be adequately sensitive to QOL in children with CF with disease severity comparable to our sample. The Cystic Fibrosis Questionnaire, a recently validated CF-specific QOL measure for pediatric samples, is likely to provide a more informative evaluation of the effects of CF NBS on patients' QOL.
OBJECTIVE: To review health-related quality of life (QOL) and associated issues and to describe a study investigating "Child Health Questionnaire" (CHQ) scores in relationship to newborn screening (NBS) for cystic fibrosis (CF) and markers of disease severity. METHODS: A total of 36 patients from 10-15.5 years old who were enrolled in the screened or control group of the Wisconsin CF Neonatal Screening Project completed the CHQ. Scale scores comprised the dependent variables. Independent variables included study group and measures of disease severity. Analyses included Fisher's exact, 2-sample Wilcoxon, and t tests. RESULTS: QOL did not differ significantly between the screened and control groups for any of the scales. None of the comparisons of CHQ scale scores across measures of disease severity were significant in this small sample, but the CHQ and power were limiting. CONCLUSIONS: Our results did not demonstrate a benefit of CF NBS on QOL; however, the CHQ may not be adequately sensitive to QOL in children with CF with disease severity comparable to our sample. The Cystic Fibrosis Questionnaire, a recently validated CF-specific QOL measure for pediatric samples, is likely to provide a more informative evaluation of the effects of CF NBS on patients' QOL.
Authors: Katherine V Barnes; Francesca R Coughlin; Heather M O'Leary; Natalie Bruck; Grace A Bazin; Emily B Beinecke; Alexandra C Walco; Nicole G Cantwell; Walter E Kaufmann Journal: J Neurodev Disord Date: 2015-09-15 Impact factor: 4.025
Authors: Kee Chan; Joie Davis; Sung-Yun Pai; Francisco A Bonilla; Jennifer M Puck; Michael Apkon Journal: Mol Genet Metab Date: 2011-07-12 Impact factor: 4.797
Authors: J B Lane; H-S Lee; L W Smith; P Cheng; A K Percy; D G Glaze; J L Neul; K J Motil; J O Barrish; S A Skinner; F Annese; L McNair; J Graham; O Khwaja; K Barnes; J P Krischer Journal: Neurology Date: 2011-10-19 Impact factor: 9.910
Authors: Christina M D'Angelo; Sylvie Mrug; Daniel Grossoehme; Kevin Leon; Lacrecia Thomas; Bradley Troxler Journal: J Pediatr Psychol Date: 2021-02-19