CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Quality of life of children with cystic fibrosis.

Study design (if review, criteria of inclusion for studies)

RCT

Participants

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 study

Interventions

Neonatal screening vs standard diagnostic methods.

Outcome measures

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.

Main 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.

Authors' conclusions

There is no 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 this 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.

Keywords: Neonatal Screening; Newborn; non pharmacological intervention - diagn; screening; diagnostic procedures;