title: Relationships among health-related quality of life, pulmonary health, and newborn screening for cystic fibrosis.
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.
authors.affiliation: School of Nursing, School of Medicine and Public Health, University of Wisconsin, Madison, WI; Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI. Electronic address: atluczek@wisc.edu.
authors.forename: Audrey
authors.initials: A
authors.lastname: Tluczek
authors.affiliation: Department of Biostatistics and Medical Informatics, School of Medicine and Public Health, University of Wisconsin, Madison.
authors.forename: Tara
authors.initials: T
authors.lastname: Becker
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Anita
authors.initials: A
authors.lastname: Laxova
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Adam
authors.initials: A
authors.lastname: Grieve
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Caroline N Racine
authors.initials: CNR
authors.lastname: Gilles
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Rock
authors.affiliation: Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI.
authors.forename: William M
authors.initials: WM
authors.lastname: Gershan
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Green
authors.affiliation: Department of Pediatrics Racine, School of Medicine and Public Health, University of Wisconsin, Madison, WI.
authors.forename: Philip M
authors.initials: PM
authors.lastname: Farrell
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D042241:Early Diagnosis; D005260:Female; D006304:Health Status; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening / Q000379:methods*; D011379:Prognosis; D011788:Quality of Life; D012129:Respiratory Function Tests; D011795:Surveys and Questionnaires; D014922:Wisconsin / Q000453:epidemiology