Impact of newborn screening for cystic fibrosis on clinical outcomes of pediatric patients: 10 years’ experience in Lodz Voivodship

BACKGROUND: Cystic Fibrosis newborn screening (CFNBS) is the optimal method to diagnose the disease during the asymptomatic period. The aim of the study was to determine how CFNBS affects long term clinical outcomes. METHODS: Data from infants who were born in Lodz Voivodship, referred to CF center as a part of CFNBS according to IRT/DNA protocol were compared to the data of children with established CF diagnosis before the start of NBS in Poland (Group CF, n = 52). RESULTS: In 37 children (during 151 referred infants) the diagnosis of CF was established due to CF NBS (CF NBS Group, n = 37). The average time of diagnosis was 1.59 month in Group CF NBS and 45.25 months in 52 children from Group CF. Pulmonary exacerbations occurred on average 4.2 times in Group CFNBS and they were hospitalized on average 0.5 times compared to Group CF – respectively 6.77 and 2.14 (p < 0.001). The number of PA infected patients increased between the fifth and eighth year of age (OR = 1.16 (95% CI: 1.04–19) (P = 0.007)) regardless of the study group (P = 0.984). Patients with MRSA infection have a higher risk of PA infections in subsequent years of their life (OR = 1.45 (95% CI: 1.03–2.03) (P = 0.032)). CONCLUSIONS: CF NBS has beneficial effects primarily on decrease of pulmonary withhope for a longer life expectancy and better and centralised treatment in multidisciplinary CF focused centres.