Evaluation of the clinical, psychological and economic effects of the Cystic Fibrosis Newborn Screening Programme: the Irish Comparative Outcomes Study of CF (ICOS) Part 2

This is a national programme of research which addresses key outcomes in the recently established Cystic Fibrosis (CF) Newborn Screening programme (NBS). Ireland has the highest prevalence of CF worldwide. NBS for CF was introduced in 2011. The Irish Comparative Outcome Study (ICOS) Part I was conducted in 2013-2017 and studied children with CF (CwCF) to age 3-5 years.

This study aims to continue this research programme in ICOS Part 2, which will follow CwCF to age 8-11 years. This study aims to continue evaluating important clinical and economic parameters of the screening programme by comparing those children detected by the screening programme with children diagnosed after they present with symptoms in terms of the following:

  • health outcomes, including growth, weight and height, lung function, infection rates, number of admissions to hospital in the early years of life;
  • timing of onset of early respiratory complications in children diagnosed through NBS;
  • the effect of gender and presence of specific genes on early development of the disease;
  • comparisons of screen-detected children's early health with results from the European registry;
  • health costs incurred by parents and by the health services following diagnosis and
  • comparison of carer burden and quality of life in parents of CwCF.

The proposed research on the CF Newborn Screening Programme will utilise both data collected by the National Cystic Fibrosis Registry and complementary additional data from parents directly and from hospital charts. Recently two important drugs have been added to the treatment of CwCF - Orkambi and Kalydeco.

It is important to conduct this follow-up evaluation of the CF NBS to see its effect in the context of these new treatments; this research is both timely and necessary, providing important information for multidisciplinary teams caring for CwCF and also the parents of CwCF.

Award Date
27 June 2019
Award Value
Principal Investigator
Professor Patricia Fitzpatrick
Host Institution
University College Dublin
Investigator Led Projects