International Clinical and Medical Case Reports Journal (ISSN: 2832-5788) | Volume 5, Issue 10 | Case Series | Open Access DOI
Samiha Sajida*
Samiha Sajida1,2*, Jordan Gertenstein1, M Gabriella Rizzo3
1Pediatric Critical Care, Cork University Hospital, Cork, IRL
2Anesthesiology and Critical Care, Cork University Hospital, Cork, IRL
3Internal Medicine, Cork University Hospital, Cork, IRL
*Correspondence to: Samiha Sajida
Fulltext PDFCystic fibrosis (CF) management in Ireland has been limited to symptomatic and supportive treatment prior to the advent of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapy. The implementation of national newborn screening in July 2011, and the development of highly effective modulator therapies have substantially altered the disease trajectory for many individuals with CF in Ireland. We describe three patients with CF treated at Cork University Hospital, each representing a distinct era of diagnosis and management. Patient 1, born in 1978, experienced the expected progression of CF in the pre-screening, pre-modulator era, culminating in bilateral lung transplantation at approximately age 22 and subsequent multisystem complications including chronic allograft rejection, chronic kidney disease (CKD) Stage 4, and Barrett's adenocarcinoma. Patient 2, born in 1988, was diagnosed in adolescence and experienced progressive lung disease prior to the availability of highly effective modulator therapy (HEMT); elexacaftor/tezacaftor/ivacaftor (ETI) improved FEV1 from 61% to 115%, however, established bronchiectasis remained irreversible. Patient 3, born in 2021, was diagnosed on day 3 of life via national newborn screening and commenced ETI granules at age two, with no evidence of chronic infection or structural lung damage to date. This case series illustrates how advances in newborn screening, CFTR modulator therapy, and centralised multidisciplinary care have fundamentally altered the trajectory of CF across successive generations of patients in Ireland. Early diagnosis and treatment may preserve near-normal organ function and life expectancy; however, inequities persist for patients ineligible for modulator therapy.
Case series; cftr modulator; Cork university hospital; Cystic fibrosis; Elexacaftor/tezacaftor/ivacaftor; Highly effective modulator therapy; Ireland; Kaftrio; Lung transplantation; Newborn screening
Samiha Sajida, Jordan Gertenstein, M Gabriella Rizzo. Evolution of Cystic Fibrosis Management in Ireland: A Comparative Three-Patient Case Series. Int Clinc Med Case Rep Jour. 2026;5(10):1-9.