Pediatric invasive long-term ventilation-A 10-year review.

INTRODUCTION: The number of children with complex physical and developmental pathologies, including chronic respiratory insufficiency, surviving and growing beyond early childhood continues to rise. No study has examined the clinical pathway of children on invasive long-term mechanical ventilation (LTMV) in an Irish setting. Our data over a 10-year period were reviewed to see if our demographics and outcomes are in line with global trends. METHODS: Children's Health Ireland (CHI) at Crumlin, Dublin is Ireland's largest tertiary pediatric hospital. A retrospective review analyzed data from children in our center commenced on LTMV via a tracheostomy over 10 years (2009-2018). This data was subdivided into two epochs for statistical analysis of longitudinal trends. RESULTS: Forty-six children were commenced on LTMV from 2009 to 2018. Many had complex medical diagnoses with associated comorbidities. Far less children, 30.4% (n = 14) commenced LTMV in the latter half of the 10-year period, they also fared better in all aspects of their treatment course. Focusing solely on children who have needed LTMV over this timeframe we have been able to isolate trends specific to this cohort. Less patients commenced LTMV on a year-on-year basis, and for those that require tracheostomy and LTMV, their journey to decannulation tends to be shorter. CONCLUSION: Over the period reviewed, less patients over time necessitated LTMV, and those patients are being weaned and decannulated with ever more success. This has implications in terms of predicting numbers transitioning to adult services and allocation of hospital and community care resources.

Neonatal screening programme for CF: Results from the Irish Comparative Outcomes Study (ICOS).

The introduction of NBS in Ireland in July 2011, provided a unique opportunity to investigate clinical outcomes using a comparative historical cohort study. Clinical cohort: children clinically diagnosed with CF born 1 July 2008 to 30 June 2011, and NBS cohort: children diagnosed with CF through NBS born 1 July 2011 to 30 June 2016. Clinical data were collected from the CF Registry of Ireland, medical charts, and data on weight/height before diagnosis from public health nurses and family doctors. SPSS was used for analysis. A total of 232 patients were recruited (response 93%) (93 clinically diagnosed, 139 NBS-detected). Following exclusions of meconium ileus (MI) (40), diagnosis outside Ireland (4), and being designated as CFSPID (2), a total of 77 clinically diagnosed patients and 109 NBS detected children were included in analysis. Over half were homozygous for F508del mutation. Being clinically diagnosed was independently associated with hospitalization for infective exacerbation of CF < 36 months (OR, 2.80; 95%CI 1.24-6.29). Diagnosis to first acquisition of Pseudomonas aeruginosa was significantly longer in NBS than clinically detected; from birth there was no significant difference. Weight and length/height were significantly greater in NBS cohort at 6 and 12 months. We provide evidence of improved growth, reduced hospitalization for acute exacerbations, and delayed P. aeruginosa acquisition (from diagnosis) to age 3 for the NBS cohort. Screening practices likely account for the non-significant difference in P. aeruginosa acquisition from birth.