Document resource
Introduction LCI 2.5 is used as an outcome measure in research trials for Cystic Fibrosis (CF) therapies, although use in routine clinical practice is limited. Spirometry is the most common lung function test is not always reliable in children under six years. Spirometry is thought to only detect moderate to severe lung disease, and therefore improvements in treatments for CF have normalised spirometry. LCI detects CF airways disease earlier than spirometry, allowing the opportunity for earlier intervention.Method In Edinburgh, the paediatric clinical MBW service started in 2022, and there has been a yearly increase in the number of tests performed. Initially, patients aged 6–16 were seen. In 2023 after further training this reduced to aged 3 and by the end of 2023 to aged 2. This has early respiratory diagnostics in a patient group where spirometry is not possible. Annual test service data are shown in figure 1.Results MBW in the group aged 2–5 years with CF has allowed the clinical team to measure the response to Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy using pre and post LCI in a patient group who were previously modulator naive. Baseline success rates in 2-year-olds seen (3) are 100% by using distraction techniques who had a mean baseline LCI 2.5 of 6.89 (6.25–7.91) only one of these has current LCI data which shows a fall in LCI post ETI.In a group of 5 3-year-olds seen in 2024 pre ETI therapy LCI2.5 mean 7.39 (6.79–8.85) and post mean 6.55 (5.94–8.29) showing a fall.In the 6 years plus group annual and ad-hoc MBW is performed alongside spirometry. A group of 6–11-year-olds showed a baseline mean of 7.01 with a trend towards improvement in mean LCI post ETI therapy of 6.31.In both groups trend data is used to find out if there is an improvement or decline in LCI.Abstract P134 Figure 1Annual test service dataConclusion The introduction of the LCI service clinically has allowed earlier access to diagnostic testing, treatment monitoring, treatment intervention and reduction in treatment burden, which previously would not have been possible.