BackgroundCystic fibrosis (CF) is a genetic condition caused by variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that primarily impacts the lungs. Treatments historically have been symptomatic to improve airway clearance and treat infection. However, CFTR modulator drugs have recently been developed that target the underlying defect. The triple combination of elexacaftor-tezacaftor-ivacaftor (ETI) was approved in 2020 in England for over 80% of people with CF aged over 12 years and in 2022 extended to those over 6 years. ETI treatment is associated with substantial improvements in lung function. The experience of children with CF starting on ETI or their views regarding future treatments have not been well studied. This study aimed to explore the opinions of children with CF, their parents/carers and healthcare professionals (HCPs) on the impact of ETI, airway clearance techniques (ACTs) and nebulised treatments.MethodsSemistructured qualitative interviews were performed with 10 children with CF, 7 parents/carers and 10 HCPs. Audio recordings were transcribed and analysed using reflexive thematic analysis.ResultsFour main themes were identified: ‘Kaftrio changed my life’, ‘Your entire life is dictated by the CF timetable’, ‘Simplifying treatment-hopes and fears’ and ‘Kaftrio is a game-changer’ along with several subthemes and an overarching theme of ‘I still can’t get my head around how three tablets can do what Kaftrio done’.ConclusionsDespite the highly positive impact of ETI on the health of children with CF some concerns remain about the longer-term outcomes of reducing ACTs or nebulised treatments. ETI has prompted a shift in treatment for many and offers an opportunity to personalise approaches.

Introduction Post–infectious Obliterative bronchiolitis (OB) has multiple aetiologies with adenovirus the most common cause in the UK. Children with OB usually present with respiratory symptoms and features of small airway obstruction. High resolution CT may show variable small airway obstruction and paucity of vascular markings to support the diagnosis. Treatment is supportive. No published studies have reported the medium term prognosis of OB in UK children. Methods We describe the clinical course of OB in 19 patients followed for up to 5 years after diagnosis. Data on symptoms, lung function, microbiology, imaging and treatment were collected. Results Median age at diagnosis was 4 years (range 1–11 years, 8 boys). Productive cough was common in up to 70% and wheeze occurred in 20%. Adenovirus was the most common cause identified (9 patients), followed by idiopathic (7 patients). CT confirmed diagnosis and repeat showed no change in 3, improvement in 2 and worsening in 2. Seven children had repeat FEV1 measurements, of those 4 had an improvement (increase in% predicted FEV1 4–25%). 69% of the cohort received prophylactic winter antibiotics and on average had an additional 2 treatment courses /year. Chest physiotherapy was used in all children, mainly during exacerbations. 3 patients required supplementary oxygen and BIPAP which we were able to discontinue by year 5. 1 teenage boy received a lung transplant. Conclusion Our cohort has been defined. This is a heterogeneous group with a variable course in disease. An improvement in disease outcome may be possible in some patients with effective treatment. However a national multicentre follow- up longer term study would help to define characteristics and prognosis on a population level.

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