Background Tracheobronchomalacia (TBM) is characterised by abnormal collapsibility of the trachea and bronchi, often seen in children with cystic fibrosis (CF). This study aims to determine the impact of TBM on hospital admissions in young children with CF. Methods A retrospective study was conducted at a single paediatric tertiary CF centre, examining medical records of children with CF born between January 2009 and June 2019. TBM presence was identified through bronchoscopy records. Hospital admission data, including the number, length and reason for admissions, was collected and analysed. Results Of 101 children included, 59 had TBM. Children with TBM had significantly more hospital admissions and longer hospital stays compared to those without TBM. The risk of all hospital admissions increased by a factor of 2.00 in the first two years and 1.89 in the first four years of life. Risk of respiratory admissions were also higher, increasing by a factor of 3.06 and 2.17 respectively. The total number of days admitted to hospital for any reason increased by a factor of 2.3 in the first two years and 2.05 in the first four years, with respiratory bed days alone increasing by a factor of 3.14 in the first two years. Conclusion TBM in young children with CF is associated with increased hospital admissions and longer hospital stays, particularly for respiratory issues. These findings highlight the need for proactive management to address the increased healthcare burden in this population, and identifies areas for further research on effective interventions aimed at reducing hospitalisations.

Background: Inhaled therapies are used extensively in the treatment of patients with CF. With progressive impairment of lung function, aerosol deposition of inhaled drug occurs more centrally in the lung. The aim of this study was to determine whether long slow inhalations with a dosimetric nebuliser might improve penetration of drug aerosol into the peripheral lung areas. Methods: A comparison of two inhalation modes was undertaken in 5 subjects with moderately severe CF lung disease (aged 12-18 years; FEV1 63-80%) in a crossover study. The pattern of aerosol distribution was compared using a) long (6 – 8 secs) slow inhalations with the dosimetric AKITA delivery system with Pari LC Sprint nebuliser, and b) tidal breathing with a standard Pari LC Sprint nebuliser and compressor. Distribution and total aerosol dose deposited corrected for attenuation was obtained using gamma scintigraphy. Results: Slow, prolonged inhalation with the AKITA was associated with a higher proportion of the delivered dose depositing in the lungs (74.5%), when compared to tidal breathing (32.1%) with significantly less upper airway deposition with the AKITA. The mean peripheral to central deposition ratios for the 5 subjects were long slow breath 2.16 vs tidal breathing 2.13 (p=0.90). Conclusion: Given that the increased lung dose achieved with long slow inhalation is likely to contain a disproportionate amount of larger droplets that are likely to be deposited centrally, particularly in the presence of significant lung disease, the data supports the suggestion that a greater proportion of the finer droplets reach the lung periphery. Hence total and peripheral lung dose are increased by controlling the inspiratory profile.

Objectives: Assuming a causal relationship between depression and the progression of CF, the aim of our study was to show that elevated symptoms of depression could be a predictor of worse lung function.Methods: 22 CF patients were recruited form our adult CF centre (13 women, 9 men, mean age 24, 18-33 years).The patients completed Patient Health Questionnaire-9 (PHQ 9) and the Generalized Anxiety Disorder 7item scale (GAD 7).Body mass indeX (BMI) and pulmonary function testing were measured.Results: Level of anxiety and depression showed no significant association with decreased lung function.This can be explained by the fact that none of the subjects had serious anxiety and/or depression and prospective study should bed one. Conclusion:The interaction between depression and disease progression could be linked to the lack of hope, optimism and perceived self-efficacy in depressed patients, consequently inhibiting their fighting spirit against the disease and necessary adherence to treatment.Thus, depressed patients may fail to respond to standard medical treatment due to the nature of their mental health problems, and may experience a greater loss of lung function than necessary.This effect may then reinforce the negative thoughts related to their own prognosis, triggering a vicious psychosomatic cycle.In contrast, patients who are not depressed and already have severely restricted lung function might be more able to make use of resources for their daily treatment routines.This, in turn, might result in stable or even slightly improved outcomes.