BackgroundPhysiotherapists (PTs) are ideally positioned to assist patients and families with inhalation therapies through monitoring, communication and education about available therapies and their proper use; indeed aerosoltherapy management is listed as part of Italian PTs' core competence and in the core syllabus for post-graduate training in respiratory physiotherapy. The aim of this study was to outline the involvement of Italian PTs working in Cystic Fibrosis (CF) centres in the aerosol delivery practice.MethodsPhysiotherapist coordinators (n = 29) of all Italian CF centres were invited to participate in a cross-sectional survey and a semi-structured questionnaire was developed and sent by e-mail.ResultsA response rate of 69 % was achieved. The majority of participants were woman and the overall mean professional experience was twenty years. Italian PTs are involved in the aerosol delivery practice, managing education, drug-device combination, dilution and mixing of drugs.ConclusionsPhysiotherapists play a key role in the care of Italian CF patients; nevertheless the Italian Group of Physiotherapists might plan interventions to harmonize the aerosol delivery practice in Italian CF centres and to sustain continuing education.

Background Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple‐breath washout test and symptoms‐limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross‐sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results Seventy‐seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53‐8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96‐5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

Background Lung clearance index (LCI) is accepted as an early marker of lung disease in cystic fibrosis (CF), however the utility of LCI to identify subgroups of CF disease in the paediatric age group has never been explored. The aim of the study was to characterize phenotypes of children with CF using LCI as a marker of ventilation inhomogeneity and to investigate whether these phenotypes distinguished patients based on time to pulmonary exacerbation (PE). Methods Data were collected on patients with CF aged < 18 years old, attending the CF Center of Milan during outpatient follow-up visits between October 2014 and September 2019. Cluster analysis using agglomerative nesting hierarchical method was performed to generate distinct phenotypes. Time-to-recurrent event analysis investigated association of phenotypes with PE. Results We collected 313 multiple breath washout tests on 125 children aged 5.5–16.8 years. Cluster analysis identified two divergent phenotypes in children and adolescents of same age, presenting with almost normal FEV1 but with substantial difference in markers of ventilation inhomogeneity (mean LCI difference of 3.4, 95% Confidence Interval [CI] 2.6–4.2). A less severe phenotype was associated with a lower risk of PE relapse (Hazard Ratio 0.45, 95% CI 0.34–0.62). Conclusions LCI is useful in clinical practice to characterize distinct phenotypes of children and adolescents with mild/normal FEV1. A less severe phenotype translates into a lower risk of PE relapse.

Increased functional exercise tolerance is related to higher static expiratory pressure in patients with mild-to-normal cystic fibrosis lung disease. Difference in exercise performance might reflect a possible difference in body composition that needs to be further investigated.

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