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Characteristics, treatment and lung function outcomes of pulmonary exacerbations in cystic fibrosis: insights from the BEAT-CF cohort

Pulmonary exacerbations pose a significant clinical burden on people with cystic fibrosis (pwCF). Whether management of exacerbations should change in the context of modulator therapy is unclear. We describe the characteristics, treatment and lung function outcomes of pulmonary exacerbations requiring intravenous antibiotic therapy (PERITs) in a contemporary Australian cohort of pwCF, in an era of rapidly broadening access to modulator therapy.

Cystic Fibrosis

Cystic fibrosis (CF) is the most common chronic, life-shortening genetic condition affecting young Australians. There is no cure but researchers are working to prevent the onset of lung disease.

The feasibility of using electrical impedance tomography to guide positive pressure airway clearance in children with cystic fibrosis and tracheobronchomalacia

Positive expiratory pressure devices are frequently used for airway clearance in children with cystic fibrosis and tracheobronchomalacia. This study aimed to establish if electrical impedance tomography is a feasible measure to titrate pressures in non-sedated children.

Young children with cystic fibrosis and tracheobronchomalacia have longer and more frequent hospital admissions

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.

Giving kids with CF a chance at a longer, healthier life

COMBAT CF is one of two long-standing international trials which have resulted in new early intervention options helping to reduce progressive lung damage in kids living with CF.

Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis

Pulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcomes should be measured.

Spring-infusors: How a simple and small solution can create king-sized complexity

The aims of the study were to investigate family and hospital staff views about the use of spring-infusor devices for administration of intravenous antibiotic medications, to examine if the device is acceptable and feasible and to map a process for implementation.

Signal-correction errors in the EasyOne Pro LAB multiple-breath washout device significantly impact outcomes in children and adults

Multiple-breath washout (MBW) is an established technique to assess functional residual capacity (FRC) and ventilation inhomogeneity in the lung. Indirect calculation of nitrogen concentration requires accurate measurement of gas concentrations.

Improving screening in a paediatric cohort for cystic fibrosis-related diabetes: A quality improvement project

André Schultz MBChB, PhD, FRACP Head, BREATH Team Head, BREATH Team Prof André Schultz is the Head, BREATH Team at The Kids Research Institute

A Small Device May Deliver King-Sized Solutions for Patients With an Exacerbation of Cystic Fibrosis

The aim is to examine whether using a portable spring-infusor device to deliver antibiotics compared with a standard infusion pump (SIP) translated to (i) improve health outcomes, (ii) reduce the length of stay (LoS), and (iii) reduce cost for treatment of exacerbations of cystic fibrosis.