The feasibility of collecting the physiotherapy outcomes airway clearance, physical activity and fitness for the Australian Cystic Fibrosis Data Registry.

BACKGROUND: Physiotherapy-related data, such as airway clearance techniques (ACTS), physical activity and aerobic fitness are not consistently included in international cystic fibrosis (CF) data registries. This study aimed to pilot the collection of ACTS, physical activity and fitness in a hospital CF clinic, as a step towards informing future national implementation. METHODS: This study was undertaken in a CF clinic within a major tertiary hospital. Patients and families were invited to participate. Participants completed self-report questionnaires on ACT use and those aged ≥ 10 years completed a physical activity questionnaire (Core Indicators and Measures of Youth Health Survey) and aerobic fitness test (the A-STEP test). Participants also completed a survey to explore the tolerance and acceptability of the fitness test, and the perceived accuracy of the self-reported data collection. RESULTS: Forty patients agreed to participate in the study (mean age = 9.8, SD = 4.1 years old; 52.5% female). All patients and/or families that were approached agreed to participate and completion rate for the ACTs and physical activity surveys was 98% and 100% (respectively). Completion rate for the fitness test was 55%, due to time constraints. Most participants agreed (≥ 90%) they could accurately provide ACT and physical activity data, and the assessments were tolerable and acceptable. CONCLUSIONS: Patients with CF and their families are able to and can acceptably provide physiotherapy-related data, and collecting self-report ACTs and physical activity data is highly feasibly during routine CF clinic visits. However, aerobic fitness testing using the A-STEP test may be less feasible in clinic environments, due to time constraints.

Feasibility of the A-STEP for the assessment of exercise capacity in people with cystic fibrosis.

OBJECTIVES: To evaluate feasibility of the Alfred Step Test Exercise Protocol (A-STEP) for the assessment of exercise capacity in adults and children with cystic fibrosis (CF); in adults to test whether demographics and/or lung function correlated with exercise capacity. METHODS: Adults and children with stable CF from two centres completed the A-STEP (a recently developed incremental maximal-effort step test). Feasibility was evaluated by: usefulness for exercise capacity assessment (measures of exercise capacity were: level reached, exercise-induced desaturation, and achievement of at least one maximal effort criteria); safety; operational factors; time to complete; floor and/or ceiling effects. We used multiple linear regression to test whether demographics and/or lung function correlated with exercise capacity. RESULTS: A total of 49 participants: 38 adults (18 male), percent predicted (pp) forced expiration in one second (FEV1 ) 29-109, aged 22-48 years and 11 children (6 male), ppFEV1 68-107, aged 10-15 years were included. Levels reached (mean (SD) [range]) were 10.2 (2.4) [6-15] (adults), 10.1 (2.5) [7-14] (children); desaturation (change between baseline and peak-exercise SpO2 ): was 8.4 (3.8 [0-15]% (adults), 2.0 (2.0) [0-7]% (children). A total of 8 (21%) adults and no children desaturated <90% SpO2 . At least one criterion for maximal effort was reached by 33 (84%) adults and 10 (91%) children. There were no adverse events. The A-STEP was straightforward to use and carried out by one operator. A total of 26 (68.4%) adults and 7 (63.6%) children completed the test within the recommended 8-12 min. All participants completed a minimum of 6 levels, and completed the test before the final 16th level. In adults, ppFEV1 and ppFVC correlated with the level reached (r = 0.55; p = <0.001 and r = 0.66, p = <0.0001) and desaturation (r = 0.55, p = <0.001 and r = 0.45, p = <0.005). CONCLUSION: In adults and children with stable CF, the A-STEP was feasible, safe, and operationally easy to use for the assessment of exercise capacity, without floor or ceiling effects. In adults, lung function correlated with exercise capacity.

Should the physiotherapy outcomes airway clearance, physical activity and fitness be recorded on the Australian Cystic Fibrosis Data Registry? A consensus approach.

BACKGROUND: Physiotherapy is a cornerstone of cystic fibrosis (CF) management, yet the Australian CF Data Registry (ACFDR) currently does not record physiotherapy-related data. This study aimed to gather opinions from lead Australian CF physiotherapists regarding the importance and feasibility of collecting physiotherapy-related data on the ACFDR. METHODS: A three-round online Delphi survey was conducted to gather expert stakeholder opinion and consensus agreement. Lead physiotherapists from all 23 Australian CF centres were invited to participate. Round one explored the potential benefits, barriers and importance of recording three physiotherapy-related domains on the ACFDR: airway clearance, physical activity and fitness. Subsequent rounds were developed based on the findings from the previous round and sought consensus (80% agreement) for the inclusion of physiotherapy-related data on the ACFDR and for the most appropriate methods of collecting such data. RESULTS: The response rate was > 80% for all rounds. Participants agreed that collection of airway clearance, physical activity and fitness data on the ACFDR was important and feasible. Findings suggested that airway clearance and physical activity should be collected using self-reported questionnaires, while fitness should be measured using a field-based test. CONCLUSIONS: Australian lead CF physiotherapists believe that collection of airway clearance, physical activity and fitness on the ACFDR is important and feasible. Future work is needed to pilot the data collection procedure to examine its feasibility in real-world clinical settings. This study demonstrates how Delphi methodology can provide a contemporary summary of expert clinicians' opinion that may underpin nation-wide health service improvement.

Health-related quality of life and psychological wellbeing are poor in children with bronchiectasis and their parents.

Children with bronchiectasis in a stable state have poor HRQoL and their parents report higher levels of anxiety and depression. Parental perception of HRQoL is associated with poorer parental psychological wellbeing. http://bit.ly/2lcf3uB.

Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline.

Physiotherapy management is a key element of care for people with cystic fibrosis (CF) throughout the lifespan. Although considerable evidence exists to support physiotherapy management of CF, there is documented variation in practice. The aim of this guideline is to optimize the physiotherapy management of people with CF in Australia and New Zealand. A systematic review of the literature in key areas of physiotherapy practice for CF was undertaken. Recommendations were formulated based on National Health and Medical Research Council (Australia) guidelines and considered the quality, quantity and level of the evidence; the consistency of the body of evidence; the likely clinical impact; and applicability to physiotherapy practice in Australia and New Zealand. A total of 30 recommendations were made for airway clearance therapy, inhalation therapy, exercise assessment and training, musculoskeletal management, management of urinary incontinence, managing the newly diagnosed patient with CF, delivery of non-invasive ventilation, and physiotherapy management before and after lung transplantation. These recommendations can be used to underpin the provision of evidence-based physiotherapy care to people with CF in Australia and New Zealand.