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Abstract Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival. This population-based cohort study used prospectively collected data from 23 Australian CF centres participating in the Australian CF Data Registry (ACFDR) from 2005–2020. Period survival analysis was used to calculate median age of survival estimates for each 5-year window from 2005–2009 until 2016–2020. The overall median survival was estimated using the Kaplan–Meier method. Between 2005–2020 the ACFDR followed 4,601 people with CF, noting 516 (11.2%) deaths including 195 following lung transplantation. Out of the total sample, more than half (52.5%) were male and 395 (8.6%) had undergone lung transplantation. Two thirds of people with CF (66.1%) were diagnosed before six weeks of age or by newborn/prenatal screening. The overall median age of survival was estimated as 54.0 years (95% CI: 51.0–57.04). Estimated median survival increased from 48.9 years (95% CI: 44.7–53.5) for people with CF born in 2005–2009, to 56.3 years (95% CI: 51.2–60.4) for those born in 2016–2020. Factors independently associated with reduced survival include receiving a lung transplant, having low FEV1pp and BMI. Median survival estimates are increasing in CF in Australia. This likely reflects multiple factors, including newborn screening, improvement in diagnosis, refinements in CF management and centre-based multidisciplinary care.

Summary: Background & aims: Malnutrition is associated with functional decline and poorer clinical outcomes for hospitalised patients. This study aimed to survey the knowledge of clinical staff concerning identification/diagnosis and management of inpatient malnutrition and to assess attitudes and knowledge of malnutrition as a shared inter-disciplinary responsibility. Methods: A survey designed to investigate the aims was distributed to clinical staff (medical, nursing, dietitians and allied health) across three hospital campuses including acute, sub-acute and rehabilitation sites. Results: Survey responses were received from 14% (n = 302) of eligible clinical staff (116 nursing, 68 medical, 20 dietitians and 98 other allied health staff). With the exception of dietitians, responses to questions regarding skills and knowledge in the areas of malnutrition identification, diagnosis, treatment and management indicated sub-optimal knowledge. Only 60% of medical/surgical staff and 42% of nursing staff self-reported the ability to diagnose malnutrition. Responses to questions regarding responsibility for malnutrition were highly variable and suggested no significant ‘shared ownership’ of malnutrition as clinical responsibility. Conclusion: Apart from dietitians, malnutrition management skills were modest and knowledge gaps of clinical staff were marked. Confusion also existed as to the responsibility for malnutrition care for inpatients. To improve inpatient nutrition further multidisciplinary engagement and improved education may be required.

Background Quality of life has improved dramatically over the past two decades in people with cystic fibrosis (CF). Quantification has been enabled by patient-reported outcome measures (PROMs); however, many are lengthy and can be challenging to use in routine clinical practice. We propose a short-form PROM that correlates well with established quality-of-life measures. Methods We evaluated the utility of a 10-item score (AWESCORE) by measuring reliability, validity and responsiveness in adults with CF. The questions were developed by thematic analysis of survey questions to patients in a single adult CF centre. Each question was scored using a numerical rating scale 0 to 10. Total scores ranged from 0 to 100. Test–retest reliability was assessed over 24 h. To determine validity, comparisons were sought between stable subjects and those in pulmonary exacerbation, and between AWESCORE and Cystic Fibrosis Questionnaire – Revised (CFQ-R). Responsiveness to pulmonary exacerbation in individual subjects was evaluated. Results Five domains, each with two questions, were identified for respiratory, physical, nutritional, psychological and general health. A total of 246 consecutive adults attending the outpatient clinic completed the AWESCORE. Scores were higher during clinical stability compared to pulmonary exacerbation (mean± sd): 73±11 versus 48±11 (p