Survival of people with cystic fibrosis in Australia

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.

Clinical registries: Not yet perfect, but essential for a high‐functioning health system

“…The ACFDR has borne witness to incremental and more recently transformational improvements in morbidity and outcomes for people with CF during this time. Adults exceeded children in the ACFDR for the first time in 2014 4 ; lung function and nutritional status continue to improve; and in the last 5 years particularly, more substantial improvements have been observed in rates of common infections, a decline in overall hospitalisations, a rapid decline in lung transplantation and a sustained increase in life expectancy 5,6 …”

“…Adults exceeded children in the ACFDR for the first time in 2014 4 ; lung function and nutritional status continue to improve; and in the last 5 years particularly, more substantial improvements have been observed in rates of common infections, a decline in overall hospitalisations, a rapid decline in lung transplantation and a sustained increase in life expectancy. 5,6 Registries must evolve with the needs of their populations, and CF registries are an exemplar in this regard. 7 During the COVID-19 pandemic, amid concerns regarding the sudden shift to telehealth, early outcomes published using ACFDR data reported that despite this, overall patient outcomes improved.…”

Critical disease burdens of Australian adults with cystic fibrosis: Results from an online survey

“…Improved treatment and management plans have led to increased survival and the need to predict disease outcomes other than lung transplantations or mortality 10 . According to the Australian Cystic Fibrosis Data Register (ACFDR), the survival age has been increasing over time, whereby the median age of death is currently 31 years 11 . Despite this projected increase in median survival age and the discovery of sophisticated therapies, quality of life is still limited due to significant clinical, psychological, psychosocial, and economic burdens 12 .…”

“…10 According to the Australian Cystic Fibrosis Data Register (ACFDR), the survival age has been increasing over time, whereby the median age of death is currently 31 years. 11 Despite this projected increase in median survival age and the discovery of sophisticated therapies, quality of life is still limited due to significant clinical, psychological, psychosocial, and economic burdens. 12 With increased life expectancy, predicting the prognosis of the patient's disease, such as progression and severity, is critical to the patient's quality of life treatment options, adherence, and psychological health.…”

Gastroenterology services for patients with Cystic Fibrosis across Australia and New Zealand: a multi-stakeholder assessment of patients' and professionals’ perspectives

“…Cystic Fibrosis (CF) is the most common lethal genetic disease in the Caucasian population, with a median age of survival of 54.0 years (1). Death is mainly a result of respiratory failure, which correlates closely to nutritional status (2).…”