Trends in cystic fibrosis survival over 40 years in South Africa: An observational cohort study

Zampoli, Marco; Kassanjee, Reshma; Verstraete, J; Westwood, Anthony; Zar, Heather J.; Morrow, Brenda

doi:10.1002/ppul.25810

Cited by 5 publications

(2 citation statements)

References 24 publications

(51 reference statements)

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“…Improving median survival age in CF can be attributed to many different factors including NBS, nutritional interventions, proactive disease surveillance at both individual and population levels (including via the ACFDR), management of respiratory infections, access to novel therapies and improved standards of care 16,32,33 . The impact of NBS on survival estimates would not be evident for several decades until babies screened at birth reached an age where they would be at risk for death.…”

Section: Resultsmentioning

confidence: 99%

Survival of People with Cystic Fibrosis in Australia

Ruseckaite

Salimi

Earnest

et al. 2022

Preprint

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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 being F508del homozygous, 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.

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Section: Resultsmentioning

confidence: 99%

Survival of People with Cystic Fibrosis in Australia

Ruseckaite

Salimi

Earnest

et al. 2022

Preprint

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Survival of people with cystic fibrosis in Australia

Ruseckaite

Salimi

Earnest

et al. 2022

Sci Rep

View full text Add to dashboard Cite

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.

show abstract