A contemporary survival analysis of individuals with cystic fibrosis: a cohort study

Abstract

Previously established predictors of survival may no longer apply in the current era of cystic fibrosis (CF) care. Our objective was to identify risk factors associated with survival in a contemporary CF population.We used the Canadian CF Registry, a population-based cohort, to calculate median age of survival and summarise patient characteristics from 1990 to 2012. Clinical, demographic and geographical factors, and survival were estimated for a contemporary cohort (2000-2012) using Cox proportional hazards models.There were 5787 individuals in the registry between 1990 and 2012. Median survival age increased from 31.9 years (95% CI 28.3-35.2 years) in 1990 to 49.7 years (95% CI 46.1-52.2 years) in the most current 5-year window ending in 2012. Median forced expiratory volume in 1 s improved ( p=0.04) and fewer subjects were malnourished ( p<0.001) over time. Malnourished patients (hazard ratio (HR) 2.1, 95% CI 1.6-2.8), those with multiple exacerbations (HR 4.5, 95% CI 3.2-6.4) and women with CF-related diabetes (HR 1.8, 95% CI 1.2-2.7) were at increased risk of death.Life expectancy in Canadians with CF is increasing. Modifiable risk factors such as malnutrition and pulmonary exacerbations are associated with an increased risk of death. The sex gap in CF survival may be explained by an increased hazard for death in women with CF-related diabetes. @ERSpublications Malnutrition and pulmonary exacerbations increase the risk of death in CF but survival in CF has increased over time

Viver Com Fibrose Cística: A Visão Pessoal Do Adolescente Brasileiro

“…O conhecimento sobre as terapias pode trazer esperança para os pacientes com FC. Outro aspecto relacionado à doença é a esperança de maior sobrevida, que aumentou consideravelmente nas últimas décadas (Kuehn, 2014;Stephenson et al, 2015). Atualmente, pacientes com FC podem ter um estilo de vida normal, considerando as limitações do tratamento da doença.…”

“…Este sentimento de esperança, que pode envolver a busca de uma cura, é diferente de negação, porque ela não afeta o ajuste para o processo da doença, e é compatível com a aceitação da realidade e adesão ao tratamento (Stephenson et al, 2015).…”

Cystic Fibrosis: The Dawn of a New Therapeutic Era

“…The dynamic nature of the CF population has been highlighted by significant improvements in survival (5,14). U.S. data have demonstrated that CF survival improved from 2000 to 2010 at a rate of 1.8%/year, and a median survival of 56 years is projected if the mortality rate continues to decrease at the rate observed between 2000 and 2010 (5).…”

A model for active and healthy ageing with a rare genetic disease: cystic fibrosis

“…Advances achieved over the last four decades have transformed CF from a fatal childhood disease to a long-term chronic condition. CF patients can now reach adulthood [2][3][4][5], although most of them still have a reduced life expectancy, and the adult population has passed from 44.55% in 2005 to 49.3% in 2010 (figure 1), the oldest patient being aged >80 years, from the 2010 European CF registry [6]. In the near future, even though CF paediatric care will remain crucial to assure the best growth for patients and transition to adulthood, CF centres may largely be considered as adult oriented [7], and we should, therefore, aim for the new concept of active and healthy ageing (AHA) in CF patients.…”