TY - JOUR T1 - Survival at low lung function in cystic fibrosis: Cohort study from 1995 to 2010 JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - P3373 AU - Majdi Osman AU - Thomas Daniels Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/P3373.abstract N2 - Background Outcomes in cystic fibrosis have seen an improvement over the past 20 years but there remains uncertainty on the outcomes of those with severe disease. An FEV1 of less than 30% predicted has been generally accepted as the cut-off for consideration for lung transplantation based on a 2 year survival of 50%. Recently questions have been raised on the clinical significance of this threshold.Aim To evaluate the survival of patients with CF whose lung function has deteriorated to an FEV1 below 30% predicted and to explore factors associated with any change in survival.Method Cohort study of CF subjects across the South of England whose FEV1 was first observed to be less than 30% of the predicted value between 1 January 1995 and 31 December 2009. Survival was examined through to 31 December 2010 in four-year sub-cohorts. At point of entry data was collated on characteristics and current therapies.Results 202 participants in total (111 (55%) male) with median follow up of 5.13 years. Median survival has not been reached for the latest cohort (2004-2009) but 5 year survival for the first cohort (1995-1999) was 37% compared to 64% for the latest cohort (2004-2009). Use of recombinant DNase was significantly associated with a reduced risk of death (hazard ratio 0.64 95% CI 0.41 to 0.99). Significantly increased risk was associated with BMI < 19 (HR 2.19, 1.35 to 3.53) and long term home oxygen therapy (HR 1.90, 1.15 to 3.94).Conclusions The use of FEV1 < 30% predicted as the most important indicator for the timing of lung transplantation is outdated and other factors should be considered. Survival continues to improve, with the importance of airway clearance treatments and good nutrition reinforced. ER -