Objectives: A Delphi consensus of adults with CF and CF health professionals from 13 UK centres identified their most important indicators of PEx(McCourt et al. 2015). These responses were used to pilot a checklist exploring PEx signs, symptoms, severity and recovery. Methods: The highest ranked indicators populated 2 Exacerbation Checklist Scores(ECS) – one for patients(PECS) and one for clinicians(CECS). One point was given to each indicator present including ≥10% FEV1 drop, trouble breathing, reduced saturations, change in sputum, reduction in activity, CRP rise, fever, exhaustion , dyspnoea, respiratory rate, increased cough or airway clearance to maximum of 10(PECs)and 12(CECs)points. In a single centre observational study to assess response to IVs for PEx each participant and study investigator independently completed an ECS on admission. Statistics: Spearman R correlation; survival analysis. Results: 31 participants enrolled, mean(SD)age 29.8(7) yrs; mean FEV1 61.19(25)%. Mean(SD)PECS was 8.7/10(1.6) and mean CECS 7.4/12(1.8). Correlation between PECS and CECS(r=0.55,p=0.001). PECS correlated with CFRSD-CRISS score(r=0.51,p=0.003). CECS correlated with FEV1(L) on admission(r= 0.5,p=0.004) . A PECS≤9 was associated with greater FEV1 recovery at end of treatment(25%(n=5))and longer time to next IVs(90 days)(HR 0 .3)than PECS=10 where 9%(n=1) recovered FEV1 and time to next IVs was 41 days. A CECS≤8 also led to a longer time to next IVs(78 days) compared to CECS≥9(42 days)(HR 0.4). Those with lower scores were less likely to need further IVs within 30 days. A PECS cut off of ≤9(Sensitivity & Specificity 75%)has a negative predictive value(NPV)of 94% whilst a CECS ≤8(Sensitivity 50%, specificity 76%)has a NPV of 91% for no further IVs within 30 days. Conclusion: A simple ECS may be useful to determine PEx severity, correlate with clinical parameters and assist in clinical course prediction. Further validation of the ECS is warranted in a larger population.
|Publication status||Accepted - 2018|
|Event||European Cystic Fibrosis Society Conference, 2018 - Belgrade, Serbia|
Duration: 06 Jun 2018 → …
|Conference||European Cystic Fibrosis Society Conference, 2018|
|Period||06/06/2018 → …|
Addy, C., Bradley, J., Elborn, J., Bradbury, I., & Downey, D. (Accepted/In press). Role of an Exacerbation Checklist Score (ECS) in the definition, assessment and outcome of CF pulmonary exacerbations (PEx). Abstract from European Cystic Fibrosis Society Conference, 2018, Belgrade, Serbia.