The CF-ABLE score: a novel clinical prediction rule for prognosis in patients with cystic fibrosis

McCarthy, Cormac, Dimitrov, Borislav D., Meurling, Imran J., Gunaratnam, Cedric and McElvaney, Noel G. (2013) The CF-ABLE score: a novel clinical prediction rule for prognosis in patients with cystic fibrosis. Chest, 143 (5), 1358-1364. (doi:10.1378/chest.12-2022). (PMID:23172242)

Abstract

Background: determining prognosis and predicting outcomes in cystic fibrosis (CF) is a complex issue and there have been very few clinically applicable models for this. Our aim was to create a simple practical outcome prediction tool for CF.

Methods: forty-nine consecutive CF patients from a single centre were studied over an 84-month period (2004-2010). All baseline clinical parameters were gathered and Forced Expiratory Volume in 1 second (FEV1) measurements were analysed over the period. Using patterns of FEV1 decline a “tipping-point” of 52.8% predicted FEV1 was identified. Other clinical variables were analysed and correlated with outcome. “Poor-outcome” was defined as death or transplantation. Using FEV1, Body Mass Index (BMI), Age and Number of Exacerbations in the last 3 months the CF-ABLE score was created. The score was validated on data from 370 patients from the Irish National CF registry.

Results: the ABLE score employs clinical parameters that are measured at every clinic visit. It is scored out of 7 points (range 0-7). If FEV1 is below 52% then 3.5 points are added, if the number of exacerbations in the last three months is greater than 1, then 1.5 points is added, if BMI is less than 20.1 or Age less than 24 years each get 1 point added.

Conclusions: patients with a low score have a very low risk of death or needing lung transplantation within 4 years, however as the score increases the risk significantly increases. Patients who score over 5 points have a 26% risk of poor outcome within 4 years. This score is simple and applicable and better predicts outcome than FEV1 alone

This record has no associated files available for download.

Contributors

Download statistics