Predictive 5-year survivorship model of cystic fibrosis

The objective of this study was to create a 5-year survivorship model to id entify key clinical features of cystic fibrosis. Such a model could help re searchers and clinicians to evaluate therapies, improve the design of prosp ective studies, monitor practice patterns, counsel individual patients, and determine the best candidates for lung transplantation. The authors used i nformation from the Cystic Fibrosis Foundation Patient Registry (CFFPR), wh ich has collected longitudinal data on approximately 90% of cystic fibrosis patients diagnosed in the United States since 1986. They developed multiva riate logistic regression models by using data on 5,820 patients randomly s elected from 11,630 in the CFFPR in 1993. Models were tested for goodness o f fit and were validated for the remaining 5,810 patients for 1993. The val idated 5-year survivorship model included age, forced expiratory volume in 1 second as a percentage of predicted normal, gender, weight-for-age z scor e, pancreatic sufficiency, diabetes mellitus, Staphylococcus aureus infecti on, Burkerholderia cepacia infection, and annual number of acute pulmonary exacerbations. The model provides insights into the complex nature of cysti c fibrosis and supplies a rigorous tool for clinical practice and research.