Improving the quality of care for patients with cystic fibrosis

Improvement in cystic fibrosis (CF) outcomes over the past 50 years has been dramatic. This article describes the factors that have contributed to the recent acceleration in this improvement and the important role of the Cystic Fibrosis Foundation.

Initiatives to improve CF care over the past decade include the refinement of a sophisticated patient registry that allows a comparison of center processes and outcomes; development of evidence-based and consensus-based guidelines regarding standards of care; cultural transformation, including the training of care center teams in a systems-oriented approach to quality improvement; data transparency; and encouragement of a patient-centered and family-centered orientation. Cystic Fibrosis Foundation initiatives have promoted an increase in the consistent provision of evidence-based care and the promulgation of innovative, proactive therapeutic approaches. Unfortunately, so far most of these accomplishments have not been documented in peer-reviewed research articles, but rather in platform presentations at national meetings, meeting abstracts, data reports to center directors, and published reports from individual centers.

While several new medications for CF lung disease have been introduced in the last decade, improvements in outcomes have been largely due to refinements in the delivery of care. At this point, the most efficient and reliably effective technique for promoting further improvement still needs to be identified. Recent discussions have centered on attempts at new innovative approaches that utilize more selective teaching of relevant systems-based quality improvement methods in the specific CF environment.