Chest
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Cystic fibrosis (CF) is a life-shortening inherited disease caused by mutations in the CF transmembrane conductance regulator gene (CFTR), which encodes for the CF transmembrane conductance regulator (CFTR) ion channel that regulates chloride and water transport across the surface of epithelial cells. Ivacaftor, a drug recently approved by the US Food and Drug Administration, represents the first mutation-specific therapy for CF. It is a CFTR channel modulator and improves CFTR function in patients with CF who have a G551D mutation. ⋯ However, a decrease in sweat chloride did not correlate with improvement in FEV(1), nor did there appear to be a threshold level for change in sweat chloride above which an improvement in FEV(1) was apparent. The lack of correlation of sweat chloride with improvement in FEV(1) speaks to the multiplicity of factors, physiologic, environmental, and genetic, that likely modulate CF disease severity. Future clinical trials of drugs that are directed to the defective CFTR will need take into account the uncertainty of using even established measurements, such as sweat chloride, as clinical end points.
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Electronic health record (EHR) order sets are common. Order sets represent one clinical decision support (CDS) tool within computerized provider order entry systems that may promote safe, efficient, and evidence-based patient care. A small number of order sets account for the vast majority of use, suggesting that some order sets have a higher value to clinicians than others. ⋯ There are general guidelines on CDS and usability that can be applied to the development of EHR order sets. Order set success requires leadership, planning, and resources as well as ongoing maintenance and evaluation. This article describes one academic medical center's experience in developing an EHR order set embedded with evidence-based principles and lessons learned for future order set development.