Ivacaftor Therapy in CF Patients: Single Center Experience

Figure 2

FEV1 trend. There was significant overall improvement () in FEV1, however more so in the younger patients. Patient number 5 (46-year-old female) who already had established bilateral bronchiectasis had a modest improvement. Patient number 4, a 14-year-old male who also had bilateral diffuse bronchiectasis and multiple pulmonary exacerbations prior to starting Ivacaftor, demonstrated significant improvement in his FEV1 presumably due to decreased mucus impaction in the airways (see chest CT scans in Figure 4).