Figure 1.

CT radiograph of a patient with advanced CF lung disease demonstrating extensive bronchiectasis and mucoid impaction. Patient was heterozygous for F508del with a forced expiratory volume in 1 s (FEV1) of 0.74 L/22% predicted, history of several pulmonary exacerbations per year, chronic hypoxemia and hypercapnia, and severe pulmonary hypertension. Highly effective modulator therapy, elexacaftor/tezacaftor/ivacaftor, had not yet been approved at the time when the patient required a bilateral lung transplantation.