Ivacaftor Therapy in CF Patients: Single Center Experience

Table 1

Demography, radiology, and lower airway flora.

Patient/; age/gender	Mutation	CT scan before therapy	CT scan after therapy	Lower airway flora before Ivacaftor	Lower airway flora after Ivacaftor	Chronic antibiotic/; insulin
Number 1; 14 yrs/F	del F508/G551D	6 months before: the multiple regions of scattered ground glass and tree in bud opacities in both lower lobes; with scattered bronchiectasis predominantly in the upper lobes with mucoid impaction in distal airways	18 months after: the multiple regions of scattered ground glass and tree in bud opacities in both lower lobes and; near resolution of the prior bibasilar ground glass and tree in bud opacities	MRSA*	MRSA	None
Number 2; 11 yrs/F	del F508/G551D	3 months before: diffuse bronchiectasis and peribronchial thickening in the left lower lobe	Not done	MRSA,; NMPA^$	MSSA^#	None
Number 3; 14 yrs/M	del F508/G551D	6 months before: peribronchial thickening and diffuse mild; bronchiectasis	Not done	MRSA	Normal flora	None
Number 4; 16 yrs/M	del F508/G551D	2 years before: diffuse bronchiectasis and mucoid impactions	6 months after: decreased mucoid impaction, improved bronchial wall thickening in comparison to CT scan 2 years ago	MRSA	MRSA	Inhaled tobramycin and colistin which was discontinued 1 year after Ivacaftor was started
Number 5; 47 yrs/F	2789 + 5G > A/G551D	Not done	Not done	NMPA; MPA^++	NMPA; MPA	Inhaled tobramycin Lispro-insulin

MRSA: methicillin-resistant Staphylococcus aureus.
^#MSSA: methicillin-sensitive Staphylococcus aureus.
^$NMPA: nonmucoid Pseudomonas aeruginosa.
⁺⁺MPA: mucoid Pseudomonas aeruginosa.