Table 2:

Change from baseline in clinical outcomes after initiation of ivacaftor

					Absolute Change
Variable	Visit	N	Mean (SD)	N	Mean	95% C.I.	P-value
ppFEV1 (GLI)	Baseline	96	82 (25)	-	-	-	-
	1 month	96	89.4 (23.9)	96	7.5	(5.4, 9.5)	<.0001
	3 month	93	88.5 (23.8)	93	6.1	(4.5, 7.7)	<.0001
	6 month	90	89.8 (23.3)	90	7.9	(5.8, 10.1)	<.0001
	1.5 years	82	87.4 (23.1)	82	4.8	(2.6, 7.1)	<.0001
	5.5 years	78	84.7 (23.3)	78	0.8	(−2.0, 3.6)	0.5739
Weight, kg	Baseline	96	52.1 (20.4)	-	-	-	-
	1 month	96	53.4 (20.5)	96	1.3	(1.0, 1.6)	<.0001
	3 month	94	54.2 (20.8)	94	2.1	(1.6, 2.5)	<.0001
	6 month	92	54.3 (19.8)	92	2.9	(2.2, 3.6)	<.0001
	1.5 years	82	58.7 (18.9)	82	6.6	(5.3, 7.9)	<.0001
	5.5 years	78	64.4 (15.4)	78	12.8	(10.1, 15.5)	<.0001
BMI, kg/m2	Baseline	96	20.4 (4.6)	-	-	-	-
	1 month	96	20.9 (4.5)	96	0.4	(0.3, 0.6)	<.0001
	3 month	94	21.1 (4.6)	94	0.7	(0.5, 0.9)	<.0001
	6 month	92	21.2 (4.7)	92	0.9	(0.7, 1.1)	<.0001
	1.5 years	82	21.9 (4.5)	82	1.6	(1.2, 2.0)	<.0001
	5.5 years	78	23 (4.7)	78	2.5	(2.0, 3.1)	<.0001
CFQR Respiratory	Baseline	96	71 (20.1)	-	-	-	-
	1 month	95	81.8 (13.2)	95	10.4	(7.2, 13.5)	<.0001
	3 month	93	83.7 (15.4)	93	11.8	(8.6, 15.0)	<.0001
	6 month	92	80.3 (16.5)	92	8.8	(4.8, 12.8)	<.0001
	1.5 years	81	80.1 (16.5)	81	9	(4.7, 13.3)	<.0001
	5.5 years	78	79 (16.8)	78	6.7	(2.5, 10.9)	0.0020
Sweat chloride, mEq/L	Baseline	93	103.9 (11.3)	-	-	-	-
	1 month	88	55.2 (22.3)	86	−48.9	(−53.3, −44.4)	<.0001
	3 month	85	49.4 (20.3)	84	−55	(−59.2, −50.8)	<.0001
	6 month	89	47.3 (19.9)	87	−56.4	(−60.5, −52.2)	<.0001
	1.5 years	79	48.9 (22.2)	79	−55.3	(−60.5, −50.1)	<.0001
	5.5 years	76	54.5 (24)	74	−49.5	(−55.0, −44.1)	<.0001