Table 4 Mean respiratory values after Lumacaftor/Ivacaftor treatment

FEV1% (SD)

(16 ±)65.7

(18.3 ±)61.5

(17.6 ±)62.6

(15.8 ±)60.6

 Change from baseline

5.7

0.7

2.6

0.6

 P-value

0.009

0.861

0.34

0.921

FVC% (SD)

(18.6 ±)82.8

(17.7 ±)80.9

(18.2 ±)77.1

(17.5 ±)78.2

 Change from baseline

7.3

4.6

1.6

2.7

 P-value

0.027

0.0371

0.0775

0.0626

FEF25-75% (SD)

(17 ±)34.5

(17.2 ±)31.1

(17.3 ±)32.6

(19.9 ±)34

 Change from baseline

4.3

0.5

2.5

3.9

 P-value

0.001

0.764

0.181

0.672

CT Score (SD)^a

-

-

-

(2.7 ±)12.5

 Change from baseline

-

-

-

-0.3

 P-value

-

-

-

0.517