Ivacaftor Baseline Demographics     n=20

Combination Baseline Demographics     n=32

Age – mean years

32.5 (18-65)

33.05 (20-53)

Male – n (%)

12 (60%)

20 (62.5%)

Height – mean cm

169 (150-182)

169.3(151-187)

Weight - kg mean

67.25 (49.05-121.7)

60.7 (38.95-85.5)

BMI

25.8 (18-36.4)

21.02(16.9-28.37)

Sweat Chloride mmol/L

98.05 (56-113)

Not done

Smoker – n

0

0

Oxygen Supplementation

Nil

Nil

Exocrine Pancreatic Insufficiency – n

18

32

CF Diabetes Mellitus – n

1

15

CF Liver Disease – n

1

12

Fat Mass %

23.03 (4-42)

21.07 (2.4-41.1)

Genotype

G551D/G551D

2

N/A

G551D/ df508

12

N/A 

G551D/Other

G524X (2), V520F (1), Unknown (2)

 N/A

df508/df508

N/A

32

Lung Function

FEV1 % predicted mean

54.5 (20-99)

41.9 (24-95)

FVC % predicted mean

71.05 (40-96)

64.1 (38-107)

Baseline Cognition values

MOCA score

27.6 (22-30)

27.5 (25-30)

Trail A – speed seconds

28.06 (23-36.9)

25.4 (12.3-38)

Trail B – speed seconds

 Not done

50.4 (20-83.60)

Cogstate™ score

 Not done

9.02X10^-17(-.95-0.80)