n (%), mean (SD)

Gender (% female)

35 (52%)

Age

29 (8.9)

Body Mass Index (kg/m2)

23 (3.7)

Ethnicity

Caucasian

57 (84%)

Hispanic

9 (13%)

African-American

2 (3%)

Medical History

Pulmonary Hypertension

6 (9%)

Cystic Fibrosis Related Diabetes

38 (56%)

Pneumothorax

1 (2%)

Lobectomy

3 (4%)

Genotype

F508del Homozygous

26 (38%)

F508del Heterozygous

33 (49%)

Other

8 (12%)

Mortality (% expired)

13 (20%)

Total exacerbations over 1 year

0.7 (0.9)

Total exacerbations over 3 years

2.3 (2.5)

CFQ-R-Respiratory Score (%)

75 (15)

Lung Function

FEV1 (% pred)

61 (27)

FVC (% pred)

74 (20)

FEV1/FVC (%)

64 (15)

TLC (% pred)

102 (15)

IC (% pred)

90 (23)

IC/TLC (%)

43 (11)

FRC (% pred)

107 (31)

FRC/TLC (%)

57 (11)

RV (% pred)

176 (79)

RV/TLC (%)

43 (16)