Table 1 Characteristics of the population before the first ICU hospitalization

Age, yr

28.1 ± 7.8

Sex, M/F

25/17

Severity of CFTR genotype, S/M/ND

30/4/8

BMI, Kg/m ²

17.8 ± 2.1

Extra-pulmonary involvement

Pancreatic insufficiency

37 (88%)

Diabetes

8 (19%)

Cirrhosis

3 (7%)

Chronic airway colonization

P. aeruginosa

39 (93%)

B. cepacia complex

2 (5%)

Lung function in stable state

FEV₁, % predicted

28 ± 12

FVC, % predicted

45 ± 15

TLC, % predicted

98 ± 22

Room air PaO₂, mmHg

62 ± 15

Room air PaCO₂, mmHg

47 ± 9

Room air SaO₂, %

89 ± 8

Annual FEV₁ loss, % predicted/yr

4.21 ± 2.65

Home ventilatory support

Long term oxygenotherapy alone

12 (29%)

Noninvasive ventilation

10 (24%)

Patients awaiting lung transplant

9 (21%)