Table 1 Sociodemographic and clinical characteristics of CF patients at the beginning of the treatment

Patient characteristics at baseline (N=108)

Age (years)

30.0 [17.0, 61.0]

Sex [Male]

59 (54.6%)

Type of mutation [ Heterozygous]

59 (54.6%)

Exocrine pancreas [pancreatic insufficiency]

91 (84.3%)

Endocrine pancreas [hydrocarbon intolerance]

45 (41.7%)

Hepatopathy

26 (24.1%)

FEV1 (%)

63.5 [29.0, 114.0]

FVC (%)

80.5 [43.0, 119.0]

BMI (kg/m2)

21.7 [16.2, 32.0]

Infections at baseline

Methicillin-sensible Staphylococcus aureus

63 (58.3%)

Pseudomonas aeruginosa

39 (36.1%)

Methicillin-resistant Staphylococcus aureus

9 (8.3%)

Haemophilus influenzae

6 (5.6%)

Non-tuberculous Mycobacterium

5 (4.6%)

Other

40 (37.0%)

Previous Treatments

Previous Modulator

46 (42.6%)

Ivacaftor

2 (4.3%)

Tezacaftor and Ivacaftor

42 (91.4%)

Lumacaftor and Ivacaftor

2 (4.3%)

Age at Previous Modulator (years)

27.0 [15.0, 45.0]

Use of ETI

Age at ETI start (years)

28.0 [12.0, 59.0]

Side Effects after ETI

6 (5.6%)

Cholecystopancreatitis

1 (16.6%)

Hepatic

2 (33,4%)

Rash

3 (50.0%)

Suspension of ETI

5 (4.6%)

Out of Stock

1 (20.0%)

Side Effect

4 (80.0%)

Transplant Unit Evaluation pre-ETI

6 (5.6%)

Transplant Active List pre-ETI

2 (1.9%)

Questionnaires

GAD7

Normal [score: 0–4]

67 (62.0%)

Mild [score: 5–9]

27 (25.0%)

Moderate [score: 10–14]

7 (6.5%)

Severe [score: 15–21]

5 (4.6%)

PHQ9

Normal [score: 0–4]

67 (62.0%)

Mild [score: 5–9]

25 (23.1%)

Moderate [score: 10–14]

12 (11.1%)

Moderately severe [score: 15–19]

1 (0.9%)

Severe [score: >20]

1 (0.9%)