Skip to main content

Table 1 Demographic and clinical characteristics

From: Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study

Characteristic Overall
(N = 209)
G551D/IVA patient group
(n = 72)
F508del/SOC patient group
(n = 137)
P valuea
Age, mean (SD), years 24.3 (12.1) 23.9 (13.9) 24.6 (11.1) 0.70
 Range 6–62 6–62 6–52  
 Missing, n 6 3 3  
Age group (years), n (%)
 6–11 33 (15.8) 12 (16.7) 21 (15.3)  
 12–17 39 (18.7) 18 (25.0) 21 (15.3) 0.19
 ≥18 137 (65.6) 42 (58.3) 95 (69.3)  
Sex, n (%)b
 Male 108 (56.0) 27 (39.7) 81 (64.8) < 0.01
 Female 85 (44.0) 41 (60.3) 44 (35.2)  
 Missing, n 16 3 12  
ppFEV1 (%), mean (SD) 73.9c (28.0) 79.8 (25.6) 70.7d (28.8) < 0.05
 ppFEV1 <70, n (%) 85 (40.7)e 24 (33.3) 61 (44.5)e 0.12
ppFEV1 (%), n; mean (SD)
 <40 32; 29.3 (6.6) 6; 28.8 (4.5) 26; 29.3 (7.0) 0.87
 40–69 51; 54.5 (8.7) 18; 57.4 (7.9) 33; 52.9 (8.7) 0.07
 70–89 50; 79.2 (6.0) 16; 78.6 (6.6) 33; 79.5 (5.7) 0.60
 ≥90 74; 103.0 (10.5) 31; 103.4 (9.7) 43; 102.6 (11.1) 0.74
Number of comorbidities, mean (SD) 1.9f (1.2) 1.5g (1.1) 2.0 (1.2) < 0.01
 Patients with ≥1 comorbidity, n (%) 192 (92.3) 60 (84.5) 132 (96.4) < 0.01
History of lung transplant, n (%)
 Yes 3f (1.4) 0g (0.0) 3 (2.2) 0.55
 No 205f (98.6) 71g (100.0) 134 (97.8)
Waiting for lung transplant, n (%)
 Yes 3f (1.4) 0g (0.0) 3 (2.2) 0.55
 No 205f (98.6) 71g (100.0) 134 (97.8)
BMI
 Aged ≥19 years, n; mean (SD), kg/m2 126; 21.6 (3.3) 36; 22.2 (3.2) 90; 21.3 (3.3) 0.16
 Aged <19 years, n; mean (SD) z-score 59; 0.001 (0.87) 25; 0.004 (0.82) 34; −0.0009 (0.92) 0.98
  1. BMI Body mass index, IVA Ivacaftor, ppFEV1 Percent predicted forced expiratory volume in 1 s, SD Standard deviation, SOC Standard of care
  2. aP values presented are for comparison of the G551D/IVA group with the F508del/SOC group
  3. bPercentages are derived from the available sample
  4. cn=207
  5. dn=135
  6. eBaseline data was missing for 2 patients
  7. fn=208
  8. gn=71