Characteristics of Persons in Groups A–D at Baseline, Defined as 2008 for the Pre-Ivacaftor Period and 2012 for the Post-Ivacaftor Period, UK Cystic Fibrosis Registry, 2008–2016
| Variable . | Group A (P = 0, G = 1) (n = 437) . | Group B (P = 1, G = 1) (n = 397) . | Group C (P = 0, G = 0) (n = 6,382) . | Group D (P = 1, G = 0) (n = 7,378) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . |
| Ivacaftor use | 0 | 0 | 397 | 100 | 0 | 0 | 0 | 0 | ||||
| Total no. of postbaseline visits | 3.0 (1.1) | 3.4 (1.1) | 3.0 (1.1) | 3.3 (1.0) | ||||||||
| Age, years | 20.4 (10.8) | 22.4 (11.2) | 20.9 (11.6) | 21.9 (12.6) | ||||||||
| Female sex | 205 | 46.9 | 186 | 46.9 | 2,971 | 46.6 | 3,465 | 47.0 | ||||
| White ethnicity | 428 | 97.9 | 390 | 98.2 | 6,150 | 96.4 | 7,043 | 95.5 | ||||
| ppFEV1 | 71.0 (23.2) | 69.7 (23.2) | 71.6 (23.3) | 72.0 (23.4) | ||||||||
| % predicted forced vital capacitya | 84.8 (19.4) | 84.1 (18.9) | 84.0 (19.5) | 84.4 (19.6) | ||||||||
| % predicted forced midexpiratory flowa | 56.3 (31.3) | 55.9 (32.4) | 60.9 (32.8) | 58.4 (31.0) | ||||||||
| Annual no. of IV antibiotic days | 18.4 (28.1) | 20.2 (30.5) | 17.6 (27.7) | 18.6 (28.3) | ||||||||
| Infectionb | 358 | 81.9 | 350 | 88.2 | 4,847 | 75.9 | 5,948 | 80.6 | ||||
| CF-related diabetes | 69 | 15.8 | 90 | 22.7 | 1,198 | 18.8 | 1,751 | 23.7 | ||||
| Smoking (yes) | 9 | 2.1 | 9 | 2.3 | 154 | 2.4 | 201 | 2.7 | ||||
| Mucolytic treatmentc | 223 | 51.0 | 264 | 66.5 | 2,992 | 46.9 | 4,882 | 65.4 | ||||
| Variable . | Group A (P = 0, G = 1) (n = 437) . | Group B (P = 1, G = 1) (n = 397) . | Group C (P = 0, G = 0) (n = 6,382) . | Group D (P = 1, G = 0) (n = 7,378) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . |
| Ivacaftor use | 0 | 0 | 397 | 100 | 0 | 0 | 0 | 0 | ||||
| Total no. of postbaseline visits | 3.0 (1.1) | 3.4 (1.1) | 3.0 (1.1) | 3.3 (1.0) | ||||||||
| Age, years | 20.4 (10.8) | 22.4 (11.2) | 20.9 (11.6) | 21.9 (12.6) | ||||||||
| Female sex | 205 | 46.9 | 186 | 46.9 | 2,971 | 46.6 | 3,465 | 47.0 | ||||
| White ethnicity | 428 | 97.9 | 390 | 98.2 | 6,150 | 96.4 | 7,043 | 95.5 | ||||
| ppFEV1 | 71.0 (23.2) | 69.7 (23.2) | 71.6 (23.3) | 72.0 (23.4) | ||||||||
| % predicted forced vital capacitya | 84.8 (19.4) | 84.1 (18.9) | 84.0 (19.5) | 84.4 (19.6) | ||||||||
| % predicted forced midexpiratory flowa | 56.3 (31.3) | 55.9 (32.4) | 60.9 (32.8) | 58.4 (31.0) | ||||||||
| Annual no. of IV antibiotic days | 18.4 (28.1) | 20.2 (30.5) | 17.6 (27.7) | 18.6 (28.3) | ||||||||
| Infectionb | 358 | 81.9 | 350 | 88.2 | 4,847 | 75.9 | 5,948 | 80.6 | ||||
| CF-related diabetes | 69 | 15.8 | 90 | 22.7 | 1,198 | 18.8 | 1,751 | 23.7 | ||||
| Smoking (yes) | 9 | 2.1 | 9 | 2.3 | 154 | 2.4 | 201 | 2.7 | ||||
| Mucolytic treatmentc | 223 | 51.0 | 264 | 66.5 | 2,992 | 46.9 | 4,882 | 65.4 | ||||
Abbreviations: CF, cystic fibrosis; IV, intravenous; ppFEV1, percent predicted forced expiratory volume in 1 second; SD, standard deviation.
a Percent predicted forced vital capacity was based on 14,556 participants and percent predicted forced midexpiratory flow was based on 5,711 participants, out of a total of 14,594 individuals , many counted more than once, across the 4 groups.
b Baseline infection included infection with Staphylococcus aureus, Pseudomonas aeruginosa, Aspergillus fumigatus, methicillin-resistant S. aureus (MRSA), influenza, Stenotrophomonas maltophilia, and Burkholderia cepacia complex.
c Baseline mucolytic treatment includes acetylcysteine, dornase alfa, hypertonic saline, and mannitol.
Characteristics of Persons in Groups A–D at Baseline, Defined as 2008 for the Pre-Ivacaftor Period and 2012 for the Post-Ivacaftor Period, UK Cystic Fibrosis Registry, 2008–2016
| Variable . | Group A (P = 0, G = 1) (n = 437) . | Group B (P = 1, G = 1) (n = 397) . | Group C (P = 0, G = 0) (n = 6,382) . | Group D (P = 1, G = 0) (n = 7,378) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . |
| Ivacaftor use | 0 | 0 | 397 | 100 | 0 | 0 | 0 | 0 | ||||
| Total no. of postbaseline visits | 3.0 (1.1) | 3.4 (1.1) | 3.0 (1.1) | 3.3 (1.0) | ||||||||
| Age, years | 20.4 (10.8) | 22.4 (11.2) | 20.9 (11.6) | 21.9 (12.6) | ||||||||
| Female sex | 205 | 46.9 | 186 | 46.9 | 2,971 | 46.6 | 3,465 | 47.0 | ||||
| White ethnicity | 428 | 97.9 | 390 | 98.2 | 6,150 | 96.4 | 7,043 | 95.5 | ||||
| ppFEV1 | 71.0 (23.2) | 69.7 (23.2) | 71.6 (23.3) | 72.0 (23.4) | ||||||||
| % predicted forced vital capacitya | 84.8 (19.4) | 84.1 (18.9) | 84.0 (19.5) | 84.4 (19.6) | ||||||||
| % predicted forced midexpiratory flowa | 56.3 (31.3) | 55.9 (32.4) | 60.9 (32.8) | 58.4 (31.0) | ||||||||
| Annual no. of IV antibiotic days | 18.4 (28.1) | 20.2 (30.5) | 17.6 (27.7) | 18.6 (28.3) | ||||||||
| Infectionb | 358 | 81.9 | 350 | 88.2 | 4,847 | 75.9 | 5,948 | 80.6 | ||||
| CF-related diabetes | 69 | 15.8 | 90 | 22.7 | 1,198 | 18.8 | 1,751 | 23.7 | ||||
| Smoking (yes) | 9 | 2.1 | 9 | 2.3 | 154 | 2.4 | 201 | 2.7 | ||||
| Mucolytic treatmentc | 223 | 51.0 | 264 | 66.5 | 2,992 | 46.9 | 4,882 | 65.4 | ||||
| Variable . | Group A (P = 0, G = 1) (n = 437) . | Group B (P = 1, G = 1) (n = 397) . | Group C (P = 0, G = 0) (n = 6,382) . | Group D (P = 1, G = 0) (n = 7,378) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . | No. . | % . | Mean (SD) . |
| Ivacaftor use | 0 | 0 | 397 | 100 | 0 | 0 | 0 | 0 | ||||
| Total no. of postbaseline visits | 3.0 (1.1) | 3.4 (1.1) | 3.0 (1.1) | 3.3 (1.0) | ||||||||
| Age, years | 20.4 (10.8) | 22.4 (11.2) | 20.9 (11.6) | 21.9 (12.6) | ||||||||
| Female sex | 205 | 46.9 | 186 | 46.9 | 2,971 | 46.6 | 3,465 | 47.0 | ||||
| White ethnicity | 428 | 97.9 | 390 | 98.2 | 6,150 | 96.4 | 7,043 | 95.5 | ||||
| ppFEV1 | 71.0 (23.2) | 69.7 (23.2) | 71.6 (23.3) | 72.0 (23.4) | ||||||||
| % predicted forced vital capacitya | 84.8 (19.4) | 84.1 (18.9) | 84.0 (19.5) | 84.4 (19.6) | ||||||||
| % predicted forced midexpiratory flowa | 56.3 (31.3) | 55.9 (32.4) | 60.9 (32.8) | 58.4 (31.0) | ||||||||
| Annual no. of IV antibiotic days | 18.4 (28.1) | 20.2 (30.5) | 17.6 (27.7) | 18.6 (28.3) | ||||||||
| Infectionb | 358 | 81.9 | 350 | 88.2 | 4,847 | 75.9 | 5,948 | 80.6 | ||||
| CF-related diabetes | 69 | 15.8 | 90 | 22.7 | 1,198 | 18.8 | 1,751 | 23.7 | ||||
| Smoking (yes) | 9 | 2.1 | 9 | 2.3 | 154 | 2.4 | 201 | 2.7 | ||||
| Mucolytic treatmentc | 223 | 51.0 | 264 | 66.5 | 2,992 | 46.9 | 4,882 | 65.4 | ||||
Abbreviations: CF, cystic fibrosis; IV, intravenous; ppFEV1, percent predicted forced expiratory volume in 1 second; SD, standard deviation.
a Percent predicted forced vital capacity was based on 14,556 participants and percent predicted forced midexpiratory flow was based on 5,711 participants, out of a total of 14,594 individuals , many counted more than once, across the 4 groups.
b Baseline infection included infection with Staphylococcus aureus, Pseudomonas aeruginosa, Aspergillus fumigatus, methicillin-resistant S. aureus (MRSA), influenza, Stenotrophomonas maltophilia, and Burkholderia cepacia complex.
c Baseline mucolytic treatment includes acetylcysteine, dornase alfa, hypertonic saline, and mannitol.
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