Table 3.
Demographic and clinical characteristics by disease course (non-transplant cohort, N=1315).
| Hospitalisation with supplemental oxygen | Hospitalisation | Intensive care unit | ||||
|---|---|---|---|---|---|---|
| N=1243 | N=1266 | N=1290 | ||||
| n/N | % | n/N** | % | n/N | % | |
| Sex; n (%) | ||||||
| Male | 51/619 | 8.2 | 115/632 | 18.2 | 16/645 | 2.5 |
| Female | 47/624 | 7.5 | 121/634 | 19.1 | 11/645 | 1.7 |
| Age; n (%) | ||||||
| <18 | 17/394 | 4.3 | 62/401 | 15.5 | <6/408 | - |
| 18-39 | 54/680 | 7.9 | 127/696 | 18.2 | 12/706 | 1.7 |
| ≥40 | 27/169 | 16.0 | 47/169 | 27.8 | 10/176 | 5.7 |
| Best FEV1; n (%) | ||||||
| <40% | 32/81 | 39.5 | 51/83 | 61.4 | 9/87 | 10.3 |
| 40-70% | 30/271 | 11.1 | 68/275 | 24.7 | 8/280 | 2.9 |
| >70% | 19/591 | 3.2 | 70/599 | 11.7 | 6/620 | 1 |
| BMI category; n (%) | ||||||
| Underweight | 23/104 | 22.1 | 42/105 | 40.0 | <6/108 | - |
| Normal | 52/624 | 8.3 | 126/637 | 19.8 | 14/657 | 2.1 |
| Overweight | 15/264 | 5.7 | 42/269 | 15.6 | 6/275 | 2.2 |
| Race; n (%) | ||||||
| White | 58/951 | 6.1 | 153/972 | 15.7 | 21/951 | 2.2 |
| Non-white | 14/81 | 17.3 | 26/82 | 31.7 | <6/81 | - |
| Black | 6/26 | 23.1 | 10/27 | 37.0 | <6/26 | - |
| Asian | 0/17 | 0.0 | 4/17 | 23.5 | 0/17 | 0 |
| Any other race | 8/38 | 21.1 | 12/38 | 31.6 | <6/38 | - |
| Genotype; n (%) | ||||||
| Heterozygous F508del | 41/516 | 7.9 | 90/527 | 17.1 | 10/553 | 1.8 |
| Homozygous F508del | 33/500 | 6.6 | 91/510 | 17.8 | 12/500 | 2.4 |
| Other | 24/225 | 10.7 | 55/227 | 24.2 | <6/235 | - |
| CF-related diabetes; n (%) | ||||||
| No | 60/945 | 6.3 | 152/959 | 15.8 | 15/955 | 1.6 |
| Yes | 34/264 | 12.9 | 75/268 | 28.0 | 11/264 | 4.2 |
| P. aeruginosa infection; n (%) | ||||||
| No | 32/636 | 5.0 | 88/643 | 13.7 | 11/656 | 1.7 |
| Yes | 64/565 | 11.3 | 141/576 | 24.5 | 15/587 | 2.6 |
| Pancreatic insufficiency; n (%) | ||||||
| No | 11/225 | 4.9 | 26/227 | 11.5 | 3/243 | 1.2 |
| Yes | 87/989 | 8.8 | 204/1005 | 20.3 | 24/1018 | 2.4 |
| CFTR modulators; n (%) | ||||||
| Any CFTR modulator | 42/713 | 5.9 | 105/726 | 14.5 | 14/725 | 1.9 |
| Iva or Elex/Tez/Iva | 27/528 | 5.1 | 68/539 | 12.6 | 11/533 | 2.1 |
| Lum/Iva or Tez/Iva | 15/185 | 8.1 | 37/187 | 19.8 | 3/192 | 1.6 |
| No modulator | 55/520 | 10.6 | 124/527 | 23.5 | 13/555 | 2.3 |
| Time of diagnosis; n (%) | ||||||
| Feb-May | 30/157 | 19.1 | 69/161 | 42.9 | 9/157 | 5.7 |
| June-Sept | 27/340 | 7.9 | 60/348 | 17.2 | 9/343 | 2.6 |
| Oct-Dec | 41/746 | 5.5 | 107/757 | 14.1 | 9/790 | 1.1 |
Column row totals represent non missing data for the column outcome. n=number admitted N=total number reported in the cohort. Row proportions are calculated from the total non-missing in each outcome (See Table 1 for missing data). CFTR=cystic fibrosis transmembrane conductance regulator. FEV1=forced expiratory volume in 1 second, BMI=body mass index. Iva=ivacaftor, elex=elexacaftor, tez=tezacaftor.