TABLE 1

Clinical and demographic characteristics of the Canadian and UK cystic fibrosis (CF) populations

Canada 1982–2015UK 2008–2013Canada 2008–2013#
Patients499374503342
Annual records56 83927 01415 226
Sex
 Female2365 (47.4)3472 (46.6)1569 (46.9)
 Male2628 (52.6)3978 (53.4)1773 (53.1)
Age at diagnosis years0.7 (0–73.7)0.422 (0–79.2)0.7 (0–73.7)
 <2 yrs3186 (63.8)5222 (70.1)2131 (63.8)
 ≥2 yrs1807 (36.2)2228 (29.9)1211 (36.2)
Birth cohort
 <1970989 (19.8)580 (7.8)400 (12.0)
 1970–19791059 (21.2)887 (11.9)484 (14.5)
 1980–19891260 (25.2)1966 (26.4)930 (27.8)
 1990–19991124 (22.5)2535 (34.0)1063 (31.8)
 ≥2000561 (11.2)1482 (19.9)465 (13.9)
Pancreatic status (ever/never)
 Sufficient814 (16.3)941 (12.6)437 (13.1)
 Insufficient4179 (83.7)6509 (87.4)2905 (86.9)
CF-related diabetes (ever/never)
 No3647 (73.0)4176 (56.1)2464 (73.7)
 Yes1346 (27.0)3274 (43.9)878 (26.3)
FEV1 % pred55.8 (8.7–138.3)69.5 (9.2–138.9)70.0 (10.6–136.7)
 <401786 (35.8)1361 (18.3)621 (18.6)
 40–691307 (26.2)2412 (32.4)1048 (31.4)
 70–89991 (19.8)2111 (28.3)871 (26.1)
 ≥90909 (18.2)1566 (21.0)802 (24.0)
FVC % pred76.4 (13.7–147.4)83.8 (12.1–146.7)86.9 (16.7–149.7)
Hospitalisations in prior year0 (0–14)0 (0–19.3)0 (0–14)
 02942 (58.9)4161 (55.9)2324 (69.5)
 1–21338 (26.8)2282 (30.6)759 (22.7)
 ≥3713 (14.3)1007 (13.5)259 (7.7)
Nutritional status
 Underweight1114 (22.3)1002 (13.4)438 (13.1)
 Normal3184 (63.8)5134 (68.9)2348 (70.3)
 Overweight695 (13.9)1314 (17.6)556 (16.6)
Microbiology
 Neither P. aeruginosa nor BCC1901 (38.1)3373 (45.3)1364 (40.8)
P. aeruginosa, no BCC2168 (43.4)3684 (49.4)1563 (46.8)
 BCC924 (18.5)393 (5.3)415 (12.4)
Transplant: yes589 (11.8)220 (3.0)197 (5.9)
Vital status: dead893 (17.9)355 (4.8)142 (4.2)

Data are presented as n, n (%) or median (range). FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; P. aeruginosa: Pseudomonas aeruginosa; BCC: Burkholderia cepacia complex. Observations on clinical variables for the last clinic visit, unless otherwise stated. #: the threshold regression model was developed using the full Canadian dataset from 1982 to 2015. The UK validation dataset had data from 2008 to 2013, so a subset of the Canadian data for the same time period was used for comparison. : UK hospitalisations are derived by dividing total days in hospital by 14 (2 weeks).