Table 1 Demographic and clinical variables of the study population, 2005–2020.

From: Survival of people with cystic fibrosis in Australia

 

Survived, N (%)

Died, N (%)

Total, N (%)

p

 

4,085 (88.8%)

516 (11.2%)

4,601 (100%)

 

Sex

   

0.8401

Female

1,943 (47.6%)

243 (47.1%)

2,186 (47.5%)

 

Male

2,142 (52.4%)

273 (52.9%)

2,415 (52.5%)

 

Diagnosis category

   

< 0.0011

Diagnosed with meconium ileus

480 (11.8%)

48 (9.3%)

528 (11.5%)

 

Age < 6 weeks or through NBS/Prenatal screening

2,747 (67.2%)

296 (57.4%)

3,043 (66.1%)

 

Age 6 weeks–< 2 years

376 (9.2%)

97 (18.8%)

473 (10.3%)

 

Age 2 – 17 years

325 (8.0%)

46 (8.9%)

371 (8.1%)

 

Age ≥ 18 years

157 (3.8%)

29 (5.6%)

186 (4.0%)

 

F508del homozygous

   

0.8641

No

2,223 (54.4%)

275 (54.1%)

2,498 (54.4%)

 

Yes

1860 (45.6%)

233 (45.9%)

2,093 (45.6%)

 

Lung transplant

   

< 0.0012

No

3,885 (95.1%)

321 (62.2%)

4,206 (91.4%)

 

Yes

200 (4.9%)

195 (37.8%)

395 (8.6%)

 

Pancreatic exocrine status

   

< 0.4361

Insufficient

2,836 (69.4%)

368 (71.3%)

3,204 (69.6%)

 

Sufficient

972 (23.8%)

110 (21.3%)

1,082 (23.5%)

 

Missing

277 (6.8%)

38 (7.4%)

315 (6.8%)

 

Mean FEV1pp across the lifetime

   

< 0.0012

Not assessed, age < 6 years3

516 (12.6%)

3 (0.6%)

519 (11.3%)

 

Mean (SD)

83.1 (21.0)

55.7 (19.7)

79.7 (22.7)

 

Range

17.0–130.0

10.0–111.6

10.0–130.0

 

Missing

138 (3.9%)

27 (0.6%)

165 (3.6%)

 

Last recorded BMI4

   

< 0.0011

Underweight

182 (4.5%)

134 (26.0%)

316 (6.9%)

 

Adequate weight status

2,670 (65.4%)

312 (60.5%)

2,982 (64.8%)

 

Overweight

1,076 (26.3%)

47 (9.1%)

1,123 (24.4%)

 

Missing

157 (3.8%)

23 (4.5%)

180 (3.9%)

 

Mean last recorded BMI

    

Adults (N)

2,359

462

2,821

< 0.0012

BMI, Mean (SD)

23.5 (4.9)

20.6 (3.5)

23.0 (4.8)

 

Range

13.9–167.5

12.5–35.8

12.5–157.5

 

Missing

89

21

110

 

Children (N)

1,726

54

1,780

< 0.0012

BMI percentile, Mean (SD)

0.6 (0.3)

0.4 (0.3)

0.6 (0.3)

 

Range

0.0–1.0

0.0–1.0

0.0–1.0

 

Missing

68

2

70

 
  1. BMI body mass index, FEV1pp percent predicted forced expiratory volume in one second.
  2. 1 Pearson’s Chi-squared test, 2 Linear Model ANOVA, 3 Lung function not captured for those < 6 years of age, 4 Adult individuals (≥ 18 years) were classified into BMI categories based on World Health Organization guidelines as underweight (< 18.5 kg/m2), adequate weight (18.5–24.9 kg/m2), or overweight (≥ 25.0 kg/m2). Children were classified as underweight (< 10th percentile), adequate weight (10–85th percentile), or overweight (> 85th percentile).
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