Demographics and baseline characteristics
| Study participants (N=123) | ||
|---|---|---|
| N | Per cent | |
| Sex, female | 74 | 60.2 |
| Race | ||
| Caucasian | 114 | 92.7 |
| Hispanic | 3 | 2.4 |
| African–American | 4 | 3.3 |
| Asian | 1 | 0.8 |
| Pacific Islander | 1 | 0.8 |
| Genotype | ||
| Delta F508 homozygous | 67 | 54.5 |
| Delta F508 heterozygous | 45 | 36.6 |
| Other/unknown/not genotyped | 11 | 8.9 |
| Pancreatic insufficient* | 117 | 95.1 |
| Age group (years) | ||
| 10–17 | 43 | 35.0 |
| ≥18 | 80 | 65.0 |
| FEV1% predicted† | ||
| <50 | 49 | 39.8 |
| 50–69 | 34 | 27.6 |
| 70–89 | 26 | 21.1 |
| ≥90 | 7 | 5.7 |
| FEV1 drop ≥10% predicted presenting for PEx‡ | 66 | 53.7 |
| Intravenous antibiotic for PEx in 6 months prior§ | 51 | 41.5 |
| Microbiology¶ | ||
| Pseudomonas aeruginosa | 79 | 64.2 |
| MSSA | 34 | 27.6 |
| MRSA | 40 | 32.5 |
| Burkolderia cepacia | 4 | 3.3 |
| Stenotrophomonas maltophilia | 15 | 12.2 |
| Achromobacter xylosoxidans | 11 | 8.9 |
| Haemophilus influenza | 4 | 3.3 |
| Aspergillus | 9 | 7.3 |
| CF-related diabetes | 37 | 30.1 |
| Mean | SD | |
| Age (years) | 23.1 | 10.2 |
| Body mass index (kg/m2) | 19.9 | 3.4 |
| FEV1% predicted at enrolment† | 55.8 | 20.9 |
| FEV1% best in 6 months prior to visit 1** | 63.7 | 23.9 |
*Evidence of pancreatic enzyme use.
†Spirometry % predicted is calculated using the Wang equations24 for females <16 years of age and males <18 years of age. The Hankinson equations23 are used for females 16 years and older, males 18 years and older.
‡As reported as criterion on pulmonary exacerbation diagnostic form.
§One or more episodes of intravenous antibiotic for reason ‘pulmonary exacerbation’ recorded in the CFF Registry in 6 months prior to study PEx.
¶Among respiratory cultures isolated at time of enrolment (n=107) or in CFF registry in 6 months prior to enrolment (n=16).
**Best of all spirometry in CFF Registry in 6 months prior to study PEx.
CF, cystic fibrosis; CFF, CF Foundation; MRSA, methicillin-resistant Staphylococcus aureus; MSSA, methicillin-sensitive Staphylococcus aureus; PEx, pulmonary exacerbation.