Patient Characteristics | MWCFC, n = 114 | CFFPR 2014 n = 14,394 | p | |
---|---|---|---|---|
Enrollment | Annualized for 2014 | |||
Male sex, fraction | 0.46 | 0.46 | 0.48 | 0.79 |
Age, Years, mean (SD) | 28 (12) | 27 (12) | 27 (12) | 0.71 |
FEV1, Percent Predicted, mean (SD) | 70 (22) | 80 (23) | 77 (27) | 0.094 |
Height, cm, mean (SD) | 167 (9.95) | 167 (10.2) | 166 (10) | 0.39 |
Weight-for-age z-score, mean (SD) | −0.17 (0.98) | −0.20 (0.93) | −0.29 (1.1) | 0.34 |
Pulmonary exacerbations in year prior to enrollment, median (range) | 1 (0–7) | 1 (0–6) | 1 (0–14) | 0.55 |
Pulmonary exacerbations in year prior to enrollment, mean (SD) | 1.7 (1.7) | 1 (1.2) | 1.1 (1.5) | > 0.99 |
Patients with no pulmonary exacerbations, n (fraction affected) | 79 (0.69) | 63 (0.56) | 7054 (0.52) | 0.46 |
Diabetes, n (fraction affected) | 25 (0.22) | 26 (0.23) | 4423 (0.31) | 0.073 |
Pancreatic Sufficiency, n (fraction affected) | 9 (0.079) | 19 (0.17) | 2239 (0.16) | 0.84 |
CF related arthropathy, n (fraction affected) | 8 (0.07) | 10 (0.088) | 697 (0.048) | 0.085 |
5-Year Predicted Survival, median (range) | 0.959 (0.094 to > 0.999) | 0.973 (0.464–0.999) | 0.967 (0.0949–0.999) | 0.17 |
Home Altitude, m, mean (SD) | 1305 (442) | – | – | – |
Infections Present, n (fraction affected) | ||||
 Methicillin Sensitive S aureus | 51 (0.45) | 67 (0.59) | 7937 (0.55) | 0.5 |
 Methicillin Resistant S aureus | 21 (0.18) | 33 (0.29) | 5001 (0.35) | 0.23 |
 P aeruginosa | 70 (0.61) | 84 (0.74) | 10,096 (0.70) | 0.47 |
 B cepacia complex | 3 (0.026) | 5 (0.044) | 782 (0.054) | 0.78 |
 S maltophilia | 7 (0.061) | 26 (0.23) | 2970 (0.21) | 0.65 |
 Achromobacter spp | 5 (0.044) | 11 (0.096) | 1579 (0.11) | 0.76 |
 Candida spp | 17 (0.15) | 17 (0.15) | 3100 (0.22) | 0.11 |
 Aspergillus | 12 (0.11) | 26 (0.23) | 3091 (0.21) | 0.82 |
 Mycobacterium avium-intracellulare complexa | 1 (0.0088) | 8 (0.082) | 730 (0.069) | 0.74 |
 Mycobacterium abscessusa | 3 (0.027) | 5 (0.052) | 527 (0.05) | > 0.99 |
Treatments in use, n (fraction affected) | ||||
 Any Form of Inhaled Tobramycin | 38 (0.33) | 71 (0.62) | 8069 (0.58) | 0.38 |
 Inhaled Aztreonam | 40 (0.35) | 47 (0.41) | 4977 (0.36) | 0.25 |
 Oral Azithromycin | 62 (0.54) | 67 (0.59) | 8595 (0.60) | 0.91 |
 Inhaled Hypertonic Saline | 71 (0.62) | 82 (0.72) | 9611 (0.69) | 0.54 |
 Inhaled DNase | 101 (0.89) | 106 (0.93) | 12,168 (0.87) | 0.087 |