Table 8. CHESS PAEDs quality-of-life measures evaluated by disease severity and treatment approach.
| Severity | Total ( N = 691) |
||||
|---|---|---|---|---|---|
| Moderate ( n = 282) |
Severe ( n = 409) |
||||
| Prophylaxis ( n = 136) |
Other ( n = 146) |
Prophylaxis ( n = 332) |
Other ( n = 77) |
||
| EQ-5D score for children > 8 y | |||||
| n | 17 | 10 | 76 | 18 | 121 |
| Mean (SD) | 0.70 (0.23) | 0.50 (0.53) | 0.65 (0.25) | 0.69 (0.17) | 0.65 (0.27) |
| Median (IQR) | 0.71 (0.27) | 0.70 (0.66) | 0.65 (0.29) | 0.70 (0.22) | 0.68 (0.27) |
| Had to reduce or give up social activities, n (%) | n = 28 | n = 17 | n = 100 | n = 28 | n = 173 |
| Strongly agree | 4 (14.3) | 1 (5.9) | 5 (5.0) | 2 (7.1) | 12 (6.9) |
| Agree | 8 (28.6) | 4 (23.5) | 17 (17.0) | 4 (14.3) | 33 (19.1) |
| Neither agree nor disagree | 5 (17.9) | 2 (11.8) | 21 (21.0) | 7 (25.0) | 35 (20.2) |
| Disagree | 9 (32.1) | 8 (47.1) | 50 (50.0) | 14 (50.0) | 81 (46.8) |
| Strongly disagree | 2 (7.1) | 2 (11.8) | 7 (7.0) | 1 (3.6) | 12 (6.9) |
| Had to reduce or give up exercise, n (%) |
n = 28 | n = 18 | n = 100 | n = 28 | n = 174 |
| Strongly agree | 2 (7.1) | 0 (0.0) | 4 (4.0) | 2 (7.1) | 8 (4.6) |
| Agree | 7 (25.0) | 2 (11.1) | 5 (5.0) | 8 (28.6) | 22 (12.6) |
| Neither agree nor disagree | 5 (17.9) | 7 (38.9) | 29 (29.0) | 7 (25.0) | 48 (27.6) |
| Disagree | 11 (39.3) | 8 (44.4) | 46 (46.0) | 9 (32.1) | 74 (42.5) |
| Strongly disagree | 3 (10.7) | 1 (5.6) | 16 (16.0) | 2 (7.1) | 22 (12.6) |
| Miss out on opportunities, n (%) | n = 28 | n = 18 | n = 100 | n = 28 | n = 174 |
| Strongly agree | 2 (7.1) | 0 (0) | 3 (3.0) | 2 (7.1) | 7 (4.0) |
| Agree | 5 (17.9) | 5 (27.8) | 10 (10.0) | 6 (21.4) | 26 (14.9) |
| Neither agree nor disagree | 12 (42.9) | 4 (22.2) | 32 (32.0) | 11 (39.3) | 59 (33.9) |
| Disagree | 7 (25.0) | 8 (44.4) | 49 (49.0) | 9 (32.1) | 73 (42.0) |
| Strongly disagree | 2 (7.1) | 1 (5.6) | 6 (6.0) | 0 (0) | 9 (5.2) |
| Feel frustrated by the influence on lifestyle, n (%) | n = 28 | n = 18 | n = 100 | n = 28 | n = 174 |
| Strongly agree | 2 (7.1) | 0 (0) | 2 (2.0) | 0 (0) | 4 (2.3) |
| Agree | 5 (17.9) | 4 (22.2) | 12 (12.0) | 4 (14.3) | 25 (14.4) |
| Neither agree nor disagree | 11 (39.3) | 8 (44.4) | 30 (30.0) | 11 (39.3) | 60 (34.5) |
| Disagree | 8 (28.6) | 5 (27.8) | 48 (48.0) | 13 (46.4) | 74 (42.5) |
| Strongly disagree | 2 (7.1) | 1 (5.6) | 8 (8.0) | 0 (0) | 11 (6.3) |
| Parent/guardian providing care for a pediatric relative with hemophilia, n (%) | n = 30 | n = 18 | n = 97 | n = 26 | n = 171 |
| Yes | 18 (60.0) | 12 (66.7) | 77 (79.4) | 13 (50.0) | 120 (70.2) |
| No | 12 (40.0) | 6 (33.3) | 20 (20.6) | 13 (50.0) | 51 (29.8) |
| Hours spent caring in a week | |||||
| n | 17 | 11 | 77 | 13 | 118 |
| Mean (SD) | 24.88 (20.92) | 40.45 (34.08) | 19.70 (24.61) | 12.00 (10.26) | 21.54 (24.72) |
| Median (IQR) | 15.0 (20.0) | 40.0 (71.0) | 12.0 (16.0) | 8.0 (4.0) | 12 (18) |
| Caregiver duties prevent you from working/working more hours, n (%) | n = 12 | n = 10 | n = 60 | n = 11 | n = 93 |
| Yes | 1 (5.6) | 3 (25.0) | 9 (11.7) | 7 (53.8) | 20 (21.5) |
| No | 6 (50.0) | 6 (60.0) | 44 (73.3) | 4 (36.4) | 60 (64.5) |
| Don't know | 5 (41.7) | 1 (10.0) | 7 (11.7) | 0 (0) | 13 (14.0) |
Abbreviations: IQR, interquartile range; SD, standard deviation.