Table 3.
Comparison of quality of life of Brazilian patients with Gaucher and Fabry diseases and a group of Brazilian elderly according to the SF-36a
| Gaucher disease receiving ERT (n = 15) | Gaucher disease not receiving ERT (n = 6) | Fabry disease receiving ERT (n = 10) | Elderly (Lima et al. 2005) (n = 1.958)b |
|
|---|---|---|---|---|
| Mean age (years) | 22.3 ±13.0 | 31.5 ±13.1 | 33.5 ± 12.9 | 60 ± 69.6 |
| SF-36 | ||||
| Physical component | ||||
| Physical functioning | 76.3 ± 26.0b | 59.1 ± 38.3a,b | 66.0 ± 26.5 | 71.4 |
| Role-physical | 70.0 ± 42.4b | 41.6 ± 46.5a,b | 45.0 ± 44.7a | 81.2 |
| Bodily pain | 76.5 ± 27.3b,c | 47.8 ± 22.7a,b,c | 59.7 ± 28.5 | 74.2 |
| General health | 71.1 ± 21.6b,d | 62.5 ± 19.1a,b | 50.3 ± 26.8a,d | 70.1 |
| Mental component | ||||
| Vitality | 67.6 ± 20.6b | 56.6 ± 16.3b | 63.5 ± 21.6 | 64.4 |
| Social functioning | 70.0 ± 20.9a,b | 55.2 ± 31.2a,b | 65.0 ± 25.4a | 86.1 |
| Role-emotional | 68.8 ± 42.6b | 38.8 ± 49.0a,b | 66.6 ± 41.3a | 85.9 |
| Mental health | 77.3 ± 9.7a | 72.0 ± 11.8 | 64.4 ± 25.3 | 69.9 |
*Data described as means and standard deviations for SF-36 components and domains
**Standard deviations of SF-36 scores not reported in original study
aClinically significant differences in comparison with group of elderly patients
bClinically significant differences between the groups of patients with GD receiving or not receiving ERT
cp < 0.05 (GD receiving ERT x GD not receiving ERT)
dp < 0.05 (GD receiving ERT × FD receiving ERT)