Skip to main content

Table 2 SF-36 quality of life scores before and during ERT

From: Quality of life and participation in daily life of adults with Pompe disease receiving enzyme replacement therapy: 10 years of international follow-up

Clinical outcome measure Follow-up time intervals (in years)
SF-36 Component summary scores Pre-ERT
Mean sp/y (95 % CI)
0 to 2 years during ERT
Mean sp/y (95 % CI)
>2 years during ERT
Mean sp/y (95 % CI)
 Physical component summary -0.73 (-1.07; -0.39)** 1.49 (0.76; 2.21)** -0.15 (-0.43; 0.13)
 Mental component summary 0.16 (-0.25; 0.57) 1.03 (-0.07; 2.13) 0.02 (-0.41; 0.46)
SF-36 domain scores
 Physical functioning -1.80 (-2.41; -1.19)** 1.81 (0.38; 3.23)* 0.68 (0.12; 1.25)*
 Role physical -1.20 (-2.92; 0.52) 9.18 (5.83; 12.53)** 2.46 (1.15; 3.77)**
 Bodily pain -2.10 (-3.01; -1.19) ** 0.76 (-1.39; 2.92) -1.96 (-2.95; -0.98)**
 General health 0.19 (-0.49; 0.87) 5.22 (3.58; 6.86)** 0.81 (0.13; 1.49)*
 Vitality -0.44 (-1.11; 0.24) 4.10 (2.44; 5.76)** 1.43 (0.77; 2.09)**
 Social functioning -0.74 (-1.66; 0.18) 2.11 (-0.16; 4.39) 0.41 (-0.54; 1.35)
 Role emotional -0.15 (-1.64; 1.34) 3.05 (-1.06; 7.16) 0.19 (-1.15; 1.53)
 Mental health 0.13 (-0.60; 0.87) 2.09 (0.57; 3.61)** 0.29 (-0.37; 0.95)
Rotterdam handicap scale score Pre-ERT
Mean sp/y (95 % CI)
During ERT
Mean sp/y (95 % CI)
  -0.49 (-0.64;-0.34)** -0.02 (-0.17; 0.13)
  1. Data shown are mean changes in score points per year (sp/y) as calculated by univariate analysis using mixed model ANOVA
  2. CI confidence interval
  3. *Change is significant at the 0.05 level, **Change is significant at the 0.01 level