Table 2 Mean (SD) HUI2, HUI3, and EQ-5D utility values per health state

Early ambulatory

0.89 (0.13)

0.81 (0.22)

0.79 (0.20)

Preserved upper limb, no daytime ventilation, without symptomatic CM

0.92 (0.08)

0.84 (0.20)

0.84 (0.13)

Mildly impaired upper limb, no daytime ventilation, without symptomatic CM

0.57 (NA)

0.48 (NA)

0.30 (NA)

Late ambulatory

0.71 (0.24)

0.64 (0.32)

0.64 (0.30)

Transitional, preserved upper limb, no daytime ventilation, without symptomatic CM

0.64 (0.22)

0.54 (0.31)

0.59 (0.33)

Transitional, mildly impaired upper limb, no daytime ventilation, without symptomatic CM

0.95 (0.07)

0.94 (0.09)

0.79 (0.16)

Early non-ambulatory

0.49 (0.12)

0.22 (0.14)

0.31 (0.13)

Preserved upper limb, no daytime ventilation, without symptomatic CM

0.61 (0.11)

0.16 (0.33)

0.46 (0.10)

Mildly impaired upper limb, no daytime ventilation, without symptomatic CM

0.49 (0.12)

0.21 (0.12)

0.30 (0.14)

Mildly impaired upper limb, no daytime ventilation, with symptomatic CM

0.45 (0.10)

0.27 (0.14)

0.29 (0.07)

Late non-ambulatory

0.47 (0.10)

0.15 (0.15)

0.22 (0.15)

Moderately impaired upper limb, no daytime ventilation, without symptomatic CM

0.49 (0.05)

0.22 (0.11)

0.22 (0.15)

Moderately impaired upper limb, no daytime ventilation, with symptomatic CM

0.52 (0.05)

0.20 (0.02)

0.27 (0.08)

Moderately impaired upper limb, nighttime and daytime ventilation, without symptomatic CM

0.49 (0.07)

0.16 (0.15)

0.25 (0.14)

Loss of upper limb function, no daytime ventilation, without symptomatic CM

0.51 (NA)

0.09 (NA)

0.26 (NA)

Loss of upper limb function, nighttime and daytime ventilation, without symptomatic CM

0.36 (0.01)

0.01 (0.00)

0.26 (0.01)

Loss of upper limb function, nighttime and daytime ventilation, with symptomatic CM

0.32 (0.27)

− 0.15 (0.11)

0.02 (0.34)