Objectives:
To determine the validity of traditional TUG and newly developed FSHD-TUG (modified TUG for FSHD, which includes supine-to-sit and sit-to-supine components) over a one-year period.
Background:
FSHD is characterized by progressive weakness and accumulation of physical disability. Mobility issues rank highly among patients. FSHD patients suffer from significant upper extremity and trunk dysfunction. TUG is an objective assessment of mobility; however, it is relatively insensitive to weakness outside the legs. A Survey of FSHD patients found getting up from a supine position to be one of the most challenging tasks in daily life. We hypothesize that a TUG incorporating a supine-to-sit and sit-to-supine components will provide a more appropriate measure of functional mobility for the FSHD community. We also anticipate the FSHD-TUG correlates to the Clinical Severity Scale (CSS) and the PROMIS-PF, a self-reported measure of physical function.
Methods:
Twenty-two FSHD and twenty healthy volunteers (HV) subjects were enrolled. Each group performed the traditional TUG and FSHD-TUG at two initial visits separated by 14 days, with FSHD subjects having an additional 3 visits every 3 months up to 12 months. Pearson correlation coefficient was used to measure the relationship between TUG and FSHD-TUG, CSS and TUG, CSS and FSHD-TUG, and PROMIS-PF and FSHD-TUG.
Results:
On average, FSHD subjects took approximately twice the time to complete TUG, FSHD TUG, and components of the FSHD TUG as HV. Strong correlations were observed between the TUG and FSHD-TUG at all visits (r≤0.95, p≤0.01). There was moderate correlation between CSS, TUG (r=0.57 to 0.59, p≤0.01) and FSHD-TUG (r=0.58 to 0.67, p≤0.01). There was also a moderate negative correlation between PROMIS-PF, TUG (r=-0.56 to -0.69, p≤0.01), and FSHD-TUG (r=-0.59 to -0.72, p≤0.01).
Conclusions:
These results support the validity of the FSHD-TUG as a clinical outcome assessment of mobility for ongoing and future FSHD clinical trials.