Table 1.

FVCs and PT outcomes.

Age (years)	FVC % Predicted	6MWT (Meters)	6MWT % predicted	GSGC (total)	QMFT	GMFM (Standing) %	GMFM (Walk/Run/Jump) %
8.1		368.5	49.5	12		84.62	87.5
9.6		401.2	54.7	10		89.74	81.94
10.2		458.0	59.7	10		87.18	94.44
12.1	87	502.9	68.2
12.4	87
13.7	74
13.9	77	511.8	62.6
14.1		511.0	64.1	14	41
14.2	74
14.7		526.1	64.1
15.3		512.6	63.1	16	44	84.62	91.67
15.8	61
17.1		480.0	59.2
17.5	57
18.6	65	508.0	62.3	13	47	89.7	95.8

Table 1 footnotes: pre-immunomodulation: <14.9 years, on immunomodulation: 14.9–17.4 years, post-immunomodulation: >17.4 years. Functional capacity for walking was measured using the Six Minute Walk Test (6MWT) conducted during clinical visits in accordance with American Thoracic Society guidelines [16]. The percent of normal distance walked was calculated using published formulas [17,18]. The Gait, Stairs, Gowers, Chair Test (GSGC) was used to measure motor function as previously described [19,20]; scores range from 4 to 27 with a score of 4 indicating normal function. The Quick Motor Function Test (QMFT) has scores ranging from 0 to 64 (score of 64 indicates normal function) and was developed to measure motor function in patients with Pompe disease [21]. The Gross Motor Function Measure (GMFM-88) is a test for motor skills, with scores ranging from 0 to 100%, with 100% indicating normal function [22].