Table 2: Study results. The three patients enrolled in the original pilot trial (P1, P2, and P3) received CsA for 2 to 3.2 years and the three new patients for 1 to 1.2 years. Megalimbs increased in all but P5. The distance walked in 6 minutes remained the same in the 2 patients able to walk. Forced vital capacity declined in all from 57.1% predicted to 45.2% predicted.

PatientAge at treatment (Years)Age at Followup (Years)Megalimbs (Newton)6 MWD (meter)FVC %
Time 0FollowupTime 0FollowupTime 0Followup

19.512.7218283NANA4724
29.811.6106142NANA4432
38.910.9106120NANANANA
49.010.2140169NANA41.731
59.110.12412221891885951
65.56.71722363003049488

Mean8.610.4163.8195.3244.524657.145.2

Megalimbs: sum of bilateral muscle strength of elbow flexion, hand grip, knee flexion, and knee extension; 6 MWD: distance walked in 6 minutes; FVC %, forced vital capacity % of predicted; NA: not able.