S.P.1 Can the six minute walk test (6MWT) reflect deterioration in function in ambulant children with Spinal Muscular Atrophy (SMA)

Abstract

Abstract In children with 5q SMA, type 3 is defined by the ability of children to achieve independent ambulation. Studies have shown, however, that a high proportion of these children will lose functional walking before adulthood. The age of onset of symptoms, functional scores, timed tests and walking distance can give some indication of long term ability to remain ambulant. Variables including progression of weakness in individual children, and rapidity of growth during puberty, however, mean that predicting the outcome in an individual child is not straightforward. The ability to predict when children are at risk of losing ambulation would assist parents and therapists in planning housing adaptations, mobility aids and accessibility problems in schools. The Dubowitz neuromuscular service at GOSH is involved in the follow up of a cohort of 25 children with SMA3 justifying a specific clinic for their needs. It was felt that while the current assessments used gave a good indication of function in the mildly and more severely affected children, the course of those in the intermediate category was less predictable. The addition of the six Minute Walk Test (6MWT) to the regular physiotherapy assessment of ambulant children with SMA has given an improved clinical picture of ability that better reflects their overall function, correlating well with other tested parameters and interventions. Our study confirms recent observation that fatigue, as detected by the 6MWT, plays a significant role in SMA.The results of the 6MWT when used in conjunction with the other parameters has proved to be a useful indicator in predicting loss of ambulation in children with SMA 3.