How do physical capacity, fatigue and performance differ in children with duchenne muscular dystrophy compared with their healthy peers?

OBJECTIVE: To compare the fatigue levels and energy expenditure of children with Duchenne Muscular Dystrophy (DMD) at different functional levels with healthy children. METHODS: The cross-sectional study was carried out in the Unit of Pediatric Neuromuscular Diseases in the Department of Physiotherapy and Rehabilitation, Faculty of Health Science, Hacettepe University between March 2015 and January 2016. Fifty two children diagnosed with DMD in Level I-III according to the Brooke Functional Classification Scale and 17 healthy children were included in the study. The Six Minute Walk Test (6MWT), Northstar Ambulatory Assessment Scale (NSAA), Physiological Cost Index (PCI), and Timed performance tests were used to assess the children. RESULTS: Comparison in terms of PCI indicated a difference between Levels 2 and 3, and Levels 1 and 3 (p<0.0083). A difference was found in ascending and descending 4 stairs after 6MWT when fatigue after activity was evaluated. CONCLUSION: The walking distances, fatigue levels and energy expenditure of DMD patients were higher than the healthy peers. This difference was more prominent with decreasing functional level.

Is there any relationship between orthotic usage and functional activities in children with neuromuscular disorders?

BACKGROUND: Contractures of Achilles tendons and gastrocnemius muscle deteriorate the performance in daily living activities of patients with neuromuscular diseases. Ankle-foot orthoses help to prevent the progression of deformities and to obtain optimal position of the joints to support standing and walking. OBJECTIVE: To investigate the relationship between orthotic usage and functional activities in pediatric patients with different neuromuscular diseases. STUDY DESIGN: Retrospective study. METHODS: A total of 127 subjects' physical assessment forms were analyzed. Functional level, type of orthoses, falling frequencies, ankle joint range of motion, and timed performance tests were examined in two consecutive dates with an interval of 3 months. RESULTS: A total of 91 patients were using orthoses while 36 patients were not within assessment dates. A total of 64 of 91 (70.3%) patients were diagnosed with Duchenne muscular dystrophy. A total of 81 (89.0%) subjects were using plastic ankle-foot orthoses for positioning at nights and 10 (11%) were using different types of the orthoses (knee-ankle-foot orthoses, dynamic ankle-foot orthoses, and so on) for gait in the study group. CONCLUSIONS: Night ankle-foot orthoses were not found to be effective directly on functional performance in children with neuromuscular diseases, although they protect ankle from contractures and may help to correct gait and balance. CLINICAL RELEVANCE: This retrospective study shows that the positive effects of using an ankle-foot orthosis at night are not reflected in the functional performance of children with neuromuscular diseases. This may be due to the progressive deteriorating nature of the disease.