Title: 2013 Cochrane Fellowship: The use of standing frames to enhance quality of life in Duchenne Muscular Dystrophy
Funder: Health and Social Care Research and Development Division (HSC R&D) of Public Health Agency, Northern Ireland

Abstract: Duchenne Muscular Dystrophy (DMD), the most common form of muscular dystrophy, is a genetically-linked disease of male children which affects one in 3,300 boys1. Muscle wasting is the main clinical presentation of this population, resulting in a gradual physical and functional decline in addition to secondary co-morbidities. As the weakness progresses, the boys often become confined to wheelchairs early in the second decade of life2. Due to advances in medical treatment these patients are now living longer and are confined to a wheelchair for longer time periods. Therefore the longevity and complexity of co-morbidities are greater. Second to this, the economic burden on healthcare services to manage their condition is continuing to rise. As a result patient management must be efficient and cost effective. A recent survey of therapists revealed that 68% of therapists prescribed standing frames for DMD3. Research has reported many benefits including improvement in bone density, reduction in bladder infections, improved digestion, respiratory improvement, circulatory benefits and reduced risk of pressure ulcers. Another suggested benefit of standing frames is the reduction of scoliosis; approximately 68-90% of boys with DMD develop scoliosis4 which has a negative impact on respiratory function, feeding, seating and comfort5. Despite the knowledge of the potential benefits of standing frames for this population, there has not been a consensus in the approach to their use. Expert consensus on the management of DMD have reported standing frames to be logical but not evidenced 6 with guidelines stating there is no consensus as to how long or how often they should be used7. As a result, there is a current need to complete a review of the literature regarding the use of standing frames for patients with DMD. The need for this review has also been highlighted as a priority by the Cochrane neuromuscular disease group. 1 Deconinck N, Dan B. (2007). Pathophysiology of Duchenne muscular dystrophy. Pediatric Neurology, 36(1):1-7 2 Emery A, Muntoni F. (2003). Duchenne Muscular Dystrophy. 3rd edition. Oxford University Press, Oxford. 3 Bakker JPJ, de Groot IJM, De Jong BA, van Tol-de Jager MA, Lankhort GJ. (1997). Prescription pattern for orthoses in The Netherlands: Use and experience in the ambulatory phase of Duchenne muscular dystrophy. Disability and Rehabilitation, 19 (8): 318-325. 4 Predictive factors for the development of scoliosis in Duchenne muscular dystrophy. (2007). European Journal of Paediatric Neurology, 11(3): 160-166. 5 Manzur AJ, Kinali M, Muntoni F. (2008). Update on the management of Duchenne muscular dystrophy. Archives of disease in childhood, 93 (11):986-990. 6 Eagle M. (2002). Workshop Report on the Muscular Dystrophy Campaign workshop: Exercise in neuromuscular diseases Newcastle. Neuromuscular Disorders, 12: 975–983. 7 Dooner R, Keddie A, Lomax D, Melville P, Morrow M, Wotherspoon L. (2007).Duchenne Muscular Dystrophy Scottish physiotherapy management profile.


Research activity codes: 7.1 INDIVIDUAL CARE NEEDS
Health categories: MUSCULOSKELETAL