Predictive factors for the development of scoliosis in Duchenne muscular dystrophy

Duchenne muscular dystrophy: the management of scoliosis

“…There is evidence that using steroids in patients with DMD can have numerous positive effects including prolonged independent ambulation, decreased progression of scoliosis, increased muscle strength, respiratory function, cardiac function and quality of life (18,(21)(22)(23)(24)(25)(26). The use of steroid therapy has been associated with a reduction in the incidence of scoliosis in DMD because prolonging ambulation means that some boys will undergo their pubertal growth spurt before loss of ambulation.…”

“…Maintaining ambulation for as long as possible can reduce the risk of the development of scoliosis and can decrease the severity of the scoliosis in the long term (18). Again, a multidisciplinary approach is essential involving physiotherapists, rehabilitation specialists and orthopaedic surgeons (19).…”

“…One such study looked at a number of factors that might influence scoliosis development including age at onset, glucocorticoid treatment, age at loss of ambulation and forced vital capacity. In a cohort of 123 patients with DMD, the administration of prednisolone reduced the development of scoliosis, prolonging ambulation and a smaller scoliosis at 17 years of age (18).…”

Psychosocial Support Needs of Families of Boys with Duchenne Muscular Dystrophy

“…By using the DMD-FNS during clinic visits, health care professionals may be in a better position to understand each family's unique priority needs and strengths, instead of having to second guess what they might be or be influenced by clinician's personal bias [Gibson, 2001;Kinali et al, 2007]. The revised DMD-FNS that was used in this study incorporates topics specific to the care of individuals with DMD, and therefore may serve as an ideal prompt for parents to discuss these issues during their child's regular clinic visits.…”

“…Therapeutic interventions include the use of corticosteroids (such as prednisone or deflazacort) for skeletal muscle weakness and afterload reduction (such as angiotensin converting enzyme inhibitor or beta-blocker) for cardiomyopathy. Corticosteroid therapy offers benefit to DMD boys by improving muscle strength and function [Mendell et al, 1989;Griggs et al, 1991], prolonging independent ambulation [Biggar et al, 2001;Schara et al, 2001], plus slowing the progression of cardiomyopathy [Markham et al, 2008] and scoliosis [Kinali et al, 2007]. As well, the introduction of noninvasive positive pressure ventilation has prolonged the survival of individuals with DMD [Bach & Martinez, 2011].…”