Prednisone is currently the only medication that has demonstrated any benefit in the treatment of Becker’s dystrophy. The dose is 0.75 to 1.5 mg/kg/day in divided doses. The muscle wasting produced by this disease is retarded or delayed by the administration of prednisone. The benefit of prednisone administration may become evident as soon as 1 month after initiation of treatment, but those benefits generally last only about 3 years. These benefits may also be somewhat diminished by sequelae resulting from chronic steroid administration.

The remainder of medical management of Becker’s dystrophy concentrates on measures and strategies to maximize functional status, maintain muscle tone, and delay reliance on a wheelchair for as long as possible. Exercising joints and stretching muscles daily can delay the onset of debilitating contractures. These exercises work synergistically with the application of various supportive braces, such as ankle-foot or knee-ankle-foot orthoses, to help maintain the ability to stand, whether mobile or not, and further contribute to the delay of debilitating contractures and scoliosis.

Becker’s dystrophy is a progressive disease that ultimately culminates in muscle-joint contractures, profound muscle weakness, and disability. When the patient becomes wheelchair dependent, the possibility of developing pressure sores increases. This possibility is further exacerbated by the chronic administration of corticosteroids. With the development of pressure sores comes the greater potential for infection and sepsis. Muscle wasting or atrophy and weakness contribute to alteration in pulmonary function, particularly forced vital capacity (FVC). Diminished pulmonary function can lead to the development of atelectasis and pneumonia. Frequent use of incentive spirometry can help prevent atelectasis and pneumonia. The decline in pulmonary function may necessitate some manner of ventilatory support, ranging from noninvasive, such as continuous positive airway pressure (CPAP), to minimally invasive, such as bilevel positive airway pressure (BiPAP), to invasive, such as tracheal intubation or tracheostomy, with or without mechanical ventilation. Tracheal intubation should be a short-term measure that should be supplanted by tracheostomy, whether or not it is combined with mechanical ventilation.