Standards of Care in Muscular Dystrophy Association Clinics

Abstract

Two hundred seventy three Muscular Dystrophy Association (MDA) clinic directors and co-directors of 167 of the 220 clinics responded to a survey designed to study physical management approaches for patients with Duchenne muscular dystrophy (DMD). Lower extremity musculotendinous release surgery was performed on at least some patients in 140 of 166 (84 percent) responding clinics at the time or before they became entirely dependent on wheelchair use. Orthopedic surgery was also performed to reduce scoliotic curves on at least some patients in 137 of 166 clinics (83 percent). The most frequent surgical method used was Luque instrumentation in 71 percent of the 137 clinics where scoliosis was managed surgically. Eighty-nine of 165 responding clinics (54 percent) also reported using thoracolumbar bracing for the prevention or treatment of scoliosis for DMD patients.Ventilatory assistance was recommended and used on an elective basis in 43 of 167 clinics (26 percent); its use was discouraged in 68 clinics (41 percent); and 62 clinics (37 percent) were managing no ventilator users at the time of the survey. The most common reason given for discouraging ventilator use was poor patient quality of life. Only two physicians who discouraged use of mechanical ventilation were familiar with newly described methods of noninvasive ventilatory aid..We conclude that great variability exists in the access of DMD and other neuromuscular disease patients to orthopedic, pulmonary therapeutic, and life-prolonging interventions.