Physical Medicine Interventions for Duchenne Muscular Dystrophy, the Including Avoiding Invasive Airway Tubes

5 I. INTRODUCTION Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease that primarily affects skeletal, but also cardiac and smooth muscle to a degree. With DMD there is less than 1% of normal muscle Dystrophin whereas greater Dystrophin denotes Becker muscular dystrophy. Since DMD patients go through ambulatory, wheelchair, and life preserving stages, we consider DMD at type 3 disorder (NMD). There is delayed acquisition of motor milestones, including walking, and later loss of this ability from 7 to 15 years of age thereby beginning the wheelchair user stage. Before the use of inspiratory and cough aids, life expectancy was about 20 years. Then with tracheostomy tubes it was about 29 years. Using noninvasive ventilatory support (NVS) and mechanical in-exsufflation (MIE) it is 10 years longer and often to over age 50. However, for quality of life orthopedic, cardiology, nutrition, surgical, physical, occupational, speech, swallowing, computers, and robotics are needed. Walking can be prolonged by an early surgical muscle repositioning protocol, and targeted therapy. Scoliosis can be entirely reversed, and tracheostomy tubes avoided indefinitely, including for those requiring continuous ventilator use in their 50s. All of these advances require knowledge of physical medicine interventions to maintain quality of life and life itself. To achieve this, it is essential to understand the disease trajectory, identify its stage, and anticipate its management. Although DMD is conventionally divided into four phases, this classification has limitations compared to current advances that extend life into advanced age through the interventions described here. Therefore, we will classify the disease into three stages, and the relevant interventions for each can be seen in Figure 1.