Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (DMD) is a gender-linked inherited condition that impairs production of an essential muscle-building protein called dystrophin. A variety of other inherited muscle-wasting disorders are also classified as muscular dystrophies, but DMD is the most prevalent and debilitating. Approximately one in 3,000 newborn males are affected.
DMD is characterized by progressive deterioration of skeletal muscles. Most boys depend upon wheelchairs by their early teens. Progressive weakening of respiratory and swallowing muscles compromise cough and pulmonary defenses. An increased risk for aspiration (swallowing and “going down the wrong pipe”) and the reduced ability to clear airway secretions leads to increasingly frequent and severe respiratory infections and pneumonias. Persistent or recurrent pneumonia and severe mucus plugging contribute to the most common cause of death – respiratory failure.
Although DMD is incurable, it is increasingly treatable. Aggressive disease management including effective airway clearance therapy and, in later stages, assisted ventilation can stabilize the lung condition.
Regular airway clearance therapy is recognized as an essential component in the standard respiratory care regimen for DMD. Many physicians prescribe vest therapy for DMD patients who cannot clear airway secretions without therapy. Therapy using techniques and methods that require a lot of effort on the part of the patient are generally unsuitable. In patients with ineffective cough, additional techniques or devices to clear secretions from central airways are also required.