Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is a sex-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 is 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. Increased risk for aspiration and reduced ability to clear airway secretions ensue, leading to increasingly frequent and severe respiratory infections and pneumonias. Refractory pneumonia and severe mucus plugging contribute to the most common cause of death - respiratory failure.

Although DMD is incurable, it is increasingly treatable. With aggressive disease management including effective airway clearance therapy (ACT) and, in later stages, assisted ventilation, extended survival is not uncommon.

Regular ACT is recognized as an essential component in the standard respiratory care regimen for DMD. Many physicians prescribe high frequency chest compression (HFCC) for DMD patients who cannot mobilize airway secretions without therapeutic intervention. ACT methods requiring active participation are generally unsuitable. In patients with ineffective cough, additional techniques or devices to clear secretions from central airways are also required.

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  • Braverman J, Nozzarella M. High-frequency chest compression: Advanced therapy for obstructive lung disease. Respir Therp 2007; 2 (2): 48-51. [Link]
  • Toder DS. Respiratory problems in the adolescent with developmental delay. Adolescent Medicine: State of the Art Reviews. 2000; 11 (3): 617-631. [PubMed]