The muscular dystrophies are a group of inherited, progressive neuromuscular disorders classified on the basis of specific genetic characteristics. Among such conditions, Duchennes muscular dystrophy (DMD) is the most common and most debilitating. Because a sex-linked recessive gene causes the disorder, it affects males almost exclusively; approximately one boy in 3,000 is born with DMD.1
In severe forms of Muscular Dystrophy (MD), especially DMD, muscles involved in swallowing and respiration weaken over time and gradually waste away. Skeletal-spinal deformities also develop.2 These impairments interact in complex ways to diminish pulmonary function. Although the significance of each of the following conditions is related closely to the extent of disease progression, individuals with severe MD are predisposed to the following life-threatening respiratory complications:
- Restrictive pulmonary disease
- Ineffective cough
- Chronic aspiration
- Chronic respiratory insufficiency
- Respiratory failure
By late adolescence, serious, recurrent pulmonary infections are commonplace. Death is most frequently a direct result of respiratory failure, usually due to pneumonia, mucus plugging, or atelectasis.
Without effective removal of retained secretions, individuals with severe MD are caught in a spiral of progressive, ultimately irreversible pulmonary decline. Many respiratory complications associated with MD are preventable or treatable.3 To delay disease progression, excess secretions must be mobilized and cleared. Routine administration of aggressive, effective Airway Clearance Therapy is the most reliable means of accomplishing this goal.
Airway Clearance with The Vest® System
The Vest® Airway Clearance System is an easy-to-use medical device for children and adults. The Vest® System is quiet, comfortable and portable. Its features promote user independence and simplify treatment routines.
The Vest® System uses a technology called High Frequency Chest Wall Oscillation (HFCWO). The Vest® System has an inflatable garment connected by Air Hoses to an Air Pulse Generator. During therapy, the inflatable garment inflates and deflates rapidly, applying gentle pressure to the chest wall. This works to loosen and thin mucus and to move it toward the larger airways, where it can be cleared by coughing or suctioning.
The Vest® System — Safe and Effective Therapy
HFCWO has been widely described in medical literature. More than 80 studies demonstrate the efficacy and safety of HFCWO for a variety of patients.
Learn more about clinical evidence and support:
- HFCWO (The Vest® System) Research
- Case Study: Muscular Dystrophy and Congenital Myopathy with Respiratory Insufficiency Pneumonia - Tom Hall (employed by Hill-Rom)
- Airway Clearance Needs in Duchenne Muscular Dystrophy: An Overview - Jane Braverman (employed by Hill-Rom)
- Airway Clearance Needs in Neuromuscular Disease: An Overview - Jane Braverman (employed by Hill-Rom)
- Chest Physiotherapy: The Gold Standard? - Jane Braverman (employed by Hill-Rom)
- Mendell JR, Sahenk Z, Prior TW. The childhood muscular dystrophies: Diseases sharing a common pathogenesis of membrane instability. J Child Neurol 1995; 10: 150-159.
- Smith PEM, Edwards, RHT, Evans GA, Campbell EJM. Practical problems in the respiratory care of patients with muscular dystrophy. New Engl J Med 1987; 316: 1197-1205.
- Schramm CM. Current concepts of respiratory complications of neuromuscular disease in children. Curr Opin Pediatr 2000; 12: 203-207.