To asssess efficacy of airway clearance provided by Vest therapy (HFCWO) in the reduction of respiratory exacerbations requiring hospitalization or antibiotic utilization in patients with muscle weakness and restrictive lung disease.

This non-randomized home based pre-post intervention study. The study will compare the efficacy of Vest® therapy (HFCWO) for mucus secretion clearance in a population of patients with muscle weakness and restrictive lung disease with frequent pulmonary exacerbations. Enrolled subjects with a tracheostomy will be evaluated for microbial load and inflammatory status as a pilot evaluation of microbial load and inflammatory status outcomes.

The total number of evaluable subjects to be recruited is estimated at 40. Subjects will be considered evaluable if they have completed at least 6 months of follow up.

Inclusion Criteria:

• Subject is at least 18 months old.
• Subject has a chest circumference of greater than 19 inches.
• Subject has a diagnosis of muscle weakness and restrictive lung disease.
• Ability to provide Informed consent from legal guardian.
• Subject has had 2 or more exacerbations requiring either hospitalization or antibiotics (IV or oral) in the past 12 months
• If inhaled Tobramycin has been prescribed prophylacticly, subjects must discontinue the medication at least 2 weeks prior to study enrollment

Exclusion Criteria:

• Subject has a diagnosed allergy with a respiratory trigger.
• Participation in another clinical trial.
• Unstable head or spinal injury.
• Unresolved pneumothorax or pneumomediastinum present
• Unresolved hemorrhage
• Hypotension requiring vasopressors or positioning
• Bronchopleural fistula
• Gross hemoptysis within the past eight hours
• Pulmonary embolism or history of pulmonary embolism within the past two months
• Burns, open wounds and skin infections on the thorax
• Osteomyelitis of the ribs
• Osteoporosis with history of fractures.

Patients who completed 12 months of prospective therapy

24 months

Study design was sub-optimal due to lack of control group and lack of randomization