2000 OPEN FORUM Abstracts
HIGH FREQUENCY CHEST WALL OSCILLATION: VIDEO DOCUMENTATION OF EFFECT ON A PATIENT WITH DUCHENNE'S MUSCULAR DYSTROPHY AND SEVERE SCOLIOSIS
Ann Gomez RCP, Ph.D., Isabelo Elisan RPFT, Karen Hardy MD; Children's Hospital Oakland, Oakland, CA
INTRODUCTION: High Frequency Chest Wall Oscillation (HFCWO or Vest Therapy) is a method of chest physical therapy, used for the past 11 years. Indications that require aggressive airway clearance include secretion removal for diseases like Cystic Fibrosis and bronchiectasis. Therapy is delivered in the hospital and at home. It is most often used for patients with Cystic Fibrosis, both in the hospital and as a home therapy. We present here another indication for Vest Therapy.
CASE SUMMARY: JB has had Duchenne's Muscular Dystrophy since the age of six. He is now 16 years old and has profound kyphoscoliosis. His pulmonary function shows restrictive and obstructive airway disease, and poor tidal volume. His cough is ineffective. JB was hospitalized in 1997, and required a prolonged stay in the intensive care, with five bronchoscopies to remove thick secretions and treat persistent atelectasis. Nocturnal BiPAP was instituted to treat persistent atelectasis. After discharge, airway clearance at home included Albuterol nebulization followed by postural drainage and percussion (PD&P) BID. However, the PD&P was severely limited due to JB's profound kyphoscoliosis. This December JB was hospitalized, for progressive difficulty feeding over three months, with significant weight loss, and the need for continuous BiPAP resulting in facial necrosis. JB received aggressive nutritional supplementation and a tracheostomy to ease the application of BiPAP and enhance airway clearance. Despite Albuterol and modified PD&P TID, he continued to have thick secretions requiring deep suctioning. Two weeks after tracheostomy, JB had an acute respiratory decompensation with pH of 7.0, and PaCO2 of 123, requiring hand-ventilation and repeated lavage and suctioning of bloody secretions. Pediatric Pulmonology was consulted. Video bronchoscopy (performed by KH.) revealed severe diffuse tracheitis, extensive mucous plugging was present, with full obstruction of left mainstream. Airway clearance methods, hydration, and deep suctioning needed to be reevaluated. We instituted Vest Therapy for 30-minute intervals at a pressure of 4 and a frequency of 15 Hz during Albuterol aerosol, QID. Heated humidity was initiated and a cut-off suction catheter or olive tip was used to remove secretions. There was significant clinical improvement in PaO2, PaCO2 and patient comfort. Days later, a subsequent video bronchoscopy showed healing mucosa and minimal secretions. Intraprocedure Vest Therapy documented significant movement of distal secretions from the lung periphery to the central airways for removal by bronchoscope.
DISCUSSION: Vest Therapy in Duchenne's Muscular Dystrophy has not been previously reported. However there have been two publications describing Vest Therapy, one in an MS patient, and another in 8 patients with neuromuscular weakness. As these diseases progress, they have increasing skeletal deformity causing worsening pulmonary function and decreased ability to clear secretions from the lower airways. Their deformity severely limits the effectiveness of conventional airway clearance techniques. We conclude that Vest Therapy is effective to mobilize distal airway secretions for easier removal as documented by video bronchoscopy. We recommend this therapy be applied to other patients with similar pathophysiology.