Aspiration pneumonia in familial dysautonomia (FD) patients is common and causes increased secretions. Hypotonia, poorly coordinated breathing, and spine curvature impede effective clearance of secretions and increase risk for chronic lung disease. Chest physiotherapy (CPT) has inherent complications due to concomitant labile blood pressures and gastroesophageal reflux. High-frequency chest wall oscillation (HFCWO) therapy (administered via an inflatable vest) creates airflow within the lungs that loosens and mobilizes secretions cephalad. Although HFCWO is used in a variety of patients, outcomes in FD have not been reported. We hypothesized that HFCWO therapy would reduce respiratory problems and medical interventions and improve lung function.
Fifteen FD patients (age range 11–33 y) were enrolled in a one-year clinical trial of HFCWO therapy. Two subjects withdrew after 3 and 6 months, respectively. Retrospective chart review of 12 months prior to entry provided baseline data regarding respiratory illnesses, medications, doctor visits, hospitalizations, and absenteeism. Daily logs provided prospective data. Evaluations were performed at one, 3, 6, 9, and 12 months for pulse oximetry, spirometry and log review.
In the first month >=85% compliance was achieved by 87% of 15 subjects contrasted with 50% of 13 subjects by study completion. Mean oxygen saturation improved by one month (93.5±3.3% vs. 96.8±1.6%, p =0.005) and was sustained at exit evaluation (97.9±0.7%). Mean FVC was the pulmonary function measure with sustained improvement from 3 months to exit (p<0.05). When retrospective and prospective data were compared, all measured health outcomes improved significantly including pneumonias (p=0.0156), hospitalizations (p=0.0161), antibiotic courses (p=0.0005), antibiotic days (p=0.0002), doctor visits (p=0.0005), and absenteeism (p=0.0002).
In this study of FD patients, HFCWO effected significant improvements in all measured health outcomes and oxygen saturation; FVC was the pulmonary function measure with sustained improvement.CLINICAL IMPLICATION: HFCWO is beneficial for FD patients with lung disease.
A.A. Chaikin, Advanced Respiratory, Grant monies,, Industry,, Discussion of product research or unlabeled uses of Product.