Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS), a progressive, fatal neurodegenerative disease, has no known cause and few effective treatments. Patients with advanced disease are unable to clear airway secretions effectively; most die of respiratory failure following recurrent, increasingly severe episodes of pneumonia.

  1. Chaisson KM, Walsh S, Simmons Z, Vender RL. A clinical pilot study: High frequency chest wall oscillation airway clearance in patients with amyotrophic lateral sclerosis. ALS 2006; 7 (2): 107-111.
    
This single center study evaluates the effectiveness of high frequency chest wall oscillation (HFCWO) when added to standard care in preventing pulmonary complications and prolonging the time to death in patients with ALS. Nine patients with a diagnosis of ALS and concurrently receiving non-invasive ventilatory support with bi-level positive airway pressure (BiPAP) were randomized to receive either standard care or standard care plus the addition of HFCWO twice-daily for 15 minutes. The addition of HFCWO did not significantly improve time to death compared to standard treatment alone (340 days +/- 247 vs. 470 days +/- 241; p = 0.26); effects on pulmonary function were non-significant. Investigators comment that this study does not exclude the potential benefit of HFCWO in select patients with ALS who have co-existent pulmonary diseases, pre-existent mucus-related pulmonary complications, or less severe levels of respiratory muscle weakness.
  2. Jackson CE, Moore D, Kittrell P, Ensrud, E. High-frequency chest wall oscillation in amyotrophic lateral sclerosis. J Neuromusc Dis 2006; 8(2): 60-64.
    
In this three month retrospective chart review to evaluate the effectiveness of high-frequency chest wall oscillation (HFCWO) therapy in 18 patients with amyotrophic lateral sclerosis (ALS), pre-treatment and post-treatment data including ALS Functional Rating Scale-Revised (ALSFRS-R), forced vital capacity (FVC), maximal inspiratory pressure (MIP), peak cough expiratory flow (PCEF), and daytime oximetry were compared. Daytime oximetry increased for 8 of 18 patients; none of the other measures changed significantly although the change in slope of FVC, MIP, and PCEF following initiation of treatment suggested a reduction in the rate of decline of each measure. Patient survey results reported that 92% felt better after HFCWO therapy and breathing was easier; 85% agreed that HFCWO eased secretion clearance and improved their quality of life.
  3. Lange DJ, Lechtzin N, Davey C, David W, Heiman-Patterson T, Gelinas D, Becker B, Mitsumoto H, and the HFCWO study group. High-frequency chest wall oscillation in ALS: An exploratory randomized controlled trial. Neurol 2006; 67: 991-997.

    This 12-week randomized controlled trial to evaluate changes in respiratory function in ALS patients after using high-frequency chest wall oscillation (HFCWO) showed that the HFCWO users (19) had less breathlessness (p = 0.021) and better nocturnal cough function (p = 0.048) at 12 weeks compared to baseline. Non-users (20) reported more noise when breathing (p = 0.027); those with FVC between 40 and 70% predicted showed a significant mean decrease in FVC; HFCWO users showed stable FVC and significantly less increased fatigue and breathlessness. 79% reported satisfaction with HFCWO.

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