No abstract

In patients with stable ALS, bulbar dysfunction (NBS < 29), PCF (< 4.25 L/s), and PCF/PVT (< 28.88 L/s(2)) could predict the risk of ineffective spontaneous cough during a respiratory tract infection.

For ventilator-dependent patients with amyotrophic lateral sclerosis, mechanical insufflation-exsufflation via a tracheostomy tube with an inflated cuff may be more effective in eliminating airway secretions than conventional tracheal suctioning.

Background Home tracheotomy mechanical ventilation (HTMV) can prolong survival in patients with amyotrophic lateral sclerosis (ALS) when non-invasive ventilation (NIV) fails, but knowledge about HTMV is scarce. The aim of this study was to determine the causes of tracheotomy and the main issues of 1-year HTMV in a cohort of patients with ALS. Methods A prospective study of all patients needing HTMV was performed in a referral respiratory care unit (RCU) from April 2001 to January 2010. Patients' informed decisions about HTMV were fully respected. Caregivers were trained and could telephone the RCU. Hospital staff made home visits. Results All patients (n¼116) agreed to participate and a tracheotomy was needed for 76, mainly due to bulbar dysfunction. Of the 38 who had a tracheotomy, in 21 it was performed in an acute setting and in 17 as a nonemergency procedure. In 19 patients the tracheotomy was related to the inadequacy of mechanically assisted coughing (MAC) to maintain normal oxygen saturation. During HTMV, 19 patients required hospitalisation, 12 with respiratory problems. The 1-year survival rate was 78.9%, with a mean survival of 10.39 months (95% CI 9.36 to 11.43). Sudden death was the main cause of death (n¼9) and only one patient died from respiratory causes. No predictive factors for survival were found. Conclusions Besides NIV inadequacy, the ineffectiveness of mechanically assisted coughing appears to be a relevant cause of tracheotomy for patients with ALS with severe bulbar dysfunction. Patients choosing HTMV provided by a referral RCU could have a good 1-year survival rate, respiratory problems being the main cause of hospitalisation but not of death.Although respiratory problems related to inspiratory and expiratory muscle weakness and to upper airway muscle dysfunction are the main cause of morbidity and mortality in patients with amyotrophic lateral sclerosis (ALS), 1 the use of appropriate respiratory muscle aids can change the course of the disease dramatically. Non-invasive ventilation (NIV) and mechanically assisted coughing (MAC) can prolong survival, 2 3 reduce hospitalisations, 2 improve quality of life 3 and relieve symptoms in patients with ALS. 2 3 Nevertheless, when bulbar dysfunction (BD) reaches a critical level, non-invasive muscle aids become inadequate 2 4 and it is essential to perform a tracheotomy or to intensify appropriate palliative care.Home tracheotomy mechanical ventilation (HTMV) can prolong survival for patients with advanced ALS, 5 and this is the procedure of choice for those who want to continue living. 6 7 However, when one examines the guidelines, the paucity of information on HTMV (the best time to start, the right procedures, main complications, real burdens, etc) is clearly inferior to the information available for other ALS management procedures. 6 7 This lack of information could be related to the small quantity and the protocol limitations of the studies available on HTMV in ALS. Although they provide new and valuable information, the existing stu...