PURPOSE: We aim to describe the indications, timing and survival rates of patients with ALS under continuous ventilatory support by tracheostomy and continuous noninvasive ventilation (CNIV).
METHODS: 30 ALS patients (7 Women), mean age 59±12 years old that, during the disease progression, required continuous ventilatory support were screened. FVC, SpO2 and PaCO2 values were analysed according to the timing both for nightime mask ventilation (NTNIV) and CNIV (daytime mouthpiece). Total time of CNIV, timing and duration of tracheostomy and survival rates were described.
RESULTS: Initial presentation was non-bulbar in 77% of patients. Mean timing of NTNIV was 8±12 months after diagnosis when patients had a mean predicted FVC of 52±14%, mean SpO2 of 93±2 and a mean PaCO2 of 46±7mmHg. CNIV was indicated after a mean of 11±18 months in NTNIV when patients had a mean predicted FVC of 37±13%, mean SpO2 of 93±2 and a mean PaCO2 of 46±8mmHg. Total duration of CNIV for all patients was 28±26 months. 8 patients (27%) with severe bulbar dysfunction required tracheostomy ventilation (TV) after a mean of 10±4 months in CNIV. Total time of TV was 14±8 months. 15 patients (3 under TV) died after a mean of 25±12 months under CNIV and 8±4 months under TV.
CONCLUSIONS: CNIV is feasible in non-bulbar ALS patients and TV is required in severe bulbar impairment. Both CNIV and TV can prolong survival in ALS.
CLINICAL IMPLICATIONS: In patients with amyotrophic lateral sclerosis (ALS) ventilatory support prolongs survival. Tracheostomy is usually recommended for continuous ventilatory support, however some studies suggest efficacy of continuous noninvasive ventilation (CNIV) in patients with intact bulbar function.
DISCLOSURE: The following authors have nothing to disclose: Miguel Gonçalves, Tiago Pinto, João Carlos Winck
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