Article Text
Abstract
Objective To evaluate the clinical characteristics and outcome of non-invasive ventilation (NIV) in an epidemiological based series of amyotrophic lateral sclerosis (ALS) patients.
Methods The study was performed using data from the Piemonte and Valle d'Aosta Register for ALS, a prospective epidemiological register enrolling all ALS incident cases in two Italian regions.
Results Among the 1260 patients incident in the period 1995–2004, 259 (20.6%) underwent NIV. Young male patients and subjects attending the tertiary ALS centres were more likely to undergo NIV. There was a progressive significant increase in the use of NIV during the study but was limited to patients attending the ALS tertiary centres. Median survival after NIV was 289 days (95% CI 255 to 333).
Conclusions In an epidemiological setting, NIV represents an increasingly utilised option for the treatment of respiratory disturbances in ALS and has favourable effects on survival, in particular among patients followed by tertiary ALS centres. Sociocultural factors, such as age, gender and marital status, strongly influence the probability of undergoing NIV. Efforts should be made to remove these obstacles in order to spread the use of NIV in all ALS patients with respiratory failure.
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Footnotes
Funding The study was in part supported by Regione Piemonte (Ricerca Finalizzata 2002, grant 12944; Ricerca Scientifica Applicata 2004, grant A317; Ricerca Finalizzata, 2009, grant 30258) and Ministero della Salute, Centro Nazionale per la Prevenzione e il Controllo delle Malattie (CCM) (grant 31, 2009). The research leading to these results has received funding from the European Community's Health Seventh Framework Programme (FP7/2007-2013) under grant agreement No 259867.
Competing interests AC serves on the editorial advisory board of Amyotrophic Lateral Sclerosis and has received research support from the Italian Ministry of Health (Centro Nazionale per la Prevenzione e il Controllo delle Malattie), Regione Piemonte (ricerca finalizzata) and European Commission (Health Seventh Framework Programme). He serves on the ALS advisory board of Biogen Idec. GM has served on the editorial advisory board of Amyotrophic Lateral Sclerosis and has received research support from the Italian Ministry of Health (Ricerca Finalizzata).
Ethics approval Ethics approval was provided by Comitato Etico Regione Piemonte.
Provenance and peer review Not commissioned; externally peer reviewed.