Determine the proportion of patients who are non-compliant with BiPAP and identify factors associated with noncompliance. This will help define strategies to improve ventilator support and better treat our ALS patients.

ALS is an incurable disease that affects around 20,000 patients in the U.S.A. The main therapies are designed to improve the quality of life (QOL). One technique is the use of non-invasive ventilation (BiPAP) when patients develop weakness of respiratory muscles. Multiple clinical trials have revealed that BiPAP improves the patients’ QOL, and has been shown to have survival benefit. However, a significant number of patients who are prescribed BiPAP, stop using it. The frequency and reasons for noncompliance have not been studied.

Retrospective study was conducted focusing on ALS patient’s compliance/noncompliance with non-invasive ventilation. Electronic medical records were reviewed, questionnaires were administered, and descriptive data were compiled/analyzed. Factors associated with noncompliance were identified, as well as clinical attributes associated with patients’ failure to comply with this standard therapy.

Data were collected on 22 patients. Patients were predominantly Caucasian (96%), male (73%), and compliant (87%). Compliant patients reported improvement regarding QOL (68%), breathing (63%), fatigue (42%), and depression (26%). However, they did report difficulties with mask discomfort (47%), air leakage (53%), mask management (11%), and claustrophobia (16%).

Non-compliant patients consistently reported no improvement of QOL, breathing, fatigue, or depression. Primary reason for noncompliance was secondary to mask discomfort (66%) and claustrophobia (33%).

Majority of patients reported being compliant with BiPAP and improvement of QOL, breathing, fatigue, and depression. A high percentage of patients, in both categories, reported significant dissatisfaction with mask fit, air leakage, and claustrophobia. 

There is an unmet need to enhance the quality and fit of masks in order to improve patient compliance with non-invasive ventilation and thereby improving QOL, breathing, fatigue, and depression of ALS patients.