Mechanical Insufflation Exsufflation and Lung Volume Recruitment in Amyotrophic Lateral Sclerosis: a Prospective Study of the Prescription Process, the Outcomes and the Experience
Introduction: Mechanical Insufflation Exsufflation (M-IE) and Lung Volume Recruitment (LVR) increase cough strength in Amyotrophic Lateral Sclerosis (ALS). Changes in respiratory measures, morbidity, physical function and outcomes; participant’s characteristics prescribed a M-IE and LVR device and their experience with devices are unknown. Finally, LVR adherence is difficult to measure.
Aims/Objectives: The primary aim was to measure, respiratory measures, morbidity, physical function and outcome in an Irish ALS cohort. Secondary aims were to examine the characteristics of participants prescribed a device; to evaluate their experiences and to develop an electronic LVR adherence prototype.
Methods: A prospective longitudinal observational cohort study evaluated 108 participants over one year. Respiratory measures, physical function and outcomes were measured. Characteristics were assessed at device prescription. A questionnaire evaluated experience. An electronic LVR adherence prototype was developed.
Results: Participants (n=108), of mean age 62.05±11.47, were recruited. Sniff Nasal Inspiratory Pressure (SNIP), Slow Vital Capacity (SVC) percent predicted, Peak Cough Flow (PCF) and Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R) declined significantly (p2O, 17.49%, 124.84L/min and 9.62 units per year respectively. One third reported a chest infection and 21 died. Participants prescribed a device had significantly lower average SNIP, SVC percent predicted, PCF and ALSFRS-R (p
Conclusion: This is the first study to quantify the rate of decline of SNIP, SVC and PCF in an Irish ALS cohort and PCF in a large ALS cohort. Device prescription is detailed. This study provides a point of reference for clinicians and future trials. The electronic LVR adherence prototype has potential to be developed for clinical trials.