mechanical insufflation in the philadelphia amyotrophic lateral sclerosis cohort (mi-pals) study

Conditions: Amyotrophic Lateral Sclerosis; Chronic Respiratory Failure; Airway Clearance Impairment; Neuromuscular Diseases

Summary: the goal of this clinical trial is to learn how doing mechanical insufflation (mi) using a mechanical insufflator-exsufflator (mi-e) device affects breathing in early amyotrophic lateral sclerosis (als). this will be a single-center, single-arm study of mi in 20 patients with als at penn. based on prior research, we believe that 6-months of mi may slow decline in cough strength, measured as peak cough flow (pcf). participants will perform mi using a device designed for mechanical insufflation-exsufflation (mi-e) known as the biwaze cough system. the biwaze cough is used for mucus clearance . it is connected to tubing and mouthpiece (or mask). the device will use programmed pressure and timing settings. an insufflation includes inflating the lungs for a maximal size inhalation before exhaling. the daily routine for the device includes 5 sets of 5 insufflations twice daily. researchers will compare how use of mi in early als affects peak cough flow compared to 20 subjects who did not use mi in early als.


Eligibility criteria:
inclusion criteria: 1. provision of signed and dated informed consent form. 2. stated willingness to comply with all study procedures and availability for the duration of the study. 3. age ≥18 years. 4. diagnosed with amyotrophic lateral sclerosis using the gold coast criteria. 5. have an able and willing caregiver to assist with mechanical insufflation on a daily basis. 6. willingness and ability to participate in study procedures. exclusion criteria: 1. age \<18 years old. 2. inability to perform a cough peak flow or spirometry manuever 3. current use of non-invasive ventilation (niv), bi-level positive pressure ventilation, or "bi-pap" or physician prescribing niv on day of potential enrollment. 4. current use of mi-e (also known as a "cough assist device") for airway clearance. please note that patients can start use of a mi-e device subsequent to enrollment while currently being followed for the study. 5. active enrollment in hospice. 6. current tracheostomy. 7. presence of cognitive dysfunction that would impair ability to complete study procedures, as determined by neurology attending physician. 8. absence of an able and willing caregiver to assist with mi twice daily as specified in the protocol. 9. pregnancy 10. medical history of any of the following: 1. recent hemoptysis 2. recent barotrauma 3. history of emphysema of any kind (including bullous emphysema) 4. history of or known susceptibility to pneumothorax 5. history of or known susceptibility to pneumomediastinum 6. chronic obstructive pulmonary disease 7. uncontrolled asthma (defined as recent exacerbation requiring corticosteroids in the previous 30 days) 8. symptomatic cardiomyopathy (heart failure) with left ventricular ejection fraction less than 50% 9. history of right heart failure or pulmonary hypertension 11. current smoker or tobacco use within the last 30 days.

Keywords: amyotrophic lateral sclerosis chronic respiratory failure neuromuscular weakness mechanical insufflation-exsufflation peak cough flow