Mechanical Insufflation in the Philadelphia Amyotrophic Lateral Sclerosis Cohort (MI-PALS) Study

Launched by UNIVERSITY OF PENNSYLVANIA · Oct 31, 2023

Keywords

ClinConnect Summary

The MI-PALS study is looking to understand how a device called a mechanical insufflator-exsufflator (MI-E) can help improve breathing in people with early-stage amyotrophic lateral sclerosis (ALS). This study will involve 20 participants at Penn who will use the BiWaze Cough system, which helps clear mucus from the lungs. Over six months, participants will perform a specific breathing routine with the device twice a day, and researchers will measure its impact on cough strength. The goal is to determine if using this device can slow down the decline in cough strength in ALS patients.

To join this study, participants need to be at least 18 years old and have a confirmed diagnosis of ALS. They should also have a caregiver who can help them use the device daily. Excluded from the study are individuals under 18, those currently using certain breathing machines, or those with specific health conditions that could complicate the study. If eligible, participants will commit to following the study procedures and can expect regular monitoring to see how the device affects their breathing and cough strength.

Gender

ALL

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.