Mechanical Insufflation in the Philadelphia Amyotrophic Lateral Sclerosis Cohort (MI-PALS) Study
Overview
- Phase
- Not Applicable
- Intervention
- Not specified
- Conditions
- Amyotrophic Lateral Sclerosis
- Sponsor
- University of Pennsylvania
- Enrollment
- 20
- Locations
- 1
- Primary Endpoint
- peak cough flow (PCF)
- Status
- Recruiting
- Last Updated
- 6 months ago
Overview
Brief 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.
Investigators
Eligibility Criteria
Inclusion Criteria
- •Provision of signed and dated informed consent form.
- •Stated willingness to comply with all study procedures and availability for the duration of the study.
- •Age ≥18 years.
- •Diagnosed with amyotrophic lateral sclerosis using the Gold Coast Criteria.
- •Have an able and willing caregiver to assist with mechanical insufflation on a daily basis.
- •Willingness and ability to participate in study procedures.
Exclusion Criteria
- •Age \<18 years old.
- •Inability to perform a cough peak flow or spirometry manuever
- •Current use of non-invasive ventilation (NIV), bi-level positive pressure ventilation, or "Bi-PAP" or physician prescribing NIV on day of potential enrollment.
- •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.
- •Active enrollment in hospice.
- •Current tracheostomy.
- •Presence of cognitive dysfunction that would impair ability to complete study procedures, as determined by neurology attending physician.
- •Absence of an able and willing caregiver to assist with MI twice daily as specified in the protocol.
- •Medical history of any of the following:
- •Recent hemoptysis
Outcomes
Primary Outcomes
peak cough flow (PCF)
Time Frame: 6 months
PCF in liters/minute as measured by use of a peak flow meter while the patient is in a seated upright position. An alternative device for measuring PCF can be the use of a handheld spirometer and using the measured peak expiratory flow by multiplying by 60 to convert from liters/second to liters/minute. The subject must be seated in an upright position and the interface must include a mouthpiece or an oronasal mask. Subjects are asked to perform a deep inhalation followed by a maximal cough.
Secondary Outcomes
- Maximal expiratory pressure (MEP)(6 months)
- Maximum insufflation capacity (MIC)(6 months)
- MIC-FVC difference(6 months)
- Forced vital capacity (FVC)(6 months)
- Maximal inspiratory pressure (MIP)(6 months)
- Maximum insufflation capacity assisted peak cough flow(6 months)
- Transcutaneous carbon dioxide(6 months)
- ALS Functional Rating Scale - Revised (ALSFRS-R) dyspnea and orthopnea scores(6 months)
- Global rate of change score for peak cough flow(6 months)