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.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
SUPPORTIVE_CARE
Masking
NONE
Enrollment
20
Mechanical insufflation (MI) is a chest physiotherapy exercise that will be performed using a type of mechanical insufflator-exsufflator (MI-E) known as the BiWaze Cough device. The device connects to a tube that can interface with a patient using either a facemask or mouthpiece. Mechanical insufflation is a chest physiotherapy exercise that passively inflates the chest with positive pressure that is delivered in coordination with the patient's own inspiratory timing until maximal inflation capacity (MIC), determined by the patient or maximal chest rise on visual inspection. At MIC, the patient passively exhales, which completes one "cycle". Prior literature has used a "dose" of 5 sets of 5 cycles once or twice daily. The maneuver is usually performed with assistance of a caregiver to hold the mask or mouthpiece in place.
University of Pennsylvania
Philadelphia, Pennsylvania, United States
RECRUITINGpeak cough flow (PCF)
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.
Time frame: 6 months
Forced vital capacity (FVC)
Defined as maximal volume, in liters, that a person can exhale forcefully after a complete inhalation. Measured using a spirometer connected to a face mask or mouthpiece in the sitting and upright position.
Time frame: 6 months
Maximal inspiratory pressure (MIP)
Defined as the maximum pressure, in cmH2O, generated when the subject exhales as much air as possible and then immediately inhales as forcefully as possible.
Time frame: 6 months
Maximal expiratory pressure (MEP)
Defined as the maximum pressure, in cmH2O, generated when the subject inhales as much air as possible and then immediately exhales as forcefully as possible. Measured using a hand-held manual or digital manometer connected to a mouthpiece or mask in the sitting and upright position.
Time frame: 6 months
Maximum insufflation capacity (MIC)
Defined as exhaled volume, in liters, immediately following a MI maneuver to maximum insufflation capacity. Measured using a spirometer connected to a face mask or mouthpiece, in the sitting and upright position.
Time frame: 6 months
Maximum insufflation capacity assisted peak cough flow
Defined as the peak cough flow generated from MIC. Lungs are inflated to MIC using MI and before the subject exhales they insert a peak flow meter in their mouth and follow with a peak cough flow as described above.
Time frame: 6 months
MIC-FVC difference
defined by subtracting the FVC from the MIC
Time frame: 6 months
Transcutaneous carbon dioxide
Defined as the average value in mmHg from a daytime in-clinic measurement over a 15-minute transcutaneous recording. Measured using a Sentec transcutaneous digital monitoring system while the subject is sitting upright in a chair or wheelchair. The transcutaneous sensor can be placed on the subject forehead, cheek, or earlobe.
Time frame: 6 months
ALS Functional Rating Scale - Revised (ALSFRS-R) dyspnea and orthopnea scores
The ALSFRS-R is a 12-item standardized questionnaire to assess the motor function status of an individual with ALS. The ALSFRS-R is assessed by a research staff member who has been certified for performance of the ALSFRS-R. The dyspnea score is one of the questions focusing on level of shortness of breath, scored on a scale of 0 to 4, with 4 being no symptoms at all and 0 being severely symptomatic with consideration of mechanical respiratory support. The orthopnea score is one of the questions focused on breathing symptoms and difficulty sleeping while lying supine, scored on a scale of 0 to 4, with 4 being no symptoms at all and 0 being severely symptomatic with inability to sleep.
Time frame: 6 months
Global rate of change score for peak cough flow
The global rate of change score for the peak cough flow will ask patients how effective they think their cough is today by rating it on a Likert scale from -7 (extremely impaired) to +7 (extremely strong).
Time frame: 6 months
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