Mechanical Insufflation in the Philadelphia Amyotrophic Lateral Sclerosis Cohort (MI-PALS) Study
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 23
- 试验地点
- 2
- 主要终点
- peak cough flow (PCF)
研究概览
简要总结
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Supportive Care
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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.
排除标准
- •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
- •Recent barotrauma
- •History of emphysema of any kind (including bullous emphysema)
- •History of or known susceptibility to pneumothorax
- •History of or known susceptibility to pneumomediastinum
- •Chronic obstructive pulmonary disease
- •Uncontrolled asthma (defined as recent exacerbation requiring corticosteroids in the previous 30 days)
- •Symptomatic cardiomyopathy (heart failure) with left ventricular ejection fraction less than 50%
- •History of right heart failure or pulmonary hypertension
- •Current smoker or tobacco use within the last 30 days.
研究组 & 干预措施
Mechanical insufflation
Participants will be asked to perform mechanical insufflation using the BiWaze cough device as 5 sets of 5 insufflations twice daily for 6 months.
干预措施: Mechanical insufflation (Device)
结局指标
主要结局
peak cough flow (PCF)
时间窗: 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.
次要结局
- Transcutaneous carbon dioxide(6 months)
- 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)
- ALS Functional Rating Scale - Revised (ALSFRS-R) dyspnea and orthopnea scores(6 months)
- Global rate of change score for peak cough flow(6 months)