An observational study in Amyotrophic Lateral Sclerosis and Motor Neuron Disease, sponsored by Columbia University. Completed at 1 site in United States. Open to participants aged 18 Years to 80 Years. Per ClinicalTrials.gov, last updated 2015-07-07.
Sponsored by Columbia University · Observational
This study will test the hypothesis that noninvasive ventilation (NIV) as prescribed in current medical practice for use in amyotrophic Lateral Sclerosis (ALS) patients fails to deliver adequate breathing support over a night of use in the patient's home. ALS patients who come to the ALS Center for their routine 3 month follow up exam and are currently using NIV will be asked to complete questionnaires regarding their quality of sleep, quality of life and general level of function, and to undergo a home sleep study, using a safe, comfortable and reliable breathing monitoring system during a night of sleep. If the questionnaires or the sleep study show failure of the breathing device, the investigators will work with the patient to fix the problem and then offer a second study to make sure that the changes were helpful. The results of this study may help to develop subsequent studies and to improve the guidelines used for care of ALS patients.
Amyotrophic Lateral Sclerosis (ALS), also known as "Lou Gehrig's Disease", is a fatal disorder that causes breathing failure due to progressive weakness of the muscles of breathing. Breathing assist devices known as noninvasive ventilation (NIV) are offered to ALS patients when their breathing function worsens. These devices deliver breathing assistance via a mask on the nose or nose and mouth, and are thought to be particularly important to be used during sleep, when breathing often becomes more shallow and irregular. However, although these devices have become the standard of therapy in ALS patients once their lung function worsens, it remains unclear how effective these devices actually are when a patient is sleeping, partly because of the practical difficulties in applying the device properly and keeping it applied throughout the sleep period, and partly because they are most commonly prescribed without objective evidence regarding how much breathing support the patient needs as the disease progresses and the breathing muscles weaken further.
717 studies on the registry are indexed under Motor Neuron Disease; 137 are open to participants now.
This study's enrollment of 25 is below the median of 109 across 217 observational studies indexed under Motor Neuron Disease.
Browse Motor Neuron Disease studies →Columbia University is the lead sponsor of 1,103 studies on the registry; 193 are open to participants now.
Of its 172 completed or terminated interventional studies of FDA-regulated products, 142 (83%) have results posted.
Counted across the registry records on this site, refreshed daily.
Patients with ALS who are using nocturnal noninvasive ventilation to treat respiratory insufficiency will be screened for eligibility as below.
Exclusion Criteria:
Tolerance of bilevel PAP
The ALS Functional Rating Scale - Revised (ALSFRS-R) will be administered to measure tolerance.
Time frame: Up to 3 months
Score on Epworth Sleepiness Scale
Objective evidence of nocturnal sleep-disordered breathing on the bilevel PAP will be measured.
Time frame: Up to 3 months
This study is completed, as verified in Jul 2015. You cannot join it, but the record below documents what was studied.
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Columbia University