Clinical Trial: Efficacy of Noninvasive Ventilation in Amyotrophic Lateral Sclerosis (ALS)

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Prevalence and Severity of Nocturnal Oxygenation and Ventilation Failure in Patients With Amyotrophic Lateral Sclerosis Using Noninvasive Ventilation

Brief Summary: 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.

Detailed Summary: 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.
Sponsor: Columbia University

Current Primary Outcome:

  • Tolerance of bilevel PAP [ Time Frame: Up to 3 months ]
    The ALS Functional Rating Scale — Revised (ALSFRS-R) will be administered to measure tolerance.
  • Score on Epworth Sleepiness Scale [ Time Frame: Up to 3 months ]
    Objective evidence of nocturnal sleep-disordered breathing on the bilevel PAP will be measured.


Original Primary Outcome:

Current Secondary Outcome:

Original Secondary Outcome:

Information By: Columbia University

Dates:
Date Received: September 27, 2007
Date Started: May 2007
Date Completion:
Last Updated: July 2, 2015
Last Verified: July 2015