Polysomnography-directed Noninvasive Ventilation in Amyotrophic Lateral Sclerosis (ALS)

Not Applicable

Terminated

Interventions: Other: Sleep study-guided adjustment of NIV
Other: Standard initiation of NIV

Brief Summary: Use of noninvasive ventilation (NIV, also known colloquially as "Bipap") has been associated in some studies with improvement in pulmonary function, quality of life and survival. NIV is typically applied during sleep, and without the benefit of sleep study to determine the optimal settings. The investigators have shown that when NIV is used in this fashion, failure of nocturnal oxygenation and ventilation is prominent. This study is randomizing patients to standard application of NIV vs application guided by use of sleep study data to determine the effect of titrated therapy on pulmonary function, quality of life and survival.

Recruitment & Eligibility

Inclusion Criteria

Diagnosis of probable or definite Amyotrophic Lateral Sclerosis (ALS) per El Escorial criteria
Between ages of 18 and 80 yrs old

Exclusion Criteria

Inability to clear secretions from the airway
Life expectancy < 6 months from a comorbid condition
Dementia sufficient to impair ability to use NIV, perform respiratory muscle pressure testing (PFTs), or complete Health-related Quality of Life (HRQOL) instruments
Inability to follow up at the ALS Center on a regular basis
Previously diagnosed obstructive Sleep Apnea

Arm && Interventions

Group	Intervention	Description
Sleep study titrated NIV	Sleep study-guided adjustment of NIV	ALS subjects in this arm, who are offered NIV for Forced Vital Capacity (FVC) \<50% as per AAN Practice Parameters, will have their initial level of NIV determined polysomnographically. They will be followed with sleep studies at 1 month, 3 months and 6 months to reassess NIV efficacy and NIV will be adjusted as necessary to optimize parameters of oxygenation and ventilation.
Standard NIV	Standard initiation of NIV	Noninvasive ventilation (NIV) will be initiated and managed as per current standard of practice guided by the American Academy of Neurology (AAN) Practice Parameters (updated in 2009), in all subjects with amyotrophic lateral sclerosis (ALS) and a forced vital capacity of \<50% predicted. Sleep studies will be performed at baseline, 2 weeks, 1, 3 and 6 months, but will not influence management of the NIV.

Primary Outcome Measures

Name	Time	Method
Change from baseline in spirometric, respiratory muscle strength, and gas exchange measures	Up to 6 months after starting NIV	FVC (forced vital capacity), FEV1 (forced expiratory volume in 1 second), MIP (maximum inspiratory pressure) and MEP (maximum expiratory pressure

Secondary Outcome Measures

Name	Time	Method
Duration that the Mental Component Summary (MCS) is maintained above 75% of baseline score for the Medical Outcomes Study Health Survey (SF-12)	Up to 6 months after starting NIV
Survival	Up to 6 months after baseline	Whether sleep study-titrated NIV is associated with trends to improved survival, compared with standardly prescribed (non-titrated) NIV
Nocturnal oxygenation and ventilation	Up to 6 months after starting NIV	Nadir oxygen saturation, number of oxygen desaturations of 3%/hr (ODI3), % time of sleep spent with oxygen saturation \<90%, apnea-hypopnea index, asynchrony index
Modified Borg dyspnea score (see description)	Up to 6 months after baseline	The Modified Borg Dyspnea Scale is a 10-point Likert scale asking subjects to rate perceived shortness of breath, ranging from 0 (no breathlessness at all) to 10 (maximal breathlessness)

Locations (1): Columbia University Medical Center
🇺🇸
New York, New York, United States

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