Early Treatment of Amyotrophic Lateral Sclerosis (ALS) With Nutrition and Non-Invasive Positive Pressure Ventilation
Amyotrophic Lateral Sclerosis
About this trial
This is an interventional supportive care trial for Amyotrophic Lateral Sclerosis focused on measuring amyotrophic lateral sclerosis, ALS, Lou Gehrig's disease, nutrition, NIPPV, non-invasive positive pressure ventilation, percutaneous endoscopic gastrostomy, PEG
Eligibility Criteria
Inclusion Criteria: Willing and able to give informed consent. If women of childbearing age, must be non-lactating and surgically sterile or using an effective method of birth control (double-barrier or oral contraception) and have a negative pregnancy test. Minorities: All races and ethnic backgrounds. Disease inclusion: Clinically possible/definite/probable, or laboratory supported probable, sporadic or familial ALS according to the revised Escorial criteria. Onset of progressive weakness within 60 months prior to study. Willing to return for visits as scheduled and adhere to protocol requirements. FVC Criteria NIPPV Arm: Best-sitting FVC between 50% and 95% of predicted normal. Nutrition Arm: Best-sitting FVC >50% of predicted normal. Exclusion Criteria: Untreated or unstable hyper or hypothyroidism, untreated or unstable asthma, unstable angina, advanced liver or renal disease, advanced cancer, untreated diabetes or chronic obstructive pulmonary disease (COPD). Diagnosis of other motor neuron or other neurological disorder that mimics ALS. Diagnosis of other neurodegenerative disease (i.e., Parkinson's disease, Alzheimer's disease, etc.) Inflammatory bowel disease or malabsorption syndrome. Use of NIPPV, mechanical ventilation, or tracheostomy at the time of consent. Inability to adhere to study visit schedule or lack of reliable caretaker if participant requires one. Pregnant or lactating woman.
Sites / Locations
- University of Colorado
- University of Miami
- University of Kentucky
- Henry Ford Hospital
- Beth Israel
- Columbia University
- SUNY
- Pennsylvania State University
- Drexel University
- University of Texas-San Antonio
- University of Utah
- University of Vermont
Arms of the Study
Arm 1
Arm 2
Arm 3
Experimental
Active Comparator
Active Comparator
Early NIPPV Intervention
Standard of Care NIPPV
Standard of Care NIPPV and Nutritional Monitoring
Participants with >80% predicted forced vital capacity (FVC).
Participants with 50-74% predicted forced vital capacity (FVC).
Participants with 50-95% forced vital capacity (FVC), and normal or impaired amyotrophic lateral sclerosis functional rating scale (ALSFRS) scores. Participants in this group will receive standard of care NIPPV therapy but will also undergo detailed analysis of nutritional status.