WELCOME TO THE ALS CARE PROJECT
If you, a loved one, or your ALS patient has been diagnosed with ALS, you may CONTACT US for information or assistance from anywhere in the USA or world. Nursing consultation includes assessment of patient problems; nursing recommendations; education on options for breathing and living; and referral to appropriate multidisciplinary healthcare providers for best management of care and patient outcomes.
WHAT PEOPLE NEED TO KNOW ABOUT ALS
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Amyotrophic lateral sclerosis (ALS) is a motor neuron disease in adults that invariably results in respiratory failure unless prevented and treated with noninvasive or invasive breathing support and adequate ventilation is maintained.
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Noninvasive ventilation (NIV) is the standard practice of breathing support for ALS.
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NIV can be tolerated unless oral secretions become severe due to bulbar impairment (resulting in the progressive inability to swallow saliva). Therefore, use of NIV is temporary, short-term treatment for those with bulbar ALS.
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In contrast, tracheostomy and invasive ventilation (TIV) can be tolerated in people with ALS, despite the severity of oral secretions, and may extend survival well beyond respiratory failure. Thus, use of TIV in persons with ALS is referred to as long-term mechanical ventilation (LTMV). ALS itself is not fatal, if TIV maintains adequate ventilation and complications are avoided.
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People with ALS who are nonbulbar (having the ability to talk, swallow and clear the throat) do not need tracheostomy and invasive breathing support.
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The validated Oral Secretion Scale (OSS) predicts tolerance of NIV in people with ALS. Survival correlates with NIV tolerance and hours per day that NIV is used. The Oral Secretion Scale reliably signals when to initiate hospice or transition to tracheosotomy ventilation.
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NIV users with nonbulbar ALS do not need a tracheostomy when upper-airway clearance is maintained.
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Cazzolli PA, Brooks BR, Nakayama, Y, Lewarski JS, McKim DA, Holt SL, Chatburn RL. The Oral Secretion Scale and prognostic factors for survival in subjects with amyotrophic lateral sclerosis. Respir Care 2020; 65(8):1063-1076.
MECHANICS OF RESPIRATION
ALS AFFECTS THE MUSCLES OF RESPIRATION, THE “VENTILATORY PUMP MUSCLES”
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ALS ultimately results in the progression of respiratory muscle weakness and under-ventilation of the lungs.
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Respiratory failure will occur unless prevented and treated with use of noninvasive or invasive breathing support to maintain adequate ventilation of the lungs.
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People with ALS have choices for breathing and living and can live beyond respiratory failure, if they so choose.
** The diaphragm is the principal muscle of inspiration for breathing in.
Breathing out is ordinarily a passive process.**
INCIDENCE OF ALS
The Incidence of Amyotrophic Lateral Sclerosis in Ohio 2016-2018: The Ohio Population-Based
ALS Registry
Andrew AS, Pioro EP, Li M, Shi X, Gui J, Stommel EW, Butt TH, Peipert D, Henegan P, Tischbein, Cazzolli P, Novak J, Quick A, Pugar K, Sawlani K, Katirji B, Hayes T, Horton D, Mehta P, Bradley WG. The incidence of amyotrophic lateral sclerosis in Ohio 2016-2018: the Ohio population-based ALS registry. Neuroepidemiology. DOI: 10.1159/000515103.
WHAT CAUSES ALS?
Risk Factors for Amyotrophic Lateral Sclerosis: A Regional United States Case-Control Study
Andrew A, Bradley W, Peipert D, Butt T, Amoako K, Pioro E, Tandan R, Novak J, Quick A, Pugar K, Sawlani K, Katirji B, Hayes T, Cazzolli P, Gui J, Mehta P, Horton D, Stommel E. Risk factors for amyotrophic lateral sclerosis: a regional United States case-control study. Muscle & Nerve 2021;63:52-59. DOI: 10.1002/mus.27085
PATIENT RIGHTS
We believe that all ALS patients have the right to accurate, necessary and understandable information on ALS and options for living. People should know that ALS ultimately results in progressive respiratory muscle weakness and that breathing failure may be prevented or treated if desired. All people with ALS should be given the choice to live, or if they so choose, to refuse or stop life-sustaining treatment and be given the right to comfort care.
