BEST PRACTICES OF ALS CARE
BASED ON CLINICAL EVIDENCE AND EXPERIENCE
TO EDUCATE ON
CDC ALS RESEARCH STUDY
In search of potential risk factors for causing ALS
WELCOME TO THE ALS CARE PROJECT
ALS (amyotrophic lateral sclerosis) is a degenerative disease of the motor neurons in adults. ALS is nicknamed “Lou Gehrig’s Disease” after the New York Yankee baseball star whose career ended in 1939. Motor neurons are the motor nerve cells in the cerebral cortex of the brain, the bulbar-region of the brain stem, and the anterior gray matter in the spinal cord. Motor neurons extend their fibers (called axons) from the brain down through tracts in the spinal cord and from the spinal cord to the skeletal muscles. Motor neurons contract skeletal (voluntary) muscles for moving the body, including the respiratory muscles for moving air in and out of the lungs.
Motor neuron degeneration causes weakness of the affected skeletal muscles. As a result, people with ALS may have difficulty walking, using the arms and hands, talking and swallowing. However, not all people with ALS lose the ability to walk, talk, or swallow. Invariably, the respiratory muscles become affected. This may occur at the disease onset or any time after the onset. Respiratory failure will eventually ensue unless prevented or treated by using noninvasive or invasive breathing support.
ALS can strike any adult of any age. The average ages of onset are 40’s to 70’s. The incidence of ALS is only an estimation. Tracking the accurate incidence of the disease has been challenging due to the widely scattered ALS population and multiple clinical settings where people with ALS are diagnosed. Other reasons are unawareness of the National ALS Registry or unwillingness of ALS patients to enroll in the registry. The Ice Bucket Challenge of 2014 demonstrates that ALS may not be as rare as reported in the literature. It is important for every person who is diagnosed with ALS in the USA to register with the National ALS Registry to assure being counted in the ALS census.
Currently, there is no treatment to stop ALS. Although ALS is incurable, the symptoms are manageable. Education on best practices of ALS care is necessary for achieving best patient outcomes. People with ALS have choices for breathing and living. ALS itself is not fatal when breathing support is used and adequate ventilation of the lungs is maintained.
MECHANICS OF RESPIRATION
ALS AFFECTS THE MUSCLES OF RESPIRATION, THE “VENTILATORY PUMP MUSCLES”
ALS ultimately results in the progression of respiratory muscle weakness and under-ventilation of the lungs.
Respiratory failure will occur unless prevented and treated with use of noninvasive or invasive breathing support to maintain adequate ventilation of the lungs.
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
Tracking the accurate incidence of the disease has been challenging due to the widely scattered ALS population and multiple clinical settings where people with ALS are diagnosed. The Ice Bucket Challenge of 2014 demonstrates that ALS may not be as rare as reported in the literature. It is important for every person who is diagnosed with ALS in the USA to register with the National ALS Registry to assure being counted in the ALS census.
WHAT CAUSES ALS?
Factors that may potentially cause ALS are being explored. These include: genetic, environmental, infectious (i.e., viral, prion), autoimmune, physical activity, trauma, and lifestyle factors. According to investigators, genetics may play a larger role in causing ALS than previously believed. Although there is no treatment to stop ALS, the symptoms are manageable.
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.