ALS
Amyotrophic Lateral Sclerosis Lou Gehrig's Disease
What is ALS
ALS, amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
Cells Illustration
The onset of ALS is insidious with muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look "thinner" as muscle tissue atrophies. The body has many kinds of nerves. There are those involved in the process of thinking, memory, and of detecting sensations (such as hot/cold, sharp/dull), and others for vision, hearing, and other bodily functions. The nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle power. Examples of voluntary movements are you're making the effort to reach for the phone or step off a curb; these actions are controlled by the muscles in the arms and legs. The heart and the digestive system are also made of muscle but a different kind and their movements are not under voluntary control. When your heart beats or a meal is digested, it all happens automatically. Therefore, the heart and digestive system are not involved in ALS. Breathing also may seem to be involuntary. Remember, though, while you cannot stop your heart, you can hold your breath - so be aware that ALS may eventually have an impact on breathing. Approximately 5,600 people in the U.S. are diagnosed with ALS each year. The incidence of ALS is two per 100,000 people, and it is estimated that as many as 30,000 Americans may have the disease at any given time. ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries. ALS can strike anyone. [10% is hereditary, for 90% there is no known cause]. Life expectancy of an ALS patient averages about two to five years from the time of diagnosis. [some less, some more, how it progresses and affects any individual is unpredictable, including pain, gastrointestinal issues, side effects of medications, etc.] While there is not a cure or treatment today that halts or reverses ALS, there is one FDA approved drug, riluzole, that modestly slows the progression of ALS as well as several other drugs in clinical trials that hold promise. Also, Northwestern University has recently published exciting news regarding the cause of ALS. There can be significant costs for medical care, equipment, and home health caregiving later in the disease.