Most people know something about Lou Gehrig, and how he faced a devastating disease with extraordinary courage. Yet few know the exact nature of the disease or its real name: Amyotrophic Lateral Sclerosis (ALS).
ALS, first described in 1869, is a progressive disease shared by as many as 30,000 Americans. Deterioration of motor neurons and pathways in the brain and spinal cord disrupt connections to muscles throughout the body, causing increasing paralysis. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. The progressive degeneration of the motor neurons in ALS eventually lead to death.
As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. 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.
With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. Although people with ALS may progress to the point where they are unable to control or use their voluntary muscles, for most people their minds and sensory nerves remain unaffected.
While ALS is presently incurable, the situation is not hopeless. The Providence ALS Center team is working diligently to better understand the disease's cause, potential cures and possible means of prevention.
Despite the serious nature of ALS, there are numerous people who live with ALS for many years, even decades, with a high quality of life. Some people have a form of ALS that is very slowly progressing. Others choose medical therapies and devices that help maintain mobility, nutrition and breathing.
