Everybody seems to be getting in on the act with the Ice Bucket Challenge for ALS research funds.
While most have heard about the Ice Bucket Challenge to raise funds for ALS, many don’t know a lot about this disease.
Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive, neuromuscular disease. It attacks the motor neurons that transmit electrical impulses from the brain to the voluntary muscles in the body. When they fail to receive messages, the muscles lose strength, atrophy and die.
The disease is sometimes called Lou Gehrig’s disease. Many years ago, Lou Gehrig was known as pro baseball’s Ironman. With strength, agility, and excellent health, Gehrig had everything it took to become a sports legend. At the peak of his career, he was diagnosed as having Amyotrophic Lateral Sclerosis (ALS) and died two years later at 39.
ALS can strike anyone at anytime, regardless of age, gender or ethnic origin. It does not affect the senses and only rarely does it affect the mind. The equipment costs for each patient average $137,000, while nursing and home care costs can be up to 10 times that amount. The average life expectancy after diagnosis is three to five years.
According to the National Institute of Neurological Disorders and Stroke, (NINDS) the onset of ALS may be so subtle that the symptoms are overlooked. The earliest symptoms may include fasciculations, cramps, tight and stiff muscles (spasticity), muscle weakness affecting an arm or a leg, slurred and nasal speech or difficulty chewing or swallowing. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS.
According to NINDS, “The parts of the body showing early symptoms of ALS depend on which muscles in the body are affected. Many individuals first see the effects of the disease in a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing or turning a key in a lock.
“In other cases, symptoms initially affect one of the legs, and people experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. Others first notice speech problems, termed “bulbar onset” ALS,” NINDS says.
It adds regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses.
“Individuals may develop problems with moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes (hyperreflexia) including an overactive gag reflex.
“An abnormal reflex commonly called Babinski’s sign (the large toe extends upward as the sole of the foot is stimulated in a certain way) also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fasciculations.”
To be diagnosed with ALS, people must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, NINDS says, eventually individuals will not be able to stand or walk, get in or out of bed on their own or use their hands and arms.
Difficulty swallowing and chewing impair the person’s ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem.
Health care professionals need to explain the course of the disease and describe available treatment options so that people can make informed decisions in advance. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. They eventually lose the ability to breathe on their own and must depend on ventilatory support for survival.
In searching for the cause of ALS, researchers are also studying the role of environmental factors such as exposure to toxic or infectious agents, as well as physical trauma or behavioral and occupational factors.