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ALS: A Famous Disease • “Fans, for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of this earth. I have been in ballparks for seventeen years and have never received anything but kindness and encouragement from you fans.” – Lou Gehrig
What is ALS? Lou Gehrig's disease is a disorder that's also called Amyotrophic Lateral Sclerosis (say: ah-my-uh-tro-fik lah-tuh-rul skluh-ro-sis), or ALS. The official name comes from these Greek words: "a" for without "myo" for muscle "trophic" for nourishment "lateral" for side (of the spinal cord) "sclerosis" for hardening or scarring
In Easier Terms • So, amyotrophic means that the muscles have lost their nourishment. When this happens, they become smaller and weaker. Lateral means that the disease affects the sides of the spinal cord, where the nerves that nourish the muscles are located; and sclerosis means that the diseased part of the spinal cord develops hardened or scarred tissue in place of healthy nerves. • ALS is often called Lou Gehrig's disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s.
What does it do? • Over time, Lou Gehrig's disease causes these motor neurons in the brain and spinal cord to shrink and disappear, so that the muscles no longer receive signals to move. As a result, the muscles become smaller and weaker. Gradually the body becomes paralyzed (say: par-uh-lized), which means that the muscles no longer work. However, someone with ALS, even at an advanced stage, can still see, hear, smell, and feel touch. The nerves that carry feelings of hot, cold, pain, pressure, or even being tickled, are not affected by Lou Gehrig's disease. The parts of the brain that allow us to think, remember, and learn are also not affected by the disease.
Who does it impact? • Although this disease can strike anyone, it is extremely rare in kids. According to the ALS Association, most people who develop Lou Gehrig's disease are adults between 40 and 70. Only 2 out of every 100,000 people will get the disease each year. Because it is not contagious, you can't catch ALS from someone who has the disease. • Among ALS cases in the United States, 5% to 10% are hereditary, which means the disease runs in certain families. This is called familial (say: fuh-mee-lee-ul) ALS. At least 90% of cases are not inherited; this is called sporadic (say: spuh-rah-dik) ALS.
Symptoms and Diagnosis • The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; 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. • The parts of the body affected by early symptoms of ALS depend on which muscles in the body are damaged first. In some cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Some patients first see the effects of the disease on 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. Other patients notice speech problems. • 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. Patients have increasing problems with moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (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 fleeting twitches of muscles that can be seen under the skin (fasciculations). • To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
Treatment Options • Currently, there's no way to prevent or cure Lou Gehrig's disease, but a number of treatments are available to people with the disease. Medicines can control symptoms, such as muscle cramping and difficulty swallowing, and other drugs can slow the development of the disease. • Physical therapy can help people with ALS cope with muscle loss and breathing problems. Special equipment is also provided when it becomes necessary. For instance, a power wheelchair can enable a paralyzed person with ALS to get around. A machine called a ventilator (say: ven-til-ay-ter) can help someone breathe.
