Baseball great Lou Gehrig was only 35 years old in 1938 when he first noticed muscle weakness was affecting his hitting, running and throwing ability. He was diagnosed with amyotrophic lateral sclerosis (ALS) in 1939, and retired from the New York Yankees that year. He died of the disease in 1941.

“Lou Gehrig’s disease,” as ALS is commonly called, affects the nerve cells in the brain and spinal column. The first symptom is often weakness and lack of control of the legs, feet, hands and arms. Some people with ALS may first notice difficulty swallowing, speaking or breathing. As nerve cells in the brain and spinal cord deteriorate over the course of the disease, ALS patients progressively lose the ability to control their muscles. Over time, the muscles shrink from disuse.

People with ALS eventually lose the ability to walk, use their hands and arms, and to care for themselves. They may lose the ability to speak. Later in the disease, the muscles that control breathing are affected, and patients need a mechanical ventilator to stay alive.

Most ALS patients die within a few years of diagnosis; about 20 percent live longer than five years and 10 percent live longer than 10 years.

ALS does not affect a person’s intellect, nor their ability to see, hear and feel. Since their ability to think is unaffected, ALS patients can learn to communicate in alternative ways. Advances in computer software have enabled people with ALS to continue to communicate and be productive for longer than was formerly possible.

ALS strikes about two out of every 100,000 people worldwide. The ALS Association reports about 5,000 people in the United States are diagnosed with ALS each year. Most people with ALS are diagnosed between ages 40 and 70, but it has been found in people as young as 12 years and as old as 98 years.

Ninety percent of ALS cases occur randomly. The other 10 percent of cases are familial, meaning a defective gene is passed on from parent to child. Men and women are equally affected. No environmental or infectious cause has been found.

There is no one test used to diagnose ALS. If ALS is suspected, a neurologist will do a physical examination, then may order blood tests, an MRI or CT scan of the head, electromyogram (to measure the strength of electrical signals to the muscles) and/or a muscle biopsy.

At this time, there are only a few medications that have been found useful for ALS patients. One is called Riluzole; it is not a cure, nor does it reverse the progression of the disease, but it may prolong the life of ALS patients. Other drugs may be used to control the symptoms of ALS, such as muscle spasticity, cramps or difficulty swallowing saliva.

Due to difficulty swallowing, some ALS patients opt for a gastrostomy tube, which allows them to get nutrition directly to their stomach without swallowing.

As the ability to walk is lost, most ALS patients use wheelchairs or other assistive devices. Physical therapy, respiratory therapy, speech therapy and massage are all used and can improve the quality of life for ALS patients.

Usually, the most important decision ALS patients and their families face is whether or not to use a mechanical ventilator. As ALS progresses, the breathing muscles weaken. Without a ventilator, ALS patients eventually die painlessly of respiratory failure.

Amyotrophic lateral sclerosis is one of the most devastating diseases a person can get. Coping with the physical, emotional and practical aspects of this disease is the most difficult challenge a person and their family and friends will face.

Kathryn B. Brown is a family nurse practitioner with a master’s degree in nursing from OHSU. Is there a health topic you would like to read about? Send ideas to kbbrown@eastoregonian.com.