Sam Shepard and Amyotrophic Lateral Sclerosis

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Dr. Gabe Mirkin
Dr. Gabe Mirkin

Sam Shepard was a prolific playwright, actor, screenwriter and director who:

• acted in more than sixty films and was nominated for an Academy Award for Best Supporting Actor for his portrayal of pilot Chuck Yeager in The Right Stuff

• wrote more than 55 plays, often focusing on the serious problems that occur in American family life;

• won the most Obie Awards (10) for his off-Broadway writing and directing.  In 1979 he received a Pulitzer Prize for his play, Buried Child, and New York Magazine called him “the greatest American playwright of his generation.”  

In his late sixties, he developed amyotrophic lateral sclerosis (ALS), the disease that killed baseball great Lou Gehrig at age 37.  Shepard died from complications of ALS on July 27, 2017, at age 73. 

A Difficult Life 

Sam Shepard
Sam Shepard

He was born on November 5, 1943, in Fort Sheridan, Illinois.  His “dysfunctional family” served as a basis for characters in many of his plays. His father was a United States Army Air Force bomber pilot during World War II who was also an alcoholic and an abusive husband and father.  His loving, supportive mother, a teacher, offset some of the pain and abuse he suffered from his father. In his early years, the family had to move every two years because of army transfers. Later his father left the service and bought an avocado farm in Duarte, California. Shepard briefly studied animal husbandry at nearby Mt. San Antonio College, but soon left school to move to New York City, where he worked as a busboy, played in a psychedelic folk band and tried to break into the theater.    

At age 35, his acting career took off  when he won a role in Terrence Malick’s Days of Heaven, with Richard Gere and Brooke Adams. At the same time, he continued to write successful plays and in 1986 (age 43) he was elected to the American Academy of Arts and Letters. 

Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig’s Disease)

In his last few years, Shepard suffered privately from ALS, but he described his experience in his last book, The One Inside.  One of the characters said that he couldn’t get up from bed in the morning and felt as though his limbs weren’t connected to “the motor” driving his body. “They won’t take direction — won’t be dictated to — the arms, legs, feet, hands. Nothing moves. Nothing even wants to. The brain isn’t sending signals.” 

ALS is a progressive disease that destroys the nerves that move voluntary muscles.  More than 6,000 people in the United States are diagnosed with ALS each year.  Nobody knows the cause and there is no cure. The brain is supposed to send messages to nerves in the spinal cord which transmit messages to the nerves that move muscles.  When a muscle loses its nerve control, it starts to twitch and can waste away to nothing. Early symptoms of ALS include 

• muscle weakness 

• twitching

• slurred speech

• inability to chew food

• tripping or stumbling.

The first sign could be difficulty buttoning a shirt, writing, or turning a key in a lock.   The disease usually does not affect a person’s ability to think and reason, so affected people are terribly disturbed by their lack of ability to control their voluntary muscles. As the disease progresses, a person loses the ability to speak, eat, walk and eventually breathe.  The most common cause of death is inability to breathe, which typically occurs about 3-5 years after symptoms start. Only about ten percent of affected people live more than ten years after first being diagnosed.

Risk Factors and Diagnosis

The disease usually starts between the ages of 55 and 75, but there are no known specific risk factors.  Military veterans appear to be twice as likely as non-veterans to develop ALS.  Possible causes could be exposure to occupational or environmental toxins such as lead or pesticides, infections or  trauma. Family history does not appear to predict the disease. 

There are no specific tests to diagnose ALS.  It is usually diagnosed by a history of the symptoms, physical examination and ruling out other causes. 

Current Treatments and Research

The U.S. Food and Drug Administration (FDA) has approved riluzole (Rilutek) and edaravone (Radicava) to treat ALS.  These drugs offer no hope for a cure, but Riluzole appears to protect nerves by decreasing glutamate, the chemical messenger for nerves that innervate muscles. Intravenous edaravone possibly slows loss of muscle function, but it costs $1,086 per infusion or a yearly cost before government discount of $145,524.  Another drug under European review is being developed by French drug maker AB Science SA (ABS.PA).  Since there is no cure, all patients should receive physical therapy and speech therapy because inactivity itself causes loss of muscle function.  

Since ALS is caused by the death of nerve cells that cause muscles to contract, the most promising line of research is through stem cells.  Stem cells are young cells that can become any type of tissue. Treatment in the future may be to program stem cells to become nerve cells that innervate muscles and then inject them into areas where the nerve cells have already died.

Dr. Gabe Mirkin is a Villager. Learn more at www.drmirkin.com

1 COMMENT

  1. My Father contacted this disease in his late 60’s and passed about 3 years after Diagnosis . Dad had 9 siblings and none were affected with this . other factors you mentioned were not present but ironically at the time Swine flu shots were being given and after speaking with others their relative also received that shot .