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Stephen Hawking, Genius with ALS

stephen HawkingOne of the world's greatest theoretical physicists died on March 13, 2018 at age 76. In spite of suffering from Amyotrophic Lateral Sclerosis (ALS) which left him able to move only a few muscles in the side of his face, he opened new ground on how we view the origin and possible end of the universe. He defined "black holes" as we know them today. 
• Max Planck (1858-1947) had shown that solid particles could be converted to and from energy.
• Then Albert Einstein (1879-1955) created his "theory of relativity" that there was a mathematical relationship between mass and energy (E=MC2).   
• Niels Bohr, Louis de Broglie, Erwin Schrodinger and Paul M. Dirac solidified quantum theory that suggested that space and time began with a "big bang" that formed "black holes" 15 billion years ago.
• Then Stephen Hawking showed that "black holes" emit radiation and therefore have a limited existence of usually 10 to the 64th power years, many trillions of times the accepted age of the universe. 
 
The old theory was that when a star much larger than our sun uses up its nuclear fuel, it has to collapse and form a "singularity," a black hole that is infinitely dense and has infinite gravity that prevents anything, including light, from getting out.  However, Hawking did not agree that nothing could escape from black holes. He believed that black holes would allow solid material to emit radiation and therefore would constantly lose mass and eventually disappear, and their mass would be transferred to other black holes (Nature. March, 1974;248:30–31). Upon showing that black holes emit radiation, he wrote, "as a black hole shrinks, they get hotter and hotter and finally explode."  However, he believed that black holes usually last many trillions of times the proposed age of the universe.  He also postulated that the universe has no beginning and no end.  In his later years, Hawking concluded that the universe has no edge or boundary and no beginning or end.  He believed that "the universe would not be created, not be destroyed; it would simply be." 
 
His Formative Years
Hawking was born in Oxford, England on January 8, 1942, exactly 300 years after the death of Galileo.  He finished in the middle of his class in secondary school, but did extremely well on his entrance exam and was accepted at Oxford.  He majored in physics, partied most of the time, and in spite of having little athletic talent was the coxswain on a club crew.  Even though he studied very little, he was graduated with high honors and went on to graduate school at Cambridge University.  
 

Gradual Loss of Muscle Control
In 1963 at age 21, he was diagnosed with Amyotrophic Lateral Sclerosis (ALS), also called Lou Gehrig's Disease, in which the nerves break down progressively so people lose all control of their muscles and within a couple years, they are unable to eat or breathe and they often smother to death. However the disease does not affect their minds so that they suffer by knowing that they cannot use their bodies for anything without external help.  
 
In his first year at Cambridge, he had such difficulty controlling his arm and leg movements that he required medical help and doctors told him he would live only a few more years.  After receiving that diagnosis, he decided there was no need to study so he spent his time drinking, listening to Wagner, and reading science fiction.  By age 23, he had recovered from his depression and started studying again.  He married a fellow student, Jane Wilde, and they had three children.
 
He gradually lost muscle function until he was able to move only a few fingers on one hand and was completely dependent on others for bathing, dressing, and eating.  By his forties, he was totally incapacitated, lived in a wheelchair and required someone to care for him all the time. At age 43, he was in Geneva and nearly smothered to death from a bout of pneumonia.  To save his life, doctors had to make a hole in his windpipe that prevented him from speaking.  He had to keep that tracheostomy for the rest of his life and was able to communicate only through a computerized speech synthesizer controlled by his only remaining functioning muscles in one hand and eventually just with a muscle in his cheek.  He and his wife divorced in 1995, when he was 53.  He married his nurse, Elaine Mason, but they divorced in 2006.
 

Amyotrophic Lateral Sclerosis (ALS)
Most people who develop ALS live only a few years after they are diagnosed. He lived for 55 years after his initial diagnosis. Most people who suffer from ALS are diagnosed between the ages of 40 to 70.  Hawking was believed to have a rare early-onset slow-progressing form of the disease and to have lived as long as he did because he had plenty of money available for comprehensive care 24 hours a day and to meet all of his needs for elaborate special equipment.
 
Nobody knows the cause of this disease that destroys the nerves that control muscle function.  It does not destroy a person's brain or ability to think and reason. At this time doctors have only two drugs to slow progression of the disease.  In 1995, the FDA approved Rilutek (riluzole) which prolonged life 3-6 months.  In 2017, the FDA approved Radicava (edaravone), which can slow progression when given early in the disease.  Doctors also have various drugs to reduce shaking, muscle spasms, muscle weakness, drooling and difficulty sleeping.
 
In 2017, scientists at Ben-Gurion University in Israel announced that they had developed a drug to improve nerve function and prolong life in mice with ALS.  They used MabThera (retuximab), a drug already approved to treat chronic lymphocytic leukemia, non-Hodgkin’s lymphoma and autoimmune diseases such as rheumatoid arthritis. ALS is thought to be an autoimmune disease in which a person's own immunity attacks and destroys his own nerves like it attacks germs trying to invade his body. This new treatment is an antibody that attaches to and destroys a person's own immune B cells so they stop attacking and damaging the covering of nerves (glial cells) and the nerves that cause muscles to move.
 

 
January 8, 1942 - March 14, 2018
March 25th, 2018
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About the Author: Gabe Mirkin, MD

Sports medicine doctor, fitness guru and long-time radio host Gabe Mirkin, M.D., brings you news and tips for your healthful lifestyle. A practicing physician for more than 50 years and a radio talk show host for 25 years, Dr. Mirkin is a graduate of Harvard University and Baylor University College of Medicine. He is board-certified in four specialties: Sports Medicine, Allergy and Immunology, Pediatrics and Pediatric Immunology. The Dr. Mirkin Show, his call-in show on fitness and health, was syndicated in more than 120 cities. Read More
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