1 in 10,000: Huntington's Disease and Woody Guthrie
June 11, 2012

Woody Guthrie’s music was a force for goodwill. His songs brought people together and dispelled hatred and fear. The magic that happened when he picked up an acoustic guitar or held a harmonica to his mouth was undeniable.

Unfortunately there was a microscopic part of Guthrie that wasn’t so rare or peaceful. It tore through his life, shredded his relationships, and ultimately claimed his life.  It was the version of Huntington’s gene that causes Huntington’s Disease and it afflicts 1 in 10,000 people.

Warning! Science Ahead

The gene that causes Huntington’s Disease, which Guthrie inherited, is quite literally an unsightly mutant. Genes, as you know, are sections of DNA that code for protein. If you reach back to that fateful High School Bio class, you’ll remember that DNA is made up of the nucleotides ACTG. When formed normally, those nucleotides have a sequence of CAG that repeats 20 times or less. The normal Huntington's gene plays nice with the brain and actually creates a protein that helps facilitate functions of the nervous system.

In the mutant version, that CAG sequence repeats 36 times or more. The resulting protein is toxic to the brain. It clumps up in the control center of the sufferer’s cells and slowly suffocates their nervous system. Most of the action happens in a part of the brain called the Basal Ganglia where movement is controlled, but many of the most profound symptoms are much less obvious.

Woody’s Symptoms

Guthrie himself writes about seeing these symptoms in his mother, "She got worse, and lost control of the muscles in her body; and two or three times a day she would have bad spells of epileptics, first getting angry at things in the house, then arguing at every stick of furniture in every room until she would be talking so loud that all of the neighbors heard and wondered about it (Bound for Glory, p.135)."

The physical spasms, also known as Chorea, are the most visible symptoms of the disease. But it is the degenerative mental effects that can be most devastating. Guthrie had written extensively about his great difficulty in dealing with his mother’s increasingly erratic behavior. When Guthrie was 14, his mother was committed to the Oklahoma Hospital for the Insane... where she would die in 1930. This was the common approach to treating Huntington’s at the time; the result was a stigma that effectively wrote thousands of sufferers off as incurably insane. Doctors and scientists understood that if one parent had the disease, their children had a 50/50 chance of also having it (think Punnet squares), but there was little understanding of the affliction.

Guthrie denied the possibility of coming down with symptoms, even through the beginning of the disease's onset. When asked by his second wife, Marjorie, if he would get sick like his mother, he replied, “No, only women.” It is impossible to tell precisely when Guthrie began experiencing symptoms, though it is probable that the initial diagnosis– alcoholism and schizophrenia– can be attributed to the disease. Because of these symptoms, Woody would burn through two marriages and spend the last 10 years of his life in and out of psychiatric hospitals before he passed.

Huntington’s Today

After Woody’s death, Marjorie Guthrie formed what would become the Huntington’s Disease Society of America– an organization that works at the forefront of Huntington’s research. The HDSA provides resources to those affected by Huntington’s and works tirelessly to raise awareness about symptoms and treatment.

There is still no cure for Huntington’s disease. It remains just as tragic and terminal as it was in Guthrie’s day. Medical advancements can, for a time, alleviate symptoms, but the question for those who have the dysfunctional gene remains not "If," but "When."

In 1993, the gene that precipitated Woody’s downfall was isolated. A simple genetic test was devised to conclusively determine if an individual with a family history of Huntington’s disease has the mutated gene that causes it. This raises a couple questions: If Nora Guthrie (Woody's mother) had this option, what would she have done? Would she have chosen to have children? Should Woody himself have had kids? Is it right to even get tested?

The answer to the latter question will always be an intensely personal and complicated decision. But it is safe to say that as short and heartbreaking as Guthrie’s life was, the world is an infinitely better place having had him and his music in it.

Further Reading

For more on Woody’s battle with Huntington’s

The history of Hungtington’s
An overview of the disease, science and all

The HDSA

Work on the disease being done at MGH

A special thanks to Morgan Thompson for all her help in providing the resources used for this entry.

Author:
Andy Short
Publication date:
May 30, 2012
Events:

August

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