Huntington's Scene In New Zealand
|Articles taken from the June 2003 Huntington's News. The Quarterly Newsletter of the Huntington's Disease Associations of New Zealand|
Woodrow Wilson 'Woody' Guthrie and Huntington disease
This brief account of Woody Guthrie is instructive to clinical geneticists. It tells the story of one famous man's understanding of, and struggle with, Huntington's disease.
Born on 14 July 1912 in Okemah, Oklahoma, USA, to Charley and Nora Guthrie, Woodrow Wilson Woody' Guthrie left an enduring legacy as one of America's most accomplished singer-songwriters. Despite the family suffering some hard-ship, his childhood was a happy one. Woody did not attain much formal schooling, but he had a thirst for knowledge and was very well read. It was evident from an early age that he had a natural aptitude for music, and Woody's ability to write songs and lyrics was legendary.
He married the teenage sister of a good friend in 1933 at the age of 21 and they would have three children together. Shortly after they were married, the infamous dust storms of the 'dirty 30s' hit Oklahoma and other parts of the USA. This prompted Woody to move his family to California, and on leaving Oklahoma, he penned one of his most familiar songs, 'So Long Its Been Good to Know You'. Woody's eyes were opened by his experiences in California. He encountered the same migrant workers who were immortalized in the novels of John Steinbeck, and he wrote hundreds of songs detailing their experiences and hardships. This was the beginning of a lifelong commitment to left-wing political causes that would eventually lead to Guthrie and many of his contemporaries being labelled as communists. At this time. Woody also began to develop some notoriety with his own Los Angeles radio show.
Guthrie eventually settled in New York where he surrounded himself with a group of musicians including Pete Seeger, Leadbelly and other significant figures in the history of American folk music. It was during this period that Guthrie wrote the first draft of a scathing composition that targetted Irving Berlins 'God Bless America'. Originally entitled 'God Blessed America', this composition subsequently became 'This Land is Your Land'. Now recognized as a patriotic American anthem, its origins were quite the opposite. While he gained a modicum of fame in New York City, Woody continued to find it difficult to cope with any degree of fame or fortune. As a result, he often uprooted his family as he moved from coast to coast. Eventually, his wife left him to try and raise her family in a stable environment. Woody began a relationship with a dancer called Marjorie Mazia. Marjorie became pregnant and the couple married.
As the United States entered World War II, it was increasingly difficult for artists with communist sympathies to retain a pacifist stance, particularly as the Soviets and the Americans had joined forces against fascism. In an attempt to avoid conscription into the military, Guthrie served in the merchant marine. Remarkably, his ship was torpedoed on all three of his sorties, but he managed to survive each attack. He was eventually drafted into the army on victory in Europe day, but never saw combat action.
However, the war period was not completely devoid of accomplishments for Guthrie. He spent much time with his new young daughter Cathy and wrote a large number of children's songs, the most memorable of these being 'Take Me for a Ride in the Car-Car'. This period also saw the publication of his remarkable autobiography, Bound for Glory, which detailed (and perhaps exaggerated) his extraordinary upbringing and life experiences. It was well received by much of the national press.
Tragedy struck Woody's family after the war. A house fire killed his daughter Cathy. This was not the first time that such a disaster had affected Woody's family. Woody and Marjorie had a number of other children together, including Arlo Guthrie, who became a respected folk singer in his own right. Much of the above information comes from the Biography Woody Guthrie: A Life by Joe Klein (1). This book begins with the story of Guthrie's maternal grandfather, George Sherman, who fell off his horse and drowned in shallow waters as a relatively young man. The death was explained as another in a long line of tragedies in the Sherman family.
Misfortune would continue to befall the family. In May 1919, during an argument with her mother, Woody's sister Clara set herself on fire. While her intent was only to scare her mother, she died of these injuries days later. The townsfolk blamed Woody's mother, Nora Guthrie, for her daughter's death. In fact, it was recalled that the Guthrie's previous home had also burned down 10 years earlier. Much of the conversation in town turned to Nora's increasingly odd conduct. Woody would later describe his mother's behaviour during that period: 'She would be alright for awhile, and treat us kids as good as any mother, and all at once it would start in something bad and awful something would start coming over her, and it would come by slow degrees. Her face would twitch and her lips would snarl and her teeth would show. Spit would run out of her mouth and she would start out in a low grumbling voice and gradually get to talking as loud as her throat could stand it; and her arms would draw up at her sides, then behind her back and swing in all kinds of curves. Her stomach would draw up into a hard ball, and she would double over Into a terrible-looking hunch and turn into another person, it looked like, standing right there before Joy and me.' (1)
She deteriorated over the years, but enjoyed spending time going to movies with young Woody. Eventually, it became harder for Nora to care for her children and the younger children moved in with relatives. In 1927, Nora walked over to her husband Charley, who was asleep on the sofa, and set him on fire with a kerosene lamp. Charley Guthrie survived, but Nora Guthrie was admitted to the state mental facility in Norman, Oklahoma. Woody visited his mother in hospital, where he was informed by doctors that she was suffering from Huntington's chorea. Nora Guthrie died of Huntingtons disease (HD) in 1929, when she would have been 41 years old. Woody briefly discussed his mother's death with his future brother in law, Matt Jennings, at that time. He stated that the disease seemed to be passed from father to daughter and mother to son, and then added, There's no way I'm gonna get that disease' (1).
While Woody had always been an eccentric, playful character, it eventually became clear that his own behaviour was increasingly unusual. As early as the early 1940s, he was clearly erratic in formal situations. This was often ascribed to his increasing use of alcohol. While aboard one of the merchant marine voyages during World War II, he confided to his friends, Im pretty sure I've got the same thing my mother had' (1). When asked how he knew, his reply was, 'Dunno, just feel queer sometimes' (1).
Crowds became increasingly uncomfortable at his performances as he stumbled on stage or botched his lines. Because playful stumbling had always been a part of his act, this was usually easily covered up. However, things were deteriorating at home as well. Woody became increasingly angry and his actions were increasingly bizarre. In fact, he was eventually sentenced to jail in 1949 for obscene letter writing. Things got no better after his discharge from prison, and he was found wandering aimlessly at times. One night, he was arrested for loitering and was assumed to be under the influence of alcohol given that there was a 'boozy, light-headed quality to him; his walk had become a lurch, and his speech often was decidedly slurred. What's more he looked like a bum' (1).
As his behaviour became increasingly terrifying to his wife, Marjorie eventually enlisted the help of friends to check Woody into Kings County Hospital. He was not specifically diagnosed, but was placed in a 3-week detoxification programme.
In rather rapid succession, he was in and out of a variety of facilities, including Bellevue Hospital (where he was diagnosed with schizophrenia) and Kings County Hospital. Marjorie was increasingly perplexed with her husband's situation, and eventually, he was assessed at the Brooklyn State Hospital. The results of this assessment are described in exceptional detail in Joe Klein's biography. The conclusions of the examining physician, Dr Marlowe, are fascinating:
This is one of those cases which stubbornly defies classification. In it, it has elements of schizophrenia, psychopathy and a psychoneurotic anxiety state, not to mention the mental and personality changes occurring in Huntington's chorea, at this patient's age. As such, examiner chooses to defer diagnosis until such time as further observation has made the picture clearer.' (1)
While it has been presumed that Woody mentioned his family history to Dr Marlowe, it appears clear that the physician in question had difficulty recognizing the clinical presentation of HD. Various physicians at the facility questioned Woody about his mother's history and this perplexed him. By this time, he felt his problems were all secondary to alcohol use, and it was his father that was the alcoholic! On 3 September 1952, he was officially diagnosed with Huntington's chorea by a neurologist named Dr Perkins. This diagnosis was not shared with Woody, who was led to believe he was going to be treated with insulin injections to treat his symptoms. On 6 September, he wrote, I dont think anybody will find any tracks of insanity or hereditary disease in me, except for my mother's death by Huntington's Chorea which I've read in a heavy book or two is not pass-onable'(1).
Woody's curiosity intensified, and writing on 12 September, he stated that:
'Face seems to twist out of shape. Cant control it. Arms dangle all around. Cant control them. Wrists feel weak and my hands wave around in odd ways. I cant stop. All these docs keep asking me about how my mother died of Huntington's Chorea. They never tell me if ifs pass-onable or not. So I never know. I believe every doctor ought to speak plainer so us patients can begin to try to guess partly what's wrong with us. If it's not alcohol which has me, I wonder what it's going to be' (1)
Three days later, he told a friend how his mother died with that 'old three-way disease of chorea consisting of St. Vitus dance, epilepsy and mild insanity. He was 'not of the opinion that chorea can be passed on to any child. I was born before mama took her chorea anyhow' (1). The next day he reassured himself that 'I've read that chorea's not transmittable from parent to child' (1). On 21 September 1952, it was reportedly disclosed to Woody Guthrie that he was affected with Huntington's chorea. The next day, he requested a discharge to help care for his family. He left stating:
'I am already tracking down all the library books, pamphlets, articles, essays, speeches, lectures on that peculiar mixture of mental diseases called by the name of Huntington's Chorea. I want to do some writing of my own on that disease, chorea.' (1)
However, it was clear that Woody could not continue to care for his family. He left Marjorie and briefly married another young woman, Anneke van Kirk, with whom he had another child. Woody returned to New York City with Anneke and it was apparent to Marjorie that Woody was deteriorating. Remarkably, Marjorie helped look after this family as well. After further troubles. Woody checked himself back into the Brooklyn State Hospital on 16 September 1954. By December of that year, he finally acknowledged to his father, 'I've got the 1st early signs and symptoms of a dizzy disease called Huntington's Chorea, same disease Mama had which lets me stay dizzy in my head everyday without paying my barman one penny' (1).
Around that time, he wrote a poem that should serve as an inspiration to all involved in HD research:
Means there's no help known
In the science of medicine
Eventually, Anneke left him and Marjorie moved back in to care for Woody, despite the fact that she had remarried.
On 17 March 1956, a benefit for Woody's family was held at Pythian Hall, New York. This concert was an overwhelming success and reunited many of Woody's old contemporaries. Many say this was the beginning of the elevation of Woody Guthrie to icon status. The concert ended with a resounding version of This Land is Your Land'. By now, Woody Guthrie was a shadow of his former self, but folk music was becoming a phenomenon across the United States and Guthrie was beginning to be recognized as a genius. One young musician who idolized Guthrie persevered to meet him while he was hospitalized in 1961. This young man's name was Robert Zimmerman, who would later be known as Bob Dylan. After several more years of decline, Woody Guthrie died on 3 October 1967. Despite only a moderate level of success during his lifetime, he is now widely recognized as one of America's great songwriters.
His second wife, Marjorie, founded the Committee to Combat Huntington's Disease. Through this association, Woody Guthrie may yet contribute to the science of medicine's quest to help others with HD.
Woody Guthrie was inducted into the Rock and Roll Hall of Fame in 1988. His name has continued to live on through the work of a new generation of musicians. Two albums of Guthrie's previously unpublished lyrics were released to critical acclaim by British folk singer Billy Bragg and the American alternative country band Wilco. These albums, entitled Mermaid Avenue, volumes 1 and 2, were released in 1998 and 2000, respectively, thanks in part to the work of his daughter Nora Guthrie.
To pay homage to Woody Guthrie, the US Postal Service issued a stamp in his honour on 6 June 1998 as part of a series of stamps honouring 'Folk Musicians' (Fig. 1). It was a fitting tribute that some might have found ironic given Guthrie's history of anti-government songwriting.
George Huntington first described the disease that would subsequently bear his name in 1872 when he was only 21 years old and one year removed from medical school (2,3). In his paper entitled 'On Chorea', which he delivered to the Meigs and Mason Academy of Medicine at Middleport, Ohio, he commented on the presentation of a previously undescribed condition which he had first encountered at 8 years of age while on rounds with his physician father.
Huntington's description of what we now know as HD was so complete that Osier himself marvelled at the succinct description 20 years later. However, George Huntington was first and foremost a complete family physician, and he did not pursue further research into HD or other matters. In fact, his presentation 'On Chorea' ended with the conclusion that this disorder was probably no more than a 'medical curiosity. It would appear that HD was indeed held as little more than a medical curiosity in the decades which followed. Even 80 years later and only miles from where Huntington made the rounds with his father. Woody Guthrie was hospitalized with classic symptoms of HD and a positive family history of the condition, but the diagnosis was not apparent to the admitting doctors. However, HD has certainly become much more than a medical curiosity over the past 50 years. It is an autosomal dominant disorder caused by CAG expansions of the gene HD that encodes the protein huntingtin (4,5).
The disorder is characterized by progressive motor disability involving both voluntary and involuntary movements, and mental disturbances, including changes in cognition and personality.
In many different ways, HD has become something of a paradigm in medical genetics research. Huntington disease is now one of many neurological conditions known to be associated with CAG expansions and polyglutamine tract expansions. Most importantly, the work on pre-symptomatic testing in HD has led to a significantly enhanced knowledge of how to work with families at risk of such an overwhelming and as-yet-untreatable genetic disorder. In addition, the expansion of services and information for families (including information on the Internet) has empowered many to take more control of their futures. While we do not yet have the cure Woody Guthrie yearned for, the information that he cried out for is increasingly available.
1. Klein J. Woody Guthrie: a life. New
York, NY: Dell Publishing/Random House, Inc., 1980.
2. Durbach N, Hayden MR. George Huntington: the man behind the eponym. J Med Genet 1993: 30:406-409.
3. Conomy JP. Dr. George Sumner Huntington and the disease bearing his name. (http://www.lkwdpl.org/hdsa/conomy.htm)
4. Online Mendelian Inheritance in Man (OMIM). Baltimore, MD: Johns Hopkins University. MIM number 143100: 09/07/2001. (http://www.ncbi.nlm.nih.gov/omim)
5. Huq AHMM, Hayden MR. Huntington disease. (Updated 30 September 1998.) In: Geneclinics: Clinical Genetic Information Resource (database online). Seattle, WA: University of Washington.
(Clinical Genetics Volume 61 Issue 4 Page 263 - April 2002)