A History of Huntington Disease and Beyond

tags: historians , Science , Huntington Disease , Thomas Bird

Robin Lindley is a Seattle-based writer and attorney, and the features editor of the History News Network (hnn.us). His articles have appeared in HNN, Crosscut, Salon, Real Change, Documentary, Writer’s Chronicle, Billmoyers.com , Alternet, and others. He has a special interest in the history of conflict and human rights. His email: robinlindley@gmail.com. In 1974, young neurologist Dr. Thomas Bird founded the first clinic for adults with neurogenetic diseases in the United States. For more than 40 years, he directed this clinic at the University of Washington where he saw thousands of patients and conducted pioneering research on conditions such as cerebellar ataxia, movement disorders, hereditary neuropathy, muscular dystrophies, and familial dementias. Over his career, he has been honored with numerous national awards and lauded for his discoveries about the genetics of hereditary neurological disorders including Alzheimer and Huntington diseases.

To his initial surprise, patients with the cruel and incurable Huntington disease became a prominent part of Dr. Bird’s practice in the early years of his clinic. Huntington’s is a progressive, inherited disease that perniciously and ruthlessly devastates the brain. It can cause incoordination, jerkiness, confusion, impaired judgment, emotional instability, depression, anxiety, social disinhibition, hallucinations, and other problems. And no two patients are alike in terms of their signs and symptoms of the disorder.

Dr. Bird addresses this perplexing disease and its many permutations in his groundbreaking new book for general readers and professionals alike, Can You Help Me?: Inside the Turbulent World of Huntington Disease (Oxford University Press). The title comes from a Huntington sufferer’s plea for help from his prison cell, and this desperate call reflects the desire of so many of Dr. Bird’s patients who, through the years, sought his unique understanding and care.

In his book, Dr. Bird vividly describes Huntington disease, traces its history and, at the heart of his book, shares dozens of accounts of his own patients in lively prose that evokes the engaging writing of renowned doctor-authors such as Oliver Sacks, Richard Selzer and Atul Gawande. He recounts the physical, cognitive and emotional challenges of his patients and the complex situations that patients and their families face every day. There are wrenching stories of neglect and abuse of vulnerable Huntington sufferers as well as stories of hope and courage and the unselfish—and vital—support of families and friends. These very human accounts come from Dr. Bird’s decades of meeting and treating Huntington patients of all ages, from early childhood to the nineties, and from all walks of life.

Physicians are still struggling to understand the clinical manifestations of this condition. No Huntington’s patient is “typical,” as Dr. Bird’s case studies demonstrate. One patient may exhibit jerky movements only, while another may be emotionally explosive with poor judgment but without an obvious movement disorder. Some may experience both severe physical and behavioral problems, especially as brain degeneration progresses. Some patients may alienate their caregivers and some may refuse care and some may lack the financial and other resources to receive care and to survive in today’s complex world.

Can You Help Me? reflects Dr. Bird’s compassion and care for patients of this dreaded disease as he offers support and treatment grounded on his trailblazing research into the genetics of neurological diseases. In offering understanding and empathy to each patient, he emulates the admonition of the legendary physician Sir William Osler: “Care more for the individual patient than the special features of the disease.”

Dr. Bird is a University of Washington Professor (Emeritus) of Neurology and Medicine (Medical Genetics). In addition to directing the UW Neurogenetics Clinic for more than 40 years, he was also chief of neurology at the Seattle VA Medical Center for 12 years and is presently a retired Research Neurologist in Geriatrics at the VA.

Although retired from clinical practice, Dr. Bird still actively researches genetic diseases of the brain and neuromuscular system; collaborates with molecular biologists and others on genetics projects; and mentors physicians in training and research fellows. He earned his M.D. from Cornell Medical College and is board certified by the American Board of Psychiatry and Neurology. He lives in Lake Forest Park, WA, just outside Seattle, with his wife Ros.

Dr. Bird sat down at his University of Washington Medical Center office and generously responded to questions about his career and the history and human stories of Huntington disease.

R obin Lindley: Thank you Dr. Bird for talking with me about your distinguished career and your new book on Huntington disease. I’d like to first ask you about your own story. When you were a child, did you dream of becoming a doctor?

Dr. Thomas Bird: I grew up in a small town in upstate western New York, in the Finger Lakes area. My maternal grandfather was a country doctor in that small town. I didn’t know him really. He died when I was five or six years old, so I only have a few vague memories of him. But our house where I grew up was just down the street from where he lived, and that was the family home on my mother’s side, and my mother’s brother, my uncle lived in that house, so I was very familiar with the house.

My aunt and uncle kept my grandfather’s old office intact. I remember wandering through it as a kid and seeing the examination chair and a little side laboratory with a microscope and shelves loaded with pill bottles. I was very impressed.

So, I had this knowledge of my grandfather, even though I didn’t know him. My mother clearly adored him so, when I was growing up as a kid, it was very clear to me that if you wanted to be the best you could be, you would become a doctor. That was never said explicitly, but it was the aura that I grew up with. Later I got really interested in chemistry and thought I wanted to be a chemist. So it wasn’t like I directly wanted to be a doctor. But when I went to college, it was certainly in the back of my mind because I became a premed major.

Robin Lindley: What inspired you to specialize in neurology?

Dr. Thomas Bird : There were a lot of lines that led to that. I’m sure that having a brother with mental retardation made a difference in how I viewed people and how I viewed medicine and the things I was interested in. Having a brother that I lived with 24/7 my whole childhood life who had something not right with his brain impacted me a lot. And, when I went to college at Dartmouth, I actually majored in psychology and who knows exactly all the influences for why I did that, but my brother was probably part of it.

I also was just fascinated by human behavior. Fortunately for my future, the psychology department specialized in biological psychology so the faculty were very interested in the neuroscience brain piece of psychology. We had Skinner boxes where we did mouse and rat and pigeon experiments on behavior, and I took one course where we dissected a sheep brain and then a human brain. I think that made a difference. My mentor was a clinical psychologist who was actually in the department of psychiatry at the medical school as well as in the department of psychology. He taught me a lot about human behavior and our interests in that topic matched nicely.

So, when I went to medical school, I thought my trajectory would be to either be a family doctor, like my grandfather, or a psychiatrist because I was really interested in human behavior. When I got there, I didn’t particularly like psychiatry. It wasn’t a neuroscience or brain-oriented department of psychiatry so I lost interest in it.

Then, mostly by chance, I ended up having my summer project with the new chairman of the department of neurology at Cornell, Fred Plum. I knew nothing about neurology, but I wanted to stay on campus and work with somebody doing research in medicine. So I got hooked up with Dr Plum. He turned out to be a very dynamic, aggressive, energetic person who eventually became world famous. He wrote a bestselling textbook in the 1960s called Stupor and Coma , and he became one of America’s leading neurologists. In the beginning I had no idea who he was or what I was getting into, but it turned out to be terrific.

I started with a clinical project in coma. I learned how to do EEGs [electroencephalograms], and I was going around the hospital with a mobile EEG machine and doing EEGs on people in comas. So I got to see all kinds of neurology and I got to see it up close and personal. Then I started going to neurology grand rounds on a regular basis. I just became fascinated with the brain and with neurology.

Back in those days, you didn’t have to decide what you wanted to specialize in until you […]

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