(5 minute read)
As Basel walked toward me, he swayed, listing slightly to his left. His face contorted into a crooked smile. Just a few weeks earlier, I’d heard that he “got his feet all tangled up” and fell, requiring eight staples in his head, but his injury had since healed. He shook my hand. That’s when I noticed a subtle pulsing in his grip – what doctors refer to as “milkmaid’s grip,” the alternate contraction and relaxation of the tiny muscles in his hand.
Basel Queen and his wife, Teresa, live down the street from my parents in the small Florida panhandle town of Freeport. Basel retired there after serving in the military for two decades, a heavy equipment operator for the Air Force’s elite heavy construction unit, RED HORSE. He operated immense cranes, drove powerful bulldozers and dump trucks, and built runways and other key structures in Korea during the Vietnam War and later in the United States. These days Basel’s driving is limited to around town. “I can’t drive on the interstate because I don’t know what I’m doing,” he said.
When I first met Basel a few years ago, he had the appearance of a guy who had spent much of his life working outdoors: lean and lanky with a weathered face and tanned, sinewy arms. His salt-and-pepper hair was neatly trimmed, a holdover from his military days. He stood quietly as Teresa and I spoke, one arm folded and clutching his opposite elbow. He seemed shy. I didn’t think much about his unusual stance at the time, but often over the years I have seen him in this position at home or around town, his arms locked in a self-imposed vise.
Basel wasn’t always given to such constraint. As a teenager growing up in Texas in the 1960s, Basel loved to dance, especially the Twist, his hips swiveling and arms waving to the rousing tempo of dance phenom Chubby Checker. Now 66, Basel does a different dance, directed by a cruel, inner choreographer over which he has no control: Huntington’s disease.
Sometimes referred to as Huntington’s chorea (chorea is Greek for “dance”) because of the dance-like movements associated with the condition, Huntington’s disease is a hereditary disorder that affects the brain, causing uncontrolled movements, speech problems, personality changes, and dementia. Although symptoms usually begin around age 40, Basel didn’t show signs until his late 50s.
Basel is one of roughly 30,000 people in the United States currently living with Huntington’s; his daughter and granddaughter are among nearly 200,000 at risk of inheriting it. Huntington’s bears striking similarity to other neurodegenerative diseases, such as Alzheimer’s and Parkinson’s, which target specific areas of a person’s brain and often appear late in life.
Many people who have Huntington’s disease retreat from the world, self-conscious about their uncontrollable movements, odd grimaces, and strange speech patterns. As I’ve come to know Basel, I’ve learned that he isn’t embarrassed by his condition. He still socializes with friends in his neighborhood and at his church, and he often goes shopping with Teresa. The reactions of people around him don’t go unnoticed, however. “Sometimes I can tell that people are staring at me, curious,” he said.
Because Huntington’s disease is so rare, few people have seen or met someone with the condition, although occasionally it surfaces in literature or pop culture. A character in the popular television show House, MD, had the disease; in one episode, she helped her brother, who also had Huntington’s, commit suicide rather than succumb to its eventualities. Perhaps the most famous person with the disease was folk singer Woody Guthrie, who died from its complications in 1967. Guthrie once wrote a letter to his wife, Marjorie, describing its effects: “No bodily pains; just like my arms and legs and hands and feet and my whole body belongs to somebody else and not to me.”
Basel loves the outdoors, but the physical manifestations of his condition keep him from doing his favorite activities. He and my dad hunted together for many years, trekking through palmetto and pine forests dressed in camouflage and snake boots in search of white-tailed deer. Since his diagnosis, Basel doesn’t hunt anymore; carrying a gun would be a bad idea.
He’s still active in his church, however, where he serves as a deacon. Basel organizes visits to church members who are sick or have special needs. But he gets anxious about things that wouldn’t have bothered him before. “He used to lead the most beautiful prayers,” my mother told me. Now he can’t speak in front of a group because he’s too nervous. Plus, he’s hard to understand. Basel’s speech slurs slightly, and he often struggles to form words, a consequence of the loss of muscle control and language processing skills.
Ordinary activities like eating and drinking are perilous for Basel. Teresa adds thickening agents to his drinks to prevent one of the more common risks people with Huntington’s disease face: aspiration – inhaling thin liquids or foods into the lungs, putting them at risk for pneumonia. But a greater danger is weight loss due to changes in the way the body metabolizes foods and the inability to swallow. Teresa said that Basel has lost more than 35 pounds since his diagnosis, a lot for an already slender guy.
“Right now I can still eat,” said Basel. “I love hamburgers and fish sandwiches, but I can’t eat them anymore. I can eat soft foods like pudding and applesauce.” Occasionally, he coughs or chokes while eating; eventually, he’ll need a feeding tube to help him meet his nutritional needs. Basel hopes to delay that as long as possible.
Last summer, I sat down with Basel and Teresa, and we talked about the fundamental cause of his condition – a single genetic mutation, or change, in his DNA, called a CAG repeat. The mutation repeats the genetic information in his DNA too many times, a sort of “genetic stutter,” like a stuck key on a keyboard. The end result: tortuous twisted clumps of protein that wreak havoc on his brain, producing the condition’s telltale symptoms.
Basel was quiet for a moment. Then he asked, “Will I become a vegetable?”
That’s a difficult question to answer. People with Huntington’s disease typically die within 15 to 20 years of diagnosis, usually from complications such as pneumonia, injuries from a fall, or starvation due to swallowing difficulties. If Basel is typical, he has about 5 to 10 years left.
Although Huntington’s is the focus of much study, including the application of new techniques like gene editing and protein visualization, none of the research will help Basel. The damage to his brain is irreversible. But it might help Basel’s daughter, Keri, who has a 50 percent chance of developing the disease. She had never heard of Huntington’s disease before her father’s diagnosis, even though other family members showed signs of having the condition. “I noticed that Dad’s aunt, grandmother, and cousin moved a lot, but I never really thought about it,” she said.
Keri doesn’t know whether she has inherited the disease, and she doesn’t want to be tested. She’s not alone. Fewer than 5 percent of people at risk for Huntington’s disease choose to undergo genetic testing to learn if they will get the disease or if they might be carriers. “My in-laws, my husband, even my friends want me to get DNA testing,” she said. “But I think that what I don’t know won’t hurt me. I don’t want to know because if I do know that my daughter or I have it, then that’s going to be a constant burden. I will always be looking around the corner, wondering, what was that? Was that a sign? What was that movement?”
Basel has a keen understanding of the mental and physical changes that accompany his illness, but many people who have Huntington’s disease demonstrate poor self-awareness, oblivious to the changes that are often obvious to those around them. His sister, whose symptoms are far worse than Basel’s, is either unaware or unwilling to admit that she has the disease. Basel believes it’s the latter. “She won’t go to the doctor. She’s in denial.”
When I asked Basel what he sees in his future, he was matter-of-fact. “Well, there’s no cure for Huntington’s disease. I guess I’ll just have to deal with life as it progresses.” Medications help. Basel takes tetrabenazine to reduce his involuntary movements and Ativan to help with his anxiety. Both seem to be working for now, although neither is 100 percent effective in reducing his symptoms. “He doesn’t move constantly,” Teresa said, “but when he gets anxious or aggravated, his movements get worse.”
Basel’s symptoms will progressively worsen over time, and the drugs will be less effective. Although some scientists hope to find new drugs to better treat the symptoms of Huntington’s disease, some doctors have mixed feelings about the research and express concern about their patients’ quality of life. Drugs that could reduce Basel’s unnatural movements and muscle problems also might extend his life and prolong his suffering – what one researcher referred to as “unpalatable trade-offs.”
The progressive physical decline associated with Huntington’s disease typically ushers in a loss of independence. But the mental changes, such as depression, irritability, anxiety, and memory problems lead to a loss of self. Even so, Basel remains upbeat. When he visits church members or hears of neighbors whose health is much worse, he’s thankful that he is still able to do so much. Eventually, he will need around-the-clock medical care.
Keri sees Basel about twice a week and notices small changes in his health, but she thinks her father is doing okay. “I know a little bit of his memory isn’t as sharp as it used to be. But we still laugh about things that have been running jokes in our family for a really long time,” she said. “He’s still Daddy to me.”
Basel stopped by my parents’ home recently. “Basel was twisting and turning like he does,” my mother said. “I told him, ‘I can do the Twist just as good as you, Basel!’” And she began twisting her 83-year-old hips. Basel laughed and grabbed her, as if ready to dance.