Bill Nye is turning family history into ataxia advocacy

Growing up in Washington, DC, in the 1950s and 1960s, young Bill Nye inherited a passion for science from his parents. His mother, Jacqueline, was a World War II codebreaker. His father, Edwin “Ned” Darby Nye, spent several years in a Japanese prison camp during the war, where he taught himself to tell time using a shovel handle, spurring Bill’s fascination with sundials.

But there was something he did not inherit: his father’s distinctive, wobbly, gliding-like gait. “I grew up with my father falling down all the time,” says Nye, who helped generations of children and their families discover the world of science with his Bill Nye the Science Guy program. “One day I came home, and there was an ambulance in the driveway because he had fallen down the basement steps.”

Ned Nye’s motor condition was a form of hereditary ataxia, a rare degenerative condition of the nervous system that includes many subtypes. Ataxia is a neurological condition that affects coordination, balance, and muscle control. The most common form of hereditary ataxia is Friedreich’s ataxia, a genetic condition that was the first to be identified as its own disorder separate from other forms of hereditary ataxia. In addition to balance difficulties and slurred speech, people with Friedreich’s ataxia are often affected by spinal issues and heart problems that can be fatal.

But that was not the type of ataxia that ran in the Nye family. According to family lore, the condition traced back to a Massachusetts Bay Colony ancestor named Samuel Darby whose family intermarried with the Nye family back in the 17th century. “A feature of our ataxia is it doesn’t kill you. You’ll die with it, not from it, as they say. But it persists through generations, and it is passed on by a specific gene,” Nye explains.

The “Darby Glide”—what the Nye family has nicknamed the hereditary condition—has appeared regularly but unpredictably in each generation. Ned had it, as did his mother and grandmother. As Bill and his siblings grew up, his sister Susan and brother Edward Jr. both developed it—but Bill did not. Until 2023, the Nye family did not know exactly which gene was the culprit and why some family members were spared while others were affected.

A breakthrough 300 years in the making

In January 2023, two different research teams published nearly simultaneous articles identifying the cause of a previously unexplained form of late-onset cerebellar ataxia. They traced the condition to a change in the FGF14 gene that causes a small DNA sequence to repeat many more times than normal. The condition was first called SCA50 (spinocerebellar ataxia type 50) but was later renamed SCA27B (spinocerebellar ataxia type 27B). Researchers have since found that it is one of the most common inherited forms of adult-onset ataxia.

“This discovery has been a tremendous benefit to the field, as has Bill Nye’s involvement, because he is such a great spokesperson for science of all types,” says Christopher Gomez, MD, PhD, the Albina Y. Surbis Professor of Neurology at the University of Chicago and founder and director of the University of Chicago Medicine Ataxia Center.

The discovery has led to an increase in new diagnoses. “With a genetic test now available, we are learning about more and more people with SCA27B,” says Sheng-Han Kuo, MD, director of the Initiative for Columbia Ataxia and Tremor at Columbia University Irving Medical Center and a scientific advisor to the National Ataxia Foundation. “It’s a very exciting time.”

For Nye, the impact was personal. “People in my family—my nieces, nephews, cousins, everybody—can get tested,” he says.

The discovery also became his call to advocacy. He had grown up watching his father struggle with the disease but had always felt the lack of treatment options left little for an advocate to do. "My brother especially would say, 'Bill, you should use your celebrity [status] to do something for ataxia,'" Nye says. "And I would tell him, 'There isn't much you can do about it.' Until two and a half years ago. Now there's something I can do, so I'm doing it."

In 2024, he partnered with the National Ataxia Foundation, the primary patient advocacy organization for the disorder, filming a series of four short videos about ataxia and his family's experience. By the following year, the collaboration had broadened into a Congressional briefing on rare-disease clinical trials and a four-part series with the biotech company Biogen on Friedreich ataxia, bringing viewers back to Bill’s iconic “Science Guy” lab. "Two years ago, very few people I met had heard the word 'ataxia,'" Nye says. "Now I meet many people who have."

What is ataxia?

Ataxia is a neurological condition that affects coordination, balance, and muscle control. There are many subtypes of ataxia, and the condition impacts roughly 150,000 people in the US. Dr. Gomez has a memorable way of explaining what it looks like: “Go to a bar at closing time,” he says. “You’ll see what ataxia looks like. People stumble out.” Unfortunately for people like Bill Nye’s father, it’s all too easy to mistake the disorder for drunkenness; Ned Nye was fired from one job because colleagues thought he was drunk.

Dr. Gomez explains that neurologists can give their patients a letter from the Ataxia Center to carry with them, explaining that the symptoms and signs in ataxia resemble intoxication but are due to disability. The National Ataxia Foundation also has a wallet card that can be printed and carried, describing the condition and providing spaces to fill in the physician’s name and contact information, type of ataxia, and emergency contact.

The clinical picture of most ataxias, generally characterized by progressive imbalance, slurred speech, and coordination problems, may result from a vast array of genetic changes. Researchers have identified more than 50 types of spinocerebellar ataxias that are dominantly inherited, meaning a child receives the gene change from just one parent. More than 600 types of recessively inherited spinocerebellar ataxias have been identified, which is when a child inherits the gene change from both parents. “Genetically, it’s one of the most diverse diseases that there is,” Dr. Gomez says.

Most of the inherited ataxias involve the same vulnerable part of the brain: the cerebellum—the part of the brain that helps you maintain balance and fine motor skills. In conversations with his patients, Dr. Gomez often uses an aviation analogy to explain its function. “The cerebellum is like the autopilot on an airplane. It allows you to do things unconsciously—until it stops working.” A pilot whose autopilot fails has to fly the plane manually; a patient whose cerebellum is failing has to consciously manage every movement that the rest of us perform without thinking.

Nye has spent decades translating complex science for lay audiences and has his own way of describing what is going wrong at the molecular level. Both his family's form of ataxia and Friedreich ataxia, he says, come down to the same kind of error: tiny DNA building blocks repeating far more times than they should. "If you get too many of them, you get the disease. When your body has bad or unsuitable instructions in your DNA, you're going to have all kinds of trouble as a living thing."

Because the symptoms overlap with so many other neurological conditions, ataxia is frequently confused with disorders like multiple sclerosis or Parkinson’s disease. Dr. Kuo sees this in his clinic constantly: “It’s actually very common. Patients have usually been seeing other doctors before they come to me, getting some sort of misdiagnosis, or just a label of ‘ataxia’ without anyone digging deeper into what’s causing it.”

Nye does not have SCA27B in any meaningful sense. He was tested, and his GAA gene repeat count was below the threshold for symptoms. “Through dumb luck, my gene repeat is very short,” he says. “For a guy of my ancestry, I’ve been pretty athletic my whole life. I don’t have the fine motor disability that my dad and grandmother had, and that my sister has now, and that my brother’s condition is definitely worsening into. I have survivor’s guilt. I never had kids because I just did not want to pass this on. I realize that is not the best decision, perhaps, but it had a big effect on me.”

His sister now uses a wheelchair; his brother’s symptoms are progressing as well. Because SCA27B has a later onset compared to other ataxias, some patients first notice symptoms in middle age or later. “Some people who are 60, 70, or 80 years old might be surprised to find out that they have the [gene] repeats,” Dr. Gomez says. “On the other hand, Friedreich’s ataxia often starts at eight, nine, or ten years old. The contrast is striking.”

The disease also has a day-to-day variability that Nye finds difficult to explain. “When you have the Darby Glide, you’re not aware of what you can’t do. Some days you can write. Some days you can swallow. Some days you speak very clearly, other days you don’t.”

New options for Friedrich’s ataxia treatment

For nearly all of the hereditary ataxias, there has historically been no disease-modifying treatment—only physical therapy, occupational therapy, speech therapy, and the gradual escalation of mobility aids. Within the past three years, this has finally begun to change. In February 2023, the FDA approved omaveloxolone (Skyclarys) as the first disease-modifying therapy for Friedreich’s ataxia in patients 16 and older

For SCA27B, the gene discovery brought a different but equally important development. A drug called 4-aminopyridine, long used to treat walking difficulty in multiple sclerosis and recently available as a low-cost generic medication, has turned out to be very effective at suppressing SCA27B symptoms—a 2024 study found that three out of four patients improved with the medication.

Both of Nye’s affected siblings have started 4-aminopyridine. “My sister is a big fan [of the medication],” he says. “When I talk to her on the phone or FaceTime, her speech is much clearer than it used to be.”

For families with a history of ataxia who have never pursued genetic testing, the process is more accessible than it used to be. “First, see your neurologist, who can recommend genetic testing, and talk with a genetic counselor who will discuss your family history and the different risks,” Dr. Kuo says. “Depending on what’s being ordered, the test may come as a kit with an oral swab. You send it back and get the result in two to three months. The price has come down a lot, and most insurance covers it now. I encourage everybody to pursue genetic testing.”

To families navigating this diagnosis for the first time, Nye says, “I just wish everybody had accepted it more, instead of fighting it and pretending it wasn’t a real issue. If we accept it, we’ll be able to live better.”