Bill Nye the Science Guy is temporarily rebranding. For now, he’s “Bill Nye the Ataxia Advocate Guy,” as he aims to bring awareness to a neurological disease that has affected his family for generations. Nye has previously spoken about the genetic disease, which makes it difficult for people with the condition to control their muscles and often leads to problems with walking and speech, in a documentary released in 2017. He said then that he didn’t have children because of concerns he would pass on the disease to them.
This year, Nye has taken a more outspoken stance in bringing awareness to the disorder, which can often be misdiagnosed as other, more common diseases like Parkinson’s or multiple sclerosis, in partnering with the National Ataxia Foundation.
STAT spoke with Nye about his history with the disease and what made him interested in talking more about it publicly. This conversation has been lightly edited for length and clarity.
Could you talk to me about your family history with ataxia?
Well, my grandmother lost her balance all the time, often. Her two sons, my father and my uncle, lost their balance all the time. My sister and brother have symptoms. My sister especially, spends a lot of time sitting down. She’s not quite confined to a wheelchair, but getting there.
It correlates with this gene repeat that somehow affects your cerebellum — this is the base of your brain in the back. It had not been understood even whether it was a genetic disorder or some other sort of thing, but a year and a half ago, two separate sets of researchers identified this gene repeat on the FGF14 gene, and so it’s called spinocerebellar ataxia, SCA27B. There are many [other] variations of ataxia.
It’s a big deal because once you find it, [you can] maybe do something about it. And the thing you would do about it, for example, my gene repeats according to genetic tests are so few that I will almost certainly not present symptoms. Suppose it had been possible for my mother to select fertilized eggs, zygotes, in such a way that the ones she selected would not have significant gene repeats. That could change the world.
You’ve spoken briefly about ataxia in the past. But why have you chosen this moment to be more vocal?
Well, the big thing is identifying the gene repeat. It may be that SCA27B is more common than people realized for two reasons.
Sometimes it’s so mild people aren’t even aware that they have it and they’re passing it on. And other times, it’s so much like other forms of ataxia that you don’t realize it’s 27B. So if we can get people who present with ataxia symptoms to come forward and send their genes — a cheek swab, blood sample — to these labs that do this analysis, then perhaps we could learn more about how it is passed from generation to generation, and then the pattern in that passing would lead to discoveries associated with all sorts of things about genetics.
It sounds like you’ve been yourself tested for these genes that are associated with ataxia. I wonder what that experience was like for you?
To me, there’s two big things. The first one is, I’m not going to get it, cool. I can keep playing disc golf and go swing dancing and these other things I do.
The other thing is the survivor’s guilt. My brother and sister have strong symptoms; my cousin, who’s about a year older than I am, has very strong symptoms. He needs an assistive device to walk or move around. His older sister, my cousin, has this crazy tricycle thing that she goes all over the place on.
There are all of these other parts of the mystery that we’re trying to sort out, [like] why some people may have shorter repeats, but present with stronger symptoms than other people with longer repeats and fewer symptoms. The more people in the world that we can get to participate in these studies, the more we’ll know about it, and it’s very reasonable to me we’ll know more about all sorts of genetic questions.
What breakthroughs and discoveries do you hope for people with ataxia in the near future? Well, we’re all working for a molecule, as they say. If you can get the right drug introduced into your cerebellum, which gets nourished with blood flow, then presumably you’d be able to reduce the symptoms in the same way certain potassium channel blockers, as they’re called, reduce symptoms and target Parkinson’s and multiple sclerosis and other diseases that are related to coordination and fine motor movements and so on.
So the cool thing is if we could learn enough about spinocerebellar ataxia 27B so that you could design a drug that would address it specifically and improve people’s quality of life in the short term, and then maybe eliminate it in the long term.
Are you hopeful that you’ll see this discovery in your lifetime?
Well, that would be great if we found a drug in my lifetime to improve my sister’s and my brother’s quality of life, my cousin’s’ quality of life — that’d be fantastic. But perhaps what I’m really hoping for is, my grand-nephew just got engaged, and I would love it if they could sort out their eggs so that they don’t pass it on. They may or may not have it; they’re so young that the symptoms don’t present. You don’t know whether or not you have it till you’re in your 50s and 60s generally.
Is there anything else you would want to tell people?
This is a big deal to me. Many people have heard of Parkinson’s, heard of multiple sclerosis, and many people have not heard of ataxia, even though the symptoms present in so much the same way that they’re often misidentified or misdiagnosed. So by raising awareness, we hope to improve diagnosis and make everybody’s life better.