A Family's Saga Through Genetic Dimentia; Public Artist in Residence

All Of It with Alison Stewart

The episode features two distinct segments: an interview with author Robert Kolker about his book 'The Vanishing Family,' which explores the

Key takeaways

• Robert Kolker's new book details a family of nine siblings carrying a rare genetic mutation for frontotemporal dementia (FTD).
• Research into FTD-related tauopathies may provide a 'backdoor' or test kitchen for developing Alzheimer's treatments.

Main topics

• Frontotemporal Dementia (FTD)
• Genetic mutations and inheritance

Notable quotes

If there was a test that could tell you... you had developed a rare form of dementia and that there was no cure, would you want to know?

Conclusion

The episode offers a profound meditation on how both biological mutati

Transcript preview

Speaker 6 (0:08) This is All of It. I'm Alison Stewart, live from the WNYC studios in Soho. Thank you for sharing part of your day with us. I'm really grateful that you're here. It's book week here on All of It. All week long, we're speaking with authors about their work. And tomorrow, we're getting... off of the radio and into the New York Public Library for this month's Get Lit With All Of It book club event. I'll be in conversation with George Saunders about his novel Lincoln in the Bardo. And our special musical guest is the Metropolitan Opera, which is adapting the novel and is joining us for a preview. Now, tickets are sold out, but you can watch the live stream. Head to WNYC.org slash Get Lit for more information. That's the plan. So let's get started today with today's book conversation with author Robert Kolker and The Vanishing Family. Speaker 6 (1:08) If there was a test that could tell you in your youth, maybe 10 or 20 years down the road, you had developed a rare form of dementia and that there was no cure, would you want to know? That's the question that faced a group of nine siblings. When their mother started to act strangely, stopped washing, stopped caring about the kids, seemed unable to manage daily life, drank, they chalked it up to... alcoholism and depression. But then one sister started exhibiting the same symptoms, and then another, and then a brother. They all had frontal temporal dementia, FTD. and it was inherited. It usually presents in your 40s and affects the part of the brain that are responsible for social cues, organizing, and decision-making. There is a 50-50 chance they passed it on to their own children. Members of the family with successful careers and loving families were suddenly someone else. But research into FTD might help find a cure for other neurodegenerative diseases, including Alzheimer's. This story is the subject of journalist Robert Kolker's new book. It's titled The Vanishing Family, Love, Fate, and the Quest to End Dementia. It is out today. Robert, welcome to all of it. Speaker 3 (2:21) I'm so glad to be here, Allison. Thank you. Last Speaker 6 (2:23) time you were on the show was for your book Hidden Valley Road, which is about a family that struggled with schizophrenia. It's a great book, by the way. But I understand the family for this book, they contacted you. Who contacted you? What did they want? Speaker 3 (2:36) Yes, I wasn't necessarily looking for another family or another book about families. Barb, the youngest of the nine siblings, contacted me two and a half years after Hidden Valley Road came out, saying, we're a family and we have a disease. But the second she started talking, I was floored by just how different their predicament was from schizophrenia. People with severe mental illness don't know how it's caused. and still there's no smoking gun gene. This family knows exactly what their genetic mutation is. They know what causes it. And people with schizophrenia can't test for it early. This family, as you said, they have this crystal ball they can look into, and they have to decide whether or not they want to know their future. And what follows, I think, I took my breath away reporting on it. It's an inspiring story of what people do for one another when the worst happens, but also it's about what the nature of the self is. If one little genetic mutation can change your whole personality, then what is a personality? And what about us is real and lasting? I think this is perhaps a more exaggerated version of what any of the listeners might be experiencing with a loved one with dementia in their life. They wonder, is the person I know still there? If she isn't, then what happened? And what is it about any of us that really lasts? Speaker 6 (3:55) What did you know about dementia or Alzheimer's before starting this project? Speaker 3 (3:59) I think I had the same misconception that a lot of the public had, which is that when you think of dementia, you think of Alzheimer's. It's almost like an interchangeable term, like Kleenex would be for tissue. But it turns out that... While in the beginning, it was just simply assumed that maybe 80 or 90 % of all dementia was Alzheimer's, now it's more like 60%. And FTD is just one other variety of dementia. There's Lewy body's dementia and corticobasal degeneration and primary progressive aphasia. And you could even lump ALS scientifically into that category. And so you have a... scientific world now that has a lot more feel for the nuance of things. And that's important because some of these conditions are actually theoretically treatable now, like FTD is in many cases. And not only that, they could be a backdoor toward treating more complicated conditions like Alzheimer's disease. Speaker 6 (4:53) Why did you decide to write about this family? Speaker 3 (4:55) At first, I was drawn to this backdoor idea. I thought, could this family cure Alzheimer's? You know, why aren't we paying attention to simple genetic diseases that genetically could end up being a test kitchen for a treatment for Alzheimer's? But then very, very quickly, I got to know the family. And again, my breath was taken away by... by just what they did for one another. And also the dilemma they faced. You have Barb, for instance, who was the first woman who contacted me. She spent her teenage years alone in the house. Her siblings were all older and at college or out of the house. And she was watching her mother change. And it was the most nightmarish time of her life, three or four years, basically raising herself. Flash forward decades later, the family is finally diagnosed after several siblings develop similar symptoms. And she's 32 years old and married and has two children. Now, she could take a test and find out if she's going to get dementia. But if she learns... bad news from that result, then suddenly she's going to look at her children and see that they're going to have the same awful experience that she had. They're going to relive the most nightmarish time of Barb's life. That's the first problem. The second is that she's going to become like her mother. That's obviously not desirable. And then the third nightmare is that if she has it, her two children have a 50-50 chance of having it too. And that was just unbearable. So she had been spending years. crusading. The whole family had, but Barb at that time was the leader in trying to get a diagnosis and then find out if there's a test. And she succeeded. It was a moment of triumph. But in that moment of triumph, she suddenly pumps the brakes and says, wait a minute, I don't want to know. And the next 10 years of her life, she ends up both not knowing. and obsessing because you can't just put it out of your mind. It's like saying, don't think of elephants. Suddenly all you're thinking about is elephants. Speaker 5 (6:52) It's interesting. You described Barb was born in 1976 and her oldest sibling, Kathy, was born I think in 1951. Describe their mother, Kathy's mother, when she was young versus Barb. Really Speaker 3 (7:05) two different women. Kathy's mother, Jean. was eventually a mother of eight children. Barb was the ninth child born much later. And she got her hair done twice a week. And she never left the house without being beautifully, amazingly dressed. And she read to her children. And she gave advice to other mothers because it was clear that their family in their suburb, they were the family that was going to send all their kids to college. Not everybody in town was doing that. She raised children who went on to not just college, but many of them got graduate degrees. It was the 70s, the older children, they kept the girls, they kept their names when they got married, which is a forward thinking thing. You know, this was a family that was really on it. But by the 80s, when Barb was growing up, it was a totally different gene. She never went out. She barely ate. She certainly didn't cook. She was smoking and drinking most of the time and watching TV. The community kind of turned on her and thought, well, she's really let herself go. Her husband moved on. And while they didn't divorce, he had a whole other life outside the house. And it was not terribly judged for it because the explanations at the time were depression, alcoholism. nervous breakdown, menopause, whatever you have it. And this is, I learned, a typical story. I've spent three years now writing about FTD, and I keep running into families where it takes 10, 15, 20 years just for some doctor to suspect that what's happening is neurodegenerative and not sociological, not something that requires a good therapist. And it's just amazing that way. Speaker 5 (8:46) Why does it take doctors so long to diagnose it? Speaker 3 (8:49) Well, I think... In a superficial way, a lot of them had the same preconception about dementia that we all did. They thought, you know, if it's Alzheimer's, it's about memory and it's about cognition. It's certainly not about suddenly becoming impulsive or suddenly not caring about your kids or suddenly drinking. We have a slightly more nuanced view of that now. But also, I don't think that, and this is still a problem today, a lot of doctors don't want to... think about testing their patients for incurable diseases, because what's the point? And that's a really difficult thing that the family has had to reckon with. No one wants to test for an incurable disease because there's no cure. But you'll never cure these diseases unless more people test for them, because you need enough patients to get the interest of drug companies and researchers at academic institutions. Speaker 5 (9:38) We're discussing his new book, The Vanishing Family, Love, Fate, and the Quest to End Dementia. a family of nine siblings who learn they each have a 50-50 chance of inheriting a rare form of early onset dementia. My guest is author and journalist Robert Kolker. The book is out today. What makes FTD unique? Speaker 3 (9:59) The researchers who study it appreciate it because it seems to be where neuroscience bumps right up against psychiatry because it affects the personality so early. I mean, I think all of us who have a loved one who has a more common dementia, like Alzheimer's, they do wonder, has the person's personality changed? But usually before that, it's preceded by years of memory problems or cognitive issues. Whereas these folks, they change right away. They become different people. And so, you know, for someone studying FTD, they think it's a window into finding out how. how the brain works, what makes it tick, how we might be able to manipulate it one day to, you know, not just stop these diseases, but perhaps, you know, in a science fiction kind of way, maybe alter other kinds of, you know, aberrant behavior before it's a problem. They really actually are dreaming of a day where you can take medicine for future dementia the same way we take medicine now for future heart disease. Speaker 6 (11:02) That's interesting. So as the name suggests, the damage is done to the frontal lobe and the temporal lobes of the brain. What role do these lobes play in a healthy brain? Speaker 3 (11:14) It was a mystery for many years. And in recent decades, we see the frontal lobe as sort of the command center, the executive function area. Speaker 3 (11:24) And that has a very resonant meaning for most of us. You think, oh, executive function, it helps you get through the day. But I think in a technical way with the brain, the brain isn't exactly telling you what to do all day. What it's doing is it's triaging all of the data that's coming into it from all the body's different senses and deciding what's important and what isn't important. So you could be sitting somewhere and there's a bird chirping and there's something cooking in the oven and the doorbell is ringing and you have to decide what... on earth is going on and what's important and what isn't important. And the folks with frontotemporal dementia, it's not just that they don't care about the oven and leave the oven on. It's that they kind of lose all of what neuroscientists call social cognition, which means they don't worry about consequences to actions anymore. And they don't necessarily understand that other people have thoughts and feelings. So they appear sometimes quite childlike. They jump to the front of the line, not because they are angry or impatient or hate waiting. They go to the front of the line because they want what's at the front of the line, and they don't think about the consequences. Speaker 6 (12:30) Yeah, there's one character in your book who shows up at a party. She's not invited to. Speaker 3 (12:33) Yeah, exactly. And the neighbors Speaker 6 (12:34) are like, why is she showing up at this party? Speaker 3 (12:37) Yeah. And then they leave a little note on the door saying, you know. to the