SCOTT SIMON, HOST:
Robert Kolker's new book is at once a tragedy, a mystery and a testament to a family's love. It's a family who discovers that some of them have a time bomb ticking away in their genes and in their brains - frontotemporal dementia, or FTD. Barb is the youngest. She has little memory of what her mother was like before she began to change. But she has seen what's happened to some of her older siblings.
ROBERT KOLKER: (Reading) Barb couldn't stop dwelling on what it would be like if it started happening to her. The signs would be subtle at first, a few random idiosyncrasies - saying the wrong thing in public, being oblivious to a friend in trouble. Barb would probably insist she wasn't changing at all. Everyone does. Next, she would start doing things the old her never would have done. She might have run red lights, tell off her boss, ignore her children, fall out of love with her husband. It would take some time, some tough conversations behind her back. But one day, the people closest to her would decide that she had become a completely different person. Would she even know it was happening?
SIMON: "The Vanishing Family: Love, Fate And The Quest To End Dementia" is by Robert Kolker, contributing writer at The New York Times Magazine. And he joins us from our studios in New York. Thank you so much for being with us.
KOLKER: Thank you, Scott.
SIMON: Tell us about this family in Pleasant Hills, Pennsylvania. The parents are Ollie and Jean. There are nine children, some of whose names we'll get to. They were happy, weren't they?
KOLKER: They were. The kids largely grew up in the '60s and '70s. The youngest, Barb, was a late-in-life baby who grew up in the '80s. There were nine of them in all in a middle-class community outside of Pittsburgh, with a high-achieving, you know, professional parent and a lot of the kids going on to graduate school. They were well-known in the community because they were a large family and because of their accomplishments.
SIMON: How did Jean, the mother, change over time?
KOLKER: She changed in a way that - I have since heard about other families going through this. They couldn't get a handle on what was happening to her. Some people thought it was depression. Some thought it was alcoholism, perhaps even menopause or just a nervous breakdown because her marriage was failing.
But this was a very put-together woman who read to her children, who was very on top of things. And suddenly she didn't want to do anything. She had complete apathy and sat around the house. She didn't take care of herself anymore. She didn't do her hair or get new clothes, and she stopped cooking for the family. It's a disease that makes you apathetic, makes you compulsive and impulsive - not necessarily angry or sad, but really a slave to your immediate desires.
SIMON: We thought it was interesting and especially cruel that FTD often begins to manifest around middle age.
KOLKER: It was breathtaking to hear it happening over and over again with this family. It happens in their 40s or perhaps in their 50s. And unlike a lot of other diseases, like, say, Huntington's or ALS, which might affect in that time of life, it doesn't necessarily affect your lifespan. So you could live for decades with a completely different personality from what you had before. The people around you are sort of left to watch you and sort of come to terms with the new person you are, and that person need - might need round-the-clock care for decades.
SIMON: What happens to Christy and then Mary?
KOLKER: Christy is one of the older children. And some years after the mother in the family dies, she starts to display some symptoms that are frighteningly like her mother's. Years after that, when Mary displays symptoms, then the family can't deny it anymore. There's something genetic happening. I think what they all slowly realize is that whether or not they personally have the disease - and there's a 50/50 chance that each of them have it - they are going to be affected by it. Either they are going to change, or they're going to watch some of the people they love the most in the world change.
SIMON: How did you come upon this story? How did you come to tell it?
KOLKER: This family found me. I had written a book called "Hidden Valley Road," which was about a family with schizophrenia. The mystery of schizophrenia, of course, is that no one knows the cause. The mystery of FTD is why we haven't cured it yet if it's just one genetic mutation. This family offered an inspiring story of what people do for each other who are really facing some of the worst things you could face. But more than that, they also offered a window into how the brain works, how the brain forms the self and personality. I was just amazed at how close we are, perhaps, to curing this disease and leading to treatments for Alzheimer's. It's a family that's sort of on the edge of the future in so many different ways.
SIMON: How could FTD, although distinct from Alzheimer's, be instrumental in helping us find a treatment for Alzheimer's and maybe other brain conditions?
KOLKER: The family knows exactly what is causing their disease. They know that there is a mutation in the gene that produces a protein in their brain called tau. As it happens, the tau protein figures prominently in Alzheimer's disease as well. And we're at a time in research where finally the Alzheimer's research community has realized that Tau might actually be a major player in Alzheimer's, and they're trying to come up with tau treatments.
Studying this family, who specifically have a tau disorder - a lot of researchers think families like this could be a back door or a shortcut to test out treatments that could be helpful to the millions and millions of people around the world who suffer from Alzheimer's. I really was amazed at the beginning. I thought, could this family actually lead to a cure for Alzheimer's?
SIMON: If science can establish that you have a 50/50 chance of developing FTD, would you really want to know that?
KOLKER: Scott, I thought I would want to know. When I first started talking to the family, I thought, well, I'm a journalist. I want the facts. I want to know what's going on in my life. I talked to maybe 15 people in the family who all have to make that choice, and they all had different takes on it. And each one, I thought, had a really satisfying rationale.
There are some people who didn't want to lose their healthcare. There are other people, like Barb, for instance, who spent her teenage years watching her mother vanish, and she had children now who are entering their teenage years. And if she had the condition, it meant that - not only that she would be repeating her mother's life, but her kids would be repeating her life. And then they might have it too. It was like a triple threat.
There are some people who went ahead and had children, thinking anything can happen to all of us. They thought, you could get hit by a bus tomorrow. Why should I not have a child because of a neurodegenerative condition they have a 50% chance of having and that might be cured decades from now? And so my mind was open to many different takes on this.
SIMON: We often see somebody with a brain disorder like dementia who seems different from the person we knew. I find it interesting when you cite the work of a British philosophy lecturer named Harry Lesser.
KOLKER: I went looking, searching for any take on the self and what happens when people have dementia. Are we still ourselves? And if so, what is left behind? Harry Lesser had an amazing take on it. He said, yes, the person still remains, even if their personality seems completely transformed. And his reasoning was, we all change. Just because it's happening more vividly and dramatically to folks with dementia doesn't mean that you should put them in a special category. We all live in time. We all change over time. We all will be different at the end than we were at the beginning, and we all are vulnerable to change. And these folks may have fallen prey to a dramatic vulnerability, but it doesn't mean that they aren't the same person.
SIMON: Robert Kolker - his new book, "The Vanishing Family." Thank you so much for being with us.
KOLKER: Thank you, Scott. Transcript provided by NPR, Copyright NPR.
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