Earlier this month, I had the opportunity to speak with Joselin Linder, author of outstanding new memoir The Family Gene (Ecco Books, 2017), in which she recounts her and her family’s experience dealing with a mysterious genetic condition. Read on to see what she had to say about how the memoir came about, genetics, her family’s experience dealing with this private mutation and much more.
Andrew DeCanniere (AD): To begin at the beginning, how did you decide to write about your story in the first place?
Joselin Linder (JL): That’s a really good question. I was in a writing group and I was writing fiction and articles and different things like that, and I had a friend kind of go ‘Remember how you told me that story about how your dad died?’ And I was like ‘Yeah.’ And she said ‘Well, you should be writing about that.’ I remember going ‘But it’s just my life. How is that interesting?’ I genuinely hadn’t thought of it as a narrative or as an interesting story. It just kind of felt like this tragedy that happened to my family, but when she said that, I started to think that maybe there is something there. I think that, for me, the entree into that world was genomics and all of these genetics technology we have now. All of a sudden, people are doing 23AndMe and Ancestry DNA. They’re sending their DNA away to these companies, and yet nobody even really knows what it means. Even for doctors, the genetics course in medical school is extremely short, but I feel as though that’s all about to change. Pretty soon, medicine is going to be looked at at a genetic level — everything from bacteria to cancer. It’s already happening.
All of a sudden, I felt it should not just be for professionals but that all of us should understand — at least on a basic level — what is happening and what these things are. Then, I also felt that having this family story and this illness that is the centerpiece of the story may make it easier for people to understand it or to follow it. For example, I thought that Rebecca Skloot’s The Immortal Life of Henrietta Lacks did a great job of bringing the reader to the science by giving us this really strong narrative arc. Basically, I wanted to do something similar using genetics and our story.
AD: And it really is amazing when you think about how recent our understanding of genetics really is. Until very recently, you can’t really say that we had a good understanding of our genes or how everything works.
JL: Right — even just to have the basics. I feel like I don’t even go that far into it. It’s really like an introduction to genetics, but at least there’s a baseline understanding. If you read the book, when you send away for your genome, you can kind of know what it all means — or you can look it up and find out more. I really think we’re at this precipice. You’re pretty much about to be handed your baby and their genome at the same time. What we’re going to do with that information is really important, and we’re going to be the ones who decide. So, it just seems really important that we have the conversation and that people understand as much as they can.
AD: And the speed of progress — at least recently — is pretty astounding.
JL: For sure. The Human Genome Project was first published in 2003.
AD: Getting to your story in particular, I could certainly see how the whole thing could have come as this complete shock. It seems like it was just totally random — as if one day you’re just thrown into this situation.
JL: I think it really felt like that, because my father was really healthy and then he just wasn’t. Even in the early stages of his illness, he was still quite functional. I think the biggest problem for him initially was the leak — the lymphatic leak. It doesn’t hurt. It’s not a painful condition. It’s uncomfortable in that you have the swelling, and you have the weight of the fluid, but he wasn’t sick. I think he felt tired and he felt a little uncomfortable, but he was working right up until he was pretty much starving to death. In the beginning, the leak was slower. For him, it happened over three years. It was a really strange thing to experience. It felt totally crazy, but it didn’t feel frantic. It wasn’t as though we were dealing with a person who was in a lot of pain. That isn’t to say it wasn’t horribly uncomfortable, and it was horrible. It was just so confusing. Nobody knew what was happening, but nobody knew it was going to be fatal. I think for most of it, none of us thought it was going to be fatal. I think we thought he would figure it out or it would stop as quickly as it started. At least from my perspective, I really wasn’t thinking I was going to lose my dad over this. I thought at some point it was going to stop and he was going to get better.
AD: And I could definitely imagine how someone could be totally blindsided by something like this. I think most everyone’s inclination is to think that if something is wrong, it will be diagnosed, and it will be treated and that will be that. You assume that there has to be some kind of an answer, some kind of a treatment and a cure.
JL: Right. It just seemed so strange we would just never know what was wrong with him, at least until my great-aunt showed up and said something like it happened to her husband. I also felt like things were so different. It was the nineties. People could figure things out better and that whatever they found out about my great-uncle could maybe be applied to my father, and they would really learn something this time. I think at that point my father kind of knew. He kind of understood that there was something very anomalous — that there was something very unusual happening — and that he needed to not only worry about himself but that he needed to worry about my sister and I, and he needed to worry about other members of the family.
AD: Which you said is not something that occurred to you — this idea that whatever was afflicting your father could also perhaps one day affect you. It was more just about figuring it out so that his symptoms would resolve.
JL: Right. Genetic diseases — or at least these adult-onset ones, specifically — are such an interesting thing, because it is generational. You hear family lore. In Woody Guthrie’s family, his mother had been institutionalized for setting fires and for killing one of her children. As Woody Guthrie got older, and he started to exhibit similar symptoms, that was when they were like ‘Oh, this is Huntington’s Disease. This isn’t some hereditary insanity. It’s a neurological condition.’ With adult-onset diseases, you have generations go by where everything is fine. Nothing seems weird. You can have a 20 year span where nothing is happening, so everyone gets kind of comfortable, and then somebody gets sick. That’s sort of what happened in our family.
AD: And that’s when it sort of all came to light that there were more people within your family who may not have been immediately aware but were impacted by this same illness. Understandably, especially if you weren’t around at the time — or perhaps were little at the time — you may not even be aware someone else had the same condition, and yet it turned out that they did.
JL: Right. I also think that in my sister’s case and my case, we had our grandmother who clearly had the gene — she passed it on to two children — but she was older, and had symptoms like swelling, but she wasn’t sick. She definitely wasn’t sick like her mother and definitely wasn’t sick like her brother. I think there was some confusion about what that would mean for us, because obviously the X chromosome link means women get it differently than men do. So, many of the women in my family have different levels of effect from the gene.
AD: It also seems as though, apart from taking a toll on the person who is ill, it understandably had an impact on all of the other family members as well.
JL: Yeah. I mean, I think that after my father died — my father had been the editor of a medical magazine for the medical community in Columbus, Ohio, where we grew up — and he had written all of these editorials, and they decided to make a little book. So, they put all of his editorials together into this little book, and they had asked the family members whether we wanted to contribute something. My mother wrote this beautiful essay that was titled ‘The Loneliness of a Rare Disease.’
I think it was really difficult for all of us. I was in college at the time, and I had tons of friends who kept asking where I kept going. I was always going to the hospital or flying home, or I took a semester off to be home with my father. Everyone was like ‘Well, what’s wrong with your father?’ and I didn’t know. I genuinely didn’t know. And they were like ‘Well, what does that mean, and what is it called? Can we look it up?’ Everybody wanted language, and there just wasn’t any. It felt really isolating and I remember wondering if everyone thought I was being dramatic. Do they think that I’m a hypochondriac or that my family is just being silly? I don’t know what people thought. I don’t think people were thinking that. This is so silly, but I was just thinking about this recently. I used to have a lot of dreams at the time where I was in a plane crash, but I couldn’t prove it. I couldn’t explain it to anybody. The whole dream would be this stress of trying to explain how I was just in this horrible plane crash. I think that it was hugely, ridiculously metaphoric for what it felt like. You couldn’t explain it. You didn’t have the language. You didn’t have anyone to back you up. I think that with the writing of this book, there’s been a huge vindication. This is real. I’m not just making things up. We have this team that has been studying us. Here’s what they’re doing for us, and here’s what we know.
I know that, with my father, a lot of his patients thought he had AIDS — which, at the time, had a huge stigma and a lot of secrecy attached to it. It wasn’t just that he had AIDS. There were questions about his honesty. Did he lead a gay life? There were these questions about the veracity of my father — almost like the character of my father. I don’t think he would have been ashamed to be gay at all, but I think that was a question that was running through peoples’ minds. It was just really hard because we just didn’t have any words, and we were like ‘We’re not lying. We’re not hiding something. If he had AIDS, we would have said that he had AIDS.’ It was just a really confusing and complicated time.
AD: I think that, as you say, a lot of that probably had to do with the fact that we’re so used to having answers — or, at least, we’re so used to feeling like we have the answers. The problem is, you don’t always have them. That sort of seems to be the assumption — even if it is inaccurate — and, if you don’t have the answer as to at least what the disease itself is, it’s somehow seen as unusual.
JL: Yeah. I don’t know that’s what has happened in my case, but that’s the thing about rare diseases. Up until the moment you have a word to describe what you’re going through, you really can be marginalized by our culture. We expect to understand what you’re saying to us.
AD: And I think that in every decade we feel like we’re living in a time when we’ve never known more than we do now, and like we’re at the forefront of technology and medicine. Like you said in your book, in the 1990s, it seemed like we had come such a long way and, in many respects, it is true. Yet, we can and do get carried away with ourselves and, in giving ourselves credit for what we have learned, I think we can easily overlook how little we still know — even with all of the progress that we’ve made. I think it’s helpful to keep in mind that’s how they felt in every decade — at least in recent history. We’re always convinced that we know the most we’ve ever known, and that we understand the most we’ve ever understood — but I also think it’s important not to lose sight of how much more we’ve yet to discover, and how much more we’ve yet to truly comprehend. That last part is the part I think we can lose sight of all too easily. Even in 2017, there’s so much more that we just simply do not know.
JL: I remember when 20 years ago somebody said to me ‘You’re going to go shopping on the Internet.’ I was baffled. My brain couldn’t wrap itself around the fact. I was like ‘But it takes 120 minutes to load a single image. Who’s going to wait for a shirt to appear?’ I was so baffled by that. I think that if you were to talk, right this minute, about what medicine is going to look like in the next 10 to 20 years, it’s going to be that sort of a huge shift. I think it is going to be as big as what happened with the Internet. We’re going to see a whole new way of treating people. I know that in March, [23AndMe] started allowing for sharing some really important health issues. You could get back your 23AndMe results and it could say you have a 60 percent chance of developing and dying of Alzheimer’s. So, let’s say you get that information and then, suddenly, in the next 10 years, you find they can weed that out. Or you find out today that they can weed it out in your child — you can make sure your child doesn’t inherit that gene. I think many people would say ‘Okay. Sign me up.’
I mean, there are some ethical questions surrounding that. Is it a designer baby if we’re weeding out Alzheimer’s or Huntington’s Disease? Hopefully, we’ll do those things, because I think those diseases are horrible and shouldn’t exist, but then you have other questions. There’s this book Andrew Solomon from the Times wrote called Far from the Tree, where he talks about deafness and the culture of deafness and how a lot of deaf people don’t see deafness as a deficit. So, do you weed out deafness in an unborn child, or do you wait until that person is an adult and let them choose? At what point do you ‘cure’ deafness in a person and is it something to cure? These are questions facing us right this minute.
AD: I mean, particularly with a condition like the one in your family, I could see how gene editing could be a complete game changer, especially since it is adult-onset, which means that, historically, you wouldn’t even know that you have the gene for the condition and could pass it on until you are in your child-bearing years or later. You could already have children. So, something like gene editing could change everything.
Speaking of being unaware that you have the gene, or that you have the condition, you also discuss how you and your sister both discovered that you have the condition.
JL: Yeah. I think we might not even have known that we have these blockages, though, until something sort of scary happened. My dad’s first cousin, Valerie — who I write about in my book — had a stroke. She had her spleen removed and a TIPS procedure — which is the same procedure to prop open her portal vein as the one that I had. She threw up what she described as coffee grinds. That’s when she knew she had a blockage, because the blockage doesn’t make you feel bad. You don’t feel unwell. Your blood finds another path because one is blocked. So they found mine because I had lymphatic swelling in my legs and in my hands, and I think that they thought something may be going on, and so I had an MRI. That’s when they saw my blockage. It became something that our doctors are monitoring, to see who else had blockages, but there really isn’t a great answer for these conditions. I think that otherwise we just kind of hoped that we were like our grandmother — that we could live 91 years and not really have any health problems, maybe a little swelling here and there. So, she didn’t have any extreme blockage in her portal vein.
AD: Yeah. I just thought that was so surprising, because in the book you had said that you went in for what you thought was a routine check-up, taking advantage of your student health insurance while you still had it, and that’s how they had stumbled upon it — at that check-up, or as a result of it, anyway.
Speaking of health insurance, you talk about how after your visit to the doctor, the doctor likely entered information about your condition into the system, and how that was probably why you had been unable to get health insurance in Ohio — how you were unable to get coverage because of this pre-existing condition. That just strikes me as particularly relevant as well, especially when you consider how there’s this extremely aggressive push from some to repeal and replace the Affordable Healthcare Act — also known as the ACA or else as ‘Obamacare.’
JL: Yeah. This particular topic is interesting, because if you have a genetic condition there’s a mediocre amount of protection under the Genetic Information Nondiscrimination Act — also known as the GINA Act, which was signed into law by George W. Bush. It actually does protect people with genetic conditions. I don’t know how that would impact me, because my disease is unnamed. So, I probably would just have this string of pre-existing conditions. I could get some protection, but it’s hard to say. Then, the other piece of it is that I live in New York state, which is a fairly liberal state, and they’re trying to pass universal healthcare. I am really hopeful it will pass within the year. It’s places like Ohio, where my sister is, that I get really concerned. I worry that for them it’s going to be much harder to pass universal healthcare and make sure she’s protected. So, I do worry about people in the family. I mean, I worry about the whole country. It’s such a stupid thing that we can’t take care of our population.
AD: I know. I certainly would like to see universal healthcare throughout the country myself. I mean, if you look around the world, there are a number of other countries that already offer such coverage to their citizens, and they are so much better off as a society — so much healthier, in many ways — because everyone has access to quality healthcare.
JL: Insurance is such a superpower in this country. I mean, it’s ridiculous. There is no transparency. Why isn’t it $300 across the board for an MRI? Why isn’t there a set amount of money? It’s different in every hospital — not even in every city. Often, you can’t find out how much your MRI will be until after you’ve had it. So, your insurer might say that they’ll cover 60 percent, but then you don’t know what that means, and you can’t find out. They won’t be able to tell you. Why can’t I know how much each place is going to charge for an MRI so that when I’m choosing where I’m going to get my MRI, I can get the best deal for the 40 percent I still have to cover? Insurance is horrible in our country.
There’s an interesting book that just came out entitled A Stitch of Time. It’s by this woman, Lauren Marks, who had a stroke when she was 27 and living in Scotland at the time. She almost died from a burst aneurysm and ended up being in the hospital for weeks. Her family wasn’t really thinking about what it was going to cost them, and they were all a little bit terrified, and then all of a sudden they got their bill. I forget what she said, but it was like maybe six thousand dollars. In the U.S., if you were in the hospital for three weeks uninsured, it would be hundreds of thousands of dollars, easily. When I lived in Prague, I didn’t feel well and ended up going to the hospital. It was unrelated to the gene, but I ended up being in the hospital all day for tests — I had a bunch of tests done — and I think I owed them something like forty-five dollars. I had to wait a little while, but really it wasn’t any longer than I’ve ever had to wait anywhere else in the U.S.
AD: And, I have to say, I think that it’s so important that your story is now out there as well.
JL: Since the book has come out, I have been approached by a few people with some similar symptoms, which have not panned out to be the same thing we had, but it has been really interesting. I’ve also had a conversation with a genetics researcher who explained that there’s hope for our condition. One of the things I think we’re learning about genes is they might cause other problems, but there’s an element that impacts all of humanity in an interesting way. I think all these rare diseases do, to some degree. For example, in the book I talk about a friend of mine whose daughter has a condition that causes global developmental delays and all of these other problems, but the kids also have these superstar immune systems. So, there’s this team studying these kids to see what we can learn from that.
In our case, our gene has a lymphatic component that’s deadly, but it also has taught us that our livers have a heartbeat. Your liver actually beats, which is something that wasn’t always accepted. It was kind of suspected, but it wasn’t really accepted as true, because they couldn’t really figure it out or hear it or understand it. So, our genes sort of prove it, and that’s a really big deal in terms of when you think about heart conditions or blood pressure conditions. Knowing there’s a second blood pressure point in the body is really valuable. We feel hopeful there is going to be a cure in our lifetime. So far, there have been six generations since the gene mutated in my great-great-grandmother, and there are 23 kids in the sixth generation, and none of them have the family gene. I feel like that’s a huge thing that I am really proud of, but now to also have this hope that maybe in our lifetime there could be a cure — that would be really amazing.
Joselin Linder is a regular contributor to the NEW YORK POST, whose work has also been featured on THIS AMERICAN LIFE, MORNING EDITION and LIFE OF THE LAW. She spoke at the TEDX GOWANUS event in Brooklyn in 2014, presenting for the first time on the subject of her family gene and the deadly illness to which it leads. Exclusive to just fourteen people, the story of the gene is told in Linder’s new book, THE FAMILY GENE, available now from Ecco Books. She lives in Brooklyn with her husband and two dogs. For more information, visit her website. You can also find her on Twitter, Facebook and Instagram.