In this week’s episode, influencer Brooke Eby joins Brain & Life co-host Dr. Katy Peters. Brooke discusses her experience living with amyotrophic lateral sclerosis (ALS) and why she chooses to share it with the world on social media. Then, Dr. Peters is joined by Dr. Richard Bedlack, Stewart, Hughes, and Wendt Distinguished Professor of ALS at Duke and director of the Duke ALS Clinic. He explains ALS and what hopeful research is on the horizon. 

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Episode Transcript

Dr. Daniel Correa:
From the American Academy of Neurology. I'm Dr. Daniel Correa.

Dr. Katy Peters:
And I am Dr. Katy Peters. And this is the Brain & Life Podcast. Well, hello Brain & Life listeners. Welcome to the podcast again and good afternoon, Daniel. So did you get to check out that recent solar eclipse?

Dr. Daniel Correa:
I saved my eyes. I got the glasses. We took a quick break in our office. I got to join some of the students at our medical school outdoors. It was beautiful weather in New York. But it was also wild how disoriented some people felt that day, in the afternoon. I felt a little weird at dinner, but who knows what the reason and cause was for that. And even those several organizations have said afterwards because so many people posted online, "Oh, I felt weird. I was all thrown off." That right now there's not any evidence that the eclipse would affect our health. But so many people noted it that maybe it's another area where we need more research to understand. Sometimes it's just the limitation of data and something like this that only happens every 20 years that probably really limits and challenges our ability to understand how things like this might impact some individuals or more people. How about you?

Dr. Katy Peters:
Well, I think you're hit the nail on the head. Maybe we should chat about this and our brain wonders how does I guess the cosmos affect our brains? I'd love to tap into that. But I felt very prepared for this eclipse. I had my special solar eclipse glasses. I got some extra for family and friends, and we all got to see the eclipse together. It does make you think about your place in the cosmos and how time passes. And it makes you think about really living each day and how fleeting time is, and how the movement of the planets and the movement of the moon, what really is happening as the time is passing on. I got quite a perspective on time on how to live every day to the fullest potential when I talked with our next guest, Brooke Eby. She's a social media star that has been sharing her life about being diagnosed with amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig's disease.
She talks about her personal journey, how she shares and interacts with people on social media and her advocacy work with ALS. She is pretty candid about her diagnosis and her life. She's young, she's in her 30s, and she also talks about the terminal nature of her illness. We talk about how she has to live life in "dog years." For example, packing seven years of life experiences into one year. She calls that ALS time. And while that may seem a little sad, she actually gives a very real hopeful and inspiring discussion about her life and her diagnosis. And later on we'll hear from one of my colleagues at Duke, Dr. Richard Bedlack, he's an expert in ALS. And he'll talk about the disease process and what are the advances taking place to treat and manage these patients.

Dr. Daniel Correa:
Wow. And also make sure if you're interested in more information about ALS, go back and check out some of our past episodes that have covered ALS where we had interviews with musician John Driskell Hopkins and advocates Lori Carey and Brian Wallach, along with great medical interviews about the condition ALS, research in the area and living with the condition. You can also find these podcasts and any articles that cover any condition like ALS on the Brain & Life website. At the top of the page, you'll find the disorders A to Z tab, and there you might be able to click and find a disorder that you're interested in, and take a look at all the different articles in our archive, which will include the podcast episodes. I hope you enjoyed this episode.

Dr. Katy Peters:
Well, hello Brain & Life Podcast listeners. I would like to welcome Brooke Eby to the podcast. As you know, she is a social media star that has been sharing her life after being diagnosed with ALS. ALS stands for Amyotrophic Lateral Sclerosis, which we also know as Lou Gehrig's Disease. It's a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. And Brooke has been sharing her journey with others and I would say she's inspiring to others, including my whole research team today. I said I was going to interview her and they were just so excited because they follow her on social media. And Brooke has been promoting advocacy for all those affected by ALS, and also some other conditions such as muscular dystrophy, I believe. So Brooke, welcome to the Brain & Life Podcast.

Brooke Eby:
Thank you so much for having me. I'm so excited to be here.

Dr. Katy Peters:
So where are you joining us from today?

Brooke Eby:
So usually I'm based out of Maryland, but I am in Florida right now. So I was here actually for the MDA Conference. The Muscular Dystrophy Association had their annual scientific conference two weeks ago, and they asked me to be the keynote speaker, which was a new gig for me. I've done speaking engagements, but never the word keynote in front of them, which makes it a little more, to me it felt a little more high pressure, like Ted Talky feeling. But it went super well. We were there for a few days and then came back to my parents' place and I've just been enjoying.

Dr. Katy Peters:
Well, fabulous. That sounds like a lot of fun. And thank you for doing that for great organization. I'm sure it meant so much to all of the attendees and to also the patients and their loved ones. So tell us about yourself. So tell us about what you're currently doing as far as work and advocacy. I'd love to learn about it. I know you're very active on social media.

Brooke Eby:
In terms of work, so I was diagnosed with ALS in March of 2022. I was 33 years old. My symptoms started when I was 29 years old, so it took me four years to get the diagnosis of ALS. As you know, and probably as your whole research team knows, it's really hard to diagnose, especially in a slow progress or especially in a young person who you don't expect to have this death sentence of a disease. Once I got diagnosed, I had already been working at Salesforce. I work in tech. I've been there for years and years. Once I got diagnosed, I took a couple months to and kept it hidden, I would say. I didn't really know how to share it with people. And I didn't really know what my life was going to look like. I'm like, "Should I quit my job? Should I move in with my family? What do I do?"
And I remember my friends being like, "Maybe just operate as normal until you feel a need to not. Don't jump the gun with this disease." So I've continued working and it's honestly a really nice dose of normalcy for me, to wake up and have minion tasks to do every day for my job. And then outside of Salesforce, I started doing the whole social media thing about a year and a half ago, and that's blown up quite a bit. So a lot of great opportunities have come from that. I've gotten to meet a ton of people like your team and so many others who do all of this research and it's been great.

Dr. Katy Peters:
Again, you're very active on social media. What do you like best about sharing your experiences in life on social media?

Brooke Eby:
It's funny, when I first started sharing, I was like, "My North Star is giving a face to ALS." That was always the thing. I'm like, "I'm going to drive awareness for this disease. I'm going to help people living with the disease." And then slowly but surely, I remember one of my friends being like, "Doesn't it help you too?" And I was like, "Oh yeah, why have I never even thought about it like that?" I think before being on socials and all of that, I was a lot more uncomfortable sharing my story. Mostly because in those one-on-one conversations, when you have to tell people about your diagnosis, it's horrible. You're like, "Here, carry this heavy weight for a second and see how it feels." I just felt I was ruining people's days left and right when I was telling them. And they never knew how to react. So usually people would cry and then I would cry and it was just bad.
So once I started sharing on social media, I was able to share it in the way that I viewed it, which was not all depression and isolation and all these bad things that people assume. I was able to share it with the humor that I've approached it with. And I think since then it's allowed me to be more comfortable talking about it. Now I'm nearly desensitized to it at this point. I could talk about it all day.

Dr. Katy Peters:
Well, I like that because you are essentially getting that reciprocal relationship with your followers. It's truly the meaning of social, of developing a community.

Brooke Eby:
It's what social media is intended for. It's probably not how it's largely used, but my mom's always like, "Was anyone mean to you in the comments today?" And I'm like, "No, if you have lost faith in the internet, go to my comment section." Everyone's just there to learn and be supportive and cheer me on and make jokes, and it's been amazing.

Dr. Katy Peters:
Don't lose faith.

Brooke Eby:
I know.

Dr. Katy Peters:
Listen to Rook. So can you share with our listeners, because we have a great audience. The specifics of how you were initially diagnosed with ALS. I know that you had some weakness. Can you delve into that a little bit more?

Brooke Eby:
Yeah, for sure. So it was 2018 when honestly it felt like overnight. I just started feeling like as I was walking, I couldn't quite push off of my left foot. I don't think I was even able to isolate that at the time. I was just like, "I think I'm walking slower. Something feels weird." And I had just moved back to New York City with my job, and that's not a place to walk slowly. People will tell you or physically tell you. One way or another you find out. And so I was walking with a colleague to a conference and she was like, I could just tell she was slowing down for me. And I'm like, "What's going on here?" I thought maybe I was just sore from a workout or something. I was relatively healthy at a very first normal 29 years of life. And it just felt like one day my foot stopped cooperating.
So I ignored it for a little bit until more and more people started pointing it out. And then I think I went to physical therapy for a bit. It didn't get better. And so at that point, once enough people said, "You're definitely limping." I went to my sister and her husband who are both PM&R doctors, and they said... They had me walk on my heels, which I didn't know this was a diagnostic test at all, but they were like, "Just walk towards me on your heels." So I was able to walk forward and my right heel stayed up as I was walking on my heels, but my whole left foot would just slam into the ground. I couldn't keep it up. I couldn't keep my toes raised.
And so they knew that was foot drop. But again, we were all largely in denial. But also just assuming it was something basic because why wouldn't it be? It probably was a pinched nerve in my back, something or other. But I got all of those tests that would've ruled out anything simple and then some. So it ended up being four years of running tests and every test you could imagine. Lots of different types of doctors. I think I heard ALS for the first time, and when I say I heard it, that doesn't mean it wasn't said before, but that's the first time I heard it was two years in, in 2020, when I got an EMG. And they're like, "You definitely have denervation in your left foot, but it's nowhere else in your body. So it's not enough for us to give a diagnosis at this point, but it's something we want to watch out for and see how it progresses."
And I panicked, but they gave me this ALS genetic test and it came back clear. So my family and I thought we were safe. We're like, "Oh, great, I don't have it." Now, come to find out, 90% of cases do not have a genetic marker on that test. So it wasn't a conclusive test, it was just something helpful for the doctor. I just misread or wasn't told what it was. So really, we just put ALS out of our minds until beginning of 2022 when I started losing my balance a little bit more. I just could tell it wasn't getting any better. I wasn't sure it was getting worse, but it certainly wasn't getting better. So went back to the neurologist, they ran the EMG, which if people don't know, that test stings. I think I've had 10 of them.

Dr. Katy Peters:
Oh, yeah.

Brooke Eby:
They stick needles in your muscles and then have you flex those muscles and it cramps them. But that way they can tell if your brain is communicating properly to the muscles, from what I understand. In the most layman's terms. You're probably like, "This girl has no idea what the actual details of that test are." But to me it was like, "Do I have it or not?" So they ran it and they saw it in both my feet this time. So they're like, "Okay, it's officially progressed." And then I think they saw some twitching in my arms and that was the point beginning of 2022 when they were like, "Okay, we can officially call it ALS."
So you have to rule out everything else first. And then on top of that, like I said, because I was in my late 20s, early 30s at the time, I don't think anyone's first assumption was like, "Let's think about this disease that we typically associate with older males." So yeah, that's my four-year journey.

Dr. Katy Peters:
Oh my gosh. That had to be just a very, the one thing I can think of about giving somebody a diagnosis of ALS or receiving it is there has to be a lot of fear associated with that. Because you have seen pictures of people having degeneration, but you're still doing well and you're on social media. How did you deal with the emotional side of the diagnosis and the unknown?

Brooke Eby:
Yeah, I think it's funny, those four years, I wish I was a journaler. I've always said that, but I never start writing in a journal. It's just not something I've ever done. But I do wish I could put myself back in those four years, because I think a lot of the time I was just truly avoiding it. I don't think I was thinking about it. I just put it out of my head and tried living my normal life. After getting diagnosed, I took a couple months to be in shock. I don't think I processed anything. I still remember the drive home from that appointment when they said, "We don't like what we're seeing," type of news. And I just got in bed. I had a couple friends check in on me and I was like, "Don't even," they were like, "We're going to come visit." I'm like, "Don't because I just feel so weird right now." I don't even think I could communicate to someone.
So I spent a couple months in bed mostly. I would Google ALS, I would close out the window, I would repeat, it was just panic and shock. But then I went to a friend's wedding and your research team probably could recite this story as well as I did because I tell it all the time, but two months into my diagnosis, I was a bridesmaid at one of my college friend's weddings. And I show up in a dress that's already a little tight because I had mostly just been lying in bed and eating M&Ms for the prior two months. And I was using a walker, the exact same walker as the brides' grandma. We had the same tennis balls on the bottom. I was really, really embarrassed, but in hindsight, it's hilarious. But I was like, "I got to get out of here. I cannot do this."
And it was all my college friends, a lot of whom didn't know what was going on. So I told my closest friend, I'm like, "Can we leave? Would anyone notice if I just left? I don't think I can do this. It's too embarrassing." And she was like, "What if Instead of it being embarrassing, we just make it really, really fun and just try to make it a really fun night?" And two hours later probably, we had the bride limboing under my walker and I was giving walker rides all over the dance floor. And it was the first time where I'm like, "Okay, people aren't feeling uncomfortable around me. They're enjoying and laughing." So I was like, "Maybe I can approach this in a way that's not as nerve wracking." I feel most people, I'm always nervous to ask questions to someone going through something heavy because I'm like, "What if I say the wrong thing? What if I upset them?"
And so my goal is to break down that wall, ask me anything because otherwise no one's going to talk about it at all. And no one's going to talk about ALS or learn about ALS. So after that wedding, I actually got COVID from it, a couple extra gifts from that wedding. But as I had COVID, I just started writing down ideas of funny things that had happened to me since being diagnosed and posted my first TikTok and just went from there. Typical millennial coping strategy.

Dr. Katy Peters:
I like that coping strategy. What you found was there's a community for you and they're your friends. It sounds they really embraced you from day one as soon as they knew.

Brooke Eby:
Yeah, it's been a wild ride. I'm not even sure at what point it really started feeling like a community. I think in the early days I was a little more shy about what I was going through online. I was vague about it. I was still a little gun shy just sharing with the internet, but now it's I'm over sharing every minute of every day and it really does feel like a community.

Dr. Katy Peters:
So you mentioned you thought you wish you could have journaled. Is there anything that you wish you could tell now in the position that you're in, that you're sharing on social media, that you're in advocacy for ALS? What would you tell yourself back then when you first got diagnosed? What would you tell them now?

Brooke Eby:
Oh my gosh. I don't even know if I could express what it's been like for the last year and a half to that person. Because when I first got diagnosed, I was just confused what anything was going to look like in the future. And honestly, I still am. I still had no idea what life's going to look like in a month, six months, a year. No one knows. But I'm just really aware that I don't know. And I think about it probably more often than the average human. But I think, I always say, "I do think I was meant for this," which not a lot of people love hearing me say that, but if you look at how my life was set up, I'm in a really good position to have something like this come in, and I'm in the right position to be able to fight back. Whereas not everyone is.
I have a strong family, friends, job, financial situation, humor, all of those building blocks allowed it so that when ALS came into my life, I could just solely focus on that head on. Whereas if you don't have those building blocks, ALS will come pummeling in and knock everything over. So I think the girl, those first couple months, I do wish I could have seen the potential of what I was able to do back then, but I don't think I would've believed it in that position. I was just taking it a minute at a time back then.

Dr. Katy Peters:
And that's okay to take it a minute to minute, a second to second, because what you're showing is also you're enjoying it and you're enjoying every second. You're enjoying every minute. So I just say kudos to you. I think that's wonderful.

Brooke Eby:
Thank you.

Dr. Katy Peters:
And how have you adapted? I'm sure you have favorite activities, favorite hobbies, now that you're living with ALS, how have you adapted to be able to do those?

Brooke Eby:
Yeah, physically my life has changed a lot. So in the first probably six or seven months after being diagnosed, I went from using just braces on my legs to a pain, to a walker, to a wheelchair in probably, it might've been eight months, but it was between six and eight. I can't really do the math because my memory's not very good. But I went from walking to being in a wheelchair full-time very, very quickly because my legs are really where it's affecting me. So life has changed quite a bit. I'm not going to workout classes anymore the way I used to. I'm not going to the gym, I'm not doing running, anything like that.
But I have found some adaptations for certain things I like. When I first got diagnosed, I would go and hit golf balls with my dad. He actually found they have an accessible cart, an adaptable cart that you can strap yourself to and still hit. So we did that early days. Now I don't even think I have the core strength to use the cart anymore, but that's an example of a hobby that can be adapted. But now honestly, most of my hobbies don't require a ton of physical activity. I've built my life around what I still can do. So it's social media. I really don't need to be getting into the gym for that. I just tuck into my phone. I'm a big reader. I spend time with family and friends and we just adapt as necessary.

Dr. Katy Peters:
So can you share your hopes and aspirations for people living with ALS today? What do you hope for?

Brooke Eby:
As hopeless as a diagnosis is, when you get diagnosed, they basically tell you go write your will and plan your funeral. There's not a lot they can give you because today there are three approved medications for non-genetic ALS, which is what I have. And one of those is actually being pulled off the shelf likely because it's proven to be not effective. So we'll probably be down to two. And those medications are expected to extend your life for a few months. It's not revolutionary. It's not anything to really hang your hat on. And so the community is forced to be very hopeful in a hopeless situation. So when I gave that keynote a couple weeks ago, what I really wanted to tell them, and I think it was how I opened closed all of it. I'm sure they're sick of me saying it, but I said, "When I got diagnosed, I really had to learn how to live life in dog years." I make every year worth seven now.
And so because of that, because I'm living at the speed of ALS, you all need to operate at the speed of ALS. We need to move faster with the research on this disease. I presented this whole framework to them where I basically said, "Write down every obstacle. What is slowing you down from living or working at the speed of ALS?" Because we need to understand that. And I don't think people do, if it's just funding, tell us that we can go advocate for it. We're going to be the biggest advocates because our lives depend on their work. So if it's funding, tell us. If it's something else, how would we know? We don't know the challenges that the science world is going through as it relates to this disease.
I think the ideal answer is ALS is just a really hard problem to solve, but we know that's not the only challenge they're facing. There's so much more red tape around what they're doing. So I think my hope is that they heard me first of all, but also just anything to get us moving faster. Again, operating at the speed of ALS is not easy, but that's my hope.

Dr. Katy Peters:
I love that. I think that's a great idea. Operate at the speed of ALS and let's get things done and become better allies and advocates for individuals at ALS. So Brooke, you're amazing. And I just want to thank you for sharing your experiences and your advocacy for yourself and for all the community out there that are affected by ALS. And you can check out Brooke on her Instagram I have and on X and TikTok. And as always, thank you to our Brain & Life listeners.

Brooke Eby:
Thank you so much for having me.

Dr. Daniel Correa:
We're excited to start taking your questions and feedback and sharing those responses here with you on the podcast. You can also email or record an audio message and send it to blpodcast@brainandlife.org. And of course, you can also reach the Brain & Life team at Brain & Life MAG.

Dr. Katy Peters:
Hello, Brain & Life listeners. Thank you for joining us today. I'm your podcast co-host, Dr. Katy Peters, and I'm honored to introduce our medical expert, Dr. Richard Bedlack, who will discuss ALS. Dr. Bedlack is a fellowship-trained neurologist with a special interest in understanding the individual needs for patients with ALS and also their families. He is the Stuart Hughes Wendt Distinguished Professor in the Department of Neurology at Duke University School of Medicine. He serves as the director of the Duke ALS Clinic.
He is well-regarded for his teaching, his patient care and his health advocacy. He's been recognized with a myriad of awards. He is also well-known for his fashionable, colorful clothing, which sadly you cannot appreciate today by listening to the podcast. But I would recommend Googling him and checking it out. Still I'm confident that you'll appreciate his knowledge, compassion, and wisdom about ALS. Welcome to the Brain & Life Podcast, Dr. Bedlack.

Dr. Richard Bedlack:
Thank you so much, Katy. It's great to be with you.

Dr. Katy Peters:
Great. Before we start on the specifics of really ALS, which is what we're here to discuss today, can you just tell us more about yourself and where you're joining us from?

Dr. Richard Bedlack:
Yeah. I grew up in a small town in Cromwell, Connecticut, and I was fascinated by neurology from an early age. I remember rolling my little brother down this hill in our backyard when I was about six or seven and wondering why he got up and was staggering around dizzy. So I rolled myself down the hill and experienced what I would later come to understand was vertigo. But back then we didn't have the ability to just Google things. So I'd run inside and I'd say, "Mom, mom, how come this happens when I rolled down the hill?" Well write it down and I'll take you to the library and you can try to sort it out. So that just happened over and over again through my childhood and teenage years that I'd have a list of things. My mom would drop me at the library and I'd try to work it out with the librarian. Why does a person shuffle when they walk? Or why does a person's arm shake or voice tremble?
So the more I learned about it, the more fascinated I got. And eventually I wound up coming to Duke University in 1995 and did my neurology residency here, and that's where I saw my first person with ALS. And of all the things I'd ever come across, I found it to be the most amazing and most terrible. Never seen a history or a collection of exam findings like the one I saw that day. And I was just horrified when the attending I was learning from came in and said, "We don't know why it happens and there's nothing we can do about it. They just have to go home and get their affairs in order." And I said, "Nah, there's got to be a better way." So decided to stay here, and for 23 years I've been trying to build a program here at Duke that gives people and their families options and hope.

Dr. Katy Peters:
And hope is so key. And I guess you really got interested in neurology at a young age. Kudos to you. It took me medical school.

Dr. Richard Bedlack:
Yeah, I think before I even knew what the brain was, I was interested. I don't know why it captured my imagination, but it did.

Dr. Katy Peters:
That's wonderful. So you mentioned ALS, so can you just give us the basics of what it is?

Dr. Richard Bedlack:
Yeah. So ALS is a degenerative disease that most obviously affects a type of cell called motor neurons. Motor neurons are like the wires that connect the front of your brain where you decide to do stuff to the muscles that carry out your actions. And so as these cells die, people affected by ALS experience difficulty walking eventually, or they're wheelchair bound, they experience difficulty using their hands to dress themselves, feed themselves, bathe themselves and toilet. Eventually they need somebody to do all that stuff for them. They experience the gradual loss of speaking and swallowing and eventually breathing. And typically all this happens pretty fast over just a few years. So lives are dramatically impacted and shortened by this disease.
I really think to appreciate the full horror of what this is, you have to use the words of a person who lived with this and she told me, "It's like when you diagnosed me, you put me in a box and every day I know it's going to be a little smaller and tighter. It's going to further restrict what I can do, and eventually it's going to get so small and tight that it's going to suffocate me." And I still get the chills thinking about that description. And it's part of why I worked so hard to try to find a way to stop this.

Dr. Katy Peters:
That would really inspire someone to want to get that patient out of that box and allow them to thrive and spread their wings. Now, are there certain types of people that are more likely to develop ALS?

Dr. Richard Bedlack:
Yeah, so Katy, if you look at the epidemiology, it's primarily a disease of older folks. Peak age of onset is about 60 years old. It's quite rare in young people. It's primarily a disease of whites way more commonly than any other race. And males are affected about one and a half times as often as females. But the thing that's important for people to remember is that there really isn't anyone who's spared from getting this. My youngest patient was 15 and my oldest was 90. And certainly I've seen just about every race come to my clinic with this disease. So even though it's more common in some demographics, nobody is spared.

Dr. Katy Peters:
Well, one of the questions that I get because I treat patients with brain tumors, they're like, "Well, why did this happen to me? What are the risk factors? What caused this bad disease to occur to me?" Are there specific things that we know about risk factors for ALS?

Dr. Richard Bedlack:
Yeah, so we divide the origins of ALS into two categories. One, we really understand quite well, that's genetic ALS. So having a family member with ALS puts you at an increased risk. Having certain genetic mutations puts you at a markedly increased risk. About 12% of everybody that we find with this disease has what appears to be a gene, a genetic mutation that caused the disease. The rest don't have those genetic mutations. We think there are things in the environment they get exposed to. And we know that ALS is more common in certain occupations, like military veterans are two to three times more likely to get ALS. Extreme athletes, like professional athletes, especially in certain sports, contact sports are more likely to get ALS. Certain behaviors like smoking puts you at a bit of an increased risk for getting ALS.
And then ALS is more common in certain parts of the world too. And interestingly, in those areas where there seems to be a lot of ALS, there also seems to be a lot of algae. And so there's a theory now that an algae toxin, specifically one called β-Methylamino-L-alanine, might cause some cases of ALS.

Dr. Katy Peters:
Well, that's fascinating about the algae and the different forms, whether it's genetic or if there's something happening in our environment. Have people even posited about climate change? If there is this algae toxin, could ALS increase or decrease?

Dr. Richard Bedlack:
Yeah. So believe it or not, the prevalence of ALS is expected to increase by about 70% in the next 20 years worldwide. Now the main reason for that is the shift in the population toward an older population across the world, we're just getting older. But I think there are other possible explanations too. And certainly when you think about climate change, yeah, algae thrive in warmer water. So as the lakes and the streams and the oceans heat up, we're probably going to see more algae blooms. And if that really is one of the reasons why people get ALS, that's probably going to increase the numbers too.

Dr. Katy Peters:
I think this is going to be a fascinating area of research. And before we talk about research, can you give us the breakdown of what are those specific neurologic symptoms that you would see maybe on exam, certain signs as a neurologist that you would see?

Dr. Richard Bedlack:
Yeah, so the things that people with ALS have, painless weakness is the main one, and it's progressive. So unlike a pinched nerve that could start suddenly, and there's a lot of pain with it, ALS usually starts gradually. So one day you might notice, "Hey, I don't seem to have the same spring in my right leg as I used to have." But unlike a condition like a stroke, this actually keeps getting worse. And so it becomes more obvious over time that you have foot drop, and then that you're having trouble standing on that leg. And that progressive weakness is accompanied by muscle atrophy, muscle twitching, muscle cramping, sometimes by other neurologic symptoms like there's a symptom complex called pseudobulbar affect where people lose their ability to regulate their laughing and crying. And about half of everybody with ALS has cognitive and or behavioral changes. About 10% actually have frank frontal temporal dementia.

Dr. Katy Peters:
So I interviewed Brooke Eby, she's a social media influencer, she's an ALS advocate. She has ALS herself. She was actually diagnosed, she's one of those young patients. She was diagnosed in her 30s and she's had ALS for a few years. She also is a fan of yours. So just to shout out to Brooke, she was a great interview, but why does the course of ALS differ from patient to patient?

Dr. Richard Bedlack:
Yeah, so Katy, first, let me say, I'm a huge fan of Brookes as well. There's hardly been a day when she hasn't put a smile on my face since she got the disease. I try to always listen to her speak. I recently listened to her speak at the MDA conference and it was amazing. So what you just pointed out is really if I had to pick my niche in ALS research, it's trying to understand why some people can do so well with this disease for so long and others can't. I would say the fastest progression I've seen is a person who got ALS and lived only about six months with it. But I've got people who've been with me for the whole 20 years that I've been running the Duke ALS program. Stephen Hawking had the disease for 50 years. So that's what I call variability between patients.
And I would say it's probably more than one thing that explains that. Certainly I think there are people who do better because of their genetics. There are people who do better because they don't have as many other medical conditions. There are people who do better because they comply with all the evidence-based therapies. But even beyond that, there's got to be something else like why did Stephen Hawking survive for 50 years with ALS? And my research here suggests that there could be something in the gut microbiome that's different about slow and fast progressors. And I've also seen some people who are progressing at an average to fast rate, and then they stopped. And in a small number of cases, they actually recovered from the disease.
So there's about 60 people that I've found over the last 60 years, some in the literature, some in my own practice who appear to have had ALS progress to where they were disabled and then recovered from it. I call them the ALS reversals. And a few months ago, my team and I found something that we're really excited about. We found a genetic mutation in about a third of these patients that's extremely rare in people with typically progressive ALS. And we have a theory for how that could actually lead to ALS resistance.
And so if this theory continues to look promising in the experiments that we have going on, we'll be launching a trial next year to target the genetic abnormality that these people have and try to give it to everyone, and see if we might be able to reverse ALS in everyone.

Dr. Katy Peters:
Well, that is amazing and exciting because I think that people with ALS, they have such a, once they get diagnosed, there's so little hope. And if you can give them an opportunity to reverse that very serious, ultimately life-threatening illness, then there couldn't be anything more exciting. So congratulations on that discovery. I can't wait to learn more.

Dr. Richard Bedlack:
Thank you. Yeah, hopefully the next few months will show us that that target continues to be promising.

Dr. Katy Peters:
And this is so reflexive of what I see for my brain tumor patients. I have some patients with the worst possible diagnosis of glioblastoma where the survival is really 12 to 20 months, but they've been hanging with me for the last 15 years.

Dr. Richard Bedlack:
Yep. Well, I've always loved oddballs, Katy. I consider myself to be an oddball because of my early interest in the brain. I was certainly an oddball in school, and certainly my outfits made me stand out as well. Not always in a good way. But yes, I love oddballs and I'm really excited that I've been able to work in a study of these oddballs that do extremely well with the disease and might've found something that might someday allow other people to do that well.

Dr. Katy Peters:
It just adds more to the idea of neuro diversity, which I think is important.

Dr. Richard Bedlack:
Yes.

Dr. Katy Peters:
So you already told us about stuff that's going on in your lab, in your research. Can you tell us about over the last year, have there been some other recent advances to help patients today with ALS?

Dr. Richard Bedlack:
Definitely. So I'll just pick two. So one is we've had this drug now for a couple of years called Tofersen. It's an antisense oligonucleotide. And what it does is markedly reduces the ability of a mutant SOD1 gene to express mutant SOD1 protein. So this genetic form of ALS, we've known about it for decades, and we've had multiple different models. And we're absolutely sure that the way this mutation causes disease is through a toxic gain of function. And therefore we know exactly what we need to do to help people with that form of the disease. We got to get rid of as much of this mutant protein as we can. And so this drug was put into trials, and it went in front of the FDA, I guess about two years ago now. And I think there was some controversy about whether this should be FDA approved. And the reason is it's hard to find people with this form of the ALS.
And so you had to do a pretty small study and you couldn't be very selective as far as picking patients that you thought might be able to show you a response on the clumsy outcome measures that we use in ALS trials like the ALSFRS-R. You couldn't be so selective because it'd be impossible to fill up the trial. And so mostly what they showed was the ability to knock down mutant SOD1 protein, and a pretty significant reduction in a biomarker that we think comes from injured neurons called neurofilament light chain. And we were so confident that those two things would lead to a later clinical benefit that we're able to get the FDA to do an accelerated approval. It was the first drug in ALS to ever get an accelerated approval.
So what's new over the past year is that we've now seen the data on people that have been on this drug for a while, and it's extraordinary. About half of them have stopped progressing and about 20% are recovering lost function. The idea that we could do this with ALS was super controversial. Many people didn't believe it. They thought the best we could do is slow it down, but it appears when we hit the right target, we can stop it and in some cases reverse it.
The other is that we've learned that there's a certain diet that seems to be associated with the slowest progression. So for years, we've wondered, people always ask, "What should I eat?" And if you Google diet and ALS, you come across a lot of sketchy websites that tell you all kinds of things. Cut out all sugars, cut out all gluten. Things where there's really not a lot of science. But we finally now know that a diet with a high glycemic index appears to be the one that's associated with the slowest progression. So it's more than just telling people to maintain their weight, which we've been doing for years. Now, our dietician comes in and talks about what's a diet with a high glycemic index looks like? How do you get foods like that into your diet?

Dr. Katy Peters:
So I think those are two very interesting. Those are the same questions I feel asking about diet and asking about where it comes from, those are what happens in our clinic. And for so many neurologic disorders, and I'm sure our listeners and their caregivers are definitely wondering about these things. Now, you mentioned a dietitian, and so I'm going to ask another question. I know that you're the physician for the patients, but it sounds like you probably need a big team to care for your patients. Can you tell me about what team is needed to take care of a patient with ALS?

Dr. Richard Bedlack:
Yeah, Katy, to build a foundation of great care in this disease, you really do need a village. So this disease can affect so many different parts of the body, so many different parts of someone's life, often so quickly that it's virtually impossible for one clinician to keep up with it. And so this model of ALS care has arisen, which is called multidisciplinary team care, and it's incredibly resource intensive. I have 20 clinicians on my team. We typically see 20 patients in a day. You can imagine that is a huge money loser for the institution. So there better be some good evidence that this works. And it turns out there is.
So people who come to these kinds of clinics have persistently better quality of life scores throughout their disease, and they live more than a year longer compared to people who don't. So on my team, I've got multiple respiratory therapists, a pulmonologist, I've got a nutritionist, I've got multiple different speech therapists, some focusing on swallowing, some focusing on communication. I've got multiple physical therapists, I've got multiple occupational therapists, I've got a variety of different kinds of nurses that have different expertise, I've got a social worker, and I've got a research coordinator as well as of course, myself.

Dr. Katy Peters:
I think that's great to provide that so much support to the patient. What do you do also to support the caregivers?

Dr. Richard Bedlack:
Yeah, so this is an evolving area. Typically, in North American medicine, the way that we do things, the encounter is scheduled with a patient, and that's the one we're providing medical advice to. But certainly, we do provide a lot of support to caregivers. My social worker, that's what she does most of the day, is fields calls from caregivers who need support, need encouragement, and I would say there's a lot of it out there. And so for caregivers that are listening who maybe haven't connected, don't be afraid to tap into the social worker in your clinic. They know a lot about those resources. And some of the specific resources that caregivers of mine have found to be most helpful. There's lots of support groups across the country. Some are virtual. There's some new websites that are just incredible.
I'm a huge fan of this website called Your ALS Guide, and also of a website called Roon. So these websites have loads of videos. Roon videos have to be really short. That's one of the things. It's almost like the TikTok of ALS care. They're all organized around a question that a person with ALS might have. For example, what's the best diet for a person with ALS? And there'll be a minute or two minute video from an expert explaining what the best diet is for a person with ALS. So tons of information out there, but I also think caregivers, they need a lot of emotional support too. I went through a neurological disease with my mom last year. She passed from advanced Parkinson's.

Dr. Katy Peters:
I'm so sorry.

Dr. Richard Bedlack:
I realized, well, thanks, but I realized I was completely unprepared for that role. Even being a physician for 23 years taking care of this patient population. It's different when you're there at night by yourself and your mom's having a spell. They just need to hear that they're not alone I think. They're not expected to be experts on day one. It's going to be a learning process. And I think one really important message is that self-care is not selfish. Yes, you're going to spend a lot of your time taking care of your loved one with this disease, but you got to take time for yourself too. And I think that makes you a better caregiver.

Dr. Katy Peters:
I couldn't agree more. And I'm sorry to hear about your mother in that journey.

Dr. Richard Bedlack:
Well, thanks. Yeah.

Dr. Katy Peters:
I mentioned before you're quite a fashion icon. I know our listeners can't see it today, but again, Google some pics, but can you tell us a little bit more about your fashion and why?

Dr. Richard Bedlack:
So Katy, from an early age, I just had this epiphany that if I put on something really wild and crazy, it made me feel good. It made me more positive, and it radiated energy too. Instead of people wanting to pick on me on the bus, they'd look at me and they'd laugh and they'd say, "Look at this crazy outfit this kid has on." And they couldn't help but smile. And so I've thought a lot about what makes a great ALS doctor. What are some of the things that we can do for these patients until the day comes when there's a cure? And I think one of them is hope. Across every disease, patients who are more hopeful have a better prognosis. And why is that? It could be that there's some mind-body connection. It could also be somebody who's more hopeful is more engaged. They're more likely to do all the evidence-based things that we know help patients with that disease, including ALS.
So I've started to think a lot about what are some hope promoting and some hope suppressing behaviors that I see? And from a hope promoting perspective, to me, if I walk in the room with a crazy outfit on the one I have today, again, it's the faces of the patient and the caregivers just light up. Very often it starts with, "Can I get a selfie for my social media with you with this crazy outfit?" It gives me a more positive, enthusiastic attitude when I talk to the patient. If I feel good about myself, then I walk in with a different kind of energy. And there's a whole bunch of other things that I've learned. But that's what I call setting the table. I think a lot on Monday nights, what am I going to wear tomorrow for clinic? And that's how I set the table for the day.
But yeah, this jacket, I told you this story earlier, this mashup of fashion and neurology has taken me to some wild places. One designer invited me to come to Men's Fashion Week and let me walk the runway during his fashion show. Another made ties that the coaches for Duke and UNC wore a few years ago for the Duke UNC game. These are not the type of ties you would typically see basketball coaches wear. I got to meet with Post Malone's tailor during the pandemic and fulfill one of my bucket list items, which was I always wanted to help design my own suit. And so we had a lot of correspondences, a lot of fabric samples, a lot of drawings, but I wanted that suit to be embroidered with lessons I've learned from people with ALS that I don't ever want to forget.
And then today's suit, I got to meet a tailor in Nashville last year, who's someone I've been reading about since I was a kid. His name is Manuel Cuevas, and he was Johnny Cash's Tailor, Elvis Presley's Tailor, Elton John's Tailor, Lady Gaga's tailor. His resume is unbelievable. And he's still working. He's in his 90s, and he was kind enough to meet with me. And first he asked me, "What band are you in?" I said, "Believe it or not, I'm not in a band. I'm actually a neurologist and I work on this rare disease called ALS." And his face just went white. And he said, "Wow, did you know my wife passed away from that?" I had no idea. And he told me, this was years ago, but he told me it was a tough time. They didn't feel like they got a lot of options or hope.
And I told him what I'm trying to do different at Duke and how it starts with what I put on. And that just prompted him to say, "I want to make something for you to wear for your patients." And so today's jacket is my favorite color. It's bright purple and it's embroidered with symbols of patients and families that he read about online that have helped me in my quest to end ALS. So there's race car drivers, and there's gardeners and there's Tesla engineers, and there's hunters and there's scholars and one family that owns a shipyard all represented on this jacket, and then he embroidered it with some symbols of his family too, to make it our story. So yeah, I always feel 10 feet tall when I wear this one.

Dr. Katy Peters:
Well, I will say that you're on fire right now because I see some flames coming out of that jacket. And I couldn't be more impressed, and I'm so excited that you shared it with us. Brooke Eby, our guest with ALS, she was just amazing. And she described living on ALS time and the way she stated it to us is she feels she has to pack seven years of living into one year. She actually called it living in dog years. Do you have any thoughts on this? I guess some completing thoughts for our listeners today, just about that thought?

Dr. Richard Bedlack:
Yeah, I'll say a couple things. So first, I always hate to hear that any person with this disease was given any kind of expiration date. I still hear patients that come to me and they say, "Yeah, they told me I have ALS and I have two to three years to live." Well, I don't know how you could ever look at a person and know that they have exactly two to three years to live. 10% of people with ALS live 10 years or more, 5% live 20 years or more. So I don't think we ever know for sure, but I have heard this expression from some of my patients. I remember one person that was popular on social media that I thought was an amazing advocate, even before Brooke, she used the term, the ALS clock. It's different than the regular healthy human clock. It moves faster.
And the more I think about this, I just feel like all of us, whether we've been diagnosed with ALS or not, we don't know how long we have. If in your mind you have a list of things that you really want to do while you're on this planet, do them today because we might not be here tomorrow. So even without a definite expiration date, I just think that's such an important lesson for all of us. We get caught up in our day-to-day activities. I can't tell you how many patients I've diagnosed who said, "Gosh, we were saving. We're putting off all these trips until the day that we retired. And now we're 55 and getting close, and we got ALS and we might not make it to be able to do all that stuff." Just make your list of things you really want to do and do it.
Knock on wood, I've been relatively healthy, but I've been clicking things off my bucket list. You've commented on my office, a lot of these pictures behind me are bucket list items that I got to do. And I've only got, I think, five things left on my bucket list. And if I'm lucky enough to check those off, well then I'll make a new bucket list. But I think it's a great mentality to have. Seize the day. Nobody's promised tomorrow. If you have a list of things you really want to do, go get them done.

Dr. Katy Peters:
Agree, agree, agree. And I will say, I just checked off something. I got to interview you, so thank you very much.

Dr. Richard Bedlack:
Well, thank you, Katy.

Dr. Katy Peters:
I want to say thank you to, Dr. Bedlack. Thank you for sharing your expertise, your stories, and your hopefulness for all of us that either have ALS, supporters of patients with ALS, or caregivers and researchers.

Dr. Richard Bedlack:
It's been my pleasure to be here, Katy, and I'm so happy that you've got Brooke to be on the show because she is definitely someone I look up to as a superstar in the ALS community.

Dr. Katy Peters:
I completely agree. And thank you to all of our listeners.

Dr. Daniel Correa:
Thank you again for joining us today on the Brain & Life Podcast. Follow and subscribe to this podcast so you don't miss our weekly episodes. You can also sign up to receive the Brain & Life magazine for free at brainandlife.org. Don't forget about Brain & Life in Espanol.

Dr. Katy Peters:
Also, for each episode, you can find out how to connect with our team and our guests along with great resources in our show notes. We love it when we hear your ideas or questions. You can send these in an email to Blpodcast@brainandlife.org, and leave us a message at 612-928-6206.

Dr. Daniel Correa:
You can also find that information in our show notes, and you can follow Katy and me and the Brain & Life magazine on many of your preferred social media channels. We are your hosts, Dr. Daniel Correa, connecting with you from New York City and online at NeuroDrCorrea.

Dr. Katy Peters:
And Dr. Katy Peters, joining you from Durham North Carolina and online at KatyPetersMDPhD.

Dr. Daniel Correa:
Most importantly, thank you and all of our community members that trust us with their health and everyone living with neurologic conditions.

Dr. Katy Peters:
We hope together we can take steps to better brain health and each thrive with our own abilities every day.

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