I'm Dying to Tell You
I'm Dying to Tell You
Fashion, HOPE, ALS Reversals: All with Dr. Richard Bedlack
Here I chat with Dr. Richard Bedlack, a neurologist known not only for his relentless ALS research but also for his unique style. This episode unfolds the fascinating story of how an encounter with legendary designer Manuel Cuevas led to the creation of a special jacket that embodies Dr. Bedlack's fight against ALS. We also celebrate the powerful alliances formed through advocacy, as highlighted by a heartfelt recognition from Zac Brown Band's John Driskell Hopkins. I loved hearing him talk about how fashion, music and medicine are all playing a part in the battle against ALS.
For 24 years, Dr. Bedlack of the Duke ALS Clinic has been at the forefront of ALS research, driven by a childhood fascination with the brain. His journey has been marked by pivotal moments that shaped his dedication to building a program offering hope and options for ALS patients. Here, Dr. Bedlack reflects on the significance of HOPE, not only as a treatment strategy but as a vital component of navigating life with ALS.
Dr. Bedlack also shares his learnings after studying the 62 known cases of ALS reversals. This episode is a testament to the relentless pursuit of a brighter tomorrow, where there is a world without ALS. Listen in and share with a friend. Hugs, Lorri
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hello hey lori, how's it going?
Lorri Carey:it's going good. How are you?
Dr. Richard Bedlack:You may have seen the story behind this jacket, but this one's extra special.
Lorri Carey:Which jacket is that one?
Dr. Richard Bedlack:This is the one that was made by a designer named Manuel Cuevas. He was the designer for Johnny Cash, elvis.
Dr. Richard Bedlack:Elton John, David Bowie, Salvador Dali, Frank Sinatra, Lady
Lorri Carey:.
Dr. Richard Bedlack:Gaga Turns out he has an ALS connection. Oh my gosh. I got to meet him and talk to him for a while and tell him what I'm trying to do different at Duke and how it starts with what I put on when I go to clinic. And he loved it and he made me this jacket. He did some research on me and he made it in my favorite color and he embroidered it with symbols of all these families that have helped me in my quest to end ALS oh my gosh, that's his own family symbols on there.
Dr. Richard Bedlack:And he said this jacket is our story and you make sure you tell it as often as you can.
Lorri Carey:So oh my gosh, I love it.
Dr. Richard Bedlack:So what came first, the um, your desire to connect with that designer, and then, after you were talking to him, you found out he had a connection absolutely so oh my gosh, I've loved fashion since I was a kid and I just happened to be at a at a meeting a couple of years ago in nashville, tennessee, and I had a free afternoon and I said to my wife I want to see if I can meet Manuel Cuevas. He's been one of my heroes in fashion my whole life. I mean he's 92 years old. He started before I was born and said I'm going to go out to his studio see if he'll meet with me. Sure enough, he was very welcoming and first question he asked me is so tell me again, what band do you play in? And I said, believe it or not, sir, I'm not a musician, I'm actually a neurologist and I take care of people with a rare disease called ALS. And his jaw just dropped. He said did you know? I lost my wife to that disease. I said I had no idea like.
Dr. Richard Bedlack:I've been reading about the pieces of clothing you make and seeing them in movies and at the Grammys and things like that, but no, I had, I had no idea oh my god, I have chills.
Lorri Carey:I thought you were gonna say, like you know, a second cousin or a friend of a friend, or incredible. I mean, I still get the chills.
Dr. Richard Bedlack:it's like it's almost like we were meant to meet and we've we've actually, I'm happy to say we've become friends. I've, you know, contacted him and his wife many times. I got to go out and stay with them at their house in rural Tennessee. A few months ago, my wife they invited my wife and I for a weekend, so we got to go into what I call the adult Disney world, which is his fashion studio. I've never seen so many amazing and crazy pieces as he's working on.
Lorri Carey:Wow, that's crazy. Okay, so the last time we saw each other was in Atlanta at Hop on and Care what?
Dr. Richard Bedlack:a night. Oh, oh, how special was that it's one of the best nights I've I've ever had in my life. I mean it was yeah it was almost like a dream the people that I got to interact with, the things that I got to partake in, you know, and then, on top of all that, to get called up to the stage to to get, you know, recognized for my work and you know I still have it right here next to my desk my prized possessions.
Dr. Richard Bedlack:I got one of my John Driscoll Hopkins Western shirts. There's a couple of pieces of clothing that I I care too much about to wear, like I have these in my office, because I look at them and they remind me of moments. That's one of them.
Lorri Carey:Oh my gosh, I forgot that. He gave you one of the shirts on his back. Basically, he's such a nice guy, sure is and yeah, I had no idea that you were going to be that Hopinacure was going to award you, you know, with funding for your research. So that made it extra special as well. So I can't believe that by the end of the year they will have donated $3 million for ALS research. So I'm so proud of them.
Dr. Richard Bedlack:Yeah, they have they have an incredible um platform yeah, you know music. Is is universal zach brown band, of course is is beloved all over the world yeah, but I mean, to take it to the next level, that the whole family, the Hopkins family, is so charismatic and thoughtful, and I just love listening to what they have to say because I always learn something from them.
Lorri Carey:Yeah, very, very brave family too. So well, let's jump back a little bit. For those that aren't in our ALS community. They might be wondering why did I start off by talking about your clothes, and who is this guy? So why don't you, you know, just do a little introduction of how you're known in the ALS space?
Dr. Richard Bedlack:Yeah, I'm Dr Rick Bedlak. I'm a professor of neurology here at Duke and I started, and still lead, the Duke ALS program. And well, I guess it depends who you ask in terms of how I'm known. But you know, if I can't be the guy who cures ALS, I want to be known as the one who created a lot of options and hope for people living with this disease, and we're going to talk a lot more about that hope word.
Dr. Richard Bedlack:Yeah, as to me, as I look back over my career, which is now 24 years, I feel like that's probably something I've been especially good at is understanding the importance of hope and trying to become a better hope giver, and I think I'm pretty good at it. I'm actually going to be putting out a movie in 2025. There's been a lot of great ALS movies, as you know, but this one's a little different because it talks about what it's like to be an ALS doctor, and it's definitely not an easy job. It's actually getting harder, I think, over time because of the medical system that we have and how it's changing, but I feel like I've been able to stay positive and generate a lot of hope for my colleagues, but also for the patients and families who come to see me here at Duke.
Lorri Carey:Yeah, I would say that as well. And so we've been in the ALS community about the same time you longer than me, but I've, you know, going on 21 years, and then I've had ALS. And I will say that if I'm talking to someone about you, I'm like, you know, dr Benlack, the guy with the really cool suits and the guy that gives a lot of hope. So you're right on, and that's how you're known. I know you've been focusing on ALS for a long time. How or what initially inspired you to focus on ALS?
Dr. Richard Bedlack:Yeah, well, let me back up and you can edit this if it's too long. But I'll back up all the way to my childhood. There were two weird things about me. When were you born? Two weird things about me from an early age. One was the clothes. You've already touched on that.
Dr. Richard Bedlack:But from the day that my parents let me pick out my own outfits for school which I think was at either Sears or JCPenney I liked really loud, crazy combinations because I felt like they were almost a suit of armor to me. They would allow me to protect my positivity no matter what I ran into. And I started to notice how they rubbed off on people around me. Like back then it was hey, you know these bullies who used to steal my lunch money. Now they see this and they're like look at this crazy kid, wow, this guy, what an outfit. And they would leave me alone. So it worked two ways.
Dr. Richard Bedlack:But the other weird thing about me was, before I even fully understood what the brain was, I was captivated by what I would later come to know as neurology. Like one of my first memories is rolling my little brother down this hill in our backyard and having him get up and kind of stagger around and wondering what's this? And I rolled myself down the hill and I'd experienced that spinning sensation. And I'd run inside to my mom and I'd say you know, mom, how come, when we go down the hill like this, we feel like we're spinning. Or I would see somebody out in public who shuffled when they walked or their hands shook, you know, when they wrote. And I'd say, mom, you know why does this happen? And you know, my parents were fantastic, they really loved us, but they didn't have a lot of education or a lot of money. And so my mom would say write down, and on Saturday I'll drop you off at the library and you can try to figure this out. Oh my gosh.
Dr. Richard Bedlack:I had this great local librarian who would pull these books off the shelves and we would sit there and try to understand what I was seeing.
Dr. Richard Bedlack:And it always came back to the brain, like what I was fascinated by was always connected in some way to the brain and it led me to like programs, eventually to a neurology residency, and that's where I first came in contact with a person with ALS and it just captured everything that I was fascinated by. Since I was a kid. I'd never heard of a story like this where a disease could take out one part of the body so specifically, so quickly and for no good reason, and I'd never seen a collection of exam findings like that in one person. And I remember when my attending physician walked into the room and said well, it's called ALS, we don't know why it happens and there's nothing we can do about it. You just have to go home and get your affairs in order. And I drove home that day and I said I think I found the disease that I want to fight and I'm going to stay at this institution and I'm going to change the culture. I'm going to build a program that's going to give people options and give people hope.
Lorri Carey:Oh my gosh. So this is, right off the bat, way more than your job, way more than really your career, more than your job, way more than really your career. I mean it's like your interest, your hobby, your passion, kind of all into one right.
Dr. Richard Bedlack:Well, I'm, in a way, blessed that I get to wake up every day and I get to work on a problem that I'm still absolutely fascinated by, and I get to do it in a way that incorporates who I am as a person. I don't have to change the things that bring me joy. I can incorporate those into my career and they make me even better at what I'm trying to do. So I feel really lucky to be able to do that.
Lorri Carey:Yeah, so you're talking about like your first encounter where you were. You had already decided that that's not good enough. I'm going to do something about that in the future, but is there anything that happened? Anyone that you met or encountered that along the way just kind of sealed the deal, you know, on your commitment to ALS research as a life, as a you know, a life project.
Dr. Richard Bedlack:Well, there's been so many different patients and families that have changed the way that I think about medicine, have changed the way that I think about ALS, both in terms of a doctor and a researcher. There have been so many patients and families that have rescued me when I was on the edge of just quitting and doing something else.
Dr. Richard Bedlack:But if I had to pick one, that really just completely changed the direction of my career. You know, I started off traveling around. I had no ALS mentor here, so I was traveling around the country and talking to the experts, watching them, you know, examine patients, watching their multidisciplinary teams, reading every article and book I could get my hands on. I was learning the science of ALS care what things are we supposed to measure and what are we supposed to offer at different measurements? And then one day probably I don't know four or five years into my career, I walked into an exam room and I was struck immediately by three unusual things. One, here's the youngest person I've ever seen with what would turn out to be ALS. He's about 20 years old, sitting in the middle of the room in a wheelchair. Two, I've never seen this many people jammed into one room. Probably at least 15 to 20 people jammed in there could hardly move. And three, this is the wildest looking bunch. They look like they're on their way to like a punk rock concert. They have mohawks and piercings and tattoos. Everybody's got tattoos. And I'm talking to him, I'm examining him, I'm explaining you know what I can do and, unfortunately, what I can't do.
Dr. Richard Bedlack:And when we came to the end of my explanation I said can I ask y'all one question? I couldn't help but notice there are all these tattoos of swallows. Like I noticed, the patient has a whole sleeve of swallows and everybody else in the room has at least one. And he said yeah, I told you, als runs in my family.
Dr. Richard Bedlack:My mom died of it when I was really young. I didn't get to know her, but she left me this book and on every page of the book there was a swallow. She just drew a swallow. I don't know why, but when I got to be 16 and my dad said I could get a tattoo, I got every swallow from that book tattooed on my arm to honor the memory of my mother. And then, when I started to get sick, we kind of knew what it was. Everybody I knew got one of these swallows from my arm tattooed somewhere on their body. And this is to show our solidarity. We call ourselves the often awesome army and we're always going to come here together and we're going to keep this upbeat and positive. And I was like wow, this is different, and they did.
Dr. Richard Bedlack:You know, every single time he came, as sick as he got, he was surrounded by this often awesome army. We laughed. They told me these incredible stories. They made the series of YouTube videos that's still out there, called often awesome the series. And you know when we, when we're coming to the end of his journey, I said, man, I've learned so much from this that I didn't find in any book or see in anybody else's bedside approach. I don't ever want to forget this. I want to be in your army. I want to get one of these tattoos.
Dr. Richard Bedlack:I did a little research to make sure it didn't have some hidden meaning that would get me fired. It turns out it's a tattoo that sailors used to get because that's the first bird you see when land is approaching. So for them it meant they weren't lost at sea. And I got it right on my wrist here because the last thing I used to do before electronic medical records is sign the chart. So it would pop out of there and it would remind me. Hey, in addition to all the evidence-based stuff, hope is really important. Not everyone has an often awesome army. Sometimes that's got to be me. So if I haven't already said something hopeful. I need to go back in there and do that, so that has always been part of my practice since I met that young man and his family.
Lorri Carey:And are you allowed to say his name?
Dr. Richard Bedlack:I do not believe it or not. Have official duke permission to release his name? Oh yeah if people go online and look up often awesome this series on youtube, you will find his name so okay, often awesome army often awesome army.
Lorri Carey:Yes okay, great, all right, and I can put a link in my show notes to that as well. Yeah, to my listeners, I won't put a link in the show notes that you can find on facebook and the website, both at I'm dying to tell you podcast, and I know I'll have several other links there as well. So, besides your awesome wardrobe and your incredible outlook and the hope that you give, has found 61 cases of ALS reversals.
Dr. Richard Bedlack:Yeah, 62 now. Believe it or not, we just found a new one a couple of weeks ago. We keep finding them. Unfortunately, we hear about a lot of people who are saying they're having an ALS reversal, and most of those we are unable to verify. But from the beginning of time, it is no test we do. There's no picture. We take blood tests. We do, biopsy we do. It's a set of criteria. So they all have the right history, the right exam, the right EMG, the right testing for mimics. They all progressed to where they were disabled from weakness and then they all recovered all or most of their lost motor function.
Dr. Richard Bedlack:This is a phenomenon that was not invented by me. This has actually been around and reported in the medical literature since the 1960s. I only learned about it approximately 15 years ago in the midst of an ALS untangled review, when I came in contact with one of these people and just about fell out of my chair when I got their medical records and saw this was something I had never heard of. But I went back and realized, wow, these have been reported but nobody's really paid any attention to them, and so I'm the first one who's trying to put them all in the same database and figure out what's different about these folks. How did they recover from ALS? How do we make this happen more often about these folks?
Lorri Carey:How did they recover from ALS. How do we make this happen more often? Yeah, before we talk about the magic question, you know the common factors that they've all had. Why do you think that people documented?
Dr. Richard Bedlack:the reversals but did nothing more with it. Well, a couple of things. I mean one I think that the supposition was that these people probably didn't have ALS. You know, like that's the most common thing I hear when I talk about this is somebody will raise their hand and say, hey, bedlock, if these people got better than they never had ALS in the first place? Well, that is a possible explanation. What I can tell you is today we diagnose ALS by checking certain boxes, and they all checked all those boxes.
Dr. Richard Bedlack:Now, someday, if somebody develops a test that comes back 100% yes or no, I would love to see all these people tested to see if they have the same thing that everybody else with more typically progressive ALS has. But until that day comes, no one can win that argument. They have ALS by today's definition. There is nothing in any published definition of ALS that says they got to do A, b, c, D, but oh, by the way, if they get better, they don't have ALS. That doesn't exist. That's not listed in any of the published criteria for making an ALS diagnosis.
Dr. Richard Bedlack:I also think that doctors are incredibly stretched, and they have been for a long time, more so now than ever. But if you go back and you read these case reports, almost every single one of them says, yeah, they recovered. And I just said, well, I don't know what happened, but go have a good life, you know? And it's on to the next patient. It takes an incredible motivation to actually add a whole nother layer of research, a whole nother direction to what you're going to work on. Layer of research, a whole nother direction to what you're going to work on without any funding.
Dr. Richard Bedlack:Like when I first started this, nobody was interested in funding this because most people thought it was never going to lead anywhere and it might not, but I would say it has created a lot of hope. Just the mere acknowledgement that this can happen it changes the way I talk to people when I make the diagnosis. Talking about the research that I do on these folks generates a lot of hope that this is possible.
Lorri Carey:Yeah, and you know, with having ALS for 20 years, I have talked to a lot of people that have ALS and now, of course, with the podcast, and I'm finding even my guests, as I interview them, are talking about hope and they refer to you and they refer to. There are some cases you know that have been documented, that are true cases of ALS reversals, and those are awesome people that I'm bringing on the podcast because they are very inspiring and very optimistic about living a life with ALS and part of your work and part of the reversals you know is a reason why so. So that's interesting. If someone is listening in and they have ALS or their family member has ALS, I guarantee what is it? Is it something they ate? Is it something they do? What is the commonality?
Dr. Richard Bedlack:Yeah, lori, I would say so far, and I haven't given up on trying to understand what's going on here, but so far, the only thing that I can see that all of them seem to have in common is that they're all very positive people. Now, what I don't know is which came first, because maybe you weren't a positive person, but somebody said well, you got this, you got two to three years to live which I hate when people say it, but they still do say it to people that way and then they were proven wrong and the person instead recovered and is now living a normal lifespan. That would probably change your entire outlook on life.
Dr. Richard Bedlack:You'd probably be a much more positive and optimistic person because of what happened. So I can't say which came first, but from a scientific standpoint, one thing that I've discovered fairly recently which I think is fascinating is that there is a genetic abnormality in these reversals. It's not in all of them, but it's much more common in these reversals than in ethnicity-matched controls which come from databases of people with more typically progressive ALS. And this gene does something that changes the brain levels of a protein called IGF-BP7. So this protein by itself has never before been talked about or implicated in ALS in any way. But this actually regulates another pathway which was of great interest in ALS. It's the insulin-like growth factor 1 pathway, specifically this protein that I found IGF-BP7, it blocks the interaction between the growth factor and motor neurons. So with less of this protein you can have a better interaction, your motor neurons can be more resistant to ALS and you get a milder case and you can recover from it. So it's the first time there's ever been really a plausible theory for how people might recover from ALS and it's a testable theory.
Dr. Richard Bedlack:And Now we did a very first preliminary test of it with a group called ALS TDI and what we asked them is could you look at the blood levels of this protein and tell us number one is it different in people with ALS and people without? And the answer is yes, it is. It's higher in people with ALS and in fact, in the literature, that protein is higher in people with lots of different neurological diseases MS, dementia, suggesting that it really is important. Two could you tell us is the protein different in people who recover from ALS versus those who don't? Well, we didn't find a statistically significant difference.
Dr. Richard Bedlack:Now, we only had 11 people with ALS reversals participating in that study. That's a pretty small number to look for a statistically significant result. We also realized that the blood levels of this protein may not reflect what's going on in the brain and spinal cord, and so what I think we need to do next is go after this, and I already have an idea for how to knock this protein down and I think we need to try to knock it down in the spinal fluid. We need to measure it in the spinal fluid before and after we start this treatment, and hopefully what we will see is that people stop or recover from ALS. But if we don't, the next question is did we actually knock down spinal fluid protein levels? Because if we didn't, well, we got to go back to the drawing board and find a different way to do that.
Lorri Carey:So is this one of the studies that you're currently doing, then this one is not.
Dr. Richard Bedlack:This is all pretty new, Okay, and we're still sort of putting a protocol and a budget and funding together and hopefully we'll open that trial in 2025, a trial targeting this IGF-BP7 protein to see if knocking it down in the spinal fluid really does change the course of a person's ALS. But to me it's pretty exciting. I mean, it is 15 years worth of work. Somebody could say, well, man, that's a lot of work to still not know for sure why this is happening. But I would say it's pretty cool that after 15 years we actually do have a plausible theory with some evidence and we have an idea for how we can manipulate a target out of these ALS reversals.
Lorri Carey:Absolutely. And you still have those people that you've identified. And you know, the more people that you identify, the more options that you're going to have to look at. So I am not a scientist at all, but when you talked about insulin growth factor one, I will tell you that I was in a trial year two, so that must have been 2005, 2006,. So that must have been 2005, 2006. With insulin growth factor one, I was giving myself two shots a day for two years and I know I was getting a drug because I would have hypoglycemia reaction. I was not getting a placebo, glycemia reaction, I was not getting a placebo. So, and you know, at that time I mean I was, you know, still pretty strong. My um FEC was really, really strong, so at the time I thought it had done something for me. Does that have anything to do with that?
Dr. Richard Bedlack:Yeah, it's interesting Laurie.
Dr. Richard Bedlack:Yeah. So the reason for the trial that you were in is because people made some observations way back then. One of the observations was that when you look across the spectrum of people with ALS, some of whom are very fast progressing, some of whom are very slow progressing, there's a difference in how much insulin-like growth factor they have floating around. People that are doing really well have more of it. So next they took insulin-like growth factor and they gave it to the mouse model of ALS and the model did much better. So they said well, now it's time for human trials. And they actually did several human trials and they concluded from these trials well, there's not really much of a signal here. This idea is dead.
Dr. Richard Bedlack:Well, maybe not so fast, because somebody actually, after all those trials were done, said do you all know that when you give IGF-1 under the skin, it doesn't get into the brain? Also, when you give it that way, it doesn't last very long, it has a very short half life. So I would say that there really isn't a conclusion from all those trials. Like, you didn't manipulate the target in the right place for the right amount of time. Therefore, you can't say the idea was wrong and it's it's gonna. It's not going to be easy to manipulate IGF-1 in the brain. You know it's it's. It's going to be pretty complicated to figure out how to do that. But what if you didn't have to manipulate the growth factor itself, but just this protein, igf-bp7, that gets in the way of the growth factor? Maybe this is a completely different way to come back to the same idea, but a much more plausible way to treat it.
Lorri Carey:Yeah Well, thank you for sharing that. I know that a lot of people are probably knocking on your door as far as traveling all over the world to come see you and hope that you have that magic answer because you have that relationship with these unique cases. So I'm glad that you are finding some glimmer of hope and some direction in all the work that you're doing. So you talked about already working with patients and how you are giving them hope and not only the patients, but the families when they come in. What is some advice that you give someone that you see on day one?
Dr. Richard Bedlack:Yeah, great question. Yeah, I mean just taking a step back and talking about that importance of hope. Yeah, so in the years since I saw the Often Awesome Army and their effect on that one patient, I've done so much reading about hope and I've really come to believe, laurie, that it isn't just an emotion, it isn't even just a coping strategy. Hope is a treatment. If you look across almost every disease where this has ever been studied and it's not been studied well in ALS, I'm going to change that in 2025. I got a hope boosting intervention I'm going to study.
Dr. Richard Bedlack:But every disease cancer, stroke, spinal cord injury, dementia, heart disease people who are more hopeful, they do better medically, they have a better quality of life, they have fewer symptoms, are less likely to be hospitalized, they live longer compared to people who are less hopeful. And it just blows my mind that we haven't thought about this in the ALS community Like, wow, yes, we should continue to try to develop traditional medicines to slow the progression of ALS, but if there are other ways to potentially slow progression and improve quality and length of life, why would we not consider those? And hope is one of those things that, to me, we really need to get more focused on in this community. So the things that I do. First, lori, it starts with me. How can I be a hope booster if I'm not positive and hopeful?
Lorri Carey:Yeah.
Dr. Richard Bedlack:So, like I was telling you before, I've got to think to myself what makes me positive and hopeful. The clothes are one thing, but there's other stuff I got to remind myself that I have in my life. It isn't just possessions, it's people. I've got the greatest support system a fantastic spouse, a wonderful father, a great brother, so many friends that keep me upbeat and positive, and so you know that sets me off on the right foot. Then, when I get to clinic, I have to be very careful about the words I choose.
Dr. Richard Bedlack:You know, when you break the news to somebody about a disease, what do you say next? Really, they leave with a very different impression and maybe that impression can't be changed. I can't tell you how many people I see that have been told these exact words you have ALS, you have two to three years to live, go and get your affairs in order. That's just soul crushing to hear the disease presented that way. It's also inaccurate. You can't look at a person on day one and know they have exactly two to three years to live and tell them that that's now when they need to go get their affairs in order. I mean what I tell people is look, you have this disease. It's a tough one. It can be disabling, it can be life shortening, but it's very different in different people. There are examples of people who've had this disease for more than 10 years and still have an incredible quality of life. I know a young lady who's had it for more than 10 years and still competes in marathons.
Dr. Richard Bedlack:Stephen Hawking had it for 50 years and was still writing books exploring the mysteries of the universe. There are these 62 people who recovered. We don't know how you're going to do with this. Let's take it one day at a time. Let's make your life the best it can be today. Let's try everything we can think of to slow it down, and in a few months, if things have changed, we'll do it all over again. We'll rethink our approach and
Dr. Richard Bedlack:this is just one tiny part of you. It does not have to define you. There are so many parts of you that ALS does not have to touch. It doesn't have to touch your spirituality, it doesn't have to touch your sense of humor, your kindness, your compassion. Those are the things that that make us who we are, not the strength of our muscles and our arms and legs.
Lorri Carey:It's all that other stuff and ALS doesn't have to touch that.
Lorri Carey:I love that and I talk to a lot of people who are just diagnosed. I get connected in a lot of different ways and I was just thinking about I always offer to do a Zoom call, you know, with them and their family. And I was on Zoom with a young lady with children and she was in her car in the driveway. She didn't want her family to hear. She was on the phone and she was just so engrossed in what was going to happen next. What was going to happen next?
Lorri Carey:What does this mean? What does this twitch mean? What does this weakness mean? She was so worked up, you know, and she was saying and your daughter, and pick out her clothes and enjoy her senior photos and like, put it on your mind, because if you put it on your mind for a moment to enjoy something really great in life, it's not gonna make a difference. Yeah, and you know, oh, I really felt for her, like I just pray for her that she will have that uh, understanding and be able to move through this in a in a different way, you know I think those words were so wise, laurie, and, and what I would say is I would even take it a step further.
Dr. Richard Bedlack:and sometimes people need a specific strategy, right, yeah, yeah, Like it's kind of like exercise. Like you walk into the gym, you know you want to get in better shape, but you're like overwhelmed at what you see. In front of you there's a thousand machines. What do I do? Yeah, you know you might need a personal trainer to say, all right, we're going to do 10 of these and we're going to build up by 10%. Each week you get a plan. So that's getting back to something I hinted at earlier about this hope boosting strategy that I want to test. So I believe there's two things that we all can do better. The first is what if, when a person gets to clinic, instead of saying, well, what are you complaining about today? What's your chief complaint? Well, what if we said tell me two or three things you're hopeful about today? But what if we said tell me two or three things you're hopeful about today? How?
Lorri Carey:does that visit look different?
Dr. Richard Bedlack:now, right, we can personalize the multidisciplinary team approach. And anybody like that person you talked to, who's out in the driveway drowning in this anticipatory grief, thinking about what? If? He said, why don't you tell me two or three things you're hopeful about today? Let's see what we can do about making those things happen Right.
Dr. Richard Bedlack:And then the other is there's this really cool instrument that was created by James Clues Ogans, a professor at University of Virginia. You may have heard of him. Yeah, his surviving family now has created something called the Hummingbird Foundation and they have this intervention that he called the ALS turbocharged living scale, and what he said was boy, it's really tough when you go to a clinic and you get all these measurements and they're all worse than they were the time before. Well, I get that, and unfortunately there's no way around the measurements. We need the measurements to know what evidence-based treatment options to offer. We also need them to get payers to pay. If we don't have any measurements, payers are not going to pay for a power wheelchair or a ventilator or a feeding tube or all these other things that we think matter.
Dr. Richard Bedlack:But what if, alongside those measurements, you included a measurement of something that doesn't have to get worse fact, something that could actually thrive in the presence of als, and so his scale. It looks a lot like the als functional rating scale, but it's it's all functions that are not motor functions. There's like a question about your sense of humor and a question about your compassion and your kindness and your empathy and your advocacy for others. And and it scored the same way as the ALSFRSR score. So while that may be calming down as your motor function deteriorates, this other scale can be rising and you can put it up next to that and say look at this, look at all the parts of you that are thriving in the face of this disease. This does not have to define you.
Lorri Carey:I love that. What has he done with that scale? Or what has his family done?
Dr. Richard Bedlack:So he put it out on his blog. If you go and look at his blog, you can get people a link to it. But what I'm going to be doing with Team Drea, andrea Peet's foundation and the Hummingbird family, we're going to study the impact of these two things that I just mentioned the question about hope and the ALS, tls, turbocharged living scale. What happens to people's hope scores when we incorporate those into the visit? Can we boost hope scores and our hope scores and ALS related to all these other things that pharmaceutical companies are spending hundreds of millions of dollars to try to slow down, like your ALS, frsr slope and how long you live? I believe that this hope-boosting intervention is going to be at least as powerful as the medicines that we have so far in this disease.
Lorri Carey:Yeah, well, I hope that you'll come back and share more about that. I'm really really interested in that. So yeah, so you already mentioned als untangled. For those that don't know what that is, can you talk a little bit about your involvement and the website? You know kind of what's out there for people to access.
Dr. Richard Bedlack:Yeah, yeah. So again, lori. Another thing I learned early in my career that no one had ever taught me is people with diseases like ALS are going to try things Like I don't know why. I didn't realize this. I guess because I had never been impacted personally by one of these diseases at that time, and so I recognized early in my career wow, people are coming in and they got these bags of stuff sitting next to them on the exam table. What is all this stuff? And they're not being looked at by the nurse and the nurses are only entering prescription medications.
Dr. Richard Bedlack:Well, these were all what I call alternative and off-label treatments. They were all vitamins and nutritional supplements and herbs and things like that that they found online. And I started to ask around to my colleagues you know my more senior colleagues, what are y'all doing about this? And really it kind of fell into two groups. One was well, I tell them it's all a bunch of crap and they're wasting their money, and I said how does that go? That doesn't sound very respectful it also doesn't seem to sound very accurate.
Dr. Richard Bedlack:What if there is something in that bag that helps the illness? How do you know, unless you look at all these things? And then the other group was like, well, I tell them, hey, why not try anything? I don't have the answer. Well, that's not helpful either. I mean, that's like too autonomous.
Dr. Richard Bedlack:What if we used our years of training, our scientific years of training, to help people actually sift through these, like to look at them, which ones are actually more plausible, which ones are safer, and to give them some guidance to make more informed decisions about what things they're going to self-experiment with? And so we created ALS, untangled to be that. It's kind of like the old X-Files TV show that I used to love as a kid. I set up a website and I field reports. So somebody in Timbuktu says I saw this website where he says he's reversing everyone's ALS. All right, we'll put it on the website under the future review section and we'll let the community vote how many people are interested in this. And if it gets enough votes, I'll assign it to one of my team members or me and we'll try to contact the person running that clinic. We'll try to get permission to visit that clinic. We'll try to talk to people who've been to that clinic and get their medical records to see did they actually have ALS? Is anyone really getting better that's paying all this money to get this treatment? We'll talk about the cost. We'll talk about the cost. We'll talk about some of the bad things that could happen and then at the end of the day, you know we're not trying to stop anyone from going there. We just want people to have all the information before they decide to spend their time and money pursuing one of these things.
Dr. Richard Bedlack:And you know, even in this I've learned some things. I've learned about the people who sell these products. You know a lot of your listeners may cringe and think these are all like snake oil salesmen. They're not actually. Most of them are not. Most of them are kind people that believe they have something. They just don't have any interest or knowledge of how to test it. And these people actually do some things really well that mainstream doctors should pay attention to. These people are all very optimistic and hopeful. These people are very respectful of patients' ideas, almost to a fault Like. I've watched some of them in action.
Dr. Richard Bedlack:I remember being in one clinic where they were doing chelation and the patient said well, I'm not really sure this is working, you know, but I did read something about Lyme disease. Oh, you want me to try some treatment for Lyme disease? Okay, we'll hook up another IV, we'll give you some Lyme disease treatment. Like, wait a second. It's one thing to be respectful. It's another thing to actually like talk about. Is there any credibility behind this idea? Is there any science behind it? Is there risks from all this stuff that's being poured in?
Dr. Richard Bedlack:But they were also very responsive and I think this is a growing problem in medicine. It's especially troubling in ALS when a person says I reached out to this center and I never even got an answer back. Or I reached out to this doctor or this coordinator and it was weeks before they got back to me. Like this is a scary disease. People need answers like in a short period of time when they have questions, especially if it's about a new treatment or a possible side effect. They can't wait weeks or months. They need an answer within a day or two of when they ask their question. And these people all do that.
Dr. Richard Bedlack:So, we need to adapt those qualities. And then I think I learned some things about the treatments. Like I would have thought most of this stuff is gonna be junk and, unfortunately, a lot of it is junk. Most of this stuff is going to be junk and, unfortunately, a lot of it is junk. We actually created something called red flags based upon all the junk that we discovered, that we debunked, and if there's something out there that's brand new that we haven't written about, take a look at all these red flags that we've published on and see how many red flags apply to this thing that you're looking at. Like, for example, is someone charging you $100,000 to inject you with something that's totally experimental? Well, that's unethical.
Dr. Richard Bedlack:Is someone claiming that they've got the cure for 30 of the world's worst diseases, which have nothing in common? Really, what do you think? The likelihood is? That someone out there has an office in a strip mall next to a Kentucky Fried Chicken and they're curing 30 of the world's worst diseases. This would be like the most famous wealthy person in the history of the world Does of the world's worst diseases. This would be like the most famous wealthy person in the history of the world.
Dr. Richard Bedlack:Does this person have any credibility? Do they have any relevant training? Do they ever plan to present the results? Are they even collecting data to find out if what they're doing makes any difference? If the answer to these things is no, no, no, then that's a lot of red flags that might not be worth pursuing. But we actually we did find some things, laurie, that to me warrant further study, and you may have seen, I posted on Instagram yesterday a paper that I just published with some colleagues, about the eight most promising products that ALS Untangled has reviewed in all of its 15 years of existence, and next year that list might be different. But for people who want to self-experiment, that's a place to start. They can take that paper to their doctor.
Lorri Carey:They can say you think any of these things might be something that you and I could do together yeah, perfect, and to my listeners, I will put a link, as I mentioned before, uh, to als untangled, but it's alsuntangledcom, and if you go to the home page it's very intuitive. It explains the method, and reviews are already done, and then future reviews that you can also submit things that you want the team to look into as well. So, so I'll put that too. And you mentioned your Instagram. I'll put that in the show notes as well. Okay, good, all right. So what do you think it's going to take to cure ALS or to come up with cures for ALS?
Dr. Richard Bedlack:It's going to take a better understanding of disease subtypes. You know, I remember early in my life seeing on TV yeah, we're going to find a cure for cancer. Well then it became obvious cancer is not one disease, we're not going to find one cure for all cancer. But we have cures now, and part of that is because we can subtype cancer so well. You know, we can biopsy it, we can look at the characteristics of the cells, we can look at the characteristics of the person's genetics, we can do imaging and find out you know where exactly is the cancer in the body? Has it spread? So that allows an individualized treatment approach which has been wildly successful in some forms of cancer.
Dr. Richard Bedlack:That used to be fatal and that's where we need to get in ALS. And I would say we already have pretty strong proof that I'm right about this. Look at what happened with the drug Tofersen. The Tofersen is only for one small subset of people with ALS, the kind that's caused by mutations in the SOD1 gene. It's the kind of ALS we understand the best because we've modeled that form of the disease for decades.
Dr. Richard Bedlack:And so it's not surprising, based upon our understanding of it, that we took a drug where we thought we knew exactly what it had to do. We put it into a clinical trial and when we, when we follow people now who are on this drug for a long period of time, the results are extraordinary. It's a home run. More than half of the people who've been on this drug for two years are stable and about 20 percent are stronger compared to where they were two years ago. So it just proves the point. When we understand a subtype, then we can make a dramatic effect. But I don't think there will be one treatment that cures all ALS. I think there will be cures for subsets of ALS.
Lorri Carey:Is there anything in particular that is happening right now that you're really excited about?
Dr. Richard Bedlack:Yeah, I mean I think there's a lot more trials underway for people with specific genetic mutations. You know, there's a product out there in trials for people with FUS mutations. There is a product that's under study for people with C9 or 72 mutations. But even for people with no genetic mutations there are efforts to subtype it and go after the specific cause of that subtype. So there's a group at the NIH and a group in Australia who believe there could be a retrovirus called human endogenous retrovirus K that causes some cases, and they're going after that. There's a man named Paul Cox who believes there could be a toxin that comes from blue-green algae called beta-methylaminoalanine, and he's going after that.
Dr. Richard Bedlack:Here at Duke we're partnering with a company in Australia called Genius. We're using a technology they've invented which is still experimental, but it's called a pathway burden analysis. We believe we can look at the entire weight of someone's genome and get an idea of which chemical pathway might be most important in killing that person's motor neurons and we can match them then to a treatment based upon this classification. And so we've got a trial underway called the ROAR-DIGAP trial that uses this technology. But we're just at the beginning of all this for people who have non-genetic ALS.
Dr. Richard Bedlack:I think in the next five years we're going to have much more subtypes and the reason I think that is because we're employing artificial intelligence now to these large data sets. So I would encourage listeners who may be living with ALS get involved in natural history studies. I know they're not as sexy as the latest new clinical trial, but they set the foundation, like AI is going to look at all the information that's in these natural history data sets and it's going to say how come somebody does as well as Laurie or Andrea Peet or these 62 ALS reversals? What's different about these folks that allow them to do this? Well, it won't be one thing. It might be 16, 20, 50 different things that combine, but AI can figure that out and once we understand how that connection works, we've identified a subtype and we might be able to learn from that subtype, how to make everyone do as well as you've done.
Lorri Carey:Yeah, and I was going to ask you you know, team slow progressor over here. Do you have any thoughts on why do some people live, you know, 15, 20, 25 years?
Dr. Richard Bedlack:Yeah.
Lorri Carey:Where others do not.
Dr. Richard Bedlack:I think it's just as important a question as the question about the ALS reversals.
Lorri Carey:Yeah.
Dr. Richard Bedlack:And there's a lot more people like you who are doing very well with this super slow progressors than there are reversals and I think it's going to be multifactorial. I think some of it's probably in the genetics. I don't think there's going to be one gene, but it's probably a combination of genes.
Dr. Richard Bedlack:I think, some of it's in the attitude, I think some of it's in the attitude, I think some of it's in the gut microbiome. We've actually got some research in the last few years here at for manipulating the gut microbiome. Now we don't expect that these manipulations would help someone who's already doing well, like you, but we do think that these manipulations could help an average to fast progressor to do much better.
Lorri Carey:And is there any like for me? I know that I don't technically qualify for studies anymore because, like you said, I'm already a slow progressor. So I would throw off the curve and I would throw off the results if I was in a study. But is there a study of those people that are slow progressors?
Dr. Richard Bedlack:Yep. So I would say there is not one study. There are studies available and again, these would be the types of things we talked about a minute ago natural history studies. So the National ALS Registry, the ALS-TDI ALS Research Collaborative, and coming soon is something called All ALS, which is funded from the National Institute of Health, where we're trying to get everybody with ALS in our clinics into the same study, and I think that these are really huge in terms of setting the foundation for the next few years, because we have the power now to identify these complex differences in phenotype and to possibly explain why these are happening, which I think will rapidly lead to better treatments. But that's only if people join these studies, like, ai is useless if there's no data for it to analyze. You got to have data. You got to have big data for AI.
Lorri Carey:Yeah, absolutely. So perhaps we can get together with those links to those natural history studies and I'll put them in my show notes as well. So we're gonna have like a thousand links now. Oh my gosh, I can talk to you forever and we can get a lot more information and resources. So I hope that you'll come back next year and we'll check in and follow up on it.
Dr. Richard Bedlack:I would love to.
Lorri Carey:Yeah, I want to know before we kind of wrap up. I want to know you talked about, you know, physicians being stretched. How do you do all that? You do? How do you see your patients? Or your running ALS untangled? How do you see your patients? Are you running ALS and Tangon? How do you?
Dr. Richard Bedlack:do it all Well, because it's a passion for me. It's not. I don't consider this my job. I consider this my life. This is my passion and, like I said, I'm lucky because I find it so fascinating and because I've been able to involve so many parts of myself that I love in this.
Dr. Richard Bedlack:So it doesn't feel to me like there's a demarcation between my work and the rest of my life. Some people will listen to this and say that's really unhealthy. Maybe it is, but that's, that's, for now, how I want to live my life. I I'm not going to say there aren't any down days. I mentioned earlier. There have been some days when I've been so frustrated, usually by the lack of urgency in our in our medical and academic systems, waiting for me to get the next big hope boosting thing off the ground and to have it take two years for me to get that off the ground. It's incredibly frustrating, but there always seems to be somebody out there that saves me. And I would just say to your listeners I totally understand that I need to be a hope giver for you, but sometimes you need to be a hope giver for me and you would be surprised how often we need that you know you want one, one story I'll never forget, which is another thing hanging on my wall over here.
Dr. Richard Bedlack:I'll just grab it. I had a really dark day a few months ago and I was about to leave here and go home and tell my wife I think I'm done. As I was walking out the door I saw this big glitter covered package sticking out of my mailbox. I said, ok, what's this? I'm back to my office. I opened it up and it said read the letter first. So I open up this letter says Dear Dr Bedlike, you don't know us, we've never met you, but we live in California.
Dr. Richard Bedlack:My husband had ALS and I just want to tell you how much your YouTube videos, your work, your passion meant to us, like they gave us so much hope and I think that allowed us to do better with the disease than we would have done. And I think that allowed us to do better with the disease than we would have done. And he said by the way, my husband was a professional magician and when he passed he left in his will one of his magician jackets that he wanted me to have. So I got this magician jacket and I haven't quite figured out all the secret compartments and things in it yet. But I left the office that day with tears in my eyes just realizing like I got to keep going. There's somebody out there that I've never even met that I've influenced in this way. You know, that's an example of how somebody gave me the hope that I needed that day to keep going.
Lorri Carey:Yeah, absolutely. Oh my gosh, that's amazing. Oh my gosh, that's amazing. Oh, I love that. Well, I just want to say that you are appreciated, I appreciate you, and I know like it's gotta be really, really challenging to keep going. I mean, I even know, like with the podcast, you know it heartbreaking. You know, like my episode I released today with Brady and Brooklyn, 11 and 8, I mean I, you know, broke down a couple times in that chat like they lost their dad and Brady talking about I didn't have enough time with him and like, oh, it's heartbreaking. And then, you know, as a physician, it's even more heartbreaking and frustrating. So I understand.
Dr. Richard Bedlack:Yeah, there is that aspect of it and it's part of it's part of the reason I mean, I have not really been able to go to funerals. In my career I build up a close relationship with a lot of my patients and families and sometimes I get invited and I have to say no, because every time I lose a patient I feel on some level like I failed.
Lorri Carey:And to go there.
Dr. Richard Bedlack:you know, I mean it's. It's probably not the right mindset. Yeah, for me that's just the boundary where I feel like I would leave there with such an overwhelming sense of failure that I could not save that person and I've got to try to focus on what I can do for people and not what I can't do.
Lorri Carey:Yeah, yeah, I imagine All right. Well, I know you listen in, so you know. I have one last question.
Dr. Richard Bedlack:Yeah.
Lorri Carey:So, Dr Penlack, what are you dying to tell us?
Dr. Richard Bedlack:Laurie, I'm dying to tell you that people can beat ALS, and I think they beat it in two ways. We talked about how people can beat it physically. That's extremely rare right now. There's only 62 people that I know of in the whole history of the world who beat ALS physically, and I hope to change that number someday.
Dr. Richard Bedlack:But I want your listeners to know that it's not at all uncommon for people to beat the disease spiritually by not letting it change the best parts of them to beat the disease spiritually by not letting it change the best parts of them, and so many of your guests are that way. Andrea, who keeps running marathons in spite of the fact that it's harder. Yeah, foundry School, hopkins, hop on a Cure. Yeah, who keeps making this beautiful music, even though it's harder? You for putting this podcast out and highlighting all these incredible people. And so I just want I want people to know that there's different ways to beat this disease, but it is possible.
Lorri Carey:Perfect. Oh, thank you. All right. Well, we'll catch up again, I'm sure. So thank you for coming on.
Dr. Richard Bedlack:You got it.