Focusing on Hope: Answering your ALS Questions with Dr. Rick Bedlack

Episode Transcript

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

Dr. Peters:
And I am Dr. Katie Peters, and this is the Brain & Life Podcast.

Dr. Correa:
Welcome back. So Katie, one of the things I like about the relationships we build with the individuals and families we support are all the updates and celebrations we get to have vicariously or experience and share in their joy. That's one of the highlights of my day, if I can have any of that. Of course, at the same time we also see and support people through the challenges with their conditions, when there's progression or worsening outcomes. How do you make sure that as a physician, neurologist, or even a friend and community member to be present for both those hard parts and celebrations?

Dr. Peters:
I think it's all about when those hard parts are happening and when the celebratory things are happening, that you have to show up and you have to be there and recognize that moment. And I had a recent experience when I was at the COGNO meeting in Australia. It is the cooperative trial group in neuro-oncology. I was invited to attend. And I gave some talks, and one of the speakers they had at one of the dinners was a husband of a patient who had passed from brain cancer. He gave this lovely speech. And in his speech he actually recalled part of my talk that I gave the day prior, which I was just so touched. And I went over and really thanked him for saying his kind words. And I just sat with him and I said, "Tell me about your wife. I'd love to know more about her." And we just sat and chatted for a while, and even though I'd never met him before, he was from a different country, even though I'd never cared for his wife, his experience was so reminiscent of what happens with my patients and their loved ones.
So, as a really a person and a physician, we need to take time to listen when you have those moments and bond over really a shared experience or feeling. So I just want to say a shout-out to Bill, and thank you very much.

Dr. Correa:
Yeah, I mean, I know that this is a clear part of your day and your practice. And we all have some aspect of it, whether it's an inpatient stroke in the neuro-ICUs. Many of the families and our listeners have experienced this in their own ways. But at the same time we also often end up being kind of our family's medical consultants. I'm always explaining and talking through med students already getting it at that stage and learning to balance all the different questions that will come up. How do you navigate this side of things? Being there and celebrating not just the personal achievements, but also the progress that people might have with the medical condition, but then also when they have bad news and difficult situations?

Dr. Peters:
You have to celebrate both parts. You can't, or not really, and I guess you can't really celebrate bad news, but you have to be there in the same way, I think. You have to be present and be able to be open to both the good experiences and the bad experiences. Because sometimes patients will have some really, you have to be open to them so that they can want to share that information with you. I had a patient in clinic yesterday where we had some really difficult conversations about their physical function, but in the other room somebody was 32 weeks pregnant. So, there are all types of things happening and we have to be open for them.

Dr. Correa:
Yeah, I mean, I think in these ways just I think of in the hospital or with patients and their families, yes, we lead with our understanding about the medicine and the science, but really still making sure that we're investing and asking questions about what matters to them, and who they are? And how to meet those goals together. And then with our families, siblings and otherwise, trying to where we can, yes, they're asking us a medical question, but where we can lead with that role and that connection that we have with them as brothers, son, family member. And be there with them, not just as the physician in the family, asking them about whether or not they're taking their medications. I feel like sometimes that we, and I can let myself kind of gain some sort of emotional distance from those things going on if I just focus on those. So, it's a challenge I think we all navigate, including the caregivers and care supporters that listen and support our community.

Dr. Peters:
Sometimes I'll often joke that I wish I had a wife like myself for ... Because I think I do a pretty good job with my husband. He's had a cold lately and I've had to do a little doctoring, but also because I'm taking care of my patients with brain cancer that I come home and he has an upper respiratory tract infection. Maybe it's not on the same skill, but I do have to show him some compassion. He's not a very good patient. And he doesn't have much patience with not feeling great either. And so I feel like I have to be more patient with him, because I'm actually in the hospital working now, and so I'm like, I come home and I can tend to him, but he's doing much, much better. He's feeling great now. I nursed him back to health.

Dr. Correa:
Well, today we'll be joined in my discussion with one of your colleagues there at Duke.

Dr. Peters:
Oh yeah.

Dr. Correa:
We're welcoming back Dr. Rick Bedlack. I think he's a great example of someone who really speaks to both of these sides, being there and present with someone as a person and what really matters to them, but also for his patients. And the members that are part of the ALS community there that he supports, being there on the medical side and helping them navigate those. We'll be discussing an update on one of our past guests that you met and interviewed Brooke Eby. She has been sharing more about her ALS and how it's progressed on social media. And gave us permission to share more together with the community along with an article that's coming out in the Brain & Life magazine. So take a look, as this is a compliment. And we'll be also going through some of the community questions we've received about ALS. I really hope everyone finds this discussion helpful. And because we need your help to increase awareness about ALS and neurologic health and brain health. So, please share this and any other past episodes with anyone you think might find it helpful.
Welcome back to the Brain & Life Podcast. Now, we've done question and answer episodes together, just Katie and I, but today I'm glad and happy to welcome back Dr. Richard Bedlack. He's a neurologist and nerve and muscle specialist, with a special interest in understanding the needs of individuals and families living with ALS. And working at Duke University School of Medicine, he's the director of their ALS clinic and the Stewart, Hughes, and Wendt Distinguished professor of ALS. Many of you may have heard him on previous episodes with Brooke Eby and discussing what we know about ALS reversals. And a friend to both Brooke Eby and the ALS community, today he's joining us to discuss some of the updates on Brooke's progress that she has shared on social media and answer some of our community's questions. Richard, thank you so much for joining me today.

Dr. Bedlack:
Daniel, it's always great to be with you.

Dr. Correa:
I would really encourage our listeners, if you haven't heard of Brooke Eby, you don't know her from social media, please go out and check our past episode with her. And take a look at the Brain & Life Magazine article that has recently come out sharing and featuring Brooke and where she shares some of the progress. And in a moment we'll have a clip that will give some background of her story from our previous episode, but if you want more, please check that out.

Brooke Eby:
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 progressor, 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 of 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."

Dr. Correa:
Now, Brooke shared with us in the past some of her early diagnosis. And more recently now she has shared on Instagram more about how her condition has progressed.

Brooke Eby:
Had ALS clinic on Friday, and it was kind of a bust. I saw PT, OT, nutrition, SLP. They basically just make sure you have what you need. But then I saw the neurologist, and it was strange. The only thing she really had me do was she said, "Let me see your tongue." And so I stuck up my tongue and she was like, "Your tongue's great." The first time I was like, "Okay, now when do I take the pulmonary function test so I could get my breathing numbers?" And she was like, "Oh, you don't have to take that test anymore, because last time you were at 27, we pretty much stopped testing after 15." And I was like, "What?" She was like, "Yeah, there's no reason to put you through that." For a disease where we don't have any benchmarks on how people progress, shouldn't we be collecting every single data point? And she just went into this spiel of like how they don't have enough funding, the whole thing. I'm like, "This is just kind of disheartening."
Then I asked, "If I were to go down there and get my breathing number and show it to you, you don't think you could give me a clear prognosis?" And she goes, "Your prognosis is not good. It's not good. You're very terminal." And that I think was the only time I laughed that entire day.

Dr. Correa:
Help us and those of our listeners, those of the social media community that have been following Brooke, help talk to us about where Brooke is now.

Dr. Bedlack:
Well, Daniel, it's important to understand, I'm actually not Brooke's physician, but I am like millions of others a devoted follower of her on social media. And what you can see in the pictures as far as Brooke's physical condition is that her voice is weaker now, and oftentimes you see her using a non-invasive ventilator. I think she also mentioned in one of her recent posts that she got a feeding tube. So, these are all markers of ALS progression in most patients. But I also, I can't help but say, one of the things that I've learned that I see in Brooke is that even though ALS may weaken a person on the outside, it may take away their motor neurons and require them to use things like we just talked about, feeding tubes and ventilators, it doesn't have to touch the best parts of someone.
And honestly, that's part of why I'm on social media, is I absolutely love to see people like Brooke, who I think are thriving in the face of this disease. I mean, Brooke's sense of humor is as great as it's ever been. I mean, her creativity, she just finished a collaboration with a clothes maker to design clothing that is both adaptive but also stylish for young people. And I mean, her charisma is as great as it's ever been. So, I think this is such an important thing for your listeners. And I have to say, I hate the words that she reports her neurologist used. I mean, I think it's really important to have a balance. It's not easy, but it's critical. If you're going to work with people with a disease like ALS, you do have to make measurements and you have to have difficult conversations, but you also have to help your patients recognize that there are parts of them that don't have to be touched.
I mean, we've got this really cool thing that we started doing at Duke last year. We learned it from a person who lived with ALS from the University of Virginia, Dr. James Plews-Ogans, it's called the Turbo-Charged Living Tool. He came home from clinic one day and he posted this thing on social media. He said, "I love going to clinic. I get a lot of good ideas, but I have to say, all they measure are parts of me that they expect to be worse. And I leave and I kind of feel like a failure, all these measurements got worse, in spite of how hard I'm fighting." He said, "What if clinics incorporated a different measurement scale? One that asked questions about my sense of humor and my kindness and my compassion and my advocacy for others."
So, it only takes 10 minutes, but it's amazing how you see people's faces light up and you see families come together and say, "Oh my God, remember that funny thing that he said the other day? Or remember that beautiful post that he did on social media?" And it just shows people that they're more than their motor neurons.

Dr. Correa:
And I think that's exactly what I see both here, for those of us who listen to that Instagram post here, but also see in that post from Brooke is, sure, yes, some of the physical measures and some of the physical features of her condition have progressed. And the motor and the nerve connections will continue to progress in this condition. Her PFT, as she said, has gone from 27 to 12, but those are just measurements of that muscle function and the function of her lungs. But I mean, to see her resilience and the light that comes out, and I wish that we had better ways of measuring that and giving that feedback back to both individuals and their families and communities. I really appreciate really that highlight that you bring to it.

Dr. Bedlack:
Well, thanks Daniel. I hope your listeners will check out the Turbo-Charged Living Tool and consider bringing that into their clinics. Again, it doesn't take much time and it's amazing how it balances out the other measurements that we have to make. But I just want to say, I mean, you mentioned earlier that I've come on your podcast before and I've talked about people who are beating ALS, rarely people beating it physically, the so-called ALS reversals. But I really, I'm blessed to have a front row seat to many people, many more people who are beating the disease spiritually. And Brooke is one of those. I mean, she's not letting it change who she is.

Dr. Correa:
And to that, she also shared another post about feedback that she's reached and heard about her progression.

Brooke Eby:
I've seen a lot of comments from those of you who's traumatized being like, "Oh, this happened so fast. This is so unfair." And it's crazy. This disease has been attacking me for seven years. The average is two to five years. So, technically I'm one of the lucky one. Think of all the people whose disease progressed faster than mine. Once I got diagnosed, when I said I wanted to live in dark years, I meant it. I went so hard. I was traveling every weekend, speaking, meeting people, doing events. I was just nonstop. And so I'm sure that didn't slow things down for me, but I'd do it again.

Dr. Correa:
What do you say to some of the people that she's heard from that feel even traumatized by what they see from what Brooke has shared about, and focus in on what they see as sad and tragic, even though as she said, she has been above average in the years that she's got it?

Dr. Bedlack:
Yeah, I mean, I think there's a difference, Daniel, between sensation, what happens when a stimulus, Brooke's image and the words that she says hit our nervous system and then what we perceive. The perception is colored by who we are. And I think we all need to make a more conscious effort to see the good in the world. It's everywhere. And I mean, when I see Brooke's posts, that's what I see. I see all the amazing good that is still in her life. I mean, she has this unique ability to enjoy living in the moment, and I think we all could learn from that. You mentioned in one of her posts that her doctor told her she was terminal. Well, we're all terminal, from the minute we're born, we have an expiration date, we're all terminal. Is not how long we live, it's how we live. And Brooke is putting herself out there. And really, if people would open themselves up to it, they could learn a lesson that would help them in their own lives.

Dr. Correa:
Now, we mentioned we were going to receive some questions from the listening and reading community for Brain & Life. And while some of these might be topics that we've touched on in our past episodes about ALS, we want to answer those questions from the community here. And some ways along the way we will include links to past episodes if you want more information. And so Richard, I thought hopefully you could help me. We heard from Jen in California, she asked and said, "When my partner started showing symptoms, it took months and several specialists before we got an ALS diagnosis. Why is it so hard to diagnose? And what should families expect during that process? It feels like it could and should have been an easier approach on our end."

Dr. Bedlack:
Well, thank you, Daniel. That's a great question, and it's definitely one that frustrates all of us who are working in this field. We refer to it as diagnostic delay and really across the whole world from as long as this has ever been studied, it's about 12 to 14 months between symptom onset and when somebody gets a diagnosis where somebody sits down and says, "This is definitely what it is." And part of the reason is ALS itself. I mean, unlike a stroke where you wake up and half your body's paralyzed, I mean, ALS starts so gradually that people aren't even sure there's anything wrong for a few months. They might notice that their speech is only affected when they have a couple glasses of wine, and they say, "Well, maybe I'm just getting older and I'm more sensitive now." Or they might notice that their right leg just doesn't have quite the same spring that it used to have. And so, their marathon times are getting longer. And they might say, "Well, maybe I just need to take a break or get a different pair of shoes."
So, it may be a few months before they see any doctor. And usually the first doctor they see is a primary care doctor, and I'm married to one, so I have a pretty good idea of how they think. They think about common things, and they should. And so, when a person comes in with a mild neurologic deficit, they're not thinking about a disease like ALS, they're thinking about, "Is this maybe a pinched nerve? Can we do some anti-inflammatories and some physical therapy?" Typically, that goes on for a month or two. Person comes back, they're a little worse. The primary care doc might get some pictures, like some MRI scans typically of the spine. All of us have abnormalities, and that might prompt a referral to a surgeon. If it's a really good surgeon, they might recognize right away, "This is not making sense. These abnormalities are not anywhere near where your deficits are, and there's no pain, there's no sensory loss."
If it's a bad surgeon, they probably just operate. 15% of everybody that I've seen, 5,000 people now that I've seen with ALS have had an unnecessary surgery that delay things a few more months. Finally, folks get to a neurologist, typically a general neurologist, very good at localizing lesions. And so very often the people that I see, their neurologist told them, "Hey, you probably have a problem with your motor neurons, but I want you to see this guy who's an expert in these kinds of problems." And you could ask, "Well, how come they don't just sit and have the conversation right then and there? This would be maybe eight months in that this is ALS." I think it's twofold. One, they might not see much of it, and since there's no test that comes back positive or negative, we might not be confident to break that news.
But two, it takes time. And I think more than ever, neurologists are in businesses where they have to see lots of patients. And I'm lucky. I mean, I can sit for an hour and a half and have this conversation that this person has ALS and answer all the questions that come up, but I don't think most general neurologists can spend that amount of time. And so they get referred to me and there's not enough people like me that may take a month or two, you can get in to see me. So that's why it takes so long, and it's not a simple problem to fix. There's companies out there who are trying to develop diagnostic tests that will come back positive or negative for ALS, but then you have to ask yourself, when would that be used in this whole story that told you. It may actually not speed up the diagnosis that much.

Dr. Correa:
But yeah, I have. And I think many people would have probably one of the many million dollar, maybe even billion-dollar questions out there in the ALS community. You said companies are trying to develop these tests, but I mean, why don't we have one already?

Dr. Bedlack:
Yeah, I mean, I think there's probably several different reasons for it. One being that ALS is not exactly the same disease in everyone. I mean, we can divide the causes of ALS into two categories, genetic and sporadic. Sporadic meaning we can't find an ALS causing gene. And even amongst the genetic forms of ALS there's at least 40 subtypes caused by mutations in different genes. That means there could be hundreds of subtypes of the non-genetic or sporadic ALS. And so, when you're trying to come up with a test, you have to figure out, what do all these forms of ALS have in common?
There may be one thing that most of them have in common, which is it involves the misplacement and the clumping of a protein called TDP-43. And so, I think that is probably the most promising area where people are trying to look for a test that could diagnose ALS sooner. But again, you'd have to ask, who's going to order that? What if tomorrow somebody developed an ALS test that was as simple as a nasal swab like we do for COVID? I mean, the person who's affected by ALS is not going to go to the drug store and buy that test the first few months, because they're not even sure anything's wrong. The primary care doctor's not going to order that test, because they're not even thinking about ALS, they're thinking about a pinched nerve.
The surgeon isn't going to order it, the general neurologist that we talked about in that algorithm, they might order it. ut then the question is, if they order it, they're going to have to have that conversation, which could take over an hour to explain it, to explain what we can do, what we can't do, where there's more resources to answer all the questions. I just don't know if a diagnostic test is going to change that diagnostic delay very much.

Dr. Correa:
Yeah, unfortunately, I think that frustrating answer that is often the reality of where things are, that we need more resources and research focused on the best way of reducing that delay.

Dr. Bedlack:
I will say, Daniel, there's some groups out there that are trying to educate primary care docs about unusual presentations that might prompt you to skip all that stuff and go right from seeing the patient to referring them to a neuromuscular specialist. And everyone kind of has their own, some people call them red flags, I call it double trouble. I think if you're a primary care doc and you see one of these two presentations, you might skip the conservative therapy and the referral to a surgeon and the referral to a general neurologist and just go right to a neuromuscular specialist.
The first is somebody who has painless progressive limb weakness. Think about that for a second. Pinched nerves, there's usually pain. And somebody that has other causes of limb weakness, like a stroke or maybe an MS flare, that's not usually going to be progressive. It usually happens. And then if it's going to improve, it improves over time. So painless progressive limb weakness, right to a neuromuscular expert. And the other is bulbar weakness, that's progressive and is accompanied by an MRI scan that shows no cause. So somebody who's got progressive problems with speaking or swallowing, you do an MRI, well, there might be a mass lesion there that explains it, that goes in a different direction. But progressive speech or swallowing problems with the MR MRI looks pretty good. Skip all that other stuff, send them right to a neuromuscular expert, even if it's not ALS, it may be another neuromuscular disease that we can intervene on something like CIDP or myasthenia gravis. It's going to be something weird where they probably need to see a neuromuscular expert.

Dr. Correa:
And I think one of the things I heard from both of the things you were saying is, this thing we talk about ALS as the condition. And really just because we're talking about the type of dysfunction, almost symptomatic dysfunction of this nerve and muscle connection. But it's probably not a single cause. This group, this acronym or this name probably names lots of different things that lead to this damage. And some of them might be related, and that's probably why it's both so hard to diagnose and also create a test to diagnose. And requires some specialized knowledge about really making sure when it should be ruled in and considered.

Dr. Bedlack:
Yeah, Daniel, and what you just said there is so important. I think it may be the most important thing that we've learned in my 25 years of working in ALS, is that this isn't the same disease. It's a syndrome. It checks a bunch of boxes as far as progressive deterioration of upper and lower motor neurons with no other explanation as ALS, but that is not the same disease. It's not the same disease any more than cancer, which is rapidly dividing cells is the same disease. And we need to learn to subtype it. Not only to diagnose it faster, but to treat it better. And so an example there is the drug, which is called Tofersen, right? So we finally recognized this one subtype of ALS caused by mutations in the SOD1 gene. We understood exactly how those mutations caused motor neuron death by causing a protein that is misfolded, and gains a new toxic function. And so the strategy became clear, we need to stop this broken gene from making this destructive protein, and we did it with an antisense oligonucleotide. And it's the first home run therapy in the history of ALS.
So more than half of the people with that rare form of ALS who go on Tofersen, their progression either stops, or they start to recover lost function. That's the kind of therapy that we're looking for. And the only way we're going to get more therapies like that is to keep going after subsets. And to go after them we got to define them. We got these 40 genetic subsets. We need to find the possibly 100s of subsets of sporadic ALS to know what to target so we can have home run therapies.

Dr. Correa:
And some of these differences and all the different possible causes is probably a big reason why we see such variation now as we've been starting to measure and pay attention to the community living with ALS about progression. Maybe why we're see as you have described some cases with reversals.
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Now, I wanted to go next to Robert's question. So Robert wrote for us, "I remember the moment we were told it was ALS, it seriously felt like the ground disappeared beneath us. How do people emotionally process such a life-changing diagnosis? And are there ways to stay grounded in those first few weeks? I'd at least like to have advice to give to others in my support groups." I love that. His thought right from the beginning is thinking of others.

Dr. Bedlack:
It's beautiful. I mean, so yeah, there's been a lot of work on breaking the news. And I think that's half of this equation. I mean, half of it is, people are different. Not only is the person delivering the news different, but the person receiving the news is different. And so we have to try to understand who's sitting in that chair across from us as providers.
And we have to kind of tailor the presentation to who that person is. But I do think that there are some themes that are important. I think first people have to kind of understand how we got to this point. We didn't just pull this out of the air. All the tests that have been done and how we now understand that you have a problem with your motor neurons and it's widespread and it seems to be getting worse and we've excluded other causes. We need people to understand that this is a difficult disease. It's usually disabling. It can be life-shortening, but I think there's also a really important thing that is not done nearly enough. We have to let people know that there's a lot of variability, that there's treatments and that this does not have to define who you are. You are more than your motor neurons. I think those parts still far too often are not stressed to patients and families.
I absolutely hate to hear somebody coming to me and they've been to three other clinics and they say, "Yeah, we were told we had two to three years, get our lives in order, go home and get our lives in order." Why are we still telling people that? I mean, we don't know that people have two to three years, they could have 50 years. And, "Go home and get your lives in order." I mean, what does that even mean? I mean, how about, "Here's some things we can do to slow it down. Here's some things we can do to compensate for your deficits. What are you hopeful about? What brings you joy in your life? How can we work together with my multidisciplinary team to keep you involved in those kinds of things?" I think that's all really important. And if that person is not getting those things from their doctor, well then they may need to find another doctor, because there are many of us now that do these types of things and can help you to stay grounded and to enjoy your life.

Dr. Correa:
On the other hand, I do think there is these experiences in our lives and in our families' lives that I do think should and hopefully provide us all an opportunity to consider, if what might happen or the surprises that may occur in our lives and our families, and that we all need to get our lives in order. I think I don't want a single person or individual to feel like it's their burden and because of a specific diagnosis, but I feel like the experiences that we have as physicians and sometimes as family members of people and going through a very challenging diagnosis sometimes allow us a perspective to reflect on, "You know what? I'm going to start thinking about these things and making sure that I'm taking the steps both in my daily life, but also in taking care of those around me and thinking of those who will follow me."

Dr. Bedlack:
There's no question that you learn a lot as a doctor working in a field like ALS. You've seen some of my crazy suits. One of them is embroidered with all the symbols of all the lessons I learned. I mean, a lesson not to take little things in my life for granted. That's not the way. For the first half of my life. It was always like, "What's the next thing I'm trying to accomplish?" Well, how about if we just enjoy what we have today? I'm much better about that now, a symbol of resilience. You often encounter people and you think that they're fragile, but many times people are much tougher than you expect them to be. A symbol of hope, which I think is much more than just an emotion. I actually think it's a treatment and I'm trying to become a better hope booster, and I know we're going to talk later about this movie that I have now that's going around the community, around the country making community screenings and going into film festivals that talks about these efforts to be a better hope booster.
But no, I do think that you learn things and when you talk about making sure that you're prepared and making sure that your family's prepared, I like to talk about that with my patients as, "Hey, we got a plan A and a plan B. Plan A, we're going to try to do everything we can to slow, stop, or reverse this disease. But in case we don't, here's some things that I see that could be coming up in the next few months that I need your help deciding on."
That's the way I present things like feeding tubes. That's the way I present things like ventilators. That's the way I present things like end-of-life preferences, goals of care. It's presented that way, because number one, I think you don't want to crush someone's hopes, but number two, I think it's honest. If I see that you might need to make a decision about whether you want CPR in the next six months, do I know for sure that you're going to need CPR in the next six months? Of course not. We know that sometimes people have these plateaus. We know that sometimes we have these conversations and somebody goes on for five years after the conversation. So it's also honest. It isn't just trying to balance hope, it's honest. I mean, it's a very difficult disease to make accurate predictions in. You make some broad predictions based upon your measurements, but you never know for sure how someone is going to do.

Dr. Correa:
And I hope that people and their families have these conversations together. And it's not just the individual living with ALS who's answering these questions. But that everyone is saying, "You know what? For what I want and right now or in the next few years, I would want CPR. Or I would want a feeding tube if I couldn't eat or swallow or I wouldn't." And have their own reasons so that we all know the people we love and around us, what matters to them if we have to be the ones along their side helping them with these decisions.

Dr. Bedlack:
And sometimes, again, those questions can be framed, because families can be resistant to these things as well. They obviously love this person and they don't want those bad things to happen. But again, they could be framed as, "Hey, mom, or Hey sis, I'm still fighting and plan A is for me to get better, but just in case it doesn't, I'm thinking that maybe I don't want CPR if it goes in a really bad direction. I just want to make sure you understood that I haven't given up, but that I've made that decision that I'm not going to cross that line. I think sometimes those conversations are more palatable that way with families and friends.

Dr. Correa:
And onto staying hopeful. We heard from Caitlin in Louisiana. "We're trying to stay hopeful, but it's hard when symptoms keep progressing. Are there ways people with ALS and their families find meaning or joy even as the disease advances? It feels impossible." And I know we've mentioned some, but I really felt like I still want to speak to Caitlin and her family.

Dr. Bedlack:
Yeah, no, Caitlin, it definitely is a challenge. I mean, again, after 5,000 patients that I've worked with, I've seen the challenge of adapting to one change where today you need to use a walker, and then three months later I tell you that it's not safe to use the walker anymore, now we got to adapt to a wheelchair. It's an incredibly cruel disease, because it just keeps changing. But I also think we can look to people like Brooke for inspiration to say, "Look how she is still living in the moment. She's adapting, you can see the new equipment that she's using, but look how she's still living in the moment. Look at how she's still able to recognize these funny things that happened." There was a great post about her dad and a joke that he had made. "And look at how she's still finding pieces of her, like her creativity to be able to go out and do new projects like the clothing line. Even as the motor symptoms worsen there are parts of her that don't have to be touched."
And I think it's just important maybe even to have those conversations with clinicians, but also families and friends and maybe utilize that Turbo-Charged Living Tool to say, "Let me sort of remind myself about all the parts of me that are still doing well, and how can I utilize those to improve my quality of life?"

Dr. Correa:
And for our listeners, we'll include a link to the Turbo-Charged Living Tool and the show notes so that you can take a look at it and consider discussing it with your clinicians and your care team. Richard, one thing I kind of think of as I turn on the side of my brain that thinks of some of the things I might hear from individuals in the community or even family members. When sometimes there are people who hear, "Oh, I have a challenging diagnosis, a progressive disease." But the answer is, look back to your own inner strength or something like that. I saw this in the TBI rehabilitation and chronic progressive conditions care clinics in the military system. Sometimes there's a real resistance and pushback. You're basically saying, "You're not going to offer me anything and you're telling me that I have to fix it myself or support myself." How do you help people bridge when that is the sense and feeling?

Dr. Bedlack:
There are things to offer to people with these conditions. Now, I mean, we do have in ALS the one home run therapy for that subset, but we also have a drug Riluzole that slows progression in everyone. And we have multidisciplinary team care where people can get educated about nutrition. There's one type of diet now, a high glycemic index diet that can slow progression. They can get educated about exercises. We know there's a certain type of exercise called respiratory muscle training that can slow the rate of decline in the respiratory muscles. We know there's pieces of equipment that can revolutionize people's lives.
I mean, another advocate that's out there that I love to follow is Steve Gleason. I mean, Steve Gleason appears to be almost completely paralyzed, except for his eye movements. And yet here he is on the ESPYs receiving the Arthur Ashe Courage Award on national television. And making one of the most powerful speeches I've ever heard using his eye movements. So, I mean, there are a lot of things that these clinics offer, and then there's always new experimental options. There's more trials happening across the world now than I've ever seen. There's expanded access programs. Accessing experimental therapies outside of trials. There's even a lot of products that people can self-experiment with. And I created a program called ALS Untangled to try to sift through those and maybe find the ones that are more promising and less likely to cause harm.
So, in addition to the self-reflection that we've talked about, the understanding of maybe what things are in your life to be grateful for, what things you're hopeful about, how you can get to achieving those hopes? Yeah, I mean, there really are things in the clinics now that we didn't have 25 years ago that are making the disease more livable than it's ever been.

Dr. Correa:
And that there are so many aspects and facets to our health. And so, there's always things that we can be working on to improve our quality of life and how we are living.

Dr. Bedlack:
Sometimes that stuff, Daniel, is already recognized by patients and families, and Brooke is somebody who just appears to have it. She just has a great understanding of herself and how to maintain a strong spirit in this difficult disease, even as it progresses. But sometimes a person's doctor has to help them understand that they have to help them refocus. And I guess if I had to say in 25 years the most important thing I've learned, it's how to be better at that. And that's why I'm proud to announce this movie that's making its way around the country, Stitching Strength. And it's about how I've come to understand myself and how that's allowed me to be more resilient and positive and to be a better hope booster and what that has meant to my patients.

Dr. Correa:
And where did the idea for the movie come from? How did it come together?

Dr. Bedlack:
Well, I've kind of got an odd story with the crazy fashion and the emphasis on positivity and hope and the alternative therapies. There's a lot of unusual parts of my career. And I was talking to a colleague, whose daughter is in film school, and this is years ago now, and she said, "We need to put your story into a movie." And it took a while. I mean, there was a pandemic that basically shut down filmmaking for a few years. But eventually the studio that she works for, GlassFire Studios, came back around and we spent a year working on this. We shot over 40 hours of footage, but we wanted to make a documentary short film, which has to be under 40 minutes. So the company that owns it, that made it, did an incredible job of distilling down a really complicated story with a lot of different patients and families all over the country in the movie telling their story. And I think it's pretty cool.
So yeah, I'd love to screen it sometime for you, but it's also starting to pop up in film festivals. It'll be, this Sunday it'll be at the Seattle International Fashion Film Festival. So, you'll start to see it at other film festivals hopefully as well.

Dr. Correa:
And as you get a schedule together, we'd love to share that, both include it in our show notes, so when a listener catches this episode they can see where's the next upcoming screenings. Or if there's a website that people can follow and get a sense of where it's going, and where they might be able to catch a screening.

Dr. Bedlack:
Well, thank you, Dan. I just want to warn people, I'm not nearly as funny or charismatic as Brooke, don't expect me to be.

Dr. Correa:
We heard from a listener, Emma in Pennsylvania and kind of coming to this question about a system of support. She shared, "My husband has ALS, and beyond just the shock, I think it's probably important, at least from our perspective, to build a support system around you. It's hard to know how to ask for help and what kind of help we even need. How do other families even begin to navigate this and find the right kind of support?"

Dr. Bedlack:
Terrific question. I think it should start with your healthcare team. Hopefully you're in a multidisciplinary program where you're seeing a neurologist and a whole bunch of other types of subspecialists, respiratory therapists, nutritionists, physical therapists, occupational therapists, speech therapists, social worker, research nurse. All of these people can help you to understand a little bit better about what ALS is doing to your loved one's body, and what the options are for compensating. But also, I think that they can be the gateway to direct you to this amazing community of people all over the world who are living with this disease. I always say, some of the strongest people I've ever crossed paths with are in this community, I mean spiritually strong people.
So there's groups out there like, I Am ALS, where you can connect with a lot of different people who are living with this and finding ways to celebrate what they have left. For example, a couple of weeks ago we were on a Zoom meeting with people all over the world who are artists. And they were exhibiting their work. And again, some of these people from a motor standpoint are quite far along, but they're still making the most beautiful art. Some of these people are really good at advocacy, and so they're out there lobbying Congress, some of them are testifying on Capitol Hill. It all sort of depends on exactly what you're looking for, but it's out there in communities like I Am ALS, Her ALS Story. There's a lot of groups, Compassion to Care, ALS, The ALS Hope Foundation, a lot of places where you can find other patients and families. And I think that they can be a great source of information, but also a great source of spiritual support.

Dr. Correa:
And these ALS community organizations and their websites, I agree, are excellent resources both to help connect with other individuals, but then to find some of the things that you are specifically looking for. But one of the other things you've pointed out is really the importance of this multidisciplinary care and ALS clinics. If a listener, they themselves or their family are not already connected with an ALS clinic, and that's something that they're looking for, where do they start?

Dr. Bedlack:
Yeah, so the ALS Association has a website that has a listing of all the clinics that are subspecialized to take care of people with this disease around the country. And there's approximately 200 of them now, which is more than twice as many as there were when I first started. And again, not every clinic is going to be exactly the same in terms of exactly what they have or in terms of maybe the things that they like to concentrate on from an attitude standpoint. And so, it may require some shopping to find one that you're comfortable with, but there's several in each state now. So you should be able to find one that has everything that you need and has an approach that you're comfortable with.

Dr. Correa:
And I think part of the answer to Dorothy and her next question will be some of working through and connecting with an ALS clinic. But I wanted to see if you have other thoughts. So Dorothy from New Jersey wrote, "I've been hearing about new clinical trials, experimental treatments for ALS, like gene therapy. It's exciting to hear about so many possibilities, but it's also overwhelming. How can I stay informed about the latest research? How do I even get into a clinical trial?"

Dr. Bedlack:
Again, the best resource is usually your treating physician. And most doctors will be willing to try to match you to a program, a research program. Now, there's different kinds of research. There's research that helps us understand the disease better, which is huge. So there's projects out there like the National ALS Registry, The ALS Therapy Development Institutes, ALS Research Collaborative, the NIH project, which is called All ALS. These are designed to try to help us understand why people get this, and why some people do so much better than others. And I think those are fundamental questions to developing the next generation of therapies. But there's also clinical trials and expanded access programs and alternative therapies that people can self-experiment with. And it is overwhelming.
There's really no single website where you can go to get matched to the program that's best for you. So you really need an advocate. You need somebody to sit down with you and understand where are you in the course of your ALS, how long have you had it? What kind of deficits do you have? How far are you willing to travel? And with information like that, they can generate a list of things that you might be eligible for that you could participate in.

Dr. Correa:
And some of that, like you said, might be also even shopping within the ALS clinics. If you would like a different perspective and approach or a different opinion as to where you are and if you have other options that might be going and seeing a different expert in team and seeing if you prefer their approach and if it feels like it fits what matters to you.

Dr. Bedlack:
Yeah, I think that's going to be really important. I mean, just as there's lots of differences in people who get ALS, there's lots of differences in people who take care of it, in terms of how much time they spend with you. How hopeful they are, how proactive they are. How much they're interested in and what you're hopeful about. So I think you got to shop around a little bit and find the clinic that meshes with what you want.

Dr. Correa:
And I wanted to leave off with a question from Adam. Because I think it takes us back to the very beginning of this. It sounds like Adam is a family advocate, very early on in this process of learning what he can do, and understanding the situation and condition and the many possible steps. He shared this question, "Is early onset ALS the same as normal ALS in quotation marks or just earlier, or are there different symptoms or a different process? My sister has early onset, she's 31."

Dr. Bedlack:
Yeah, I would say the short answer is, we don't know. So there is a form of ALS called juvenile onset ALS. This tends to be teenagers and people in their early 20s. And a lot of folks with that extremely early onset ALS do have the same genetic mutation. It's a mutation in a gene called FUS. I would say 30 is a little bit earlier than the average age of 55. But if you think about it as a bell shaped curve, age 15 is way out on one end and age 90 is way out on the other end. But 30 to 70 is where most people are getting this. It's not that unusual to see someone in their thirties with ALS. And I would say we don't know why. Some people get it sooner, some people get it later. These are questions that could be answered in those natural history studies.
But when it comes to being a family member or a friend who's trying to figure out, "How can I help my loved one?" I think the first thing is to have a conversation with the person and say, "What are you hopeful about? What hopes do you have that I could help you to achieve?" Because without that conversation, you may be sort of reading your own hopes and saying, "W`hat am I hopeful about for this person and how can I help them get there?" And that might not be at all what that person is hopeful about.

Dr. Correa:
Yeah, I like that, because I heard in this, aside from the question about the specifics, is that Adam wants to be there for his sister. I can completely understand that and feel that in my own family. But yeah, just wanting to give an opportunity to have a conversation about what she's hopeful about, what matters to her, what she's scared about, and really openly listen to that and then ask the ways that you can help with any of that.

Dr. Bedlack:
Yep, exactly.

Dr. Correa:
So Rick, let me finish with asking those questions to you. What are you hopeful about? What are you scared about, and what do you want for yourself and for the ALS community?

Dr. Bedlack:
Well, thank you for asking that question, Daniel. I want desperately to find a way to end this disease. I've just seen far too many amazing people's lives be cut short by it. If I'm not able to find that by the time I retire, then I hope that what I have found that I talk about in the movie Stitching Strength, I hope that it matters to patients and families. I hope that clinicians can find a message there that allows them to stay more resilient and positive and be better hope boosters. I think those are the things that I want to be my legacy. I still want it to be, "He's the guy who ended ALS." But if it can't be, "He's the guy that found a way to boost hope and that showed us that hope is a treatment and it matters."

Dr. Correa:
And there was a part there too. I asked, and I want to kind of own together. Because our patients, their families also have fears. What are you afraid about right now for yourself or for the ALS community? And maybe then ask, what can we all do to help support?

Dr. Bedlack:
Yeah, I guess I'm afraid that the world is moving in a direction that is going to make it more difficult for us to take care of people with diseases like ALS. And for us to do research on diseases like ALS, it's moving in a direction of profit seeking. There's a lot that I see now a lot more that I see now that's all about money, whether it's government, whether it's sports, seems like money has become the main thing. And I hope we can figure out a way to move back to put the emphasis on people and what we can do for each other. I mean, I've never valued money. That's not what I want my legacy to be. I don't want to die with a lot of money in the bank. I'd rather die poor having done something that changed the world.
And so, I guess that's what I'm afraid of is that we keep moving in this direction and somebody one day tells us, "You can't run these multidisciplinary clinics anymore. They're losing too much money. And you can't do research in this disease because there's no profit to be made." At the end of the day I'm going to keep going as long as I'm allowed to, but that's what I'm afraid of.

Dr. Correa:
Yeah, I share some of those same fears, and I think what I want to do, what I want to ask everyone listening and everyone out there, is that if these stories touch you, whether or not you have ALS, know someone with ALS, have ALS in your family, that you reach out and contact some of the decision makers, the policymakers in your communities, and share how much you value the joy and the happiness and the health that's important. And that we need continued research and investment in this multidisciplinary care for all neurologic conditions, but especially including some of the rare and progressive conditions. Because from those, we may end up learning possibilities that can impact everyone.

Dr. Bedlack:
Its well said. And I really do think it comes down to how many people care about an issue. And at the end of the day, that's part of why I am so proud of what Brooke is doing. Because she's making so many people who may never have heard about ALS or didn't care about ALS, she's making them care about it.

Dr. Correa:
Yes, our hearts are with all of you, with Brooke. Thank you, Rick for joining us.

Dr. Bedlack:
Thanks for having me.

Dr. 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.

Dr. Peters:
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Dr. Correa:
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Focusing on Hope: Answering your ALS Questions with Dr. Rick Bedlack

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