Thriving in the Kitchen with Chef Dan Jacobs

Episode Transcript

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

Dr. Peters:
And I am Dr. Katy Peters. And this is the Brain & Life Podcast.

Dr. Correa:
Okay, nice to have a little bit of time off. Hopefully we're all together with family. Happy Thanksgiving to you and your families. We're all so glad to be celebrating it with you here on the podcast and during the holiday break. And of course with a food-focused holiday, food's always on my brain. We had to bring you an episode with a chef as a guest. Katy, did you watch the last season of Top Chef in Wisconsin?

Dr. Peters:
So I caught a few of the episodes. I'm always impressed with Top Chef. I'm always so impressed with how much creativity they have. First of all, I just want to say happy Thanksgiving to you, Daniel and to your family. And I'm so thankful to be chatting with you today, and I'm very thankful for our listeners. But again, I did catch a few of those episodes. It really does take skill and creativity. Now, are you creative in the kitchen?

Dr. Correa:
I would call myself a cook. In college, I worked for a caterer and I really enjoyed cooking. I like cooking at home. I love cooking around Thanksgiving and the holidays. And so I really enjoy Top Chef, but I'm always just wowed by their ability not only to create the things they do, but under so much pressure and a time limit. It's not necessarily the level of cooking that I would consistently do. I like my weeknight rice and beans. And so for this week's episode, we are featuring a discussion and interview with Chef Dan Jacobs. If any of you did see the Top Chef this season, you probably recall him. He was a key contestant in this last season. And I won't spoil it for you, but he's definitely a fan favorite. He also shares with us in this season that he lives with a condition called Kennedy disease. And throughout the different contests and challenges, we see how he physically adapts, works together with the other team members, and still is able to make some amazing food and win several challenges. I'm excited that Dan also offered to share one of his recipes with us and you, our listeners. I'm definitely adding it to my list for the fun cooking projects this weekend. You can find a link for that in the show notes. Katy, do you have a favorite Thanksgiving or holiday recipe?

Dr. Peters:
Well, I love cooking Thanksgiving. We always go get a heritage turkey from a local farm here in North Carolina. I can't wait to see Dan's recipe. I definitely have to put it in my repertoire. My favorite thing is to be creative with leftovers. I make a wonderful turkey risotto where I use the turkey meat and the turkey skin, and also a little bit of gravy. It also has some crispy thyme leaves on it, which is really pretty and it's just very attractive and a crowd pleaser. How about you? What do you bring to the table?

Dr. Correa:
Well, I also like with the leftovers, sometimes I will turn the leftover turkey into a turkey mole, but that's always just one of those things of whatever's left and gets thrown in. But the consistent thing I really like is on the table there's the cranberry sauce and there's all the other things on there and the gravy. But I like something a little brighter and tangier and not so sweet to go with the Thanksgiving table items and the leftovers. So in addition to those typical sauces, I like to make a salsa verde. And so in that I often will throw in herbs, onion, green pepper. So it ends up being really not so much of a sweet sauce, and much more of a brighter accompaniment. And we'll make sure to include a link to our favorites of these turkey risotto and salsa verde recipes so you can explore those weekend.

Dr. Peters:
That sounds awesome. I'm ready.

Dr. Correa:
And also in the show notes, in addition to the recipes, make sure that you check out and find the link for our listener survey. Please visit brainandlife.org/survey to participate in this and get a chance to enter a drawing for one of five $100 Amazon gift cards. I think that will be a nice addition to one of us being able to get a few nice things for our family members.
Welcome back to the Brain and Life podcast. Today I'm joined by Chef Dan Jacobs. He's a Chicago-born, Milwaukee based chef many of you may have recently seen on the latest season of Top Chef Wisconsin. He has multiple restaurants with the EsterEv group including DanDan and EsterEv. And since his own diagnosis and sharing with the top chef audience, he's also been working in many different ways to increase awareness about Kennedy disease.
That's one name that many of you may not really know or heard of or understand what it is. And we're going to get more into that in this episode in our discussion with Dan and also with our medical expert following that. So please stay tuned. Dan, thank you so much for taking the time to join us both in the magazine and on the podcast.

Dan Jacobs:
It's awesome. It's cool. It's funny, because my sister-in is a neurologist and the fact that I'm going to be on a neurology magazine that she gets in her office I think is hilarious.

Dr. Correa:
It'd be one of the ones where it's a publication that you get that she didn't get. So Dan, I wanted to just know a little bit more about you and your path to becoming a chef and meeting your wife.

Dan Jacobs:
Sure. I grew up in Chicago in the city, went to high school, had a pretty normal childhood experience, nothing really out of the ordinary. Got in a little bit of trouble, nothing really serious, pretty normal upbringing.
My parents were great. They worked two jobs to keep me and my brother going to a really good school. We went to public high school. After getting out of public school or high school, we didn't really know what to do with the rest of my life. Went to college for a year, just kind of floundered at it. Didn't really do well. Now looking back on it, I definitely had ADHD when I was a kid growing up. I would test incredibly well but would just do pretty mediocre in school. There was nothing that was really grabbing my attention.
I did enjoy cooking. As a kid growing up, it was always something that started out first as a way to get out of chores. So if I was helping out in the kitchen meant I didn't have to do anything else, didn't have to vacuum, or dust, or clean the bathroom or anything.
So first it started as that, but then there was something about it that it was really fun, just kind of... And nothing serious, like making tuna salad, chicken salad, making really pasta, sauce, chili. Just helping out with a very basic foods.
Fast forward, I'm like 19 years old. I moved to Door County Wisconsin for the summer with a couple buddies of mine just to have a blow off summer. I wasn't going to go back to college. I wasn't really sure what I was going to do. I was just going to see what's happening.
So I got a job as a short order cook doing breakfast, and it was addictive. It was great. There was something about the cadence or the intensity of a kitchen that just kind of made sense. So I got a job at the nicest restaurant in Door County. This place at the time called the Inn at Cedar Crossing. And I was hired because the chef liked my T-shirt. He thought my nobody knows I'm Elvis T-shirt was worth hiring me for. So I worked like salads and did some broiler stuff. But the other line cooks there had gone to culinary school and so they were like, "You should go to culinary school."
So when winter sets in Door County, Wisconsin, there's not really a whole lot to do. So I went back to Chicago and started culinary school. That kind of started my career in Chicago. And from there I just worked around Chicago for about 15 years, worked in some really great restaurants along the way. I met my wife, Kate Riley. We met at a Halloween party and just kind of hit it off. And next thing I knew we were moving in together and we got married in 2008.
And then sometime in 2010, we both kind of got laid off. Both kind of didn't really know what we were going to do with our next step, which led us to Milwaukee, Wisconsin. We just thought we needed a change. And we thought... Actually, I remember when we first moved here, we were really considering just possibly doing something else, going somewhere else. And then we made friends, and next thing we knew we were embedded here. We were part of a community, and it felt wonderful. It was awesome.

Dr. Correa:
Wow. I mean, it's such a path between Chicago to Milwaukee and carving your way through different restaurants. When you met Kate and then in those early time and years there in Milwaukee, at that point, do you feel like you had any early on symptoms of what you've noticed later on?

Dan Jacobs:
Looking back at it now, sure. But I don't think, I just thought it was, "This must be what getting older is like." Me and got married when I was 30. We met when I was 28. And so some of the things that I look back on whenever, not being able to do a bunch of squats or lunges, I think that it was just in my head was like, "Oh, it must be that I'm just getting older." Now that I look at it was obviously these were just the beginnings or some of the cramping I would get in my hands. I feel like that was early on stuff.
But I mean it's really hard to tell. It very much mimics those just simple things. I wasn't until much later that I think... Not much later but a few years later that I think we really started to know the difference.

Dr. Correa:
So when was it or what were the symptoms that really made you feel like something else seems to be going on? This is not just little aches and pains of getting older or from the hard work days that you have there in the kitchen.

Dan Jacobs:
I mean, yeah, kitchen work is intense. There's definitely a lot to it. If the cooler's in the basement, there's a lot of walking up and down the stairs with carrying loads of things. There's a lot of long hours, hot conditions. I feel like sometimes, you just take it for granted a little bit.
But we were both working out with a trainer at this gym. All the workouts were based on your body, so it was all using body movements, body weight, and just kind of working out that way. But at a certain point, Kate was excelling past me. And I kind of hit this kind of wall and just stopped. And it wasn't that Kate's a woman, I'm a man. It's just that she's physically smaller than I am, being able to do more than I can. She could lift more than I can. And knowing that, we were like, "Man, something's off."
And I remember talking to a physical therapist friend of mine and he basically was like, "I think you need to see a neurologist." And I remember being like, "There's something wrong with my brain. And he is like, "There's more to neurology in your brain." It's also really funny because at the time my sister-in-law is in med school to be a neurologist. I'm like, "You're right. I should know a little bit more about this then."

Dr. Correa:
And then at that point, you were noticing a difference in what you could do in terms of your capacity and strength compared to Kate, but were there specific things that you were noticing with your arms or legs or were there challenges you were noticing in the kitchen with the hard work that you do?

Dan Jacobs:
It wasn't necessarily in the kitchen so much as I think it started, I used to run, but I couldn't run anymore. I couldn't run. I'd get way tired way early. I used to be able to put in eight or nine miles not really feeling like I was going to die. And I felt like that's where it was going. It felt like it was like I'd get a mile in or something and I'd be like, "Man, I'm tired." And I think that was something specifically on my right leg. My right leg would start to almost numb. It was like my foot would feel like it was falling asleep. And that was kind of the impetus to go see a neurologist.

Dr. Correa:
Yeah. So you noticed changes in your sensation and strength. Were you noticing any differences in how your muscles looked from one arm or one leg to the other?

Dan Jacobs:
Yeah. It's funny when you say that. Definitely in the hands. You could see the muscle waste in my right thumb as opposed to my left. Now it's a little bit, now they're both getting about the same, but you could see the muscle waste a little bit in my right thumb. And I remember that was something that the neurologist pointed out at the University of Chicago.
It was like serendipitous that my sister-in-law was in her residency at the University of Chicago for neurology, and this was somebody I needed to go see. So I remember going to see her attending physician Dr. Rubeiz for the first time, not really knowing what to expect.

Dr. Correa:
So one of those situations where you went around the typical pathway necessarily to getting in the hospital, and I understand that, because sometimes I've had to advocate or contact people for different family members of mine to make sure they get in to the right place near them. It sounded like your sister did the same for you.

Dan Jacobs:
Sister-in-law.

Dr. Correa:
Sister-in-law.

Dan Jacobs:
Yeah. So Andrea, she was at the University of Chicago. So I got in to see the attending physician and I went by myself. From Milwaukee, drove down there and didn't really think much of it. I didn't really know what was going to happen. It wasn't until they were running some tests where they were putting needles and sending a little bit of electric current through each muscle group that was causing to see the reaction of it. They would send little things through to the muscle groups and then they would register. And I remember the students who were doing the tests, they were very excited. They were just kind of like, "Whoo, whoo, whoo." And I'm like, "Yo, what's"-

Dr. Correa:
Not always good sounds to have from a neurologist.

Dan Jacobs:
And I'm like, "Yo, what's going on? What's happening?" And they're like, "Oh, your doctor will tell about it." I was scared all of a sudden and they start throwing out some stuff. "We're not really sure, but this might be ALS, this might be Parkinson's, this might be MS." I mean, these are scary things and they just wanted to be honest with me. And it was like we were all kind of freaked out. My business partner's freaking out, because we were in the process of opening up our first restaurant. We hadn't opened up yet. And so he was, we're about to open and then we find this out. They run one more test. We do a blood test that we find out it's Kennedy disease. And again, I don't really-

Dr. Correa:
This was all on the same day or you mean-

Dan Jacobs:
We ran the blood test. Well, they took the blood, but then we found out a couple days later that 100%, this is Kennedy's disease. Dr. Rubeiz was really good at being able to, "I think this might be it." We did this one blood test, that was it. And then my brother took a blood test. He was also positive, and so we knew exactly what it was. At that point we're like, "Okay, this is what this is."
I didn't really think anything of it. It was like we were in the middle just about to open up a restaurant. We were three months out from opening, and there was so many things going on and I was just kind of push it to the back burner. I had a six-month checkup in October and I was like, "All right, we'll just do that." I remember going to the six-month checkup in October and me and Kate had kind of made, we were like, "Oh, we'll make a weekend of it. We'll go to Chicago, we'll go out to dinner. We'll have some fun."
I think that's when everything really sunk in was at that checkup that they weren't going to be able to do anything for me. There was no pill, there was no treatment, there was nothing. And I think that's when things really sunk into. It was like, I'm going to have to live with this my entire life. This is something that will continue to go down this degenerative road, which I'm going down right now from now. I used to not have to wear any sort of brace. I now wear AFO braces on both my legs to keep me moving around.

Dr. Correa:
We jumped over a moment there where you were told in the office by these team of training neurologists and students that, "We don't know what's going on." We're seeing really interesting, but you don't necessarily want to hear that from those teams, abnormalities. And they're telling you it might be ALS, it might be all these other conditions. Until you really had that discussion with Dr. Rubeiz or until you really got the lab results of this other test that this other condition they said it might be, what were you processing together with Kate about what might be going on?

Dan Jacobs:
It was weird. You had this relief that there was something wrong. It was like, "Okay," in my head, there's definitely something here. But I think to say you're afraid was an understatement. But I can't, and this is the way I've always been, and for better or worse is who I am. I'm going to worry about what I can deal with. But things that are out of my control, whether or not I have this disease or don't have this disease, I'm not going to sit there and really ponder this. I'm going to wait until we get some results and then move on to the next thing. At this point, I'm pushing it all aside just to... I guess it's a way of dealing with it.

Dr. Correa:
Yeah. Well, I mean, as you said, you were so busy trying to open a restaurant. And it had so much going on. In some ways maybe that was a welcome distraction. And it wasn't until you went back for that follow-up, as you mentioned. And so now at that point when you and Kate have taken a moment to step away from the restaurant, have a weekend to really think about, probably you didn't plan on necessarily being a weekend to think about your condition. But how did you two talk about the fact that they were sharing with you a diagnosis for a neurodegenerative condition, but thankfully wasn't as severe as some of the other things that they were telling you about?

Dan Jacobs:
Well, first and foremost, Kate is an incredible support. Unwaveringly. She's like, "This is better or worse. It doesn't make a difference. I'm here for you." And I'm not trying to fast forward too much, but I mean she has to pick up a lot now along carrying things. So she has to do a lot of that heavy lifting now for us.
But initially, I remember we went out to dinner and I mean, I was mad at the world. I wasn't mad at anyone. I was just mad at everything. And it was a real tough thing to kind of accept, like, "Okay, now what do we do with this?"
We were both upset about it. But one of the coolest things I think that could have come out of it was Dim Sum, Give Sum, which was our annual fundraiser that we do was birthed that weekend because I remember I was like, "Look, I can't solve this problem. I don't have the education to solve this problem. But what I can do is raise money."
And I remember we went back to the restaurant, we told the staff about it. I think the staff was very emotional as well. But again, it's like I've leaned on our teams for so long, I couldn't do what I'm doing without them being here and doing that for me.

Dr. Correa:
I mean it speaks to just how deeply you feel your staff and team are really your family, that that soon after finding out about your diagnosis, you come home to include them and share with them in that.

Dan Jacobs:
I kind of had to. I felt like sharing, the more I shared it, the easier it was to process it. I felt like the more I put it out there, the more I talked about it, the better I became at dealing with it.

Dr. Correa:
And you talked about there's a lot of hard work in the kitchen. I worked catering and I love cooking, but it's nowhere near the kind of work that you guys do in the kitchens. And you said you can't really carry things upstairs. Several things are challenges for you. And then you signed up for Top Chef.

Dan Jacobs:
So I've always wanted to do Top Chef. The way my brain works, and part of this again is because I have ADHD is this is going to be really... I think this is where I'm going to be really good.
I went into doing Top Chef with the full expectation of winning Top Chef. It was something that I've wanted to do for a long time. I think the first time I tried out, I was 28 or 29. There's a certain period of time where you stop asking them and they just start asking you to try out.
And so I'd say the last eight or nine times, I worked on it myself probably the first couple times. And after that, every year they'd be like, "Hey, do you want to try it again? Hey, do you want to try out again? Hey, we're going to do a new season. We'd love to see if you're a good fit this time."
And I remember them asking me, I think it was in early '23 if I was interested. Again, I remember me and Kate talking about being like, "All right, this is going to be the last time I'm going to try out." I was like, I'm just kind of, because man, it's a grind. You know what I mean? It's a grind. And so it was one of those things where I was like, "I don't know if I could physically do this after this." And so I was like, "Okay, let's try out." And I kept just getting a little bit further and a little bit further, but I kept pushing it in the back of my head being like, "Ah, something's going to come up." Because this has happened so many times where it's like you get to a certain point and they're like, "Oh sorry, you're really close. But we moved on."
I think part of it was, and we didn't know this up until I think a couple weeks before, I think maybe it was a couple weeks before I found out I was going on the show that it was going to be in Wisconsin. So we didn't know this going into it, but I think there was, part of it was there was going to be in Wisconsin. But I think that my story for whatever reason, resonated. And I've had a bunch of friends who have done the show and that were like, "You should bring this guy on. This guy's the real deal.
And I think I'm also fun. So I make good television. I say things that, you forget you even have a mic on and you're just like, you're babbling to yourself. It's funny when you rewatch it. But I don't know, for whatever reason, this was the year and I'm grateful to have had the opportunity to showcase myself on a national spotlight like that.

Dr. Correa:
It was just a great season. I have to admit, I'm East Coaster in New York. At first I was like, "Oh, I don't know. Top Chef Wisconsin, what are they doing?" And then I loved the season. It was one of the most enjoyable ones. You came on full force. In the first few episodes, you hadn't really yet shared what you were going through and some of your own challenges and limitations with the other contestants. What you or what changed in helping you feel that you needed to open and share with the fellow contestants?

Dan Jacobs:
Well, for the first episode, I did it all without using my cane. I refused to use the cane, and I tried to run into the Whole Foods and I was like, "Well, that didn't work." And it was just one of these things where it was like I was trying to push it and I was just like, this just isn't working. Once we put shorts on, they're going to see that I'm wearing a brace. At the time, I only had brace on my AFO on my right leg. And so it's like they're going to see that I'm wearing this brace on my right leg. So eventually there's going to be the questions. And I was like, "I don't want to make it up."
And one of the reasons why I went on the show was I wanted it to be something I could talk about. I wanted to talk about on a national level. I wanted to raise the awareness of it. I wanted to help raise more funds for Kennedy's disease research. I wanted to be able to do these things. So it was always kind of in the cards.
So the judges didn't know about it. The producers knew, but apparently they didn't say anything to the judges. During the second episode, one of the guest judges was a friend of mine, and he mentioned it to the judge. He was just like, "It's amazing what he's doing considering his condition and everything." And they were like, "What condition?" And then it kind of came out.
So one of the executive producers pulled me aside before we went to judge's table after the second episode, after the second challenge. And he was like, "Hey man, just so you know, this person talked about your condition. Are you okay with the judges want to talk about it a little bit?" And I was like, "Yeah, no man, this is what I wanted to do." And I told all the contestants, some of them I'd already kind of told already they had seen the brace and they're like, "What's up with that?" And I shared with them a little bit.
The second episode was the first team challenge, and my team knew that I was not going to be the person who was going to be running around. I never was. I've said this 1,000 times during the show. They'd be like, it's funny, when you watch the beginning promo, all the chefs are running up to the table to grab hops.
And first off, for the four dishes we're doing, you need a hop. So it's in part that so that flavor, it's very strong. And you just see me in the background walking off to the side, because for me it was the more important to get to a station that was easier to move around in than to grab a handful of hops that I don't even need. And it was funny because the other contestants, I was totally slow and steady wins the race. I never was one that was running around doing stuff, but you have to think a bit more. And I knew that there was only going to be a couple, it's not anybody on the season who could think as well as I could about food, who knew as I did about food.

Dr. Correa:
It looks like it is incredibly mentally challenging in your intellect with food and intent and strategy that you take really comes across. For our listeners, if you haven't seen Top Chef Wisconsin, I encourage you all to watch it, but we're going to keep talking about it. So there's going to be some spoilers. I think most people who are fans of Top Chef don't realize how much of a competition it is even to get in. It's not just like a single audition or a few auditions. You really did a lot of work even for many years. And in your experience trying to get on the show.

Dan Jacobs:
It's weird when somebody, it's like their first, there's a couple of them, Savannah and Daniel's like, "Oh yeah, it's my first time." I was like, "Seriously? Get out of here."

Dr. Correa:
And then you went through the season itself, which just looks like an amazing and incredible feat of work to get through all the different challenges. How long does it actually take to get through, before the finale? How long were you there?

Dan Jacobs:
So we were here in Wisconsin, in Milwaukee and Madison for six weeks.

Dr. Correa:
In six weeks you do all that. Wow.

Dan Jacobs:
Yeah, it was six weeks. It's pretty intense. I mean, they have us on a pretty tight schedule. You don't have a lot of full days off. You have a couple of these, I think every three or four days you get, they call it an interview day. So you have to do interviews where all the background stuff happens. But pretty much you're off. Besides that, you just have to go sit in interviews for two and a half hours and just answer questions. But it shows you the ebbs and flows of that show.
It's like right after that episode I end up, I go on a tear. It's like I won the episode before that episode, I was on the bottom, and then I go on a tear where I'm on top the next five episodes or something like that, in one Restaurant Wars, which is great. That was a real cool one. That was fun to, I mean, it was definitely a team challenge. It was a team effort, but I had the best dish of the day and that always feels good.

Dr. Correa:
And as we'll review with our following guests, Kennedy disease is a nerve and muscle condition that does progress with time. And you're going through all of these challenges and work on the show. Did your perspective on rests have to change some for just being able to recoup from all of this work that you're doing?

Dan Jacobs:
100%. You really have to, I was a big subscriber to stretching and yoga in the morning, stretching and yoga at night. Just say keep yourself going. I would wear a compression... I basically was fully compressed at all times. I joke around compression. I had a compression shirt on. I had compression, I had these compression pants that they had screws on each side. Not screws but dials that you could compress your legs more or less. But yeah, I'm a subscriber. After that, compression socks are the way I'm a full subscriber. I don't care how stupid I look. They're great.

Dr. Correa:
Well, I enjoyed watching and particularly a lot of the work that you did and put into your menus, and your recipes, and just really embracing being in the moment.

Dan Jacobs:
I just tried to have fun, man. I feel like that was the biggest thing. It was like you got to be like water. Let everything kind of just go with the flow with it and then just try and have a good time. I had, there was five things. Somebody smarter than me told me how they approach some of these challenges. And there was five things, but the last one was have fun. And it was always the thing. It was like, because you cook from... It's not a quantifiable thing, but happier cooks make better food. It just is what it is. And it's like if I'm having a good time with it. I always feel like I'm cooking better.

Dr. Correa:
Well, we had a great time watching you and cheering you along. I know many people in the neurology community, people living with neurologic disorders, our team at Brain & Life, we're all definitely cheering for you, not just because of the love of your food, but to see you there representing members of the community.
Since the show, unfortunately you didn't win. It was a great finale. I think all three menus were amazing. But since the show, how has it been hearing from members of community, of people living with neurologic conditions, people with Kennedy's disease? What has changed for you and how is it connecting with people?

Dan Jacobs:
So it was really cool. I think the coolest thing about being on the show was the messages I would get after the shows aired from people that had ALS or people that had, or family members, somebody who had passed away from ALS. Or you had other people who had Kennedy's disease. You had people that were dealing with Parkinson's or MS would reach out to me and be like, "What you're doing is just amazing." I remember there was a teacher, she was like, "I love teaching. And just with MS, I thought it was just going to be too difficult. But watching you just deal with your disease and be able to continue moving forward with it," she's like, "I want to do the same thing."
And that's the stuff that was, that's why I did this. I wanted to raise awareness and inspire people. And the fact that people were inspired by that, it means everything. That's the best thing that could have happened. It was better than 250,000. I mean that. I'm not just saying it.

Dr. Correa:
And have you felt like an impact for your work and for the restaurant and your team?

Dan Jacobs:
I mean, absolutely. I think citywide in Milwaukee, it's kind one of those situations where high tide raises all boats. But talking to other restaurant owners, they see a bump in business this summer just from Top Chef. We've definitely seen it in our restaurants. Just being able to push our team forward and financially compensate better. I mean, that's all it really matters is keeping the teams together and moving people forward.

Dr. Correa:
And as I mentioned, Kennedy disease is a progressive condition. You talked about how during the show you were wearing one AFO or one brace on your ankle. I took from that, that meant that you're wearing a brace now on the other side.

Dan Jacobs:
I am. Yeah. So every year I go to... So Kennedy's disease has such a small amount of people that I actually go to the ALS clinic at Froedtert Hospital, which is the large hospital here in Southeastern Wisconsin. And I meet with, it's like one day and it's like three hours and I meet with nine specialists. And I was meeting with the occupational therapist and I was describing what was happening with my left foot and she was like, "Unfortunately it's time or the FO," but wearing the two FO braces. And it's cumbersome and it's a little annoying, but it's the difference between me being able to do my job and not. It's that simple. It's the difference between me being able to play restaurant and not.

Dr. Correa:
Yeah. And if it's what you enjoy and you're enjoying it now, that's awesome.

Dan Jacobs:
I love it, man. It's weird. It's so funny to me. It's like I don't know, restaurants are insane businesses that make almost no money yet. Somehow you just really got to have a love of the game for it. And it's something about hospitality that I've always enjoyed. And there's something about having somebody come in and just being able to change their day from something they came in a bad mood and they leave in a good mood. It's magic, it's beautiful.

Dr. Correa:
And when things change, I mean, how do you check in with yourself, check in with Kate and your team just to say, it seems like it's changing? Maybe my left leg is not working the way it was before. How do you determine where you are and involve other people in talking about when you need either more help or change in the responsibilities that you're doing?

Dan Jacobs:
I think I said before, it's like the more I talk about it, the easier I think it is to have that conversation. But also, asking for help is a sign of strength. It's not a sign of weakness. We've always been that way. It's the same thing in a kitchen. Sure man, you're going to get overwhelmed on your station or get overwhelmed with your prep at certain times. Asking for help and knowing when you need it, to me, is the sign of ultimate strength. And I feel the same way about it in the circumstance. And we're blessed with having an amazing team at both restaurants that are willing to pick up the slack that I genuinely have. I mean, again, my brain is sharp as a pencil. Sometimes the limbs don't work the way they want to. But I mean all the information's up there still.

Dr. Correa:
I know you have cooking and a team to get back to. I wanted to particularly thank you, Dan, for taking the time to join us and our listeners for wetting all of our appetite to understand Kennedy disease a little bit more. Thank you so much, man.

Dan Jacobs:
Thank you so much. I appreciate it.

Dr. Correa:
Make sure that you check out and find the link for our listener survey. Please visit brainandlife.org/survey to participate in this and get a chance to enter a drawing for one of five $100 Amazon gift cards. I think that will be a nice addition to one of us being able to get a few nice things for our family members.
All right, so I know it's Thanksgiving day. We're all hungry or probably need to get back to some cooking, but I'm hoping that you stay tuned now for our expert discussion with Dr. Chris Grunseich. And so I'd like to continue this and learning about what Dan shared with us about his living with Kennedy disease with an expert who works within the condition.
Dr. Christopher Grunseich is a neurologist and researcher with a focus on neurodegenerative conditions working at the NIH and National Institute of Neurological Disorders and Stroke. It's a lot of different words and acronyms. It summarizes NINDS, but he dedicates his efforts to deeply understanding how neuromuscular diseases affect our bodies, including Kennedy disease. Dr. Grunseich's research actually helps at the frontline of taking science from the lab to potentially future effective treatments that could improve the quality of life of people living with these variety of conditions. Chris, thank you so much for joining us today.

Dr. Grunseich:
Thank you Dr. Correa, it's a pleasure to be here and discuss this topic with you.

Dr. Correa:
So Chris, Dan shared with us that he disclosed his diagnosis of living with Kennedy disease on Top Chef. I don't know if you were watching or have heard about it. Has this been in the topic of discussion in the community?

Dr. Grunseich:
I've heard about it. We've been very interested to hear about Dan and we've also learned about it through our Kennedy's Disease Association that Dan is very prominent individual with the disease and very curious to see what he has to say about his experience with KD and also what he hopes will happen in the future for patients living with KD.

Dr. Correa:
Yeah. I mean, I'm glad it's bringing more attention and awareness to this rare condition. But for our listeners, can you help us understand a little bit better what is Kennedy disease and what is its genetic or other aspects that we should be aware of?

Dr. Grunseich:
Yeah, Kennedy's disease, also called spinal and bulbar muscular atrophy, SBMA, is a disease that's caused by a CAG repeat expansion in the androgen receptor gene. So the androgen receptor gene codes for the androgen receptor protein, and it's a protein that serves many functions throughout the body. Most prominently in males, the testosterone binds to the receptor, and the receptor then moves from cytoplasm into the nucleus and turns on a whole host of genes that allows for male features in males to be expressed.
Now in SBMA, that repeat expansion changes the function of the receptor and results in toxicity. We think that the toxicity is both in the muscle, and to some extent also in the spinal cord and the motor neurons. And as a result of that toxicity, features usually involving weakness or cramping affect males with this condition.

Dr. Correa:
Now, yeah, you were just describing that it's the androgen receptor or really that hormone. And you've mentioned how it affects males. Does that mean that this condition doesn't typically affect females, or how is that different?

Dr. Grunseich:
Yeah, it's a great question. And so females are carriers of the disease. So females who are XX, the disease gene is inherited on the X chromosome. And so females typically will have, if they are carriers, will have one normal copy of the gene and the copy on the other allele is the copy that can have the expansion.
And what we typically see in females is that there is no or very minimal weakness. Females can have sensory changes, neuropathy, cramping, but usually the symptoms are much less pronounced in the female carriers.
And female carriers have about a 50% chance then of passing on that affected copy of the X chromosome to their children. And when males inherit that copy, their XYs, they only have that one copy with the expansion. And it's thought that that inheritance of the expansion together with the testosterone levels that are higher in males, that both of those are needed to result in the disease.

Dr. Correa:
And then when we think about it, I mean I'm sure this is a big aspect of an individual being diagnosed and thinking about family planning. Does this mean that every male or XY descendant of that individual will have the condition?

Dr. Grunseich:
That's a great question. So if there's an affected male, that means that that individual's sons will not have the condition because an affected male will only pass on the Y chromosome to their sons. So their sons will not have the disease. However, if an affected male passes on their X chromosome to their daughters, that daughter is what we call an obligate carrier. The daughters of an affected male would be carriers. And those carriers then have a 50% chance of passing on that affected copy of the X chromosome to their children.

Dr. Correa:
Okay. Wow. So yeah, genetics can be its own little puzzle. It's at least helpful to understand some of the aspects of it. We will of course also include in our show notes some more resources where people can go to learn more, and especially even understanding some of these genetics with this condition.
Now, Dan shared with us some of his initial early symptoms of having challenges using his hand, having challenges mobilizing, and really having issues with one of his legs. And he shares and shows on the show how he adapts. But I'm wondering across the community of individuals who get diagnosed with Kennedy disease, are there certain consistent symptoms that may be different or compared to other neuromuscular conditions?

Dr. Grunseich:
Yeah, that's a great question. So typically, some of the earlier features that we can see in patients can vary across individuals with SBMA. It's common to see cramping and tremor early on in the disease. Of course, patients can present with weakness. And that weakness can be either in a proximal muscle up in the shoulder or weakness down in the hand. It can be weakness in the lower extremity or the upper extremity.
Some patients can also present with very prominent bulbar weakness, so they can have difficulty with speech or swallowing. And also what sets this disease apart from other mimics of the condition is the sensory neuropathy. So patients can also have loss of sensation, and this typically happens in the distal parts of our extremities, in the fingertips and the toes. And that sensory change can be detected on the nerve conduction studies that patients may complete as part of their diagnostic evaluation.
And in addition to those muscle features and nerve features of the disease, sometimes we also encounter a loss of androgen function in the patient. So the disease is complex, and in addition to the toxicity from the mutation in the androgen receptor, there's also a loss of androgen function that can happen in patients. And so patients can have early on in the disease gynecomastia. There can be breast enlargement. Patients can also have infertility. And so there's both toxicity to the nervous system and to the muscle. And there are also other symptoms that can happen throughout the body. And the more the research is done, the more we're learning about how other organ systems can be affected in this disease.

Dr. Correa:
And that sensory involvement is quite unique within neurodegenerative conditions that affect the nerve and muscle. That sensation can be affected by other conditions that affect the nerves or neuropathies. But why is it that Kennedy disease affects the nerves when many other neurodegenerative conditions of this type don't?

Dr. Grunseich:
Yeah, we don't know for sure how the mutation in the androgen receptor results in this toxicity, and it's likely that there is damage to both the muscle, that we know the receptor is expressed in skeletal muscle, and there's likely some degeneration that's happening in the muscle and in the nerve. And it's likely potentially a combination of both of those that's resulting in the symptoms in that patient's experience.
But what we see is vulnerability to those nerves in the body that are a length dependent process. So the longer nerves tend to be affected in terms of the sensory function, but the muscles and the nerves that are impacted as a result of weakness don't necessarily follow that same pattern in that we do see proximal and distal involvement in terms of the weakness that occurs in the disease. But it's thought that it's toxicity to both the nerve and the muscle that's contributing to the symptoms that patients experience.
Now there are other diseases that can mimic SBMA, and one of the main diseases that is frequently misdiagnosed as being SBMA is amyotrophic lateral sclerosis, ALS. And ALS, we know it to be more of a neurogenic condition that the disease is driven primarily by degeneration of the motor nerves. And so the disease process though in ALS is different than in SBMA, in that the disease is much more rapid and has more severe progression compared to the average rate of progression of a patient with SBMA.

Dr. Correa:
Before it gets to the stage of being able to get the genetic testing, is there really any way other than the speed of the progression to be able to distinguish whether or not someone might have ALS and Kennedy disease? Many patients that in the few that I've seen with Kennedy disease in my training before have often been told that they had ALS until later on, additional testing came back or it wasn't progressing and then more testing was done.

Dr. Grunseich:
No, that's a great question. Typically, what we see in patients with SBMA is some mild elevation of a level, a metabolite that can come from the muscle, the creatine kinase can be mildly elevated. And this elevation is also shared in patients with ALS. But what is typically seen in patients with SBMA that is a little bit unique is that sensory finding.
So patients will have on nerve conduction studies, a loss of that amplitudes with sensory nerve function throughout the body. And it's that loss of the sensory nerve amplitude action potentials that can distinguish the main clinical features that are present in patients with SBMA compared to ALS.
Now there also are other metabolic features that typically can occur in patients with SBMA. Patients can have alterations in their fasting lipid levels, some evidence of insulin resistance. And those features can occur early on in SBMA and may occur less frequently in patients with ALS. But one of the main features that we see that can distinguish clinically SBMA from ALS is the changes on nerve conduction studies with sensory neuropathy and SBMA.

Dr. Correa:
All this brings to mind the fact that Chris, your day-to-day work, whether it's in the research or even working with individuals and family members, really plays along this gray margin of what we don't know in medicine. How is it for you really being there each day talking to individuals about some of what we know, but often probably talking much deeper into what we don't know or understand about their condition?

Dr. Grunseich:
No, and it is a challenge. We need to focus on the research. We need to emphasize what we can do to put the field in a better position for the future by learning more about different markers that will inform us as to whether or not different candidate drugs are working. And we need to be very collegial with that and to try and as a field, come up with better ways that we can learn from those studies that we plan to do in the future to learn whether or not there are better ways for tracking the disease and understanding the impact of candidate therapies in the future.
I think we are optimistic that as a field, we're learning gradually more and more about how to evaluate therapies in SBMA. But the hope is that in the future, we'll have something that's meaningful that impacts the quality of our patients' lives, and we can hope to get to that point in a stepwise fashion. We need to gradually learn more about which markers we should be evaluating in clinical studies, and then employ those markers in future clinical studies to find out if therapies are having the desired effect.
But I'm really open about communicating that process to our patients and encouraging them to learn more and to get involved if there's a clinical study that's taking place that they're interested in, to learn more about that and determine whether or not that's an appropriate study for them to be involved in.

Dr. Correa:
And where does the NIH, and specifically the section that you work within the NINDS, stand in this pathway to figuring out not only more of the basic understanding about medical conditions, but directions for treatment? I mean, you guys have patients that come there sometimes for further evaluation or for some of these studies. But what is its role as a hospital or a research center compared to the many other ones that are around it?

Dr. Grunseich:
Yeah, that's a great question. So the focus of our work is to better characterize the disease and to put ourselves in a better position in the future for developing a treatment. And so the way that we do that is by carrying out studies that are focused on that goal. We have what we call a natural history study, which is designed to target a specific patient population, patients with SBMA, and to do a battery of tests involving tests of muscle strength, muscle imaging, collecting different fluid samples to develop biomarkers, and to bring patients back in longitudinally to track how those measurements change.
And that type of work is very helpful for both understanding more about how the disease progresses and which measurements are sensitive at detecting that progression, and then helping us to use that data then to design clinical studies in the future. And so our team is in the process of conducting that type of natural history study. We share the data with our collaborators and with other research teams that are interested in developing therapies for patients with SBMA.
And so the work that's being done at the NIH is hopefully getting us to the point so that we're better able to conduct those types of clinical studies in the future in a shorter timeframe with fewer patients, because we know clinical studies are very expensive. And so the goal with refine these different biomarkers and endpoints is to make those studies more feasible for us to conduct with fewer patients, less time, so that we can move on to the next drug if the first one is not successful, and eventually continue that process until we find a drug or treatment that is impactful on the disease.

Dr. Correa:
So Dan himself mentioned it, but I mean it's sounding like what I'm hearing is that we're not yet at the stage for a definitive treatment that could stop or maybe modify the direction of the condition. Are we at a stage right now that there are even treatments to help manage symptoms and some of the other progress of the condition?

Dr. Grunseich:
Yes. So we do have a number of therapies that are in the pipeline, meaning they are undergoing evaluation and research right now. Some of those studies have been recruiting patients, some are in the process of continuing to recruit. And so I think we're really excited about the prospect of the results from those studies and the possibility that some of that data will show evidence of efficacy that would be helpful for getting us to the point where we have something that's able to modify the disease.

Dr. Correa:
Okay. So there are strategies and ways of connecting with a supportive team to help a person live better with Kennedy disease. But right now, not treatments that will help stop or change the progress.

Dr. Grunseich:
That's right. Unfortunately, right now, we don't have a therapy that modifies the course of the disease,

Dr. Correa:
And all of us are familiar to hearing now that there's technologies to just insert fixed genes in different ways. Can't we just fix the gene?

Dr. Grunseich:
Yes, that would be a wonderful therapy if we could do that. I think that there is an interest in trying to target the expression of the mutant end receptor, perhaps with technology such as antisense oligonucleotides, that an ASO could be developed perhaps to reduce the expression of that mutant form of the receptor in the spinal cord or the muscle. And so there is interest in that particular avenue of therapy now to actually go into the patient's gene and edit that gene to remove the expansion. I think that would be terrific if we had the technology to do that. I think we just need to find a safe way for that type of therapy to be delivered in the future.
Right now, I don't think we're quite there yet, but it is definitely a hope of ours that in the future we could go in and try to edit that gene, and that would be a useful therapy because it would still allow for the normal function of the AR and help to reduce the toxicity throughout the body.
So I think that right now, some of the therapies that are being investigated do provide some level of precision and reducing the ablation of the end receptor. But I think gradually as the field of genetics and CRISPR technology evolves, that hopefully in the future we'll have a safe way of delivering that type of therapy for our patients.

Dr. Correa:
Yeah, it's amazing that it's progressing. But I do remember back to my med school days when I learned that the genes aren't just a manual that gets read once and the body follows suit, that they exist in every single cell in our body. And so depending on these technologies right now, they're mostly being designed to be personalized and targeted to very specific tissues if it's even that possible. But in this situation, it seems like I mean, you have the challenge of trying to deliver it to many, many different cells and organ types throughout the body.

Dr. Grunseich:
Yes. No, that's right, Daniel. Because we think that the muscle is also a site where there's toxicity, and we know that the muscle is a multi-nucleated syncytium. And with all those millions of nuclei, we don't know if we were to deliver this type of therapy, how many nuclei we would need to target before we saw some benefit.
But I think we have to keep trying, and we have to be open to this possibility as a field, and think about ways that we could start out by safely testing some of those approaches. But yes, the disease is multi-systemic. We know that there's involvement in many different organ systems, and finding a way to deliver that type of therapy safely would be really important.

Dr. Correa:
So this point, not as simple as finding the one broken Lego block or the miscolored one, taking it out and replacing it or fixing it. We have a ways to go.
Now we've related how there are some similarities to the condition. We use the acronyms ALS. And there's been several new treatments that have gone through research and some that have come out to the community within ALS. Is it possible that a treatment for ALS or another neuromuscular condition that shows some overlap with Kennedy disease could move over for Kennedy disease, or are they too different?

Dr. Grunseich:
Yeah, that's a great question. It's our hope that some of these therapies will transcend the disease condition itself. So perhaps a therapy that is found to be efficacious for patients with ALS or another form of muscular dystrophy would be beneficial for patients with SBMA. And I think we need to consider that.
So for some of the therapies that are designed, let's say for a specific form of inherited ALS or other muscle that it may be less likely to be beneficial in SBMA. But depending on where toxicity is and where the drug's mechanism of action resides, there's the possibility that that drug or target could be helpful for patients with SBMA.

Dr. Correa:
And you mentioned before that there were several things in the pipeline. So by that, you mean there are possible treatments and therapies that are undergoing investigation right now?

Dr. Grunseich:
Yes. So there are three main studies that are ongoing. There is a study of a curcumin analog called AJ201, and that's a drug that's been developed by a company in Taiwan. And here at the NIH were one of the sites throughout the United States that participated in a phase one two, a randomized double-blinded study to look at the safety and tolerability for co-kinetics of that drug. And that was a drug that was also tested by Dr. Fischbeck in animal and cell models of SBMA and showed benefit in the animal and cellular models. And now we're completing that early stage of that study in our patients and hopefully that that data will be promising and we could move on to a larger study of that agent in SBMA.
There's another study that's being done by a team mostly in Italy, Dr. Gianni Soraru of Clenbuterol. That's a drug that has a beta. It's a beta2 agonist, and just spoke with Dr. Soraru. That study is underway and hopefully there's evidence of efficacy from that work. And the other study that is recruiting it looks very promising, is a drug that is an androgen receptor modulator. So it's a drug that binds directly to the receptor and changes the receptor's function. So it still preserves the normal function of the air, but it interferes with the toxic function of the receptor.

Dr. Correa:
That's great to hear that progress. And you mentioned that there at the NIH also have what we call a natural history study. In that, what are its goals? And for that study or any of your other work, how can people reach out if they're interested in learning more?

Dr. Grunseich:
Yeah, thank you Daniel. So the natural history study is designed to follow patients with SBMA and we follow patients longitudinally. We have them visit the NIH and spend two to three days performing a battery of different tests focusing on evaluations of muscle strength and function. We do imaging of the muscle by MRI, by ultrasound. We do some laboratory tests looking at blood and urine.
There are optional procedures during the study to do a lumbar puncture to collect spinal fluid, and also a muscle biopsy of a muscle in the lower leg. Again, those are optional. They're very well tolerated.
And then we also have patients wear devices that they bring back home. These devices are accelerometers that patients can wear on their wrist, because we're really interested in capturing data. And that data is hopefully a reflection of the activity level of our patients when they're back in their normal environments at home. And we want to understand how the data that we're collecting when the patients are at the NH is related to their total activity level when they're in their lives back home.
And the purpose of the study is to help us refine the tools and the techniques that we use to track the disease and the tools that we use to develop clinical studies in the future. If we have a tool that very accurately predicts how the disease is progressing, we can use that measurement to help us design clinical studies. So if we have a reliable way of measuring that progression, we can use that data to help us power the studies for testing therapies in the future. So how many patients to include, how long they should be followed. And the goal is to put us in a better position of designing studies that are involving fewer patients, less time that would save us money and also save the patient's time so that we can hopefully test several candidate therapies in the future with less effort and would help us identify a candidate therapy faster for less cost.

Dr. Correa:
We can be hopeful. There's a funny thing sometimes in medical research that the team approach and even just the engagement in a research study sometimes shows a benefit in the control group or the people who aren't getting the test medicine, and sometimes that skews the results. I'm wondering just even in your interactions with individuals and families, do they feel that there is a benefit to participating in the natural history study, or maybe even it's just a sense of purpose and giving back?

Dr. Grunseich:
Yeah, no, that's a great question. And so we do help patients engage with the team approach that we mentioned before. And we know that the symptomatic management of the disease is very important. And sometimes it's just that discussion and reviewing the symptoms that are most burdensome to our patients, and identifying ways for those patients to mitigate that burden by finding specialists who can help them with their concerns. And working with a physical therapist and occupational therapist, that can have a big impact on our patients because they could find ways to improve their activities of daily living and do the same types of tasks with less wearing out and less fatigue.
And so just in the process of seeing the patients and completing some of those discussions and recommendations, there can be a benefit to our patients. But that's something that needs to be incorporated into the design of our studies in the future.

Dr. Correa:
And if you could go back one-on-one to the many individuals and their families who've helped contribute to your work, what would you say?

Dr. Grunseich:
I have to say thank you. I'm totally appreciative for your commitment, for everything that you've given for our work, and then we can't do it without you. Really, this is definitely a project that involves a lot of participation on behalf of our patients and families, and so we're very grateful for everything that you're doing for us. And hopefully we can give back something meaningful for our patients in the future.

Dr. Correa:
Well, we're thankful for your careful and intentional approach to really move this science forward. And hopefully not just bring improved treatments and strategies for those living with SBMA or Kennedy disease, but all of those living with a variety of neuromuscular, neurodegenerative conditions. Chris, I really appreciate you taking the time with us and our listeners today.

Dr. Grunseich:
Thank you, Daniel. It's a pleasure to speak with you.

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. You can also sign up to receive the Brain & Life magazine for free at brainandlife.org. Don't forget about Brain and Life en Espanol.

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

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

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

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

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

Dr. Correa:
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