The Art of Overcoming: Liz Nugent on Storytelling and Dystonia Part Two

Episode Transcript

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

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

Dr. Correa:
Welcome back. As you might see, this is a part two of our episode that we started last week with Liz Nugent. Make sure, if you didn't get a chance to check out last week's episode, start there, hear some more about Liz's own story and her experience with dystonia. We're going to be continuing that with a very specific situation and a type of treatment that she's had experience with, it happens to be related to brain implants. And how, whether it's from the news about Neuralink, or maybe science fiction shows like Black Mirror and Severance, West World, or the movie Manchurian Candidate, I've heard from different members in the community about concerns related to mind control or recording of thoughts, and our private data through medical devices that interact with the brain and spinal cord.
Katy, have you heard any concerns in these areas, or have thought about it in watching these shows?

Dr. Peters:
It's blowing my mind now. Wow. Nobody has brought this to my attention. I haven't really heard about these concerns. I guess I'm more worried about real life scams, that I guess are a little more low-tech. Those phony emails, or those texts that you get that are phishing for someone's data. I know people that have fallen for those things and they're pretty low-tech. I think what you're talking about has to much more high-tech. I think that when we think about medical devices, that technology really will continue to grow and will continue to challenge us.

Dr. Correa:
Yeah. I think we need to take the community's concerns very seriously as we think of how this technology grows and changes over time in its advances. I think sometimes we're not as immediately concerned with it. I find it's an interesting thought exercise when I think of something like Severance or other shows. I'm often reassuring to friends, family, and other people, and I know watching these shows, we're nowhere near any of that level of concern. The kind of technology that they're thinking up in the science fiction sections is not really the level and the type of interaction and concern that are recording or type of level of detail in connection to the brain as what's communicated in these shows.
But there are many devices in much simpler formats that are very well studied in safety and efficacy that help treat things like epilepsy, various movement disorders, and there's ongoing studies showing benefits for other conditions. Some of these devices have also helped people rehabilitate or overcome impairments from brain and spinal cord injuries. We really want to get you more information about this.
This week, we'll continue last week's episode with Liz Nugent to hear more about her journey with dystonia management and her initial experiences with deep brain stimulation, or DBS, which is one of these types of devices. Then we'll go back to Dr. Alfonso Fasano for his experience in helping counsel patients about these different devices and treatments, and their role in management of different conditions.

Liz Nugent:
There began another saga. In December 2023, I had deep brain stimulation surgery in the London clinic in London. I noticed no difference at all for the first three months. They were playing around with various settings, you know this remote control you get with the ... Do you want to explain with the DBS thing is?

Dr. Correa:
For our listeners, this is where electrodes with a small generating device are placed in the area that controls or is related to the abnormal network that leads to the dystonia. Then it causes a stimulation to help decrease the response that's leading to the dystonia. We're going to get into more detail about deep brain stimulation and some of the treatments for dystonia with our medical expert following this.

Liz Nugent:
Okay. It's very minor brain surgery. It doesn't hurt at all because there's no nerve endings in the brain. I woke up the next day, and I think I was given aspirin. I was expecting to go on an Oxy holiday, but I think I got aspirin. Yeah, I was fine. It didn't hurt at all.
For the first couple of months while they played around with the settings, I noticed no difference at all. Then, that was December, and it was January really when it was turned on. Around April, I started to notice that my arm was going into spasm, my right arm. They tell me I was an unusual case, but they told me that the surgeon had targeted exactly the right place, with .01 of a millimeter of the target area. He had got that so precise. He said I was his "finest work."
Unfortunately for me, whatever settings it was on, it set off a series of spasms in my right arm so I completely lost the use of my right arm. Beforehand, I couldn't type with my right hand, but I could use a knife and fork, I could do up a zip, I could do up my bra. I could lift things, I could carry things. But at this point, I just completely ... This is last year, from April to August, September, my right arm was in spasm, painful spasm all of the time, 90% of the time.
Then there was another form of Botox that had come on the market called Xeomin. My doctor said, "Look, let's just try this." He tried it and the spasms released and they went away, and went back to the way I was before. Only, that I hadn't had physiotherapy because they asked me not to have physiotherapy for the year after I'd had the DBS because they wanted to make sure that if there were improvements, that they could put it down to the DBS and nothing else. I was a year without physiotherapy and no improvement from the DBS.
Now I'm a little bit worse than I was before I had the surgery. I'm not saying this happens for everybody. For a lot of people with dystonia, DBS is a life-saver and a life-changer, and particularly for those who are born with it. I have it as an acquired brain injury. It's never a good thing when your doctor says, "You're a very complicated case." I was a very complicated case.

Dr. Correa:
Since then, they've still tried other settings and right now, it's not at a point where it's working for you?

Liz Nugent:
Yeah. Nothing has improved it so far. We're a year-and-a-half now past the surgery. I'm thinking it's not going to work.

Dr. Correa:
But now that you've gotten some response with that new version of the Botox and you're back to your physical therapy?

Liz Nugent:
The Xeomin only worked for that time.

Dr. Correa:
Ah.

Liz Nugent:
It's never worked since on any of the spasms in the other parts of my body. It's never worked since. By some miracle, it worked that one time. In my bicep, and my forearm, and my hand, and in my shoulder, it worked. But since then, it hasn't worked. I often walk with my right foot postured in the air, so I'm walking on my heel. I'm not walking on my whole foot. They've tried injecting it into my shin to see if it would prevent that, but it made no difference. Then they tried a higher dose, and they were scared of trying too high a dose because you can get a dropped foot from that. But even with the higher dose, it made no difference whatsoever. I'm back to square one.

Dr. Correa:
Yeah.

Liz Nugent:
Luckily, my mental health is, despite my time in the psychiatric unit, my mental health is fairly robust and I'm an optimistic person. I make a living with my writing. I'm very fortunate to be married to a very nice man. I have a good life. I could be a whole lot worse.

Dr. Correa:
Yeah. You started off by describing a lot more about the physical challenges of dystonia, and you were just speaking to your own mental health and resilience, but those emotional and psychological aspects of the experience with a pain disorder or muscle disorder like dystonia can be quite a significant aspect. What do you think along the way helped support you in developing those skills to cope with the pain issues and the emotional challenges?

Liz Nugent:
I just kept thinking, somebody said to me once years ago, "Would you swap your disability for somebody else's?" Would you swap your disability for blindness, or deafness, or with somebody who had MS, or somebody who had some other neurological disorder? It's the devil I know. Dystonia, this condition that I have, I know how to negotiate it. I know that I can't walk very far. I know that if I'm going somewhere where there's stairs that I have to make sure there's a lift in the building. I can do stairs, but very awkwardly. Because of my books, I'm often interviewed and I always ask, "Please don't put me on a high stool." I ask for a chair that's not too deep because I can just slide backwards into the chair.
Oh, sorry. I'm having a spasm as I'm speaking to you. At the moment, my knee is the biggest problem because I have no cartilage left in my right knee. I can't have a knee replacement surgery because of the dystonia, because it would just go into crazy spasms and I wouldn't be able to withstand the physiotherapy required afterwards. It's bone on bone pain. That means I'm limited in the distance that I can walk.
My arm now doesn't bother me really because it's not obvious. When people see me they don't think, "Oh, what's happened to your arm?" They go, "Oh, what's happened to your leg?" My arm doesn't really bother me because I'm back to being able to lift things, and use a knife and fork, and do all the normal things. If I was sitting across the table from you, you wouldn't notice that there was something wrong with me, only when I stood up to go somewhere. Often if I'm meeting people for the first time, I'll make sure I get there early and I'm sitting down so that they will take me at face value, rather than see that I have a disability.
I recently have had to resort to a crutch from time to time, or walking stick. I guess that's in my future. Maybe a wheelchair is in my future. I don't like to think about that too much. But yeah, I'm looking at my beautiful house and thinking I may have to move to a bungalow, a house all on one level. That's for future me, future Liz to worry about.

Dr. Correa:
Well, it's heartening to think and to hear that you're really thinking about taking care of yourself and what you need now, but also thinking a little bit ahead towards future Liz.

Liz Nugent:
Yeah.

Dr. Correa:
Yeah.

Liz Nugent:
Yeah, a friend did tell me, "If you think you're going to end up disabled, get all that in place well before you think you need it because you don't want to wait until the day where you cannot get up the stairs before you need to do something." Actually, my 92-year-old mother is at that stage as well now. She's refusing to move out of her house. The kitchen is downstairs, the bathroom and the bedroom are upstairs. She's living on three levels and she can barely walk, but she's absolutely refusing any talk of moving and she lives by herself. She won't have carers in the house so we're doing a lot of it. But at the moment, I'm not actually because I can't get up the two flights of stairs to her front door.

Dr. Correa:
Wow, yeah. So many unique challenges that each person can experience.
I was thinking just now about your writing. I think these are the characters of your life. I'm wondering how has your own experience with dystonia influenced the way you write characters into your storylines, and the themes of control, or vulnerability, or resilience for some of your novels?

Liz Nugent:
My third book Skin Deep dealt a lot with facial disfigurement, and how noticeable that was, and how self-conscious it makes you. I have never written a character with a limp because everybody would know that that was based on me. I can't really put myself into a book, especially if I'm the bad person. They'll know it's me.
All my characters are very ... Well, apart from Sally Diamond, all of my main characters are either psychopaths or sociopaths. Yeah. Sally Diamond herself has neurodivergent issues. What does Oliver have? Oliver has abandonment issues. They all have something, all of the characters have something. I have another character in Unraveling Oliver who has a port wine stain on one side of his face. Yeah, facial disfigurement actually comes up in two characters. There's another character with Down's syndrome in one of the books. That's Unraveling Oliver as well.
If I write a character with a limp, everybody's going to know it's based on me.

Dr. Correa:
Yeah.

Liz Nugent:
I don't appear in the books at all. I'm not a character in one of my books. I have to be careful about that. But I always talk very highly of nurses and doctors, and the people who working in the healthcare services who ... I have Facebook friends now who were cleaners in the hospitals that I was in. Catering staff, and doctors, and nurses.
I've made very good friends with a girl. I ended up in hospital in 2019, I was in a ward with a girl who had MS. At the time, she was really bad. She couldn't use her hands, she was doubly incontinent, she was blind in one eye, and she was deteriorating quite badly. But they gave her an infusion of something, I don't know what MS infusions are, and she started to recover. When I went to the rehab hospital, about two days after I got there, she arrived into the same ward. I watched her recover. I watched her go from somebody who wasn't able to do anything to somebody who I visit now, who I go to her house, and she's walking around and looking after her two children. It's incredible what science has done for her. It's incredible. It's a fantastic recovery story.
Yeah, I've made some good friends through my journey. I'm going for dinner with my retired neurologist Michael Hutchinson shortly and his wife. We've become good friends because we both like classical music and we both go to the Kilkenny Arts Festival in Ireland every year in the southeast of the country. We also meet up there every year. This year we said, "Let's have dinner." Yeah, me and my husband are going with him and his wife. That should be fun.

Dr. Correa:
Well, I'm glad to hear so much of both your own progress, and how you've had the opportunity to see how so many others have been really able to find a new way and adapt with their own experiences and challenges.

Liz Nugent:
Yeah.

Dr. Correa:
It sounds like in a way, you explore or process some of those challenges with your books and the characters, just in a different image.

Liz Nugent:
I talk about physical pain a lot, but it's more mental torture that I deal with.

Dr. Correa:
Exploring the creativity behind it.

Liz Nugent:
Yeah.

Dr. Correa:
It was something I found interesting. I write your books often out of a chronological order. How do you see the pieces of the puzzle in the timeline of these universes coming together? Is it just by inspiration?

Liz Nugent:
Yeah. I think [Our] Little Cruelties starts in 1990, and then it goes back to the next chapter is 1973, and then the next chapter is 1992. It's completely out of order, but I wanted to build up a picture, a snapshot of three brothers who grow up together in the same house with the same parents, the same education, who grew up to be entirely different characters. I just decided that the best way to do that was by just taking a snapshot of their lives in each year from each one's point of view. Some of the stories overlap, so you're getting both sides of the story of what happened the day they went to see the Bob Dylan concert. Or what happened the day they went to the Cannes Film Festival because one of them is a film producer. One of the other brothers is a former pop star now actor.
Yeah, the chronological thing, it just worked for that book because I had to build up the enmity between them so that you would see, by the denouement of the book when one of the brothers murders one of the other brothers, I've built up the enmity between them. Because the first line of the book is, "All three of the Drumm brothers, but one of us was in the coffin." You know that one of them is dead, but you don't know which one and you don't know which one has killed him. I had to build up the rivalry and the enmity between the brothers and that's how I did it, just taking a little chapter in each life where the small things, the small cruelties became bigger cruelties, and betrayals. And sleeping with each other's wives, and having affairs. Just awful. One brother ripping off another brother. Just lots of reasons for them all to hate each other enough to kill each other. At the end, you're waiting to find out which one is dead.

Dr. Correa:
It's great storylines. Thank you for sharing some of the chapters of your own story. We really appreciate you connecting with us and our listeners and sharing your own experience with dystonia.
Now in a moment, we'll be going to our medical expert discussion to talk more about the treatment, and management, and deep brain stimulation and the other options there are for dystonia.
Thank you, Liz, so much.

Liz Nugent:
Thank you. Very appreciate this. Thank you.

Dr. Correa:
Want to learn more about the conditions discussed in this episode and other factors that could impact your brain health? For the latest on causes, symptoms, diagnosis, treatment, and management of more than 250 of some of the most common and rare neurologic conditions, please visit brainandlife.org/disorders.
Now, the surgeon that took care of Liz Nugent in the UK and placed her DBS told her he got it exactly where it needed to be. Yet, after the initial processes so far and the months, she's not really been having a response and improvement. She's described that she's in a watch and wait phase at this point, and they're working on modulating and adjustments. Can you explain why this occurs and how to adjust or set expectations after DBS is placed?

Dr. Fasano:
Yes, absolutely. This goes back to my earlier point, education and managing expectations, which is very human. As a person going through a procedure, everybody will hope to get the most out of it because it's not an easy decision to being operated with the insertion of device in your head. Putting myself in a patient's shoes, I will thoroughly trying to picture of this in my mind the best possible outcome even to get the courage to go through it. But expectations need to be managed.
Now, acquired dystonia, dystonia coming from a brain lesion, a trauma, a hemorrhage, are the most difficult to treat with DBS. That's because first of all, there's nobody like this person in the world, so you cannot go by what's been done by others because everybody has their own lesion in a different shape and a different moment in life. There's not another brain like someone with a lesion causing dystonia. The same happens, for example, in multiple sclerosis. The combination of other lesions, what happened to the brain throughout the life. That's why it's quite difficult to predict the outcome.
Usually, the outcome however can be predicted somehow in these cases. We usually say that if there is mainly dystonia determining problem with functioning and quality of life, then the outcome of surgery will be more positive. As I said before, the weakness is not going to improve. The spasticity is not going to improve. Now, weakness is easy to diagnose, but spasticity often is together with dystonia, and it's difficult really to disentangle the two. If the trauma caused mainly spasticity that was mistaken for being mainly dystonia, then DBS is not going to help.
Another thing that we go by is the movements. If a person has a lot of movement, tremor or involuntary movements that look like a dance, we call this kinesia, that is a good sign. It means that DBS will help. As opposed to a fixed posture. A fixed posture is more difficult to be helped, also because a fixed posture can come from spasticity, but it can also come from those contractors that I was telling your earlier. If someone is having a problem for 30 years, by the time something is done, the body itself has taken that particular shape. Changing the brain activity is not going to change the bone, the tendon, and so forth.
To complicate the issue is also that, in dystonia, whatever we do when we program doesn't translate into an improvement immediately. Dystonia is a slow disease and therefore when we do adjustments, we need to wait sometimes weeks or even months to see an effect which complicates the programming. Programming at this stage is still a trial and error approach. We try something and we need to wait. My patients, for example, are given homework. I give them a remote control with different programs. They go home and they try one for one week, another one for another week. They take notes, they take videos. They come back to see me a month later, we go over their notes and we say, "Okay, this seems better than this other program, let's continue with this. Do this adjustment," and the process continues. It might take even a year to find the right parameters.
Last, but not least, I want to just end with this. The electrode needs to be in the right place. Now, I understand that this is summing it up in this case. But being operated is not like taking a pill because if the electrode is slightly misplaced, it's like you never got DBS. Yes, in theory you had DBS, but the electrode is not where it's supposed to be and that's why you don't get any benefit from it.

Dr. Correa:
Wow. It sounds like it is a deep discussion and relationship to build together with not only in the neurosurgeon who's placing it, but then the neurologist who's going to be managing it with you. Things can take time and there can be lots of reasons for each individual.
Now, you've talked already and mentioned about adaptive DBS as potentially a new direction in various situations. Like in epilepsy, these recording of these intercranial EEG or these intercranial electrodes is helping us learn and modulate a lot more of our opportunities and options. I'd love to hear from you, what do you think are going to be some other advances in the research and treatment that you're excited about for those living with dystonia?

Dr. Fasano:
Yes. That's a one million dollar question. I can say that dystonia is just a label that we give to a very complex condition. For example, when I was a trainee, when I was a resident even, we only knew of one gene. Now, one gene actually quite common in people with dystonia living in New York. This gene is called Tor-1A and that was it. This was really a breakthrough because back then, we could not really understand if dystonia was a genetic condition. But now, the list of genes causing dystonia, I can think of 20 genes or more.
Now with this, I want to say two things. The first is that for particular individuals, we know what's happening. We know what's happened to their brain and why they have dystonia. This might not translate into anything differently done now, but gene therapies are used more and more. Or also, more specific drugs that target particular parts of the brain affected by the mutation. For certain individual, in the near future we'll know exactly what's happening and we'll most likely have a more tailored treatment.
But that's not it, and that's the second message. In any case, these genes, even if we're dealing with someone with no mutation of any of the known genes are telling us what's dysfunctioning in dystonia, and perhaps they're opening new avenues of intervention. I realize I haven't given you a clear answer, but I can just say the more we know where dystonia comes from and how it's produced in the brain, the closer we get to more that is not just something symptomatic, something that just masks the symptoms, but it's something that goes deep down into the mechanism of the disease and therefore changes the natural course of the process.

Dr. Correa:
It sounds like then one of the most significant things that could help to advance research and treatment is if you know someone or are living with a dystonia, that you engage and connect with a movement disorder center because they may be able to involve you in some of the many research opportunities to help the science move forward.

Dr. Fasano:
Yeah. In this case, for people listening and wondering, "Oh my God, where do I find a dystonia specialist?" Usually, it is a movement disorder specialist. It's not difficult, you just need to look for specialists in Parkinson's. Parkinson's is the most common of the movement disorders. If you come across someone that knows Parkinson's, chances are that this person will also know dystonia or is working in a center where there's a dystonia expert. That's probably the easiest path. I can also mention dystonia associations, patients associations. Dystonia Medical Research Foundation has done a lot for dystonia, research in dystonia. We live in a different era where people are connected. You go on the internet and you can find these associations.
Be careful going on the internet, though. I guess I'm stating the obvious, but there is a lot of noise and it's difficult sometimes to discern the real information. Always good to go to the dystonia coalitions or dystonia medical foundations, or website of known entities rather than just random people on the web. I also know that patients nowadays are very well-connected through social media, which is good. But it's also something dangerous because there's a lot of misinformation that can be available unfortunately in this media. Always, it's good to share stories and to be connected with a large community because people dystonia think they are unique. Instead, they realize there are many people suffering from the same problems. But it's also important to recognize that you are not talking to a healthcare professional, so don't follow blindly what Mr. Smith from the middle of some state is telling you what to do. They might be right, but always good to go back to your neurologist and discuss options with them.

Dr. Correa:
Yes. For our listeners, we'll list some of the US-based patient advocacy organizations for those living with dystonia in our show notes.
Lastly, I just wanted to hear for fun, since you commute per se between Milan in Italy, and Canada there in Toronto, do you see a difference in those living with dystonia between the two communities in Canada and Italy?

Dr. Fasano:
Oh, thank you for the question. Yes, I have a role also in Humanitas University in Milan. That's why I tend to spend time in Milan. I'm Italian, so it's actually nice to be connected in my own country. I'm Canadian too, but primarily Italian as my accent also testifies. Also, throughout my career, traveling a lot for conferences and meeting people really from any corner of the world, I can say that deep down, humans are the same everywhere. Especially when challenged by diseases, the questions, the values, the aspirations, the fears, the hopes are the same everywhere which is the reason why anybody in science doesn't understand politics, doesn't understand wars, because we are all the same. I know this might sound cheesy, but through my experience, and of course in dystonia is the same, speaking to a lot of people with these problems, I can recognize that we are really part of one big family.

Dr. Correa:
Thank you so much, Alfonso, for joining us and sharing everything that you have and your time with our listeners to talk more about dystonia, its management, and some more details specifically about the unique treatment of DBS.

Dr. Fasano:
Thank you. Thanks for having me, and for giving so much importance to this condition that clearly needs more attention also by the lay public. A lot more needs to be done, but for sure you guys are doing an excellent job.

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 Brain & Life Magazine for free at brainandlife.org.

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. You can follow Katy and me and the Brain & Life Magazine on any of your preferred social media channels. We're your hosts, Dr. Daniel Correa connecting with you from New York City and online @neurodrcorrea.

Dr. Peters:
And Dr. Katy Peters joining you from Durham, North Carolina, and online @katypetersmdphd.

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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The Art of Overcoming: Liz Nugent on Storytelling and Dystonia Part Two

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