Ashley Brooks on Advocating for Herself and Others with Myasthenia Gravis

Episode Transcript

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Dr. Daniel Correa:
From the American Academy of Neurology. I'm Dr. Daniel Correa. This is the Brain & Life podcast. We are deep in summer. In this episode, we're going to be including some activity and movement cues. We've heard from a lot of listeners that they like to listen to the podcast while going on a walk or doing their other physical activity. Shout out to my mom. And we would like to now include a few cues in some of our episodes for those who want to increase their activity levels or consider listening to the podcast during an activity.
Recently, this summer, in June, we just published a special issue of the Brain & Life magazine on myasthenia gravis or MG, and our guest was featured in a section of the magazine that highlights community members. Ashley Brooks has been working to advocate for myasthenia gravis to increase understanding about myasthenia and reduce the stigma of living with this and other chronic diseases. In the coming weeks, you'll hear an interview with one of our guest hosts and the associate editor of the Brain & Life magazine, Dr. Sarah Song, who speaks with Dr. Roy Hamilton about agnosia. So, a term I'm sure I've mentioned a few times now in lead up and teases for episodes, but you'll be hearing more about it and how we interpret the whole world around this.
Next, you'll hear about the experiences of families who have a loved one with a rare epilepsy called SYNGAP1. So, make sure to catch these upcoming episodes and not miss them by subscribing to this podcast wherever you're listening. Now, if you're in a place where you can get up for a walk or start an activity and you want to explore that with us, and if you haven't already started, join me in some of our walking listeners on a walk or your activity as we start with our interview. In the first few minutes of any activity, make sure to take it slow, listen to your body, warm up the muscles and joints. After the first five minutes of the episode, we'll take a quick break in to cue you to increase your activity level.
Welcome back to the Brain & Life podcast. Today, I'm joined by Ashley Brooks. She not only works as a respiratory therapist and a healthcare colleague, but also lives with myasthenia gravis, an autoimmune disorder that affects the chemical signals between the nerves and the muscles in the body. Since her diagnosis, she has dedicated her life to raising awareness about living with the rare disease and helping other people who are living with this same illness to find strength. Thank you, Ashley, for joining us here on the podcast today.

Ashley Brooks:
Hello everybody, and thank you so much, Daniel, for having me today.

Dr. Daniel Correa:
I wanted to hear a little bit more about your life and what were you doing back in 2017 before all this changed?

Ashley Brooks:
Yes. So, let's go back to 2017. I was actually a junior in college. I attended Georgia State University, so I was downtown in the middle of the city just basically trying to get ready to enter my career field. I was studying to become a respiratory therapist. So, being in the healthcare field, my senses are always on a hundred. I'm always trying to see, "Am I feeling okay? What's going on with myself?" So, I started noticing little small things at the beginning of the year, 2017, January, my eyes at the end of the day, they would just be really, really low. I'd wake up in the morning and it would be gone and we'd be like, "Okay, maybe we were just really, really tired or something." But that kept going on.
And finally, around March, my birthday, we went out to eat and we got steak. I love a good steak. I had my steak medium. I could not chew my steak, and it was just really hard for me to chew and swallow my food. And I just started to notice at the end of my days, I was on the phone with my sister talking and they could not understand me at all. So, I finally broke down and went to the doctor and told them what was going on. And I told the doctor, "Hey, these are my symptoms." And I'm in school for this and we learned about this maybe a month ago, this disease called myasthenia gravis, but this is what I think I have. And they ran the test, and sure enough, it came back that I had MG.

Dr. Daniel Correa:
So, from day one already you had spent the time thinking about it and you had an idea that what might be going on?

Ashley Brooks:
Yes, because the symptoms were so specific, the ptosis, the droopy eyelids, the slurred speech. It was very weird because in the morning when I woke up, everything would be back to normal. I would be back at my normal strength when I woke up. But at the end of the day, I could not do much of anything. I couldn't walk up the stairs.

Dr. Daniel Correa:
But it took a few months of having these symptoms and then learning about it in class and realizing, "Oh my gosh, these are my symptoms."

Ashley Brooks:
Also, being a 20 something year old busy in college, after I noticed the symptoms, I did go into the doctor. They had been calling me for few weeks, months after I went to the doctor in March, they'd been calling me. They didn't get a hold of me until the end of April.

Dr. Daniel Correa:
What were you doing that was making you so busy that you thought maybe you were just too tired from your schedule?

Ashley Brooks:
I was a junior, so when you're doing healthcare, you have to do your clinical rotations. So, on Tuesdays and Thursdays, I was in the hospital from 7:00 A.M. until 4:00 and then, I was still full-time student and I'm also was pledging to be in a sorority. I was working two jobs. I had my own organization, nonprofit organization I was running at that time. So, I had quite a few things that I was dealing with. So yeah, I missed all of my doctor's phone calls. Yeah.

Dr. Daniel Correa:
Okay. You were running things already?

Ashley Brooks:
Yeah, pretty much, yes.

Dr. Daniel Correa:
And what do you think held you back from just making those appointments? Just your schedule or was there some already a sense of a little bit of worry and fear about having something and dealing with it?

Ashley Brooks:
I think more so I was just putting it off. The doctor seemed very concerned when she was going through my symptoms and she was just like, "Oh no, we need to do all these tests and blood work." So, it did freak me out a little. So, when I left that appointment, I was kind of nervous, but she also told me, she was like, "Don't worry, you are young to have MG." So, I did leave the appointment thinking, "Okay, don't worry about it." And I kind of just put it in the back of my mind. I didn't want to deal with it to be honest. Yeah.

Dr. Daniel Correa:
And at this stage, you came in with the weakness that would come on after activity or fatigable weakness. Had you had any issues with breathing or respiratory distress?

Ashley Brooks:
So, for me, I did not have any initially. It was more so my limbs, and even now to this day, my limbs and my muscles for talking, speaking, chewing, swallowing, those were affected. I was choking on my spit, but I wasn't having issues with shortness of breath. The only time I noticed having respiratory issues was when active myasthenic crisis is when I noticed respiratory issues.

Dr. Daniel Correa:
Let's take a quick break and check in. If you're feeling good, feeling warmer, the joints are moving, then now would be a good time to try and increase your speed or your activity level. The total minutes of activity level for your brain health should be around an effort level where you're just a little out of breath. It could be hard to keep a conversation or finish a few sentences. Over the week, try and see if you can add up to 150 minutes of this type of activity. You can do this with just 20 minutes a day. If you do a walk or other activity during the full podcast, you'll already be nearly halfway to your weekly minimum goal.
Right off the bat, when you started talking with your doctors about this diagnosis, did they talk to you some about respiratory and myasthenic crisis and was that something that helped you understand it or was that kind of shocking and concerning right early on?

Ashley Brooks:
So, immediately after they did the blood work and it came back positive for that specific neurotransmitter receptor they're looking for, they referred me to a neurologist.

Dr. Daniel Correa:
How was that discussion when you were starting talking about initial treatments and with the options? I mean because with myasthenia, many other autoimmune conditions, there can be a lot of different initial options and a lot of side effects to consider.

Ashley Brooks:
Oh, for sure. I was really scared. Most autoimmune diseases, they start off with the steroids, the prednisone, and me being that I was in healthcare, I've seen my patients on steroids and see the change in the weight, the appetite, and some of the other side effects with their bones. So, I was really scared and I told him, I was like, "Look, I don't want to be on steroid." But my neurologist was like, I mean it's necessary right now to get your symptoms managed." So, my initial treatments were 60 milligrams of prednisone steroids, also another infusion medication called IVIG or immunoglobulin, Mestinon, and also azathioprine.

Dr. Daniel Correa:
The steroids also help reduce some of the initial response and inflammation and the autoimmune process and hopefully get you over to some of the other medications. But yes, there's always the challenge of the side effect issues, and really oftentimes, the other medicines hopefully help you reduce or get off those steroids and move to something else in the future. And the Mestinon you mentioned, is that medication that tries to replace that chemical that's being depleted in your body?

Ashley Brooks:
Yes. I always refer to that as my Band-Aid because it'll help me for my little momentary weakness, but not a cure.

Dr. Daniel Correa:
And then, you mentioned the azathioprine, that and some of the other medications are, yes, they were originally designed often as chemotherapy medications, but they're medicines that also help you reduce the dose of the steroid that you're on and stops the autoimmune process or slow down the autoimmune process that's going on. So, that sounds like you've made the transition that is very similar to many other patients living with myasthenia. Each person's severity of illness is their own and will be their own journey, but those are often a lot of the steps that need to be taken. Now, some people, they also get pictures taken of their chest to check out their thymus. Did you end up having that done and how was that discussion with your doctor?

Ashley Brooks:
Yes, so they did another chest x-ray to see if the thymus gland was enlarged. I didn't actually have any, it wasn't enlarged. I mean it was probably slightly, but because I was very adamant to my doctors and my care team about, I was 22 years old when I was diagnosed, I was, in my mind, thinking long term about my medications. Like I said, I didn't want to be on the steroids and I wanted to eventually have kids and just worrying about other things. So, my doctors have kind of set up a plan with me so I could wean myself off of steroids.
But it did include me having to get the open chest surgery, the thymectomy, they thought that if they were more aggressive and did remove my thymus because they felt like that would help my immune system response. So, I did end up in December of 2017 getting my thymus removed, so they did a thymectomy. So, I did get a sternotomy and plates in my chest.

Dr. Daniel Correa:
It is a significant surgery, but it's an important one that sometimes it should be considered for people living with myasthenia. And we're going to make sure to go into a lot more detail to explain what that is and why it helps and for people in our medical expert portion of the interview. Tell us, Ashley, where are you now with your treatment and how do you consider yourself with the condition? Do you feel like you're cured? Are you in remission? Is it really more of a chronic management situation? How do you consider these terms that are out there?

Ashley Brooks:
Even though I do have good days, I don't consider myself to be in remission. I just think that my symptoms are more manageable. I have a better understanding of my limitation and my triggers now for my symptoms, my MG symptoms. So, I'm able to live within a certain means so I don't aggravate them as much and I'm not like uncomfortable. Currently, I am no longer on the steroids. I no longer take Mestinon. I am on a new treatment that's FDA approved that's called Ultomiris. So, I am on that and I just had my two loading doses, so I can't tell you much how, but so far so good. I would say I've made some pretty good strides. I'm off the steroids. That was a goal at 2017 and I got off of them and I only go back on them unless I'm sick.

Dr. Daniel Correa:
And this all came up while you were in college as you say, that time when we're all there with peers, both having fun and aspiring to many goals. How did you deal with the mental health impact and the self-esteem impacts from the condition while you were in college? And what have you been doing to help manage it since?

Ashley Brooks:
It was so overwhelming. The first thing I did was after I got that call or we went in person, they were like, "This is what you have." I reached out to the mental health facility on campus at my school because I knew for a fact, "Okay, I can do this, I can go to the counseling facility." But they do an intake where they have you speak to someone. And again, I was just really fortunate after I explained to the intake therapist what was going on and how it was just, I guess, having a pretty significant life change. They were able to get me in with a therapist twice a week until I graduated.
I went through all the stages, grieving, denial. I went through that with my therapist and I always recommend people when they're first newly diagnosed, you should just get some help, mental health wise, it was everything. And I think because early on, I reached out for help and was honest with myself.

Dr. Daniel Correa:
I mean, that's great that you were able to connect so quickly to someone and then have that support through already what was it incredibly challenging time in your life with all the transitions that you were working on. And then on top of that, to have a new chronic medical condition and diagnosis. We really encourage, especially with major changes in life, it's just getting involved and seeking support right off the bat, even if you're not noticing issues with depression, anxiety, or even just grieving. And you mentioned your website. So, in the course of getting your diagnosis, you started a blog and website with community information about myasthenia, but what information and messaging did you feel was missing when you were looking for this information?

Ashley Brooks:
Yeah, so when I typed in myasthenia gravis, I understand it was rare, but I just would see general information like, "Oh, this is a rare disease." But I didn't see anyone who looked like me. I didn't see any black women who could give me their experience, "Okay, I see what the NIH says this is." Or "I see what WebMD says this is," just to understand what the disease is. But I wanted a real person's perspective. I wanted to know are they still living life? How are they functioning? But I could not find any information and it was just very weird. So, I thought to create a website where I can make people aware, you would look at me and you would probably think like, "Oh yeah, she doesn't have an autoimmune disease."
But I think the beauty of it is you can't really tell or look at someone and tell if they have an illness, a disability. I wanted that to be what the message was. I love makeup, I love beauty related things, do reviews. But I also wanted to educate people on how it is for me, a 20-something year old living with this rare condition so people can maybe understand, have more respect for it. I have autoimmune disease, this is how life looks.
So, that's what the purpose of the website was. Like, you can still live and this is how life could look or it looks for me. I get emails from people all over the world. My friend from Romania, she's like, "I saw your website, I was just diagnosed, but seeing your website let me know that I could still live my life." That's everything to me because that was the goal. You're still going to live your life.

Dr. Daniel Correa:
Are there misconceptions that you found online that you felt like you just needed to address and explain further not only on how it relates to you, but for the community?

Ashley Brooks:
With MG, just in general, people don't understand it's the physical manifestation of my limits, basically. A lot of people didn't understand here's the blog and it outlines in an easy way, not a bunch of medical terms, layman's terms of how this disease affects my life and how it is because it could be confusing for some people. They'd be like, "The messages aren't communicating." And I'm like, "If I move my hand too much, if I talk too long, my mouth would stop moving. My hand is going to stop." No matter how much I want to force my voluntary muscle to do something, it won't. And I wanted people to understand that because it was confusing even for my mom. It was like, I can't positively think my way or sheer willed, make myself, it just will not.

Dr. Daniel Correa:
What do you think changed in your goals and expectations for your life and for yourself?

Ashley Brooks:
I wanted to make money and have titles. I wanted to be a respiratory therapist eventually. I was putting more emphasis on doing career jobs, whatever that are like within my means that aren't forcing me. When I was working as a respiratory therapist, I would push myself so hard and I believe that is partly why I spent a lot of my initial first years being diagnosed in the hospital so much and in the emergency room, ambulance rides, because I was still trying to get to the certain level, obtain this functionality. I was just making myself sicker. So, now, I put emphasis on, yeah, I would love a career that I enjoy, but I put more emphasis on being comfortable.
So, yeah, I'm putting emphasis on being comfortable, happy Ashley not, "Oh yeah, I'm out here doing this, that, and the third. And a lot of people, I think, are focused on titles and being perceived by society to be super busy and productive.

Dr. Daniel Correa:
Now, we're back again for a quick activity check in. If you're on a walk, jog, bike ride, we are at the halfway point now. If it's right for you, it might be time to turn around and start your way back. If you are planning an activity as a one-way trip to the grocery store or another destination, then let's keep going. So, you're present now and taking care of yourself.

Ashley Brooks:
Yes.

Dr. Daniel Correa:
Now, I want to go back though then to the day you were diagnosed. What advice or support would you give to yourself or to someone else or maybe even to your mother or family member of someone diagnosed at that time?

Ashley Brooks:
I would just probably say just give yourself some grace. And that sounds cliche, but it's something I always have to remind myself. Giving yourself grace, being honest with yourself about your limitations, your boundaries. It's okay to say no. You can cut yourself some slack and just listen to your body. That's going to be a big one. Listening to your body and being honest with yourself. Yeah, it took me a while.

Dr. Daniel Correa:
And is there any way that you feel like it's important that family members and friends help support someone who lives with MG?

Ashley Brooks:
Oh, yeah. I would say your support system is everything. I have a pretty solid one. My mom and my sister, they're just like a sounding board, reminding me when I'm doing too much, my boundaries. I know I may seem like I can able to advocate for myself, but sometimes I do have a tough time. If I'm in the presence of my doctor or nurse, it gets intimidating. Even if I'm a person in healthcare, it's sometimes hard to speak up for yourself, but your support system, that's what they're there for.

Dr. Daniel Correa:
Oh, Ashley, I want to honor and thank you for spending your time and energy with us today. I know it's precious, especially for you.

Ashley Brooks:
Yes, yes, yes. No, absolutely. It was my pleasure. Thank you so much.

Dr. Daniel Correa:
Is this episode leaving you wanting more? Get the latest tips on healthy living and management for more than 250 neurologic conditions by visiting brainandlife.org where you can learn more about neurology every day powered by trusted neurologists.
Welcome back to the Brain & Life podcast. So, now, after that discussion with Ashley Brooks, I'm joined by Dr. Gordon Smith, a professor and chair of neurology at Virginia Commonwealth University. He's an internationally recognized expert in the diagnosis and treatment of nerve and muscle disorders, and he's been a leader in multiple clinical research trials for various nerve disorders. Today, we are talking with Gordon about supporting the quality of life of people living with myasthenia gravis and some of the new treatments. Thank you so much for taking the time to join us.

Dr. Gordon Smith:
Daniel, I'm very excited for the invitation to be able to talk with you today.

Dr. Daniel Correa:
In our past episode with Suzanne Rogers and our expert discussion with Dr. Nicholas Sylvestri. We heard some about the basics about what is myasthenia. But can you recap for us how you explain myasthenia gravis to other people who are new to the condition?

Dr. Gordon Smith:
Sure, Daniel. It's a complicated thing to explain, so I'll go through how I try to make this digestible. So, myasthenia gravis is an autoimmune disorder. And so, this is disorder where your immune system gets confused and rather than attacking a foreign invader, a virus, or a bacteria, it attacks your body. And in this case, it attacks the receptors on the muscle membrane that receive the nerve signal that triggers movement. And I think of the metaphor of satellite television. I used to live up in the mountains and had a satellite dish. And the way the nerve communicates to the muscle is by releasing a chemical called acetylcholine that floats over and binds to essentially a satellite dish on the surface of the muscle.
And so, when this happens, a couple of things occur. One, the signal can't get through because it's being blocked by this antibody. And so that would be like having snow in your satellite dish. The signal doesn't get through. The other thing that happens is the antibodies that your body makes against the acetylcholine receptor, that satellite dish on the muscle actually triggers damage. And I'm sure we'll talk more about how that occurs because it informs the new therapies we have, which are very exciting. But this would be the equivalent of not only snow in your satellite dish, but someone ripping the satellite dish off the roof of your house and actually poking a hole in your roof.
And so, this obviously causes muscle weakness and it causes a lot of fatigue for patients. And the thing that I think is particularly perplexing is the variability in symptoms between different patients with myasthenia gravis. Most patients initially will have weakness in eye muscles, so drooping eyelids or double vision, but most patients probably 60% over a couple of years will progress to have weakness of other muscles, and that can be speech and swallowing. So, slurred speech or choking on foods or weakness in their limb muscles, trouble working over their head or walking or even difficulty with breathing.

Dr. Daniel Correa:
When Ashley was in college, she was at that time starting to learn and start her initial training as a respiratory therapist working hard multiple jobs. Initially, her symptoms started. She had some of that fatigue and occasionally would have some episodes of blurred vision or double vision. She explained it away, of course, because she was working so hard and not sleeping well. And initially, when she really started to have the more significant symptoms, it was unique that it was only a few weeks after her first course where she learned about myasthenia and almost started that diagnosis on herself.
And so, her symptoms included double vision, blurred vision, a weakened smile, slurred speech, and eventually, some right facial droop. But as you talked about, there's lots of other symptoms that come along with myasthenia. What is it beyond weakness that many people live with?

Dr. Gordon Smith:
Well, the fatigue is important, and as neurologists, Daniel, we're taught that fatigue in myasthenia gravis is fatiguability. In other words, the phenomena where with repeated movement of a muscle, it becomes weaker. That kind of fatigue patients appreciate as weakness, but it's also common that patients with myasthenia gravis have true fatigue, just exhaustion associated with their disease. And then, there are other aspects to this that it can be particularly troublesome.
For instance, many patients have a great deal of difficulty tolerating heat. There are reasons for that biologically, but the fatigue can be much worse and the heat and the ability to get things done in the heat can be problematic. It's actually the only condition where I've ever written a prescription for an air conditioner in my career.

Dr. Daniel Correa:
Ashley, when she was learning and was already working in a respiratory therapy program when she started to have symptoms and have some awareness of what might be going on, she luckily was able to get to a neurologist and to get to more diagnostic and early treatment very quickly. But it seems like that's not necessarily the case for everyone who has symptoms that might be myasthenia or eventually diagnosis myasthenia. What is the usual or typical delayed diagnosis that we have seen and what are some of the problems with that?

Dr. Gordon Smith:
Yeah, Daniel, that's a great question. And I think in some ways this is similar to giving prognostic information to patients. I think the average experience isn't particularly helpful because there's a broad spectrum. Some patients who are medical professionals, as is the case with Ashley, get to a diagnosis fairly rapidly. But others have a significant delay and there is a lot of diversity in this. And I think a lot of care disparities. I work in Richmond, Virginia, which is a very diverse community. I moved from Utah and one of the things I observed coming here is the impact of care disparities.
And by that, I mean, not only urban populations, but we care for a large rural population that often has difficulty accessing care. And even when patients are able to access care, they sometimes don't have access to subspecialists or even neurologists. As you know, there are not as many neurologists as we need. And so, there's a lot of variability. Some patients get diagnosed quickly, but for some it takes a long time and even after diagnosis to get the appropriate care can be a real challenge.

Dr. Daniel Correa:
And Ashley shared with us that early on, she was placed on steroids and then started on azathioprine before moving on to some other newer medications. Can you explain for our listeners some of the differences between emergency or crisis treatment and more long-term treatments for myasthenia?

Dr. Gordon Smith:
Sure, Daniel. And I think that's a question that's becoming more complicated in a good way because we have all of these new treatments and trying to figure out where they should be applied is an active area of investigation and discussion. Traditionally, chronic management of myasthenia has been focused on use of medicine that helps boost the signal in between the nerve and the muscle. So, Mestinon or pyridostigmine, which blocks the breakdown of that chemical signal. That's helpful for many patients, but for most it's not enough.
After that, we've traditionally relied on prednisone or steroids which worked for most patients. They're very effective, actually. But the challenge is there are a lot of side effects, and I suspect Ashley may have commented a bit about these as well. It's hard for patients to take prednisone. The approach we typically take with prednisone is to gradually decrease the dose over time to minimize those side effects. Oftentimes, it's difficult to get to a low enough dose that is both effective and doesn't have side effects. And for that reason, we typically have used medicines like azathioprine or another common one is mycophenolate mofetil. These are drugs that are commonly used to prevent organ transplant rejection or other immune diseases.
In this context, we call them steroid sparing agents. They help people reduce steroid doses, but these medicines have their own side effects and risks and require monitoring. And so, while that approach works really well. It has problems, particularly in terms of medication side effects. For acute treatments for patients who are in a crisis, perhaps in an intensive care unit or in the hospital, the two approaches that we've typically used are, one, something called plasma exchange, which is often described as a dialysis type treatment. That's not really how it works, but it's kind of a construct that works to explain it.
It basically gets rid of the antibodies in the blood. And this is a treatment that requires a large IV catheter, a central line that is similar to those that are used for dialysis and is done about every other day. Usually, we use maybe 3, 4, 5 of these treatments. The other is in infusion of immunoglobulin or intravenous immunoglobulin, IVIG. Both of those are effective in treating patients who have a crisis. Occasionally, we use them chronically, in particular, IVIG can be used to either bridge patients in a crisis situation into their maintenance therapy or sometimes we'll use it as a chronic therapy for patients who really can't be taking steroids. And of course, we'll talk more in a moment, Daniel, I'm sure about the new agents which are really transforming care.
But this overall treatment approach is good. And in fact, probably 80, 90% of patients do well on that from a medical perspective. The doctors will say, "Gosh, my patient's doing really well." But if you ask patients, many, probably more often than not, are unsatisfied. In other words, maybe their myasthenia gravis is under good control by the neurologist perspective, but they may have side effects from the medicines they're taking. And I think that's the promise of the new therapies is really to not only provide a higher level of disease control, but to do so in a way we hope that will leave patients with fewer side effects.

Dr. Daniel Correa:
I wanted to remind our listeners, as Gordon brought up, there is this term of crisis or attacks in myasthenia. Please listen to our past episode with Suzanne Rogers and our expert Dr. Sylvestri, where we talk about and explain what the crisis or attacks are in myasthenia. So, Gordon, tell us where have we gotten with these new medications and how are they different from the past medicines that we use for management of myasthenia?

Dr. Gordon Smith:
Well, Daniel, they're more targeted. So, if you think about the treatment approach I described with steroids or IVIG and plasma exchange, there are a couple of things that I didn't say, which is precisely how they help. Now, the plasma exchange gets rid of antibodies, so that makes sense. But steroids, steroid sparing agents and IVIG are probably pretty blunt tools. They're messy in that they have lots of different mechanisms. The two classes of medications that are now available to us are more targeted. And so, the one class that was first approved are complement inhibitors. These are Soliris or Ultomiris or eculizumab or ravulizumab.
Complement is the way that the immune system rips the antenna off your roof and pokes holes in it. So, complement's a really confusing system, but it's this network of proteins that ultimately lead to a number of different activities in the immune system. And this is obviously the way your immune system gets rid of bacteria, for instance. One aspect is they attract a whole bunch of chemicals that increase inflammation, but the pointy end of the stick, as it were, no pun intended, is that it ends up poking holes in cells and causing the cells to be damaged.
And so, this turns out to be a really important mechanism by which these antibodies that cause myasthenia gravis, particularly the acetylcholine receptor antibodies, is important to recognize that there are patients who have different antibodies for which complement may not be important, but for the 85, 90% of patients who have these acetylcholine receptor antibodies compliments the way that muscle membrane is damaged. And, so the eculizumab, Soliris, ravulizumab, Ultomiris treat that mechanism. And there are others in development that we might talk about.
The other new class of agents is exemplified by Vyvgart, the non-trade name is efgartigimod, so Vyvgart's easier to say. Vyvgart is another way of getting rid of antibodies that uses a system called the FcRn system, which is a pretty cool system. I don't know if you've ever thought about this, but how do antibodies get across the placenta from the maternal circulation into the fetal circulation or how do babies get antibodies into their blood from mother's milk? And it's this system, right?
And this system is not only important for fetal and neonatal antibody regulation, but it's also a mechanism by which our body regulates antibody levels. And so, inhibiting the system basically forces the cells in your body to get rid of antibodies. And so, you can think of it as a more targeted plasma exchange, but it's an infusion, which means it's a lot easier to use. And so, these two different classes of medicines both work very well. It appears, we have 60 to 70% of patients respond. Keeping in mind in clinical trials, there's always 30% or so of patients who get the placebo get better too, but they're very effective and they seem so far to be well tolerated.
There are some nuances to each that we might talk about. But these, I think, promise to transform care in a lot of ways. One is it gives us other treatment options. Two, we can think of creative ways that we might use them. For instance, maybe these can be used in acute settings. They certainly can be used to treat patients who have intolerable side effects from prednisone or other medicines.

Dr. Daniel Correa:
And then, to follow that up with another treatment that is used and increasingly used but isn't necessarily another medicine. So, Ashley also had a thymectomy. When and why are you discussing thymectomy with patients living with myasthenia?

Dr. Gordon Smith:
It's a question for which now we have better answers than we used to. We've known for a long time that the thymus gland is important in myasthenia. One, some patients with myasthenia gravis have a tumor in their thymus gland that needs to come out. But what's more, we've thought for a long time that taking out the thymus gland in people who have myasthenia gravis, even if they don't have a thymoma or a tumor in the thymus gland may help with long-term control. But there wasn't really good data to guide us in this, and it was variable whether patients got this or not.
We now have good data from a very large clinical trial that demonstrate that taking out the thymus gland and younger patients early in the disease can be helpful or is helpful in reducing the disease severity over time and allowing patients to get onto lower doses of prednisone, which as we've talked about is really important because prednisone causes a lot of side effects for patients.
And so, for younger patients, early in the disease, we recommend a thymectomy or being evaluated for a thymectomy. Older patients, those who've had the disease for a long time on multiple treatments, probably not as good at candidate. There's still a lot of questions that are out there, and I'll throw one at you that I think our listeners may be particularly interested in, and that is how do we treat ocular myasthenia?
As a neurologist, I was always trained that ocular myasthenia is sort of mild myasthenia, right? And even if you look at the MGFA, Myasthenia Gravis Foundation of American Classification System, the mildest form is ocular alone. But when you talk to patients, ocular symptoms are very disabling to them. And as a community, there's been discussion about how nice it would be to have data regarding whether thymectomy would work in patients who have significant ocular only disease. So, we'll need to await further clinical trials for that. But for now, at least, we've got a pretty clear answer that thymectomy and the right patient population is both appropriate and effective.

Dr. Daniel Correa:
Thank you, Dr. Gordon Smith for taking the time to join with us and speak with our listeners about your perspective and treating of community with myasthenia.

Dr. Gordon Smith:
Well, thank you, Daniel. I appreciate all you do with the Brain & Life podcast. It's a great resource for our community.

Dr. Daniel Correa:
Thanks for listening to the podcast throughout your weekly activities. And I hope our activity cues today were helpful as you think about how to increase your activity time each week to help with brain health and move better with your abilities.
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 and even get the Espanol version. 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 by email to BLpodcast@brainandlife.org and leave us a message at 612-928-6206. You can also follow the Brain & Life magazine and me on any of your preferred social media channels.
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Ashley Brooks on Advocating for Herself and Others with Myasthenia Gravis

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