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Nicholas J. Silvestri, MD: Welcome to Medscape InDiscussion: Myasthenia Gravis, or MG for short. I'm your host, Dr Nick Silvestri. This is the sixth and final episode of our first season of this podcast, and we've saved the best for last: managing patients with MG in the primary care setting. Ideally, what role do primary care physicians (PCPs) play in the management of MG? With a lack of specialist availability, should PCPs manage most care other than diagnosis and treatment planning? And what are the key guardrails to know? For expert guidance on these questions, we've invited Dr Michael Hehir, the James F. Howard II, MD Professor of neuromuscular disorders in the Department of Neurological Sciences at the Larner College of Medicine, and division chief of neuromuscular medicine at the University of Vermont Medical Center in Burlington, Vermont. In addition to his work at University of Vermont, Dr Hehir served and continues to serve in leadership roles with the Myasthenia Gravis Foundation of America, the American Association of Neuromuscular and Electrodiagnostic Medicine, and the Neuromuscular Study Group. Welcome, Mike.
Michael K. Hehir II, MD: Thank you so much for having me. It's an honor to be part of this program.
Silvestri: Always a pleasure. Mike, you're known for your work with patients who have MG. Can you tell us how you got interested in neuromuscular medicine and what made you want to focus on people with MG?
Hehir: When we were training, neuromuscular medicine was such an exciting field to me. In terms of MG, we take care of patients who have conditions that affect their ability to function during the day. MG impairs their ability to walk, eat, breathe, hold down a job, and do things for their family. But in our short careers, what we've seen for a lot of neuromuscular disorders is that we now have treatments that can help these patients.
We're observing genetic therapies for conditions like spinal muscular atrophy and muscular dystrophies. We're seeing several treatments come out for patients that are going to improve our ability to care for them, and in the past 2 years alone, there have been four new treatments approved by the FDA in the United States. So it's really an exciting time and we can do a lot of good for our patients, which makes it fun to do what we do.
Silvestri: I couldn't agree more. Thank you for making me feel better about myself by calling our careers "short." In the time since we were in training, we have certainly seen an explosion in treatments, and there's a lot more benefit to come for patients, so it's definitely an exciting time to do what we do.
Okay, Dr Hehir, let's get started. My first question to you: What role do PCPs play in the management of MG, including ongoing monitoring and coordination of care with specialists?
Hehir: When coordinating care, it's important to first recognize when your patient with MG might be getting worse, and particularly recognize developing situations where they might need to be hospitalized due to weakness. That happens when MG starts to affect a patient's breathing muscles or their swallowing muscles.
These situations are often triggered by conditions that a PCP might see a patient for; they may have just been getting over an upper respiratory infection or a urinary tract infection, or had a recent stressor in their life and are experiencing anxiety and not sleeping, which is triggering the MG to get worse.
Or maybe they just had a recent surgery or medication change and then suddenly is reporting to their PCP saying they're having trouble swallowing or are feeling short of breath with exertion. Those are situations where the PCP would want to send the patient to the emergency room because they might need to be treated with some kind of a rescue therapy, like intravenous immunoglobulin (IVIG) or plasma exchange.
The other important thing to recognize is that the majority of the medicines we use to treat MG cause our patients to be immunocompromised, which definitely requires intervention by a PCP — to keep patients up-to-date on age-appropriate vaccinations. This is true for some newer medications, like this new class of medicines we're using called complement inhibitors, which require vaccination for Neisseria meningitidis; this is a vaccine that's often not given to older patients. And we're diagnosing more and more patients with MG over the age of 60, so this would seem like an easily overlooked vaccine for them, based on their age.
It's also important to recognize that patients coming in with regular medical conditions, like an upper respiratory infection or a urinary tract infection or any infection, may not show all the signs and symptoms of an infection because of the immune-suppressing medicines we treat them with. So it's important to treat patients with MG more aggressively than just the average patient in these instances.
Silvestri: That's a good point. Looking at the whole picture is what PCPs do really, really well, and I think we could probably do a better job of being holistic, but it's important to work with them to take care of the whole patient.
Hehir: Yes. I think there are lots of aspects to that. A PCP could be the first person a patient calls when they're not well, from the standpoint of MG. It's important to recognize that patients with MG can get weakness, and when their weakness develops, sometimes it can develop rather rapidly. MG affects certain muscles in the body more than others. When it's starting to affect muscles that impair the person's ability to chew or swallow, or they're starting to get respiratory symptoms, it's important to know that we have to act quickly in those situations; you may need to be directing your patient to an emergency room, or you may be directing the patient to call their specialist so that an intervention can happen and try to turn it around before a patient might need hospitalization for worsening weakness.
When patients are worsening, they can be triggered by things that they may have seen their PCP for. They could have an infection, just gotten over the flu, or had a lot of stress in their life and be dealing with a lot of anxiety, or something that's affecting their ability to sleep and that's triggering them to get worse. A medication change can also trigger worsening, so think it's important to recognize those sorts of things.
Silvestri: These are all great points, Mike. With the lack of specialist availability in some regions of the country, should PCPs manage most care other than diagnosis and treatment planning? And a related question: How can homebound patients or patients who live in rural areas get better access to care for MG?
Hehir: That's a tough question, but I think MG in 2023 is really becoming a condition where there are just so many treatment options for patients that it may be challenging for a PCP who doesn't focus on MG specifically to manage the ins and outs of the treatment of that condition. I don't know how it is for you, but I feel like I'm drinking from a fire hose sometimes when I'm trying to explain to patients the different treatment options that are available for them for MG, and I do this every day. I think there is a role for a specialist in caring for these patients. But in a rural area, particularly like the one I practice in, I think it is possible to have a partnership where you could work with someone to advise, or you could come up with a plan together to manage some of these treatments, like some of the infusions that we're now giving for our patients.
There are also a lot of ways that people can leverage access to care now that maybe we weren't as adept at just a couple of years ago. With the COVID-19 pandemic, we've all gotten really good at virtual medicine, and particularly for MG, we got very good at it in the past couple of years.
There's about to be a video exam that'll be well validated, that's easy to administer in 5 minutes on the computer, which gives you an excellent idea of what's going on with a patient, and I think you could use that to guide therapy. So even if there's not a specialist in someone's town, there might be somebody that they could access virtually. I know that we do that for patients who live a distance from here. That could be good for a homebound patient as well.
For a homebound patient, it's important to ask whether they are homebound because of MG. If the answer is yes, it's probably a good idea to reassess their treatment strategy for MG because potentially the treatment strategy could be improved and maybe they wouldn't be homebound. But obviously, people can be homebound for many reasons.
Also, there are several good resources that a PCP or a patient could access to help with finding a specialist. The Myasthenia Gravis Foundation of America maintains a Partners in Care program which provides lists of physicians from around the country who are adept at treating patients with MG. It's not a perfect list and there are definitely some areas of the country with fewer specialists, but you can usually find somebody within a few hours by accessing the Myasthenia Gravis Foundation of America resource.
Silvestri: All great points, and that's a fantastic resource to highlight. So, what are the key guardrails that PCPs should consider when treating patients with MG?
Hehir: I think it's important to recognize when a neuromuscular patient is starting to develop symptoms that could become serious or require hospitalization. In MG, a patient who's beginning to develop respiratory dysfunction could report a number of symptoms, including shortness of breath with exertion. The person could report having a headache upon wakeup in the morning, or they could start to feel short of breath when they're lying in the supine position. I think those would all be triggers that somebody's MG could be getting much worse. The other thing that can severely affect patients with MG is when they begin to develop difficulty with swallowing.
The patient may be having difficulty swallowing liquids or solids, or they may have fluids come out of their nose when they drink. Those are red flags, and you need to get the patient to an emergency setting, because they might need IVIG or plasma exchange to try to turn their condition around.
It's also important to recognize that rarely do those two situations — respiratory muscle weakness and swallowing weakness — happen in isolation. Usually the patients are having other symptoms, like their shoulders might be weak or their hips might be weak, or they're having more double vision than usual because of eye muscle weakness.
Those things usually come together. And that's another important point for a PCP: If a patient is presenting with isolated respiratory dysfunction and no other symptoms of MG, think about their other medical conditions and whether it could be causing the presenting symptom.
Silvestri: That's a great point, that latter point. We all care for older patients who have medical comorbidities, which may include things like heart failure or pulmonary disorders, and it's sometimes difficult to tease those out. But rarely does respiratory failure occur in isolation due to MG. It certainly can happen, but I think it's the exception rather than the rule, as you point out.
Silvestri: Can you talk a little bit about the importance of physician-to-physician communication when it comes to caring for patients with MG?
Hehir: These patients in particular need a team-based approach. Our patients are complicated. We're diagnosing MG in older people more and more, and they may have multiple medical issues that require attention. So it's important to work together.
It's also important to work together because the number of new treatments and the number of mechanisms by which the medicines that we treat MG patients with are constantly shifting; the complications and side effects that can come along with those things are different from what we're used to. So it's important to have an open line of communication related to that.
There are lots of situations where it's good to have that relationship because something else could develop in the person's medical history that would require a team-based approach. I recently had a patient who, unfortunately, developed melanoma that was metastatic and was going to need to be treated with one of the new immune checkpoint inhibitor therapies that turn on the immune system. We had to change the monitoring plan for this patient because it could potentially make their MG get much, much worse. So we changed the plan. We came up with a plan where I was seeing this patient monthly by video visits to make sure we were keeping tabs on this patient more than their previous, every-6-month schedule that they had with us. So in situations like that, it's really important to make sure that there's good communication.
Silvestri: I couldn't agree with you more. I mean, I think it's just good medical practice — and certainly that relates to more than just MG — for a lot of the reasons you talked about before, like MG becoming more complicated in terms of the way we treat it, and how with new and novel mechanisms of action of medications, it's even more important. And then there are other considerations, such as the importance of vaccination prior to the administration of complement inhibitors. Often we need to work hand-in-hand with PCPs to get that done. Understanding why that has to be done and the importance of it, and what to look out for, are all key, so thanks for bringing that up.
Hehir: Yes. And another area that we didn't really touch on yet is this list of medications that sometimes can worsen weakness for patients with MG. It's important just to recognize that this list exists; I don't think PCPs need to memorize the list, but just know where to find it.
An updated list is always available on the Myasthenia Gravis Foundation of America website, and you can also encourage your patients to download the Foundation's smartphone app, which includes that list. They can pull it out if they ended up in the emergency room or they saw another specialist, because there are some common medicines — like beta-blockers, for example — that sometimes can worsen weakness. And if you can choose an alternative from the medicines on that list and still take care of your patient, that's always optimal.
Silvestri: That's another great example of where physician-to-physician communication is important. I always appreciate when PCPs and other docs call me and say, "Hey, I was thinking about using this medication in your patient with MG; is this okay?" Or "What do you think about that?" I think that's a great starter for conversation as well.
Hehir: I totally agree.
Silvestri: All right, Mike, we've gone through a lot of information. I really appreciate your thorough and candid answers. What is the one thing that you want listeners to do differently after hearing this podcast?
Hehir: Boy, save the easiest question for last, right? I think it's important to recognize that MG is treatable, and our goal for patients is to have minimal symptoms with minimal side effects, and to recognize that there are lots of options to get people to that clinical status. Also recognize that your patients are immunocompromised. Always feel free to reach out and discuss care with their specialist or with their neurologist or neuromuscular neurologist who might be seeing them. We love it when people reach out to us; we're always willing to talk on the phone, return emails, chat about our patients, and talk about MG. As a whole, I think the community really likes that. So please always reach out with any questions.
Silvestri: Absolutely. Thanks, Mike. I agree. In a nutshell, what we've learned today is the important role of PCPs in the treatment of our patients with MG. The communication between neurologists, neuromuscular specialists, and PCPs is so critical when taking care of and treating patients with complex diseases like MG.
Thanks again for all the information you've given us today.
Hehir: Thank you for having me. Always a pleasure to work with you.
Silvestri: Thank you, Mike, and thanks to our viewers for joining our discussion with Dr Hehir. Be sure to check out the Medscape app and share, save, and subscribe if you enjoyed this episode. This is Dr Nick Silvestri for Medscape InDiscussion.
Resources
New and Emerging Treatments for Myasthenia Gravis
Myasthenia Gravis With Elderly Onset at Advanced Age
Myasthenia Gravis, Respiratory Function, and Respiratory Tract Disease
MGFA Partners in MG Care Program
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Cite this: Managing Myasthenia Gravis in Primary Care: Best Practices - Medscape - Oct 24, 2023.
