Transcript
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Dr. Bril:
This is CME on ReachMD, and I'm Dr. Vera Bril. Here with me today is Dr. Hans Katzberg.
Dr. Katzberg, what is myasthenic crisis? Are there early signs of its impending risk? And can we intervene to limit its impact on patient outcomes?
Dr. Katzberg:
Thank you, Dr. Bril. Myasthenic crisis is one of the most serious effects from myasthenia that clinicians have to be on the lookout for. It is defined as a worsening or exacerbation of myasthenia that often can affect breathing or swallowing to the point that someone may be in a medical emergency due to the serious nature of these effects. One can imagine that if there is serious swallowing difficulties, there may be aspiration. And if there is significant respiratory disturbances, there can be hypercapnia and, worse, an arrest of breathing that can lead to an arrest.
The symptoms themselves can be difficult, sometimes in the early phases, but as symptoms progress, they can become more obvious. Things like coughing or not being able to clear saliva while eating or drinking, fluids coming out from the nose while drinking, low volume speech. The breathing difficulties sometimes manifest as a very obvious shortness of breath, but sometimes it's more difficult to identify, and it can be more subtle. Someone having more difficultly with breathing when they're lying or in the supine position. And the tests for these, again, should be done during the clinical examination. One can do something called the forced vital capacity if one has the capability to do that; however, that usually requires some equipment and assistance from therapists or resources in hospital in order to measure this.
When someone is undergoing a myasthenic crisis, it's usually important to maintain and support them with the appropriate resources, again, usually in hospital, with the assistance of a medical team, a respiratory therapist, as patients may require support from noninvasive ventilation, so the bi-pressure assistance, as well as occasionally intubation if the noninvasive options are not helpful. If patients are not able to swallow, often an NG tube is needed for support as well.
Myasthenic crisis is serious. Fortunately, it does not happen frequently. However, it can occur over a lifetime in up to 15% to 20% of gMG patients, and one has to be alerted to this at any point in someone with myasthenia, particularly if they're progressing with symptoms.
Dr. Bril:
Thank you, Dr. Katzberg. I think myasthenic crises terrify our patients. As they learn about the disease and this risk, they're very frightened that this will happen to them and they'll end up in an intensive care unit. And if they have that happen to them once, they certainly don't want to have that happen to them again.
I think one of the most important things to recommend to our patients is that if they're feeling increasingly short of breath or with any of the symptoms that you mentioned, liquid coming out through their nose, and they can't clear saliva, or they're feeling like they're drowning in saliva or phlegm, I think that the advice that we give is that they attend their nearest emergency room, letting the physicians know they have gMG so that the physician can be alerted to the possibility of a myasthenic crisis or impending respiratory failure. Because that's what a myasthenic crisis is. The patient is unable to breathe by themselves and is suffocating, and that is just terrifying for them and needs to be addressed as a medical emergency.
With that, our time is up. Thanks for a great discussion, Dr. Katzberg. And thanks to our audience for tuning in.
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