A Conversation on Myasthenia Gravis and Exercise

From an advocate’s perspective:

First, let me say that the conversation on Myasthenia Gravis and exercise is a sorely needed one, no pun intended. It remains a controversial and delicate subject as many struggle to maintain stability in their daily life with MG outside of any augmented incorporation of exercise. The community by and large faces a great deal of misinformation as it relates to our weakness and overall stamina in the medical community with too many clinicians and well meaning family and friends telling us that we would feel better if we would just exercise more, inadvertently missing the entire picture of what MG is, how it is embodied, and what such a prescription would mean for a large majority of sufferers.

This latest study (referenced at the bottom of the article), I fully believe, was brought about in an attempt to deal with this hesitation and a topic that is so fraught with misconception.

I will start out by saying that I appreciate Dr. Macko’s validating depiction of MG altering quality of life, often significantly, and I appreciate her endeavor to help bring a certain level of clinical assessment and consensus as a broad basis to help other clinicians foster a potential exercise program that may be beneficial to some.

With that being said, as an advocate, I have hesitations and concerns about this study that was recently published in a neurological journal. To make it a little easier to discuss, I organized my concerns into bullet points.

  • Too broad and poorly applied. Right off the bat, the study cites application to those who are categorized as stable with mild presentations while moderate myasthenics are added in later as potential considerations. What does mild or moderate and stable MG look like clinically? This study fails to correctly and appropriately define this salient premise upon which it largely based its foundation for success. Without a defined consensus of what these terms mean, their discrepancy proposes a potentially dangerous gray area left to individual clinical interpretation that may or may not be appropriate to the Myasthenic.

 

  • Contradicts itself in the tailoring to a disease that fluctuates (as cited within the study,) sometimes hourly and most certainly daily. For example, if an exercise regimen is developed based off a guided assessment from a skilled practitioner (which I will address that in greater detail in a moment) on a particular day of stability and controlled symptoms and several days later, or perhaps even later that day, the disease manifests differently and the stability from before is now a flare that requires hours or days of rest, where is the application of a tailored regimen and how can it be effective? What day are they tailoring to and how is it possible to stick to this? Is it something that the patient only adheres to on days similar to the assessment? I think this key issue comes back to the poorly defined definitions of mild and moderate MG and stability.

  • Oversight for this, even in more mild and well controlled cases, requires a skilled therapist and clinician to help ensure the proper balance between beneficial exercise and inducement of exacerbation with potentially serious adverse setbacks. My main issue here is finding skilled therapists and clinicians who understand this disease and it’s minutiae well enough to be able to execute such a program safely. As it is, it is almost laughable to think about when you consider how hard it is for the average Myasthenic to find a skilled clinician to care for the disease itself. Impossible? Not at all. Probable? Not highly likely at this juncture in the current relationship between the overall clinical world to MG.

 

  • Focuses on the probability of sustained stability that is not clearly defined or applied. Stability can be a fleeting principle when discussing MG as efficacy and timing of treatments, clinical skill, and patient response are all wild cards and as the autoimmune component waxes and wanes (as noted in the study from a myriad different sources, some controllable but many not). Even under optimization of environment and overall health, MG is nothing short of unpredictable.

 

  • Fails to provide a control set of those who are defined by this study as mild and moderately stable who are receiving skilled care and are responsive to therapy who are not in an exercise regimen to see an equitable, nonprejudicial comparison between exercise participants and their controls. This fails to offer a comparable foundation to see if, in the long term, exercise benefits those who are stable enough to tolerate it or if there is too broad an application from a more general understanding of how exercise benefits the body in those who are not struggling with MG.

 

  • While muscular wasting and atrophy (by-products of the underlying flaccidity inherent to the disease itself) and secondary myopathy (medication induced) is something Myasthenics actively need to guard against, an exercise regimen provides a potentially contradictory ideology in its claim to help dispel fatigue and improve overall function based on the very premise of the mechanics of the disease (i.e. varying levels of muscular fatigue or lack of contraction seen after inducement of changing activity levels from increases in antibody production, changes in nerve transmission as a result of fevers, infection, temperature changes etc., exacerbation from co-morbidities, incorrect medication regimens or poor clinical response, overall nutrition deficits due to fatigued bulbar muscles used to typically chew and swallow safely etc…), which ultimately circles the drain back to the definition of mild, moderate and stable.

 

  • It fails to aptly define fatigue. Are they referring to muscular fatigue (flaccidity), which is defined by the study’s own criteria as a causation of repeated, sustained activity? Are they referring to the mental fatigue common amongst autoimmune disease and due, in part to the inflammatory process of a constantly fluctuating immune response? Research has indeed shown that exercise boasts great benefits mentally, emotionally and physically and improves overall health but the research across the board gets muddy with autoimmune disease. Without knowing the cause of the immune abnormalities and mind numbing mental fatigue produced in autoimmune disease, generalized applications based on exercise benefit from those outside this criterion is inequitable.

 

  • I do believe that mild* and some moderate Myasthenics who are well controlled symptomatically, are not at risk for pending exacerbation and do not have a predominance to respiratory and bulbar weakness, will benefit from a highly individualized and carefully executed regimen. The point in this rebuttal is not to say that exercise should be thrown out the window or taken off the table, but to point out the flaws that have been established on too many assumptions, variance and lack of clearly defined clinical presentations.

*(I am defining mild MG presentation as a Myasthenic who is not hospitalized for their MG, is optimized on their treatment plan and does not require more aggressive therapies to maintain stability, is able to tolerate temperature extremes with less noticeable adversity, enjoys greater sustainable activity and occasionally struggles with a temporary flare that is resolved with a day or two of rest.)

 
My conclusion:

For far too many Myasthenics, (including moderate cases that are able to perform somewhat functionally on a daily basis) routine activities like bathing, dressing and eating, let alone driving, raising children, cleaning, cooking or running errands, take sustainable energy and contractual strength that is often not there or not there in any reliable measure. Incorporate exercise and now you have Myasthenics who are often put In places of greater temporary weakness than before they began the exercise, most likely compounding the very issue meant to be allayed by the exercise to begin with.

For those who are able to exercise and improve overall health, reduce stress and increase stamina in varying degrees through exercise, this is a move in the right direction but the minority response cannot define or imply clinical approaches to the majority without creating a potentially harmful clinical perspective on patient outcome, effort and treatment. Prior to the publishing of this study, there was already in existence a clinical misnomer about patient effort as it relates to overall patient prognosis. Physicians who are treating MG without a solid and capable understanding of the mechanics of the disease, tend to extol exercise as a means of augmented therapy, insisting that it will provide benefit to those who try. Now, there is a published article in a respected journal to further strengthen their approach without appropriate consensus or definition and I am afraid that the interpretation will be rooted in even more potential misunderstanding and poor application.

I appreciate the conversation but not the application of this study.

– Rebekah Dorr
Founder, Myasthenia Gravis Unmasked

 
From a Physical Therapist’s perspective:

Rebekah has already highlighted a lot of my questions/concerns about the data included/reviewed in this article. But, as a physical therapist who now suffers with MG I have a few issues of concern as well. First of all, please keep in mind this is the summation of a literature review and that the primary conclusion drawn is that there is a need for the development of an “evidenced based protocol” for MG patients in order to determine what type and what level of exercise will aid in being able to maintain or improve their physical functioning. This article is not stating that exercise is what all MG patients need to do across the board and it should not be interpreted that way by anyone. My interpretation is they are merely looking to validate the need to develop a protocol. Protocol by one definition is a written set of detailed instructions to guide us in the care of a patient or to assist in the performance of a procedure. Please note this says “guide” and “assist”. Unfortunately, protocols have allowed the clinical decision making process to become robotic and not the critical thinking of an intelligent human being who is looking for guidance or assistance. Additionally, the article itself acknowledges the variability of this disease, patient to patient, day to day and within a day. This is very frustrating for patient and clinician both, but to me it also obviously contradicts the need for a “protocol” in the first place!

The premise behind the article highlights one of the flaws of our current medical system. Find a box to fit the patient in and then treat them with a list of pre-determined medications/procedures regardless of the specific presentation of the individual in front of you.  I am not arguing that research to develop a body of quality evidence based information about the effects of exercise on the MG population is sorely needed. Not only will this help us further understand the disease itself but will enable us to optimize the benefit of the rehabilitation provided. However, this is an extremely daunting task if you truly consider the number of variables the myasthenic population presents with and the fact that this is a rare disease and by nature offers a limited population of subject with similar presentations to do the studies on. Several of the articles cited in the references were conclusion based on the review of surveys of self reported activity levels and quality of life, or they were case studies of very limited or specific patient populations. This is not the type of information that I would use to draw conclusions about what is appropriate exercise recommendations for the MG patient. Unfortunately, it will take years and money to develop a body of quality information and this will allow many practitioners and patients to continue to flounder with the issue of what to do now!

I believe the focus needs to be laid on the education of the medical community as to the nature of the disease itself. Doing this will enable the medical/rehabilitation practitioners to apply the skills we were taught to the specific patient in front of us, NOW. As a therapist it is my job to evaluate the entirety of a person and apply the principles of exercise, tissue mobilization, energy conservation, body mechanics, posture, etc to develop a plan to improve that patient’s functional status without causing further harm. If I am unfamiliar with a condition my patient presents with, I either educate myself about it or find someone who knows. It is truly sad that medicine is now so driven by the need for evidence based research and protocols to tell us what to do and to provide the validation so that insurance will cover it. The thought that unless I am provided the research that says my patient should do 3 reps instead of 10 per set that I can’t determine that for myself is very disturbing. Not only that, but there is no way you could develop a protocol of specific exercises to address this population as a whole. This is not a surgical procedure with defined healing constraints or mechanical limitations that would outline the treatment for you. This disease requires knowledge of the disease not a list of pre ordained “guidelines” to tell you what to do.

Lastly I must say we need to redefine what the word “exercise” means. For nearly all of us it conjures up tight clothing, a gym, x sets of x, sweating and feeling the burn. I will just tell you there were many times in this process that getting make up on and hair fixed felt like a P-90 X work out to me!!! Individualized therapy treatment could easily lead to an exercise prescription for things like: take the long way to the bathroom using your rolling walker at least 2 times per day, stand with your arms out in front of you for 10 seconds before you sit in a chair, each time a commercial comes on pump your feet up and down until the program comes back on. I swear to you this is where I started myself. Things this simple can be exercise programs when you are significantly debilitated But keep in mind, I would not prescribe these type of exercises for an MG patient who is still working, getting their own groceries and doing their yard work. I understand there are folks that function at that level and for them I may prescribe the 3 sets of 10 of X, Y, Z exercises and see how they tolerate it. There are MG patients who are stable enough to run 5K’s, half marathons and more and their exercise program would be entirely different from both of these. If we (medical practitioners and patients alike) would step out of our need to have uniform treatment protocols for diseases and humans that are not uniform, a lot of this issue could be put to rest!

I do want to specifically address the concern Rebekah raised about a program being developed based on one presentation and the next day the patient is different and how do you now apply that program to the new status. Part of any treatment plan is educating the patient on the “what if’s” of the situation. If the patient has been dealing with this condition long enough they may have been able to identify patterns or trends of how their symptoms fluctuate or present, i.e., more upper extremity, more lower extremity, swallowing, respiratory etc… I would expect any good therapist to work with the patient to take this information and develop an “if this then that” addendum to any treatment plan. If there is not an identifiable pattern to presentation then the therapist and patient, together, should develop the plan in a way that allows the patient freedom to alter as needed and still have a level of achievement and continue to move them forward to a goal of improved function. Even if that means do nothing. Just as I feel the need for clinicians to rely on our critical thinking to make clinical decisions, patients need to rely on their critical thinking to take what instructions/information we are given and determine what is best for their particular situation.

– Erin Brantley PT, CLT
Study referenced:

http://myasthenia.org/LinkClick.aspx?fileticket=IFsAhUwqGV0%3D&tabid=40

Empire’s Myasthenia Gravis

Empire is Fox’s hit TV show that millions of people can’t help but love. For Myasthenics, this show in particular was put on the map in our communities when the writers chose to use Myasthenia Gravis as a dramatic element for one of it’s main characters, Lucious Lyons, played by Terrence Howard.

Back in 2015, awareness efforts on Twitter and Facebook were at a frenzied pace. I remember living on my phone almost the entire month of June that year, to the point where I wouldn’t realize the passage of time or interact with my family. Myself and several others pushed hard for awareness on Twitter, reaching out to everyone we could for retweets, shout outs and love. Empire had premiered in January of 2015 and the buzz reached an excited pitch when Lucious Lyons had been diagnosed with MG later on in the first season. Our Twitter awareness efforts extended out to the Empire writers and cast as the news broke about his wrongful diagnosis from ALS to Myasthenia Gravis.


Pictured Terrence Howard (Lucious Lyons)
EMPIRE ©2017 Fox Broadcasting Co. CR: Chuck Hodes/FOX

And I watched as the instantaneous reactions lit up Twitter and blogs with headlines and articles lamenting their “disappointment” that it wasn’t ALS, that it was “just Myasthenia Gravis” and how “boring that was” etc etc etc…

Just like that, the precedent was set and stigmas and incorrect information were resurrected and affirmed for the masses. Hundreds of articles were quoting the teeth grinding ideology that “most Myasthenics lead nearly normal lives and respond well to treatment”.

Other than spotty encounters with MG over the last two years with some off beat depictions that sometimes get part of it right, Empire writers have stuck to the plot line that MG would be a peripheral issue for Lucious Lyons. It is a disease the writers have subsequently left to the shadows of the show in a mercurial form that is pulled back into focus when needed for some dramatic flare but no permanent harm.

Leading in to their season premiere (April 12, 2017), the community began to buzz again as trailers teased, showing Lucious struggling on a vent and our hope once again became palatable. Last night, the season opener started on the mark with Lucious struggling to speak and breathe and a doctor urgently seeking to get him to a hospital, telling him that if it wasn’t treated, it could be fatal. They intubated him and cut away with Lucious laying there, struggling. I felt myself growing more hopeful that this time, Empire would get it right.

They come back to Lucious still on the vent, supposedly sedated but very much alert and able to comprehend what is happening around him. The plot line moves on as other characters move deliciously in and out of their woven drama, entwined in complicated relationships with one another. The camera comes back to Lucious yet again and we see that he is being threatened by a beautiful female character on the show and she pulls his vent tube from the machine. His eyes widen and he begins to panic and his breathing distress augments. She smiles until she looks down and sees him pull out a gun keeping his arm close to his chest but still able to hold and aim it at his would be threat. My hope rapidly began to dwindle. Call me a nitpicker or overly critical, I can handle that. But from my perch as a Myasthenic who has almost died far too many times to count and has advocated for more Myasthenics in crisis than I can recall, I began to pick apart the reality of the depiction from his lack of weak neck flexion and his ability to gesture and use his arms quite prolifically with a critical eye. Yes, we are all different. No, we will not all have the same crisis experience for those unfortunate enough to walk in that. But the truth remains that there are foundational principles that MG falls back into, in spite of the uniqueness in presentation from person to person.

In my experience as a patient and a patient advocate, if your diaphragm and intercostal muscles are flaccid enough to need a ventilator for assistance, weak neck flexion, bulbar and bilateral extremity flaccidity is also typically present to varying degrees. The presentation may vary and not all will have every symptom, but it is almost unheard of to have it strictly manifested as a respiratory distress.

At this point, my mind is racing and I am now tensely watching the next cut to Lucious as the show goes on after he draws his gun and is still on the ventilator. Next thing the audience knows, Lucious is off the vent, sitting up in bed with a perfectly bilateral smile, strong neck flexion, able to lead a meeting, freely gesturing and while his speech is gravely and perhaps slightly hypophonic from the vent, his recovery is nothing short of a rapid miracle. Drama ensues with his son and the board meeting he called (which, in most cases would have further weakened him due to high stress post crisis) and the scene cuts away with him sitting up in bed, alone.

The next time the audience sees Lucious, he is miraculously walking into an election party, sharply dressed, no sign of obvious weakness, talking, stirring up some good old Hollywood drama, singing and playing the piano.

EMPIRE: Pictured L-R: Guest star Taye Diggs and Terrence Howard (Lucious Lyons) in the “The Naked Villainy” episode of EMPIRE ©2017 Fox Broadcasting Co. CR: Chuck Hodes/FOX

For anyone who has ever had to recover from crisis, this ending scene was cringe worthy. To go from a flaccid diaphragm that is struggling for basic contraction that allows continued breathing/respiration, being on a vent a short time, being removed quickly and then bouncing back to go to a party, let alone singing and playing piano expeditiously is so against the basic science and reality behind the disease, it isn’t even funny. In fact, it is down right insulting.

While many may argue with me for my “critical assessment” of his vented performance and recovery, I do not know anyone who could refute the knowledge that no MG crisis ever recovers like that. Ever.

Myasthenia Gravis is nothing if not a complicated nuance. We look for the big picture symptoms and miss the small details and, in my experience, it’s often the small details that matter most when tackling accurate individual care and saving a life in the critical moments.

Even in the best case scenarios with appropriate treatment and accurate triage, the Myasthenic in crisis doesn’t recover so swiftly and completely.

As a community, we now face an audience of millions of viewers who have no other reference to MG other than what they may Google about it or see on a show like Empire, House or Grey’s Anatomy, all of which have fallen far short of tapping into the reality of MG. Our uphill battle in fighting against stigma and pleading for awareness has just grown steeper in the lay community. While not all will take the Hollywood portrayal as gospel, the influence has cast a long shadow against the idea that MG is not easily treated, managed or lived with, it does cost lives, treatments have their own hell and for most, normalcy is a dirty word in a rear view mirror.

Yes, this is Hollywood. Their main game is ratings and making money and MG was their ticket for just the right amount of controllable drama to catch attention and tease out the nature of the disease while still maintaining control that it won’t keep their main character down for long as he seeks to stay at the top of his game. But, I will argue all day long that when they chose MG, they silently accepted a responsibility to thousands of sufferers who are desperate for a voice to show millions of people that our struggle is real, to make it resonate and bring validation. It is what it is. You cannot profit from a disease and not have the courage to get dirty with it’s truth, with the lives it touches. MG doesn’t need a writing staff to lend it drama. It is drama and it isn’t going away.

Empire, you have once again angered and deeply disappointed me. You have made me feel personally invalidated and quite frankly, insulted. While my struggles to live with MG may not look like someone else’s, my unfaded memory of blue lips, shallow gasps, doctors telling my screaming parents I was just anxious as I slumped down onto my side, unable to move or speak, a paged code and the eerie awareness that I was on the line of living and dying tinges my critique. I’ll admit that I am biased and happily so. I earned that bias. My struggle and thousands of others deserve better from you, Empire. We deserve your time, your diligence and your skill.

Just one woman’s two cents (and some change).

– Rebekah Dorr
Founder, Myasthenia Gravis Unmasked

Help Tyler Get a New Mobility Scooter

Hi, I’m Tyler, the admin of the MG Hope blog. I have MG among many other chronic illnesses and I use a mobility scooter to get around on my college campus.

I’m a part time college student studying for a B.S. in Computer Information Systems & Technology. I started college in 2011 as a full time student before my diagnosis and kept going while trying to get a diagnosis. I am determined to finish and my mobility scooter is my lifeline to get to and from classes and meetings as a student leader. Without a new scooter, I will likely be unable to finish school.

My current scooter needs about $500 – $600 worth of parts and it is 15 years old, bought used. I need a new one as I can’t see putting money into something that may or may not last, but I lack the funds to buy it. My insurance won’t pay for it as I don’t need or use it within my home to complete my “daily living activities.”

I greatly appreciate any help you can provide and I hope you’ll consider sharing this link.

https://www.youcaring.com/tylermorris-801596

Thanks,
Tyler
MG Hope Admin