Offering Hope and Love to Myasthenia Gravis Warriors

“Walking with us doesn’t have to be complicated, it just takes a choice.”

We read the well meant phrase often enough; “let me know if you need anything!” as we stare back through a screen, connected in the most disconnected of ways. But how many times do we find ourselves wishing that the cliched, albeit good intentions of another translated into practical help and support where we need it most, without the awkwardness or shame of having to send a message of S.O.S.? 

Myasthenia Gravis came into my life like a whirlwind, it’s symptoms quickly escalating from severe fatigue to respiratory weakness in a matter or ten days. My family and I  found ourselves in the emergency room four different times within a two week period, so severe and fast moving were my symptoms. The diagnostic process involved two spinal taps just 48 hours apart; the second spinal tap left me paralyzed from the waist down due to several rare complications. I was sent home a week later after being kicked out with a security escort, still paralyzed, after we insisted something was very wrong. 

I spent the next six weeks in a wheelchair at home or confined to a bed due to the paralysis and my increasing weakness. 

Concerned friends, family and neighbors dropped by and brought meals or called to check on us and we heard each time the invitation to call if we needed anything. But we found ourselves unsure how to ask, the needs were so great.  One evening however, a friend of the family dropped by unexpectedly after a long day at work and he had with him a special gift; he had taken the time to build me a ramp so I could get down the step from my front door in the wheelchair. 

After he was done installing it and I got to test out my new gift of freedom, he sat with us and visited. He looked at me and said something I will never forget; “Rebekah, that ramp out there? It is temporary. I made it that way on purpose because I believe that you’re going to walk again. And I want you to hang on to that.” He spoke with the grace and love of a father and I wept at his gift, overcome with emotion. He saw a need we didn’t know how to articulate and he showed up without us ever picking up the phone.

It has been seven years and like he promised, the ramp was needed for just six weeks, but it taught me a crucial lesson that I have never forgotten. We have opportunities in our lives to walk with our fellow human beings throughout many ups and downs. Some of us choose to stick around only for the good moments and others still simply don’t know how to respond to the bad, but those choices of investment change lives. 

Over the years, I have heard countless iterations of the invitation to call or reach out if I need something and while I always appreciate the heart that is behind such an entreaty, I find myself thinking back to the unexpected gift of a ramp and how a friend took the time to quietly walk with me and show up in the middle of the messiness. 

Sit with me during treatments, take me to the doctor, help me weed my flower beds or cut my grass, bring meals and paper plates, come sit and talk with me and forgive my messy home, call me once a week and tell me you’re thinking of me. Yes, it takes time and effort to pour into someone who is hurting, but it isn’t complicated. We just need you to show up, no matter what you are able to offer, because it matters far beyond what you could ever imagine. 

I believe we have to learn how to walk with our fellow human beings in this life; how to listen with patient grace, how to find ways to use whatever talents or resources we have to connect, support and stay the course when things get hard and life overwhelms us. It is easy to offer the entreaty that we are available to someone if the person struggling takes the time to call, but it can be hard for us to take you up on that. We don’t want to burden you or interrupt your life and sometimes, the truth of it all is that we just don’t know what to say, we just need someone to show up and let us know that we still matter. 

You see, it is easy to type something online and walk away when life is business as usual but it is a whole other thing when suddenly you are the one in need of support. After all, we once were like you too. Some come walk with us, let us tell you how we really are, no remedies or advice, no cliched responses; just the patience and grace to hear and affirm the truth of it all.

Walking with us doesn’t have to be complicated, it just takes a choice.

The Life I Knew Changed with Myasthenia Gravis

I am writing this letter on my own behalf. I feel it is necessary although not common yet for patients to be advocates for themselves in these situations. I am an honest person, a wife for ten years, mother of two children eight and ten, college graduate with honors, a loyal employee, and U.S. citizen. I have gone through dramatic changes in the last four years. I was diagnosed with Myasthenia Gravis when I was approximately 23 years old, in spite of this autoimmune neuromuscular disease I finished college while working full time.

Upon graduation I became a surgical/ medical instrument representative for a well know company. This job required me to travel, sometimes a great distance, on an almost daily basis. Prior to September 2014 I had Myasthenia Gravis, with daily symptoms and worked fulltime. At that time, I didn’t realize I was pushing my body too hard, therefore suffering physically and my Myasthenia Gravis (MG) not improving. There were times I couldn’t speak and would have to cancel appointments, I didn’t entertain clients where food was involved as chewing food was difficult at times or I would have to use the restroom multiple times due to rapid transition of the bowels, a side effect for me from the mestinon.

Somedays my body wouldn’t move well or my fingers at all, somedays I would get sick and it would take weeks to recover. I would then have to cancel appointments again. My life as I knew it came to an abrupt halt in September of 2014 when I had an MG crisis and was put on life support in hospital near the town I was living. I was on life support for over two weeks at times not know if I was going to live or die but I fought because of my family. During the month of September 2014 I went to the emergency room as I thought I had a cold or bronchitis. I planned on being back home within four hours per the emergency room protocol. Four days later I remember waking up and not knowing where I was, why I was in that room, why I was hooked up to machines, why I couldn’t move or talk, and so on. I remember waking up the next few days having the same feeling. I was sent to the Intensive Care Unit on life support, had bilateral chest tubes, the Rhino Virus, the Entero68 virus, pneumonia, was given paralytics in the Emergency room which is contraindicated for a myasthenia gravis patient, was given antibiotics that was verbally communicated and documented was a contraindicated medicine for a myasthenia gravis patient, had pneumothorax, and had pneumoperitoneum.

After three weeks on life support and being bed ridden I had to learn to breathe again, which is something we are born doing naturally, so to learn how to do this and accept it emotionally was overwhelming. I also had to learn to swallow, control my pee, poop, talk, sit, stand, walk, exercise, and more. This is just the physical part I had to do in the hospital. The emotional part came later. The ICU doctors and team had to do their part as well to keep me alive, with daily bronchoscopes for eighth days after being extubated. I had chest tubes having to be reinserted after pulling them out too soon. I also had a wound to my lip due to being intubated resulting in permanent scaring. Three years has passed since that incident.

I have gone to multiple physical therapy appointments, counseling appointments, primary care doctor appointments, chiropractic appointments, massages, pulmonologist, neurologists, rheumatologists, dermatologist, and more. I have done 16 doses (four rounds) consisting of one day a week, 4-7 hours each day, for four weeks of Rituximab. Approximately 135-225 doses (forty-five rounds) of intravenous immunoglobulins (IVIG) consisting of 3-5 days, 3-5 hours each day every four weeks. I currently am having a hard time with vein access for the (IVIG). Having to do this treatment plan makes me lethargic, I sometimes have flu like symtpoms, my immune sysytem is lowered, I tend to get infections easier, I am unreliable due to my phsyical pain and emtional truama, I dont know when the Rituximab is always needed therefore making long term commitments is difficult.

The physicaly therapist have done all they can and the pain that I have daily is not something they can address or fix. I have been going to counseling appointments over the last two years. I have come a longway but still have problems with large crowds, germs, being away from home for very long, going anywhere overnight, going to any type of healthcare setting, watching anything hospital related on television, and more. I have tried to volunteer in the community but always tend to only do half or less of what is planned initially due to my current condition. I go through daily emotional trauma from the experience. I currently have anxiety, was diagnosed with PTSD, and insomnia (side effect of prednisone, mestinon, anxiety and PTSD). I have daily physical trauma from the experience. I currently have pain in my neck, thoracic and torso area.

I have numerous scars that hurt with the changing weather. My immune system is extremely low due to a previous thymectomy, being on prednisone, doing regular IVIG treatments and the Rituximab treatments. My body, and emotions are not the same as they were before, the current medical treatments needed for my body keeps me from be reliable and committed to things beyond my control. In my opinion, I came out of that tragedy a totally different person and I believe I will never be the same person. I had counseling, have anxiety attacks and am afraid to travel over two blocks to take the children to school preferring not to even leave our home (my safe place). I can no longer talk comfortably to people and do everything possible to avoid crowds. I try to volunteer when I can but have to cancel a lot. The worst part is I found out the hard way that autoimmune disorders can be passed on to your children. My son was diagnosed last year with colitis. I pray everyday a cure is found for my son and I.  Thank you for listening!

Author
Keri Soto

Patience in the middle of the mess with Myasthenia Gravis

Patience- the capacity to accept or tolerate delay, trouble, or SUFFERING without getting angry or upset.

The natural doesn’t make an ounce of sense. Here’s the deal. Surgery was started, incisions were made, the robot was put in place and then my oxygen levels dropped to dangerous levels so they had to stop surgery. The surgeon thought that maybe there was a blood clot in my lung- which is common after IVIG treatments- took me for x-ray and NOTHING! No explanation. They took me to ICU where it took 3 hours for me to come out of the anesthesia… which resulted in me needing the respirator. 🙊🙊🙊 that was the pits! Panic and discomfort are the words to describe it.

Once I got my bearings I was told the news… no thymectomy. I couldn’t believe it. Everything that had been done to prepare for this surgery- my students not having their teacher, the unpaid leave, the expensive treatments, the pain and discomfort I was feeling- all in vain. How could this be?!

“The capacity to ACCEPT SUFFERING without anger.” I must accept that God knows what is best and for some reason He stopped the surgery. I cannot get wrapped up in the expenses, the delays, the suffering. I’d had very specific prayer requests… for the anesthesia to not make me panic, that I wake up normally from the anesthesia, for my recovery to go well, and for my cure. I didn’t even realize when they gave me the anesthesia, the recovery is going well (my MG hasn’t flared up at all- this is HUGE) the respirator was only for 3 hours Vs days, and my cure is up to Him.

So even though this didn’t go the way we’d hoped we still hold steadfast to our hope in Him.

“But blessed is the one who trusts in the Lord,
whose confidence is in him.
They will be like a tree planted by the water
that sends out its roots by the stream.
It does not fear when heat comes;
its leaves are always green.
It has no worries in a year of drought
and never fails to bear fruit.”
Jeremiah 17:7-8

Author: Linda Nulisch

Huperzine A, Mestinon and Myasthenia Gravis

Huperzine A gets a lot of attention as a natural (and sometimes necessary) alternative to pyridostigmine bromide, especially when there are adverse side effects to the synthetic treatment option. So what is the best choice and how do we begin to navigate the options in front of us?

First of all, let’s define what exactly Huperzine A is. Huperzine A is a dietary supplement that is “derived from the Chinese club moss Huperzia serrata and works as a cholinesterase inhibitor — a type of medication that works by improving the availability of the neurotransmitter acetylcholine”, needed for muscle contraction. (1) By definition, this is a similar mechanism of action found in pyridostigmine, also known by the brand name of Mestinon. Pyridostigmine is also classified as a cholinesterase inhibitor which helps to inhbit “the destruction of acetylcholine by cholinesterase and thereby permits freer transmission of nerve impulses across the neuromuscular junction”. (2)

While Huperzine A has been used for centuries in the Chinese culture to treat everything from “rheumatism and colds, to relax muscles and tendons, and to improve blood circulation”, it gained attention as a potential use in the treatment of Alzheimer’s patients. (3) In recent years however, it has sparked interest in the potential treatment of Myasthenia Gravis as an alternative to pyridostigmine. Many cite adverse side effects and intolerable reactions to the pyridostigmine as a motivator for switching to Huperzine A. Others still prefer a more natural approach to their medication regimen.

        

It is important to know that Huperzine A has only been clinically studied, and at specific doses, in relation to Alzheimer’s disease. This has no clinical backing in the potential treatment of Myasthenia Gravis in the way of controlled studies. (3)

Let’s dig a little deeper though into how these two treatments break down in our bodies and are used to either our benefit or detriment.

  • Side effects, adverse reactions and cholinergic crisis:
    Within the Myasthenic communities online, you may stumble upon individuals who have made the switch from the synthetic pyridostigmine to the natural Huperzine A, citing they could not tolerate an ambiguously defined set of side effects and/or adverse reactions. This is very important to talk about and unpack as much of the movement in favor for Huperzine A rests right here. Pyridostigmine bromide works by stimulating the parasympathetic nervous system, which inevitably includes an over stimulation of the gastrointestinal tract that can lead to bloating, cramping and diarrhea. (4) For many, these side effects can be harsh and debilitating both inside and outside the home and leave many with social hesitation or anxiety of being too far from the nearest bathroom. Pyridostigmine can also cause some mild muscular twitching and systemic cramping, although these side effects are notably and significantly less than their GI counterparts. (4) Understandably so, this would motivate anyone who experiences such side effects to seek a potentially more gentle alternative that is billed as safer and effective for those who made the switch.

 

  • But, we need to talk about definitions here. Few have ever clarified if they experienced the above reactions and subsequently classified them as “adverse” or “side effects” or if there is more to the picture than meets the eye. You see, pyridostigmine, when taken in a greater dose than what is needed*, can cause what is called a cholinergic crisis which has the potential to produce adverse effects and potentially life threatening scenarios in it’s most severe form. These effects can come about in rapid sequence (usually they begin 45-60 minutes after ingesting an oral dose) and not all symptoms may present in all individuals. Moderate to severe twitching, miosis, excessive salivation and lacrimation, nausea, vomiting and severe diarrhea, severe GI cramping, difficulty breathing, swallowing and speaking, sweating and flushing are all part of the potential presentation. (4) Since it can mimic in presentation with a Myasthenic crisis (disease induced rather than medication induced), it is not always easy to pick up on, even with a trained eye, and can leave many assuming that it is simply an unpleasant side effect of the drug itself. This lack of clarity between true side effect and cholinergic crisis, even in a more mild to moderate form, leaves the Myasthenic with some of the puzzle pieces missing. *Please note that overdose is not the fault of the patient in that it is rarely intentional. As environmental factors and immune fluctuations occur, the need for pyridostigmine can vary and the lack of adjustment to those influencing factors often are the culprit in producing cholinergic crisis.
  • Huperzine A, as noted above, has the same mechanism of action as does it’s synthetic counterpart, meaning that you have the exact same potential to repeat the side effects and cholinergic crisis risks as you did with pyridostigmine. It may take a much higher dose and the impact may be slightly minimized, but the risks are all still there. The selling point it is safer since it is natural. Again, we need to go back to definitions in order to get a clearer picture of what safer with any treatment really means. As it currently stands, “safer” is a loaded word with ambiguous meaning here. We have already talked about it being in the same class as pyridostigmine and working on the same mechanism which, ergo, offers the same risks. Huperzine A still must be processed by the kidneys and liver, just like the synthetic version and it’s renal toxicity is almost identical. (3,4) But we must also recognize that, as a dietary supplement, it is unregulated and that makes it more difficult to ensure safety, efficacy and viability. There are no studies or trials that offer patients or treating physicians a standard for dosing, peaks and falls like we see in pyridostigmine, further augmenting the potential for cholinergic danger and under dosing.
  • The typical half life of pyridostigmine bromide is 3- 4 hours after renal elimination with an estimated half life of Huperzine A to be an estimated 10-14 hours after renal elimination. (3,5) This astounding difference in half life clearance solidifies a marked difference in safe dosing regimens, further augmenting the concerns over the lack of regulation in ingredients and the absence clinical studies to offer up a clear understanding of integral information (ie., when peak release and half-life begins and ends etc..). This leaves both patient and physician zero dosing standard and narrowly contributes a vague and unchecked approach to treatment.

Lack of access, cost and delayed diagnosis can leave many without alternative options other than to reach for Huperzine A and there are those who firmly believe that Huperzine A helps them control their Myasthenia Gravis with fewer side effects than pyridostigmine. While these scenarios and individuals may find great success with their switch to Huperzine A, it begs discernment and caution in understanding all of the potential pros and cons before moving forward.

Author: Rebekah Dorr
Founder & Administrator, Myasthenia Gravis Unmasked

References:
(1) (http://www.mayoclinic.org/diseases-conditions/alzheimers-disease/expert-answers/huperzine-a/faq-20058259)

(2) (Miller RD. Pharmacodynamics and pharmacokinetics of anticholinesterase. In: Rueg-heimer E, Zindler M, ed. Anaesthesiology. [Hamburg, Germany: Congress; Sep 14-21,1980; 222-223.] [Int Congr. No. 538], Amsterdam, Netherlands: Excerpta Medica; 1981)

(3) (https://www.drugs.com/pro/pyridostigmine.html)

(4) (http://www.mims.com/malaysia/drug/info/pyridostigmine%20bromide/?type=brief&mtype=generic)

(5) (https://www.medicines.org.uk/emc/medicine/865)

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

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Myasthenia Gravis Showed Me a New Purpose

“Ma, I can’t open the car door.” Those words were my introduction to Myasthenia Gravis. As my mom looked at me she saw my eyelids began to droop right before her eyes. Trying to open the car door again, my hands slipped. There was no grip at all. At that moment I was rushed to the hospital.

The tensilon test was how I was diagnosed. Upon the injection my eyes flew open and my hands were strong. I don’t remember anything else that day because I was scared. I was 12 years old and documented as the youngest MG patient at Stony Brook University Hospital in NY. It was 1989.

Fast forward two years. I had a thymectomy. I was 14. Most of high school I was home schooled with so many doctors appointments and being sick a lot.

Time went on but my fears were dwindling as I became more educated about MG. I almost died twice due to complications from MG, but I am still here! Then I realized I had a purpose, to help support others with the same illness.

Now at 41, my symptoms have progressed to difficulty walking, climbing, swallowing, toileting, speaking, basically every human function. However, with God’s love, my husband, friends, and my mg support group, I still push on.

I can’t stay home alone anymore so I go to an adult center, but I’m using my story to help others at the center know they can push through. I don’t know what will happen, but I want my fight to be my legacy.

-Shirrel Miles

shirrel and rebekah

Shirrel and Rebekah at an MG Support Group Meeting