[Douglas Manning]

I like the term refractory MG. It does identify the likely low percentage of those of us with generalized MG who have mostly been through a ‘been there, done that’s experience with treatments. My neurologist used the term “refractory” to describe my case back in 2017. As mentioned by others in this string, at times, the treatments can be worse than the disease. After going through the whole gamut, I am fortunate enough to be minutes away from an Apheresis center so that I get a plasma exchange every 10 days followed by an infusion of Soliris. I’m also on a 20mg/daily dose of prednisolone and buckets of pyridostigmine (780mg total between 60mg and 180mg ER caplets). The good news, I am stable and haven’t had a crash since 2019! Life is fragile, but cherished

[Douglas Manning]

As I reach tiring points throughout each day I will experience diplopia and dysarthria regardless of where I am in a treatment cycle. They are almost like old friends after managing MG for 7 years. But the one symptom that continues almost unrelenting in my 7th year is chewing and swallowing fatigue. As others in this stream have commented I have had swallowing studies to confirm that my swallowing is quite disregulated and so far we haven’t found an answer. The choking and coughing makes we weaker which the exacerbates other symptoms. It has also caused 2 aspiration pneumonia episodes this year. So, yeah, swallowing is always hard.

[Douglas Manning]

I was 58 when diagnosed with Achr+ MG. I began with double and blurred vision, difficulty speaking for more than a few moments at a time, chewing fatigue, swallowing issues, that progressed to sided weakness, and fatigue. It progressed quite quickly from first symptoms to my first “crash” about 6 weeks later. I do not believe anyone (except the Opthalmology Resident – yep, that’s right) took the initial diagnosis too seriously and believed we had time to react. After the first crash, I was placed on high dose steroids, twice weekly PLEX, felt better and started on Cellcept. I crashed a second time 6 months later. During the second admission (and second round of being placed on vent), a CT scan indicated that I had an enlarged thymus gland. After discharge, an MRI with contrast indicated an enlarged thymus gland. It took a few more months of back and forth and ongoing gMG symptoms, but a laparoscopic thymectomy was planned. I find no fault with my surgeon in whom I had great trust, but the thymectomy was awful. My lung was nicked and ended up collapsing, and the thymus gland was huge and the procedure turned into an open chest procedure and although found not to have a thymoma, it had began to spread out and attach to lymph glands in my left armpit and parathyroid glands. In short, a long extensive surgery. I had to recover from the surgery and collapsed lung. I also went through a year of AFib before it finally went away.
If it all sounds awful, it was! The good news we were able to begin tapering down on steroids, and PLEX treatments slowly went from twice weekly to once per week. We continued through trialing every ‘tool in the box’ with the most ironic moment being a paradoxical response to retuximab when I had two crashes.
So, although it sounds like I might think otherwise, I would definitely recommend a thymectomy after age 50 if your case progresses to unstable gMG. For me just to be able to reduce the steroids was a win.
The good news is Soliris came on the market and now I am stable with biweekly PLEX and Soliris infusion treatments, 12mg Prednisolone daily, and a mix of long acting and standard Mestinon to address basic symptoms.

[Douglas Manning]

Unrepentant optimist and lifelong learner.

[Douglas Manning]

I have a two-part strategy to help with these paperwork jungles.

1) Like David in this thread has said, I am also most fortunate to have my spouse attend almost all appointments with me, so I begin forms and she completes them if I tire or cannot see well enough.

2) I am able to keep most of my medical care within the same medical system, and our system (Geisinger in Pennsylvania, USA), has a marvelous electronic patient records system. This allows us to print out a complete abbreviated health summary. This way, I just put my name on whatever form I am asked to complete and just write in “see attached health summary.”

My spouse and I often wonder if the physician we visit EVER looks at these forms that are often completed hastily before an appointment. So the attached printed summary is more complete and legible.

[Douglas Manning]

I’ve had ports (Powerflow Apheresis ports) in my upper right chest since the summer of 2018. They have been an absolute benefit for me as my gMG is tough. I need biweekly Apheresis treatments and eculimazab infusions. It makes access so much easier, nearly painless, and virtually foolproof. It is also wonderful to have my arms free during treatment and it takes away stress on the caregivers who need to access my veins for treatments.

I’m tall and relatively broad-shouldered so, I cannot say that I have ever felt as if the ports restricted my movement, nor do I find that they are intrusive when I lay on my back, side, or stomach. I also really like that the access points at my elbows no longer look like so beat up, (On more than one occasion I’ve seen ER staff have raised eyebrows when they need to do a blood draw and almost immediately ask me if I have use illegal intravenous drugs). I do have a small set of bruises on my chest from the ports because they are accessed so frequently, but that is not a bother to me.

Perhaps the biggest advantage to the ports is how much faster my Apheresis treatments are because of the higher flow rates made possible due to the ports.

I’ve have only had the ports clog on a very few occasions, and each occurrence was quickly rectified through a TPA treatment of the port.

SO, I can think of only positive things to say about my experience. I hope that if ports might be recommended for you that you will have the same excellent results.

[Douglas Manning]

Wow! You’ve encapsulated the whole of the generalized MG experience. Like you my “work” was in large part how I identified myself. I saw that role hammered away both by my age and the disease. MG is such a ‘silent’ disease, it affects us in such profound ways and yet from the outside people say, “Well you don’t look sick….” Ugh, how depressing, deafening, defeating and disheartening this can be! My work was exciting as it was one that allowed me to be active, interactive, and instructive. But it was one that meant I was on my feet most of the day, having to ascend and descend stairs / ladders throughout the day as well as bending, reaching, lifting and keyboarding. All of the while dealing with blurred and double vision throughout the day, weakness that made stairs and ladders daunting. All the younger engineers and technicians working for and with me were all to ready it seemed to chalk it up to my age while failing to see the disease. Layer on top of that time away from work for apheresis treatments and infusions and the multiple “crashes” (once a year it seemed for the first six years), and I was all but relegated to the ‘dust bin’ career path.

So, like you it gave me a chance to reflect on my whole life, my self worth, and what value I had to give. In a stroke of luck, the enormity of my ongoing symptoms and the pile on secondary infections and interactions with my other autoimmune diseases allowed me to accept permanent disability. What doors it opened form me! It allowed me to reenter the lives of my adult children in ways that they found helpful; it allowed me to be more active in the life of my church family in ways that didn’t require my ‘physical’ skills. Finally, it allowed me to understand the meaning of grace. So many people, beginning with my family, have been my eyes when I could not see, my legs when I couldn’t walk, my driver, my caregiver, my teacher! I’ve grown in ways I never thought possible. I do really miss what I had to leave behind, but in the end, I discovered that my work did not need me, it only used me as long as I didn’t ask for too much in return. Conversely my new life is one where I not only still use all my gifts but the energy that comes back to me tenfold! In my clear-eyed hindsight, I could not have asked for more, (although I would be lying if I didn’t wish that MG could now go away!).

[Douglas Manning]

I think the Muscular Dystrophy Association gives the best explanation of the conundrum that MG is not hereditary but has a genetic component:
What is the genetic susceptibility in MG?
Although MG and other autoimmune diseases are not hereditary, genetic susceptibility does appear to play a role. It seems likely that genetic factors also contribute to the pathogenesis of MG. Certain human leukocyte antigen (HLA) types, cell-surface proteins that are responsible for the regulation of the immune system, have been associated with myasthenia, including HLA-B8, DRw3, and DQw2. MuSK antibody-positive myasthenia is associated with haplotypes (clusters of genes inherited together) DR14 and DQ5.

Most studies suggest that if people have a relative with an autoimmune disease, their risk of getting an autoimmune disease is increased — the closer the relative, the higher the risk.

Even for identical twins, however, that risk is relatively small. Most studies suggest that when one twin has an autoimmune disease, the other has less than a 50% chance of getting the same disease.

Also, people who already have one autoimmune disease have a greater risk of developing another one. It is estimated that 5% to 10% of people with MG have another autoimmune disease that appeared before or after the onset of MG. The most common of these are autoimmune thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus (a disease that affects multiple organs).
References

1. Carlsson, B., Wallin, J., Pirskanen, R., Matell, G. & Smith, C. I. E. Different HLA DR-DQ associations in subgroups of idiopathic myasthenia gravis. Immunogenetics (1990). doi:10.1007/BF02115001
2. Niks, E. H. et al. Strong association of MuSK antibody-positive myasthenia gravis and HLA-DR14-DQ5. Neurology (2006). doi:10.1212/01.wnl.0000218159.79769.5c

Borrowed from the website: https://www.mda.org/disease/myasthenia-gravis/causes-inheritance

Hope this is helpful.

I believe I am in the category that I have another autoimmune disease (celiac), and MG piled on in 2014 at 57.

[Douglas Manning]

I feel remarkably fortunate in my journey. At the first onset of symptoms in 2014 I knew nothing whatsoever about MG. I had very swift and severe onset of symptoms and at age 58 I mostly feared I was having a stroke with the vision, swallowing, sided weakness all happing at once. At the ED, once stroke was ruled out the Opthalmology resident after a thorough exam, determined that all my symptoms pointed towards MG. A blood test was ordered and I was AcHr+.

The Geisinger system here in central Pennsylvania was able to get me an appointment with neurology within a week, and my treatment regimen began. As luck would have I had my first myasthenic crisis about 10 later and had my first hospitalization and experience with a vent.

Despite this traumatic first month, I’ve always felt that I’ve received the best care possible. I have a wicked case of generalized MG and thanks to the support of the Geisinger team, my spouse and family I thrive mentally despite the physical challenges the disease has given me. It is also great that despite our wonderful rural setting, Geisinger offers a class of care that has supported me through multiple crisis events, secondary infections, the onset of other conditions, I feel safe and cared for, a state i hope that we all can teach if remission-like periods haven’t come yet.

[Douglas Manning]

Peanut butter cups, peanut butter M&Ms! So easy to eat – no worries about chewing fatigue – just holes in my belt, 😂

[Douglas Manning]

I really appreciate the comments regarding:

1) The journey for each of us with MG is unique for each of us,

2) The “toolkit” of medicine available to physicians is filled with medicines that met a standard that was a patient receiving the medicine during clinical trials had an improvement that was statistically significant compared to not taking it. As stated the percentage improvement has a broad range. I think that wide range is reflected in the wide range of the state of control that each of us lives within,

3) Having a physician/specialist who understands our disease state is so very important, but even more important is that we each learn as much as we can so that we can be a contributing part of our healthcare plan.

4) Every medicine has a plus/minus impact on our lives.

For me, prednisolone is the foundation medicine in my cart. I’ve tried and failed to get positive results taking every immunosuppressant and none knocked out my ACHr + status until we started Soliris. But even now I still require plasmapheresis every 14 days and 12.5mg prednisolone. Like others the much higher doses of prednisolone has gifted me with diabetes, cataracts, multiple muscle tears, and lots of miscellaneous infection events, BUT I’m still here! And I can feel the lack of a dose of prednisolone within hours if I miss a dose.

I do hope for the next medicine that might allow prednisolone to be in the rearview mirror… someday.

 

 

[Douglas Manning]

Life’s ultimate marathon

The chances for joy just go on and on and on.

[Douglas Manning]

<p style=”text-align: center;”>I’ve had both plasmapheresis and IVIG treatments. I am Achr+ and have fun the full gamut of treatment schemes since first being diagnosed and then experiencing my first “crash” about a month after first symptoms 8 years ago. Initially high dose Prednisone and plasmapheresis got me past the first crash without requiring ventilation. I left the hospital with an Rx for Mycophenolate, Mestinon, and Prednisone and received plasmapheresis treatments two times per week waiting for the Mycophenolate to come up to speed. It never really did and I had a second crash and need for ventilation about ten months later. Prednisone dose was increased and plasmapheresis frequency was increased to two times one week and three times the next for two months. During this time a series of scans identified that I still had the presence of a large thymus gland (without thymoma). So, thirteen months after initial diagnosis, I had a thymectomy and the surgeons discovered that the gland had developed ‘tendrils’ that appeared to connect to my parathyroid glands as well as lymph nodes in one armpit. The surgery was extensive to separate the the ‘tendrils’ from those areas; two of the parathyroid glands were removed and the work to free the thymus from other connections seemed successful. At age 59, this was a lot! So, the plan was to stay on a higher dose of Prednisone and the Mycophenolate dose was maxed out and cyclosporine was added. Within three months kidney function quickly degraded and cyclosporine was stopped and tacrolimus was substituted to continued poor kidney function. So that was stopped and my medical profile now lists that class of medicine as a “no-go” for me. So at 18 months post diagnosis I had a third minor crash (without ventilation) and we tried to switch from plasmapheresis to IVIG therapy. For the first month I felt really great, but symptoms returned and despite remaining on elevated doses of Prednisone and Mycophenolate, I became symptomatic after about three weeks. So we went to one treatment each month. At the time, my insurance company made the decision that IVIG treatments at once per month was not cost effective! (You’ll never have to worry about the “death panels” predicted by a certain party in the USA when  we have insurance companies, 😆). So we went back to plasmapheresis at twice per week, Prednisone, and Mycophenolate. At the time retuximab burst onto the seen and it was seen as a possible breakthrough. For many it has been, but for some reason my immune system went crazy and I had my worst crash yet. So, back to plasmapheresis, and continued Prednisone and Mycophenolate. With the help of my neurologist we reached out to a different hospital system for a second opinion. After three frustrating months of verifying once more that I had Achr+ MG they came to the conclusion that we didn’t stick with retuximab long enough. We gave it another round and crashed once more. So, back to plasmapheresis, Prednisone, and Mycophenolate. We at last decided that the Mycophenolate was not doing much so we tapered off and found that indeed I was no worse off it than on it. We also experimented with dose levels of Prednisone and plasmapheresis frequency. I developed a serious infection and crashed again. After the crash though Soliris (eculizumab) became available in the USA and we began to use that. Over the last 2 years we have been able to reduce the frequency of plasmapheresis treatments to once every two weeks. So life is predictable now and I haven’t had a crash since 2019. I tolerated IVIG quite well, as I do the plasmapheresis treatments. As time has passed and I look at the cost of Soliris, it is almost quaint that IVIG was considered too dear (not cost effective). But it is likely more related to the effort and huge number of donors it takes to create IVIG such that it should be reserved for those for whom it is the only workable option.</p>
Whew! Long winded, but to conclude, both IVIG work well for me, IVIG perhaps a little better, but at a rarity and cost that makes living with plasmapheresis, Soliris, and relatively (at last) reduced dose of Prednisone an option I can live with!

[Douglas Manning]

My neverending journey

[Douglas Manning]

The impact on my ability to sing is probably my single greatest “loss” with MG. I can deal with the more limited mobility issues, vision issues, swallowing issues and weakness that pop up, but one symptom that is with me always is getting breathless, losing control of my breath, slurring words after several minutes and hoarse and hushed output. Bummer. Nevertheless, I still love to sing for myself, as well as listening to and take great joy in seeing others use their talents. I keep looking forward to the day when I take a few more steps towards a remission/more controlled state. And even if my “voice” remains elusive, I can still enjoy having music in my life.

[Douglas Manning]

<p style=”text-align: left;”>I think the need for ports is really related to the evolution of your case of myasthenia gravis. In my case MG has been an ever present and relentless traveler in my life’s journey since 2014. The neurologist and team that support me have been positive, helpful, and forward-looking. I have been what I hope is a good natured, knowledgeably informed and compliant patient. On top of that my spouse has been tireless and equally relentless partner with and for me. I share all this to tell you that plasma Apheresis treatments have been part of my life on a schedule ranging from 2 times per week to now when they are once every two weeks. To get me to this reduced schedule, we finally found an add on therapy in Soliris and moderate doses of oral prednisolone and pyridostigmine. In the mean time we’ve trialed IViG, Rituximab,  cyclosporine, tacrolimus, and mycophenolate mofetil and I had a thymectomy.  It has been exhausting at times so after 3 years of very frequent needs for access for treatment we discussed having access ports installed. So in 2019 when Soliris was the better part of a year from approval for use in the United States, we looked at trying to make this one part of my a little easier. After some research I had Bard Powerflow ports implanted in my chest. Oh my, what a difference it has made! It speeds up treatment, access is always a breeze and as painless as can be. So, a long story, but if the frequency of your Apheresis treatments is anything more frequent than once per month, then I would seriously consider this option to allow access for your treatment.</p>

[Douglas Manning]

Hello. For me, swimming is my absolute go to for exercise. I can stretch, do water aerobics, and swim as many laps as I am able. One of my favorite things to do is just tread water. Having been a swimmer my entire life, I find this relaxing and allows me to breathe deeply using the light pressure of the water on my chest as resistance to taking deep breaths. The only negative is how hard it is to get out of the water! Going from near-weightless to once more having gravity – ugh.

I’m also a big fan of circuit training at my local gym/YMCA/Planet Fitness (whatever your preference) for weight-bearing resistance exercises and walking on a treadmill, ellipticals bikes, and most dreaded the stair climber.

You’ll see that I like my exercises to be in a closed protected setting where I can stop to rest whenever fatigue starts to win the day.

As others have said, I stick closely to my medicine regimen. Finally I keep a journal to celebrate milestones, understand better where how my body is reacting, adapting to and my plans. It also helps me pinpoint issues to discuss with my support team.

[Douglas Manning]

What great posts highlighting just how bewildering this condition can be! I am trying to conjure up a “D” word for the invisible nature of the disease. Maybe its “Doesn’t get seen by most people”? How did I change from hiking, running, lifting, and standing for hours at a time and then suddenly in the span of onset and the first “crash” seemingly lose it all? How many times have well-meaning friends and family said something akin to “Well, you don’t look sick at all!”? When the weakness hits you and you can no longer bound up several flights of stairs, or you go to shake hands (pre-pandemic of course) and “miss” because you reached for the wrong hand? I’m sure you can insert an episode for each of the “D’s” when you were met with a perplexed look because your look fine, not ‘sick’ at all. So, as we all manage our personal journeys (I also use music therapy to tame tinnitus – someday this will be listed as a symptom), how do we kindly and calmly educate those who are well-meaning?

[Douglas Manning]

What a great read! My approach has perhaps been a little simpler. My family often jokes that I am too optimistic and as a result, I may on occasion “overshoot the mark”. Regardless, I wake up each day imagining what is possible, not what I cannot do. To that end, my family and I have taken the plunge into researching our family story. We’ve had our DNA testing done and it has launched us on a fabulous adventure of discovery. So, on a day when other things may not be possible, I can log on to our chosen ancestry searching website and discover a few more facts about our family! Sometimes, it’s just for a few moments while I’m resting waiting for some muscles to rebound, or it can be for the better part of day when I am getting an Apheresis treatment and Soliris infusion. Either way, it is focusing on what I can do and what is possible. One thing I’ve learned through this hobby in searching for my family history is that this amazing family from which I’ve come from is that in every generation are examples for me to follow of people who focused on looking at each day and looking at what is possible to do on that day. And it’s given me the motivation to walk a few more steps, swim a few more laps, and even say one more prayer.

[Douglas Manning]

As I sat in my chair today for my final treatment of 2021, I could not help but reflect on the season. Thanksgiving is past and we are coming to the end of Advent Season and what follows will bring us back to the beginning of the great promise. We celebrate our blessings before we renew once more the journey on our road to redemption. So, I begin by giving thanks for ongoing well wishes, aid, and prayers I have received from friends, family and an amazing health-care team. Today I had the blessing to sit by a window during one of my biweekly treatments on a sunny, if cold, early winter afternoon gazing for a time as the last leaves fall along with a Christmas Eve snowfall, dancing across the roof, floating to the lawns, and tree line below. In the moment I felt fortunate that unlike so many of the patients with whom I share time, my treatments are not for cancer. My fellow patients I muse are hopeful that their treatments will end and a new normal can begin. The one thing we do all share is the desire to be better, to get better. That was my greatest moment of reflection – how do we both celebrate and yet yearn at the same time? In a larger sense, how do we transition from being Thanksgiving people to becoming Advent people. I do have so much to celebrate as we passed Thanksgiving week. The infection that caused me to have a knee replacement hardware removed is gone, the infection that took away my ability to walk is gone. I am free of pain, have a new knee, and I am taking steps once more; I am blessed! And yet, I have so many steps to go, and then so much more life to live and myasthenia gravis to manage, but still I am always indebted to family, friends, community, and God. Everyday then I have the chance to celebrate blessings given and the opportunity not only to yearn for one more but to be a blessing to others. Each of us, all of us on every day are a Thanksgiving people and simultaneously Advent people. But I want to be b-e-t-t-e-r. That was my original thought, wasn’t it? For me, it has come in the form of pure joy. I am blessed! The grace bestowed upon me throughout my life makes me strong. My family beginning with the one I was born into and now the one I have created with my wife and children is ever nourishing, and extended family and dear friends fortify us. It is not all pumpkin pie though. It seems our world is gripped by near endless tumult, the planet’s history books reluctantly confirm it if we read thoughtfully. Are we bound forever to be an Advent people waiting on the world to change? I hope not. Instead, I live each day in joy knowing that every hill I climb has a splendid view waiting for me at the top.

I try to remember this everyday – grace abounds – be thankful always – and realize that I am both a Thanksgiving and Advent person who declares hopefully that I will have better health, be a better family member, friend and live in harmony with the community and this disease.

 

[Douglas Manning]

A reply another forum member Norm is so valuable I think. That is to trust your care to a neurologist who has experience with patients with MG. If you are fortunate to have that specialist close to you, so much the better. In my case we reached out to Johns Hopkins neurology for a review of my case when it seemed that I was at point where progress was stalling.

Our experience with Johns Hopkins was less than optimal to be honest. It was a multiple visit process to diagnose that I had generalized MG – surprise, I did, both clinically and via anti–acetylcholine receptor (AChR) antibody (Ab) testing. It felt like I wasted several months and we did not arrive at a better endpoint. At the time the team at Johns Hopkins seemed positive that Rituxan therapy was essentially failsafe. For me it was not. We did follow their recommendations however and for a second time proved that it was not the answer for me.

What we found was that the care team I had in the Geisinger Medical Center was experienced, the most knowledgeable and provided the most holistic care for me. Additionally I am most blessed to have a primary care provider who is both a partner and coach. So, my best advice would be to find a primary care provider who can be your guide and coach as you navigate your care journey. I have access to the best specialist I can imagine, but it is my primary care provider who has taken the time to helped me “call the shots” in my journey.

[Douglas Manning]

What an interesting topic! As I live my life as a patient with generalized MG, my life has all its chapter written before the disease where my only comorbidities were Celiac disease and carrying some extra weight. The chapters I’ve written since have been full of the ink smears of ’caused-morbidities’. I’ve gained weight, developed cataracts, type-2 diabetes, have had steroid-caused (we think) tendon tears, and because of immunosuppression have had several systemic infections, and two debilitating osteoarthritis episodes. All this rides with bouts of ocular symptoms, swallowing, neck, weakness that comes with generalized MG. I’ve averaged one crash a year requiring hospitalization and breathing support.

Treatment has been an ongoing series of trial and trial again – steroids, cyclosporine (damaged my kidneys and pushed me to stage III kidney disease), tacrilimus, Mycophenolate, rituximab (paradoxically sent me into a crash twice), IvIg, pyridostigmine, thymectomy, and Plasma Apheresis. At last, at last, we have been on a ongoing regimen of prednisolone, Plasma Apheresis, Soliris infusions and pyridostigmine to conserve acetylcholine. For the first time in 7 years I feel as if we’ve found a steady plateau AND the last ACHR antibody test showed no measurable antibodies!

So, each new page in my story is written with joy. Life is beautiful (no matter how challenging), and I am blessed to have a family and care team as doggedly determined as me to see what the next  sentence in that book will be. So, no matter what may come, I believe that another step is possible. Please know that despite all the extras that come with MG, you have to keep putting the puzzle together.

[Douglas Manning]

I’ve had experience with feeding tubes, ng tubes, and central line nutrition.

Central line nutrition is far and away the most expensive and was used only when a crash was so severe that the hospital stay and recovery looked to extend for weeks. Although intrusive it is easy to live with and allowed me to work with speech therapy to restore my speaking, chewing, and swallowing.

NG tubes were, for me, easier to onboard and much less intrusive day-to-day and allow speech to happen so much easier! as to worrying about the trauma of insertion I can only say, it happens quickly and so take solace in that.

Feeding tubes are truly intrusive for me as a patient. It’s so hard to talk, makes my gag reflex happen on an hourly basis and is an awful experience if it happens again and again if they are not “left in” for days at a time.

In the end, I would say to face any of these options as bravely as you can with the hope that it is part of getting you out of crisis. If you have the choice, I would choose an ng tube first and as part of your healthcare plan make this option known to family and your healthcare team.