[Karen]

I had a port placed >7 years ago; which had to be replaced by 2 “vortex” ports to support PLEX. Like any implanted medical device, there are risks—I’ve had my vortex ports in for 6 years and last year one had to be removed b/c I had blood clots in the subclavian and internal jugular veins. Prednisone killed off my venous excess, so I still have one in place (and chronic blood clots)—fortunately, clots in the arms/neck are less likely to cause pulmonary embolus. I have opted to have an a-v fistula created so I can eventually get rid of the other port—this is not standard; however, I’ve been surviving on PLEX q2wk for the last 5 years and I can’t risk losing access. In the beginning the ports where painful, then annoying and now I don’t even notice it—it is far better then being stuck 7-8 times to find a tiny vein that blows anyway

[Karen]

I was able to get insurance under the Penn State alumni association—many big schools provide insurance options with limited health info—-only only got $65k for $650/y—but it’s better than nothing. Life insurance through your employer (if applicable) many not require any health evaluation—I was fortunate to work for the federal government and can keep my life insurance even though I’m on disability retirement

[Karen]

I have a sign on the window by my front door asking that anyone who enters the house wear a mask (and I have some by the door if they don’t have one). I’ve never had anyone take issue with it.

[Karen]

My total income is too high for any programs, but fortunately I was able to enroll in a catastrophic insurance prior to having issues…while they have a $100000 deductible, they count what my primary insurance pays in addition to what I pay and one the deductible is met the cover everything my primary does pay (I.e copays, co-insurance..) this year is my deductible period and I started rituximab in Jan. My cost is ~$2000 for 2 infusions (loading dose followed by standard dose 2 weeks later… maybe I’m just lucky, but my CD19 count is 0, And I am now taking 1/2 the Méstinon dose..I was previously on plasmApheresis every other week for 5 years with numerous hospitalizations for respiratory failure.. as I’m seronegative (actually LRP4 (+)) I am not able to get Vygart even off label (which is weird b/c my insurance said no pre-approval was ever received, but the company says I can not get it)..after clots related to the ports and now having an a-v fistula for PLEX I’m thrilled at the possibility of reducing PLEX sessions—my husband has a whole life insurance policy that we can borrow from and pay back when we can afford it (they charge interest of course, but rates are still reasonable)—-once we meet the deductible for our catastrophic insurance—- it seems totally in fair that having and income makes you ineligible for aid and the cost of the care makes your actual income lower than that needed to be “eligible” for aid

 

[Karen]

I have been told that I could not switch providers within a group b/c by accepting anyone in the group I was by default accepting all of them as the cover for one another; however, I believe that who you choose as your primary neurologist should be your decision–even if you are taking the chance of having the one you dislike “covering” at some point. Not everybody gets along–I’m pretty sure there is no  “medical necessity” clause requiring a provider to “release you from their care”. At the same time, unless they are the only players in town, I’d want to stay as far away from that practice as possible– you can also contact the board of medicine for your state and get their take

 

[Karen]

I’m glad to hear that it helps you; however the title of this post is. very mis-leading. Blue Light DOES NOT ease MG eye muscle weakness–you explain that you are wearing glass that BLOCK blue light, therefore it is the LACK of blue light that is helpful.

RESEARCH HAS SUGGESTED THAT BLOCKING BLUE LIGHT CAN EASE EYE STRAIN IN ALL PEOPLE.

I agree that trying glasses that block blue light is definitely worth trying. Unfortunately, for me they did not help with my double vision

[Karen]

I’m seronegative..I thought I’d responded to this post earlier but I don’t see it..I’d love to get involved in the zoom meeting.

[Karen]

I was started on Mestinon empirically with a drastic improvement in sx and have not stopped it since—my official dx (SFEMG as am ACH and MUSK (-))

[Karen]

It sounds like most of the respondents are generally in better health than me—I have chronic respiratory failure from MG and up until 2 months ago was sure to walk with 1 crutch around the outside of my house (with numerous breaks) ~ .2 mi, and go up the stairs 1/d (down does not work safely for me—gravity takes over..I have an elevator in the house).

I get PLEX every other week which keeps me at a baseline I can deal with.

~2mo ago I had severe Back pain (to the extend that when I put weight on my right leg I was suddenly on the ground before noticing the intense pain. After a few week of hellish pain and inability to get an appointment with anyone who could do anything helpful (I did get an MR done showing severe foraminal narrowing with root compression on the right side at L4/5 and L5/S1 with moderate to servers narrowing at L3/4. This is due to a congenital abnormality which is now becoming prominent from age related degenerative changes (I’m 54). I finally went to the ED and they admitted me for evaluation— I got steroid injections into L3/4 and L4/5 which made movement and transferring possible again; however I can no longer walk for more than a few steps with my crutch before the pain become unbearable and my leg starts to shake..I finally saw my neurosurgeon (I’ve had prior neck surgeries) who said there is nothing he could do given my MG and the risk of making things worse.

I’m staying as active as I can and do my PT exercises. I’m managing the pain with advil and gabapetin (Neurotin). I’m really concerned that I won’t be able to walk again.

Is anyone starting/ has started from a functional level like mine? I want to try swimming again, but my pulmonologist. Is against as I’ve had numerous respiratory infections and tend to micro- aspirate and with the heat getting out of a pool may not be feasible.

I’ll keep moving but want to do more

[Karen]

I didn’t consider low pressure as I don’t live in a high altitude; however, I cannot fly b/c they don’t pressurize the planes well enough. I found out the hard way on a short flight (Orlando to Richmond VA). I was hard to arouse when we were near Richmond (my husband just though I fell asleep)— I was having problems breathing. Fortunately I had my Trilogy as a carry-on-he got it, I put it on, and by the time it was time for us to “de-plane” (last b/c of my wheelchair) I was feeling much bettter and could take the trilogy off.

[Karen]

Definitely HEAT takes me out—above 80 degrees results in emended fatigue—84 F in the sun and its too much—I overheat (for some reason I do not sweat much at all—my skin gets very hot) and I need to get cool quickly and am out for the rest of the day.

I use a cooling vest (Polar products?) so I can go out at all in the spring/summer (I live in VA).

I missed all of my kids graduations and many sports event b/c I can’t tolerate the heat

[Karen]

Hope is what I desperately need!

I am very disappointed that Vvygart (efgartigimod alfa-fcab) was not approved for sero-negative patients. While clinically, it is “loosely” referred to as an in-vivo PLEX (PlasmApheresis), I strongly believe it should be available to all MG patients who have responded to PLEX as well as those in the clinical trial participants.

That being said, I am being keep alive by PLEX every other week—my ports are old >5-years, I have clotted off several veins in the upper arms and neck (likely due to the presence of the ports) making replacement of the ports a risky option.

I had an A-V fistula (like they use for dialysis) placed in my L arm so that the ports can be removed and they can “clean-up” the clotted areas; but it is still maturing, and has clotted 2x’s already.

There are no other options now—I use a Trilogy at night and for 1-2 hours during the day and will now be started on a negative pressure (Hayek) non-invasive ventilator b/c I’m desaturating at night. Any lung infection could take me out and I’m slowly losing ground.

I want to be alive and functional for my youngest son’s college graduation (2024). At this point its a toss up on who will die 1st, my 85 year old healthy mother-in-law or me (54) and the odds are not in my favor.

Being told not to get stressed about it is NOT helpful.

[Karen]

I am sero-negative and after >30 years (officially ~15 years..the rest of the time it was “all in my head”) my baseline now means I can’t chew much, have chronic respiratory failure due to muscle weakness, extreme heat intolerance, fatigue that great limits any activities after ~3pm ( even after my daiy nap)…I’m extremely mad about the FDA excluding seronegative patients for the new tx

[Karen]

…while not “illnesses” per se, I am also diagnosed with neurogenic bladder, chronic constipation (requiring a “cleanse” every 4 days), esophageal dysmotility, GERD, gastroparesis along with my seronegative MG…and also AVN of my R hip from years of IV steroids

[Karen]

I have sero-negative gMG and am surviving due to PLEX every other week—I’m really disappointed that the FcRn antibody treatments are only for ACh (+)…. As they are considered “in vivo” PLEX (blocking formation of IgG only instead of removing all antibody types) it should be made available to all patients with MG in whom PLEX shows benefit. While statistical significance was not achieved (likely due to low sample size) a trend of improvement was noted—all the other FcRn antibody drugs in development are excluding seronegative patients from their trials— essentially writing us off…being a rare subgroup within a rare disease drastically reduces our treatment options—this is NOT acceptable. Sorry for the rant PS I had a difficult time establishing an account and waiting to get “verified”—I’m not sure what the totally # of users here is; but at best you can expect ~10% of that # as the max. Based on typical clinical trial enrollment, a more realistic estimate would be around 20% of that 10%— but count me in!—Karen (I do not know how to PM in this platform

[Karen]

I’m not sure how to go about it, but we must petition the FDA to reconsider approving the drug as all current clinical studies have excluded seronegative patients; alternatively we could petition the drug company to perform a clinical trial in seronegative patients, but that is not cost-effective for them; therefore unlikely.

[Karen]

The mechanism of action is to block the FcRn (neonatal (formally fetal) receptor) which is needed to create IgG antibodies— almost all auto immune diseases result from production of IgG antibodies which erroneously target and interfere with the normal function of a receptor (such as the ACh receptor in MG). There is no reason to suggest it would not work in patients who are seronegative in whom PLEX works. The data submitted to the FDA showed a trend of improvement in the small number of seronegative patients included; however, statistical significance was not achieved. I’m not sure how to go about it, but we must petition the FDA to reconsider approving the drug as all current clinical studies have excluded seronegative patients; alternatively we could petition the drug company to perform a clinical trial in seronegative patients, but that is not cost-effective for them; therefore unlikely.