LEMS and Immunosuppression

[Mary Hudak-Collins]

I was originally on Cellcept and Prednisone before I was diagnosed with LEMS.  They thought I had Myesthenia Gravis.  I weaned off Prednisone shortly after starting Firdapse.  Currently I am on Cellcept 500 mg twice a day and Firdapse 10 mg four times a day.  My doctor hopes to wean me off of Cellcept at some point, when he is sure the Firdapse is carrying the weight of my symptoms.

Before I started Firdapse, the Cellcept and Prednisone did help to some degree, but didn’t make such a significant difference.  It did decrease the severity of my symptoms but not to the point that I was able to do much.  Before Cellcept and Prednisone, they put me on Mestinon (Pyridostigmine) and that seemed to help quite a bit but after 6 days I stopped taking it because it made me sooooo sick that I was spending my entire day in bed, unable to eat.

I’m 60 yrs old and the only medical history I have is 2 C-sxn and a Cholecystectomy.  I have never really been sick other than maybe a brief head-cold.  I began having symptoms on Aug 1, 2020.  Diagnosed with Myesthenia Gravis Oct. 2020.  Requested to be seen by out-of-state Neuro.  Diagnosed with LEMS March 2021.

[Price Wooldridge]

@ashleygregory757 what a timely topic! There is plenty of talk about a booster vaccine for Covid, especially for those immunosuppressed, or over age 65.
With many LEMS patients on some form of suppression, getting a third booster vaccine should be part of decision making in the next few months for maximum protection.
We need to be watching for recommendations and guidance which should happen soon.

[Kristina Patafio]

Hi, thank you for the add to the group. I am currently on 5 mg of prednisone and I do Rituxan infusions around the 9 month mark. I had started back in 2017 when I was diagnosed in the hospital with 80 prednisone then to rehab came out on 30mg, since my infusions and Firdapse I have been able to lower my dose over the past 3 years to the 5mg. This is currently my only autoimmune (I think anyway). This regimen works really well for me.

[Price Wooldridge]

We’re glad to have you. That’s a lot of immunosuppression. I’m glad you’re on IVIG and Firdapse and that it’s working for you.

[Kristina Patafio]

I am actually on Rituxan every 9 months, 5mg of prednisone, and 70 Firdapse. IVIG was a negative effect on me

[RDT]

So timely!

I just had a FU visit with my Neuro yesterday. As I’ve failed multiple standard LEMS treatments, looking towards next steps.

Since I have several other autoimmune diseases & other medical conditions/diseases as well. They are trying to determine if one immune suppressant can cross hit some of my other conditions.

Right now, they are trying to determine which to try first. The 2 options on the table right now are: Imuran vs. Cellcept.

My Rhuematologist just weighed in and thought the Imuran could also cross benefit a few of my other conditions he’s trying to treat like reactivate arthritis (aka Reiters Syndrome), Lyme arthritis, Sjogrens and possibly joint pains from my EDS??

So will be waiting for now to see what neuro decides as they hold a round table to discuss & decide my next steps.  My labs need to be a bit more stable anyways to start. So I will keep the group posted.

In the past I’ve tried:

steroids, Plasmapharesis, ivig, 3,4 Dap, rituxan iv, Humira, leflunomide, methotrexate, sulfasalazine & plaquenil.

I have a tendency to develop pretty serious reactions to medications/vaccines etc. due to bad MCAS (mast cell activation syndrome). Also doesn’t help my 2 primary immune deficiencies of CVID & IgA deficiency or my myeloma!

🤦🏻‍♀️ Good Grief! 🤦🏻‍♀️

 

I do think our zoom meet & greet was very helpful! Hearing others experiences with LEMS was very insightful to my own journey!!

Thanks for holding it!! 🙏🏼🙏🏼😇

[Price Wooldridge]

@rdt we’re glad you were able to join us for the first Zoom meet. Best of luck as you try a new medication. Keep us posted and hopefully join us again next month and give a report.

[Ashley]

So Sorry for the delay on my part everyone!

Hey @mhudakcollins !

Thanks for sharing with us! Do you feel that the firdapse and cellcept keep your symptoms pretty well controlled? I also tried high dose steroids for a few months (there was an IVIG shortage and I went without it for 3 months in 2019) but they didn’t help much. So far, IVIG is really the only thing that made a huge difference to me!

I’m glad you were able to get diagnosed correctly, although I’m sorry it took a long time and you were stuck with treatments that didn’t help. Hopefully things are smoother now!

 

Hey @pricewool !

Thank you! Stay tuned for a topic from me tomorrow on the third booster vaccine!

 

Hey @kpatafio !

Welcome! We’re so glad you found our community! Wow! I’m so glad you were able to lower your prednisone dose! I keep hearing a lot of great things about Rituxan which keeps me wondering if it might be a better option for me. Do you experience many side effects from it? I’m glad to hear that it keeps everything controlled for you!

 

Hey @rdt!

I’ve heard good things about Cellcept. Maybe you can talk to @mhudakcollins about her experience with Cellcept. I know we all react differently to medications, but maybe she could give you some pointers or a better idea of what you might expect. I did read that Rituxan cross treats LEMS and other AI conditions, but I know you had a reaction with that so it’s unfortunate. Hopefully you will have better results with immunosuppression, please keep us posted! I’m so glad you enjoyed our meet and greet! Hope to see you again next month! 🙂

[Mary Hudak-Collins]

Good afternoon all!  The Cellcept with Prednisone did a pretty good job…gave me the opportunity to be out of bed and wheelchair and allowed me to do 1-2 light tasks about 3 times a week.  I still had a lot of symptom issues, but the combination helped some.  When I started the Firdapse, my neuro weaned me off Prednisone but wanted to keep me on the Cellcept until I was at a point I could also wean off it.  I’m just now in my second month of Firdapse and am at 40 mg/day.  We have that spread out throughout the day at 4x/day.  I’m not quite where I would like to be at yet so I’m just taking one day at a time.  We’ll see how next month goes for me.  But yes, the Cellcept/Prednisone combo helped but I had to be on a higher dose of Prednisone so that wasn’t ideal.

[Price Wooldridge]

@mhudakcollins just from my perspective, I’m glad to hear they’ve reduced the prednisone. Be sure and understand the long term effects of being on a steroid like this. It’s also good to hear you’re on Firdapse. If you think it’s good where you’re at (40 MG Daily) then fine. But do know you may be able to titrate higher if needed, with the coordination of your neurologist. Maximum daily dose total, per their fact sheet, is 80 MG.
I hope you continue to improve!

[Mary Hudak-Collins]

Having been a healthcare professional all my adult life, I’m familiar with most medicines and side effects.  If I’m not, I am very proficient in doing my research before making any decisions.  I didn’t particularly care for being on any steroid, but it did help my issues with my eyes.  Since being off of it, the droopy lid and blurred vision is back…not necessarily as bad as it began but bad enough to keep me from driving.

I do understand that I can increase my dosage of Firdapse, with the max being 80 mg/day.  I’m not satisfied at 40 mg/day but my doctor wants to slowly increase…month by month…because of my hypersensitivity to medications.

I read testimonies where people have returned to work and their ADL’s are minimally affected after starting on Firdapse.  I’m patiently awaiting that point in time when I will be able to say the same 🙂

[Price Wooldridge]

@mhudakcollins I’m glad to hear you’re on top of everything. As an RRT myself, dealing with respiratory issues sometimes requiring steroids, is unavoidable. For me it has always been short courses with a taper. I do understand that at some level of problem, long term steroids may be the only option.
We all have to follow the guidance of our neurologist, and it sounds like you’ll get to where you need to be soon enough. I hope you are able to attain the level of wellbeing you’re after.

[Mary Hudak-Collins]

yes, I agree to some point that we need to follow the guidance of our neurologist…I also believe that we are our very best patient advocate.  I am very verbal with my neuro when it comes to my health.  I don’t let grass grow under my feet.  I always tell people that if they don’t feel they are getting the treatment they feel is necessary or their doc doesn’t listen to them, then look for one that will.  Had I just sat back and listened to the first neuro I saw, I would still be laying in bed waiting for “statin induced myopathy” symptoms to go away.  The neuro I have now is willing to listen, negotiate, and work out a plan together.  He reviews the research I send him and we go from there.  He’s great!  He’s humble and willing to admit when he wasn’t aware of something.  I am truly blessed to have found him.

[Kristina Patafio]

Thank you so happy to be here, its always a great thing sharing info to help others. I have learned a lot along the way with the FB group as well. IVIG had a negative effect for me I was super weak until it would wear off. I had immediate positive results with the Rituxan. They start it very slow for the first of the two infusions. You are premeditated prior for any reaction and I have not had any side effects luckily on it. I like the fact that the infusions last me a good 9 months (it takes at least 4 weeks to get results). Two infusions back to back within two weeks so its not interfering with my daily to do things.

[Tescha]

Good Morning Everyone!!!

I have LEMS no other autoimmune diseases and for the last year I’ve been taking 80 mg of Firdapse,  Prednisone 40 mg-10 mg but recently tapered down to 10 mg yay!  Plasmapheresis and IVIG every 2 weeks.    My doctors are discussing changing me to Rituxan which obviously comes with risk like everything else.  I’m unable to receive the covid vaccination or any vaccination at this time.    I’m going out to Mayo clinic next week and hope they will have more guidance as which treatment direction I should go.  Take care!!

Tescha

[Price Wooldridge]

@tcarr20 Hey Tescha! Good luck with your visit to Mayo. Let us know how it goes for you and the treatment plan you guys arrive at. Plasmapheresis is very invasive so I only do IVIG every 4 weeks. Also, I believe Plasmapheresis is not an option in the VA healthcare system for maintenance. I’ve also opted not to do steroids due to concerns of long term use, and they reek havoc on diabetes which I have. I’m glad you have been able to reduce your dose. I’ve heard very good things from patients on Rituxan. Keep us updated!

[Ashley]

@kpatafio!

Wow! Okay, I was wondering if it was immediate or more like IVIG/Steroids which take a while to fully work. It’s definitely something I will consider, it would be really nice to have an infusion every 9 months instead of every 4 weeks. how long are you usually at the infusion center?

 

@tcarr20

Glad you were able to taper down on the prednisone! That’s Awesome! Keep us informed of your appointment at Mayo and if they recommend Rituxan and your next steps

[Kristina Patafio]

It has been a tremendous help for me. I do 9 months because I get that timeframe out of it. Some people 6 months or even longer. We have figured out through COVID and not wanting to go how long it actually last because I became weak again.  The first of the two back to back doses runs slower to make sure accepting it with no issues then second dose they run in about 2-2.5 hours. For me its an all day affair as I go to U Penn and takes 2 hours to leave drive park and check in. I dont mind because I’m close to the city and that in itself can take even longer travel times

[Ashley]

@kpatafio

I’m so glad it’s working so well for you! That’s good to know! I usually am at the infusion center for six hours two days in a row, so I know the feeling of an all day affair there well!

[Morgan]

I am currently taking 50mg of Prednisone and I started IVIG October 1-4. My Neuro is hoping to wean me off Prednisone if I have success with IVIG. The medications for LEMS I can’t take because I have epilepsy.

I’m newly diagnosed with LEMS but I was first diagnosed with MG.  I was put on Mestinon but it didn’t help. I started having breathing issues and could barely put one foot in front of the other so one of my neurologists put me on Prednisone. It helped immediately but I hate the moon face & weight gain. 🤦‍♀️ I hope the IVIG helps.

[Price Wooldridge]

@morgan1028 As an RRT, I well know prednisone and have used short runs of it myself for gout attacks and refractory asthma attacks. It’s a powerful anti inflammatory, but you’ve experienced the long term effects which aren’t good. The strategy to move you off it, if possible, is good.
I understand your inability to take amifampridine given your medical history.
I will tell you, IVIG (immunoglobulin) is probably the most powerful med in my plan.
We have a whole forum section on it, so read through topics you think may be relevant.
I can counsel you about it as needed, so reach out. I’ve been on it for a couple of years, with all the good and bad of it.

[Desiree Taliancich]

Hello Price! I just got home from 2 consecutive days of plasmapheresis. This was my very first treatment ever. I was diagnosed about 2 weeks ago. My insurance has stalled with approving my IVIG and my autonomic symptoms became pretty terrible so we opted for this “quick fix”. Now onto IVIG hopefully in a few weeks. I am my Neuro’s only LEMS patient. I feel I may need to establish with another neuro in the event the IVIG isn’t as effective as we hope. Scary to read that some don’t respond to it well enough. Besides my autonomic neuropathy, I don’t have any other chronic diseases. Hoping to be able to avoid steroids and the more complicated meds. Hoping I may pick your brain once I get the name and frequency of my IVIG.

[Price Wooldridge]

@deztali1980 Hey Desiree! I hope the Plasmapheresis went well and has given you some goodness back. I suspect many neurologists have only one, or may never have had a LEMS patient. The ones most suited to treat us are neuromuscular specialists. If you establish a good, knowledgeable relationship with this doctor, a change might not be needed. But, I know you’ll keep an eye on it.
Now officially diagnosed, you have a number of treatment options. You can read about many of them here on the forum. IVIG is a well established treatment for LEMS, so I hope you have no issues with insurance. If you get the green light to start, let me know and I can help you through with suggestions and what to expect. Other frequently prescribed meds are Mestinon (Pyridostigmine), and Firdapse (amifampridine). Firdapse is the only FDA approved drug for LEMS. You should definitely talk to your neurologist about this medication. I can help you with this medication as well. If there’s any suggestion of steroids, that’s a big discussion with your neurologist. They have well known undesirable side effects.
Good luck and keep me updated.

[Ashley]

Hey @morgan1028

I hope IVIG went well for you!

If you want to read some about IVIG specifically for LEMS, we have some information on the LEMS home page that you can find here

Or

We have had many topics and discussions in the forums on IVIG and you can view them all here

Hope this helps you! I know this website can be overwhelming with so much information!

[Morgan]

Hi @pricewool and @ashleygregory757!

Those articles and forums are great! Thank you! Yes, it can be overwhelming.

The first IVIG treatments at home went really well. The loading dose was 4 days, then it will be 2 days every 4 weeks. The brand I received is Gammagard Liquid. I was thankful I didn’t have any side effects. Price, I read you didn’t have a good experience with that brand but I know like with all medications it affects everyone differently. I’m curious if the high dose of prednisone I’m taking possibly kept me from having any side effects?

The great news is today is 2 days post last treatment and I seem to have a little bit more strength! I noticed I haven’t had to use my walker as much around the house. 😀

[Price Wooldridge]

@morgan1028 @ashleygregory757 answered your question on steroids. Regarding my issues with that immunoglobulin brand, I remember it well, having fairly significant flu-like symptoms a couple of days post infusion. The infusion provider pharmacy moved me to another brand for subsequent infusions. This is a good lesson to watch yourself during and after (up to several days) for any adverse reactions.

[Ashley]

@morgan1028

I’m SO happy to hear that your IVIG went well and you are seeing the benefits! Most of us are on the every 4 week schedule with 2 days of infusions so we know what that’s like!

Prednisone could definitely be preventing some of the IVIG side effects. I get a small dose of solumedrol with my infusion to prevent side effects