Please Allow Me to Introduce Myself…

[Debbie Lewis]

Hello everyone,

My name is Debbie Lewis. I’ve just received my diagnosis of LEMS last week. That really sent my husband and I through a loop, esp after doing some reading up on it. It’s a scary diagnosis.  After 5 yrs of struggling it’s nice to have a name for what’s been going on inside of me.

For me, it started with intense fire at the base of my throat that made me feel as through someone was choking me. I would  actually choke on my food! This was about 5 years ago. I couldn’t eat or drink without choking or having my food/drink regurgitating several times before actually going down and staying there. Sometimes, hours later it would regurgitate. From there it progressed to not being able to eat fruit, nuts, grains, nightshade veggies, etc. My diet now consists of chicken or beef stock with vegetable stock for 2 of my 3 meals. I can tolerate salads & steamed veggies, about 1 to 1 1/12 oz of meat & sometimes sourdough bread. What doesn’t help me is that I have sensitivities to certain foods that will kick off my Crohn’s.  So I already had a very limited diet, now it’s even more so. Like I mentioned I also have Crohn’s & hypothyroidism, so fatigue isn’t a new thing or is problems with eating certain foods. However, this was different….

In 2017, I had my Lower Espophgeal sphincter (LES) stretched to help with a spasming esophagus.  Helped some along with adding a calcium channel blocker. Then last year, July, I had another endoscopic procedure and had my LES stretched again. This time things went really bad for me. I was extremely nauseous, unable to eat or drink without making the nausea worse. Then my balance, which was never great, started to get worse. I now use a walking stick to keep me steady & feel safe walking distances. Or I would hold onto my husband. Last year, I could walk 5 miles in about 55-60 mins. Now I can’t do 2 1/2 miles in that time. I love to hike and challenge myself to walk faster, hike faster & further. I also used to lift weights now that just wears me out. I now find that any exertion causes me pain, I struggle to take deep breathes, my hands shake esp early in the day. Walking now fatigues my legs quickly. I wake up with my arms feeling like I’ve been lifting weights for hours! They’re so heavy. When I’m tired I’m starting to have trouble to focus. I also struggle to talk. I struggle to pick things up or hold onto to them sometimes. I’m beginning to have a small amount of salivation at the corners of my mouth. Dry eyes too. My voice leaves me or becomes very gravelly sounding. I can see the word in my head but I can’t say it or pronounce it correctly. Being a sign language interpreter this is a problem! I’ve cut back my hours at work because I don’t have the stamina I once had. I have decided to retire before my treatments begin. I’m slated to start IVIG in June because I want to go to GA to see my granddaughter graduate from HS at the end of May. If that hapoens. If not, we’ll celebrate her nonetheless. My dr doesn’t want me to be out around others when I start the infusions because of the suppression of the immune system.  Again, my job would not work with that restriction.  So for June/July/Aug I’m scheduled to get my IVIG infusions.  So my quarantine will continue through Aug. My dr wants me to stay home during those 3 mons.

During my reading about LEMS I realized that the calcium channel blockers were not a good idea nor was taking magnesium both of which I’ve stopped taking. My husband & I feel that either the meds they used for sedation or the act of the stretching caused LEMS to come to the forefront.

I was encouraged to read that being able to walk, hike and travel doesn’t have to end. Life can and will continue. What it’ll look like is the big question.

I went through a series of tests before my Chiropractor suggested checking out the Vagas nerve. So I sought out a neurologist. After waiting 6 months, I got in. At our first meeting and listening to my symptoms he commented on seeing ‘fasciculations’ as was talking. He took 16 vials of blood and did a lot of blood tests. He told us at our appt that he suspected that my nerves weren’t ‘talking’ to my muscles to activate. It turns out that he was right.

Sometime in the next 2-3 months, hopefully,  I’ll see a specialist neurologist who work with rare diseases. I’m still waiting for them to call and get that ball rolling. I’m also suppose to start working with an Occupational Therapist to help me to be able to swallow better.

I’m grateful to have this resource & all of you available to me. I get so overwhelmed with this and feeling like my life is over. Yesterday, was a hard day mentally. But now I know there is hope. I can, in turn, help my family know there is hope. Thank you. I’m looking forward to learning more.

[Dawn DeBois]

Wow @dlewis, you certainly have quite the story- and this is such a scary time to have a rare disease diagnosis come along as well. However, I’m quite positive that once you start your treatments, you will find that the most difficult part of this diagnosis is when you haven’t been diagnosed yet. There are many treatments that help greatly- some of them work in coordination with each other.

As far as your choking- that was why I started IVIG and it was amazing at how quickly it improved my swallowing. I rarely have any swallowing issues now, like maybe once a year?

Having multiple autoimmune diseases isn’t uncommon with LEMS. The good news is that those patients who have multiple other autoimmune diseases tend to have the NT type of LEMS (Non-tumor caused). However, I do hope your neurologist is going to scbedule a CT scan just to make sure.

I’m so glad you found the information about calcium channel blockers and magnesium causing LEMS symptoms to flare- it’s crazy how bad it can get.  I can’t walk up my cellar stairs if I have chia seeds, that are high in magnesium!

We are happy to have you  join us, please take a look around the different sub forums as well as my column, LEMMe Tell Ya. You’ll find lots of info and we will be here to help you process it.  You finally have a diagnosis, so now your neurologist can work on a plan and, yes, many are able to hike and do all the fun things they enjoyed before LEMS- I can!

[Debbie Lewis]

Hi Dawn,

Yes, he is. I’m scheduled for a Chest CT this Thursday, April 30. He’ll do another one in 3 mons and again in another 3 mons. Then if they all come back clear then he’ll do another one in a year.

It’s encouraging to know that the IVIG can help with the swallowing issue. Hopefully,  it’ll help me to being able to form words. And maybe projection? There are days I think I’m talking loud enough but am being told to ‘speak up’. So frustrating.

Thank you for the encouragement.  I’ll be looking at and participating in some of the other forums.

Debbie

[Ashley]

Hey Debbie,

Thank you for sharing your story!

I’m so glad you were able to finally get a diagnosis and am truly sorry that it took so long.

I didn’t have problems with choking, but I believe that is due to the fact that I had such a “short” window to a diagnosis thankfully. I did have problems with serious acid reflux that at times caused my throat to feel like it was narrowing.

I am also a lover of hiking, so I understand how tough it has been to have a decrease in the distances you are able to go now. I really struggled with having to give this up until we got the kinks of my treatment plan worked out, now I am able to hike like I was prior to my diagnosis for the most part (as long as I get my IVIG).

I also find myself losing the “name” of things and having it at the tip of my tongue all the time. Brain fog is a real thing and something a lot of us here understand!

[Ruth Nixon]

My name is Ruth Nixon and I live in Ireland. It’s so good to read what other people are experiencing, as sometimes I feel like I am losing my mind, with the variety of weird symptoms that can change daily. Neither myself, or any of the Consultants I have been dealing with, have come across LEMS. I don’t know of any other cases in Ireland. I read the forums and it’s good to feel that I’m not alone. I feel like such a failure because I have had to give up work due to LEMS, and hiking has been the hardest thing to give up. 2 1/2 years ago I was running marathons and climbing mountains. I would be so interested to hear how you have managed to hike again, Dawn. To get back to that place would be my biggest hope.

[Ashley]

Welcome Ruth! I’m glad you have found comfort in our community, we are happy to have you and you are not alone! I’m so sorry you have had to give up hiking, I had to give it up as well in the beginning of my diagnosis, but now that I have a treatment regimen that works, I’m able to again! Have you tried any treatment or medications for your LEMS? I have been getting IVIG since 2016 and it has been a lifesaver for me and given me my life back.

[Debbie Lewis]

Yes, welcome Ruth! So glad you found this forum. Like you, I’m new to this diagnosis and haven’t started my treatments yet. I, too, am looking forward to hiking and long distance walks again. That is my hope too. You’re not alone. I look forward to seeing you reach your goal of hiking again.

Debbie

[Ruth Nixon]

Hi Ashley and Debbie,
I’m so amazed by your quick response. Thank you so much. I was diagnosed with LEMS in June 2018. But none of the doctors had ever come across it before. I was started on IVIG – Flebogamma, and have been having a 5 day treatment every 5 weeks since then. I was also started on 3/4 Diaminopyridine ( which sounds like what the forums are calling Firdapse)? I also take immunosuppressants, anti muscle spasming drugs and painkillers, but sometimes I feel like the Consultants are just throwing drugs at it because they don’t know themselves. The IVIG has helped a bit, but I struggle with exhaustion, walking , climbing stairs and expending any amount of energy. At this moment, climbing mountains seems like a million miles away but I’m holding on to that hope. I feel like I’m trying to work it out myself, so it is great to have some insight into it from people in the know. Thank you for your response. It means a lot.

[Debbie Lewis]

Hi Ruth,

Well, you’re ahead of me on treatment.  I’ve been struggling with this for 5 years now. I received my diagnosis about 2 weeks ago. In June I’ll start my IVIG treatments with 4 day ‘loading’, then 2 days in July & Aug. Then there will be a reassessment to see if that helped me. I’m sure, like you and others, there’ll be trial & error of medication to see which combination works best for me. From what I’ve read on this forum – everyone has a different combination of meds. Maybe your doctors haven’t found the right combination for you yet. I’m not a doctor so I can only guess. It’s frustrating to be sure.

I’m experiencing the same exhaustion from exertion from walking, climbing stairs (which is avoid or take slowly), hiking or anything. I still make myself go out for walks but my legs feel as though I’ve been walking for hours, when in reality it’s only been 20 mins. I also struggle with balance.  I’m hoping that after my IVIG treatments that’ll stop. But I really would like to not struggle so much with swallowing and nausea when I eat. I’m keeping my fingers crossed. 🤞

I’m glad you & I found this forum, it gives me hope and a feeling of ‘not being alone’ on this path.

Debbie

[Ruth Nixon]

Hi Debbie,
I am not sure if this is the right way to use forums, as I have never used them before, but I was very interested in your comment about magnesium and Dawn’s response about flare ups. Where did you find this information. Are there foods that should be avoided? I find that I have flare ups that I can’t account any reason for, and have been trying to follow a paleo diet, but maybe that’s not the right route to take?

[Debbie Lewis]

Hi Ruth,

My neurologist told me to stop taking magnesium as a supplement and to avoid soy. These make the symptoms worse. Dawn noticed that chia seeds, which are high in magnesium.  She had a bad reaction to the magnesium. If you google foods that are high in magnesium it will list several foods you wouldn’t guess that would be high in magnesium. I have no idea if you need to avoid all of those foods or only the ones you notice a reaction to. Which would mean eliminating them one at a time while avoiding the others so as to make sure you don’t mistake which food item it is you’re reacting too. This is where I’m at in figuring out which ones I need to avoid and which ones I can safely eat.

Good luck! Debbie 🤗

[Debbie Lewis]

Look at this article Dawn wrote about her experience with magnesium. You’ll see a link connected to the word ‘magnesium ‘ that will take you a list of foods high in magnesium.  https://lamberteatonnews.com/forums/forums/topic/diet-what-diet-changes-do-for-lems-symptoms/

Debbie

Diet- What diet changes do for LEMS symptoms

[Dawn DeBois]

Hi Debbie and Ruth,

I’m so happy to see you supporting each other in the forums!

The magnesium concern with LEMS (and MG) is something that is missed by many neuros, unfortunately. My neuro told me the very first thing upon my diagnosis “Whatever you do, don’t take magnesium as a supplement!”  She knew about the issues it could cause and explained to me that with Multiple Sclerosis, patients are told to take magnesium, as well as patients with migraines.  Not ALL magnesium causes this, such as found in foods with trace amounts. The challenge comes when you eat too much of it.  For example, chia seeds- I had a horrible experience with it when I was adding a tablespoon to cranberry juice to thicken it like jello and help with satiety- as it expands and fills the stomach. I was shocked at how difficult it was to lift my legs.  When I’d have a reaction, I’d look up the amount of magnesium in the food. I found dried papaya and dried mango cause the same result with me- dried fruit it’s easier to eat a lot more than one serving, which is where I found the issue thinking I was “eating healthy.”

I literally can feel the difference come on about 20 minutes after eating something high in magnesium. My neuro was nice enough to answer my messages when I’d tell her which food I felt it with and the magnesium amount per serving….Chia seeds have 95 mg of magnesium per ounce. That’s high. Bananas only have 35 mg of mg for one medium banana, so, I have no issue with those. If you google “magnesium content” with the food it will bring it up quite easily.

I’ve learned to look at labels for protein shakes, vitamins and such to make sure I don’t go over 30 mg per serving.  One LEMS patient I knew was having difficulty managing his symptoms, and it turned out he was eating a can of sardines each and every day, with 36 mg of magnesium per can!

Dawn

[Ruth Nixon]

Hi Debbie and Dawn,
Thank you so much for this information. I cannot believe that I have gotten more information over the last few days on this forum about trying to manage LEMS than I have received from any source ( Medical professional or otherwise) since I have received my diagnosis. I have been finding it so frustrating to have times when, like you say Dawn, I have been suddenly unable to lift my legs, when half an hour before I was able to do so!! Sometimes I have felt like I’ve been loosing my mind. I am going to start a food diary right away and monitor those times and see if I can find links. I have already worked out the negative effects of heat but if there are any other triggers, I would be so interested to hear, as this journey so far has been like trying to navigate without a map or compass. Thanks again. So much appreciated.

[Dawn DeBois]

Hi Ruth,

I’m so happy that you are finding the info you need here in the forums! Being ultra-rare, it’s unfortunately ultra-rare to find providers that are well versed in LEMS treatment and management unless they are researchers themselves.  Most neurologists have never had a diagnosed LEMS patient. However, I’m questioning how rare we really are with how long it takes so many TO be diagnosed!

I definitely recommend keeping a daily log of what you are eating, and how much you are “doing”, temperatures, etc to figure out what your triggers are. With autoimmune disease, stress is even a trigger.  When I was only on pyrostigmine bromide (Mestinon), after first getting diagnosed, I was amazed not only at how it helped with strength and alleviated my dry mouth- but I was also shocked when I was stressed over something and the benefits wore off faster than usual.  When I asked my neuro, she told me it’s a very common thing with neuro meds- they do get processed by the body quicker when under duress.  Our entire planet is under duress right now with COVID19- so any declines can also be due in part to that. Stress management is so important.

We are so happy to have you here!

[Ruth Nixon]

Thanks so much, Dawn. That is already so helpful. I was told 2 years ago that I had LEMS, and have been given little or no information on how to deal with it day to day. However, just in the past few days, since reading your post about stress, I have been taking note of the times of the day when I feel stronger and when I can feel the benefits of the 3 4 Diaminopyridine, and using that time more effectively, and not beating myself up when I have to rest. I can see now, looking back on stressful situations, how much it would have benefited me to know about the effects of stress. Can I just ask, and please don’t feel you have to answer if you’d rather not. You mentioned that you were on Mestinon. Are there any other medications that people with LEMS use to counteract symptoms, other than immunosuppressants or the IVIG treatments? I’m trying to see if the 3 4 Diaminopyridine which I believe is a variation of Firdapse? is the best option, or are there other medications I should be aware of? Any further information would be very helpful.

[Dawn DeBois]

Hi @ruth,

I apologize for taking so long to respond. My notifications aren’t working properly at the moment.

The way I look at treatment for LEMS, with what works best for me, is having the medicine that gets more acetylcholine into the neuromusclar junction by taking 3,4 DAP/Firdapse, keeping the amount of acetylcholine from depleting too fast by taking Mestinon, and, for many, something needs to be done to stop the antibody attack that causes LEMS in the first place. If the LEMS is tumor caused, then finding and treating the tumor is supposed to stop the antibody attack (although I know patients who have successfully beat cancer and still deal with LEMS symptoms). For those with autoimmune LEMS, it means adding an immunosuppressant or immunomodulator. I added IVIG first and have since transitioned to sub q Ig. I finally  had most of my LEMS symptoms managed once I added IVIG! I know some people take Cellcept and others take Rituxan. Others do plasmapheresis.  Are those available in Ireland?

[Linda]

Hello everyone my name is Linda. I’m from Massachusetts about an hour away from Boston. I am looking forward to sharing with other people the kinds of things that I have been experiencing. I have been looking for a site like this for a while. My daughter Hayley actually found it for me.

I was diagnosed with LEMS in August of  2011 after much testing. I first noticed a problem when I was in a parking lot and a car stopped to let me pass so naturally I tried to walk fast and realized my legs no longer allowed me to run, it was a very strange experience. Then stairs were a problem I have moved my bedroom downstairs for fear of a fire I would not be able to get out in time. I can’t even walk fast mostly I wobble like a penguin. I was given Dap and I did fairly well on that, good days and bad days but I was functioning and still working. In October I was diagnosed with small cell lung cancer which has a connection to LEMS. I had six weeks of chemo and six weeks of radiation, all was good cancer-free. I lost all my hair which was very hard to go through I would not wish that on anyone. Then I had brain radiation because that is the next place it travels. I can’t even remember how many treatments I had for that. After that my brain doesn’t function the same. I have lost most of my short-term memory. I can remember things from 20 years and now I don’t remember 10 minutes ago. Because of this, I can no longer work. I worked out of my house for years doing data entry and I typed pretty fast. Now I barely know the keyboard and type with 1 finger.

I started taking this new drug Firdapse and I’m not doing very well on it. I have fallen backward on the floor many times. I start walking forward and my legs take me backward. I have lots of bruises, whacked my head on the floor a few times, one fall gave me a fractured shoulder. I can’t lift my legs very high so I trip on things all the time. Taking a shower is difficult just getting in and out. I can’t stand and get dressed anymore I have to sit. In April of this year, I had a scare. I was in my kitchen making a grilled cheese sat down ate it. The next thing I remember I was in the emergency room with a doctor asking me if I knew my name and what year it was. At first, they thought I had a stroke but then determined I had a seizure, I  have never had one before.  Apparently, seizures are a side effect of Firdapse. Now I am on seizure medication and I can’t drive for at least six months which is probably best. My eyesight has gotten worse and my depth perception is really off. I think I am most interested in how Firdapse has affected other patients. Looking forward to chatting with you all!

[Linda]

Hello everyone my name is Linda. I’m from Massachusetts about an hour away from Boston. I am looking forward to sharing with other people the kinds of things that I have been experiencing. I have been looking for a site like this for a while. My daughter Hayley actually found it for me.

I was diagnosed with LEMS in August of 2011 after much testing. I first noticed a problem when I was in a parking lot and a car stopped to let me pass so naturally I tried to walk fast and realized my legs no longer allowed me to run, it was a very strange experience. Then stairs were a problem I have moved my bedroom downstairs for fear of a fire I would not be able to get out in time. I can’t even walk fast mostly I wobble like a penguin. I was given Dap and I did fairly well on that, good days and bad days but I was functioning and still working. In October I was diagnosed with small cell lung cancer which has a connection to LEMS. I had six weeks of chemo and six weeks of radiation, all was good cancer free. I lost all my hair which was very hard to go through I would not wish that on anyone. Then I had brain radiation because that is the next place it travels. I can’t even remember how many treatments I had for that. After that my brain doesn’t function the same. I have lost most of my short term memory. I can remember things for 20 years and now I don’t remember 10 minutes ago. Because of this I can no longer work. I worked out of my house for years doing data entry and I typed pretty fast. Now I barely know the keyboard and type with 1 finger.

I started taking this new drug Firdapse and I’m not doing very well on it. I have fallen backwards on the floor many times. I start walking forward and my legs take me backwards. I have lots of bruises, whacked my head on the floor a few times, one fall gave me a fractured shoulder. I can’t lift my legs very high so I trip on things all the time. Taking a shower is difficult just getting in and out. I can’t stand and get dressed anymore I have to sit. In April of this year I had a scare. I was in my kitchen making a grill cheese sat down ate it. The next thing I remember I was in the emergency room with a doctor asking me if I knew my name and what year it was. At first they thought I had a stroke but then determined I had a seizure, I have never had one before. Apparently seizures is a side effect of Firdapse. Now I am on seizure medication and I can’t drive for at least six months which is probably best. My eyesight has gotten worse and my depth perception is really off. I think I am most interested in how Firdapse has effected other patients.

Looking forward to chatting with you all!

[Price Wooldridge]

Hi Linda!  We’re very happy to have you here.  I am a Moderator on the forum and will continue to do so for another month or so, until the senior moderator returns.
My first thought, based on your story, is please be very careful of falling.  I’ve used a walker before, and it can be be a huge aid.  Also, I would hope you can add bars in your bathroom, around the toilet, and any other aid you may need.  With my first major problem with LEMS, I got all of this, and a shower chair as well.
Regarding Firdapse (amifampridine), I’ve been taking it for quite some time.  I do 60MG total for the day in four 15MG doses.  I believe it to be an important part of my treatment plan, but certainly not the end all.  A common drug given for myasthenic problems is pyridostigmine, aka Mestinon.  I also do antibodies treatments getting immunoglobulin by infusion every four weeks.  There are other options as well.  Please let us know your treatment plan.  And welcome!

[Linda]

Hello Price thanks for the pointers I do have two handles in the shower and I know a seat is next, trying to not to think about that yet. Just wondering if we take the same medicines. I take Firdapse 10 mgs 8 times a day, pyridostigmine Br 60mgs 6 times a day, and pyridostigmine ER180 mgs at bed time.

[Price Wooldridge]

Thanks for your med plan update.  You can discuss with your physician, but I believe 80MG a day is max dose for Firdapse, though I am aware of a couple of folks on 100MG.  And yes, seizure warning comes along with amifampridine too.  I take 60MG Mestinon four times a day along with my Firdapse.  Anecdotal evidence is the combo works well together.  I was having overnight weakness problems as well, and also take the 180MG time release Mestinon before bed.  Something not on your plan I do is 90 Grams immunoglobulin infusions every 4 weeks.  This is a “major” part of my treatment plan.  It provides a load of pooled antibodies to counteract the antibodies issues that come with LEMS.  I hope my plan may shed some light for you.

[Linda]

I have asked my doctor many times is there any other treatmets and Firdapse is all he has to offer.

[Price Wooldridge]

Have you specifically asked your neuro about IVIG?  After Mestinon, IVIG was the second medication I was given.  It’s THAT well known as a treatment for LEMS.  If you don’t get a satisfactory answer, it will be your decision, based on what you’re told, how to proceed.  Be proactive!

[Linda]

I have never heard about infusions and I do take the max amount of Firdapse. I have an appt with my neurologist soon I will inquire about it.