[Dawn DeBois]

Carmen,

I’d be very interested in what the Myasthenia Gravis listing includes for essential oils!

[Dawn DeBois]

Hi Ashley,

I absolutely believe that outlook plays a big role in helping to manage our LEMS symptoms. I recall within the first year of diagnosis, when my strength had returned via 3,4-DAP and Mestinon, I suddenly found my symptoms needing me to take my meds on a shorter time interval than usual. I was stressed about a relationship in my life an d found my dry mouth and the need to take my meds was shortened to just under 3 hours instead of the usual 4-5 hours during the day.  I asked my neurologist about it, including the part about my being stressed when this happened and she wasn’t surprised. She told me this happens all the time with ALS, Parkinsons and MS patients- when they are stressed their meds don’t last as long. The body processes them faster.

Since that conversation I’ve been more keenly aware of how much stress I carry. When stressed, I try to exercise, or blast DISCO music- who can be sad listening to that?), I write in my gratitude journal, get outside, or go find some live music someplace to escape to. If my symptoms are flaring- I try not to stress about it, and just rest and recover, knowing that is what my body needs and refusing to feel guilty about it.

I’ve also learned to also limit how much “daily news” I pay attention to because that can be a constant stressor with the advent of 24 hour news channels.

Lastly, I always remember that there are rare disease patients out there without treatments, without the ability for symptoms to be managed, and who face a dire prognosis because their disease is progressive. I feel blessed every day that if I had to have a rare disease diagnosis, God gave me LEMS. (For patients reading this who may still be in the frustrated mode with their disease diagnosis, if your symptoms aren’t controlled yet, I hear you and pray that the right treatment combination is found for you soon so that you can live your best LEMS life! )

[Dawn DeBois]

That’s an amazing story Carmen!
I’m so glad your neurologist recognized that you couldn’t take either of the amifampridine meds due to your seizures. They are very strong and I have recently heard of a patient who was hospitalized for over a week due to multiple seizures (he has epilepsy and had been taking one of the products.)

IVIG is simply a miracle product for me in regards to managing my LEMS. It stops the antibody attack. It’s tough on a monthly schedule with working full time, I’m sure; but I just last week starting receiving immunoglobulin G subcutaneously, at home! Hizentra is indicated for CIDP, which is the first autoimmune neuromuscular disease indication. Look for my column this week for more info! It may be something you can transition to in order to make it easier to receive the miraculous “liquid gold” and not have the side effects interfere with your life so much.

As for you AIP protocol….OMG……I’d have such a hard time cutting coffee!! I will look into that more, you definitely make a good case! Being single and on disability right now, my grocery budget is small and it’s so unfortunate that eating super healthy is super expensive. Also, I use an NSAID to control my psoraitic arthritis, without it my hands are falling apart and I can’t type! 😉

Thanks so much for sharing your story! I’m sure you will get lots of questions!

[Dawn DeBois]

I learned early on with my LEMS diagnosis that heat was my nemesis.  I can fortunately take hot showers to relieve my neck and shoulder tension; but any hot humid weather that I can’t find relief with air conditioning will bring on crazy LEMS weakness, including facial drooping. I wrote about the experience of trying to save money by taking my conditioners out early on fall in my blog, The Battle Within. I paid dearly for it.

Another LEMS trigger for me is any food that is has a lot of magnesium in it. Magnesium is contraindicated in LEMS and MG patients because it slows down the neuro pathways, and ours are already compromised. When I was doing Weight Watchers, I decided to make a “jello” with chia seeds and sugar free cranberry juice for a more full feeling in my stomach. When I could barely walk up my basement stairs, I looked at the amount of magnesium in chia seeds (95 mg per oz) and sent it to my neuro- she said that absolutely could bring on the flare I was feeling.  My neuro cautioned me from day one about taking any magnesium supplements but many neuros never even mention it. I also found dried papaya and mango have high levels of mg because it’s condensed.

Stress, illness and not getting enough sleep are triggers as well. Those I try to alleviate by managing myself. IVIG has helped me greatly in regards to avoiding illness!  All those extra antibodies are super helpful!

Lastly, not receiving IVIG during the shortage, just like Ashley found, was a real eye opener to the benefit I was receiving. I was shuffling around like a grandpa, barely able to life my legs. I was exhausted, weak, and most of all, I was having trouble with conversations. It turns out the type of antibodies I have, type-N, are highly prevalent in the brain synapses and when they once again took over without the Ig in my system, everyone I spoke to knew something was dreadfully wrong. My first IVIg treatment after the delay immediately brought relief of those symptoms. Some describe “brain fog” as a sign of a flare with autoimmune disease. My term for what I went through with the shortage was “brain paint,”  There was no way to see through it and grab any words I was trying to say. It was incredibly scary.

[Dawn DeBois]

Ashley,

Your resilience and strength in juggling all that you do is going to be of benefit for all LEMS and multiple autoimmune syndrome patients to witness here in the forums as well as in real life. It can all be so overwhelming at times, especially when numerous diagnoses are thrown at you at once. I’m so proud of you and thrilled to have you as my co-moderator here in the forums!

[Dawn DeBois]

Ashley,

Your resilience and strength in juggling all that you do is going to be of benefit for all LEMS and multiple autoimmune syndrome patients to witness here in the forums as well as in real life. It can all be so overwhelming at times, especially when numerous diagnoses are thrown at you at once. I’m so proud of you and thrilled to have you as my co-moderator here in the forums!

[Dawn DeBois]

Price- I need to start putting “The Office” on more. Humor is the best antidote to all of the scary stuff on the news these days. The Aaron Hernandez story was definitely tragic and a worthwhile watch. Many people think that NFL players came from families that could afford expensive training camps, etc. Sometimes people are just born with a gift and get the chance to use it. Unfortunately, demons still can penetrate any amount of success.

Ashley- Yes, I binged YOU when I first saw it available during it’s first season. Very good!  I haven’t yet gotten back into the 2nd season yet. I’m trying, as you know, to read a lot more and meet my Goodreads reading goal of 36 books this year. Starting off 2020 with “East of Eden” took a lot of time away from binge-watching TV; but that’s ok!

[Dawn DeBois]

Carmen,

I’m so glad you are finding the tips helpful! I learned 90% of what made my infusions successful from other patients.

I do need to caution you on the coconut water during your infusions because aside from being a natural source of electrolytes, it is also a diuretic. You need your veins to keep all the extra fluid possible during the infusions and until you get through that post IVIG flu-ish feeling. After I start feeling better, I’ve used coconut water to help release the fluids gained during the process. Another option to Vitamin water is smart water with electrolytes.

Yesterday I started my first sub cutaneous ig treatment, to replace the monthly infusion cycle. Look for me next column to tell you how it went!

[Dawn DeBois]

Ashley,

I am trying to read more; but when the focus is off due to exhaustion, I will turn on Netflix or Hulu!  One Netflix binge I watched in a day was Diagnosis.   We as rare disease patients can relate to all of the patients who had rare medical conditions that no doctors could diagnose, so a New York Times writer decided to give them all a platform to get crowd sourced ideas of what their symptoms might be! Does that sound familiar in the LEMS world?? I also watched Killer Inside, the mind of Aaron Hernendez in just about one sitting and Dead to Me.  I love that Netflix will delve into topic areas that the FCC won’t usually allow over the air!

When I need a laugh, all I need to do is watch a couple of episodes of Schitt’s Creek to get into a better mood!

[Dawn DeBois]

Hi Tescha,

I’m glad Catalyst Pathways has been helpful for you! The PALS are a great asset to the treatment team.

I think that for most patients, Firdapse is first line therapy before going to immunomodulation with IVIg or immunosuppression. I do know patients who are only on Firdapse or only on Firdapse and Mestinon. If a patient is in a crisis, then the neuro may go for the “big guns” right out of the gate with IVIG. Journaling how the medicine affects you and your symptoms based on the dose you are taking will be helpful for your neuro to assess how things are going.

Good luck to you! Please keep us posted on how you are doing!

[Dawn DeBois]

Hi Ashley,

Oh what a learning experience I’ve had with IVIG. People who are prone to migraines, as I am, definitely have a greater risk of post infusion migraines with IVIG. My first few were doozies.  Thankfully my neuro is a migraine specialist so she started me right out with 500 mls of saline before and after, and I took benedryl and tylenol throughout. However, I don’t have Solumedrol added to my infusion because when I had two doses of only solumedrol, my LEMS symptoms escalated prior to having relief. It was really scary and it turns out can happen with some LEMS patients.

Things I have learned since starting IVIG were to drink electrolyte filled beverages during and for a few days after my infusions- Sugar free vitamin water is my favorite. Immunoglobulin is thick and changes the blood chemistry- making sure to hydrate with more than just water is essential.  I also learned to keep taking my tylenol and benedryl for a few days after helped stop a headache from happening.

My migraines are now controlled with a preventative, and that has really helped lesson the severity of post infusion migraines as well.

Oh, and lastly, keeping my infusion rate really slow. The highest I go to is 162- if I go higher I wake up the next day with a headache that mimics Aseptic Meningitis- I’ve never gone to the ER though, I just hydrate, rest, and take tyelenol and benedryl- Sometimes tylenol 3 if need be.

The most important part of all of the potential side effects is the reality that once the side effects subside, my LEMS is controlled. My weakness disappears, and I’ve been able to live a full life. It took me a year to write about all that goes along with IVIG treatment- but I felt it was important to share the full story about being able to live life again thanks to IVIG because it truly is a miracle treatment for me!

I feel horrible for those who don’t start IVIG because of the fears of the side effects.

I’m so sorry the orders got messed up for you on Thursday and am glad they got straightened out so that your entire treatment regimen wasn’t disrupted!

[Dawn DeBois]

Hi Michael,

I’ve lived with the challenge of a spinal fusion and rod from scoliosis surgery when I was 13 complicating having an epidural and going through the lumbar puncture process leading to diagnosis. Put simply, I know the horror all too well, especially if you chance a provider who isn’t meticulous when accessing the spinal fluid. Ugh.

I had to have general anesthesia for my c-sections and then with one of my lumbar punctures, it was termed “traumatic” in the results due to the amount of blood in the spinal fluid sample. I was also traumatized by a 2 week spinal migraine.  The pain of your brain sitting on your skull instead of bring cushioned in spinal fluid is unmatched. So, I can’t imagine being an SMA patient, with a fusion, needing the treatment that works so well but that can only be administered via lumbar puncture? Ugh!

My conversations with my neuro in regards to the possibility of a port for my IVIG treatment were always coupled with concern expressed from her over the risk of infection. Many times an expression says more than the words; and her expression told be I want to explore all other options, first. She would have ordered a port, however, if I requested it. Thankfully I have the sub q route to turn to for my IVIG instead of getting a port at this time.

[Dawn DeBois]

Lori, I hope that you get to meet my friend Christina in real life some day, who has been managing her chronic illness since she was 19. I know you’ve talked but to truly understand that a chronic illness doesn’t have to dictate your life, you just need to see all she has accomplished! Ashley is just like that with LEMS- she doesn’t even let her recovery time from IVIG slow her down!!

[Dawn DeBois]

And we appreciate you and all you do to make awareness of Rare Disease, Romy!

[Dawn DeBois]

Hello Sandra,

Thank you for joining our Forums! Also, thank you for your compliments on the Lambert-Eaton News site. It means a lot! We are starting a large endeavor to improve the overall site experience for all of our sites here at BioNews so keep watching for great things to come! We were just meeting over the last week in Philadelphia in regards to this new initiative, which is why my response is delayed. I do apologize.

Thank you SO much for your feedback about transitioning to sub q. I just received approval from my insurance company to start sub q treatments! Like you, I needed IVIG treatments every 4th week for multiple days in the infusion center, which meant my entire life needed to be scheduled around it. Next week will be my last IVIG infusion, hopefully, before I start training with a nurse on how to self-infuse at home. I am so excited- especially after reading about your experience!

[Dawn DeBois]

Lori,

That is absolutely wonderful to read!!! There is no doubt that IVIG has allowed me to live my best possible Life with LEMS thanks to IVIG. Many patients across multiple disease states refer to IVIG as “liquid gold”…..it is amazing the difference it makes!!

I’m beyond thrilled for her and all of you that love her!!

[Dawn DeBois]

Saline needs to be prescribed but your nurses can suggest it to your neurologist. My neurologist started me out with the saline because I suffer from chronic migraines and so the hydration is exceedingly important. I also have blood pressure drops on IVIG from my already low BP levels so the extra fluids help counteract that side effect.

[Dawn DeBois]

That sounds like a good plan Ben. I was on 3,4 DAP during the compassionate use phase, and kept mine in the fridge as well.  If they are going to go through all of the trouble to ship it in a cooler, there’s a reason! 😉

[Dawn DeBois]

Carmen it’s in the PIS for IVIG, have him look at the dosing for CIDP, which is another neuromuscular disease that they treat with IVIG.

[Dawn DeBois]

I converted your dosage to lbs to figure out where your dosage was set at based on the dose you shared.  Based on your kgs, you should be getting around 60 grams per dosing. 1g/kg . The smaller doses are for those with autoimmune suppression whether from chemo or other chronic illness.  In order for IVIG to work for the autoimmune disease state, the donated antibodies need to flood the system and take over, not just enhance.  That would make sense as to why you aren’t feeling the typical boost.

As far as posting your kgs, it would take someone figuring out how to convert them 😉

[Dawn DeBois]

Oh Carmen, I didn’t put this together. BioNews Services, the parent company of Lambert-Eaton News is doing a lot of work to make this a great site for LEMS patients. They also have pages for multiple other rare diseases. The team is amazing. I just feel fortunate that new patients can find info easier now than I could when I was diagnosed and am happy to be  part of bringing it to the LEMS community.

Sharing info and experiences really helps others. I can’t imagine having moved a lot throughout my diagnositic process. However, once you get the right “team” in place it’s great! I a at a good place with all of my specialists right now and am very grateful for them!

Some patients have been able to get off of most meds based on supplements and diet/exercise. Kudos to you for giving that your all!

[Dawn DeBois]

Oh Beta Blockers are so bad with LEMS!  No wonder you flared so horribly. Speaking of what to watch out for, has your neuro told you to be careful of how much magnesium you get in your diet?  Chia seeds are horrible for me, and other dried fruits. I realize quite quickly when I eat something very high in magnesium due to my body’s reaction.

I saw 3 neuros in the years before diagnosis. Fortunately, my 3rd neuro, and current neuro, is my own Dr. House.

[Dawn DeBois]

You are a teacher!  That’s amazing!!

The loading dose is 2g/kg. The maintenance dose for IVIG autoimmune protocol is 1g/kg.  Unless you weigh 55 lbs, that dose isn’t the correct maintenance dose. Definitely keep your neuro in the loop.  Try not to be scared of your insurance company. They have done the peer to peer review and realized it is needed.  It is normal for it to take a while to figure out your best dosing.

For example, I started on 2g/kg every six weeks. The dosing was horrible migraine-wise. And, because the half life is 3.5 weeks, I’d crash starting around the 4th week. My neuro and I went back to the drawing board and decided to try 1g/kg every 4th week. I get pretty tired in the few days leading up to it but overall do REALLY well with that schedule.

As far as a day early vs. a few days later, double check with your infusion center to see if they can check with your insurance company to see if it would be an issue. I’ve had to tweak my dates before and usually it’s my infusion center that reaches out to my neuro if a new order is needed.

[Dawn DeBois]

Carmen, we are both from the same mindset. I was relieved to find out what had been plaguing me for most of my adult years as well.  To finally have an answer, and know it wasn’t in my head, and a plan in place was a huge sense of relief.

For most people with autoimmune LEMS, it doesn’t seem to be progressive with all of the treatment options we have now. I feel fortunate for that. There are definitely some days that are not good; but when everything is as it should be med-wise, stress-wise, and treatment- with my IVIG, I do really well!  Last summer my IVIG treatments were delayed due to the shortage and I went downhill fast. It was very scary and eye  opening. I’m so grateful every day for that miraculous product! Without it, I would have a very different view of how LEMS was affecting me.