[Bernie]

I have titrated up to 90mg/day.  I take 15mg every four hours, including overnight. I was able to work with my neurologist to obtain the 90mg/day.  He is hesitant to increase it further because of the gu8idelines regarding stroke but he might be willing to increase my dosage if the recommendation is increased.  I am not sure if it would help me or not to take more but I would be willing to try it.  My symptoms are not what I would call controlled, I still have lots of neuropathy and weakness.  Other symptoms have decreased, like lack of saliva and other autonomic issues, but I attribute those to the Rituxan infusions I get every four months.

I lobby for an extended release version of Firdapse, like I have for Mestinon.  Every time I speak with Catalyst I request it and get told that they are not looking at it right now. I know the ER Mestinon makes a big difference for me.

[Bernie]

I’d say #3, some days are better than others.  I wish I could determine some cause and effect for which I get, that would be great.

[Bernie]

I am negative for all antibodies.  My diagnosis was based on SFEMG and symptoms.

[Bernie]

Hi Debbie. I, too live in Washington State. I am near Bremerton/Poulsbo. My health provider is Kaiser. They have an excellent diagnostician in Federal Way, he’s the one that diagnosed my case. I started symptoms in January 2021 and am now being treated with Mestinon, Firdapse and Rituxan infusions. I have no indications of Small Cell Lung Cancer (SCLC) but I am being monitored frequently to see if it appears. I would say that I am at about 25% of what I could do before LEMS. I do seem to be improving slowly so I have hopes of being able to travel and do more things.

Don’t be too afraid. You can live your life with LEMS, it will just be different. Advocate for yourself, do your research and don’t let the MDs blow you off. Even though my neurologist was excellent in diagnosing my condition, I feel that I know nearly as much if not more than he does about this disease.

Good luck. Since we are nearly local, feel free to reach out to me if you want to talk or meet.

[Bernie]

My journey with xerostomia (Dry Mouth).

 

I first started noticing a sour taste in my mouth around a month before any other LEMS symptoms started making appearing. I was concerned enough that I asked my dentist about it during a routine cleaning, but they could not find any obvious causes. Soon after, I started getting the leaden feeling in my legs, upper arms and back. My saliva production continued to decrease to the point I could no longer eat various foods: chips, pretzels, breads, French fries, etc. Those things turned into a solid, dry lump in my mouth that I had significant trouble swallowing. About this same time, I noticed that my gag reflex was enhanced to the point that if I had certain foods that were too fibrous or I got too much in my mouth at one time, I would gag until I almost vomited.  Eating times increased, since I had to chew everything pretty thoroughly before swallowing.  I also noticed that my swallowing muscles would get tired and the longer I ate, the harder it because to swallow. These things combined to give me weight loss.  On New Years Day 2021, I weighed 195.6. By the middle of September, I was down to 167.1.

 

Snacks during the day became an issue if I couldn’t eat essentially anything from a fast food place. My wife started concocting smoothies with fruit and protein powder.  She was concerned about my weight loss and pushed protein at me any chance she could. She changed our menu to have higher protein and minimize things that are harder for me to swallow. I discovered that I could eat wraps, if they had enough moist ingredients (tomato, relish, mayo).

 

Note that I have seen several ENT specialists about my xerostomia.  None can provide a cause other than the autonomic effects of my autoimmune disease.

 

In June of 2021 I was diagnosed with LEMS.  I immediately started on Mestinon(pyridostigmine).  At first, the improvements were subtle but when I had to go off the meds for a few days, they manifested clearly. I went back on and also was started on Ruzurgi.  I started to get a bit of voluntary saliva production within a couple of weeks.  It is nowhere near “normal” but helps.  If I am careful I can have a hamburger now and again, or a slice of pizza. I have since been transferred to Firdapse, which I am not sure does quite the same job that the Ruzurgi did.

 

Since these things decrease saliva, I essentially gave up any alcohol or caffeine. It’s annoying, but I am trying hard to control what I can to minimize variables that are affecting my health. The LEMS meds have started to produce some voluntary saliva, which has allowed me to cautiously experiment with pizza, hamburgers and an occasional sandwich. I have gained a few pounds since the first of 2022.

 

I have an issue when I try to hydrate using water-it makes me worse. If I slug down a pint of water, within 15 minutes my mouth is parched.  If I drink something with artificial sugar or electrolytes, I can do much better.  I typically have a liter of Crystal Light Mango Iced Tea with me wherever I go so I can moisten. It probably isn’t for everyone but works pretty well for me.

 

I started trying various things to keep my mouth hydrated. I was prescribed Salagen(pilocarpine) that enhances the output of my saliva glands. If I take one ~30 minutes before a meal, I can do better with eating.  I also take one before bed.

 

Bed time is tough. I am a side sleeper, and my mouth falls open.  This leads to more mouth breathing and I ended up with a mouth fungus (thrush) that happens when your mouth dries out too much. I decided to try a strap designed for CPAP users to hold my jaw up. It works pretty well and is not particularly uncomfortable. Commonly available on Amazon, they are pretty inexpensive.  I have two so I can always have a clean one.

 

There are various over-the-counter remedies for dry mouth.  Act and Biotene both make full lines of various treatments.  I like the Biotene toothpaste and mouth spray. I do not like their mouth wash, it has a slimy feel.  I prefer the ACT mouth rinse just before bed. I have not had any luck with either brand’s mouth lozenges. I tried Xylimelts, a tablet that is supposed to moisten your cheek but I never got much from them n I tried some products from Smart Mouth, and they were not palatable to me. Oddly, I discovered that using Cepacol throat lozenges helps with the dry mouth, so I typically will have a couple in my pocket anytime I leave the house.

 

Later in 2021, I started having more trouble at night. My sinuses would close up, but it was non-productive.  In other words, I could blow my nose until the cows came home and nothing came out. Since the air gets dry in the PNW in the winter, I started with a humidifier.  It only seems to help slightly.  Then I started treating it with nasal saline, as I knew it would have very few if any side effects. I also started taking Salagen at bedtime to enhance head moisture. This worked for a couple of months. Then I had to use it more and more so I spoke with my Primary Physician and she prescribed me a nasal dialator Azelastrine. That has done the trick so far, although sometimes I still get clogged up and have to take additional Salagen to get back to sleep.

Regarding voice, when my drugs are tapering off I have trouble enunciating.  It also gets a sort of husky tone.  My wife can tell within seconds if I am needing more pills.

Thank you for this forum to share our information.  I hope that some of my data points can help others in their journey.

[Bernie]

I have done the transition.  Unfortunately, my other medical issues have clouded my observations of the effects.  At the same time, I had some AFIB episodes and was put onto Diltiazem, which most certainly affected my Mestinon/Firdapse. I was wrecked. I called them back and asked for something else and was prescribed Metoprolol. It seems to be less intrusive to my LEMS drugs, but I am unsure as to how much that is due to the change in AFIB or Ruzurgi->Firdapse. I may need to do some experimentation and see if I can replicate where I was before all of the changes.

I do find that the Firdapse has fewer side effects than the Rugurzi; tingling lips, tingles in hands, shakiness which goes away within an hour.

The process was well performed by both Anovo and Panther. I was low when it was decided to make the switch.  I was able to achieve the switch without a gap or any real drama.

[Bernie]

Price, I was able to convince my neurologist to try time release Mestinon. You have mentioned that you take one 12 hour pill and three 4 hour pills.  Would you care to elaborate why you do this instead of taking two 12 hour doses?

Thanx!

 

[Bernie]

I have trouble getting good sleep, but for a different reason. My dysautonomia affects my bladder control so I am now getting up anywhere between 3-7 times a night to void. The good news is that I am able to return to sleep pretty easily most times. The problem is of course that I do not get any more than a couple of hours at a stretch, ever. Sometimes it is rather less than an hour.  That’s pretty frustrating, let me tell you.

I have an appointment with my urologist next week and he will investigate other potential causes for this.  However I am not optimistic because prior to the onset of my LEMS symptoms I seldom got up more than once or twice a night. At least we can potentially eliminate “normal ” causes of such behavior.  Then I suppose it will be up to my neurologist and I to continue to tailor medical treatments to see if it can be corrected.

I have tried melatonin and it did not seem to change things much. I tried a CBD product and although the calming effect was nice, the THC component felt just like the nerve tingles I get when I run low on my Mestinon/Ruzurgi.  So, not pleasant.

I do run a vaporizer in my room, as well as taking Pilocarpine just before bed in order to combat my dry mouth.  I also wear a head strap meant for CPAP users, to keep me from mouth breathing.

[Bernie]

When I began my journey into this autoimmune world, one of my first concerns was a CT scan of my lungs showing what they called “cysts”, which I will assume are the same as what you are calling nodules.

Because of my profession, I have been on a lung monitoring program for decades where I would get an annual chest x-ray. Unfortunately these x-rays (which I still have in my possession) do not show the cysts discovered this January. Recent discussion with my pulmonologist tells me that they are monitoring the situation with twice-yearly imaging to determine if my condition is changing. Other than that, he did not seem to be concerned about their long-term effect. If they do progress, I may have cause to go back to my employer for compensation because of work-related damage.

Since I am currently SCLC negative, they will also continue to monitor for that as well.

 

[Bernie]

I just joined the community and would like to attend.

[Bernie]

I was recently diagnosed with AFIB, three trips to the ER so far this year. At first they gave me Diltiazem, which is supposed to also regulate the wayward heartbeat that I now occasionally get. However, it also nullified my Ruzurgi and Mestinon. That was no fun.  They replaced that  with the Metoprolol, which seems to be a little less intrusive to my LEMS drugs, but allowed another episode on Monday. I was told previously that my next options would be either ablation surgery or to start taking Tikosyn, which I would be admitted for observation for 72 hours for initiation. Right now we are pretty wary of this drug that would have that level of hazard, much less spending so much time under care during the pandemic.

I cannot take lisinopril, as I was on it for almost ten years before having a lung reaction to it. The good (?) news is that my bp has been acceptable since I became symptomatic with LEMS.

[Bernie]

Price, I purchased my set of poles back before LEMS for some more adventurous hikes I had planned. I found them on Amazon, a pair for about $20.  They are aluminum and collapse quite small. I am sure you can do better than REI for pricing.

Any more, I do not leave my house without either the hiking sticks or the cane.

[Bernie]

Price, do you know if there is a pathway for existing patients to obtain Ruzurgi after the mandate takes effect?  It would certainly seem counterproductive to take away one medication from people that really need it.  Perhaps this is an FDA question?

I have only had Ruzurgi, but am worried that if I transition my life will become more difficult. It would be nice if there were a backdoor to return to where I am now.

Thanx!