When Life Gives you LEMS

This is what LEMS looks like. This recliner, heating pad, pillows and low light have been my refuge, for the last 4 years.

My first blog post was about the pain I've been experiencing for most of my life. The pain was not just from my severe scoliosis diagnosed and operated on as a child; but also from my body attacking itself, leading to multiple diagnosis' of auto immune diseases. The reactions and comments I have received from friends and strangers who have read it have been heart felt and heart warming. It is exceedingly difficult to open up about things that make you stand out from the rest, especially when your life has always stood out from the rest (and not only because I am close to 6' tall!) A blog can be entertaining, informative, thought provoking and sometimes something that people stop reading in the first paragraph. This post is going to be the first of many that will be quite serious. I do plan on sharing funny stories through my journey, however, as they are what has gotten me through.

Last week I underwent an intensive steroid infusion therapy. I have have whined to my friends in Facebook-land, and I've had some fun with it too.

A Snap Chat filter scared my most gullible friend when I sent her this after she asked me how I felt.

When someone struggles with weight due to all that life has thrown at them, the last thing you want to hear is that you are going to receive a treatment that could cause you to balloon up even more. In the whole scheme of things, which you will soon understand, I knew there was no option. I'm sure many of my friends who themselves have undergone the same intensive therapy due to MS or Cancer, or whom have witnessed loved ones undergoing the same therapy, have probably thought I was quite the drama queen. And I was.  Here I'll explain why.

You see, life has given me LEMS. I love lemonade; but unfortunately I can't make lemonade with  LEMS. What is LEMS? The full name is "Lambert Eaton Myasthenic Syndrome", named after the two scientists who discovered it.  LEMS is an EXTREMELY rare, severe, auto immune disease of the nervous system. About half of the time, LEMS is caused by a tumor. This is why I underwent a full body CT scan a few weeks ago. It scanned from my chest to my pelvis, looking for a tumor. With my auto immune history  and monthly blood work done to monitor everything, my doctors were confident that it wasn't a tumor; but we had to make sure before starting the intensive infusion therapy. No tumors were found and so I "juiced up" with 5 days of 1000 mg methylprednisolone infusions, in a row. (A typical steroid dosage is usually around 40 mg per day for the duration of the treatment). My 1000 mg infusions were the loading dose to start my therapy for LEMS. My therapy will continue with another 1000 mg infusion each month.  While going through it I referred to the A.S.S. effect when asked about my side affects by the nurses. The side effects were Annoying, Scary or Surreal. 

How long has this LEMS been going on?  Honestly, it's been going on for a very long time. For the last 5 years I've had symptoms that have mimicked Multiple Sclerosis. I've had numbness on one side of my body and MRIs have shown areas of concern. For a period of about two years I underwent many tests from multiple MRIs, Evoked Potentials exams, and don't forget wonderful lumbar punctures. (Those were the most exciting due to them having to do a puncture for spinal fluid, someplace where away from my rod and fusion.) Nothing was conclusive. Both my first Neurologist, as well as  one who has practiced for decades ,came to the conclusion that the areas of concern that were seen in my MRIs were probably from the severe migraines I've had. The pattern of demyelination spots found in my brain was more consistent with those that looked like migraine related ones than ones found in most Multiple Sclerosis patients . However, both neurologists also said that the brain is a tricky thing and they could be wrong. The evoked potentials test was inconclusive because of that mighty rod in my back.  The harrington rod blocked being able to interpret how the nerve pathways were working from my brain to my feet.  When my MRIs remained consistent over a 2 year period with no marked change, my original neuro and I decided to shelve it unless I started experiencing worsening symptoms. 

Evoked Potentials Test
Sept 6, 2013
Inconclusive due to the waves being blurred by my Harrington Rod, or the shirt, not sure which.

Friends from my former places of employment remember full well how I always worked in low light. Florescent lights threw me into migraines. My home never had more than one light on at a time. I'd walk around friends houses' turning off their lights when I was visiting. Light made me so uncomfortable.

The other unexplained issue that I was dealing with was EXTREME muscle tenseness. No amount of therapy - PT, OMT, Massage therapy-would help. Many doctors have been shocked and said "it's as if you have rocks in your back."  When I'd try to "do the stairs" instead of the elevator to burn those blasted calories, my leg muscles would freeze up, eventually pulling my kneecap to the side.  Cue another MRI, and nothing was wrong that they could diagnose. Swim in warm water my doctor said; but when I did I could barely turn my head to the side to breath. That time in the pool was incredibly tough to deal with. From being one of the top swimmers in the state recruited by the University of Maine, to being unable to swim the length of the pool, it literally broke my spirit in half.

In the top 3 for the 50 free at our state meet in 1987. Notice how undeveloped my calf muscles were. No matter what training I did, I couldn't strengthen my legs, especially my calves. Lower leg weakness is one of the symptoms of LEMS.

At this same time as my medical issues were flaring, my kids were one by one venturing out of the nest. I had "me time" for the first time in over two decades. Not just being a mom; but a single mom for most of their childhood, will do that to you. What did I want to do with my time? I wanted to travel, I wanted to be active- hike, swim, do 5k's. I wanted to do weekend trips to anywhere. Now, all of the prior mentioned medical issues wrecked major havoc on my finances. Tests, procedures, meds, physical therapy, OMT, massage therapy all proved to leave me with not only a poor credit rating but no fun money despite my grocery bill dropping with my three jolly green giants out of the house.

My boys and I when they were 19, 18 and 21 respectfully. The fact I got them to adulthood in one piece with only two broken bones and two totaled cars is a minor miracle!

Even if I had the finances to go and do whatever I wanted, when I wanted, the truth is I couldn't.  I spent 75% of the time when I was not at work, in that damned recliner. When I'd start working out, I would do okay for a couple of days and then be in extreme pain and back on the recliner again. I described it as pulling a rubber band too far and having it snap back hard, hurting me. The trouble was, I never knew what was too far.  Being an athlete, I always mentally wanted to push myself harder; but physically I was incapable. There have been many phone calls to friends with tears over the years; and I don't cry easily. As much as I wanted to get in shape, a Fit Bit wasn't going to fix this.

So, how did my amazing new neurologist, Dr. Roople Unia, diagnose LEMS? I landed in her office this past spring, soon after starting treatment for my Psoriatic Arthritis. Within days of starting Sulfasalazine my facial numbness came back with a vengeance. My rheumatologist, Dr. Asha Shrethsa immediately stopped treatment and communicated with my new neurologist. After tests upon tests that were inconclusive, Dr. Unia suggested doing an extremely expensive blood work panel to see if I had an auto immune disease attacking my nerves. The words "Your N Type Calcium Channel Binding antibodies were high" have changed my life. I will be forever grateful to Dr. Unia for digging until we found the answer. I'm also extremely grateful for "Obamacare." The reason I have worked so long despite my extreme pain was for health insurance. With the Healthcare Marketplace, I have been able to afford insurance, even when not working. 

In my next post I'll be sharing what the treatment was like- the A.S.S. effects; and what my first week post treatment has been like. The human nervous system is the most insane electrical system to experience a repair & reboot.  For now, know that my recliner has been empty more in the past week than in the past four years; and all the lights in my house are turned on tonight.

Gentle hugs,

Dawn