Wednesday, January 21, 2015

2015: Let the "Fun" Begin

I've been contemplating on what I should write and I've decided to write a current update because I'm having surgery next week and writing is a coping tool for me; I know I'll "feel better" once I get it off my chest.

Warning to the squeamish types: there are 2 pictures near the end that might not be your cup of tea. It's NOT blood & guts; just a scapula shifted out of position. I'll post another warning when you  get closer to it. 

As I reflect back on 2014 the first thing that comes to mind is: what a long, hard, crazy, exhausting roller-coaster year!! It was a year full of so much travel seeing physicians that I had been referred to throughout the United States. We traveled approximately 7,542 "medical" miles! These miles were divided between trips to Pennsylvania, Massachusetts, Colorado, Minnesota, and of course, Illinois. Talk about a lot of flying and driving just to see a doctor!!

When 2014 started I thought it was going to be a year of moving forward and having the surgeries that were proposed in 2013 done. Instead of moving forward though, it seemed like there was some higher power constantly putting obstacles in my path so those surgeries would not happen. At the time I was so frustrated and angry at my situation. All I wanted was to have the surgeries done so I could begin the rehabilitation process to regain some of my arm function and get my life back. When you're living with a chronic medical condition 24/7 and all the various challenges and pain that goes along with it, it's really, really hard to see how things could possibly work out because you're too close to the situation. When doctor appointments don't work out, my family and I constantly tell ourselves, "That doctor wasn't the one meant to help me." When I look back now on how all the events unfolded in 2014 I know I had somebody looking out for me the whole time. It's kind of an eerie feeling because the two big surgeries that I just wanted to get done and over with would have never worked.
Fast forward to November 2014. I had finished doing two and half months of extensive physical therapy in Colorado trying to narrow down what is wrong with my neck, upper back, both shoulder joints, and both shoulder blades. It was now time to head to the fourth state to meet with yet another doctor; this time we were off to Minnesota. I can't begin to express how scared, anxious, and nervous I was. I felt so sick the day of appointment due to the nerves.

November 17, 2014
Total game changer- Thank God for Dr. E! He has seen 6 other patients similar to me. All 6 are female, have Ehlers-Danlos Syndrome (EDS) or a cousin of EDS, are tall/thin, were once very athletic, and problems started at around age 16. It's a very difficult problem to treat and it's really hard for me to put into words what the issue is. In layman's terms, it's a very, very, rare, messed up/altered muscle activation pattern that Dr. E has only seen in this category of patients. The altered muscle activation pattern causes the scapula (shoulder blade) to "dance" because there is no stability. This causes both scapular and posterior (back) shoulder joint instability, decreased strength, and very limited motion. The first step of trying to treat this was to have botox injections into my trapezius and serratus anterior muscles. It was supposed to take about a week to kick in, would peak at around 3-4 weeks, and would take 3 months to be out of my system. As luck would have it, I reacted completely opposite of what the botox was supposed to accomplish. Things started feeling worse on day 3. Instead of relaxing my muscles it made my muscles ridiculously tight, decreased my motion even more, and skyrocketed my pain. I kept trying to tell myself it's only been X amount of days/weeks there's still a chance it will get better...time would tell. In regards to my neck, there's a major problem and I have "extreme hyperlaxity" in it. A neck brace was ordered and we're trying to see if it will stiffen up my neck. I don't think it's stiffening too much but it does help with some of my symptoms which I'll take. In regards to my right side it too has this weird muscle activation pattern, but it also has some weird nerve problem that is still being investigated.

When we arrived home from Minnesota, Thanksgiving was literally just a few short days later. It was the first time my mom and I had been home since August. It's kind of funny that the only picture that was taken of me on Thanksgiving was when I was messing around with our dog Daisy and not with anyone in my family.
I tried to get it so it looked like she had on a babushka :)
Apparently my nephew thinks my neck brace makes a good mask too :)
In December I was really not feeling well at all. Between the botox and the climate change my body was reacting negatively. My joints in my upper torso were more unstable than they were when I was in Colorado. When I was in Colorado I felt better and had even gained almost five pounds which was a very good thing. I've been trying to put more weight on. Within a few weeks of being home I lost the weight I gained, plus some. I emailed Dr. E to see when I was supposed to come back for a follow-up appointment. He said in January or February depending on how I was doing. I scheduled an appointment for January 7th. I had called mid December to see if I could get in sooner and the good news was there was a same day cancellation! But wait....the bad news....the appointment was at 4 o'clock and it was already 11:30. I didn't have enough time to get from Illinois to Minnesota. Did you really think I would get in sooner?..haha that would be too easy :) Too bad we don't have those tunnels like in Super Mario that transport you quickly from one location to another :) That could have been pretty convenient.
Me, my niece, and my dad at the holiday zoo lights.
There's no way I could walk the entire zoo with all the
upper body stuff going on so we rented a wheelchair. It's
way more enjoyable to be pushed around than to be really
stubborn and hurting a ton trying to walk.
Also, scarves work really, really well to cover up the neck brace
if you're self conscious about it!
On Christmas Eve I got the most amazing tea mug from my brother and sister-in-law. I use it just about every day and every time I do, it always makes me smile.

So true haha
My favorite picture of my nephew Justin. His facial cracks me up.
January 7, 2015
We were back at Mayo in Minnesota meeting with Dr. E. I was reevaluated and the botox did in fact make me worse and he had never seen any reaction like it before. Gooooo figure! My parents and I were told I have a lot of problems and despite all the previous surgery I've had, surgery is needed. Instead of going into the surgery with the mentality of, "I'm going to fix everything" Dr. E made it very clear that he only wants to fix one problem at a time to see how I respond. During this surgery Dr. E is going to be attaching an allograft (cadaver) tendon between my scapula and my spine. We're hoping this surgery will help me just to get my arm to shoulder level. The allograft will medialize my scapula and keep it in internal rotation (basically if you were to shove my scapula in towards my spine). My biomechanics are completely screwed up and this is completely opposite of normal. Dr. E doesn't know why this is the only way I can move my arm but the point is it does move this way so we have to help my body so it can. My mom asked Dr. E, "What do we call what Meg has?" and Dr. E smiled and said, "The Megan Syndrome". I officially have my own category and I am not the same as the other 6 individuals. I'll be braced 6-8 weeks after surgery full time. The good thing is if I don't respond well to the surgery, it's easy enough to cut the allograft out with local anesthetic in the office and Dr. E has backup plans. 
My new custom brace that took almost 2 hours to make.
It's nice because there's no weight on my shoulders or neck.
My sister bought me foam so I can put it between my ribs and the brace.
My ribs don't like the pressure of the brace at all but the foam at least
gives a little bit more padding. It's pretty gross feeling my cartilage
slip over my ribs when I move.
To the squeamish types, the 2 pictures are below this paragraph...

So here we are today. In exactly one week I will finally be having my surgery on January 28th. I've tried everything under the sun for the past 2 years trying to get better conservatively and it's not working. My symptoms are getting worse, I've lost my arm motion & strength, my neck is worse, my pain is off the chart, I rely on so much help, and I've dropped 15 pounds due to the pain. I'm happy to finally have a treatment plan in the right direction. I'm not really nervous about the surgery itself. I'm more nervous about how my right side is going to react and how I'm going to get through the next few months because neither arm will be working. One will be immobilized and the other can't move at the shoulder; it's all elbow motion. I'm going to have to learn to accept a ton of help. My family is up for the challenge; wish them luck.  

This happened last week when my mom and I went
to run a couple errands. When we got home I had to
go lay down. An hour went by and I took my shirt
off to have my mom apply cream and this is what we found.
My scapula shifted waaaay out of position. No wonder I didn't feel right.
This is what happens when I try to "push" the wall.
It has been a very long week since this happened.
I've had to immobilize my arm every day. 
You might be asking how did this happen? How did I injure myself? The primary diagnosis in my chart is Ehlers-Danlos Syndrome which is causing all the laxity, which is causing all of my problems. I didn't sustain any specific injury and I don't have some amazing heroic tale to tell. It comes down to faulty tissue. The good news is I have a doctor who is totally invested in my case and has amazing communication skills. I have a doctor who believes my crazy symptoms and doesn't think this is all in my head. I have a doctor who is optimistic that he will help me get more use of my arms again; it's just going to be a long road to get to that point. I feel really good about working with Dr. E. 
The other day my nieces, nephew, sister, mom and I all made a pie. There was
a lot of love put into it and it tasted really good. We made a s'mores pie. 
I don't know how soon I'll be writing after I have my surgery. The best way to get current updates is to go onto Facebook and "Like" Meg's EDS Medical Journey My family will be posting updates there.  
My neighbor posted this quote yesterday and I found it very fitting