Yesterday was round three of the Botox injections, and hopefully not the last. Hopefully whatever replacement I find for my ending health insurance will cover Botox as it has been a tremendous benefit and provided relief. The photograph above is of the very old EMG machine used to check and make sure the Botox needle is in the right location prior to injecting medication. I liked the creepy mad scientist look it gave in the photo. :)
So the injections went as well as can be expected, and I am actually grateful for the fact that I have sensory loss in my legs because looking at him using the muscle to bend the needle back behind my shin bone it occurred to me that this should probably be painful. Yes, I supervise and watch because when anyone is coming at my body with a needle that size I prefer to monitor exactly what they are doing with it. Plus it is a control thing. I am noticing a pattern though. All three times after the injections I have crashed within a relatively short period of time. Yesterday was the shortest as I was not out of the office building and I was starting to feel dreadful. We stopped to get me something to drink so I could load up on meds, and then I focused on breathing during the hour and a half long drive back to my house. The drive sucked. By the time I got home I was beyond pale with a lite tint of green around the edges but I had managed not to vomit in my Dad's truck, for which I think I deserve bonus points. I was knocked out pretty much for the rest of the day. Today I am hung over not just from the crash but the day after Botox hangover - sore weak muscles, flu like feeling, low grade fever. I slept until noon today, woke up long enough for a cup of coffee, and then slept until 4:30pm. I will try writing a more witty, insightful, and informative entry some time this weekend but I am exhausted and foggy from the combo hangover. My poor legs look like I went to a fencing contest in Lilliput and lost. Thank you for all the prayers and support!! I am so blessed!

According to the government of this great country, I do not qualify as being disabled. I have been denied Social Security Disability and now must begin the oh so fun process of appealing. Looking at my alphabet soup of medical conditions and the severity of the dysautonomia and dystonia I would really hate to see how ill they would like me to be for me to qualify on the first go round. I am working with a company that deals with Social Security Disability, courtesy of my Long Term Insurance company, and so today we began the additional reams of paperwork over the phone. I think I am now responsible for killing an entire acre of the Amazon Rainforest for all of the paperwork completed. My medical records may be responsible for half of the deforestation. So as she is asking me questions we have the following conversation.
Her: Could you do your previous job today?
Me: **Laughter** Oh wait, you are serious? Lets see, I can't hold my arms over my head to blow dry my hair but sure I can pick up and position children weighing 50+ pounds. It was full contact teaching, not sitting at a desk and reading aloud to students.
Her: Could you work for 8 hours?
Me: **Laughter** In one day? Or across a week? There are days my blood pressure is so low I can not sit upright, and I take two strong antinarcoleptic medications and still sleep 16+ hours a day most days. Plus I don't have the ability to sit upright that long without severe pain.
Her: If you could work would you?
Me: **Indignant snort** Do you really think that at 29 I would rather spend most of my days stuck in this house or doing the job that I got the best education in the world to prepare for and loved more than anything?

I think one big issue with my application  is that even with all of the diagnositc testing and specialists we have yet to arrive at a clear cut diagnosis. We can diagnose the symptoms, we can treat symptoms, but we can not identify the main disease or disorder that is causing all of the damage. It is believed to be something genetic but beyond that we have eliminated but not identified.

My biggest fear, or concern, is that I had been praying to qualify for Medicare before my COBRA insurance coverage runs out. Michigan is a "skip state" which means something like we skip the first appeal and go straight to the second and more comprehensive appeal. This translates to a process that generally takes 7-34 months. Without access to health insurance, it will be a mute point to determine if I am disabled or not. I am holding on as hard as I can to God's promise to provide for all of our needs in His perfect timing.

The day before Thanksgiving I was lucky enough to have a double schedule of a standard neurology appointment to be followed by Botox injections from the Movement Disorder Specialist Neurologist. I arrived early for the first appointment, that with the general neurologist. I am not sure why I bothered, as she has yet to ever even manage to be on time let alone early in the year or more that I have been seeing her. So I waited, and waited, and waited. Finally, just when I was due upstairs in the Movement Disorder Specialist's office she walked into the room. Now her office knew I had an appointment with her colleague upstairs at a specific time and chose to keep me waiting downstairs and finish that appointment first. This comes into play later. Her big revelation is that I am far too complex to receive care under a general neurologist anymore, and she wants me to receive care for the dystonia from the Movement Disorder Specialist and for the Dysautonomia from another Specialist and to try to get to the Mayo Clinic if I ever get off of their waiting list for the waiting list. I waited over an hour to be told you have no idea what to do with me? Seriously? And you want my copay for this too?? She did order a bunch of labs because I have further loss of sensation and proprioception in my legs and feet which is strangely mirrored pretty closely in my arms and hands. By this point I am 40 minutes late for my appointment upstairs with the Movement Disorder Specialist for the Botox so my chart is shoved onto my lap and I am told to head directly upstairs, do not pass go, and do not dream of collecting any money.
I arrive in the office upstairs as fast as I could wheel, hand them my chart, and am asked very coldly if they were running late downstairs. Um, no, I decided to go out for drinks between appointments. Duh!! I was asked this same question no less than three times by every nurse, nurse practioner, and even the doctor. Apparently what they wanted me to do was leave downstairs, come upstairs at my appointment time, and then go back downstairs to be worked into the schedule. Great, I would have done that had anyone communicated that to me. So it was a rushed appointment, which I generally am not fond of when it involves long needles and my body. The Botox injections were less painful at the time of injection than last time, but more painful in terms of aching muscles later in the day and over the next couple days. I also got a weird headache right after the injections this time. I asked the Movement Disorder Specialist a question that the neurologist had fielded to him, and did not particularly like the answer I received. Lately I get what feels like the same increased tone in my legs in my lower to mid back. When this happens if I am standing or attempting to take steps with my walker it is very difficult if not virtually impossible for me to move my legs (I can move them a little better if lying down). He feels that this indicates that there is something going on involving my spinal cord, but since I have been imaged repeatedly, and my insurance charges a small fortune for any type of scan (Damn Green Cloak and Green Dagger) we will just watch it for now and see how it progresses. Oh fun!! And to test the progression of the sensory loss another round of EMG testing may be in my future. Santa, that was NOT on my Christmas list!!
Other than having to make an appointment for a check up with my internal medicine doctor, who is now holding my prescriptions hostage until I see him as it has been too long between appointments, I should have a decent stretch of doctor free time. Now if only my back would stop hurting, I might be able to see how much of a benefit I get from my new leg braces during peak Botox effectiveness!!

I adore words, finding in them an almost magical power that extends far beyond the representation of concept or emotion. There is a lyrical, musical, ballet dance to words and the sound and feel of the word is just as critical as the intent of the word. Discombobulated has long been one of my favorite words, ever since I first tasted its weight and tone as a small child. Nothing else quite captures the state of jumbled up, tangled, frazzled, out of my own control, not sure which way is up or down as being discombobulated. My reason for not writing anything in a long time is that I have been living in a perpetual state of discombobulation.

My dysautonomia has become significantly more active as the fall days turn colder, and I do not know if that is just a coincidence or if I am in for a long winter.I have been held hostage by my body all month thus far inside of the house, and I am getting very tired of spending my days on the couch or curled up in my bed. Tomorrow I plan on forcing my body to suck it up and deal with a short outing to Target as I am out of some of my medications and they don't deliver (I got spoiled by the old fashioned pharmacy that delivered in the smaller town I used to live in!). Emotionally I tap dance along the spectrum from anger to depression (more like mourning or grief for what I lost) to days of acceptance. I should have paid better attention during those dance lessons as a child as I keep getting the steps wrong and ending up all tangled and befuddled.

I can not believe that it is already time for me to need to get working on making my Christmas cards, and I don't even have my craft table officially set up in my bedroom. Thankfully this year's planned card is simple in design and should be an easy assembly. I had considered doing homemade Christmas gifts as well but that is so not happening this year. Maybe next year if I start in August I will have a prayer of getting done in time.

I have my next round of Botox injections scheduled for the day before Thanksgiving, and I am hopeful that this appointment is not cancelled (the first one for this round was cancelled as the doctor had to go out of town). It has been an unpleasant process to gradually regain the spasticity that was taken away by the first round of injections and I much prefer the range of motion and comfort that I had following the injections. I also see my neurologist on that day, and we have many details that need to be discussed including what symptom management will look like since it appears the search for a diagnosis has been surrendered and if this new back pain is progression of the dystonia and how I can stay hydrated to prevent the recurring bladder/kidney infections when I have absolutely no sense of thirst (thanks to the dysautonomia).

In a completely random note, it has already snowed here. Excuse me, but I don't remember placing an order for an early winter or cold weather. Thankfully the little snow that stuck melted quickly the next day. Snow sucks when you are in a wheelchair and the cold sucks when you can not regulate your body temperature. I love looking at the snow, but I hate having to go out in it when it is windchill negative freeze your breath. I still want to find the genius who thought that metal push rims would be a great idea for a wheelchair that would be used in a Northern state with frigid winters. It is like that scene in A Christmas Story when the kid gets his tongue stuck to the pole, only with my hands on the push rims. I had to buy a new, very warm winter coat as the coat from last year did not fit and matching hat, scarf, and gloves. Unfortunately, with my KAFOs, there are no winter boots that I have found that fit over them so I have to be very, very careful transferring in the winter. 

I will hopefully be back soon to write something more creative, more insightful, and more entertaining. I am writing this tonight as I try to lull my brain into a relaxed state during an annoying bout of insomnia. Therefore, I promise absolutely nothing in terms of comprehension or logic. :)

As a small child I was hypersensitive to the tags in my clothing. My mother had to painstakingly remove every last reminant of any tag from every single item of clothing before I would agree to wear it. I would hear her grumbling as she picked at seams with sewing scissors after every back to school shopping excursion. But those tags in something new were intolerable to me, I could continually feel them grating against my fragile skin and what to many would be a light touch was magnified to torture. Yet in old hand-me-down clothes that had been through the washer countless times and the tags had been softened with age I did not demand that every tag be removed because they had been softened with time. They were tolerable because they were in something broken in, something that had taken the abuse of the world and worn away the hard edges. It took time for the raw scraping to transfer to a soft and gentle touch; time and wear and use.

I realize after reading comments to my last post that it may come across that I have a huge chip on my shoulder or extremely high expectations when it comes to how others act towards me in relation to my disability. Using a wheelchair, using leg braces, not being able to walk independently - those are all brand new tags in my life. After two years they are not as intolerable as they were in the beginning but they are still no where near as soft and flexible and gentle as the other tags in my life that I have lived with for so much longer. I admit that it stings more when I feel someone react to my using a wheelchair because that tag is still grating on me. I use humor as a huge coping mechanism, and that is why I frequently just laugh at the situations that feel awkward or out of place. I also know that because these tags are not worn soft with time, I need to keep in my heart and mind the fact that most people have good intentions and just generally have limited experience with individuals with different abilities.

But I do also hold society to a high standard, because if we do not have high expectations than nothing will ever change. I will gladly educate and advocate. The inclusion of individuals with disabilities in our mainstream society has come so far, but has so very far to go. Ignorance is not an excuse, as it was not an excuse during the Civil Rights movement, but an opportunity to educate. Sometimes I need to remember that it is an opportunity to educate and ignore the rubbing of the tags. Ignorance is a tag that rubs raw, but it is one that is only softened by education and by teaching those who "don't know any better". It is showing the world what it means, and does not mean, to have a disability. It is working to create a world where having a disability is not equal to having a handicap*.

I hope this post makes sense. I tried so hard to explain what is on my heart in a manner that would be easier to understand.

* A disability is a difference of abilities as compared to a norm. A handicap is when a difference prevents the individual from being able to engage in a desired aspect of society. So it is possible to have a disability but not a handicap, and technically to have a handicap without a disabilty. In the game of wheelchair basketball, being ambulatory is a handicap because you are not used to using a wheelchair and you will struggle to keep up and not end up as roadkill.

This time of year seems to be a very melancholy, introspective one for me. Last year I thought it was because it was full of all of the first "last times" - a year since I taught my kids, a year since I got sick, a year since I was able to hike, a year since I walked independently, a year since I became dependent on a wheelchair, a year since I lost so much. Apparently those anniversaries are just as powerful the second year as the first because I started to get into a funk as October began, and it was the beginning of October when I really started getting sick two years ago. I feel the old emotions creeping back in - the frustration, the anger at my body for betraying me when I had everything I had ever wanted in life (a job teaching special education, awesome friends, and was ironically the healthiest I had ever been before this happened with hiking and teaching all day and doing yoga), the aching longing to have all of the unanswered questions answered and to give this monster a name so I can call it out into the light, the grief for what I lost. Now it sounds like I am sitting around feeling sorry for myself and that is absolutely not the case, but when I least expect it I find these old feelings creeping around again when I thought they were long gone. I guess there is a season to everything and this is my season to mourn. Soon enough it will be my season to dance and celebrate and be joyful. Even in the mourning I am thankful, for in order to feel loss I had to have had some incredible experiences and opportunities and I am forever thankful for them and for the life I have been given. Considering I was never supposed to live beyond my first birthday, every day is borrowed bonus time.
I have another entry that I want to write soon, but this is what came out when I sat down to type so I am going to go with it for tonight and save the other for another day this week. This is more real to what I am experiencing now, a real image of the fact that there are still seasons of mourning two years out and probably ten or twenty years out just as there are seasons of celebration and seasons of joy and season of dancing.

* photo courtesy of iStock Photo

I am officially moved into my new apartment that I am sharing with my mother, and the apartment is awesome. However, moving is perhaps one of the most frustrating and exhausting processes on earth. We have boxes stacked in every room in the apartment except the bathroom, and our goal is to have everything unpacked and put away and organized by Christmas. What is scary to think about is the fact that I could literally throw away every single box I have that is still packed, and other than missing my journals, I  would have every single thing I *need* and could replace anything else easily. Needless to say, as I unpack I am very carefully sorting through items and either donating or throwing away any items I have not used in the past 4-6 months. Possessions have never been a big deal for me, and I am driven in life by making memories and interacting with others, not with acquiring things. I am actually most happy when I have just the basics that I need and a few wants.
So when we moved into the apartment we apparently forgot to make some sacrificial offering to the toilet gods because on the second day the toilet made a strange sound and then proceeded to do a very realistic impersonation of Old Faithful. Water was not running over the edge, it was erupting from the toilet. Before I remembered the wall shut off valve it had flooded not just the bathroom but the entire hallway. Maintenance showed up 30 minutes after we called carrying nothing more than a plunger. Um, Dude, we're gonna need a bigger boat. An hour later, the toilet is precariously "fixed" and he has made an attempt at the flood with a wet dry vac and we go to bed with fans blowing in the hallway to dry the carpet. I am especially thankful that I choose to get carpet without padding to make it easier to maneuver my wheelchair because that makes this mess a lot less of an issue. Three days later I have prayed over the toilet and flushed it, and it is refilling when I hear a sound that I can only describe as the sound one would expect to hear if the bowels of the earth opened up and proceeded to suck down Niagra Falls as the toiled completely drained of water. Apparently somewhere along the six stories of pipe there was an obstruction, and that god awful sound was the sound of it breaking away. Either that or the toilet gods decided we had paid our dues and removed the curse. I still pray before I flush the toilet and try not to get too close to it unless necessary.
Amidst the chaos of moving I also had my follow up appointment for the Botox injections to determine how well the first round of injections had worked and plan for what the next round will target and such. It was a very short appointment, which frustrated me as I drove 90 minutes each way just to talk to the doctor for 5 minutes - no exam even. I am getting new braces made as my old ones never fit right and now totally do not fit right since I have achieved a more normal position and the ability to have my legs positioned and held in even more normal positions. This time I am getting KAFOs, or Knee Ankle Foot Orthotics, because when I stand and walk I lock all of my joints for stability. The problem with this is that I have joint hypermobility and to lock my knees I actually hyper-extend them which is causing pain and throwing me even further off balance. So the KAFOs will provide the locked knee support without allowing me to hyper extend my knees.
Right now I am temporarily set up with the internet running from the main phone line in the middle of the living room because the phone guy failed to install phone service in all of the rooms of the house when he came out, and the soonest they can come back out is Tuesday. So my internet access is still limited as it is a hassle to finagle it out here. Which is okay because I have not been feeling up to doing much more than working with my mom on a box or two a day and then resting. I don't know if I have some sort of infection or if the dysautonomia is just warning me that I have been pushing too hard for too long.
I will try to update from the box piles again soon, for all three people reading this LOL :)

Two weeks. The magical number for maximum effects from the Botox injections in my legs to be seen. I still have a large bruise from one of the injections that apparently went awry, but it is slowly fading from angry blue black to shades of purple and yellow-green. So how much can change in two weeks? Well, a lot. The spasticity in my legs is reduced and my feet at rest are much closer to a natural, neutral position. When I wear my braces it is no longer a wrestling match to try to get my feet into them and I can stand with my feet facing forward for short periods of time (before the knee and hip rotation kicks in and turns them). My feet are the closest to being flat on the ground that they have been in almost 2 years. Toes are unchanged, which means they are still curled tightly under and unable to be straightened. But perhaps next time with the injections on the bottoms of my feet (yikes!) we can get my feet fully flat and my toes to relax some. It is hard to tell how much is permanent contractures and how much is still just spasticity in the toes. Side effects were not too bad, but I do have increased weakness in my legs and have to be very careful when doing any weight bearing that I am safely supported and when in my wheelchair I still must use the straps on my footrests to hold my legs in proper alignment. Other than that, I felt kind of generally yucky for a day or two (could have been an autonomic response to the trauma of the multiple injections) and bruised and that was it. Definitely worth the short term pain for the longer term gains!!!
Just in an unrelated note, the Dysautonomia has been kicking my butt recently and I am currently living on soggy cheerios and green tea and Zofran and headache meds and muscle meds and BP meds and sleeping, sleeping, sleeping.

On Wednesday I had my first appointment for Botox injections. I knew just enough going in to be dangerous to myself, but not enough to be well informed and relaxed. Almost all of my information came from my experiences as a special education teacher, so it was framed through the pediatric framework. Knowing that this is a process done under anesthesia for children, I was a bit anxious about how much it would hurt. The reason it is done under anesthesia for children is their difficulty remaining still and the trauma of multiple injections into the muscles. Using the EMG machine with two electrodes and a special needle, the doctor targeted specific muscles in my lower legs that are responsible for rotating my feet inward and pushing them downward. I received somewhere between six and ten injections per leg with a small dose given per injection. I remained seated in my wheelchair for the process and simply removed my braces and socks. I was pleasantly and delightfully shocked by how much less it hurt than I had anticipated. I was all ready and prepared to swear creatively, since Mythbusters demonstrated that swearing increases pain tolerance by 30%, but I never even needed an old fashioned "Ouch!"!! Not that I am saying it was particularly fun, but it was no where near the pain I expected. One injection did cause significant bleeding and a large bruise formed with a golf ball sized lump underneath, so it looks like I am trying to shoplift from a golf pro shop via a hidden pouch in my leg. My legs are sore and achy, but nothing more than I would feel after expending myself too much and overworking the muscles. I go back in four weeks to have the doctor assess the impact of the Botox injections, and then in 13 weeks for the next round of injections. Based on the muscles response during the injections, the doctor thinks next time he will need to inject the bottom of my feet to attain the best possible results and get any results from my toes. According to our conversation, those particular injections "hurt like hell" so I am sure it will be an adventure. I did point out to the doctor that none of this hurts him, and he was quick to point out that if he feels any pain during the procedure something has gone wrong. I have two words buddy - voodoo doll LOL :) So now I wait - for the bruise to go away, for the aches to go away, for the results to begin to appear (any time within the next 2 weeks I should see the effects). Just the fact that this offers a chance to get relief from the tight muscles and the twisted feet makes it worth the experience, let alone if it actually works!!

Well, my self imposed vacation lasted a record 24 hours ;) I had decided not to write because I felt that all that I had been writing lately had such a negative or melancholy tone to it. But the more I thought about it, and with some advice from a friend, I realized that not everything about living this life is going to be easy and that if I want to be honest here I need to write about the good, the bad, and the ugly.
In medical news, I am not sure what I wrote in the last post but I am currently very angry at the Dr who assured me that he knew exactly what was going on and was so positive of the diagnosis of Mitochondrial Disease - so positive that he had me receiving benefits from the MDA. So positive until the DNA results came back negative. He sold me hope, and I was so careful to ask him again and again if he was sure because I have been down the road of false hopes so many times before. He wanted to then refer me to the Mayo Clinic, but financially I can not afford the expenses of travel, lodging, etc. that go along with such a medical trip. Therefore, it appears the medical consensus is that I have an unidentified progressive neurological disorder that shall not be named. Treatment will be symptomatic. As much as this sucks, and it sucks on a grandiose scale, I am weary of all of the testing and the appointments and the feeling like a laboratory specimen and the false hopes and the never ending cycle. I just want to have some sort of a life.
I made the appointment for my Botox injections today, and the soonest the could do the injections was August 4th. By my calculations, that is 34 days away - nothing like prompt service with a smile. :) I have one neuro appointment on the 20th to figure out who is coordinating care and to discuss symptom management, and the fact that I prefer to have the general neuro rather than the specialist in charge of my care (especially after the false hopes).
This Tuesday I have an appointment with a new GI doctor for the dysphagia that has returned with a serious attitude problem and the severe nausea, stomach pain with eating, bloating with eating, and other fun stuff it brought along. I am back to eating Gerber products - if you ever need a recommendation on their baby or toddler food, let me know I have eaten just about all of it. I almost choked to death last night on the bready part of a bagel and so am stepping down from soft foods to more blender/pureed consistency. My throat and chest still hurt today from that choking episode. I definitely do not want my tombstone to read "Death by Bagel"!  Now death by chocolate.... ;)

Ring around the Rosies
A Pocket full of Posies
Ashes, Ashes
We All Fall Down

Because we apparently missed a test to run on my brain and body, yesterday I had an EEG conducted prior to my appointment with the Dr. E to consult on the administration of Botox to my legs and feet. I drew the first lucky card of the day and became a guinea pig for someone just learning how to conduct EEGs. While she was very nice, I think I ended up with at least a dozen extra red pencil marks on my forehead and scalp as she measured and remeasured. Then she very slowly applied the leads using the theory that more adhesive and conductive goop is always better. Have I mentioned that I am allergic to all things adhesive? So we finally get approval from her supervisor and do the EEG testing. Results - my brain is using its wires according to plan. I would have been really upset had there been any signs of seizures or abnormalities considering that I am on a mega dose of Topomax (antiseizure drug) already for migraines, and another antizeizure drug that helps control muscle tone. As a result of the overzealous technician I had a delightful bulls eye on the middle of my forehead from her firmly and securely attaching a lead there. My hair was also glued down to my scalp, but luckily this was not my first go round with this test and so I had brought a hat. Washing my hair last night took about 20 minutes, a lot of scrubbing, and a lot of warm water to soak off the goop and the shreds of gauze still stuck to my head and be able to get my fingers through my hair let alone a hairbrush. It still itches today, so I think I may need to do a good scrub, round two this evening.
Then I had my consult appointment with Dr. E to determine whether I qualified to receive Botox treatment in  my legs to hopefully assist in releasing some of the excess tone. However I once again drew the lucky card and was given a Medical Student to play with first. She was a cute little thing and I wondered if her mommy knew that she was playing doctor. I tried hard not to laugh when she sat down and eagerly asked for my medical history. Instead I politely informed her that she would need more that that one sheet of paper if she wanted my entire history and that perhaps we should agree upon the cliffs notes version. After going over a condensed medical history, and explaining to her about how I am impacted by Dysautonomia, I then became exhibit A. I had to take off my shoes and braces so she could see my distorted and contorted feet. She asked me the stupid questions of the day.
"Where did you buy those? (pointing to my AFOs)"
"Those are custom made AFOs made by X Orthopedics."
"So did you get to choose the design?"
"Yep. I figure people are going to stare anyway, so I might as well give them something interesting to stare at."
Uncomfortable silence on her part....crickets chirp.....I try hard not to laugh.
"So to put your shoes back on do you have to straighten your toes?".
"Um, I have tried that and the thing is, they just don't straighten. So even with shoes on my toes are curled like this."
"So can you straighten your feet?"
"I can pull them towards neutral for brief periods of time, but this rotated position is how the muscles pull them. I can only hold them for a very brief (seconds) period of time in any other position because it is like pulling against a gigantic, powerful, and tight rubber band."
"So it is more comfortable the way they are (turned under)?"
"No, it hurts like this. My ankles hurt, my toes hurt, my legs hurt. I would prefer they not do this, but the extreme tone in my legs pulls them into this position. Does this look comfortable?"
"oh. Um, not really."
When she did the part of the neuro-exam to check for sensitivity, I did not have the heart or the patience to correct her and explain that you poke with the pointy end of the safety pin, not the rounded end. I am curious how she got into medical school and am just really hoping that she just began her neuro round. I had to play nicely with her for an hour. Then the real doctor came in and it took all of a ten minute discussion to decide that yes, I would in his opinion benefit from Botox injections. He wants to target the muscle on the back of my leg, one behind my shin bone (I am trying not to imagine what is involved in accessing that one!) and one or two others. I will receive the injections at a yet to be made appointment once they receive approval from my insurance and then order the proper dosage and quantity of Botox. Dosing is not an exact science, especially since this is an off label usage for it (which I found strange since I am more familiar with using it in this manner through teaching kids with spastic cerebral palsy and other disorders that caused excessive tone than cosmetic reasons) so the first dosage is like any other medication really - the best calculation but each person reacts differently. It takes 2-3 weeks to see results and results peak at about 2 months. I do plan on warning the doctor when he does the injections that a recent episode of Mythbusters, which proved that women can tolerate more pain than men and redheads more than any others, also showed that cursing increases pain tolerance by 30% and I may take the liberty to test that theory myself - afterall he is going to be injecting a muscle BEHIND my shinbone!!
All added together I was at the neurology clinic for 3.5 hours - 1 of which I spent as Exhibit A for the medical student. Because of my "unusual" medical history and presentation I tend to be lucky and draw med students and interns and residents frequently. But all in all it is good news - the wiring works in my brain, and we have a new tool to try and reduce the spasticity/dystonia in my legs.

I am not sure if I have ever posted pictures on here of exactly how the dystonia/extreme spasticity in my legs has greatly altered my feet. My toes are contracted up into a curled position from which the can no longer be coaxed straight.

My feet are rotated inward at the ankle so that when I weight bear it is not on the bottoms of my feet but the outside edges and ankles, causing several physical therapists and doctors to comment on the fact that they feel it is only a matter of time before I break an ankle. I choose to remain optimistic and take calcium to strengthen those bones!
My feet are also tightened in such a way that the toes are pulled in tightly and thearch has pulled up and to the inside. Bones have shifted creating a new arrangement on the area of my foot that takes the brunt of my weight when I transfer or take a few steps. Even in my AFOs I tend to go into a tight "toe point" with my heel pulled up and my toes tucked downward which causes pressure sores to form if I am not very careful.

This month I am supposed to meet with another neurologist to undergo evaluation for botox and obtain insurance approval to try and release the spasticity and tension in my lower legs. I also rotate inward so that when I stand my feet point in opposite directions at each other and even when sitting my knees rotate inward towards each other all the way from my hips. Some of this is corrected by the positioning of my wheelchair.
I apologize for the poor quality of these pictures, but they get the job done. And these were taken while on a cocktail of medications to treat the spasticity. I am a bit like an awkward ballerina always stuck in the wrong position. :)

Today I had a follow up appointment with my regular neurologist. She was thrilled and excited to hear that the specialists at the MDA clinic had been able to give me a working diagnosis that he is confident will be proven through subsequent testing. The focus today was the treatment of the symptoms rather than diagnosis. So we rearranged my medications that are used to treat the spasticity/dystonia in my legs to try to focus in on a combination that works. Really good news - the florinef seems to be working as my BP at the office today was 114/75!!!!! I NEVER see numbers that high!!! If we can get it a little higher and stable, then we may be able to try the Baclofen again - I got great results from the Baclofen but it caused a dangerous drop in blood pressure that led to a tour of the local emergency room when I hit numbers like 78/45 and was none to happy about it. The doctor noted that my endurance and strength have declined - for example I used to be able to stand with my braces or minor support (the counter) to complete a washing of dishes. Now after 2-3 minutes I have to sit back down in my wheelchair because otherwise my legs are going to go out from under me. I used to on a very good day be able to wheel about a mile, now I am lucky if I can wheel a block to the grocery store and library on a very good day. We are going to try to add in a low dose of an ADHD medication to treat the overwhelming fatigue because there are some days when even on a solid dose of narcolepsy medication I sleep until 3 or 4 in the afternoon and I am so exhausted that getting dressed is a massive victory. It is difficult to plan anything because I never know if it will be a day that I have energy or a day that I can barely get up out of bed to lay on the couch. Things that influence my energy include how I slept the night before, if I have any lingering or low level (let alone full out) infection, how the dysautonomia is behaving, and sometimes apparently the flight pattern of a butterfly in Brazil. :) I totally forgot to ask her what my PFT results were, but I am assuming if they were dangerously abnormal I would have been notified. I was also diagnosed as having myoclonus, and in a few weeks have to have an EEG done to make sure there are no signs of seizures and that it is originating in the muscles. On the same day I will be evaluated to determine if I would benefit from Botox injections in my legs, and then they will begin the process of convincing my insurance company that it is medically necessary - you know, that I am not treating wrinkles in my feet for cosmetic reasons. ;) The idea of being able to straighten my toes and get my foot flat again is an amazing concept that seems beyond belief.
Today started out far too early for my brain and body to keep up, but it was worth it because on the way to the appointment my father took me out to breakfast at a little hole-in-the-wall place that had awesome breakfast food. We shared one breakfast and I still could not finish my share!! Through all of this I have really been amazed and so appreciative of how my family has come together and been there for me. Never has my father or stepmother or brother complained about the appointments they have taken me to, about the waiting in waiting rooms, about the long drives (because the best neurologists are over an hour away), about the seemingly endless tests. My mother has been my emotional support, and she has never complained once when I woke her up in the middle of the night (nights are my toughest times) in tears of frustration or when I let it all out on her. Everyone has adapted to having to accommodate my wheelchair and my new limitations without making me feel like I am a burden or that I am making things more difficult for them. I am so blessed.

Lately I have been reflecting backwards on just how much my life has changed, and I realized that there are big things I miss from my "before" life but also a lot of little things. So I decided to put together a list of the things that I miss the most. I also decided that I would then complement that list by listing all of the things that I truly appreciate that have happened in the "after" life.

Memories
* Driving a car
* Hiking
* Reaching things on higher shelves without having to ask for help
* Being able to wear low socks (AFO braces require high, basically knee socks underneath them)
* Being able to buy shoes easily (AFOs do not fit in hardly any shoes - thus I rock converse with dresses)
* Being able to attend concerts (I lost that to the dysautonomia)
* Being able to take a shower instead of a bath
* Painting my toe nails in less than a two hour process
* Carrying a purse instead of a backpack
* Not looking up to everyone (I have a great view of belt buckles and backsides)
* Teaching
* Swimming without a flotation device
* Being able to break a glass and not have to call someone to come clean it up for me so I don't pop a wheelchair tire
* Wearing my polka dot rainboots and splashing in puddles
* Living in Virginia
* Sitting in a chair without strapping my feet down
* Not glowing in the dark from countless MRIs, CTs, Xrays, and other medical tests

Appreciations
* I totally skip the line at airport security and am through in about 15 minutes total no matter what
* I can run into people who annoy me and then play innocent and no one yells at the chick in the wheelchair
* I am saving a ton of money not having to pay for gas
* I have learned to truly live in the moment because nothing is guaranteed and to appreciate all I have as I am so blessed
* I have some of the most amazing friends and family in the world who have walked this journey with me and never let me fall so low that I could not get up again (Steph, you are an angel)
* God has a great sense of humor
* Laughter can do amazing things (Steph, you made me laugh in a place and time I thought I would never laugh - the tree and the words "hang in there"...priceless)
* Doing the right thing is always worth the cost
* I have a totally new appreciation and perspective on what my kids go through and all that is asked of them, and they are now my heroes even more than before if that is possible
* Every time I was at my weakest God sent me just the person I needed to be there for me and offer me the words and support I needed - a nurse at PWH, a dietician at GWUH, my home healthcare nurse, my amazing friends, prince not so charming
* I have learned to dance a new way to the music of my heart
* Watching how Little Bit sees me, how she asks questions but sees me as just the same Aunt Beth has been the most priceless treasure - my leg muscles may not work so well anymore but I am still her silly Aunt Beth and she loves "helping" by pushing me (even if she can not quite see over the wheelchair to steer!)
* Relearning to eat all over again has taught me to appreciate so many little things that seem natural and go unnoticed until they become a struggle - I appreciate each breath I take, each time I can coordinate movement, each sensation I feel, each swallow, each heartbeat, each taste of food not pureed or from gerber!
* Knowing that God has been with me all of the way and that He has plans to turn what to many would seem a disaster into something beautiful to glorify him!

Frustration has become a frequent visitor as I am constantly reminded of how different my life is from those of my "peers", and as I continually discover obstacles big and small where they never existed before. Most of the time I am pretty good at using my skills honed as a teacher to problem solve and either create alternatives or adaptations. Some things I have yet to figure out and are just annoying, like the fact that at least once a week I manage to forcefully jam a thumb between a rapidly moving wheelchair tire and the immovable brake gear. This happens because for some odd reason I can not figure out, when I am not thinking about it I push my wheelchair by the wheels more than by the push rims. I partially blame the "genius" who never asked me what kind of push rims I wanted and instead made an executive decision for chrome. Because shiny metal is so easy to grab, does not possibly rip skin from your hands and cause frostbite when it gets wet in the cold Michigan winter, and could not ever become burning, scorching hot in the summer. My patience for the stupid that seems to bloom in people when they see someone in a wheelchair has been worn very thin, and I am not yet at the point where I feel like being an educator and advocate about disabilities. Children do not bother me - they are curious, I love when they ask questions, I love watching them process it all. Adults who assume that because my legs do not work according to standard operating instructions that my brain must also operate on a different frequency frustrate me. People who assume that I have become hearing impaired because I am sitting down make me laugh, but annoy me. Anyone who asks me where my parents are or who is with me or if someone forgot me triggers the redheaded anger, which is not recommended. I am tired of the world defining me by my body when I am not my body. My body is a tool that I use that allows ME - my thoughts, my ideas, my emotions, my personality, my existence- to navigate through life. I am so much bigger than my body.

Bigger Than My Body - John Mayer
This is a call to the color-blind
This is an IOU
I'm stranded behind a horizon line
Tied up in something true

Yes, I'm grounded
Got my wings clipped
I'm surrounded (by)
All this pavement
Guess I'll circle
While I'm waiting
For my fuse to dry

Someday I'll fly
Someday I'll soar
Someday I'll be so damn much more
Cause I'm bigger than my body gives me credit for

Why is it not my time?
What is there more to learn?
Shed this skin I've been tripping in
Never to quite return

Yes, I'm grounded
Got my wings clipped
I'm surrounded (by)
All this pavement
Guess I'll circle
While I'm waiting
For my fuse to dry

Someday I'll fly
Someday I'll soar
Someday I'll be so damn much more
Cause I'm bigger than my body gives me credit for
Cause I'm bigger than my body now

Maybe I'll tangle in the power lines
And it might be over in a second's time
But I'll gladly go down in a flame
If the flame's what it takes to remember my name

Yes, I'm grounded
Got my wings clipped
I'm surrounded (by)
All this pavement
Guess I'll circle
While I'm waiting
For my fuse to dry
For my fuse to dry

Someday I'll fly
Someday I'll soar
Someday I'll be so damn much more
Cause I'm bigger than my body
I'm bigger than my body
I'm bigger than my body now

It has been a very long time since I have written anything here. Extremely long by my standards. I have been busy cleaning house in many ways. Well, first I was busy sending out prayers of Thanksgiving for the invention of TamiFlu as I dealt with a low grade version of the flu my mother got for Christmas. She got really sick, I got preventative Tamiflu and was able to get through with minor symptoms. Considering I am a living, breathing risk factor I call that a victory. I ended up staying with her for about 3 weeks instead of the 10 days I expected around Christmas because I wanted to make sure she was over the flu before I left, and I selfishly did not want to bring the germs home with me. :)
When I got back to my apartment I realized that I had left it in a mid-Christmas state of disorder. I dislike disorder a great deal. Now, my apartment is tiny - no more than about 500 square feet on a good day- so something as simple as a few items of clothes on the bedroom floor make a big difference. This was disorder and chaos. I totally rearranged just about every storage system in my house - my bookcases, my "office space", my kitchen cupboards, my dresser, and my arts and crafts storage. I am ignoring my closet because as the only closet/built in storage area I have decided it is a useless cause and as long as I can close the doors it is all good. I have no coat closet, no linen closet, no storage area so my closet serves more functions than is probably legal. Then I ended up washing almost every dish that I own. I hate dish washing because 1) I am the dishwasher and 2) the sink is not handicap accessible so dishwashing involves this awkward sideways contortion. I have tried using my braces and standing to wash dishes, but since my legs turn an awful shade of purple gray with white blotches after I stand for more than a few minutes, and I eventually develop syncope if I do not sit down, this has failed to be successful. Apparently my dysautonomic circulatory system has issues with sending blood to my legs and retrieving it when I am in an upright position. It is quite charming - I call it cadaver legs. ;) Anyway, I also vacuumed and mopped the entire apartment - I love hardwood floors. Vacuuming and mopping in a wheelchair should be Olympic sports with points for not tying yourself up in the vacuum cord and not creating tire tracks on the freshly mopped floor.
Mentally I have also been cleaning house. For some reason I was rather delusional and thought that the first year of dealing with all of this medical stuff and all of the changes in my life would be the most difficult. I was wrong. The second year is actually proving to be harder, I think because the true reality of it is sinking in and I have to let go of the illusion that there is ever going to be a simple answer or simple treatment/cure. Also it has been difficult because this illness is proving to be a relentless monster and it continues to progress. It is now impacting the strength in my arms, and I am noticing more changes in my lung function (I do not have the results from the PFTs done in December yet). I have been referred to the Muscular Dystrophy Association Clinic, for which I need to make an appointment, and I also need to follow up with my primary neurologist for treatment of the symptoms - primarily the dystonia, and to assess the upper body weakness. So I have been dealing with the frustrations and the fears, the doubts and the sadness. I have been cleaning away the emotions and the false beliefs that will not do me any good and making way for a new start, for the hopes and dreams and gratitude and joy that usually define my life.
I hope this makes sense. I have every intention of writing more soon, and writing more often now that the thorough housekeeping has been addressed.

This past weekend was the family Christmas activities for my father's family. A tradition of sorts has been established that each year the families gather at a hotel that has a swimming pool (easy entertainment for the kids grown and not) for the weekend. We have a big family meal on one day and then a smaller get together the other day for presents and such. This year the chosen hotel was just one town away from where I live, so the original plan was for me to spend the day with everyone and come back home at night. However, my stepsister's husband had to work and so she invited me to join her and her kids in their hotel room. It was a wonderful weekend where my braces and wheelchair and crutches were insignificant, where no one treated me any different and where I could just be a part of a family celebration. It was just assumed that I would be included rather than looking at obstacles and thinking it would be too much work. I have not always been close to my stepsister and brother, or to my stepmom, especially when I was growing up but they have genuinely formed a family that cares about me and accepts me and is protective of me without limiting me and that just gets that in spite of all the medical stuff I am still Bethany. I thoroughly enjoyed being able to swim so much, as in water I can move my muscles so much easier than I can in any other situation. I do cheat and use a flotation belt because I struggle to maintain balance and to hold my legs up when swimming, but the difference in motion is amazing. In the pool I could participate in all of the fooling around and playing without struggling with my wheelchair. I was able to play with my nieces in an environment that was without barriers. On Saturday evening, the spa was cooler than usual so I was able to safely sit in the warm water and place my feet in front of one of the jets for what was an incredible "massage" that helped to loosen up my muscles.
Two funny stories. The first involves being completely and inappropriately dressed for an occasion. At the hotel, the swimming pool was directly next to a large conference room (actually it connected through sliding glass doors and could be opened up so the pool and room were connected). As we were swimming Saturday morning, complete with children splashing and jumping into the pool and shouting and being kids (and some adult males being kids with them), we discovered that a funeral memorial service was being held in that room directly next door. Now, not only was it bad planning to place a somber event next to the pool the weekend before Christmas but the only way from the pool back to our room was either to go up a flight of stairs and across the upper floor then down a flight of stairs (um, apparently the wheelchair was not a clue to the hotel staff that this plan was not a reasonable option) or to go right through the buffet area for the funeral. So not only did we have to parade soaking wet, in flipflops or little bare feet through the somber set up but my swimsuit cover up? A nice wrap with brightly colored skulls and cross bones all over it. I have now officially crashed a funeral in my swimsuit and a skull and cross bone cover-up.
Second story requires some background knowledge. Last week my stepsister graduated from nursing school. Awesome! My dad and stepmom obviously went out to her graduation. I forgot what day her graduation was and so when I had a toilet emergency on that day I called them to see if they happened to have a plunger I could borrow. As soon as I called, I realized where they were and that from 3 hours away they were not going to be able to help me out. They actually did help me through a call to a friend who lives in the same apartment complex who hunted down a plunger for me, and all it cost me was some embarrassment. So yesterday while we were opening presents my dad and stepmom announced that they had a very special present for me. With a large red and green bow on top and red ribbon curling around it I was presented with a plunger complete with a tag reading "In Case of Emergency". I had to laugh, and everyone who knew the saga laughed along with me. So now when I am asked what I got for Christmas I can proudly say "a plunger!". :)
Tomorrow I am going over to my Mom's to spend Christmas with her and then on Saturday we are going to my Aunt's house for Christmas with my Mom's family. I am more nervous about that event because no one from her family has seen me or interacted with me in person since I became sick with whatever this dystonia/progressive spastic paraparesis/unknown syndrome is. I just want people to treat me like me and not be uncomfortable or all weird because of the chair and the changes in my life. But I am so excited to be celebrating Christmas with far greater health than last year, and not desperately ill and an 11 day inpatient stay right up until late on December 23. And I can even eat all of the Holiday foods this year too!! What a difference a year makes!! I am so blessed!

Expectations. I think the biggest issue, besides my inherent dislike of the teaching university approach to medicine, was a failure to match up expectations. My expectations for the appointment with the specialist, the neurogenetics specialist, was that he would be offering a second opinion and possibly assist in naming the overall syndrome that has caused the dysautonomia, the immune system difficulties, the ataxia, the sensory losses, and the abnormal muscle tone. These expectations came from the fact that my neurologist whom I adore sent me to him for his opinion on whether or not this is progressive spastic paraparesis and for a diagnostic second opinion. The doctor's expectations seem to have been that he would provide a diagnosis for the symptoms and begin a treatment plan, but not address the overlying issues or try to put the pieces together. Thus when he made the "profound announcement" that my feet and lower legs seem to have dystonia I was less than impressed. We went through that diagnosis months ago. I was hoping for something new. I already knew I have "dystonia" - abnormal muscle tone- in my legs. We need to know WHY and regarding that all he could say was that he was puzzled. Really? I drove 2 hours each way and waited 3 months to see you to get a diagnosis I already had and to have another doctor admit that they are puzzled? He thought he was accomplishing something great by pronouncing it dystonia, and I was just trying not to cry because we were back to where we were months ago. He even reverted back to the "toxic medication" diagnosis that was tried back in December 2008 and debunked when I was removed from virtually all of my medications and continued to get sicker. So with that off the table, he proposed some treatments. One, I have to stop taking the Reglan that I just started a month ago because Reglan can cause and aggravate dystonia. The only problem is that the reglan is what is allowing my digestive system to function and for food to progress out of my stomach in anything near a normal time frame. So now I am stuck trying to problem solve with my internal medicine doctor (I do not have a GI doctor, and really don't want to add to the village of doctors) something that will help because otherwise by dinner I still have breakfast sitting in my stomach. Then he wanted me to start on L-Dopa a few weeks after the reglan cleared my system. Something about L-Dopa triggered a warning in my mind but I could not pull to memory why. Of course it was not until after I got home that I remembered why L-Dopa was setting off alarms. In college I was given L-Dopa to try and treat muscle spasms associated with restless leg syndrome and had a horrific reaction to it that caused hallucinations and very abnormal behavior throughout the night. I prefer not to see things that are not really there, so I need to talk to MY neurologist and see what we can do for an alternative plan. The doctor at U of M also seemed to think he was taking over my care, but that was not my expectation and is not something I am agreeing to unless the doctor I currently see refuses to be my neurologist anymore. I trust her more, I prefer her style of medication, and she "gets me" better - she listens when I talk, she appreciates my knowledge base, she treats me as part of my own medical team. Yesterday I was treated repeatedly as if I were a teenager instead of a closer to 30 than anything adult. Curses upon looking so darn young for my age. The doctors also overlooked critical information like the newly emerged heart murmur, the association of the onset of muscle weakness with the inability to eat, the fact that my legs turn "cadaver leg" purple/gray after standing for just a few minutes, the loss of sensation, and the pain after using muscles as well as weakness and fatigue. While I am thankful the doctor confirmed that there is indeed something abnormal going on, I am frustrated that my expectations and his did not overlap virtually at all. I now hope when I meet with my neurologist (she said to see her after this appointment) we can discuss these expectations, my experience, and how to incorporate this into what she already believed and knew and go forward from here. Two steps forward, two steps back, but at least steps are still being taken.