No dreams of front porches with wooden rocking chairs
To while away the hours of an autumn in a life
No illusions of grandchildren gathered round and drawing near
To listen to stories of way back when and years ago

No dreams of newborn nurseries with wooden rocking chairs
To gently soothe the restless hours of spring in a life
No illusions of children gathered round and drawing near
To listen to favorite bedtime stories just one more time

No dreams of a world without the presence of a wheelchair
To make accessible the fragile summertime of a life
No illusions of replacing those old hiking boots
To draw nearer to God in his creation once again

No dreams of a world with seamless, endless guarantees
To string days upon days without illness, without winters end
No illusions of youths invincibility, immortality
To draw bravado and contentment with simply being alive

No old dreams remain, those dreams from before
To hold on to them is to grasp at a wisp of a ghost

No illusions of promises but no fear of tomorrow

To live life with passion, to create no regrets

"He that conceals his grief finds no remedy for it." Turkish proverb
"Give sorrow words. The grief that does not speak whispers the o'er fraught heart, and bids it break" William Shakespeare
"If you suppress grief too much it can well redouble." Moliere
"The risk of love is loss, and the price of loss is grief. But the pain of grief is only a shadow when compared with the pain of never risking love." Hilary Stanton Zunin
"There is no grief like grief that does not speak." Henry Wadsworth Longfellow

This is going to be a heart-wide-open, raw and vulnerable entry; there will be no attempts made to dress up my emotions in their Sunday best and tie pretty ribbons around the truth nor is it dressed in rags for dramatic effect. It is real, it is pure, and it is honest.
Lately I have been dealing with a grief I have never experienced before, and a grief that is difficult to process or explain to others. I hurt deeply, ache as though my heart may fall apart, have times of feeling bitter anger, and at times am sadly resigned to the situation. I am mourning the loss of so much of my life, the things that brought me such joy and defined who I was as a person and shaped my existence, and the loss of countless dreams that are now forever out of reach. It is not just a loss of what was but a loss of what could have been, a loss of the past as well as the future. In many ways I feel like some part of me has died. I go on with my life, I have good days and days of laughter and treasured memories, but something is achingly absent like a part of me has been cauterized. I don't want that old self to die, I don't want to surrender those old dreams, I don't want to admit defeat and have to allow this to change my life forever when I was so blessedly happy before. For the past 18 months I was able to lie to myself and hold on to the illusion that there was still some chance that there would be a treatable, curable diagnosis identified and I would be able to resume the life I had been living as if it had just been put on pause for a while. Now that I have a diagnosis of Mitochondrial Disease, I have to accept that there is not only no cure but no treatment. That this is a progressive disorder which may continue to steal away parts of my life, continue to rob me of dreams, continue to deny me of aspects of life that I use to define myself. If I did not live my life full out, living in every moment, living with no regrets, living at 100% with passion I would not have so much to grieve, but then I would not have so much love in my life either. It hurts more because I live more, I push boundaries, I defy expectations and definitions, I play by my own rules and love openly - love life, love others, love God.
Yet I mourn so many losses. I mourn the loss of my independence, for I have always been fiercely and proudly independent. I miss being able to just decide to go somewhere, to go exploring or on a short day adventure and not be limited to the ability of others to transport me. I mourn the loss of my physical abilities. I will never again chase after my niece and scoop her up in my arms as she squeals in laughter. My feet will never feel the rocky ground beneath them as I hike up the side of a large hill, using my arms to pull myself upward. Kneeling on a tiled floor I will never again guide the feet of a child as they take their first tentative steps. I mourn the loss of many of the things that brought me great joy and served as ways by which I defined myself - my work as a teacher and a volunteer, my passion for photography (it is much harder to access places to photograph and the angle is different in a wheelchair), my love for nature and hiking and exploring. I mourn the dreams lost, large and small. Dreams of ever being allowed to provide foster care or adopt children (I long ago understood that I can not risk having children of my own), dreams of hiking parts of the Appalachian trail, dreams of missions trips, dreams of dancing someday at my wedding, dreams of classrooms full of children.
I recognize that I can, and am, creating new dreams and a new sense of self but for now I am still mourning what I lost. The wound where it was ripped away is still too tender and too fresh to withstand the abuses of life without pain. It takes time, it takes patience, it takes growth of those new dreams and that new hope, and it takes acceptance of the grief.

Apparently this year there is going to be very little of the season that I adore called "Spring" and a great deal of the season that my body detests called "Summer". The dysautonomia leaves me rather like a reptile in that I can not adjust my body temperature very well, so when it is hot outside I am very prone to heat exhaustion. It would help if my body could make the effort to sweat, but by the time we get to that point I am already in trouble. While I also am miserable in extreme cold, I prefer winter over the heat of summer because you can always put on layers but you can only (legally) take off so many. Another huge issue that arises to the forefront this time of year is one that is virtually impossible for someone with a functioning brain to comprehend. I do my best to explain it, but it is as if I am from some weird other planet. I do not have the capacity, ability, brain signals, awareness to feel thirsty. I can and have easily gone 24 hours without drinking anything other than the sips of water required to take my medication. (On a side note, I also do not have the capacity, ability, brain signals, awareness to feel hunger or full like normal either. ) Obviously this poses a big problem because I am rather fond of my kidneys and they already take a beating from all of the chemicals they are asked to process from my medications, not to mention we know at least one has a cyst on it. Also, with my extremely low blood pressures being dehydrated is like playing on a tightrope blindfolded without a safety net - just a bad idea. To explain what I mean by not feeling thirsty, I never get a signal that my body requires liquids no matter how desperately it may indeed need them. When I do drink it is because I am forcing myself to do so or because a beverage tastes good. If my mouth is dry I chew gum or swish a small amount of water and swallow it and then have no need to drink more. To try to understand, imagine you have just eaten the largest meal of your life and are absolutely and unquestionably full. Now someone sets down a 7 layer chocolate cake in front of you and hands you a fork, telling you that now you must eat your dessert. You have no desire to eat that cake, your brain is not telling you to eat that cake but everyone else is telling you that you need to eat that cake. This is sort of what it is like for me to drink something - I feel no need to drink, because I feel no need I often end up feeling nauseous from drinking a full glass of something because my body does not recognize the need for the liquid, and I am content without touching it yet everyone is telling me I need to do it. I can't quite imagine what thirst feels like, but I do recognize that my brain is obviously malfunctioning and I think I understand the concept from reading and studying development. So we have tried numerous things to get me to drink. First there was the theory that I just needed to do it - the Nike approach "Just Do It". That worked as well as one would expect. Then there was the theory that what I needed to do was carry a water bottle everywhere with me and take a drink from it whenever I remembered. Here's a hint, if I don't feel thirsty do you think I remembered the water bottle let alone to drink from it? I left a trail of countless water bottles in my wake each with maybe three sips taken before I totally forgot. Now I am supposed to be on a 3 hour timed schedule. Every three hours, when I go to the bathroom (my neurogenic bladder requires timed elimination) I am then supposed to get myself something to drink and drink it. Any guesses on how well this is going? Some days I do really well, and other days I am lucky if I get 16oz in over the entire day. What is really bad is that I take Topomax, which comes with a big bold warning that it is imperative to drink while taking it because it is known to cause kidney stones. Oops. Thankfully so far my kidneys show no damage from a lifetime of abuse, and they keep chugging along. But how much longer can they take this? At my follow up MDA Clinic appointment this is an issue that I know we need to seriously address and problem solve because 1) I am constantly on the edge of significant dehydration, 2) this is not helping my low blood pressure, 3) I feel better when hydrated, 4) kidney stones would be bad, and 5) my kidneys are the only ones I have and I think they have been abused enough.
I should write another time about how weird it is to have an abnormal/absent sense of hunger and abnormal/absent sense of full and the joys that brings including frequently forgetting to eat until my blood sugar plummets. I don't do much better eating on a schedule than drinking on one most days. :P With that glitch you would think I would be skinny, but nope - I have my back up belly in case the dysphagia gets severe again (that's what I am calling it, so it seems purposeful instead of just weight gain from the inability to exercise anymore).

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.

* Photo from the last hike I took in September 2008, Prince William Forest.*

I had all sorts of plans for splendid entries on this journal (I still cringe at the word blog, I do not know why - maybe because it rhymes with "frog" and I have serious issues with amphibians?). Entries about the mixed emotions of sending off the blood work for the DNA testing for mitochondrial disease, entries about the amazing day that Mother's day turned out to be, entries about how when you are in a wheelchair you are all too often invisible both literally and figuratively and how this nearly turned me into a hood ornament for a Buick. But this crazy little thing called life kept stepping in and changing up my plans. So now I will be presenting My So Called Life, the abbreviated version.

* A week and a half ago I finally had the opportunity to access a hospital based laboratory to draw the single (Large!) tube of blood required to run the DNA testing for Mitochondrial Disease. The technician was very pleased with how "idiot proof" (her words) the self contained kit was, and I was pleased that I did not have to put that theory to the test at the scary, run by idiots local hospital. The downside of living in a very small town is that the medical care locally is sketchy at best - my primary doctor is a good 45 minute drive away, my specialists are about 90 minutes away. So now comes the waiting for results. I have mixed feelings as to what to pray for with the test results - the Dr is confident enough in his diagnosis that he has registered me to receive services through the MDA, and this diagnosis is the first thing that explains my entire medical history so a positive result would be a nice confirmation and would spare me the need to undergo a muscle biopsy. Given that I am on blood thinners, a muscle biopsy would be a bloddy mess and given that I have severe dysautonomia, a muscle biopsy which requires sedation would be a tightrope walk. Yet it just feels wrong to pray for test results to be positive for a disease that has no cure and no treatments. Not that any result is going to change what is going on inside of my body - either way there is a progressive disease process active and frequently making its presence known.

* On a much happier topic, Mother's Day was a day filled with priceless memories that I will treasure forever. My brother managed to orchestrate a series of surprises for just about everyone involved and had my two aunts come in to town to meet up with us for lunch. Neither my mom nor I knew anything - we thought we were going out to eat with my brother's family-, and my one aunt did not know the other was coming. I forgot to take my camera so I have no pictures but it was so much fun. Someone at the restaurant made the mistake of positioning the 9 of us front and center in the restaurant, obviously not comprehending the fact that any family gathering is a loud and rowdy affair. :) I spent half of the time at lunch at the "kiddie" end of the table with my nieces and nephew - well my nephew had his iPod in his one ear and was busy texting so the only time he spoke to me was to call me a tattle tale when I warned his mom that he has sabotaged her drink (I called him a teenager and he returned to sulking), and my older niece was constantly running from the table to check the score of the Tigers baseball game when she was not sulking because she stayed up too late the night before so really I visited with Little Bit. Little Bit is highly creative and can entertain herself for hours with just about any found object, so we were thrilled with crayons and a piece of paper but I did have some trouble following the ever changing rules of her games. Suspiciously, the rules changed every time she started to lose. :) I spent the other half of the time at lunch with the "Big People" at the adult end of the table where there was much laughter. After lunch, my one aunt came back to my mom's apartment to visit and we had a great time. I also had the honor of giving my mom a very special present that has a great deal of meaning to both of us.

* Being in a wheelchair is often like donning Harry Potter's Cloak of Invisibility. Suddenly people talk over your head, to the person with you, and about you but never to you. People will look everywhere but at you if you do talk to them, and they feel some bizarre compulsion to both shout and to revert to near baby talk. Because I obviously became hearing impaired and lost more than half my IQ points the minute I sat down in my wheelchair. What I failed to realize until the other day was that I am also literally invisible to many people. I was rolling in a parking lot at a large superstore, when all of a sudden a large vehicle whips out of a parking spot directly at me at about 30 mph. I used my emergency brakes (my hands) and my horn (screaming) to alert the driver and by the time we both came to a stop there was no more than an inch between my knees and her vehicle. I must admit that with enough adrenaline pumping through my body to pick up and toss her vehicle, I used a few words that I would not normally use in public and asked her whether or not she had seen me. She protested that I was in her blind spot, to which I answered that this is the reason as a driver you check your blind spot before whipping out of a parking spot and I questioned whether she should still be allowed to drive. I am now skitterish to even venture into parking lots and Lord help me crossing streets!!

* This week I got up, bathed, dressed, and out of the house four days in a row - Sunday for mother's day, Monday to pick up a prescription, Tuesday to go shopping for warmer weather clothes (last spring/summer I was still skinny from the dysphagia, this year definitely not so much), Wednesday to pick up the remainder of the prescription that they had not bothered to order in time for Monday and to return to my apartment (I had been staying with my mother for about 2 weeks). None of those days except Sunday and Wednesday involved getting up before noon, and those days I got up at about 10:00am. I was back home by 4:00pm every day. Yet my body is making me pay for so much activity. The dysautonomia is having a temper tantrum - I can not maintain my body temperature anywhere near normal, my blood pressure is running lower than my new normal, my GI motility is all messed up. The neurological disease is collecting its toll with headaches, severe muscle pain, and an increase in the dystonia and poor muscle control and balance. We are working out the terms of a peace treaty now, and I am hoping that by next week all will be forgiven especially since Monday I have a Dr appointment to evaluate my legs for Botox treatment as well as an EEG. I highly doubt the EEG will show anything because even if I were to have seizures I am on enough antiseizure medications that it should stop anything fast.

* I realize this post has been neither stunning nor brilliant, nor particularly entertaining. It has been long, if that counts for anything :) I shall work on the next entry and try to achieve brilliance, creativity, stunning humor, and perfect length. Hey, it is good to have goals :) Bonus points to you for making it to the end of this!!!

I remember at one point not all that long ago having this mysterious substance called energy. I think I got a hole in my fuel tank because energy is a very rare commodity. Today my BIG, HUGE accomplishment was getting dressed and going to the store Right Next To my apartment building. It is so close I can see the back of the store from my window. I managed to roll there, with a stop to rest once I got inside the store, and get basics to stock my cupboards and fridge for the next few days. Coming home was easier because the rear driveway of the store is a HUGE slant, and the very best part of the exhausting trip. I perch at the top of what is basically a launching ramp and check carefully for traffic on the side street at the bottom. Thankfully this is small town Michigan, so traffic is rare and I can spot any vehicles easily. Then I eek my chair out over the edge of the driveway and throw my arms up in the air. At the bottom of the downward trajectory is a double bump of curb and pothole that adds some serious air time. I usually come to a stop somewhere in the middle of the road, although I have made it completely across the road before. This feat scares the dust out of the mainly elderly population that happens to witness it. So, anyway, by the time I arrived home I had used all of today's supply of energy and more than likely a good share of tomorrow's. There are many dishes in the sink waiting to be washed, and the floors need to be vacuumed and mopped (hint, that takes many days to accomplish and I may be seeking assistance with that as it eats up about a week of energy), and I am too tired to do anything about it. Not sleepy tired - although I did crash with some weird system errors earlier (I was violently shivering, freezing cold, every muscle hurt, and I was groggy) and took a nap then- but physically exhausted to the point that I can feel the weight of my eyelashes tired.
Here is a good example of tired. My blood sugar crashed this evening, and I was in the kitchen fumbling to mix up a protein powder that I keep for when I don't feel good enough to eat. I was having trouble opening the new milk jug, so I placed the half gallon container between my knees (now would be a good time to point out that my knees rotate inward with spasticity) to hold it and then firmly twisted the lid. Apparently my knees were applying significant force to the jug because I created a milk fountain. After a few choice and creative curse words, I looked at the large puddle on the floor and looked at my pajama pants which were already wet. I then stripped off my pajama pants and tossed them on top of the milk puddle and let them soak up the mess. I was too tired to go get a towel, or to dig under the sink for a roll of paper towels. I was then too tired and shaky to go get new pajamas, so I wore nothing but a t-shirt until the next time I went into my bedroom for medicine and then I snagged a dry pair of pants. The shakiness is gone thanks to the milk protein drink, and my soaked pajama pants are spending the night in the bathtub until laundry day tomorrow when I will pay a little extra to the wonderful woman who does my laundry for having to deal with stale milk smelling pajamas.
My BIG goal for tomorrow? I am running out of dishes, so I think I need to wash at least a few of those so I have some plates and bowls to eat from.

I am sleepy as I write this, so the odds of it making any sense at all are at best 50/50. :) Make your wagers now! This photograph is of the image on my new favorite T shirt from Zazzle. I may rely upon a wheelchair for mobility, but I still have attitude. For a while now I have been feeling like my life is stuck in this sick and twisted version of the movie Groundhog Day where each day is a repetition of the day before and there is no escape. I thrive on having to think, to problem solve, to be creative, to engage and interact, to create new ways that have never been tried before, to celebrate successes that to the outside world seem insignificant but to the world in which my heart dwells are achievements akin to climbing Mt. Everest backwards while blindfolded. For too many months my life has been on hold. I have been in a stagnant holding position, a medical limbo, a place of existing but not living my life to the fullest. That is not me. I need to live my life and live it out loud, to live it to the limits, to live with passion and pursuing the gifts I have been given and the marvel of truly being alive. I can either wait in this holding position to die, be that at age 35 or 75, and just wander through the intermittent time, or I can take control back in my life and live every single moment that I have no matter what life throws at me. I will rebel against this disorder that continually whispers "you can't" "you lost", "that is impossible" and throw in its face all that I can do, all that is not lost, all that is possible. I will chase my dreams, even if those dreams have been changed slightly, and I will not remain stagnant any longer. It will take time to get a plan of action in motion, but I am beginning the process. I am breaking free of the hold that this has had on my life and declaring that I will live my life boldly, unabashedly, in faith and mercy, and refusing to sacrifice a moment. I must create something powerful, something beautiful, something of change and impact from this situation. I must choose to live instead of just being alive. In the future I see myself returning to college to pursue a Master's Degree, regaining my independence so I no longer feel trapped by relying on others for my access to the world, and reclaiming the passions in my life. I, and only I, will define who I am and what I am capable of, and my life will be limited only by my ability to imagine and to believe and to create adaptations as needed.
If this sounds dramatic, I apologize but this past week has been rough emotionally. This was a break through to realize that life will not be like this forever and that I can change so much of my reality. This life is mine to life, or mine to surrender. I am a rebel with a cause again!
P.S. Please let me know if this does not make any sense and I will rewrite it when I am more awake. I am very groggy and sleepy but wanted to write this tonight.

There are bad days, and then there are bad dysautonomia days. Today is a bad dysautonomia day. I have spent the entire day curled up on the couch and unable to maintain an upright sitting position for more than 5 minutes. Toasting waffles for dinner was a race with my blood pressure to see if the waffles would be toasted before my blood pressure hit the floor (and took me with it). Waffles won, but not by much. I have fought to stay hydrated, which is rather difficult when you never feel thirsty and even more difficult when your intestines decide that they need to rebel and stage a mass evacuation drill and even more difficult when your stomach decides to try to work in reverse, causing severe nausea. I have been alternately a good degree or so below normal body temperature, huddling under my favorite blanket, and a good two degrees above normal, my skin feeling on fire and burning to the touch of anything. My heart decided to join in on the fun and created some funky new rhythms as it also alternated between not quite too slow and not quite too fast. Yet with all of that going on, I still laughed today. I still shared jokes, I still celebrated friendship, I still wrote, I still listened to an audiobook, I still watched favorite tv shows, I still planned and schemed and dreamed. I still lived and laughed and loved. Even on a bad day, on a day when I wanted to cry but honestly could not find the energy to do so, I lived each moment as much as I could. That is a priceless lesson I have learned from this Mitochondrial Disease- even the bad days need to be lived to the fullest. And just for the record, I still hate Pollyanna.

Courtesy of Dysautonomia and the working diagnosis of Mitochondrial Disease, I have no functioning sleep wake cycle. Circadian rhythm is a joke. My poor brain is very discombobulated and without chemical assistance is never able to fully awaken and is never able to fully fall asleep. It is like an endless night of the living dead. I take my "lets convince the brain to wake up medicine" as soon as I get up in the morning, but there is about a one hour lag between when I take them and when they convince my brain to wake up. That one hour has led to some great family stories of the hysterical things I have reportedly said and done, and more than a few unexplained bruises. This morning was a great example of my brain NOT on drugs.
I made myself a cup of coffee, which I can do in my sleep thanks to a coffee maker upgrade back last fall. You pour boiling water across your lap once because of a combination of not being awake and poor arm strength and you quickly problem solve. I love Klaus my Keurig coffee maker. So I took my heat resistant, silicone lidded cup of coffee with me into the bathroom where I get dressed (I know, some may say eww but I was not using the toilet or flushing it so hush). I set my coffee down in the bathroom and then went to gather up my clothes. I then tossed my clothes into the bathroom (my wheelchair does not fit - by a freaking inch- so I transfer to a walker) and went to turn the light on. I stared at the two switches. I tried one, but that only made the room darker so that was no good. I put that back where I found it. I tried the other one, but that just made an annoyingly loud noise, so I put that back where I found it. OK, come on I know one of these had to be the light switch! TWO minutes later I realized the lights were already on!! Nice. As I was getting dressed I discovered that when I got dressed yesterday morning after my bath I had not only put my underwear on backwards but inside out - and had not noticed it for 24 hours. Bonus. I managed to get dressed with everything in the correct position, sipping coffee along the way. Teeth brushed, hair pulled back into sloppy ponytail, I finally looked in the mirror. I decided I needed to apply Cetaphil. I took the lid off of the jar and stuck my finger inside only to discover I had just stuck my finger in my coffee. Coffee as a facial was not going to do it for me. I tried that again with the correct lotion which worked so much better. Then I came out into the living room to put on my AFOs and shoes. Putting my AFOs on is a wrestling match (if you want to know why look at the post two entries ago with photos of the spasticity and dystonia in my feet), so at first it did not occur to me that I was having an unusually hard time getting my brace on. Five minutes later it occurred to me that it was the wrong brace. Odd thing is that the curve of the wrong brace was a more comfortable fit to my foot in many ways - it let me keep them curled.
This is why I have a rule with anyone who might come in contact with me during that hour lag period that nothing I say or do can be used against me. I am not to be held accountable to anything you might convince me to agree to doing and anything I say that makes no sense can not be used to humiliate me. The same thing applies to the lag period between when I take my "convince the brain to go to sleep medicine" and actually fall asleep!!

On Thursday night I completed an at home study to determine whether I am able to maintain my oxygen levels while asleep and whether my heart rate drops during sleep. It was a simple test, all it involved was wearing a pulse oximeter on a finger and sleeping with it. The challenge was in the poor planning of the designer of the system. Actually, the first challenge is the fact that in my sleep I can escape from anything and everything regardless of its medical necessity and have no memory of doing so. While in the hospital I often came close to removing my IV in my sleep because it was bothering me. Bandages have been removed, socks are always removed. So I figured the little finger clip had no chance of lasting through the night. Considering my tools at hand, I broke out the medical tape and fastened that clip to my finger in such a way that I would wake up before victoriously removing it from my finger. Houdini would have been proud of me. So back to the challenge posed by the designer. The finger clip plugged into a monitor and recoding box about the size of an old cassette walkman, and there was exactly 12 inches of cord between the clip and the monitor. Just enough cord to tangle and to hang myself. There was no way to set the monitor beside the bed, it had to climb on in and sleep with me. Not an issue until you consider that someone thought it would be helpful to have color coded LED lights visible from the moon on the recorded that flashed approximately every second. I tried stuffing it in a sock to smother the light and it made no difference. Finally I smothered it with an extra pillow and fell asleep. I woke up at one point during the night to the shock of my left arm being violently jerked straight out from my body as the recorded plummeted from the bed and, since the clip was fastened to my finger securely, took my arm with it. Trust me, that wakes you up quickly. It looked like I was fishing for alien testing equipment over the side of my bed. I reeled it back in, apologized for having committed the crime of moving in my sleep, and promptly fell back asleep. So I should have any results from the test next week some time. One funny moment was just after getting it hooked up and laying down I got the hiccups. In order to stop the hiccups I took a deep breath and held it. I then watched as the oximeter slowly began to register down to 93...90...88..86. Oops, but it got rid of the hiccups!! I included a little note explaining that one on the printout.

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.

Today I heard words that I never expected to hear, words that I was too battle weary to hope for anymore. As I sat in my curtained off section of the physical therapy office converted for the day into the MDA clinic, I listened in a bewildered daze as the most amazing of doctors informed me that ALL of my symptoms make sense - that as he listened to me and reviewed my chart he immediately began to get an understanding of what he thinks is going on. After spending 28 years with an ever growing list of medical issues that no one could ever put together or explain, and after 18 months of extreme frustration in which I have watched my life change in ways I never imagine, he thinks he knows where we need to focus our attention in diagnosis. He gave the monster a name, and he is confident in pursuing this diagnosis. It was not the monster I was expecting to possibly hear called out, but I am so thankful to have a name for it, to have a direction, and to have new found supports. The monster is Mitochondrial Disease and the fight is on!
I qualify as a patient under the MDA clinics, and so will be continuing to see the neurologist and team of specialists there. We are also going to do genetic testing for the specific subtype of Mitochondrial Disease that the doctor is considering most likely. In the near future I will be having an occupational therapist evaluate me at home for safety, functionality, and to suggest any equipment or changes that could be of assistance. We are working to set up PT, OT, and SLT (speech language therapy) that will be covered by my insurance and that I can afford. I am also going to undergo testing to make sure that my oxygen levels maintain an acceptable level during the night. Finally, I was started on a low dose of Florinef (sp?) to attempt to coax my blood pressure into a more acceptable range as the neurologist feels that just the constant low blood pressure can cause severe headaches, fatigue, difficulty concentrating and with memory, dizziness, and shakiness which certainly is not helping the situation.
I physically am not any better than I was this morning; but by having a name for the monster I feel empowered. By having a name for the monster we can also have a general understanding of what is within the realm of expectations, and what can be done to fight back. By having a name for the monster I can educate myself, I can access "community", and I can see the monster for what it is, and for what it is not.
If this is a bit jumbled, I am very tired after a long day and I am still processing this information. I am thankful for the amazing neurologist and for the hope I received today, and for the MDA clinic that allowed me access to top specialists. I just need to get acquainted with this monster and then tuck it away in its place.