The continuing bumpy, lumpy saga of a hobbyist equestrian whose life was turned upside-down and inside-out by rare diagnoses and ultimately, brain surgery. Mmmm...braaaaains. Ooh, and horsies!
One of them, Kristy Boxberger, set up a GoFundMe page for me so others could hear of my story, share, and donate if they were able. I wanted to be sure and share it here, just in case, you know, stuff.
Y'all are amazing. Seriously. "Thanks" does not begin to describe the depth of my gratitude.
And now, speaking of giving thanks, here are a couple of pics from Thanksgiving Day 2014. These definitely capture the spirit of Britvec Normal.
Just about the only kind of selfie I make, because the photobombers are so cute
The beach is a pretty awesome place for Thanksgiving morning...even if seconds after hubby took this pic, big bro came up to us complaining little bro was throwing sand. Again.
I swear, hubby smiles. We joke he's like Agent Mulder--this exchange from the X-Files movie is the only thing I remember about it because it perfectly describes stonefaced (but handsomely so) hubby: "You were scared! You had your scared face!" "What?! This is my scared face!" :::completely expressionless::: "THAT'S IT! That's the face you made!"
I feel awkward and embarrassed sharing this, but realized if this were a good friend of mine, I'd be sharing the GoFundMe link to support them. So...I'm being a good friend to myself.
And so, uh...here's a picture of my eldest drying off ever-tolerant and patient Firefly yesterday before putting her rain sheet on, because, CUTE.
He said to show this to his dad, saying, "Look! He found a green horse!" Thus his facial expression. Firefly's expression says, "Whatever. Just feed me when you're done."
I had my follow up with Dr. Bolognese on Friday, November 14, via telemedicine (Skype). During that appointment, he referred to this image of my cine MRI:
Braaaaiiins
Um...pretend this is animated, because it's supposed to be, but a screen capture is all I could manage. Cine MRI=cinematic MRI; it shows the pulsation of cerebral spinal fluid, not just the anatomy. He said we need to see two columns of cerebral spinal fluid (CSF) pulsating on either side of the spinal cord. (CSF it the light colored lines outlining my spine, going up into my skull.) What we see is one line on the left (anterior) clearly going up into my brain, and the one on the right (posterior) going up and...stopping. Where we should see CSF, it's black, indicating a significant blockage leaving that part of the skull starved of the flow of spinal fluid. My brain herniation is plugging the flow of CSF at the base of my skull like a cork in a wine bottle, he said. While the size of my herniation may be a cause of debate for docs, he explained any doctor with any knowledge of Chiari would look at this MRI and say, without question, I have a problematic Chiari malformation--not just an "incidental finding" as some may have said. Or, in fact, as some did say.
On top of this, my headaches are getting worse. More frequent, more severe, more constant. Truly, I have a near-constant headache now, often just a dull ache, but not uncommonly a throbbing, inescapable pain. The one I have now is enough that it makes it harder to type, but I'm pushing through, because it's what I've done for so long. One learns how to live with pain when it cannot be fought. I'm grateful this one allows me to function, if in its own annoying interfering way. Jerk.
Starting about two months ago, the headaches started getting so severe when I lie down on my back, adding pressure to the back of my head, that my eyes started going bloodshot. The process of getting cine MRI pictured above, in fact, was absolutely torturous. They were unable to get the images they wanted, so it took three attempts and 45min instead of the 15min they had casually said it would require before beginning the imaging session. The pain in the back of my head grew intense, and unable to move during the test and relieve the pain and pressure, it swallowed my head whole, bringing nausea and severe eye pain with it. Finally finished, I was dizzy a bit when standing but hobbled to my changing room, grateful to be upright again. It was when I was using the mirror to put my earrings back on (because you do NOT want to wear metal in an MRI--have you seen the video going around Facebook?) that I was taken aback by my eyes. They were red, from the outside corner to the iris. I continued changing, and the eye color improved as I was upright, but I grabbed my phone to document this bizarre visual. It's a bit tricky getting a picture of one's eyeball with an iPhone, but I managed a few that somewhat captured the bizarre look.
My, what pretty eyes you...ewww, never mind
It repeated with the nasty headache I had after my son's Trunk of Treat Boy Scout event.
Appropriate for Halloween, I suppose
And really, has repeated to where it's a near daily occurrence--like my headaches, it varies in severity. Dude, the body is WEIRD.
Dr. B reassured (rather unnecessarily, as I wasn't concerned about it as much as I was curious) that the redness isn't due to a hemorrhage or anything like that, but are turning red like this as the nerves react to the pressure. He said it was a bit like going pale when feeling faint.
On top of all this, I was diagnosed with sleep apnea back in September and fitted for a CPAP machine. That's just...weird. Apnea? Huh? I've never had an issue with that, and I don't fit the typical profile. And yet, around February or so, my husband nudged me awake for the first time in our 13+ years sharing a bed to alert me that I wasn't breathing. Whuh? Turns out, both central apnea--where the compressed brain stem stops sending the signal to breathe--and obstructive apnea are extremely common in Chiari. A study showed 73% of adult Chiari patients had one type of apnea or the other. (The good news? 90% of that group saw their apnea disappear after decompression surgery.) Thankfully, my type of apnea is obstructive, where the muscles in the back of my throat relax and collapse over my windpipe; while not fun, it's not as lethal as central apnea. They aren't sure why this type of apnea is so common in Chiari patients, but one theory is the compressed cranial nerves interfere with the signals to the throat, causes the muscles to go lax. This is also why some patients have issues swallowing, which I haven't noticed being an issue.
All of these recent additions to my body's par-tay--the clear blockage on the cine MRI, the increased severity and frequency of the headaches, the eyes going bloodshot with the headaches, the sleep apnea--resulted in Dr. Bolognese offering me surgery.
Brain surgery.
He cannot guarantee it will help all of my symptoms, as neurological issues are bizarre and unpredictable. But, he is confident it will greatly reduce my severe headaches, eye pain, and the feeling of pressure at the base of my skull. The dizziness, balance issues, limb tingling and numbness, tremors, hyperreflexia, joint and body aches, heart palpitations, vision issues, fatigue...a reduction (or erasure) of these would just be icing on the cake.
But still... EEK. Especially considering the procedure he recommends involves cutting out the herniated portion of the brain. He said it has not function, BUT STILL!
I asked Dr. B flat out about riding my horse, which I haven't done for a few months due to muscle weakness, numbness, spasticity, and balance issues. He recommended the same for both pre and post surgery for me--leisurely, walking trail rides only. (And that's him knowing I'm a somewhat skilled rider--otherwise he would tell a nonrider not to get on at all). I cannot risk head injury or being bounced around much. This is both terrific news--I can get on him again!--and heartbreaking. I'm still processing the reality that brain surgery is in my future--this is just another piece that's terribly hard to swallow. Is it really...never again?
There's no knowing. For now, I'm grateful just to be able to get back on him, when I have the strength.
So I did.
I was still smiling on the way home
I have a friend's Barefoot Cheyanne saddle out on trial, and so far, I love it. It's light, comfortable, and secure, and yet I still can feel him underneath me. It's perfect for our 20 minutes of walking only.
Tril also looks adorable in it.
He looks adorable in everything, actually
Monday, shit got real when Bolognese's surgical coordinator called to schedule my decompression surgery. She still needs to confirm with the hospital to make sure the OR is available that day at the hospital, but right now, my surgery is set for January 13, 2015. In New York.
Eep.
Our initial plan was to bring the boys along, but the more we think about this, the more we are rethinking that. For one thing, there's the length of stay. I need to be at the hospital on January 12 for presurgical testing, and I do not want to fly in on the 11th to dive straight into that. So, we'll arrive the 10th, maybe, then I have surgery the 13th, then I have a 4-5 day night hospital stay, and then I won't be cleared to travel for at least two days after discharge--which means we'll be in New York at least 10 days. A weeeeee bit rough on and with young boys.
New York--in freakin' January. Yaaaaaaaay
I'm trying not to be overwhelmed by everything. The flight, the traveling itself, finding accommodations, the animal care, the boys' care (whether they're with us or not), the insurance headaches, the money--holy crap, the money--and lest I forget, the surgery itself. EEP.
One morning this week, feeling overwhelmed by everything and physically particularly crappy, I decided to sit in a corner of Tril's stall and sip my morning coffee, breathing and taking in the sounds of the birds and the horses eating their breakfast.
Of course, they had to thoroughly inspect it for carrots and peppermints
Worrying wastes my energy, which is in such precious supply. Breathe, be in the moment.
(Every now and then, that moment is full of EEK! and some tears, admittedly.)
I have hope that my life can begin anew starting January 14. Until then...breathe. (Freak out.) Breathe. (Freak out.)
But--the day my surgery was scheduled, I had such a severe headache I couldn't freak out. All that mattered was relieving the pain--and having something on the calendar, with relieving the pain as the ultimate goal, is actually...exciting. Hopeful. Scary as hell, but hopeful.
Okay, so it's been a long time. Like, a long time. But! But! I have a good excuse! I have a doctor's note! ...Or three. Or ten. Or...hell, I lost count a long time ago.
I eased off my blog several months ago when my health took such a turn that I could no longer work, and as the fun-filing-paper-pushing started, I thought it best not to blog. Well, that and my energy comes as a rare commodity, and it usually got sucked into something else online like, you know, important things. Uh...like Facebook.
In this time I've been off-blog, I've been busy collecting doctors and diagnoses like Breyer models. First diagnosis, as I posted about here, was Hashimoto's thyroiditis, an autoimmune thyroid disease. Then came the nebulous fibromyalgia. Then came Arnold Chiari Malformation, type 1, followed most recently by obstructive sleep apnea.
The most problematic--in many freakin' ways--is the Chiari malformation. The whosie-whatsis? Chiari--as in:
I shall put my teacher hat on (actually, I never really take it off; I think it's grown into my skin now and there's no removing it) and explain.
The back of the brain has these parts that hang down in the back, under the cerebellum, called the tonsils. For most folks, your brain and tonsils look something like this:
Ah, it's so cute
Lookit all that happy space! Uh...no, on the right. Up more. Okay, see that tube thingy coming down out of the skull? The spinal cord? Well, follow it up into the skull (the nobby thing it forms at the top is the brain stem). Around where that tube thingy curves slightly to the left on the right you see a blob of brain--the tonsils. See all the dark grey? That's the cerebral spinal fluid, flowing happy and free. The tonsils are up a good centimeter or so above the skull opening, and all is as it should be in brain tonsils-land.
Then there's me:
I actually fell asleep during this MRI. Zzzzzz...
My tonsils hang down out of the skull opening, a centimeter lower than they should be and about half a centimeter out of the skull. See how the cerebral spinal fluid ain't so happy and free? Turns out, over time, cramped CSF starts getting all pissy and acts out in bizarre ways.
Okay, still a bit unclear, I'm sure...so, here's a labeled diagram I made using fancy software (or, just what came on my MRI CD).
Look, arrows...oooohh
The cerebral spinal fluid/CSF is narrowed and perhaps compressed in the areas marked with arrows on the left and top. The right arrow measures the herniation, which I showed as 3.4mm but officially on a few radiology reports is 4mm. Cramped CSF and a brain stem that's all emo about getting touched work together to create a bizarre collection of symptoms.
Let's see...well, there's the classic Chiari headache, severe, dull, unresponsive to any medication, that seems to grab the back of my head and squeeze it like a nutcracker. The pain radiates forward and out my eyes. In short, it sucks, like, a LOT. Lying down makes the pressured pain worse; oftentimes, a lot worse. During a recent MRI, where I was required to lie still on my back, the pain became so severe I grew dizzy and nauseated. It was excruciating. Then, bonus, when using the mirror afterwards to put my earrings back on, I was shocked to see both of my eyes were severely bloodshot, presumably because of the intense pressure.
Sometimes the headache just appears, and sometimes things like coughing fits, loud talking, singing, and even laughter trigger it. Sneezing? Well, with a lot of Chiari patients that's a trigger too--but for me, sneezes don't trigger pain. They trigger the feeling of a thousand ants crawling up the side of my head. Yaaaaay.
I tell people I have this big grab bag of symptoms, and every day my body reaches in and grabs a couple of fistfulls. There is no real rhyme or reason. I've lost temperature sensation in my lower right leg (touch me with an ice cube, and I know you're touching me, but don't feel the cold); I have constant hand tremors that vary in severity; I have obnoxious fatigue that my mother has described as me being half dead, not just asleep; my muscles will spasm with minimal use, painfully seizing up in protest like I've run a marathon; my vision will go hazy, like looking through dirty glasses; words get tangled up in my mind and struggle to exit my mouth; I stop at green lights because of bizarre "brain fog;" I'll become dizzy out of nowhere; I'll become nauseous (and occasionally dry heave or vomit) out of nowhere; my hands will swell like when I was 9mos pregnant; my joints will ache and throb in body-swallowing pain...yeah. The list goes on. Par-TAY.
Worse, though, is Chiari has the double whammy of being rare and misunderstood. Doctors are uninformed, and pass that misinformation on to their patients. Despite the research everywhere saying herniation size doesn't matter, symptoms do, I've had multiple doctors tell me my 4mm herniation is too small to be behind my symptoms. I've also had a neurologist and two neurosurgeons mismeasure my herniation and try to tell me I don't have a Chiari (yeah, no; three radiologists and two experts disagree).
Frustrated, I was ready to give up on Chiari completely when a young woman I met on an online Chiari support group encouraged me to send a Hail Mary pass to Dr. Bolognese, a specialist considered to be one of, if not THE, world expert on Chiari malformation. UCLA neurology, no slouch themselves, defer to him and called him and his former work at The Chiari Institute (he now has his own Chiari Neurosurgery Center) as the best in the world. I shrugged and filled out his 19-page questionnaire, packaged it up with copies of my brain and cervical MRIs, and sent it to him in New York. I was shocked when his office called wanting to set up a Skype videoconference appointment--he uses this often with his out of state and international patients. Bolognese does not see you, in person or on Skype, if he doesn't think he can help you. So, hubby took the day off and we spent nearly an hour Skyping with Bolognese, whom we found funny, attentive, thorough, and innately curious--he wants to know what is up with me.
Hi, I'm Dr. Bolognese, and you're not
He described how many doctors are very conservative about treating Chiari, which is understandable as the only treatment is a type of brain surgery called decompression. These docs only operate when the herniation is 5mm or greater (come ON, one measly millimeter?!) and all the boxes are checked indicating Chiari is an issue. This gives them a high success rate; however, he said such an approach inevitably leaves some patients out in the cold. Since Chiari can be so bizarre and not give a crap about what boxes it checks, a lot of patients can be left out in the cold in this approach.
Dr. Bolognese appeared on this episode of Mystery Diagnosis, describing and treating this poor girl's combination of Chiari Malformation and Ehlers-Danlos Syndrome.
But, when you get a 4mm herniation in a patient that "smells funny" like me (we laughed when he said that and hubby starting sniffing my hair), you go in for a closer look. And well, that's what we're doing right now: putting on the magnifying lens and putting our noses to the page to see what is revealed. I just sent him a copy of a special MRI that studies the flow of cerebral spinal fluid; we'll see what he says, hopefully by the end of the week.
I sent him a few still images, including this one:
Seriously, world of Tron-weird, ain't it?
What you see on either side of the spinal cord is the dark grey CSF, and it appears at least in this image not to get past the location of the brain tonsils--indicating a blockage. Dr. B stated at least as much in the email reply, with the important disclaimer that he can't say for sure until he sees the entire study.
Bonus, I was diagnosed with obstructive sleep apnea about three weeks ago. That is completely out of left field for me; I don't fit the type, and it just...showed up out of nowhere back in February or so when hubby first nudged me to tell me I wasn't breathing. I am getting a CPAP hopefully in the next week or so (son is dressing as Darth Vader for Halloween, but I get to BE Darth Vader! Oooh aaah, ooh aah...). Apparently, apnea of both types is extremely common in Chiari. The scarier type is central sleep apnea, where the compressed brain stem "forgets" to tell the body to breathe. Mine, obstructive, is thought in the case of Chiari to be because cranial nerves are compromised and the throat muscles relax more than they should, collapsing into the airway. Dr. B said that could in fact be one more piece of the Chiari puzzle...complicated little bastard, isn't it?
I am now a full time patient, and it sucks...but I still get out to the barn. I don't trust my body to be on Trilogy's back for now; my balance is wonky and my muscles are weak and numb, and the last thing I need is a fall. He wouldn't throw me, but that doesn't mean I couldn't wind up coming off of him. Until the dust settles, I'm staying off of him.
But I'll be damned if I'm giving up my barn time. Enter Firefly and harness-driving goodness:
"Waaaaiiittt...this is work, isn't it?"
We all know who the biggest horse is in this arena
Throughout all this, Trilogy has been worked in hand, and Firefly has begun working in earnest. It is so much easier on my body while remaining vital to my soul. What's additionally awesome is my kids can ride with me--each boy can sit on either side, and we go for a ride, blowing bubbles and chatting about anything and everything.
I hope to have Tril trained to pull a cart as well; he has the mind for it, and his back issues will be moot when there's no weight on it.
And now, I must sign off because--surprise surprise--my head hurts. But I promise to be back before, uh, another 10 months have passed. F'reals.
Happy trails are happier when there are treats involved
Tril: "I'm feeling playful! Ooh...WHAT THE FUCK IS THAT?" Me: "It's a manhole cover, dumbass, same one that was here yesterday." Tril: "But it might eat me today!" Me: "WHATever. Move on." Tril: *pout* Me: "Dude, walk a straight line, would you?" Tril: "But...what's THAT? And THAT? And..." Me: "Knock it off. I know you're not actually scared. Walk a straight line." Tril: "WHATTHEFUCK!" *props and freezes in place* Me: "F'real? Tril, move forward." Tril: "NO! There's a THING!" Me: "TRIL. I know you. You aren't afraid of anything. But you love to play with everything. Move forward. ...Still no? Alright, then." <use excess rein to smack him on the butt> Tril: "OH! Oh. Oh, I see you actually mean it. Moving forward then." Me: <rolling eyes> Tril: "OHMYGODWHATTHEFUCK is THAT?" <spooks hard in place, spins on his haunches, tries to bolt home when a flock of birds take off--REALLY? BIRDS?> Me: "Oh NO YOU DON'T." <shorten one rein, spin him back the other way, kick him forward> "Enough of this crap." Tril: *sigh* "Well...now I'm bored with this game because you won't let me play. I'll walk along at a leisurely pace, in a straight line, and on a relaxed rein now." Seriously, he was perfect the rest of the ride. The manhole covers, weird looking tree stumps, birds, Mysterious Things et al didn't even cause him to do more than flick an ear again. Somewhere, his antics have worked for him--but not with me.
