I'm not crazy.

Since seeing Dr. Liu in October, I have actively been trying to find a surgeon to treat my neck. At this point I have reached utter desperation. Though I can't have surgery yet, I know how difficult - if not impossible - it is to find doctors that treat "people like me".

Before traveling to Florida I tried Dr. Sandhu, Dr. Henderson and this really awful guy named Dr. Patel. Previously to finding Dr. Liu I had also tried a few others ("spine specialists") who laughed in my face about neck instability. I've learned that anyone with the title "orthopedic" anything behind their name do not like people with EDS. I've been told no by so many "medical professions" in my life who just don't care. I've made up my mind. I won't get told "no" or "there's nothing wrong" anymore.

So I've declared: no more messing around. It's go big or go home..which would basically mean becoming bed ridden again. My great neurologist, Dr. Chehrenama, suggested I see Dr. Franck in Tampa. So back in November I called and scheduled an appointment for a consultation with him for this Friday (12/18). He wanted an upright MRI (gosh those cursed things), and happened to have a facility he works with right there in Tampa..who didn't call to cancel an hour from my appointment.

Ariel, my adopted manatee.

My mom and I decided we would make it a three day adventure and go see all sorts of animal things. We got to drive to Homosassa Springs promptly after landing in Florida to visit my old adopted manatee. It was amazing. We got there right before the last boat left and basically had the park to ourselves. It was a great way to de-stress after flying and it completely took my mind off of my impending doom.

The next morning I went to the MRI facility to have my "Franck protocol" upright MRI. Let me tell you..an hour and a half MRI, sitting upright, with your head strapped to the wall, with sides squeezing you...it is not fun. Then, they force your head and neck into awkward positions and you can't breath, move or swallow. It was a great way to start an already stressful Friday. Thus, we went and stress ate fries at Ruby Tuesday's before my appointment with Franck.

After lunch we headed to Dr. Franck's office and started the never ending paperwork a spoonie faces. Over 10 pages of questionnaires. They're the same thing every time and they never seem to ask the right questions. Like "what hurts?", why not "what doesn't hurt"? Once finally finishing all the paperwork, Dr. Franck brought my mom and I into the room and spent over two hours going over my entire medical history.

He spent an hour examining me, watching me walk and taking down vitals. Apparently I walk really funny. No one has told me that. At this point he tells my mom that he already knows what's wrong and what to do. After a few minutes he calls us back into his office to go over my MRI and DMX-ray. My initial impression was that I didn't have instability and that he was going to tell me that he couldn't help me. Nope. He gets to the lateral part of my DMX and starts showing me how my C1 on the right side slips 7mm off of C2 (standard instability patients who get fusions are a 4.6mm) and it slips 5mm off of the C2 on the left. I had a momentary rush of relief that wow, he might be able to fix me.


Then he starts looking at the MRI and says that I indeed have a herniated cerebellum...also commonly known as Chiari. My herniation is not caused by development, but by trauma - so he didn't want to label me with Arnold Chiari Syndrome. Yay being a horse back rider with two traumatic brain injuries, multiple concussions and whiplash...oh plus EDS. So here I am sitting here relieved that the doctor I came to for a cervical fusion agrees I need one, but now he's telling me that something I thought had been completely ruled out by my favorite doctor and surgeon ever...was a real thing? I still haven't completely been able to come to terms with it and am kind of in shock. I've heard about chiari a ton in the last year, and I was so relieved when it was taken off the table for me. Just the shock and sadness of being told that, "no you do have it"...was a lot. My tonsils have dropped 3mm and are being pressed on by the C1.

I also have scar tissue build up from the synovial joint damage on the C1, that is commonly referred to as a pseudotumor, that is causing stenosis to my spinal cord and CSF flow. No wonder I'm still not 100% after a stent that should have really fixed mostly everything. The stent has still made a major improvement in my life, but until we get my neck stable and the stenosis fixed it won't be able to work to it's full potential. Apparently the ligaments in my occipital, C1, and C2 are damaged. My alar ligament is extremely stretched out from compensating for the other ligaments that are damaged (yay EDS). He worked his way down my c-spine and noted that I do have the start of degenerative discs. However the biggest problem is the herniated cerebellum and the tendon damage at my C1.

Thus...we get into game plan mode. He says craniectomy and my heart falls. I know too many other zebras who have had these procedures completely butchered. Very few have been successfully decompressed and usually have problems later on. I thought we were past that. I was fine with the stenting, but this?

Then we get into the fusion - and I get over the craniectomy for a minute. It all seems like a fairly simple procedure. It's a three hour surgery, with the craniectomy and fusion, then I'm discharged that night. I do have to stay in Florida for two weeks post op, but I will be on my feet and able to be a tourist - pain permitting.

Once I got past the fact that the two week post op stay in Florida was non-negotiable, I started to think further on to the future. I'll be in a brace for 6 weeks and able to return to school when I feel ready. Which was a major concern for me. I may even be able to return to work sooner, if accommodations can be made.

Of course I asked about riding. Luckily Dr. Franck is a fellow equestrian and understands my "pain". No heavy activity for 6 months and no riding for a year.

A year. I had no other reaction but to turn towards my mom with tears in my eyes. A year of not sitting on a horse. A year my baby horse will be sitting in a field and not being ridden. A year that neither of us will be meeting my "deadline" for us. I had so many plans for my super pony. She'll be 5 this Spring, and 6 by the time I can ride her again. It's just crushing. I've hit so many road bumps in my "equestrian career" with Luna having just as many problems as myself. Now that it's all had a chance to sink in I've become rational and realize without these procedures I won't be able to ride anyways.

I'm excited, scared, overwhelmed, sad and happy. I came down to have my cervical instability confirmed, not for a diagnosis of chiari. My mom keeps reminding me that it's not truly chiari that it's just that my cerebellum has herniated. But really, truly, it's chiari. Any other doctor would call it chiari. It even shares the same insurance code as chiari. I'm hopeful that the decompression will "fix" it, if not for a little while.

I'm so thankful I have found Dr. Franck and even more thankful that I have answers, even if some of them are unwanted. I will be undergoing these procedures as soon as I can come off of the blood thinners from my stent. The goal is first week of February.

For now, I will enjoy my horses while I can and enjoy spending the holidays with my family.

Billy Bob the Stent.

I left off with finding Dr. Liu at UVA.

I sent Dr. Liu my entire medical history on Tuesday and his staff booked an appointment for that Friday. They scheduled me for an MRI there at UVA before I saw Dr. Liu. I had plenty of emergency room MRI's and CT's, but none of them compared to the images that they were able to get at UVA. 

After getting my MRI, we went to Dr. Liu's office where were were immediately greeted by Dr. Liu's amazing assistant Travis. Dr. Liu had been called into surgery and was running late, so his nurse took my vitals and did my physical exam. 

I have to say, UVA is one of the most organized medical centers I've been to. I literally had to do nothing but show up for my appointments and everything was taken care of. My MRI was already uploaded onto the computer in the exam room I was assigned to. They even let me play with the images while I was waiting for Dr. Liu (I was majoring in biology, I love looking at scans - I was highly entertained for a long while). 

Once Dr. Liu came in the room, he had already looked at my scans and went straight to the point. He didn't think I had Arnold Chiari Malformation and that he didn't think he could fix my problems. He asked me again, in person, if I could pick one thing for him to fix what would it be? Well..at this point I was so cognitively back logged, that all I wanted to do was think clearly and not feel like my brain was going to explode. 

He then told me that he saw some narrowing of the veins in my head and that he would like me to come back for an angiogram and an ICP bolt monitor. I was expecting that it'd be another month before he could do anything for me, and he simply asked if I could just come back next week. 

So four days later, we made the trek back to Charlottesville to find out the 'unknown'. Dr. Liu is very conservative and did not want to give me any false hopes or expectations, which I appreciate. Tuesday morning I was in pre-op waiting for my angiogram. Dr. Liu really has a fantastic set of nurses and residents that made me feel extremely comfortable. I had never had anything this serious done before. I've had oral surgeries and such, but no "surgery" surgeries. His team made me feel like we were just going to hang out and not shove a probe up my femoral artery into my brain. Dr. Liu came in to warn me that he might not find anything at all, but he was going to look despite his doubts. His anesthesia nurse, Anita, was fabulous. She gave me a pre-party cocktail and I was good to go.

I was warned that angiograms are extremely uncomfortable and that I would feel them digging around. Nope. I was asleep. The entire time. All I remember was them asking if I was okay and telling me that they were almost done. I wake up in post-op with Dr. Liu there to tell me that he indeed did find something. My transverse sinus was narrowed causing the CSF to not be able to flow properly. He asked me if I wanted to do the ICP bolt test, that since he found something he didn't need to do it. Me, being the nerdy bio lover, opted for the ICP test so that he and his team could get more numbers on us weird EDS-y zebras.

Around noon on Tuesday, I was brought up to the OR to have the bolt monitor placed. At this point, we had already decided that I would get my transverse sinus stented. The plan was to do it that evening if they could get an OR booked. Yet again, I had an amazing anesthesia team who had me nice and schnockered before I even went into the OR room. I woke up in recovery and had no weird side effects of anesthesia (that's a first). 

I was brought back into my room so that I could rest before my impending stent placement. At this point, I hadn't eaten any food for over 18 hours and was STARVING. By 6:30 the nurses had all started to come in for shift change and I still had not heard about an OR or my procedure. Dr. Liu himself came up around 8:30pm to check on me and talk to my mom and I about the stent. Since it was a last minute procedure and he had already pulled the OR card for my unicorn horn, he was unable to get one that night for the stent. I had already volunteered to do the stent under sedatives instead of general, but they had no staff left for the procedure.

Dr. Liu warned me that if we did the stent, that it would be months before anyone could touch my neck. He gave me a get out of stent card to give me a chance to have my neck fixed first. I decided to go for the stent. I had been told no by every doctor I'd seen about a cervical fusion, and I was struggling at school and work to just function like a human. The stent was within grabbing the distance, the fusion was still far far away. I had already made up my mind and I wasn't going to be talked out of it.

So the game plan changed. The stent would be placed either in an OR or under sedation on Wednesday. My one and only question was: can I eat? Dr. Liu said I could eat whatever I wanted until midnight. I took that very seriously and sent my mom out on a massive conquest to get me as much carbs and cheese as possible. I don't think I've ever eaten that much, so fast. Thank goodness for Zofran.  

I managed to get very little sleep that night as they had to check the ICP every hour. Which consisted of me laying down, sitting up then standing. I finally got a few hours of sleep in the early hours of the morning during shift change. I was told at 8:30am that they would be coming to get me soon to go down for my stent placement.

Once there, my good friend Anita prepared me some early morning cocktails and had me nice and snoozy. The only thing I could feel was a piece of popcorn going off behind my ear...the stent was in. I was feeling pretty proud of myself, only having remembered being out for the entire procedure.

I woke up in the recovery room and was feeling kind of lousy. They sent me back up to my room where I slept for three hours. After an angiogram, you're required to lay still and flat for at least three hours. So when I woke up and saw the time I was very excited that I would finally be allowed to stand up and go to the bathroom. 

Then the head nurse came in to take out my unicorn horn. Holy cow did that hurt. Having a bolt screw unscrewed from your skull while you're wide awake. Then she stitched me up and sent in the other nurses to help me get out of bed. 

Well, with my luck we go to get me out of my bed only to find that I've been laying in a pool of blood because I blew a clot from the angio site. The nurses all jumped on it and started cleaning and putting pressure on the incision site. They had to hold pressure on the wound for almost an hour. The resident told me I would need to lay flat for yet another 6 hours before being allowed to move, in case I blew the clot again. That put me at 11:30pm when I could have freedom. The incision stopped bleeding much earlier than that, but with my EDS and being on blood thinners they had to be extremely cautious. Once I was able to sit up I was allowed to eat, so then I slept through the night.

The next morning Dr. Liu came in bright and early to tell me that they would have sent me home last night had anyone told him about my little bleeding episode. I guess bleeding out is something you can manage at home. Everything looked good and my numbers were coming out normal. He told me that they were going to discharge me and let me go home.  

I was prepped for discharge and the nurses made a makeshift stitches shield so I could shower before the long drive home. Let me tell you..that was one of the most uncomfortable most miserable showers yet. I was determined to be clean and de-hospitalized. Once dressed and packed, I was debriefed on my new med changes and procedures for the stent. 

My mom and I decided my new "addition" needed a name...henceforth Billy Bob the Stent.

We headed home and about 5 minutes into the drive I realized it was not fun to be out of the hospital at all. How could I have forgotten to ask for something for nausea for our three hour drive home?

Me being me, decided to sleep. Sleep is my way of hiding. My way of treating pain. It was a miserable drive back, even with dosing in and out. We somehow made it home and I walked myself from the car to the bedroom (probably a bad idea, but I'm me and I do stuff myself). 

I think I slept for 8 hours straight. Me, also being me, decided that I didn't need help getting to the bathroom or getting food. Also, not a good idea. By day two,  I somehow convinced them to let me go to an appointment with my ankle surgeon. My mom insisted I use a wheelchair, as I was getting injections and just had brain surgery. That was probably way too much, way too soon which sent me into a sleeping beauty slumber as soon as we got home. 

By day three, which was halloween, I was already going upstairs to bake cupcakes and ransack the fridge. I do not do this whole sitting still thing. My dad already commented on how much better I looked and sounded. I hadn't really noticed yet because I was still sleeping off all the drugs, but I guess mentally I did feel better. That Sunday I had a major migraine that had me in bed for a day. It was definitely on the top three of worst migraines. I was prepared to do anything to stop another one.

So when I started feeling migraine symptoms on Wednesday, I decided that I already had a metal implant, what was one more? I went to Exposed Temptations and got a daith piercing, which supposedly can help with migraines. I don't know if it was all the adrenaline from a new piercing, or the fact that it actually worked - but it kept that headache from becoming a full blown migraine.

Within two weeks post op, I had returned to work and school. I was feeling mentally better, just not quite there physically. Given the fact that my neck was still unstable and my body had been basically on bed rest for two weeks, I got why I hurt all over.

Already, Billy Bob had stopped the neuropathy in my arms and improved my blood pooling. We don't know if he has really helped, or it's the awesome combination of blood thinners. I was still somewhat symptomatic and having pre-syncope episodes regularly. It came down to another Dr. Abdullah appointment who said that it very well may be the blood thinners. He was very concerned with my continuing symptoms related to my neck and suggested I go to the Chiari Institute in NY. He agreed with Dr. Chehrenama that my neck is unstable and I might possibly have some kind of chiari.

So far I am cognitively feeling much better, the pressure headaches are significantly decreased and my neuropathy has been gone. It's month two post op, so I'm very happy with my decision to get the stent. I'm so lucky to have found Dr. Liu and his team and to finally have found a doctor willing to go out on a ledge for me. The saga continues, but I finally have a piece to my puzzle.

Billy Bob, the stent.

How it all started.

I was diagnosed with dysautonomia in fall of 2010 by Dr. Fischer at the Mayo Clinic in Minnesota. I was 17 at the time. I had been struggling all through high school with symptoms that were leaving me homebound and unable to be a "normal" teenager. At 15 doctors diagnosed me with Fibromyalgia because they could not explain or manage my symptoms. I had a few good months here and there, but always struggled with pain and other unexplained symptoms.

At the Mayo Clinic in Minnesota, post dx.

After seeing Dr. Fischer he changed my diet, treated my anemia and suggested some lifestyle changes. The dietary changes put a bandaid on my symptoms for about a year. I was able to be somewhat productive that year by getting a job and I got to ride my horses.

In the fall of 2012, while slowly going back down hill I started seeing Dr. Abdullah at the Children's Heart Institute. He was the first doctor to prescribe me medications to manage my symptoms. He also confirmed my diagnosis of dysautonomia, but also diagnosed me officially with atypical POTS. Dr. Abdullah was able to get my symptoms to a manageable level and keep me in school.

Just a usual monthly appointment at the
Children's Heart Institute.

I continue to see Dr. Abdullah on a 6-8 week basis for maintenance and to track my condition. Up until 2014 he was able to keep my symptoms at a level where I could ride and manage my pain with OTC medications. I continue to do PT, massage and chiro as well.

In 2014 my symptoms started declining in the fall, so medications were adjusted and I started seeing a pain management doctor that winter. I resumed the trigger point injections every two weeks, like I was doing in high school on a weekly basis. They started managing my pain with opioid medication, as well.

The first trail ride out with the baby horse.

The spring of 2015 was a good one, no injuries and I was feeling good with that current med cocktail. It wasn't until the good ole Virginia summer heat, that I started experiencing more and more symptoms. Dr. Abdullah finally gave me the diagnosis of Ehlers Danlos Syndrome, something we had expected all along due to numerous tendon/ligament injuries and other text book symptoms. New symptoms hit me like a wave; a very fast, very strong, brutal wave.

For the first time in my life, I wasn't just cold sensitive but I was also extremely sensitive to the heat. If I did go outside, it was in cooling clothing with a cooling vest. Heat would trigger migraines that lasted for days. My ears would ring and sometimes I even lost hearing in an ear. Something that used to help my pain, now made it 10x worse.

My EDS-y body was popping and hurting more each day. Things that didn't pop before, had started to pop and overextend. I started having neuropathy in August, that went from tingling fingers to my entire arms to sometimes not even being able to feel or have awareness of entire limbs. Bending over caused me to lose vision, get tachycardic and have full body waves of nerve pain. Moving my neck while driving caused excruciating muscle pain and sometimes shooting pain through my arms or legs. I was still in school and making really good grades for once, so I pushed through.

From being outside for less than 20 minutes, with limited
physical activity.

By fall, Dr. Abdullah had doubled my dose of midodrine for me to just stay "stable". The syncope was getting more frequent if Remi didn't catch it or I wasn't being cautious enough. I started experiencing more bradycardia than tachycardia, and severe pressure headaches. I was feeling mentally exhausted with severe brain fog - to the point forming sentences was hard. On September 30th, I went to the ER due to one of the worst migraines I've ever had. I was passing out from the pain and barely coherent. They did a typical work up and head CT - showing absolutely nothing, as usual. They sent me home on IV meds and no new answers.

A day in the life of a spoonie service dog.

By that evening the neck pain and headache returned. That week, I went to see my pain specialist in hopes that he may be able to get my neck pain under control in order to not trigger anymore headaches. Well..that didn't work. I had my usual trigger point injections that lasted about two hours before sending me into a deep spiral of excruciating pain.

On October 1st - I started seeing Dr. Chehrenama, who after reviewing my very long, very detailed history suggested that I may have chiari zero. I was kind of shocked that it wasn't just an unstable vertebrae pushing on a nerve, that there could actually be something wrong with my brain and spinal cord. She agreed with the neck instability and ordered an upright MRI to confirm.

Waiting to see Dr. C.

I waited weeks for an appointment at the nearest upright MRI facility, in Maryland. The morning of my appointment, an hour into my drive, they cancelled. I was absolutely devastated. I was counting on this MRI to give me answers so that I could FINALLY get some kind of treatment. My symptoms were worsening to the point of missing classes and barely able to go to work. I was beginning to think I was going to go back to where I was five years ago.

I frantically started looking at MRI facilities that offered this scan out of state. While I was frantically looking for someone or something to help me find the answers, my fairy godmother suggested I look into seeing Dr. Liu at University of Virginia. Within twelve hours his office had emailed me back to schedule an appointment that week. I was shocked, excited, relieved and overwhelmed that I might finally be seeing someone who could do something to help me. This is where I leave you.