It seems as though I had symptoms my entire life. As a baby, I couldn’t keep formula down. The doctors attributed it to an allergy and came up with different types of concoctions for me to eat. As I grew, I became extremely klutzy. My sisters use to laugh and tease me because I was great at tripping on air, as they called it. I even fell down when I was standing in line at the grocery store, imagine that. But we continued to just laugh it off and figure I was just a klutz. It wasn’t until I was a teenager that new symptoms started to arise. I had my first black-out spell when I was around 14 or 15. I was sent to a neurologist who performed an EEG and said that they couldn’t find anything wrong with me to explain why I was having these spells. And so we dealt with them the best way we knew how.

     The sever headaches really became an issue as I entered my 20’s. I went to the doctor several times and kept getting the same thing, it’s just a migraine. Often times, the headaches were so bad that I would end up in the hospital, as all the other medications would not even touch the pain. It took them several years before I even got prescribed Imatrix to help take the edge off. Although, I was finding that the medication wasn’t really helping either. During this time, I suffered from Bell’s Palsy, or so they thought. I was out working, driving a school Bus, when I noticed that the right side of my face was suddenly not working. With nothing showing up on the CAT scan, they had no better explanation than Bell’s Palsy. It lasted for a little over a month before I could get any movement back on that side of my face. I suffered with depression, due to the constant headaches that I continued to suffer. It isn’t easy when you have to live in pain every day and nobody knows why it’s happening.

     In my early thirties, I was finally prescribed preventive medication for the “migraines”.  Although, it didn’t keep the headaches from coming, at least someone was trying to do something for me. The trouble I kept running into was when I told my Dr that my headaches always started in the back of my head and neck, they said it was all caused by stress. I took every measure I could to try and live a less stressful life, but we all know how impossible that can be especially if you suffer from daily headaches.  As the headaches progressed, so did my symptoms. I started having tingling sensations in my face, hands and feet. At first the symptoms were ignored, but then they stated that the cause was from my medication.  Apparently Topamax can cause tingling in the face, but this didn’t explain my hands and feet.

     June 2011, really started the ball rolling. My summer route had just ended, but I was on the call list to help out when needed at the bus yard. One morning, I was awaken early because they were short an aide on one of the special needs buses. They wanted to know if I could get to work ASAP. Just as I was hanging up, my friend was at the door. She had come to pick me up because it was her route that needed help. I took a shower, got ready, and together we drove to work. As I was aiding, I noticed I was starting to feel strange. (The rest of the story is what I have been told happened, as I do not remember.) When we got back to the yard, we went upstairs to fill out our time-sheets. There is a half wall that separates some of the desks upstairs and I apparently walked right into the wall as if it wasn’t even there. We all laughed it off, but my friend was really concerned. When we got down stairs, I started to panic. I couldn’t find my car anywhere. My friend kept reminding me that we drove in together and my car was safe at home. It took a few for this to register before I would get into her car so she could take me home. We made plans for me to go over and cook dinner for her and her family, it was just something we did throughout the summer, we were spending a lot of time together. When we got to my house, as I was bending down to get the meat and stuff out of the fridge, I blacked-out. My friend got nervous and though maybe it was because I hadn’t eaten yet that day. So she gave me a granola bar and finished getting everything together. She then asked me to pack an overnight bag because she felt that I needed to have an eye kept on me. When we arrived at her house, I went straight to the kitchen and started preparing dinner. It was fairly easy as I have a roaster, so all that needed to be done was toss everything in and let it cook. After I was done with the preparations, I went and laid on the couch to watch a movie with the kids. It was during this time that I started to feel even worse. I’m told I called my friend over and told her something was wrong and then I got up and went into another room and called my sister. The two of them decided that I should go to the ER and be seen. By the time we got to the hospital, I was walking like I was drunk, my face wasn’t working properly and I was slurring my speech and talking really slow. I had all the classic symptoms of a stroke, but we just thought maybe the Bell’s Palsy was back. After the CAT scan was done and nothing abnormal showed up, they decided I should stay the night and be seen by a neurologist the next day.

     The next day, I was sent in for an MRI and an EEG. Other than that, they just kept me comfortable as my head was back to pounding again. The following morning, very early at that, the neurologist came in to talk to me. They finally had a diagnosis for me, I have Arnold Chiari Malformation I. I was very scared and felt so alone at the time, I had no idea what she was talking about. She did show me a picture of my MRI and I was able to see where my brain had herniated into my spinal canal. But what was worse was that she gave me false information and had me 100% more scared than I already was. I was told that it was serious and that I might need emergency surgery, so she was calling the Neurosurgeon on call at the Neuro-hospital to see what they wanted to do. After it was decided that I didn’t have to have surgery that day, I was sent down for another MRI, this time of the neck. The results came back that I had a small Syrinx on top of the Chiari. They kept me a week while they tried to manage my headache the best they knew how, and then I was released with new medications and a referral to a neurosurgeon.

     It took me over a month before I got a neurosurgeon to see me. It was decided that I needed to have the Decompression Surgery, as I had no fluid passing from my spinal area to my brain. Two weeks later I was in the OR and the surgery was completed, however, because my NS said I decompressed the moment I was opened up, he felt there was no reason to remove and replace the dura. So all that was done was the removal of bone near the base of the skull and the removal of the cervical vertebra C1, as well. My recover in the hospital was not an easy one, but we were hopeful that this did the trick, at least for now.

     One month later, almost to the day, I was sitting out by the fire with my friend (who had now become my roommate, they took my son and me in because I had no income and couldn’t afford my apartment anymore) when I noticed the back of my head was wet. We called the NS on call and was told I needed to get to the ER as soon as I could. It was really rather comical that night. When we got there, they rushed me right back. The nurse looked at my head and was able to find where I was leaking (the top of my incision site). When the Dr came in, she couldn’t see it at all. So the nurse had to come in and show her where it was leaking. They wanted to get a sample, but were unable to do so. The NS came in and he also was unable to locate the place where it was leaking, so once again the nurse had to come in and show them up. Even through the headache, I had to laugh at all this. The nurse was a riot and he kept us giggling the entire night. Once it was confirmed that I had a CSF leak, I was admitted into the ICU until my NS could see me the next day. When he showed up early in the morning, he checked my head and said I had two options: We could open my head back up and see if my dura had a hole in it or we could put in a lumbar shunt to help pull some of the fluid down. He kept saying he was curious about the dura, but the more we talked the more the shunt sounded better. And so I was in surgery later that day. The shunt, however, seemed to have made my headaches 100% worse. They went from being a constant headache to a constant positional headache. The only thing I could do to ease the throbbing was to lay down. All it really did was ease the banging in my head, but not remove the headache. A few days later, they changed the setting of the flow on my shunt, but with little change. I was again given the option of surgery, this time to shut off the shunt. At first I agreed, but than changed my mind, why not give it a chance to work so I don’t end up leaking again. As the time went on, I continued to have headaches and a new symptom of throbbing pain in my side where the shunt went around to drain into the abdomen. I spent the majority of my time in bed because the moment I stood up or sat up, my head would start throbbing and I would get dizzy, almost motion sickness. My unsteady gait problem, persisted as did many of my other symptoms.

     In January 2012, after there was still no change with the headaches, we were back to deciding if I should have the decompression surgery over or turn off the shunt and see if it helps any. I opted for the lesser of the surgeries.  We decided not to remove the shunt as a precaution if decompression surgery was in the future. I was also weened off of some of the medications in hopes that the tingling in my face would disappear. Unfortunately, the only thing that changed was the type of headaches, they were no longer positional and back to being just a constant everyday pain. On top of that, I was suffering with the increase of pain when I coughed, sneezed or strained, which was horrible as I had picked up a cold.

     It is now February 2012, and we have decided to go ahead with the second Decompression Surgery. A few weeks ago we did another MRI and discovered that the CSF flow is once again diminished. So, on March 8th, I will once again go in for surgery, my forth one in a total of six months. This time my dura will be replaced with part of my skin taken from another location, and he will be doing an exploration of the brain to see if there might be a reason that I continue to have these daily headaches. I don’t know if I am more scared this time because I have already gone through the surgery once and know what to expect, or scared because I don’t know how I will react this time. Luckily, I will have the ones I love close by (Mom’s flying in to be here with me)  which helps me feel a little more protected, if that makes sense. Either way, I remain positive and hopeful that this will help me be able to return to a more sustainable life. Chiari at this time, is not curable, but surgery and medications can help ease some of the symptoms or at least the progression of them.

     Chiari doesn’t just affect the person who suffers, but also all those around them (family and friends). I have been blessed with an amazing support system, and I have also gained a lot of friends and support from different online support groups. Zipperheads for Chiari, a support group on facebook, always helps to encourage and support each and every fellow Chiarian and their families as we struggle through this disorder. Also, Chiarisupport.org is another great support group for anyone needing to get a grip on whats really going on. Although Chiari remains on the Rare Disorder list, we are discovering that there are a lot more of us out there than they once thought. 1 in ever 1,000 people are diagnosed with this disorder. It’s really time to make some noise and encourage our medical community to start researching and looking for a cure!

❤ Heather

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