My Experience with Chiari
Chiari malformation is a condition where the brain tissue extends into the spinal canal which can block cerebral spinal fluid (CSF) from passing easily through your brain and spinal cord. This blockage or slow flow of CSF can cause an array of symptoms from debilitating headaches, sensory sensitivities, and even muscle weakness and trouble swallowing depending on the severity. It can be chronic and even become more progressive over time but fortunately, for many it can be treatable. It can often be misdiagnosed as migraines or other neurological disorders. There’s still so much we are learning about Chiari but I wanted to tell my story in case it helped others out there. It can be difficult to connect with others who have this condition due to privacy laws.
I started having headaches when I was in elementary school (about 8/9 years old). I remember being in the nurse’s room, sitting out of gym or going home for headaches. I had an MRI and they said that my cerebral tonsils were enlarged with a one-centimeter herniation, but they decided not to intervene because my head was still growing and because the headaches were off and on they decided to monitor it. Headaches continued into my twenties and I was always sensitive to light, closing blinds in my room and preferred living in a cave.
Flash-forward to April 2012, when I was 27 years old - one day the pressure headaches just started out of the blue and continued for 14 weeks straight. The pain was constant, debilitating and the pressure was so extreme that it felt like my head was in a vise.
My primary care physician at first thought it might be migraines, which is a common misdiagnosis. My close friend and then roommate, Katie, was a neurosurgical intensive care nurse at Georgetown University Hospital. I told her about my headaches lasting more than a week. She took a look at my scans from when I was 9 years old and said that it looks like 'Chiari Malformation'. Of course, I immediately Googled what Chiari was because I had never heard of it before. She sent me information on top doctors at Georgetown that she knew had experience with Chiari. I got an MRI and made an appointment with one of the doctors.
May 2012: On Monday I had an MRI, Tuesday I saw Dr. Mason. He immediately confirmed that it was Chiari I with an 8 mm herniation. Chiari I is the most common form, there are three types. Dr. Mason recommended getting a CINE MRI which would help determine if I would be a good candidate for surgery and if it would be effective. The CINE MRI takes images from your cervical spine (upper neck area) and also is able to measure if cerebral spinal fluid is actually being blocked. It took me five visits to different locations and multiple confused attempts from radiologists before I found my CINE-Genius in Clinton, MD. He knew exactly what to do and I will be forever grateful for his expertise.
On Friday that week, I woke up with a shooting pain on the left side of my neck and all down my left arm and shoulder. I could barely move, and it didn't improve so I went to the emergency room at Georgetown Hospital. I drove myself there pushing through the pain and unable to fully move my neck—not the smartest decision I’ve ever made.
The ER doctors said that my occipital nerve was being pinched which was causing the acute pain. I sat in the hospital bed and felt so deflated. Everything was happening so fast and it was a really low point for me. I had been suffering with terrible headaches every day for a few weeks now, I just found out a few days prior that I had a congenital brain disorder and might need "brain" surgery and then had this occipital nerve pain due to the Chiari. I was only 27 years old and felt like my body was failing me. The ER talked to my neurosurgeon and prescribed some pain medication and steroids and sent me home. The pain from the occipital nerve went away soon after.
The following week I met with Dr. Mason and he confirmed that CSF was severely blocked—I believe that he said only 4% was getting through—and said that I would be a good candidate for surgery. He recommended a suboccipital craniectomy and duraplasty for surgery. The suboccipital crainectomy involves removing part of the skull and spinal cord and the duraplasty involves expanding the outer covering of the brain and closing it with another soft tissue. These two steps would help create more space for my long cerebral tonsils and would allow the CSF to flow more easily thus releasing pressure that was causing the headaches. The other option that I had was to not get the surgery and try to live with the headaches with pain medications. He said I had an 85% chance of the surgery being successful. I decided to take my chances with the surgery because the pain from the headaches was so intense, I couldn't imagine living with them for the rest of my life.
A few weeks later, I experienced some muscle twitching in my hands that lasted a few days - another symptom of the Chiari. I also experienced some blurred vision, which made it difficult to work. Yes, I continued working through all of this. They were incredibly supportive during this time. Especially because the strong pain medications (valium, oxycodone) were not really conducive to a productive workday and made me feel drowsy. This was also back when working from home wasn’t typical, so it made for driving to and from work while on pain medications impossible.
I finally managed to get an appointment with Dr. Kalhorn at Georgetown University Hospital. I wanted to get a second opinion on surgery recommendations and liked that he primarily worked out of Georgetown Hospital and would be close in case anything happened post-surgery. I scheduled the surgery for July 20th, 2012.
I went in for surgery on the morning of July 20th, my loved ones waiting not-so-patiently in the waiting room. They wheeled me into the surgical room, and Dr. Kalhorn asked me to think about a place to vacation, “Bora Bora” I said, they joked that they all wanted to come too—and then everything went dark. The dura was constructed out of the lining of a cow’s heart. It was an interesting concept for me because I hadn’t eaten beef for seventeen years, so now the cows were here to save me.
After 3-4 hours or so I was out of surgery and in the neuro-ICU. When I got out, my friend Katie was waiting there for me and held my hand so that I didn’t feel so alone.
“Katie, is it really you?” I said. “Yes.” Katie said smiling down at me. She later told me how I kept asking for Rob, my now husband but my boyfriend at the time.
The first night after the surgery was the worst. I was nauseous from the anesthesia and got sick from the potassium that I had to drink because your potassium levels go down from the anesthesia. I did not stay ahead of my pain and trying to be tough, so I was waiting until I absolutely needed the pain meds, and it was much more difficult to come down from a nine pain level. I slept a lot and the nurses there were amazing.
They had to wheel me down to take a post-surgery MRI to ensure that everything looked good. I remember passing this woman in the hall who was there visiting in the Neuro ICU. She had a short buzz cut and was dressed like a monk, with a long flowing orange gown. There were a few women with her down the hall. As I was wheeled to the elevator, the woman came up to me and held my hand and said some encouraging words. I felt such a calmness with her. She smiled lovingly at me and I attempted a smile back.
I was able to get up with some help from a nurse the first night to go to the bathroom. I was surprised at how quickly I was able to recover. They said that I could go home after just one night in the neuro-ICU but if I wanted to, I could stay a second night. I was mostly concerned about any bleeding from the duraplasty, so I stayed. The second day I was able to get out of bed by myself and walk to the bathroom and sit in a chair in the corner of the room. I was grateful to be able to keep most of my hair, they shaved the bottom for the operation but kept the top. A hairstyle that has become popular in modern culture today.
My recovery was pretty tough. It took me a few weeks before I felt like more of myself again. Buckets of ice cream helped. I was pretty much a walking zombie for the first few weeks. I also had grand intentions of reading lots of books during all of this time away from work but with all of the meds and drowsiness I would barely get through a few pages. I found a few television series that I savored and binged. The Good Wife was one.
I thought that I would be able to keep track of my own medication, but one day when I was home alone, I accidentally took an extra muscle relaxer when I was supposed to be taking a pain medication. Katie came home and found me sitting in a chair, drooling on myself and completely out of it. After that, I put Rob in charge. He would wake me up in the middle of the night when it was time to take my medicine and help track it throughout the day.
I was out of work for eight weeks. It was difficult coming back mostly because I wasn't used to sitting in a desk chair for eight hours. My work organized for an ergonomic company to come in and help me with my workstation and chair set-up so that I would be more comfortable. I also had regular physical therapy which helped a lot because I basically had no rotation in my neck after the surgery because they have to cut through all of your neck muscles.
I am currently nine years post-op and my headaches are all gone. I have also found that the sensory sensitivities I had to light have gone away. I enjoy sunny days where I can open the blinds and let the light in. I haven’t experienced any trembling in my hands or nerve pain.
There are a few things that I was unaware of and learned after my surgery. I was sad to learn that because of my surgery, I am unable to donate blood. This is due to the cow dura, Red Cross is concerned about mad cow disease and so after giving blood for ten years, I’m not able to anymore. It made me sad to hear because I have blood type O+ which is a universal donor, meaning my blood can be donated to any of the other blood types. I was sad to lose the ability to help in that small way.
Going to the hair salon when they put your neck in the washing station still makes me incredibly uncomfortable. I ask to have two towels down to cushion behind my neck. If I am sitting there too long, I become nauseous. The same is true for scarves or shirts that are wrapped too tightly around my neck can be irritating.
My hair has grown back but I still have a scar in the back of my neck that looks like a zipper. A reminder of all that I went through. I joke that my brain was just too big for my head and that I very rarely 'moo'. I will be forever indebted to cows. My surgery was a success, and I am grateful for the doctors and nurses that took such good care of me. And for my brilliant friend, Katie, who was the first to diagnose me. I was fortunate that my case was not one of the more serious cases and that I was able to get surgery. I hope that anyone out there dealing with the same issue finds hope in my story and also relief from their pain.
I've included an MRI image and post-op pictures below. I should note that I have a birth mark in the same location as my surgery which is why that area continues to be red throughout my recovery: