(Note from Chip- I'm happy to say that Joanna manages the "Chiari Kids" support group for WACMA.
If you are a "Chiari Kid" or have one, We would love to have you participate in the support group. )
I have a great story. I�m one of the few who can say my finger has felt the inside of my head, but I�ll get to that later. All of my life I have had Chiari symptoms; I always had headaches, neck pain, shock like pains, remittent blurry vision, and a myriad of other symptoms. I have never known anything different. In fact, I thought everything I was feeling was normal; I was under the impression that everyone�s head hurt all of the time.
In the beginning of 1998, at the age of 12, my symptoms became more intense. In January of that year my back spasms started. To me, the back spasms were the most troubling of all my symptoms. The headaches were the most chronic, but the back spasms were horrendous. They would start in a specific spot in my thoracic spine, which--looking back now--corresponds approximately with where my syrinx ended. Anyhow, the spasms would start around the T5-6 area and it would feel as if someone had stabbed a knife right in that spot. Then a tingling feeling would overtake my entire back, neck and head. It felt as if the wind had been knocked out of me. The pain was so intense that moving and talking were out of the question. When I first started experiencing the spasms they would only occur a few times per week.
By 1999 I had just about lost all of my excess energy. My symptoms had become a great deal more intense and more of them had developed. I slept all the time. They thought that maybe I was depressed or something to that effect because all I did was sleep, but I pushed on and went to school, although my work did suffer. Perhaps we should have seen this as a clue that something was really wrong, but it just didn�t register. I didn�t really talk about it too much. Like I said earlier, I didn�t think there was anything wrong with me, I thought I was normal. Over the next two years my symptoms proved themselves to be very aggressive. My headaches were awful; there was just a constant, unrelenting pain. I describe the feeling of my headache to people by asking them to imagine an ice cream headache, and then to imagine that it never went away--ever. I also had a constant pain at the base of my skull and in my neck and shooting and burning pains that went into my spine and extremities. My back spasms were happening several times daily now. I started to notice numbness and tingling all through my extremities, especially on my right side. There were other weird symptoms, my eyes always seemed to hurt, and sometimes, I would be trying to move my eyes and they just wouldn�t budge, or they wouldn�t move in the direction that I wanted them to. By March 2001, I was miserable. I was missing a lot of school and had absolutely no energy. I started flunking tests, which was not like me at all. I was moody all of the time and had pretty much severed all of my contacts in my social life. I really didn�t want to go out or have fun, and I just wanted to sleep away the pain. I had a MRI of my thoracic spine in April 2001.
Back in 1998, I had a trampoline accident where I tore tissue in my back--or so they said. Between 1998 and 2001 the trampoline accident is what we attributed my back problems to, and why my parents did not do as much investigation at first. But what didn't make sense was the different type of symptoms I had and why they were progressing.
I wasn�t expecting them to find anything wrong on the MRI, I�m not sure why, I guess I still thought that I was �normal.� About a week later, my curiosity got the best of me and I asked my mom if she knew the results from the MRI yet. She casually told me that there had been a problem found with my CSF (Cerebral Spinal Fluid) and my spinal cord, and that she didn�t know too much about it. What she was referring to was my syrinx, or Syringomyelia. At this point in my life I knew very little about the nervous system besides what I had learned in my science classes, so this meant nothing to me. She was trying to underplay the situation, and she did a good job. I barely gave it another thought.
Later, I was to find out that the MRI showed not only a gigantic syrinx, but also a Chiari 1 Malformation with a herniation measuring approximately 17 mm. I remember the night my mom told me as if it was yesterday. She said, �Honey, they found something else on your MRI�� And then she paused for what seemed like a million years--time literally stopped. �You�re going to have to have brain surgery.� The words echoed throughout my entire being: my mind, my body, and my heart� And then I burst into tears. Brain surgery wasn�t the kind of thing that happened to you, that was the kind of thing you heard about on the news, or someone saying �My friend�s cousin�s, ex-girlfriend�s, stepbrother had brain surgery.� It didn�t happen to someone you knew, let alone to yourself, but it was happening to someone I knew: it was happening to me.
The following day, my dad and I went for our first Neurosurgery consultation. We knew, going in there, that we would hear that surgery was a necessity for me because of my syrinx. The neurosurgeon said just that: �She needs surgery, and the sooner the better.� He examined and performed neurological tests on me, which revealed more neurological problems and anomalies than I had realized on my own. We liked him well enough, and he said he had performed about 50 of these procedures, but we only saw him one more time after that. My mom spent a lot of time on the phone with various neurosurgeons all over the country. They all invited us to go to their hospitals for my surgery, but I really wanted to stay in Atlanta. We asked them for recommendations locally, and they all recommended the same doctor.
I think we all knew within 5 minutes of meeting him that he was the guy for us. Being the chief of Pediatric Neurosurgery at Emory University, he definitely knew what he was talking about, but more than that, he cared. Looking back, I don�t think I could have, or do for that matter, trust a doctor more than I do him. We were very lucky in finding him. His recommendation was the same. However, I did need surgery, and soon.
In June, my family and I went to the Northwest for 3 weeks, on a much-needed vacation, and had a wonderful time. After we got home, I was a counselor for 3 weeks at a performing arts camp that I�ve been attending for years, and it was a blast. It was only after I got home, in those two weeks before my surgery that I really started to learn about Chiari and Syringomyelia.
On July 27th, 2001, I had a craniectomy, laminectomy, duraplasty, and adhesions/scar tissue removed from my brainstem and cerebellum. I remember looking at my parents while being rolled into the operating room after hugging and kissing them goodbye, and thinking, �What if I never get to say I love you to them again?� Thankfully, the medications started to work, and I became very relaxed and woozy, and was unable to sorrowfully ponder that idea for very long.
The next memory I have is from the Intensive Care Unit (ICU) and it isn�t much. I remember voices, lights, and pain. I remember hearing murmurs of my parents and the resident�s voices discussing more morphine, and then saying it was dangerous because my blood pressure was too low. The first few days are a blur. The only real problems I had were pain management, and my vision-- for the first several days, everything was distorted. I had my first allergic reaction to a medication on my third day post-op. The Toradol gave me a bad rash and my skin was burning and swollen like there was no tomorrow. A few days later, I was released from the hospital.
I went home to begin what, I didn�t realize then, would be a very rough recovery. I made my first trip to the ER on my 12th day post-op. I had broken out in huge red hives all over my body, and I was very swollen. It turned out to be the second of many allergic reactions. This one was to Percocet. I was placed on three new medications, including a steroid to help with the reaction. I had x-rays, and had my stitches removed, and was then released again. The next few days I was feeling better, I even went back to school part time, which would later prove to be a mistake.
At 4 weeks post-op, I was lying in bed watching TV and resting, when all of a sudden my head started feel a little wet. I figured I was imagining it and tried to ignore it, when it didn�t get any better, I got up to look and satisfy my curiosity. Well I sure wouldn�t have been able to guess what I was about to see: On my pillow there was blood, and plenty of it. I realized that the bleeding hadn�t ceased and was continually running down my neck. Unfortunately, I was home alone. Realizing that I had to stop the bleeding, I retrieved a washcloth and was planning on putting pressure on the incision where it had opened. I started to run my finger along the incision to find the opening, but I didn�t get too far. My finger actually slipped inside the wound, and came out all bloody. Yes, you have probably guessed this is the part about the finger in the head. Kind of gross, I know, but still, it�s unique!
I went to the ER again, and was placed on an antibiotic as a precaution, and they taught my parents how to bandage and cleanse my wound several times daily. It turns out that my incision burst due to an allergic reaction to the internal sutures. I spent the next couple of days with a low-grade fever, and vomiting. It was in fact, another allergic reaction to the antibiotic. I was taken off the antibiotic because the chance of infection was so low, and because the reaction to the medication was making me absolutely miserable. I assure you that repeatedly throwing up doesn�t feel good 4 weeks after brain and spine surgery. We continued to bandage and cleanse the incision about 5 times a day, and I was taken out of school for two weeks so that I could rest and allow the incision to heal.
A couple of days later, my spine and head started to ache horribly. I had an awful headache, and it hurt to move. In addition to the pain, my incision had started to ooze a yellow and green substance--it�s gross, I know. We called my neurosurgeon, and he said that if I was in that much pain we needed to get to the ER. We arrived, and by this time everyone in the ER was starting to remember me. They took a culture from the wound; I had a spinal tap, and was given a CAT scan. The culture revealed a high white blood cell count, which indicated an infection, and the CAT scan showed little pockets of air inside my head from the incision opening up. My neurosurgeon admitted me and decided I would go back into the OR the following day. At 6 weeks and 1 day post-op (9/8/01), I was rolled into the OR once again. What was found was a Staphylococcus (Staph) infection. The infection was cleaned out thoroughly, and I had a PICC line inserted. A PICC line is a special I.V. that they put into your arm that goes directly towards your heart. It is inserted for long-term therapy, and/or I.V. treatment at home. I was put on two very strong I.V. antibiotics. After this surgery, I felt like a different person. I couldn�t believe the turn around. My neurosurgeon was blown away and how much better I was. I was released 3 days later on September 10th. I finally felt like things were going in the right direction and that I was on my way to recovery. I didn�t know then, but I couldn�t have been farther from the truth.
We had been told to take my temperature a couple of times a day, just in case. The following Saturday night, September 15th, my parents were out for the evening, and I was just about to climb in bed. I took my temperature just like I had been, it was about 99--which is a little high for me, I�m usually at 97. I just figured I was overheated, so I went to sleep and didn�t worry about it. The next morning I woke up not feeling so well. We took my temperature, and it was up to 102, so off to the ER we went.
We got there, and they couldn�t find anything wrong with me, except that my blood pressure was dangerously low for some reason. They gave my plenty of fluids via I.V., and remedied the situation very quickly. I was put on major fever reducers, and was sent home with the diagnoses of a virus. My fever actually broke for a while, but when it came back, it came back with a vengeance. That night I was lying in bed shivering and shaking because I couldn�t get warm. I couldn�t sleep; my body just hurt. It was around 11:30 pm, and I decided to take my temperature just to see what it was, it was up to 104. I went up to my parent�s room, and we rushed off to the ER. By the time we got there, it was up to 105. I was, of course, admitted to the neuro floor this time.
I spent the next couple of days in and out of deliria. I had general pediatrics, neurosurgeons, neurologists, virologists, hematologists, and infectious disease doctors�no one could figure out what was wrong with me. The second day I was admitted, something very scary started to happen. Besides the 105 fever and swollen lymph nodes, my blood cell counts got lower and lower everyday, it was almost as if my bone marrow was shutting down. I was put in isolation due to the danger of having a low blood count. I was being tested for every virus, infection, and condition available. But nothing could be found. Finally, someone decided it would be a good idea to take me off the I.V. antibiotics to see what would happen: My fever started going down, and my counts started going up. It turned out that the staph infection had spread to my lymph nodes, and that�s why they were so swollen and tender. I was released several days after my fever, and counts started to return to normal. So it was yet another problem to be caused by allergies. I was put on an oral antibiotic to treat the staph in the nodes, which to no surprise landed me in the ER several days later; thankfully the staph had been knocked out.
A few weeks later I tried to go back to school, but it didn�t last for long. I started developing horrendous headaches again. The first was at school--I started seeing bright lights, and then everything went black. I could hear my teacher calling my name, but I couldn�t respond, and all I could do was sit there. I was taken to the ER once again, and was diagnosed with post-operative complex migraines. It was thought that the migraines were due to the complications I had along with probable extra swelling from the infection. Soon after this I began developing blackouts.
The first blackout was so frightening. It felt like I was in a tunnel that never ended. My vision felt like I was looking through a TV with static, my ears ringing, my body going numb, everything feeling very heavy, and then I woke up on the floor, but I was unable to move because my body felt like it weighed 5000 lbs. Confusion took me over. I had no idea what had just happened to me, my head was hurting; I had hit it on the way down. It was very upsetting, and I remember just lying there and crying, unable to move. I was pulled out of school and was put on the homebound program, which is a way for kids who are too sick to stay up with their schooling; a teacher comes to your house to help you. I was given a 48 hour EEG that proved that they weren�t seizures. The next thing I knew, I had a cardiologist diagnose me with Neurocardiogenic syncope. We think that my syncope is due to the infection and high fever that I had, and that somehow, due to those factors, I have some sort of brainstem dysfunction. Soon after this I developed a very odd type of blackout, neither my cardiologist nor my neurologist have heard of this before, and we have no idea what it�s from. In December, my migraines slowly disappeared, and in January we started managing my blackouts with medication. I went back to school second semester, and did great.
All four of the post-op MRIs that I have had show that my syrinx is shrinking and that I am adequately decompressed. I am doing wonderfully. I finally was allowed to get my license, and I love driving and the independence that comes with it. I am maintaining a 4.0 GPA, I�m very involved with my school, I go out with my friends all the time, and basically, I have no complaints. What can I say? I love my life! I am so happy, and I feel so much better. I couldn�t have done this without everyone who has helped me, especially my parents, who put everything on hold to get me the best care possible, my wonderful doctors, friends, and of course the very special people at WACMA and ASAP. Now, my next big venture: college and then of course, medical school!
It was a very bumpy ride for a while: Two surgeries, multiple hospital admissions, more than half a dozen trips to the ER, and tons of diagnostic tests. I don�t regret what has happened to me over the last year, it was hard, there were days that I didn�t want to get up, days when all I wanted to do was curl up in a ball, and never uncurl. But I did uncurl, I did it; I�m a survivor.