. In January of 1998 (I was 12) I started experiencing an increase in headaches and many other symptoms. One of the most troubling symptoms was my back spasms. The spasms in my back were horrific. They would start in one spot on my back and it would feel like someone put a knife in that spot, but it got worse. The spasm would then spread all over my back, neck and head, I describe it to people as feeling like the wind had been knocked out of me. During the spasms I couldn't speak or move, the spasms lasted for about ten minutes and in the beginning it would only happen a few times a week.
My symptoms progressed slowly but surely at first for the next three years. More symptoms came on, strange little feelings all over, numbness and eye things. I wasn't a complainer and I rarely mentioned it. By 1999 I pretty much lost most of my energy, but I still pushed on, but everyday I would come home from school and fall asleep on the couch doing my homework, I just couldn't stay awake. I just brushed it off most of the time through the end of the year 2000. As long as I can remember I have had a headache 24/7. The thing is, though, I didn't know it was what people were talking about when they had a headache. I thought everyone's head hurt all the time, which is why I never complained about any of it, I thought everything I was feeling, was in fact normal. So I never talked about it.
That all changed in 2001. The headaches became greatly more intense, the back spasms were a daily occurrence, and a host of other things had come on suddenly. My headaches were horrible, I explain them to people like this: First I ask them to imagine an ice cream headache and then I told them to imagine one that didn't go away, ever. My symptoms were so agonizing that I was missing at least 1 day of school a week, checking in late, or leaving early. Getting through the day became extremely challenging. Apparently, my ability to pretend like nothing was really happening to me diminished greatly. My grades started to drop, and I had absolutely no energy. I decided it was time to stop pretending, so in February '01 I finally told my parents that the symptoms I had were worsening. I had an MRI of my Thoracic spine in April.
In 1998 I had a bad trampoline accident where I tore tissue in my back and in my shoulder and that is what they were attributing my symptoms to. Which is the reason that I didn't often complain and is also the reason that my parents didn't do as much investigating, because there was a reason for my symptoms, but what didn't make sense was why they were progressing. After the MRI I didn't think about it too much, I wasn't really expecting them to find anything wrong. About a week later I started to wonder what the MRI said, so I asked my Mom if they had the results yet. My Mom casually said that they had found something that had to do with my spinal cord and Cerebral Spinal Fluid (CSF), but she didn't know to much about it. Knowing absolutely nothing about the nervous system at that point, I just brushed it off and didn't think about it. The truth was that my Mom was trying to underplay it and make it sound like it didn't need to be looked into or anything like that, and she did a good job. I didn't give it another thought.
In actuality my Mom knew it was a very big deal and was doing the Mom thing of trying to shield me from reality. The truth is my dad and his having a science background (Ph.D. in Biophysics) understood the logistics of Chiari and Syringomyelia a lot more. Also, he has access to a huge library of health archives. As he and my Mom were researching Chiari/ Syringomyelia furiously, I went on about my daily life and tried to manage the best I could. My intuition, however told me that there was something they weren't telling me. I would be sleeping in the afternoon and I would wake up to one of them just sitting on a chair in my room watching my sleep. Little things like that kept hinting at something going on.
A week later I found out there was, they told me about the Chiari and more about the Syringomyelia and the actual seriousness of it. Two days later I had my first neurosurgery consultation. He was nice enough, and spent a good hour with my dad and I. He explained the Chiari and Syrinx in depth and said surgery was a necessity and I needed to get it soon. Also, the only thing stating the Chiari was the report from the initial body scan done by the radiologist, there were no films of the Chiari so he was unable to comment directly on that. The report stated: 'A very obvious Chiari Malformation approximately 17 mm and a large syrinx reaching from C1-T6.' So that was all we had to go on, he did say that my spinal cord was substantially swollen. So he ordered a brain and new cervical and thoracic MRI's to be taken immediately. I saw this Neurosurgeon one more time, but that was it.
My Mom spent a good deal of time on the phone with people from all over the country. We called Washington, Alabama, Florida, North Carolina, to talk to
the various neurosurgeons and their staff. Every single one of them invited us to come to their state and have surgery there, but I really wanted to have my surgery here. We asked each of them for recommendations in our area. Every single office recommended the same person. So that was our next consult. After five minutes of knowing him we knew that he was the one. Not only is he an exceptional surgeon, he is also one of the most compassionate and caring people that I have met. I can't say that I could trust a doctor anymore than I do him. Neither he nor my family and I predicted how complicated my situation would be, but he was great and was willing to help with the extra care that I ended up needing. We were very lucky in finding him. His recommendation was that surgery was a necessity also. And so we scheduled it. July 27th was the date.
We went away for the first 3 weeks of summer for a much needed vacation and enjoyed it immensely. We came back and I was a counselor for three weeks at a camp I have been going to for years. I got back two weeks before my surgery. It was only then when I was home for those two weeks that curiosity got the best of me and I started to learn about Chiari. Little did I realize how complicated it could get.
had my surgery at 8:30 AM on July 27th of 2001. I came through it with flying colors. I was doing very well and was released from the hospital 5 days later. I had my first reaction to a medication on my 3rd day post-op; it was to Toradol. Pain management was a problem and so was my vision (I couldn't make out anything well for a few days following surgery.) I continued to do pretty well with my recovery and was put on Percocet in addition to Ibuprofen with it. At 12 days post-op I mad my first trip to the ER. I had broken out in huge hives all over my body. I was bright red and swollen and not happy. It turned out to be a reaction to the Percocet. I had x-rays, my stitches removed, and was put on 3 new meds including a steroid to help with the reaction.
I went back to school part time at 3 weeks post-op. I was doing pretty well for a week, and then I met another complication. I was lying in bed watching
TV and resting one afternoon when my head started to feel kind of wet. I got up to see what was going on and there was blood all over my pillow and it was still bleeding. I realized my incision had burst open. Needless to say I had my second trip to the ER. My incision had burst due to an allergic reaction to the internal sutures. I was put on a general antibiotic as a precaution and had my incision packed with iodine strips and bandaged. This was a Friday and on Monday I had my 1-month post-op appt with my neurosurgeon.
On Saturday morning I woke up feeling pretty awful. I spent the next two days having a low-grade fever and vomiting. Turned out to be another allergic reaction. My NSG took me off of the antibiotic on Monday, as he didn't want to add to my misery, which I appreciated greatly. The chance of infection was so low that I was left off of antibiotics and had my incision cleaned and re-bandaged about 5 times a day.
Exactly two weeks after my incision opened up (now 6 weeks post-op) it started to ooze; it was pretty gross. I also just started to hurt and ache all over horribly. So we called my Neurosurgeon and he said if I was in that much pain I needed to come to the ER. My NSG and the neurosurgery resident met us there and I had a culture of my incision taken, a spinal tap, and a CT Scan. They weren't sure what exactly was going on, but my incision wasn't looking good. I was admitted at 6 PM and had surgery the next morning at 7 AM (September 8th...6 weeks and 1 day post op from decompression.)
A Staph infection was found; they cleaned it out thoroughly and also checked out my dura. I also had a PICC line inserted and was started on Vancomycin and Rocephin IV antibiotics. I immediately felt so much better after the infection was cleaned out. My doctor couldn't believe how much better I was, I felt like a new person. I was released on Monday night. I had a home health care nurse coming to my house to infuse the IV. I was feeling really great all week, unfortunately this feeling was short lived. I was told to take my temperature several times daily, so we did.
On Saturday evening my parents were out and before bed I took my temperature. I had a 99.5 temp, which is a little high for me (I'm usually 97) I just ignored it and figured I was overheated or something and I went to bed. On Sunday morning I woke up feeling awful, we took my temperature and it was up to 102. Off to the ER we went, this was trip 4 and by now mostly everyone knew me. They took blood from my PICC line and did various other tests. All came back clear. They gave me a couple liters of fluid because my BP was about 70/40; also they put me on fever reducers and sent me home.
I was feeling much better and my fever even broke for a while. I went to bed at about 10 PM, but I couldn't sleep, I was having horrible chills and just didn't feel good at all. Around 11 I decided to take my temp just to see what it was. It was 104. I got up and hurried up two flights of stairs to my parents room, which took me about 10 minutes as I was going very slowly since the last surgery. By the time I made it up there it was 104.5. We rushed to the ER, when we got there about 15 minutes later my temp was 105.5. By this time I was very disoriented and feeling just miserable. This time I was of course admitted.
They didn't know what was wrong with me. In addition to the fever my glands were very swollen and painful. I was put on the neuro floor, although I had Virologists, Neurologists, Neurosurgeons, Hematologists, Infectious Disease, and General Pediatrics. No one could figure out what was wrong with me. While the fever and the lymph nodes were very worrisome something else started to happen that was scary; my bone marrow essentially shut down. Everyday my counts got lower and lower. I was put in isolation in my hospital room and no one could get too close to me, and they had to wear masks. My condition was getting more and more serious. I could see it in my parents eyes when they would come back into my room after the doctors asked to speak to them outside.
I had been tested for viruses, along with a host of other diseases and basically everything I could be tested for came back negative. At this point they were completely stumped. It was decided to take me off of the Vancomycin to see what would happen. Little by little my counts started to grow, and my fever went down. So it turned out to be another very, very, severe allergic reaction to the Vancomycin, and Rocephin. And as for the swollen lymph nodes, the Staph had spread down to them, so I was put on an antibiotic to treat that. That, thankfully, was taken care of very quickly. Soon I was released from the hospital on an oral antibiotic, which "surprise, surprise" landed me in the ER with another allergic reaction.
I went back to school a few weeks later. This didn't last for very long. Soon I developed postoperative complex migraines. After this I began developing blackouts. Having my first blackout was very scary. I remember feeling like I was in a tunnel, my eyes going fuzzy, my ears ringing, my body going numb and then waking up on the floor in pain unable to move because I felt that I weighed 5000 lbs. My doctors weren't sure what they were from. I had a 48 hour EEG which said they weren't seizures, I had a MRA which said it wasn't a vascular problem, the blackouts were very curious. I was pulled out of school and my neurosurgeon wrote orders for the homebound program. I developed several different kinds of blackouts. They were happening at least 1 or more times a day.
Next thing I knew I had a Cardiologist and was diagnosed with Neurocardiogenic Syncope. We think that due to the infection and high fever I had that my brainstem has some sort of problem, and for that reason it is not communicating with my heart properly, so my heart doesn't send enough blood to my brain, so I blackout. When you are recovering from neurosurgery it really isn't good to be hitting your head a lot. So I began spending most of my time in bed out of fear of hurting myself. Soon after that I developed a one of a kind blackout, which is very complicated. My Cardiologist has never, ever heard of this kind of blackout. No one knows what it is from. In December my migraines slowly disappeared. In January we began managing my blackouts with meds. I'm on two different meds for the blackouts and they are helping a great deal.
As of right now I am doing so much better. I get occasional headaches, and I have a few other neurological problems, but it is felt that those are most likely from nerve damage from my syrinx. Every post op MRI shows (4 MRI's) that my syrinx is shrinking and that I am adequately decompressed. Which is such a relief after 2 surgeries, multiple hospital admissions, tons of diagnostic tests, and 8 trips to the ER.
I am back at school full time and am enjoying every minute of it. I am maintaining a 4.0 GPA average. I have gotten my license and a car and am driving well. This past year will probably be the hardest year I will ever have to encounter. I won't lie, it was horrible, but I am actually thankful for everything that has happened to me in the last year, I have grown up so much through all of this and am a much better person. I have now decided what I want to do with my life: I want to be a physician. I know that I am going to be so much better in the field of medicine because of my health problems. I know it's going to be hard, but I refuse to let my limitations rule my life. I enjoy everything I do; I don't take things for granted anymore. I try my best at everything and I know the value of things now. I am so thankful for everyone who has helped me through this journey, but I am particularly grateful to my doctors who have been wonderful and also my parents who put absolutely everything on hold to get me the best care possible. Thank you.
- Joanna
