It's not every day that you learn you have a rare brain condition. The day that I heard I did, was a changing day for me - it changed my whole world; my whole life. It changed the course of my life. The course I thought I was on with my life was suddenly altered. This diagnosis I was hearing, shifted everything and threatened to send me into a tailspin, both emotionally and physically.
I was in the prime of my life. I had two very young and wonderful boys and suddenly I was unable to be the kind of mom I had been - the kind of mom I wanted to be, and had planned to be long before I had kids. I was suddenly unable to be - me.
The symptoms were unnerving. They snuck up on me like a cunning fox, slowly and nearly undetectable. I wasn't even sure anything was wrong - at first. There were little tell-tell signs, but nothing so obvious that I suspected anything as devastating as a brain cyst. I didn't suspect there was a problem with my brain at all. I thought maybe I was tired, getting forgetful, or maybe even imagining things. But one day when I couldn't find the milk in my grocery store where I shopped regularly, and not just the milk, but the entire dairy section, I realized there was something very wrong with me.
The day I couldn't find the milk, I got nervous and decided to leave the store - to head to the school to get my older son. But suddenly I couldn't find my way out of the store. I was lost in the grocery; disoriented and confused. I had to rely on my four year old son to lead me out of the store, making a game of it, "Show mommy where the door is. Do you remember where the door is?" Nothing was making sense, and nothing was clicking with me, not even the normal roads I took to get my older son from school. I couldn't remember how to get around my own town, and it was then, that panic sunk in.
An MRI caught images of a very large brain cyst on the back of my brain. So big, in fact, that it took up nearly a third of my brain - the entire back portion of my brain and wrapped around to the right side. It was enormous, even by the doctors' standards. Most doctors who saw it, told me it was either the largest or one of the largest brain cysts they'd ever seen in all their years of practice. I too, was in awe at what I was looking at. And soon, everything I'd been living with and dealing with - clicked. Now, it made sense as to why I had pain and discomfort at the back of my head, especially when I'd lie down on my back, or rest my head on the back of the recliner or car seat. There were headaches in the back portion of my head, confusion, numbness in my extremities when I lied down due to the cyst putting pressure on my brain stem. Dizziness. I started having balance problems due to the compromising of my cerebellum, I couldn't think straight....nothing was making sense, and just in general, weak and lethargic.
I just knew this cyst had to be the culprit for everything I was living with, but the doctors disagreed. They tried hard to convince me that these cysts are normally asymptomatic and don't normally cause problems for people. I disagreed. To me, there was no way this cyst wasn't the culprit of all my symptoms. I believed it was, but now, I just had to convince the doctors that I knew what I was talking about. I had to get them onboard with this discovery.
It would take months of searching for a doctor that would listen to me; one that understand these cysts, and believed me with all my new and unnerving symptoms. All the while I continued to go downhill. I was sinking lower and lower into a pit of despair. My breathing was now compromised when awake and upright, and I just knew that it was only a matter of time that something terrible was going to happen if someone didn't do something about this cyst soon - a seizure, stop breathing altogether and die - something.
It felt like a miracle when I found Dr. Spetzler. A friend of mine knew his name, and knowing my situation, she gave it to me with his number. I sent him all my records and films I'd had to-date for him to review, and give me his professional opinion. Boy, was I shocked when I got the call. "You need surgery," the voice said. "Brain surgery." I had to sit down. Although it was what I suspected, just hearing those words made me lightheaded. It felt so surreal. I couldn't breathe for a minute.
"Brain surgery?" Was all I could say.
He explained too, how I needed it right away. As soon as I could get it scheduled and get out to Phoenix. Still in pure and utter shock, I had a lot of work to do to get family told, and someone to fly to Albuquerque to watch the boys. And I did it all while in a fog - partly due to my cyst, and partly due to just being shock at the fact that I needed brain surgery. I was in shock that I was going to go through with it, and have it....with a man, and doctor I'd never even met in person yet.
I had to sit my boys down and tell them. That was hard - so many questions. And the looks on their faces that told me they were worried may have been the hardest of it all. Knowing my boys were worried and I had no solid proof that I would be ok - that I would be coming home the same mom, or even at all, tore my heart out. But I did my best to ease their worries.
The surgery went off without a hitch. I not only lived through it, but went home feeling like I had a new lease on life. I felt like everything was going to be fine and I would be back to my old self and to my old life in no time. But that would not be the case because within three months, all my symptoms had returned and I was heading back to Phoenix to find out that I needed another surgery. This time to put in a shunt. Oh, that was something I didn't want - a device in my body that would require monitoring and adjusting in order to keep me feeling okay. But in order for me to feel okay, I had to have it.
My shunt, my shunt, my shunt! What a nightmare my shunt turned out to be. The doctors just could not get it adjusted so that I felt good. It was not doing its job of keeping the pressure off so that I felt good, and symptom-free. Up and down, down and up they went with the settings, trying to find the right one that would give me relief and help me to feel as much like my old self as possible. The one doctor who followed me in my hometown just did not seem to care, and what was worse, was that he didn't believe me that I was still having pressure problems, despite having the shunt. He treated me as if I were some crazy woman off the streets, a hypochondriac, an overemotional woman. I had to fight him every step of the way to get him to listen. How many nights did I spend crying because I felt defeated? Too many to count. I just knew the end was near, and especially if I couldn't get the shunt set right to get me some relief.
I had been set at a lower setting for several months - about 3, when I started having even more odd and much different symptoms than ever before. I was getting a pain on top of my head that spread like a ripple effect in the water. It started as a quarter-sized area of pain almost directly in the middle of my scalp. It then began to spread outward, all over my skull. Before long, I was having terrible memory problems and the pain became so intense that the only position that was comfortable - and I mean, just a little comfortable, was lying down. So gradually, over time, I was spending more and more time in bed. I was essentially bedridden. I was nauseas and throwing up, I had no balance left, I couldn't think straight, and it just seemed like I was sinking deeper and deeper into a pit of despair. I was dying. I just knew it and my doctor would not allow me to come into his office to be seen. I didn't understand that.
When I couldn't remember my children's names, the first time ever that something so personal as my own children's names had gone blank and I could hardly stand, let alone walk, we decided we needed to get me back to Phoenix - back to the doctors who cared. The doctors who had operated on my brain twice and had been up close and personal with the very organ that controlled everything about me. So we did. We packed our bags and made the 8 hour trip back to Arizona.
An MRI in Phoenix revealed the secret. My brain was sagging and I now had a Chiari Malformation. What? Sagging and a Chiari? I was in utter shock. "How did this happen?" I asked.
"Your shunt over-drained and when it drained too much fluid for too long, it dries your brain up till it sags. Then, the sagging causes the brain stem to fall lower, down into your spinal cord, called a Chiari Malformation." I cried. I knew that something was dreadfully wrong and had tried to tell the neurosurgeon in my hometown who was supposed to be following me. And he should have seen the signs of too low of pressure. He is a neurosurgeon and had actually talked to my husband and me a couple of times about the danger of too low of pressure with shunt settings. He seemed to be purposefully ignoring me all those months and refusing me to come see him despite him knowing what was going on. What was happening to me? Why? We don't know, but we suspect that it started with the fact that from the beginning, he never believed me with my symptoms of being under pressure, even with the shunt. He had always treated me like a hypochondriac and like someone who was crazy and too sensitive or something. So he got to where he just refused to see me, at all.
The answer to my new problems and diagnosis was to turn my pressure back up, to get more fluid in my head and around my brain in hopes that the extra fluid would float my brain back up to where it was supposed to be. I hated to do it, after all the trouble I'd had with too much pressure on the brain, even with the shunt. But who was I to argue? He was the doctor and he knew what was best - and this group definitely had my best interest at heart. They cared and they wanted to do everything possible to get me better.
I was scared. I laid there trembling as he adjusted my pressure up a little to get some more fluid in my head and around my "drying-up" brain. It took about 3 days before I noticed a significant difference, but it finally started to relieve the low pressure symptoms. It just wasn't enough, so they went up again, and again. Over the next couple of months, I went from a pressure of 80 to 130 and although my symptoms were getting better, my brain had just suffered too long without adequate fluid that there was only so much it could do. The damage was done and I was left with plenty of symptoms - permanently. I was devastated. I had wanted and hoped all the sagging and Chiari symptoms would dissipate over time with the pressure raises, but that just wasn't the case.
It has been six years now since my first surgery for the cyst, and five years since the brain sagging and Chiari started. I have since gotten divorced this past year, due largely in part to my husband losing his compassion for my situation and what I have been left to live with. I still live with a significant sagging to my brain and still have a significant Chiari which are responsible for a lot of my symptoms now.
I have been told by the neurosurgeon I now see at Duke University, since my move to North Carolina in 2007, that he would not feel comfortable operating on my Chiari because he is afraid he will make my sagging worse. I have been told that I would need to go to the Chiari Institute in New York to see if they could help me. But at this time, being divorced and on my own, who has time for brain surgery; much less a major brain surgery? I don't. Not to mention I am afraid to rock the boat. I am afraid that having another surgery is taking a big chance and that I could come out of this one with many more problems than I already live with.
Going through what I've been through has taught me a lot - a lot about life, and more importantly, a lot about myself. One thing - a major thing, is that doctors need to listen to their patients. If they don't, then they run the risk of a problem getting worse and doing much more harm than if they listened right away and believed. The other thing is that I am much stronger of a person than I ever thought I was or gave myself credit for.
Over the years I have discovered that you cannot control your future. No matter how much you plan, you cannot make things go the way you want or think they should go. Sometimes you just have to go with the flow, and you have to listen to your body and trust in yourself. Once you do that, you are well on your way to healing.
