Chiari is not my friend, yet

I have a Chiari Malformation Type 1. You may have never heard of it. I know that I had no clue what it was when my doctor called me and diagnosed me with it. An MRI showed an abnormality of the cerebral tonsils, part of the cerebellum of my brain. I understood her to say that there was part of my brain that was sticking out of the opening of my skull. I'm sure that was not what my doctor really said but that is how I interpreted it at the time. I remember thinking "great, now I have to tell my husband that I'm a freak of nature!". I was in shock. My doctor was going to set up an appointment with a neurosurgeon for a consult. Of course I looked up every everything I could find about Chiari and became my own advocate. I had plenty of time on my hands to educate myself and plan for the what ifs. I had to wait 4 weeks to get in which felt like an eternity. My crisis wasn't any bit more important than anyone elses. I had been waiting for ten years to be diagnosed. I had been suffering with chronic headaches for what seemed to be a lifetime. In recent years I had developed vertigo, left sided body weakness, tingling and pain. I had severe hip and leg pain. I was seeing flashing colors and seeing objects coming at me that weren't even there. The last straw for me was when I blacked out while I was with my two children. After that I set out on a mission to find out what was wrong with me. I had seen doctors before but all of my tests including neuro exams were unremarkable. The MRIs shown an area deep in the brain that concerned the radiologist but in the end was diagnosed as being just space. Nothing apparently was wrong with me and the sometimes disabling pain that I was experiencing must have been all in my head - (literally as I came to find out later on).

Chiari malformation is a condition in which brain tissue protrudes into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward. Chiari malformation is uncommon, but improved imaging tests have led to more frequent diagnoses. The adult form, called Chiari malformation type I, develops as the skull and brain are growing. As a result, signs and symptoms may not occur until late childhood or adulthood. Treatment of Chiari malformation depends on the form, severity and associated symptoms. Regular monitoring, medications and surgery are treatment options. In some cases, no treatment is needed. Headaches, often severe, are the classic symptom of Chiari malformation. They're typically precipitated with sudden coughing, sneezing or straining. People with Chiari malformation type I also can experience:
Neck pain (running down the shoulders at times)
Unsteady gait (problems with balance)
Poor hand coordination (fine motor skills)
Numbness and tingling of the hands and feet
Dizziness
Difficulty swallowing (sometimes accompanied by gagging, choking and vomiting)
Vision problems (blurred or double vision)
Slurred speech
Less often, people with Chiari malformation may experience:
Ringing or buzzing in the ears (tinnitus)
Poor bladder control
Chest pain, in a band-like pattern around the chest
Curvature of the spine (scoliosis) related to spinal cord impairment
Abnormal breathing — specifically, sleep apnea characterized by periods of breathing cessation during sleep In some people, Chiari malformation can become a progressive disorder and lead to serious complications. In others, there may be no associated symptoms, and no intervention is necessary. The complications associated with this condition include:
Hydrocephalus. This accumulation of excess fluid within the brain may require placement of a flexible tube (shunt) to divert and drain the cerebrospinal fluid to another area of the body.
Paralysis. This may occur due to the crowding and pressure on the spinal cord. Paralysis tends to be permanent, even after treatment with surgery. Syringomyelia. Some people with Chiari malformation also develop a condition called syringomyelia, in which a cavity or cyst (syrinx) forms within the spinal column. Although the mechanism connecting Chiari malformation with syringomyelia is unclear, it may be associated with injury or displacement of nerve fibers in the spinal cord. When a cavity forms, it tends to be filled with fluid and can additionally impair the function of the spinal cord. The diagnostic process begins with your doctor taking your medical history and with a complete physical examination. Your doctor will ask whether you're having symptoms such as head and neck pain, and will ask you to describe them. He or she will also check your fine motor skills and swallowing ability.
If you have symptoms such as head pain, and the exact cause isn't apparent to your doctor, you'll likely undergo a magnetic resonance imaging (MRI) scan of your skull, which is the definitive diagnostic tool for Chiari malformation. Using magnetic fields and radio waves, this test produces 3-D, high-resolution images of structural abnormalities that may be contributing to your symptoms. It can also provide pictures of the cerebellum and determine whether it extends into the spinal canal. A dye or contrast medium can be injected, and once it travels to your brain, it can enhance the MRI images. An MRI is a safe and painless test. Over time, repeat MRIs can be used to monitor the progression of this disorder. Your doctor may use other imaging techniques as well. For example, your doctor may recommend a computerized tomography (CT) scan, which uses X-rays in conjunction with a computer to produce precise, sectional images of the bone tissue that surrounds the spinal column.Treatment for Chiari malformation depends on the severity and the characteristics of your condition. If you have no symptoms, your doctor likely will recommend no treatment other than monitoring with regular examinations. When headaches or other types of pain are the primary symptom, your doctor may recommend pain medication. Some people experience symptom relief with anti-inflammatory or pain-relieving agents, such as indomethacin (Indocin). This approach may prevent or delay the need for an operation. Reducing pressure by surgerySurgery is the approach doctors use most often to treat symptomatic Chiari malformation. The goal is to stop the progression of changes in the anatomy of the brain and spinal canal, as well as ease or stabilize symptoms. When successful, surgery can reduce pressure on the cerebellum and the spinal cord, and restore the normal flow of spinal fluid. In the most common operation for Chiari malformation — called posterior fossa craniectomy or posterior fossa decompression — your surgeon removes a small section of bone in the back of the skull, relieving pressure by giving the brain more room. The covering of the brain, called the dura, is then opened, and a patch is sewn in place to enlarge the covering and provide more room for the brain. This patch may be an artificial material, or it could be tissue harvested from your own leg or neck. The exact technique may vary, depending on whether a fluid-filled cavity is present, or if you have hydrocephalus. The operation takes about two to three hours, and recovery in the hospital usually requires two to four days. Risks and follow-upThe use of surgery carries risks — the possibility of infection or problems with wound healing. Discuss the pros and cons with your doctor when deciding whether surgery is the best alternative for you. The operation reduces symptoms in most people, but if nerve injury in the spinal canal has already occurred prior to surgery, this procedure won't reverse the damage. After the operation, you'll need regular follow-up examinations with your doctor, including periodic imaging tests to assess the outcome of surgery and the flow of cerebrospinal fluid. Information provided by www.mayoclinic.com

I went to my neuro consult educated about my condition, with a list of questions and most importantly with a support person. My neurosurgeon, whom I ended up respecting, was upfront with me and told me that I only had a 50% chance of resolving my symptoms with the surgery. After much contemplation I chose sugery because for me, it was a quality of life issue. When I blacked out while being a lone with my children, I knew that I had to try to be better. Before scheduling the surgery my Neurosugeon required a psych eval to make sure that I was a surgical candidate. He wanted to make sure that I was mentally compitent for a surgery of this calibar. Did I have a support system afterwards? If there were complications would I be able to handle it emotionally? At the time, I felt this was fairly odd but looking back I can appreciate why he required this. My suggestion to the Neurosurgeon now would be - require counseling post recovery which is much more of a challenge.

I thought I knew everything I needed to know going into surgery. I signed my life away and off I went. During the prep, the nurse did scare me a bit when she said that I may wake up at some point during the surgery. She said do not panic, that there would be gel over my eyes so that the tape holding them in wouldn't stick. She also said I would have goggles on. She said I would be in an upside down position and that I would have small metal rods in my temples, holding my head in place. I felt fear at that point...... count backwards 10, 9, 8, 7, 6, 5 and then the surgery was over, 6 hours later. I woke up in ICU with the worst headache I had ever had in my life with a handsome male nurse changing my catheter. I didn't take the time I should have to recover. I had two little girls that depended on me. Due to financial restraints my husband didn't take any time off of work. My mother came as much as she could. I looked scary, half my head was shaved and I had a 5 inch insicion that ran from the top of my neck to the top of my occiput. I was stapled together and it didn't look pleasant to look at. The girls were afraid so it was my goal to be as normal, as possible as quick as possible.

As the months went by I felt better and better. Most of my symptoms were gone. At my post surgical visit, the Neurosurgeon said that the herniation was worse then the MRI had shown and that as soon as he decompressed the area it restored the flow of the cerebral spinal fluid. The cerebral tonsils were still herniated but the pressure had been released. The surgery had worked. 6 months later my headaches returned. An MRI showed no post complication of the surgery and that the area was still decompressed. The neurosurgeon said I was apparently now a migraine sufferer. Within 2 years post-op I had all the symptoms return except the black outs. 5years post op which brings me to today, I am the same as I was 2 years ago except I have new symptoms. I have trouble swallowing, I have devloped a twitching in my left eye and I have far more bad days then good. I have awful nausea and digestive issues that come and go without notice. The nausea is apparently the 4th venticle, the nausea center of the brain being affected by the pressure as a secondary issue from the Chiari. My post surgical neck is stiff and painful everyday. The muscles were cut during the surgery. There may be scar tissue that can cause the pain and or nerve pain. The neurosurgeon suggested building the muscles in the upper body to prevent further injury. He suggested Isometric ecercises for the neck and lap swimming for my upper arms and trap muscles. He also suggested always protecting my neck from the cold which would intensify my discomfort. The neurogurgeon suggested not doing anything that caused me pain and the tasks that I have to do so that it's comfortable. For example, while reading a book I have to hold it equal to my eyes so that my neck is not tilted down. He basically told me that I had to modify my life to live with what I have. He suggested I try to avoid another surgery in order to prevent more scar tissue which would only create more pain and nerve damage.

I see a neurologist every 3-4 months who monitors my chiari but mainly focuses on my daily headaches. He believes my Chiari is not an issue. He feels that I am a migraine sufferer and tells me that once a migraine sufferer, always a migraine sufferer. Finding the cause is likes like finding a needle in a haystack. Apparently it could be almost anything - once you have migraines anything can trigger them. When he told me this at my last appointment I immediately felt tears well up in my eyes and tingles in my stomach. I felt like he was telling me that I didn't know anything and that he was throwing me in a category of "catch alls". Frustration overwhelmed me because Chiari research shows that low lying cerebral tonsils itself cause symptoms. And that is where it stands today. I have tried to get into a Chiari Clinic but my HMO will not approve the testing required for the consult. So for now I have decided to take it one day at a time. The good thing is.... things could be so much worse. I have daily headaches but most days they are managbale. On the days they aren't I may have to shut down but I get through. There are days my neck feels so painful that I feel I can't carry my head on top of my head, but I'll get through it. My body hurts most days but I'll get through it. I don't feel sorry for myself....... I feel sad for my girls. Most days I don't feel like running around with them, or riding bikes, or even going anywhere. Until recently I haven't let them have friends over to our house because the noise just echos in my already painful head. They have had to pay the price for my pain and that makes me feel incredibly sad. Having Chiari has been life changing in many ways. I've decided that I have to find a way to make it into a positive experience. I can't let the pain defeat me. I have to find a way to make Chiari my close and personal friend. :)