But for now ...we need to have a little talk about my brain. I'm going to structure this post as an FAQ. As someone with a little-heard-of neurological problem, I get questions every day. Unfortunately, Chiari is something that is very difficult to sum up for someone in 60 seconds or less. Even the people closest to me sometimes have a hard time understanding what I'm dealing with. Tell someone you've got cancer, and they know what that means. They understand what you're facing, they know someone who's been through chemo and what that looked like. If you tell someone you have Chiari, or one of the other names for it - hindbrain herniation, or tonsillar ectopia, you are generally met with silent confused blinking and blank stares. Who can blame them? It's not exactly a household name! Let's begin by going over some basics.
What is Chiari Malformation?
In the simplest terms, Chiari Malformation is a neurological condition whereby the contents of your brain do not fit fully into your skull. The bottom part of the brain, the cerebellar tonsils, become herniated through the base of the skull opening. This herniation acts as a cork, blocking the flow of cerebral-spinal fluid, or CSF for short. This can lead to several serious neurological issues, such as spinal cysts, syringomyelia, brain stem compression, nerve damage, and obstruction of CSF flow. The neurological system exists in a delicate balance, and any disruption of normal function can cause a whole array of symptoms in virtually every bodily system. The standard diagnostic criteria is a herniation of 3-5mm. Mine is estimated to be about 15mm.
What does that look like on an MRI? Can I see a picture of your Chiari?
Sure! I am a visual person, so it helps for me to be able to see what exactly we're talking about here. Since I happen to have an image handy of my brain, I can show you what it looks like. The first picture is a normal, non-Chiari brain. The second picture is my brain. See? It's so big and awesome, it can't even fit in my skull! :) I will be posting some more detailed pictures in another post, for you science nerds out there.
What symptoms does Chiari cause?
Chiari is a tricky disease. Some people can go their entire lives not knowing they have a brain herniation. It does not cause them any problems, and they will live a normal life. Others are plagued with problems from a young age, as young as infancy. Most people with Chiari Type I (which I have) are diagnosed as young adults in their 20s or 30s after years of symptoms that are notoriously hard to describe (and thus notoriously hard to diagnose). Since there is such a huge variation in symptoms in Chiarians, I will list my own symptoms:
- Pain. Daily, chronic, 24/7 pain. My pain is mainly in my neck and upper back and shoulders. I also have pain that radiates down my right arm and into my fingers on that hand.
- Headaches. Almost all Chiarians have very severe headaches, from the compression in their brain and nervous system. I have a headache just about every day. This is made worse by any change in position, as well as "Valsalva manuevers" - these include laughing too hard, crying, sneezing, coughing, or straining.
- Pressure in the head. This goes hand and hand with the headaches. The longer I am upright, the worse the pressure in my head is. It builds through the day. It is made worse by bending over and then standing, exertion, or external pressure changes (such as flying on an airplane). In my case, I have a CSF obstruction in my brain, so the pressure is actually from the fluid dynamics not being able to equalize properly. A normal person can bend over, pick up a piece of paper, and stand back up again...without a searing pain and pressure in their head. I can't do that. My head does not have the ability to comply with the small fluid fluctuations involved in a simple task such as bending over. The pressure and pain from doing that can last for hours.
- Muscle twitches and spasms. My body twitches constantly, all over. This is likely from nerve compression and damage. The muscle spasms can be quite debilitating, and occur most frequently in my neck. Ever turn your head too fast, and get a sharp painful crick? My muscle spasms in my neck feel exactly like that, only that sharp pain will last for days. During this time, I lose all mobility in my neck and am unable to turn my head.
- Weakness and fatigue. This is pretty self-explanatory! If I ever cancel on you at the last minute because I'm too tired to go out, don't be offended. Sometimes, I just have to lay down. I think this level of fatigue is mostly due to the bodily stress of being in chronic pain. In any case, I'm very tired most of the time. Insomnia is also very common with Chiarians, due to underlying changes in the brain. Luckily I combat that with my BFF, Ambien.
- Eye pain and pressure, and painful eye movement. Again, this is related to level of physical pressure in my head - both from the "clog in the pipes" which is my brain herniation, and the CSF obstruction. I do not get a normal level of CSF to my optic tracts, which causes intractable eye pain. It is very painful for me to look hard up or to the side. I also have visual disturbances, including blurred vision, seeing flashes of light or "floaters" that aren't really there, and double vision while lying down.
- Numbness and sensory loss in the hands and feet. This is the result of nerve damage. My feet feel like they are asleep and have pins and needles all of the time. I have lost some ability to sense the difference between hot and cold. I often burn myself by grabbing things that are too hot, because I can't feel the temperature. I also have patches of numbness in different areas of my body. This can have some advantages, though - last time I was in the hospital, they gave me a huge shot in the arse, and I didn't feel it a bit! :)
- Dizziness and light-headedness. I sometimes have a hard time with driving or going to the store, because I feel as though I will pass out. Usually this is the worst when my pain is not under control.
Some other common symptoms that Chiarians can experience (but I have so far dodged) include:
- Difficulty swallowing
- Bladder issues/incontinence
- Nausea or vomiting
- Seizures
- Ringing in the ears
- Facial pain
- Drop attacks
What can be done to treat Chiari? Is it life-threatening?
Although there are many ways to manage the symptoms of Chiari, there is no true cure for Chiari. Most of the care is aimed towards alleviating pain and nerve issues, so that a good quality of life can be maintained. If the Chiari is progressing, and causing CSF obstruction, significant nerve damage, and/or spinal cysts, surgery should be strongly considered. But, even surgical intervention is aimed to halt the progressing of Chiari; it can not "fix" your brain in the truest sense. It is a treatment, but it is not a cure. A lucky minority who have symptomatic Chiari will find their symptoms all alleviated from one decompression surgery, but they truly are a minority. I will talk more about Chiari surgery in my next post. Once you have Chiari, there will be life-long modifications that you will need to adhere to and be attentive to.
Some subtypes of Chiari are life-threatening, but the majority of people have Type I, which is not often fatal. The fatalities of Type I Chiarians are most often due to post-operative infections, surgical complications, meningitis, swallowing difficulties and aspiration, medication overdoses, and syringomyelia (a type of spinal cyst common to Chiarians) which extends upward to the brain. But most people with Chiari Type I will not find their life expectancy shortened.
Since I am starting down the barrel of my second decompression surgery (8 days and counting!) I will be back on Friday with another post about Chiari surgeries, what they entail, and what they can and cannot do.
Very interested in how it turned out!
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