When Your Brain is Falling Out: My Chiari Diagnosis

Two days ago, I was diagnosed with a Type I Chiari Malformation and the diagnosis changed my life. Chiari is common, but somewhat rare because not many doctors seem to know about it. However, this condition affects 1 out of 1,000 births in the US but most go on without never being found because not every gets brain scans. I personally went for an MRI because I had headaches every single day.

I also found myself sleeping most of the day, dealing with hand tremors, sharp neck pain, and failing eyesight, and of course muscle weakness/lack of coordination which has put me out of lifting heavy weights for quite some time now. It has also been tough to drive some days which is why I have been home so much lately.

Thankfully, I knew about Chiari because my sister in law almost lost her life with her Chiari battle (hers was worse). So when my doctor had called me to tell me my MRI brain scan results, I almost dropped the phone. “Why me?” I asked myself. I asked God.

This week has been cruel. Just a day before, I had bought plane tickets for my family to California and paid a $500 deposit on my breast explant surgery only to find out it may have to be put on hold because my breast surgeon does not want to touch me until I see my Chiari specialist. I am still waiting to hear back on this, but it looks like no surgeon will want to remove my implants due to this being a brain issue. Anesthesia can cause pressure within the brain which could lead to further leaks, bleeding, or herniation. Sigh.

My life has been changed and I face having a possible decompression surgery if not now then definitely in the future. There is not a cure for chiari. The surgery will stop the progression and relieve symptoms only. But that is brain surgery guys..  I am terrified of this.

My first step in my plan of action is to find a Chiari specialist-luckily we have one in mind that my sister-in-law used. We are sending my MRI results to him and will wait for further interpretation to see how bad my case is and what can be done. I will likely need to see a Neurologist and Neurosurgeon for the rest of my life. I won’t be able to go on rollercoasters anymore and the way I have kids-if I can have anymore-will be changed as well if my case is severe enough. I hope my symptoms can be controlled with medication because I want surgery to be a last resort. But I must see what the degree of my brain herniation is before we can make that call. The doctor who called me on the phone literally could tell me nothing about Chiari which was disheartening, but something most docs will never have to face.

I also learned that Chiari is hereditary which means it runs in my family on either side. This also means that there is a huge chance that my kids, my two beautiful babies, could have this. This is not a fun feeling, but at least we know and can watch for it as they grow.

How I am feeling? Distraught. In shock. But life must go on. My family depend on me so I will fight to get better.

If you want to learn more about my condition, read on.

What is Chiari?

Chiari malformations are structural defects in the base of the skull and cerebellum, the part of the brain that controls balance.  Normally the cerebellum and parts of the brain stem sit above an opening in the skull that allows the spinal cord to pass through it (called the foramen magnum).  When part of the cerebellum extends below the foramen magnum and into the upper spinal canal, it is called a Chiari malformation (CM).

Chiari malformations may develop when part of the skull is smaller than normal or misshapen, which forces the cerebellum to be pushed down into the foramen magnum and spinal canal.  This causes pressure on the cerebellum and brain stem that may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)—the clear liquid that surrounds and cushions the brain and spinal cord.  The CSF also circulates nutrients and chemicals filtered from the blood and removes waste products from the brain.

What causes these malformations?

CM has several different causes. Most often it is caused by structural defects in the brain and spinal cord that occur during fetal development. This can be the result of genetic mutations or a maternal diet that lacked certain vitamins or nutrients. This is called primary or congenital Chiari malformation. It can also be caused later in life if spinal fluid is drained excessively from the lumbar or thoracic areas of the spine either due to traumatic injury, disease, or infection. This is called acquired or secondary Chiari malformation. Primary Chiari malformation is much more common than secondary Chiari malformation.

What are the symptoms of a Chiari malformation?

Headache is the hallmark sign of Chiari malformation, especially after sudden coughing, sneezing, or straining. Other symptoms may vary among individuals and may include:

• neck pain
• hearing or balance problems
• muscle weakness or numbness
• dizziness
• difficulty swallowing or speaking
• vomiting
• ringing or buzzing in the ears (tinnitus)
• curvature of the spine (scoliosis)
• insomnia
• depression
• problems with hand coordination and fine motor skills.

How to treat Chiari:

In many cases, surgery is the only treatment available to ease symptoms or halt the progression of damage to the central nervous system.  Surgery can improve or stabilize symptoms in most individuals.  More than one surgery may be needed to treat the condition.

The most common surgery to treat Chiari malformation is posterior fossa decompression. It creates more space for the cerebellum and relieves pressure on the spinal cord.  The surgery involves making an incision at the back of the head and removing a small portion of the bone at the bottom of the skull (craniectomy).  In some cases the arched, bony roof of the spinal canal, called the lamina, may also be removed (spinal laminectomy). The surgery should help restore the normal flow of CSF, and in some cases it may be enough to relieve symptoms.

Next, the surgeon may make an incision in the dura, the protective covering of the brain and spinal cord.  Some surgeons perform a Doppler ultrasound test during surgery to determine if opening the dura is even necessary.  If the brain and spinal cord area is still crowded, the surgeon may use a procedure called electrocautery to remove the cerebellar tonsils, allowing for more free space.  These tonsils do not have a recognized function and can be removed without causing any known neurological problems.

The final step is to sew a dura patch to expand the space around the tonsils, similar to letting out the waistband on a pair of pants.  This patch can be made of artificial material or tissue harvested from another part of an individual’s body.

If you think for one second that you could have Chiari, please visit your doctor and tell them about your headaches and demand an MRI. It is the only way to tell! No blood tests will pick up on this.

Thank you all for your support. This is a new journey for me and I will gladly share more when I know more.

Info from: NIH