Chiari Malformation

September is Chiari Awareness Month

Severe headaches of unknown cause can leave doctors and patients puzzled and frustrated.

The following stories are real, but the names have been changed to protect the privacy of those involved.

It was 2006.

I met my friend Lulu at church. She was 30-something, had a family, was working, everything was fine. Until it wasn’t.

She had been having extreme pain down the left side of her body and the doctors didn’t know why or what was going on. For months.

Fortunately for her, she ended up in the ER.

The ER physician knew just what was wrong.

Chiari malformation type 1.

Once she researched and asked the doctors about it, she told me all about it. Back then it was considered a rare disorder.

She said there are different types and that most neurologists don’t know what to do for it.

She ended up opting for surgery, and the surgery did not help her at all.

purple ribbon - sept is arnold chiari malformation month

It was 2013.

I met my friend Donte at work. In his late 20’s and a very pleasant guy, he enjoyed being around others.

One day he remarked that his pills were gone from a drawer in his office.

“Pills?” I asked.

Yes. He was on fioricet for his excruciating headaches.

Matter of fact, he had such frequent and extreme headaches that he had missed a LOT of work in the past 18 months. He called out sick regularly and had used more than his allotted vacation time.

Wow.

About a year after that, we were working at different places, but our families still went to dinner sometimes.

We met them for dinner, and he explained to me that he had found out why he was having the headaches.

Chiari malformation type 1.

In 2019, I asked how he was doing.

He found an eating plan that helped tremendously.

He doesn’t get the headaches nearly as much.

It was 2012.

We met our now-family friends, Daisy and her son Jeff through The Teen’s elementary school. The Teen and Jeff were in the same class and were friends.

They moved away after 6th grade.

We keep in touch and he visits regularly.

He told me on his last visit that he takes medicine to prevent his extreme headaches.

Headaches?? In a teen boy?

Wow.

I asked his mom Daisy about it.

She said, yes. She gets them, also, but not as strong as his.

Hers had been corrected surgically years ago.

She rarely gets them now, and they’re not as severe.

“Surgery?” I asked.

“Yes. I have Chiari malformation and so does Jeff. They think it’s genetic. I have been able to get relief from the surgery.”

I have 4 friends with Chiari malformation and before a few years ago I had never heard of this syndrome?

This is turning out to be quite a common theme in my set of friends for such a rare disease.

Just what is Chiari malformation?

Chiari is a malformation/structural defect of the base of the skull and cerebellum¹. If the skull is too small or misshapen, it can push the brain down into where your spinal cord is supposed to go, causing extreme pain and headaches.

This condition, which used to be listed as rare, is now considered common.

How Do They Detect It?

According to Lulu, the doctors diagnosed hers by an exam, CT scan, and MRI, and they measure how far off her brain is from where it’s supposed to be each time she gets a new MRI.

They do MRIs regularly to check the status and see if there are any changes.

According to the NIH site, CT scans and X-rays are also used, along with a physical exam and some tests of balance, memory, and other items.

As seen in the graphic below, pain and headaches are not the only symptoms.

(By sleeping, they may mean insomnia, as that is listed on the NIH website.)

Currently there are 4 types of Chiari malformation, with type 1 being the most common.

You can read more facts about Chiari types, diagnosis, and other information on the NIH Chiari Malformation Fact Sheet. Mayo Clinic website also has an informative write-up about Chiari and treatment options.