Chiari Warrior

Part One:

“My head hurts.”

Who knew that one statement would put us on the wildest medical rollercoaster ride of our lives? 

After being as sick as I’ve ever seen Caleb to be back in September 2022, he began lodging that complaint every so many days for a couple of months. Thinking it very odd that a six year old would experience pain in the occipital region of his head, I made an appointment with a neurologist. 

The doctor agreed that it was highly unusual for a child of that age to be having migraines, even more so to have them in the back of the head. A brain MRI was ordered, medicine prescribed, and encouragement given to make sure Caleb’s lifestyle was as healthy as we could manage. Short of embracing the suggested meditation, we tried and did it all. The result of the medication was that it was ineffectual. The result of the healthy lifestyle was that it did not change Caleb’s discomfort.  The result of the MRI was “borderline low cerebellar tonsils. The right cerebellar tonsil projects 5mm below the plane of the foramen magnum and the left projects 3.5 mm below the plane of the foramen magnum. The posterior fossa has a somewhat crowded appearance in general.”  And that’s when we first heard the term Chiari Malformation. It is the condition in which your cerebellar tonsils (the tissue at the bottom of each lobe) descend slightly below your skull because of a structural defect in your skull. Basically, the bottommost part of Caleb’s brain was hanging out in the top of the spinal column, crowding the area and crashing the proverbial party as a third-wheel participant.

Our neurologist believed that if this was the disorder Caleb truly had, then it was beyond her purview. So she referred us to a neurosurgeon. This next doctor assured us that the degree of herniation was nothing to worry about; his headaches probably weren’t even from the tonsillar ectopia (another term for Chiari) and since he looked really good upon his physical exam, she was confident surgery was not going to be necessary. However, in order to rule out another issue that can often appear in those with Chiari, she ordered a second MRI. This time it would take images of both the brain and the cervical (neck) area. 

Caleb’s first MRI was conducted in May 2023 and the second one in July of that same year. The result of that one? “…measure cerebellar ectopia below the level of the foramen magnum at up to 5.5 mm on this exam….Mild Chiari 1 Malformation with no syrinx (fluid-filled cyst found in spinal column that causes neurological issues) or other findings seen.” Our follow up with this neurosurgeon was most disappointing. She reiterated that Caleb didn’t need surgery and she was moving to CA, so good luck, hope all goes well, too bad about those headaches. 

Meanwhile, Caleb’s original complaint was quickly followed by several more.

“I have cold pricks in my palms. But it’s contagious or something because one hand will get it and then it travels to the other.”

“When I get up in the morning, my legs just say ‘nope!’ and I crawl back into bed. Sometimes, I can’t move my body at all. I’ll sloooowly move my head from side to side and then work hard to pull up my knees.” 

“When you drove the car around the corner, I was looking down at my book and then my head just immediately jumped to an 8 ½ (on the pain scale) and it rolled around. I can’t really describe it, but it all just–rolled around and felt really weird.”

“My legs felt weak and just didn’t want to do it but I made them run anyway. When I run, my feet thump, thump, thump, so I try to run lightly but that slows me down so I have to really concentrate on my steps.” * 

While these symptoms were being revealed to me through conversations with Caleb, we were working on our third opinion from a Boston Children’s neurologist who was hesitant to even agree that Caleb had tonsillar ectopia.Though she never reviewed the actual images from the two MRIs Caleb had had, she was confident in the radiologist’s report and believed two things: the tonsillar ectopia was an incidental finding (as in, an unexpected discovery unrelated to the reason why Caleb was having the MRI in the first place🤨) and something was causing these occipital headaches, but it sure couldn’t be the fact that his brain was drooping into his spinal column😑. 

She was a calm and competent neurologist, but she was also completely unconcerned. I’m sure it had something to do with the fact that during the neuro-physical exam of Caleb, he was perfectly capable of touching his nose with his pointer finger, hopping on one foot without falling over, squeezing her fingers with equal strength in both hands, feeling a light touch on his face, and running down the hallway and back without any issues whatsoever (his desire to show how fast he was overtook his memory of what running is truly like for him*).  Again, we were told to try a round or two of medicine, make sure he was eating well and staying hydrated, and we were offered a visit with a psychiatrist. I don’t think I could have rolled my eyes any louder. I was very thankful, however, that we walked out of there with an order for an EKG to check for (ir)regular heart function and a third MRI. This time of the full spine to rule in or out even more issues that Chiari patients suffer, such as a syrinx farther down or tethered cord (an abnormality where your spinal cord is anchored to the tissue around it, preventing free movement). 

As frustrating and defeating as that experience was, I wasn’t entirely surprised about the way it had gone. Prior to that visit, I had been joining group after group on Facebook where others were coming together to discuss their trials, questions, symptoms, and ordeals with neurologists and neurosurgeons, so I was aware that there are too many doctors out there who know very little about Chiari Malformation and can incidentally lead someone down the wrong path for a long time. Thanks to the hours I spent reading so many anecdotes, one name continued to be mentioned as an expert in the field, a doctor who practices out of Providence, Rhode Island: Petra Klinge.

Caleb’s third MRI was completed in January 2024 (and I made sure to come home with the disc that day), the EKG came back normal, and I managed the legwork it took to book an appointment with this highly sought after neurosurgeon whose practice is only an hour and a half away from our home. Knowing how many people I read about online who already had Dr. Klinge as their provider, and how many more were working on referrals and appointments, it can only be said that getting the first visit scheduled within months of the scan was the Lord’s doing. 

In the interim, I watched countless videos on how to read MRIs  and spent hours reviewing the images, comparing and contrasting them to ones available online. We also received the report of the scan through Caleb’s online patient portal. The most noteworthy parts that the radiologist stated were “mild cerebellar tonsillar ectopia” and “no substantial blockage, pressure, or narrowing affecting the flow of spinal fluid.” As I digested that summary, older symptoms were becoming more frequent and/or intense and new symptoms were mentioned by Caleb and catalogued. 

“My tongue is itchy.”

“My ears hurt.”

“It feels like someone is nailing something into my arms.”

“I feel like I can’t do anything, even though I know I can. But when I do something, it hurts.”

The appointment day finally arrived and we set off with the disc of images, a heart full of doubt, and a notebook of questions tucked in my purse. The previous doctors had told us that Caleb might have Chiari and maybe his headaches were a problem, though had we tried a healthy diet and exercise? Meditation? A good sleep routine? Medicine? I was quite anxious to find out if this next expert was going to suggest the same “treatments”.

*****