HOW TO GET FLEXIBLE BODY
WHEN BEING FLEXIBLE CAN CAUSE YOU UNBEARABLE PAIN
KRISTINA MARIE SUFFERED NONSTOP, DEBILITATING HEADACHES FOR YEARS. THEN SHE GOT A SURPRISING DIAGNOSIS.
Imagine a headache so excruciating you can’t focus on anything but the pain. This headache crushes your skull, knocks you flat, obliterates all other sensations. Now imagine living with it for a year — straight.
This was the hellish reality for Washington-based writer Kristina Marie, now 29, who was a senior in college when her epic headache first began.
“I was really terrified in the beginning,” she recalls. “I thought I might even be dying because I’d always been super healthy as a kid.”
The headache struck out of nowhere while she was in a ballet class. She somehow managed to drive herself home before going to the ER. Before that day, she’d never set foot in a hospital.
“The doctors told me it was probably a bad migraine and gave me an IV cocktail that didn’t help at all,” she says.
Little did they realize that she would experience the same torturous symptoms every day for a year — extremely uncharacteristic of a migraine.
“I got shuffled from specialist to specialist,” she said, “and everything with my brain and neck checked out pretty normal.”
It didn’t help that a few weeks after the onset, she suffered a car accident severe enough for her airbag to deploy right into her face, giving her whiplash. Despite the ensuing months of physical therapy, her jaw became so tight that she had to eat a liquid diet for a month.
But there was something strange about her condition that her physical therapists consistently noted with amazement.
“You’re so flexible,” they would say. “Even though you just had this bad car accident, you still have such a great range of motion.”
Kristina visited countless professionals over that first year — internists, chiropractors, naturopaths, massage therapists — who all had their own erroneous theories about what was wrong with her.
Even after she fainted at her college graduation ceremony and had to see a cardiologist, no one put her constellation of symptoms together.
That is, until she saw an orthopedic surgeon whose suspicions would change everything.
“How far does your pinky bend back?” the surgeon asked her.
She wasn’t sure what her pinky had to do with her headache or her jaw tightness or her neck pain, but she complied. Her pinky bent back past 90 degrees. Then he asked her whether she could bend forward and place her hands flat on the floor without bending her knees. She could. She’d always been flexible, she told him. That’s why she was so good at ballet in her childhood.
The surgeon explained that he was testing her for a measurement called a Beighton score, which assesses patients for flexibility. Most people score a 1 or a 2 out of 9 questions. She scored a 7.
His suspicions were confirmed: Kristina had joint hypermobility syndrome, a condition in which joints can easily move beyond the normal expected range due to weak connecting tissue. It tends to be inherited, though no single gene has been isolated that would provide genetic confirmation of the diagnosis. Depending on the case, the symptoms can range from mildly annoying (being double-jointed, having periodic aches) to totally debilitating (joint dislocations, muscle spasms because the surrounding muscles have to work overtime to keep joints in place).
Still, over the ensuing six years, she didn’t understand why she got such terrible headaches. Because medication didn’t help, she eventually learned how to decrease her pain levels through heat, ice, and other tools taught by her physical therapists — though nothing works 100 percent.
“I’ve had a headache for too much of my 20s,” she laments. “I almost always have a low-grade headache in the back of my skull.”
Though she’d “learned to function” with the pain, a new general internist whom she visited last year insisted on digging further. Kristina had already had a standard MRI of her brain in the past that was normal. But once her hypermobility syndrome was diagnosed, her internist realized that she needed an upright MRI rather than the regular lying down, closed tube.
“There should be almost no movement at all where head meets spine,” says Dr. Harold Rekate, professor of neurosurgery at Hofstra. “When a patient has a connective tissue disorder, the upright MRI will show you that movement spontaneously, while you would have to make it happen if the patient is supine.”
Kristina’s symptoms were always better lying down, but in an upright position, the MRI could easily capture the natural — and painful — positions of her brain and neck.
“I chickened out for a year after the doctor ordered the upright MRI,” she admits. “I was feeling a little better, so I thought, maybe I just don’t want to know.”
But then last fall, the pain in her head and neck worsened again. From the moment she woke up, it would torment her. So she finally went for the upright MRI this June. Rather than lying prostrate, she sat up and watched a movie for two hours.
A few days later, the shocking result came back: She had a Chiari malformation — a hernia in the back of her head where her brain had fallen into her spinal column. Such structural abnormalities were once estimated to occur in 1 in 1,000 births, though modern imaging techniques show they are actually more common. They are usually only discovered during the course of treatment for another disorder.
Most are congenital, but hers was acquired in adulthood as a result of her hypermobility syndrome and may have been aggravated by her car accident. Now she finally had an explanation for her intractable headaches — and a potential cure.
“I cried tears of relief and tears of doom and gloom,” she remembers, “because I knew the treatment for it is most often surgical.”
The standard treatment is to undergo a procedure in which the surgeon removes a small piece of the base of the skull to create more room for the spinal fluid to flow. This relieves pressure on the spinal column and give more space to the cerebellum, a structural region in the back of the brain that is important for motor control.
However, hypermobile patients face extra risks because taking away part of their skull can create more cervical instability and actually worsen their symptoms. Dr. Rekate sees 75 to100 patients like Kristina per year, the majority of whom have been made worse by a previous surgery.
“In regular Chiari patients, their head doesn’t move,” says Dr. Rekate, who is director the Chiari Institute in Great Neck, New York. “When you’re hypermobile and the angle changes, there’s an apex where that angle changes that actually pushes into the front of the brain, so by straightening it out you are taking pressure off the brain stem.”
Because the surgery can be more dangerous for patients like her, Kristina is proceeding with caution. She’s consulted two neurosurgeons — one in New York and one locally in Washington. She’s also going to meet with a spinal surgeon for another opinion before she makes a decision.
“There’s no going back from neurosurgery,” she says. “It’s a long, tough recovery, but if surgery will help me move forward, it’s probably worth it.”
And there’s nothing she wants more than to put her headaches behind her, especially now that she’s engaged to her boyfriend of two years. And now that she at last has a diagnosis, she’s emerging from the isolation of suffering from a chronic mystery condition.
“It’s been hard dealing with the stigma of it,” she admits. “Some people would say to me, ‘Your car accident was seven years ago, why aren’t you better yet?'”
But her family and friends have served as a crucial support system, as well as a Facebook group of other individuals coping with the same condition. For anyone else who may be going through a terrifying medical odyssey, she suggests seeking out a support group for solidarity and resource-sharing.
“Don’t try to go through it alone because it can eat you alive,” she advises.
And most importantly: Figure out what matters to you.
“I certainly wish I would have been diagnosed sooner,” she says, “but it’s also a blessing because a serious health problem forces you to take a step back and re-prioritize.”
Rather than running around busy from morning to night, never able to say no, she focuses on what’s important — her family and friends, her writing, her slower lifestyle.
“I’m so much more thankful for little things that I took for granted when I was so busy,” she says. Things like going to the grocery store, or hanging out with her fiancé, or days when her pain is low.
She’s facing surgery with trepidation, but also courage.
“I’m ready to move forward with the next chapter,” she declares. “I’m glad treatment is available, even if it’s not the treatment I’d hoped for.”
Even as she speaks, the pressure in her skull is intense. It almost never ceases. In fact, she barely remembers what it’s like to live without it.
But then she pauses, and the smile is clear in her voice. “The possibility of not having headaches anymore is just amazing.”