Sandi's story

    Chiari malformation

undiagnosed

Sandi fondly recalls the moment when she really knew her health had been transformed by the care she received at Mayfield. She was in her back yard, tossing a nerf ball to her young son when a happy thought occurred to her. "A year ago I couldn’t do this without getting a headache," she said aloud. Her husband, watching with a smile, amended that. "Ten years ago you couldn’t have done that without getting a headache."

Like so many people who have suffered from Chiari malformation, Sandi coped with her symptoms for many years. For most of that time, however, Sandi didn’t realize that she had a medical condition that could be treated. Chiari malformation is a condition in which the lower part of the brain, called the cerebellum, herniates through the skull and down into the spinal canal.

"Looking back, I’d had headaches ever since I was a teenager," says Sandi. "I noticed it more when I was doing something active, like playing softball or basketball, an activity that had a repetitive motion. Later, while I was in nursing school, I thought there was something wrong with me. But of course when you’re reading about all of these conditions you tend to think they’re all happening to you. I thought I was being silly and didn’t do anything about it. Over time, I adapted to it. I just didn’t do things that precipitated a headache."

Her ability to cope with the symptoms began changing after the birth of her second child. When a particularly bad headache struck, she would have to lie down. While at work one day, the discomfort became unbearable. "I hadn’t felt good that morning, and at work I was sick to my stomach and had a really bad headache," she recalls. "Then it got worse. What really hit me was my vision."

While trying to fill out paperwork, Sandi stared at what she knew was a single block and saw three.

That night she ended up in the emergency room. A CT scan revealed that her sinuses were fine, and a lumbar puncture was normal, showing no signs of suspected meningitis. The next day her primary care doctor ordered an MRI, which might have raised alarms but didn’t.

"He said the only unusual thing on the MRI scan was a Chiari malformation," Sandi says. "He said he didn’t know what that was so he had to look it up. He called a neurologist, who told him that most Chiari malformations are asymptomatic. He suggested we revisit it six months from now if I was still having problems."

untreated

But Sandi was having difficulties right then and there. She still felt significant pressure in her head, behind her ears. "He looked in my ears and told me I had water in my ears and told me to take a decongestant every day."

Sandi’s most severe symptoms resolved, but she continued to feel pressure in her head. She sought a referral to an ear, nose and throat specialist at The Christ Hospital, and a few weeks later she brought her MRI films to the office of Michael Wood, MD.

Dr. Wood looked in Sandi’s ears, assured her he saw no lakes or streams or even a drop of water, and then talked to her about Chiari malformation. A week later, he arranged for her to meet Robert Bohinski, MD, PhD, a neurosurgeon with Mayfield.

validated

Dr. Bohinski needed only one look at the MRI to observe that Sandi had "a significant Chiari."

While the average Chiari herniation is about 8 to 10 millimeters, Sandi’s cerebellar tonsils extended 25 millimeters into her spinal canal. Fortunately, Sandi did not have a syrinx, a fluid-filled cyst within the spinal cord that, over time, can damage the cord.

"To me, Sandi’s case seemed very clear cut," Dr. Bohinski recalls. "After seeing her scan and hearing her description of the headaches she was suffering from, I knew she was someone who could benefit from the surgery. She had substantial tonsillar herniation and manifested the classic syndrome of occipital pressure headaches made worse by strenuous activity."

Another symptom also intrigued Dr. Bohinski. Sandi suffered from severe sleep apnea, a condition in which a person experiences chronic interruptions in breathing while sleeping. Sandi had been prescribed a CPAP (continuous positive airway pressure) ventilator to help her sleep, a turn of events that she described as "traumatic for a 34-year-old" because the ventilator was "not very attractive and difficult to wear."

Dr. Bohinski wasn’t sure whether Sandi’s sleep apnea would improve with surgery. But he did observe that "she did not seem to fit the classic picture of someone without a Chiari malformation who suffers from that condition."

Dr. Bohinski ordered an additional MRI and a flow study to determine the extent to which the malformation was obstructing Sandi’s cerebrospinal fluid, a clear, watery-like liquid that flows within and around the brain and spinal cord. The obstruction, the test revealed, was almost complete.

One month later, in October 2010, Sandi underwent surgery. Dr. Bohinski performed a craniectomy, opening a hole in the skull to make room for the tonsils, and then performed a partial laminectomy at C1-2 and C-2 (the first two vertebrae in Sandi’s neck), because the tonsils were herniated down to the level of C2. During the partial laminectomy, Dr. Bohinski says, he was careful to preserve the critical stabilizing attachments between the vertebrae.

Dr. Bohinski warned Sandi that she would have pain following the surgery, and his warning proved correct. Nevertheless, Sandi went home three days after her surgery. She was on pain medicine round-the-clock for two weeks, but by week six she was back at work.

recovering

Today, Sandi is free of her Chiari-related symptoms. Her cerebrospinal fluid flow is normal, and her pressure headaches in the back of her head are gone. Her severe sleep apnea has also vanished.

"It was very rewarding to hear from Sandi that her sleep apnea had completely resolved by 6 months after her surgery," Dr. Bohinski notes. "For Sandi, this freed her from lifelong dependence on wearing a CPAP ventilator at night to sleep."

Most importantly, Sandi is no longer fearful.

"With Chiari you can become paralyzed if you go untreated, so I’m glad to not have to think about that," she says.

Her life is full and busy with her family and nursing career. Her fitness routine includes a low-impact Xbox workout and bike riding.

"I’m grateful for the resources I had, and I feel bad for patients who don’t have all those resources," Sandi says. "It takes years for some people to be diagnosed, and, considering everything, I was diagnosed early after I had a major episode. Most people aren’t diagnosed until their 30s because their symptoms are vague or similar to other problems. Lots of people are diagnosed with fibromyalgia, multiple sclerosis, or psychiatric issues. And, as my own experience showed, many doctors don’t know about Chiari.

"If you have the blockage and the symptoms, you should do your research when you look for a surgeon, and then you won’t have to be afraid," Sandi says. "Looking back, I would definitely have the surgery again."

"I’m grateful for the resources I had, and I feel bad for patients who don’t have all those resources," Sandi says.

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