Watching Beyoncé and Jay-Z rock Gillette Stadium July 1, 2014, Dana Kimmelstiel, 19, felt nothing when a stiletto-wearing concertgoer’s heel trod directly atop her foot. It wasn’t simply that she was enrapt by her favorite artists—it was the final straw tossed on a weeklong stack of puzzling symptoms, confirming something was seriously amiss.
The next morning, she checked into Tufts MC’s Michael Neely Neuroscience Center for a series of tests to diagnose her situation. Otherwise the picture of health, Dana’s parents—and even Dana herself—were initially hard-pressed to think such a step would be remotely necessary.
“Probably a week before everything got really bad,” recalls Dana, “I woke up, and I could tell that something was wrong.” She had lost sensation in her feet—more than just pins and needles. “I thought I slept weird. I thought I could walk it off,” she says. Later, running water in the bathroom sink “felt like it was burning my hands.” Her mom tested the water and deemed it not too cold, not too hot. They both shrugged it off.
That afternoon, out walking with friends, Dana couldn’t keep up and told them to slow down. They told her they were walking at a normal pace. Her father, Dr. Carey Kimmelstiel, Director of the Interventional Cardiology Center at Tufts MC, suspected this may have been due to a delayed peripheral nervous system reaction to her 103-degree fever and virus a few weeks prior which should resolve on its own.
Several days later, her symptoms continued, and then some. “I realized I was dragging myself upstairs—dragging my arms up the railings,” says Dana. “I think I had adjusted myself to look normal [walking] on flat ground,” but ascending a staircase shone a spotlight on the problem. Her mom called the pediatrician, who was unconcerned overall.
So Dana worked her hostess shift at a nearby restaurant that evening, but “it felt like my legs were full of lead—like they weighed 300 pounds each.” In light of all this and the spike-heel concert incident, the Kimmelstiels agreed it was time for a doctor visit. Dr. Kimmelstiel made arrangements for Dana to see colleague Dr. David Thaler, Tufts MC’s Neurologist-In-Chief.
Dana was glad to go in when she did. “It had gotten really bad when I woke that morning,” she says. “I had the same numbness, [but now] up higher on my legs. I didn’t have any feeling in my calves at all, all the way up to my knees. I had a huge gait change—almost how a toddler looks when learning to walk, wobbling from side to side.”
During Dr. Thaler’s 45-minute neurological examination, “I realized I was definitely much sicker than I thought I was, because things that should have been easy weren’t.” Dana’s reflexes failed to respond to standard taps, she was unable to push her legs and feet when asked, and “I couldn’t feel him touching my legs at all.”
Thaler ordered further testing for Dana, suspecting she had developed Guillain-Barré Syndrome (GBS)—a rare yet typically passing inflammatory disorder in which the immune system attacks the protective coating of the peripheral nerves, thereby preventing appropriate communication with the muscles. GBS affects perhaps 1 in 25,000 people, and can strike at any age.
Results of an electromyography, or EMG, assessing the health of Dana’s muscles and muscle-controlling nerves, suggested demyelination, or damage to her nerves’ protective coating—consistent with GBS. Inspection of spinal fluids extracted from a spinal tap revealed increased protein without an increase in the white blood cell count—also consistent with GBS.
A series of spine-examining MRI (magnetic resonance imaging) tests ruled out spinal inflammation, thereby excluding any possibility of multiple sclerosis and other central nervous system (CNS) disorders. “Guillain-Barré is usually monophasic, which means it happens only once, and is a much better diagnosis and prognosis” than often-degenerative CNS diseases.
Dana explains her GBS like this: “The antibodies my body had created to fight off the fever I had gotten two weeks before had gone AWOL and were attacking my nerves. My nerves were basically just dying from the outside in. There’s no way to stop it—it’s an autoimmune disease.”
Thaler concurs, and adds: “When you get a bug—a virus—your body fights it off with your own immune system, which creates antibodies and activates white blood cells.” With GBS, the immune response mistakes a person’s nerve sheathing (myelin) for a foreign object, and induces inflammation.
“In time, the peripheral nerves restore themselves,” he continues, “somewhere in the order of weeks to months. The treatment is time. Somehow, the body figures out it should stop attacking itself, and it does.”
Therefore, Thaler explains, “the ultimate [degree of] improvement is not usually affected by the treatment,” which is instead designed to accelerate the healing process. Most GBS “patients reach their nadir (the worst they will be) within a couple of weeks, and then they spend weeks or months improving”—hopefully, but not always, returning to normal.
“The inpatient stay is only for the treatment, and once it is finished, the patient can go home,” he says. Accordingly, Dana quickly completed her prescribed IVIg, or intravenous immunoglobulin series, and just five days after her admittance to Tufts’ NeuroIntensive Care Unit, was released with an “excellent prognosis.”
A Neurological Revolution
Positive post-recovery prospects are common for today’s GBS patients, but years ago, “a GBS diagnosis was grim. The fault lay in the inadequate care available for neurological patients,” Thaler explains. The development of NeuroIntensive Care Units and their round-the-clock, “detailed care of the neurologically injured patient” changed all that.
Case in point: Tufts’ Michael Neely Neuroscience Center, which, incidentally, boasts “the only Gamma Knife in New England. It’s the newest unit in town, opening in 2011,” says Thaler. “It is because of detailed neurological intensive care like ours that so many GBS patients today get better.”
Dana certainly felt these benefits. “I could not have asked for a better team of people,” she says. “They never treated me like a patient because they knew I didn’t want to be treated like a patient—because I didn’t feel like a patient. I just wanted to go back to school and go back to being a normal 20-year-old.”
“I really felt like I was being cared for in the best way possible. The staff was great. I loved the nurses so much,” she adds, particularly citing a nurse named Dani, who helped her feel like her normal self throughout her stay. “It’s a really hard feeling to describe—that you’re totally there mentally, but your body is just giving up.”
Following her discharge, Thaler prescribed physical therapy, a return to normal activity in keeping with Dana’s comfort level, and, after one follow-up visit—satisfied that the GBS was abating as expected—Dana recalls he joked, “‘I don’t ever want to see you again unless you’re coming to visit me for fun.’”
Though Dana left the hospital with a substantial limp, by the time she returned to The George Washington University in August, it was gone. She listened to her body as she eased back into her routine as a member of GW’s dance company. “Now I feel like I’m totally back,” she asserts. “I’m stronger than I was before it all happened. And I was in the Nutcracker this past Christmas.”
Paying It Forward
But Dana’s story has an even happier ending—a potentially everlasting ripple effect. For the remainder of the summer of 2014, Dana found herself reflecting on her GBS battle at Tufts MC. “The nurses and doctors kept asking if I was pre-med or in nursing school because I was so interested in what was going on,” she remembers.
In time, she realized the emotional side of being a patient had left the most indelible mark of all. Upon returning to school, she signed up for a psychology course, after which she promptly declared herself a psychology major. “I realized I loved it,” she says, and spent the summer of 2015 as a cognitive psychology intern.
In the end, Dana’s temporary GBS and her inpatient experience at Tufts MC resulted in a permanent, positive effect on the course of her life and potentially the lives of many others. “It pushed me in this new direction of wanting to work with patients and help people,” she says.