By John Kelly
Wednesday, January 7, 2009
We are conditioned these days to regard fat as a bad thing, but the body has all kinds of important uses for it. It's a handy insulator, as any blubbery sea mammal kept warm in a frigid ocean could tell you. Combined with proteins, fat insulates in another way: It comprises the insulating sheath of myelin that covers our nerves, allowing messages to be sent to and from our brain. Doctors call this myelin "white matter" to distinguish it from "gray matter," the neuronal tissue of the brain and spine.
It was this sheath that in the fall of 2007 started to break down in Sean Abercrombie's body. The Frederick 17-year-old had awoken with pain in his neck so severe that his parents took him to the emergency room. There he was given an escalating series of painkillers before his pediatrician ordered an MRI.
"I remember my mom telling me I had myelitis," said Sean, who after the MRI was transferred to Children's National Medical Center.
"Transverse myelitis is part of a bigger category called acquired demyelinating disorders," said Adeline Vanderver, a child neurologist and director of the myelin disorders program at Children's. "The underlying premise is your body all of a sudden has an inappropriate immune response."
Sean had been fighting a viral sinus infection, and it's likely that his body decided to broaden its attack, turning the guns inward in a devastating case of friendly fire. Said Dr. Vanderver: "The myelin disorder actually attacks the cells that make myelin. It's the body's normal immune response that's misdirected."
Dr. Vanderver started Sean on steroids to tamp down his immune system, as well as morphine to dull the intense pain from his haywire nerves.
"The second day I was there, I went to get up and go to bathroom and I couldn't walk," Sean said. "I lost feeling in my legs. I couldn't pick up my fingers. It really scared me."
Said Dr. Vanderver: "Sean got steroids. Then he really wasn't responding that well, so we gave him another treatment that is a little less typical, though now it's used more, called intravenous immune globulin."
Immune globulin latches on to bacteria and other foreign cells so that immune cells can target them. Injecting immune globulin that has been extracted from the blood of many donors swamps a patient's immune system. "There are so many immune globulins that the cells are no longer able to attack those specific cells," Dr. Vanderver said. "They're overwhelmed."
Sean's body stopped consuming its own white matter.
The high school athlete confessed he sometimes felt a bit out of place at Children's. "I was a lot bigger than everybody else around me," he laughed. "But the nurses and everybody were awesome." Sean felt even better when his case manager hooked him up with a laptop and Internet access. "I could update my friends," he said. (He insists he didn't get too specific on Facebook, no "Sean is unable to feel his legs.")
After two weeks at Children's, Sean was discharged. He was able to whip himself back into shape in time to make the basketball team. He's now a freshman at West Virginia University, studying athletic coaching education.
And he was changed by his health scare. "I'm more grateful now," he said. "You never know what's gonna happen. That happened in a matter of like three days. I couldn't walk in a matter of three days."
Doctors aren't sure what causes a white matter disorder such as Sean's. The cause, they say, is multifactorial and idiopathic. "It's just bad luck," Dr. Vanderver said. And good luck for Sean that he was able to seek treatment at Children's.
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