Within days of developing a viral infection following a routine ear piercing, a 15-year-old British dancer lay paralyzed in a hospital bed, unable to move or communicate except by blinking her eyes at her devastated mother. Her diagnosis: Guillain-Barre syndrome, a rare and potentially deadly immune disorder that attacks the nervous system.
Doctors at Evelina Children's Hospital in London said that Grace Etherington might
remain motionless for the rest of her life, dependent on a ventilator for every breath.
"It was a fate worse than death. She would be trapped in a lifeless shell," her mother, Sharon Etherington, 41, of Sittingbourne, Kent, told The Daily Mail.
"I wanted to tell mum I loved her and I could only do it by blinking," the now-healthy teen told the newspaper. "Being stuck in my body was horrible."
Grace defied the dire predictions rendered last November, the British newspaper reported. She took her first steps in December and by March, intensive physical therapy had her walking again. In May, she achieved her dream of dancing with her troupe in London. With the exception of some ongoing fatigue, she has recovered completely.
Grace's story is exceptional on many levels. Through an obscure route, this young, otherwise healthy young woman contracted a rare disease affecting one in 100,000 people worldwide. Guillain-Barre can affect anyone at any age; about two-thirds of cases originate in the immune system. But Dr. Kenneth C. Gorson, a neurology professor at Tufts University School of Medicine and neuromuscular specialist at St. Elizabeth's Medical Center in Boston, said Wednesday he'd never heard of a case triggered as hers apparently was.
"This would be an extraordinarily unusual complication of ear piercing and a skin infection causing Guillain-Barre syndrome," Gorson said.
Doctors unfamiliar with the disorder would have been unlikely to suspect Guillain-Barre when Grace first showed symptoms. Veteran neurologists would likely have seen her illness taking a fast, familiar course. A few days after the ear piercing, Grace developed a viral ear infection. According to what her mother told the newspaper, a doctor dismissed the tingling in her toes as nothing. But Guillain-Barre typically begins with weakness or tingling in the feet or hands.
When Grace began to experience difficulty walking a few days later, her mother took her to a nearby hospital. During her overnight evaluation, Grace stopped being able to move her legs, the Mail reported. Then one side of her face became numb. Next, she began choking, so doctors placed her on a ventilator to regulate her breathing after muscles of her diaphragm became paralyzed. About 20 percent to 30 percent of adults will end up on a ventilator, Gorson said, and the pattern in a teenager would hew more closely to what happens in an adult than a child.
Grace was transferred to the London hospital, where specialists recognized the progressing paralysis of Guillain-Barre, which remains incurable more than 150 years after it was described. In 1859, Jean Baptiste Octave Landry de Thezillat reported the 10 cases of what he termed ascending paralysis, so called because it often begins in the feet, although it also can descend from the cranial nerves of the head. In 1916, three French physicians, Jean-Alexandre Barre, Georges Guillain and Andre Strohl (whose work wasn't acknowledged at the time) made a scientific presentation on what later came to be called Guillain-Barre syndrome.
The autoimmune disorder most often follows viral maladies such as upper respiratory infections, or diarrheal infections. More rarely, it's set in motion by surgery, trauma or pregnancy. But the condition may be best-known as a side effect for hundreds of swine flu vaccine recipients in 1976. Although Guillain-Barre remains a documented risk of vaccinations, it produces one extra case for every million people vaccinated for the flu, Gorson said. People are far more likely to get it as a complication of the flu than from the vaccine to prevent the flu, he said.
There is no known cure for Guillain-Barre, although two standard treatments can hasten recovery by 50 percent, Gorson said. Most doctors try a five-day course of intravenous immunoglobulin infusions, which weaken the body's attack on itself, because they're simpler to administer. Plasma exchanges require a central line to remove blood from the body, take out the plasma and then return the red and white cells, and this poses a higher risk of infection.
Extensive paralysis has been well-described in medical literature as a complication of very severe Guillain-Barre. In some cases, "you can be completely paralyzed such that you are in a locked-in state," Gorson said. About 5 percent of cases end in death. The more extensive the symptoms, the less likelihood of full recovery, but 60 percent to 80 percent of patients do recover well.
The good news is that the peripheral nerves that are attacked by the disorder have the ability to regain their protective myelin sheaths and regenerate. Recovery, even if slow, can continue up to four years after the illness takes its neurologic toll.
Although there are interesting experiments being conducted on animals, a check of the National Institutes of Health's clinical trial listings finds none on Guillain-Barre. However, Dutch researchers have been experimenting with a second IVIG, or intravenous immunoglobin, course for patients who don't respond to the first. That trial is expected to be expanded into a multi-center international study within the next year.