Medical Mysteries

A Sore Throat That Turned Deadly Serious

By Sandra G. Boodman
Special to The Washington Post
Tuesday, September 23, 2008

During the worst moments of her ordeal last month, her mouth gaping as wide as possible into an oxygen mask in a labored effort to keep breathing, Nancy Szokan remembers wishing she could just pass out.

"I couldn't stand it anymore," recalled Szokan of the several hours she spent in the emergency room of tiny North Country Hospital in Newport, Vt. "I was that frightened."

Szokan could no longer speak. She could see that her husband was visibly alarmed by the strangulated, high-pitched sound of her breathing. And she was becoming increasingly panicked by the behavior of doctors and nurses who kept trooping in and out of the room to make phone calls or search their computers, apparently stymied in their efforts to figure out what was wrong with her.

"Can't get air!" she frantically scribbled on a tablet.

It took an astute country doctor with a good memory, roused from sleep by a dawn phone call from the ER, to figure out what was causing Szokan's sudden, life-threatening problem. Denise Niemira's instincts saved the 57-year-old Washington Post editor from serious injury or death from a malady that two decades ago was a complication greatly feared by pediatricians. Although the problem is rare among adults, many historians believe it killed George Washington.

Until that night, Szokan's summer vacation had been uneventful. She and her husband, Rick Nichols, his two sons and their five young grandchildren had hiked, swum and hung out at a lakeside cabin in Vermont's remote Northeast Kingdom, near the Canadian border, where the couple had vacationed for years.

On Aug. 6, Szokan had developed a sore throat, which she figured she'd picked up from one of the children. The next evening she had gone to bed at 9, hoping a good night's sleep might improve her throat, which felt increasingly raw.

She awoke at 2 a.m., realizing that her throat had become so painful that swallowing brought tears to her eyes. She got up and tried to take an acetaminophen capsule, but it "shot out of my mouth and across the room," Szokan recalled. The same thing happened with a smaller pill.

Maybe, she thought, infant pain reliever would work. She awakened her daughter-in-law to ask for it but found she couldn't swallow the liquid, either.

Alarmed, her daughter-in-law dialed 911.

The first EMT was on the scene a few minutes later; he lived next door. Others arrived soon afterward and peppered Szokan with questions about bee stings, unusual foods, allergies, new medicines -- anything that might explain her problem. She could think of nothing.

One EMT took her temperature and discovered Szokan had a fever of 102.

"I remember thinking, 'Good, I'm not making this up,' " she recalled. Her throat hurt so much she found it hard to speak and began communicating by scrawling notes.

The medical team loaded her into an ambulance, which bumped over pitch-black country roads alive with deer, bound for the hospital 20 miles away.

"That was the one time I was calm," she said. Doctors, she felt sure, would figure out what was wrong and she'd be on her way back to the cabin.

Szokan's optimism turned out to be short-lived.

The nurses and ER doctor asked her the same questions about allergies, food and drugs. She told them she had been diagnosed with the beginnings of asthma and that a case of acid reflux had once caused similar but much milder symptoms. When they asked her to drink something, she took a few futile sips, then started croaking that she couldn't breathe. That didn't make sense: She had been wearing an oxygen mask.

Suspecting infection, the doctor ordered blood tests, a rapid strep screen and a chest X-ray.

Szokan meanwhile worried that despite her increasingly desperate notes, the staff didn't understand that breathing was getting harder. And why did they keep leaving the room? Only later did she learn that the doctor had been consulting with the ER at the closest large hospital, Dartmouth Medical Center in Hanover, N.H., 30 minutes away by air ambulance, just in case.

The strep test was negative, but the X-ray showed a small spot that looked like pneumonia, so the doctor gave Szokan an antibiotic called Rocephin. A few minutes later, Szokan's breathing changed dramatically; she began making the abnormal, high-pitched sounds called stridor.

"This is new!" she scrawled. "None of the medicines seem to have made any difference at all."

"That's when everyone got tense," her husband recalled, adding that one doctor murmured they "might have done more harm than good." Fearing she might be having a severe allergic reaction to the antibiotic, the doctor quickly administered a cocktail of epinephrine, a steroid and the antihistamine Benadryl to counteract it.

Szokan, meanwhile, was breathing through the oxygen mask, her mouth open as wide as possible, a demonstration of what doctors call "air hunger." The staff was also worried about her elevated heart rate and her increasing anxiety.

Shortly before 6 a.m., the ER doctor called Niemira, the on-call physician, who was asleep at home.

As he recounted Szokan's history and the steps that had been taken, Niemira said her first reaction was that "the story didn't quite add up."

Is she wheezing or stridorous? Niemira asked the ER doctor. When he replied stridorous, Niemira suspected she knew what was wrong.

Her hunch would require one more test: a lateral X-ray of Szokan's neck, which she hoped would confirm her theory. Niemira ordered the X-ray and headed to the hospital.

A few years earlier, Niemira's colleagues at North Country had treated a case similar to Szokan's: A woman had been admitted to the intensive care unit after she developed epiglottitis, a rare bacterial infection of the epiglottis, the small flap of tissue that covers the windpipe and larynx. That case had been diagnosed through a neck X-ray.

Epiglottitis, once relatively common in children, whose airways are smaller than those of adults, can be rapidly fatal. If not treated promptly, infected tissue becomes swollen and obstructs the airway, making it impossible to breathe. Before the advent of the hemophilus influenza vaccine in the mid-1980s, which has largely eradicated epiglottitis in children, the infection was a feared complication of strep throat and other respiratory diseases in children younger than 5.

These days, its victims tend to be adults. The most famous is said to be Washington, who died in 1799 at age 67 at Mount Vernon, two days after developing a sore throat.

Stridor is characteristic of epiglottitis, as is fever, air hunger and an elevated heart rate -- all symptoms Szokan was exhibiting.

When she got the call about Szokan, Niemira said, she remembered that earlier case at her hospital.

"I hadn't treated this woman, but it was one of those little facts you tuck away somewhere. When you're in a small hospital and you have an unusual patient in the ICU, people talk about the interesting case."

Szokan's X-ray was definitive: Her epiglottis was swollen.

The next task was treating her quickly. She would need to be intubated and placed on a ventilator that would breathe for her while antibiotics and steroids knocked out the infection and shrank her epiglottis. Doctors warned her husband that inserting a breathing tube was not without risks, because it could trigger a spasm that could close off the airway.

Szokan awoke 36 hours later with a sore throat ("the good kind" she said), the result of having been on a ventilator. Her fever was gone; she could breathe and swallow. She has since fully recovered.

One of her first visitors was Niemira, who told Szokan she had probably contracted a staph or strep infection that had settled in her epiglottitis, an occurrence so rare Niemira compared it to getting struck by lightning.

"If I had not tucked away that little fact, I'm not sure it would have come into my consciousness," the doctor said. "I just sort of lucked out."

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