The afternoon she walked into the office of internist Wayne Meyer, Deborah Kaplan had never felt worse. Hours earlier Kaplan had been sent home from a Maryland emergency room with a diagnosis of chickenpox.
She was too sick to cool her heels for the customary 2-1/2 hours in the waiting room of her regular physician, and a family friend had recommended Meyer. Kaplan knew she had to get help for the horrible itching sores that blanketed her face and body and were invading the inside of her mouth.
Thirteen years later, Meyer said, the image of his new patient, then 30, remains vivid.
"It was horrendous," he recalled, adding he hadn't seen anything like it before or since. "She was quite ill, but the thing about Debbie is that she always looks put together." He'd seen chickenpox in adults, Meyer said, and "this didn't look like that. But I wasn't exactly sure what it was."
Hunch was right
In the end, the doctor's hunch about what was wrong turned out to be right. Kaplan's illness, triggered by an action repeated every day by tens of millions of Americans, is so rare many doctors never see a case. For both physician and patient, the arduous days that followed proved to be a crucible that tested them in different ways and continues to resonate.
Kaplan, who lives in Germantown, Md., and works as a manager of an architectural firm, was generally healthy and rarely went to the doctor. A few weeks before she saw Meyer, she had visited a walk-in clinic because of a bothersome cough. A nurse told her she probably had bronchitis; Kaplan was given a prescription for Bactrim, a widely used and inexpensive sulfa antibiotic.
Kaplan, who'd used the drug before, took it for 10 days as prescribed and felt much better. "I had no upset stomach, no problems," she recalled. "I thought the next time I needed an antibiotic I would certainly ask my doctor for this excellent medication."
A day after the last dose, Kaplan noticed several itchy bumps on her legs and thought it was the recurrence of hives she'd had as a child. Later that night, she discovered the bumps were spreading. "The itching was so bad it felt like poison ivy all over my body, inside and out," recalled Kaplan, who had developed a fever. "I couldn't sleep at all."
At 8 a.m. the next day, a Sunday, Kaplan dragged herself to a drugstore near her home. Her face was red and somewhat swollen; the pharmacist sent her to a clinic down the street. A doctor there diagnosed an allergic reaction, possibly to the Bactrim, and gave her a prescription for the corticosteroid drug prednisone, which is used to treat severe allergies.
About 12 hours later, a friend took her to the emergency room of Shady Grove Adventist Hospital. "By this time it felt like bugs were crawling under my skin," Kaplan said. The sores, which seemed to be everywhere, were oozing. Her eyes were puffy, her vision blurry and urinating was painful. Eating was out of the question: The inside of her mouth had been colonized by painful canker sores. "It was gross," she said.
The emergency room doctor asked if she'd ever had chickenpox. Kaplan said she told him she was pretty sure she had. He conferred with two other physicians, she said, who seemed mystified by her illness and suggested it might be a drug reaction. But the doctor in charge was adamant: He told her she had chickenpox, which can make adults very ill. She should stop taking prednisone, which can worsen the infection, he added. When she tearfully begged for something to control the itching, he gave her a shot of the antihistamine Atarax and sent her home.
The next morning, after another wretched, sleepless night, she made an appointment with Meyer.
Although her high fever suggested chickenpox, the skin lesions looked somewhat different, Meyer recalled. An extremely severe case of mononucleosis could cause the blisters, he thought, and there was always the possibility of an unknown infectious disease. But Meyer believed the most likely culprit was a rare, life-threatening allergic reaction known as Stevens-Johnson syndrome, or SJS.
Stevens-Johnson, which attacks the skin, internal organs and mucous membranes, is most often caused by an allergy to sulfa, but other drugs, including anti-seizure medications and even certain NSAIDs, can cause it. Between one and five cases per million are believed to occur; as many as 40 percent of cases are fatal. Prompt treatment in a hospital, sometimes a burn unit, is usually required.
As with burn victims, treatment involves trying to prevent dehydration and organ failure, and ensuring that any skin infections are treated rapidly. No one knows why some patients, Kaplan among them, develop a violent allergy to a drug they have previously taken with no problem.
Meyer put Kaplan back on prednisone and sent her home with an agreement that she would call him twice a day. He told her she could stay out of the hospital as long as she could drink. "I probably should have hospitalized her, but she was still managing," he said. "That didn't last long."
Less than six hours later, after her fever spiked to 104.4, Kaplan's mother drove her back to Shady Grove. Unsure at first whether she was contagious, doctors and nurses donned gowns and masks and whisked her to a private room.
The next six days were a blur, Kaplan recalled. She was given high-dose steroids intravenously, along with a non-sulfa antibiotic to prevent infection, and was fed intravenously because she had trouble swallowing.
Meyer remembers initially wondering whether Kaplan would survive. "The uncertainty was scary, and I worried about whether I was doing the right thing," he recalled. He saw her three times a day and began to relax only when her skin started to heal after several days. In addition to her skin lesions, Kaplan developed eye problems — corneal scarring and even blindness is a risk of severe Stevens-Johnson — as well as heart palpitations; her liver and colon were also affected.
Kaplan said she was unaware of how dangerously ill she was. She knew she was a novelty because nurses trooped into her room saying, "Are you the SJS patient? I've never seen a case before."
"At the time I was more irritated" than frightened, she recalled. "Dr. Meyer told me, 'Everyone was scared except you.' "
That came later. A year after her illness, Kaplan said, she developed severe panic attacks — something she said she had never experienced. Meyer, who remains her internist, said he believes they are a post-traumatic stress reaction. Kaplan said they have largely receded.
No permanent damage
She still has trouble tolerating heat or sun on her skin, and her eyes are more sensitive than before, but she suffered no permanent physical damage. "I was very lucky," she said.
Since then she has taken an occasional antibiotic, but only when absolutely necessary — and always with trepidation. "I was terrified the first time I had to take one," she recalled.
Meyer said that with rare exceptions, he no longer prescribes sulfa drugs because other alternatives abound. "After seeing someone like Debbie Kaplan, it's real hard for me to prescribe it," he said.
The irony, Meyer said, is that she almost certainly did not need the drug in the first place.
"Ninety-nine percent of bronchitis cases don't need an antibiotic," he said. "It just kills me that it caused this."