By Sandra G. Boodman
Special to The Washington Post
Tuesday, September 8, 2009
It was the middle of the night when 4-year-old Brooke Dawn suddenly awoke in her Northern Virginia hospital room shrieking, "Ow, ow, ow!" But it was what she said next that her mother, Patricia Dawn, says she will never forget.
"Mommy, it hurts so bad I can't stop screaming," said the normally self-contained little girl.
"That's when I realized how bad things were," Dawn recalled.
By then Dawn and her daughter had spent several days at a Northern Virginia hospital, as doctors tried to figure out what was causing Brooke's high fever, sore throat, red lips, stiff neck and headaches so severe they sometimes left her writhing in pain. Baffled by the constellation of seemingly unconnected symptoms, specialists had ruled out meningitis and juvenile rheumatoid arthritis. Brooke had been given intravenous antibiotics for a possible throat infection and had developed a severe measles-like rash that blanketed her torso and limbs. Doctors worried she might be in the throes of a dangerous drug allergy.
In the end, what was wrong with Brooke turned out to be none of those things. A week after she became ill, an infectious-disease specialist made the correct diagnosis. Treatment was begun swiftly, in an effort to avert potentially lethal complications that might not surface for years.
"She was diagnosed in time," Dawn said. "Others are not so fortunate. I often wonder what would have happened had we not fought her discharge from the ER."
On Oct. 27, 2008, Brooke and her parents had just returned from two weeks in Florida. The trip, which included a visit to Disney World, had been a treat to help Brooke cope with her mother's protracted hospitalization and recovery from a ruptured neck disk. "She was very traumatized because I was bedridden for months" unable to care for her, Dawn said. The Florida trip had been a big success.
The night the family returned, Brooke woke up crying at 11 p.m., clutching her head and complaining of a bad headache. She had no fever, and her mother gave her Tylenol for the pain, which seemed to work.
Brooke seemed fine the next morning, and her parents chalked the headache up to sinus problems, a frequent occurrence. But after preschool she seemed lethargic and soon spiked a high fever. The headaches returned and at times were so severe Brooke was writhing in pain. Her parents kept giving her over-the-counter pain relievers, which didn't help much.
The next morning she developed a new and more worrisome symptom: The right side of her neck hurt and seemed rigid. Alarmed because she had had meningitis at 14, Patricia Dawn immediately took her daughter to a pediatrician, who performed a strep test and took some blood. The doctor decided meningitis was unlikely and believed Brooke, whose white blood cell count was elevated, might have contracted a virus.
That night, after she seemed unable to move her neck, Brooke's parents took her to an area emergency room. A CT scan showed a swollen lymph node in her neck and an infection behind her throat. Doctors gave her intravenous antibiotics and told her parents she might have nicked the back of her throat with a sharp straw. At 2 a.m. they told the Dawns to take Brooke home.
"I pitched a fit," said Dawn, a former hospital chief financial officer who has many relatives who are physicians. Brooke, she insisted, was too sick to be discharged. The little girl was admitted.
She was no better in the morning, even though a second antibiotic had been added. An ear, nose and throat specialist told the Dawns that if Brooke did not improve, surgery would probably be necessary to drain a possible abscess.
The next day was Halloween, Brooke's favorite holiday, but she showed no interest in any of the hospital-sponsored festivities, further alarming her parents. At this point, her mother said, Brooke had stopped eating and drank little. Her neck was so painful that the only thing that helped was packing it in ice. At the Dawns' request, an infectious-disease specialist was called in; he agreed it was a probable infection and adjusted her medications.
The drugs seemed to help, and that evening Brooke started to feel better. But her parents' relief was short-lived. In the morning, she awoke with a red rash covering her legs and groin. Worried that she might be allergic to one of the antibiotics, as she is to penicillin, doctors prescribed an antihistamine to counteract the possible allergy.
The rash got worse, spreading to her hands and feet, which began to swell. Brooke's lips turned red, as did her eyes. Doctors worried she was developing Stevens-Johnson syndrome, a life-threatening drug allergy that can cause a similar rash and redness.
Concerned that a throat abscess might have become walled off, preventing antibiotics from reaching it, the ENT specialist recommended surgery.
By that point, the fifth day of Brooke's hospitalization, the infectious-disease specialist was reconsidering. Suspicious of the red eyes and red lips, which are not typically associated with a drug allergy, he decided she might have Kawasaki disease, an unusual cardiac problem.
Hours later, a pediatric cardiologist, concurred. Blood tests showed elevated levels of C-reactive protein, and other lab tests were consistent with Kawasaki. All of Brooke's symptoms, except the headaches, were symptoms of the disease.
There is a narrow window for treatment to prevent cardiac complications -- within 10 days of the initial illness -- and eight days had already elapsed. Doctors began intravenously administering a blood product called IVIG, which has been shown to reduce heart attacks, coronary aneurysms and sudden death in Kawasaki patients. Without treatment, the disease may disappear on its own, but 25 percent of its victims will eventually suffer sometimes fatal heart attacks and aneurysms, often in adolescence.
Named after the Japanese physician Tomisaku Kawasaki, who first described it in 1967, Kawasaki disease is a severe inflammation of the blood vessels; its cause is unclear. It is most common among children of Asian, especially Japanese, ancestry; more than 2,000 cases are diagnosed annually in the United States. Among them was John Travolta's 16-year-old son, Jett, who died last year after suffering a seizure; he had received a Kawasaki diagnosis as a toddler.
Eighty percent of children found to have the disease are younger than 5, although rare cases can occur among teenagers and even young adults. There is no single diagnostic test for the disorder, which mimics other ailments, including measles, meningitis, Stevens-Johnson syndrome and juvenile arthritis, according to a 1999 article in the journal American Family Physician.
The infectious disease specialist in Brooke's case declined to be interviewed, but Candace Gibbin, a pediatric cardiologist who specializes in treating Kawasaki at Johns Hopkins Hospital, said that drug allergy is "a very common red herring." The disease, she added, is often missed by physicians, despite guidelines to improve its detection promulgated in 2004 by the American Heart Association. Those guidelines say that doctors should consider Kawasaki in children with an unexplained fever lasting five days, when accompanied by other symptoms including red eyes without discharge, red lips or mouth, a rash and a swollen lymph node in the neck.
"Red eyes and red lips are an unmistakable sign of Kawasaki disease," Gibbin said.
"The reason people miss the diagnosis is that they don't sit down and put the signs and symptoms together," Gibbin said. "You have to have somebody leading the wagon train who can come in and synthesize what is going on."
Although Brooke improved after her first dose of IVIG, she required a second dose a few days later when some of her symptoms returned. After nearly two weeks in the hospital, she was discharged.
She continues to be monitored closely by a pediatric cardiologist and so far shows no signs of heart damage. But her ordeal continues to have psychological reverberations.
"She had a tough time in the hospital. It was such acute pain, and they were constantly drawing blood," her mother said. "She'd never been afraid to go to the doctor before, but now if we're heading for an appointment she's always asking, 'Do I need an IV?' "
As her mother discussed her illness, Brooke and a friend played "hospital." Their patient was the family dog. He was being treated for Kawasaki.