BOTULISM: Rare disease among infants was caught in time to save a life

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Published: June 6, 2009

During the awards banquet honoring her father, Terri Sebelin grew increasingly uneasy. Sebelin, a first-time mother, had her 3-month-old son, Garrett Perschy, in tow, and he was sick. The baby had a slight fever and seemed restless. He was also drooling, which the pediatrician told Sebelin, a registered nurse, meant that he was teething. Sebelin thought that odd because she couldn't feel any tooth buds. Her mother, a retired nurse who had raised seven children, was skeptical, too.

"All during dinner people kept coming up and asking me what was wrong with the baby," Sebelin recalled of the events of the Memorial Day weekend in 1999. She watched closely as friends and relatives passed her son around, noticing at one point that "it looked like they were passing around a rag doll." She tried not to overreact; she had talked to the doctor several times that day and had been assured that the problem didn't sound serious.

A few hours later, after another call to the pediatrician, who instructed Sebelin to take the baby to a nearby emergency room, the family arrived at Lehigh Valley Hospital in Allentown, Pa. Garrett was admitted, and the ER doctor told her he probably had croup. Sebelin and her mother doubted that, too: Garrett wasn't coughing. "I know you don't have croup," Sebelin wrote that night in her journal.

She was right. Less than 24 hours later, her baby was gravely ill, and doctors were frantically ruling out one diagnosis after another. It took an astute specialist to figure out what was wrong, a cause so unlikely that the doctor who made the diagnosis had never seen a case before -- and hasn't since. But diagnosis was only part of the problem. At the time there was only one experimental drug to treat Garrett's illness, and getting it to Allentown required the approval of federal officials. That process would take days -- time the baby clearly did not have.

Ten years later, pediatric neurologist Martha Lusser vividly remembers her tiny patient. Lusser said she believes Garrett's ailment is "clearly less well recognized than it should be" and remains easily overlooked by pediatricians. She is convinced that some fatalities attributed to sudden infant death syndrome were probably caused by the extremely rare problem she diagnosed in Garrett.

Until that weekend, Garrett had been a normal, healthy baby, according to Sebelin, who lives in Palmerton, a small town about 30 miles north of Allentown.

The day before the Sunday banquet, she had noticed he was constipated; she had taken him to a local mall in the morning, where he began to seem out of sorts. She later discovered he was running a slight fever, common when babies are teething. By the time she got to Lehigh Valley Hospital 36 hours later, his fever was gone but he seemed utterly wrung out.

At the hospital, doctors ran some tests and, after listening to his lungs, decided he didn't have croup. The staff thought he might have a virus and told his parents he would probably be discharged the next morning.

By then, Sebelin remembered, he was much sicker.

The hospital staff began an urgent search for a cause. A spinal tap ruled out meningitis. Garrett showed no signs of child abuse, such as retinal hemorrhages or broken bones. Toxicology tests to check for the presence of drugs or poisons came back negative. Both a CT scan and an MRI showed nothing wrong with Garrett's brain, such as a tumor. He no longer had a fever, his blood counts were normal and there were no signs of an infectious disease. One doctor said he suspected Garrett might have a rapidly progressive neuromuscular disorder but had no idea what the disease might be.

"We thought he was going to die," Sebelin recalled. Mystified, the staff called in Lusser.

She examined the baby, noting his floppiness, the way his pupils reacted to light, the reports of drooling and the history of constipation -- the last scarcely unusual, but an important clue.

In Lusser's opinion, all signs pointed to infant botulism, a malady she had never seen in more than 20 years of practice. The only way to be sure was through a stool test that had to be sent to the federal Centers for Disease Control and Prevention.

The more immediate problem was treatment. In 1999, the only drug for infants with the illness was a tightly controlled investigational compound called BabyBIG (Botulism Immune Globulin Intravenous). Garrett's best hope for survival was an immediate transfer to Children's Hospital of Philadelphia, which could get the drug on an expedited basis and had treated other victims of infant botulism. Lusser quickly arranged it, and the baby was whisked away by ambulance, accompanied by his terrified parents.

Infant botulism occurs when a baby less than a year old ingests spores of Clostridium botulinum bacteria, which is present in honey -- the reason it should never be fed to babies younger than 12 months. The bacteria produce a toxin that breeds rapidly in an infant's immature digestive tract, impairing the ability to move, eat or breathe, according to a 2002 article in the journal American Family Physician. (The other form of botulism, which can occur from home-canned foods, affects older children and adults.) BabyBIG, developed by researchers at the California Department of Public Health, was approved by the Food and Drug Administration in 2003 and is administered to about 100 victims of infant botulism each year in the United States.

Although doctors kept asking Sebelin if she or anyone had given Garrett honey, she was adamant that he'd never ingested it. The only other way he could have been exposed was through soil containing the bacteria. Pennsylvania, Lusser knew, is a hot spot for botulism, as are Arizona, California and Utah.

She concluded he was probably exposed when he came in contact with his father, a telephone lineman, before he showered after work, or with his grandfather, an avid gardener.

Lusser said her suspicions the baby had infant botulism, later confirmed by the CDC, were bolstered by the pattern of weakness and droopy eyelid; the drooling, which indicated a loss of muscle control, not an incipient toothache; and constipation, which is among the first signs of the illness.

"Recovery is 100 percent if this is diagnosed and treated early, and babies don't suffer brain damage," she said.

One of Sebelin's most vivid memories occurred in the intensive care unit at Children's Hospital after her son got his first intravenous dose of BabyBIG. "He got it at 10 a.m., and by 1 p.m. he opened his eyes and then raised his little arm," she said.

After eight days, the baby was transferred back to Lehigh Valley Hospital for a week, then discharged.

His extremely unusual illness did not affect his development, his mother said. He recovered fully without incident and recently celebrated his 10th birthday.

"My mother and I both knew something was wrong," Sebelin said. "But we certainly didn't think it was this."



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