By Jim Schutze
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By Lauren Drewes Daniels
By Anna Merlan
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Bacteria associated with meningitis are easily killed with antibiotics, but endotoxin, a poisonous byproduct, is unaffected by antibiotics. A small amount of endotoxin is not harmful to the body. You remove endotoxin from your gums in very small amounts when you floss your teeth. With meningococcal sepsis, an overwhelming amount of endotoxin floods a victim's blood, and the body's natural defenses cannot react fast enough to fight off the effects.
"This bacteria has endotoxin in 100- to 1,000-fold greater excess than other bacteria of its similarity," Giroir says. "It is really the toxin that is produced that causes this horrendous shock state and clotting and heart dysfunction and everything else. It affects all the organs, including the heart and its ability to pump."
What Giroir saw in BPI was a substance that the body already produces to fight infection. It stops bacteria and neutralizes endotoxin.
"What you are really doing is augmenting the body's own ability to deal with disease. Normally you can deal with these things because your body has defensive mechanisms, but when you have such an overwhelming kind of infection, particularly in children, you cannot match the onslaught," Giroir says.
BPI had been in development for 20 years when Giroir saw the poster. Eventually, he would make contact and befriend the man who discovered BPI, Peter Elsbach, a clinical professor at New York University School of Medicine.
Elsbach says early research showed that the white blood cells that help humans fight infection do "subtle damage" to bacteria. Between 1973 and the early 1990s, Elsbach and his colleagues tried to find out what part of a white blood cell actually damaged bacteria. What they eventually found led to their discovery of BPI.
"That was a long time ago, and that search led to the isolation of the single protein," he says.
They cloned the protein, establishing its complete amino acid sequence and demonstrating that one-half of the protein carried all of the anti-bacterial properties.
"That half of the protein, and that is now Neuprex, that half of the protein has the ability to block the activity of what is the main component...that causes many of the symptoms of sepsis, not just sepsis from meningococcemia but sepsis from other causes," he says.
Giroir says what he saw in BPI was a substance that could flood the body with a weapon to attack the poisons produced during the rapid progression of meningococcal sepsis.
"BPI had properties of endotoxin binding and neutralization. It was a protein that your body normally made and circulated and, of course, it made me feel better that it was much less likely to cause a lot of bad effects because you normally run around with it," Giroir says. "So, basically from that moment I made a commitment, and certainly my group made a commitment, to try to get this involved in pediatric trials."
That commitment led Giroir and the staff at UT Southwestern to embark on the study that started with Tashica Jimmerson.
Between 300 and 400 children or young adults die, and about the same number lose extremities to meningococcal sepsis-related gangrene annually. Some also suffer strokes, which cause "neurologic devastation" and "take out half their brain," Giroir says.
Harley Beaty was just shy of her third birthday when the 1999 outbreak struck. Her case is similar to those that doctors such as Giroir must contend with once meningococcal sepsis is diagnosed. Even with good medical treatment, there is often little doctors can do to stop the ferociously rapid progression of the disease. On May 2, 1999, a Sunday, Harley and her family had an outing at Lake Livingston, near their home in Shepherd.
"We were Jet Skiing, boating, swimming. The whole family was there," says Donna Brock, Harley's grandmother and caregiver. "All my nieces, nephews, brothers, sisters were there. Harley was fine."
The next day, Brock says, she woke up at about 5 a.m. and got dressed and ready for work. Harley called to her.
"I went and got her something to drink, and she laid back down and went back to sleep. I thought nothing of it," Brock says. "I went to work."
At the time, Brock worked in Houston, about an hour away. She probably got home about 10 hours later, she says.
"My husband greeted me at the car and I asked him what he was doing at home. He told me that Harley was real sick and he decided to stay home in case she had to go to the hospital. He said she'd been running a fever and been throwing up and had diarrhea. I went into the house and she was lying on the couch," Brock says.
Harley's fever had been going up and down all day, according to Earl Brock, Donna's husband, who had been giving Harley cold baths and fever medicine during the day. She began complaining that her arm hurt, and what looked like a little bruise appeared on her hip, Brock says.