TUESDAY, March 4, 2003 (HealthDayNews) -- Smallpox, anthrax, plague, botulism. The words spark considerable anxiety among Americans amid all the warnings about possible bioterrorism attacks.
For those with primary immunodeficiency (PI), a genetic immune system disorder that afflicts more children than leukemia and lymphoma combined, the potential danger looms much larger.
"Regardless of the agent we're talking about, these kids are more vulnerable," says Dr. Allan Lock, director of the developmental immunology program at the National Institute of Child Health and Human Development. "We're talking about an at-risk population."
PI afflicts at least 500,000 Americans, many of them children, but often goes undiagnosed or is diagnosed only after patients become extremely ill or chronically debilitated, sometimes with life-threatening conditions, experts say.
Proper diagnosis is all the more critical in the face of the threat of bioterrorism, according to experts who gathered in Washington, D.C., on March 3 for a conference titled "Primary Immune Deficiency Disorders: New Research and the Threat of Bioterrorism."
The conference, part of an ongoing effort to increase awareness of PI, featured physicians, immunologists, infectious disease specialists and bioterrorism experts.
"We thought it was very important in the context of world events to bring these people together," says Dr. Philip J. Landrigan, the conference director.
"My strong suspicion is most doctors haven't thought about this intersection of bioterrorism and immune deficiency," adds Landrigan, director of the Center for Children's Health and the Environment at Mount Sinai School of Medicine in New York City.
The conference addressed such key topics as ensuring that PI patients are diagnosed and don't receive live-virus vaccines such as the smallpox vaccine. (The smallpox vaccine has been given to health-care workers, but widespread public vaccinations have not begun.)
"The thing we've been concerned about is how many people out there who might not be aware that they have primary immunodeficiency and might go ahead and get the [smallpox] vaccine and could get very sick or even die from it," says Dr. Lisa Kobrynski, a pediatric immunologist at the Emory University School of Medicine in Atlanta.
PI patients also could be threatened by the live vaccine that was given to, say, family members or day-care workers. Patients could be infected by the pustules that form on people who have received the vaccination, Kobrynski adds.
The Immune Deficiency Foundation recommends that any smallpox vaccine programs include questionnaires at vaccination locations designed to identify undiagnosed PI patients and instructions to recently vaccinated people informing them of the potential for the virus to spread to others.
Kobrynski also says anthrax vaccines, which have been given to some military personnel, would not be effective in most PI patients because they don't produce antibodies.
PI patients' parents must exercise vigilance to shield their children from bioterrorism and take steps such as keeping them indoors in sealed rooms if an attack occurs, Landrigan says.
"Parents of children with immune deficiency disorders will need to keep those children under very close cover," he says. "Those children are really going to need very careful protection."
PI includes more than 70 conditions that can be traced to a genetically faulty immune system. Those conditions can range from chronic sinusitis to "Bubble Boy Disease," which leaves the immune system with little or no defenses against infections.
Each type of primary immunodeficiency has somewhat different characteristics and symptoms, depending on the parts of the immune defense system that are compromised. Some deficiencies are deadly; others are mild. But they all share a common trait, experts say: They may pave the way to multiple infections.
Most types of primary immunodeficiency can be diagnosed with a simple blood test, according to the National Institutes of Health.
A child should be tested for PI if two or more of these warning signs are evident: eight or more new ear infections within a year; two or more serious sinus infections within a year; two or more months on antibiotics with little effect; two or more cases of pneumonia within a year; failure of an infant to gain weight or grow normally; recurrent deep skin or organ abscesses; persistent thrush in the mouth or elsewhere on the skin after age 1; a need for intravenous antibiotics to clear infections; two or more deep-seated infections such as meningitis, cellulitis or sepsis; or a family history of immunodeficiency.
The sponsors of the Washington conference included the Center for Children's Health and the Environment of Mount Sinai School of Medicine and the Jeffrey Modell Foundation.
More information
For more on primary immunodeficiency, visit the National Institute of Child Health and Human
Development or the National Primary Immunodeficiency Resource Center.
