A Scary Diagnosis Hits Home
Tuesday, May 27, 2008
The pink marks that appeared last summer on the back of Elizabeth "BB" Blanchard's left calf looked like three slightly raised rows. Neither the teenager nor her parents thought much about it, because they occurred where a stinging caterpillar had left similar marks a few years before. Bug bites are not unusual in Baton Rouge, where the Blanchards live.
At her school physical, BB's pediatrician examined the area, chalked it up to irritation and provided some lotion to soothe it. That didn't have any effect. The rash grew more prominent and spread behind her knee and toward her foot. In September, Blanchard tagged along when her older brother went to the dermatologist. BB's mother, Anne, asked the physician to check her daughter.
The diagnosis that ultimately resulted -- leprosy -- turned the Blanchards' world upside down and rippled through the lives of many people they knew or had contact with. It also raised issues that are often confronted when any contagious disease is diagnosed, particularly one with scary connotations: What precautions should be taken to protect the rights of the affected individual as well as the health of the community? Where can reliable information be found? This case -- from the Blanchards' initial contact with the dermatologist to reactions of friends and teachers -- reveals the steps that have been accomplished in providing treatment and information and in overcoming stigma.
"It was an unusual rash," remembered Courtney Murphy, the dermatologist. "Usually, you are pretty sure when you see a patient. It might be a little bit of eczema, a little bit of psoriasis. But I was not sure exactly what this was, so I suggested taking a biopsy."
Two stitches and 15 minutes later, Murphy sent a tiny tissue sample to the lab. BB, who is an active dancer and competes on the St. Joseph's Academy swim team, was sidelined from activities for a few days while the incision healed. But she otherwise felt great.
But the seemingly innocuous test assumed more importance a few days later, when her father picked up the lab results from the doctor. He called his wife immediately. "I don't want to scare you," Steven Blanchard said, "but they think it could be leprosy."
"Do people still get leprosy?" Anne Blanchard recalls asking. "I was freaking out and about to start crying."
A.k.a. Hansen's Disease
For the Blanchards, some of the answers lay almost literally in their back yard. Baton Rouge is home to the National Hansen's Disease (Leprosy) Clinical Center, part of the U.S. Public Health Service. Founded in 1894 as a state leprosarium in nearby Carville, La., the center is considered the premier research and treatment facility in the world for leprosy.
About 300,000 new cases of leprosy are diagnosed annually, according to the World Health Organization. Now known as Hansen's disease, after the Norwegian scientist who discovered the mycobacterium that causes the illness, it affects about 2 million to 3 million people worldwide.
Where it is left untreated, Hansen's disease is a leading cause of disability and devastating deformity. It remains endemic in Bangladesh, India, Brazil and elsewhere.
In the United States, roughly 6,000 people have the disease. One hundred to two hundred new cases are reported annually, and, like BB Blanchard, about two dozen of those new patients have never been beyond U.S. borders. About 60 percent of the new cases are found in California, Louisiana, Massachusetts, New York and Texas.
Widely held but inaccurate perceptions of leprosy help fuel the fear and stigma surrounding this biblical disease.
Hansen's disease is infectious but only mildly contagious. About 95 percent of people are thought to be naturally immune to it, according to James Krahenbuhl, director of the Hansen's disease center. The remaining 5 percent who are susceptible must be exposed to the microbe in some way to contract the disease.
How transmission occurs is a mystery. Humans and the armadillo are the only two creatures known to get the disease. No one knows where the microbe hides in nature, although the suspicion is that the leprosy mycobacterium may be airborne like its bacterial cousin, tuberculosis.
Most people think of leprosy as a skin disease. But the rash that BB Blanchard had and the disfiguring lesions often associated with it are just a symptom. The mycobacteria burrow into nerves, where they often remain undetected for years or even decades.
The nerve damage done by the microbes can eventually reduce feeling in fingers and toes. Repeatedly injured because of this limited sensitivity, these digits can slowly resorb, giving the impression of falling off.
Had BB Blanchard's leprosy been diagnosed 60 years ago, her life would likely have taken a far different course. But the drug therapy developed at the Baton Rouge center now effectively treats leprosy, and when the illness is diagnosed early, the terrible complications can be avoided.
Modern public health measures and the ability to get a vast amount of medical information from the Internet are helping to erode the stigma surrounding leprosy and other infectious diseases. The center in Baton Rouge is conducting seminars to better inform the public and the medical community about the disease.
An erroneous report in February by an Arkansas television station shows why that's necessary:
The station aired a story saying that recent cases of leprosy among immigrants from the Marshall Islands could blossom into an epidemic that might affect Americans. The item was later corrected by the television station, which chalked it up to bad information from some local doctors, but not before it was publicized online by the Drudge Report.
The Arkansas Democrat newspaper published a story about the miscommunication and weighed in with an editorial informing readers that the supposed "Great Leprosy Scare of '08 . . . was a rumor/controversy/nonstory."
"I still cannot believe it," BB says of the events of the past nine months.
When the first lab report suggested that she might have leprosy, her mother logged on to the Web. "I looked at every single thing I could find," Anne Blanchard says. "But I didn't tell BB a thing, because we still weren't sure she had the disease."
BB's first biopsy showed classic cell signs of reaction to mycobacterium leprae, but doctors couldn't find any of the disease-causing microbes. That's not unusual for leprosy cases that are diagnosed very early, according to Barbara Stryjewska, the physician who treated BB at the National Hansen's Disease Center.
A second biopsy still didn't find the microbe. "We were baffled and unsure," Anne Blanchard says. The options were to do more biopsies, to simply monitor BB for another six months and then take another biopsy, or to try a month of treatment with the anti-leprosy drugs dapsone and rifampin. If the red rash improved, the diagnosis would be confirmed. The Blanchards mulled the options and decided it was time to tell BB, then 14, that she might have leprosy.
"Mom, how could I ever get that?" BB asked.
The Blanchards opted to try the drug treatment. Anne Blanchard also informed her daughter's dance teacher and swim coach about why BB had been sidelined.
The Hansen's disease center wrote a letter to BB's school explaining that, although she was under treatment for leprosy, she could resume all her usual activities and would not be a risk to anyone else -- standard practice and an attempt to thwart the widely held misconception that leprosy is easily spread.
"I was surprised that the disease was still around," says Linda Harvison, principal of St. Joseph's, an all-girls Catholic high school with 833 students, where BB was just beginning her freshman year.
Harvison sought advice from George Karam, a parent at the school who is an infectious disease specialist at the Louisiana State University School of Medicine in New Orleans. Unbeknownst to Harvison, Karam's daughter is a very close friend of BB's. The two had spent a sleepover together at Karam's house just days before BB was officially diagnosed.
"He told me, 'You don't have anything to worry about,' " said Harvison, who then spread that same message to the faculty and staff. "We informed her teachers because people have this image of this horrible contagious disease," Harvison said.
One faculty member expressed some concern. "But once we reassured her," Harvison said, "she was very, very comfortable with it."
BB's friends and classmates also rallied around her. "They were really supportive," she said. "One of my closest friends Googled and read everything about it on the Internet. Then she said, "Ask me anything about it, because I know all about it.' "
That's a far cry from what used to happen when leprosy was diagnosed. It was common practice just decades ago to expel children from school and burn their papers, desks and books in a mistaken effort to prevent spread of the disease. State and local laws required all those with the disease to be treated at the Public Health Service hospital in Carville, La. People with the disease were removed from their families, their children were taken from them and for decades they were even denied the right to vote.
Karam understands the instinct parents have to protect their children. But as an infectious disease physician, he knows it's important to protect those who are ill, too. That means getting as much information as possible before taking action.
By doing that, he says, the school was able to make sound decisions. "In situations like this, if you can get to the right people, then the job becomes much easier," Karam says. As a result, he says, BB "didn't have to go through a lot of stuff that could have made a frightening situation horrific."
Earlier this month, BB finished taking her medication. The marks on her leg are smaller, smoother and have turned from pink to brown -- confirming that the infection was indeed leprosy and that it was getting better. BB developed anemia, a complication of the medications, that left her tired. But that is now gone. She is a bubbly, vivacious 15-year-old, eager to get on with her life.
"At my last doctor's appointment, I told my mom that I am kind of happy I have this," she says. "I learned a lot about this disease and got more educated about it. I also find it really cool that I am part of the 5 percent of the world that is not immune to leprosy. Why me? I don't know. But it has been a really cool experience having a biblical disease."
Now that final exams are over, BB and one of her friends are looking ahead to next year. They're planning a science project on their new favorite subject: leprosy. "This is the perfect thing to educate my school and everyone about it," BB says. "I have really great knowledge about it now."