Curious bug infects tough girl

[Guest guest]

Curious bug infects tough girl

Pulfer Focht/The Commercial Appeal

Hart was able to leave Le Bonheur after two months and nearly

dying from a rare infection. Her mother, Yarbrough, is at

left. Hart family photo

http://www.commercialappeal.com/mca/local/article/0,2845,MCA_25340_50

34153,00.html

Hart, before contracting the rare infection Pythium

insidiosum.Story Tools

commercialappeal.com (subscription) - Memphis,TN

Millington case one of only a few in the world

By Powers

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October 1, 2006

It began as a red bump on her eyelid.

Her mother thought it was a bug bite. When the eyelid began to

swell, her mother gave her some Benadryl.

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Less than two weeks later, a CT scan showed a dark spot behind the

girl's left eye. She was wheeled into a Le Bonheur Children's

Medical Center operating room so doctors could find out what it was.

It would take more than a month to confirm the real problem.

The struggle to save Hart's life would balloon to include

efforts of researchers and laboratories in three cities and two

experimental therapies.

Doctors at the National Institutes of Health and the U.S. Food and

Drug Administration would call Le Bonheur for progress reports, and

churches across the Mid-South would remember the Millington girl in

their prayers.

's case also would force one of her doctors to confront the

possibility the treatment she recommended might kill her patient.

Her family would wonder if they soon would be planning her funeral,

rather than a belated 11th birthday party.

Now doctors say her case is destined for a medical journal.

Always happy

's doctors call her a lovely girl.

She is a patient who thanks her anesthesiologist, downs pills the

nurses hand her, gives away helium balloons to younger patients and,

early in her hospitalization, urged her mother to " stop crying all

the time. "

In a photo her cousin took this summer, 's glossy, dark blond

hair falls below her shoulders. She wears a battered cowboy hat and

denim jacket. Her expression is serious as she gazes straight into

the camera.

is the middle child of Yarbrough, a cook and kitchen

manager, and her ex-husband, Hart, a restaurant manager.

She loves reading, computers and riding her bike.

At her father's Bartlett home, she's a tomboy, playing cowboys and

Indians with her three stepbrothers.

Her grades put her in the top three of her fifth-grade class last

year, and she served in the student council.

" She's easy to please, easy to get along with, " Yarbrough

said. " She's always happy. "

And she's always been healthy. repeats her mother's

observation about her ordeal: " Maybe if it had happened to another

little kid, they might not have been able to handle it. "

A familiar feeling

Dr. DeVincenzo saw for the first time in late July as

she was heading to the operating room for a third biopsy. DeVincenzo

is an infectious disease specialist and an associate professor at

the UT Health Science Center.

The first biopsy had shown the dark spot on the CT scan was a mass

of inflammatory tissue known as an orbital pseudo tumor. The second

suggested a fungal infection.

All the while, was getting worse. Her left eye was swollen

shut and distended. Tissue across the bridge of her nose and her

forehead was swollen and hard to the touch. It didn't have the

squishy feel common to fungal infections.

" I remember thinking this feels like the other kid, " DeVincenzo

said.

The " other kid " was a boy he'd seen several times in the clinic

nearly 10 years earlier. DeVincenzo was still pretty new to the

hospital and the patient was recovering from a rare infection with a

fungus-like organism called Pythium insidiosum.

Like , the youngster was healthy until swelling around his

left eye brought him to Le Bonheur's emergency department. Like

, the swelling spread despite treatment.

It had taken doctors more than two months to pinpoint the boy's

problem. It was another two months before he went home, the first

Pythium patient ever cured with medication.

When DeVincenzo read 's pathology reports listing two molds as

the likely problem, he was skeptical.

" There were too many strange things going on here, " he said. " We see

a lot of eye infections. The feel of this one just wasn't right. "

So the infectious disease doctor and the pathologist looked at the

tissue again. Under the microscope, the organism's branching thread-

like filaments gave it the look of a spindly tree in a stark winter

landscape.

" To myself, I said: 'I wonder if this is Pythium. It's hard to

believe lightning striking twice.' "

Worldwide the medical literature includes only 32 confirmed human

cases of the infection, including 25 Thai patients.

In North America, there were only four, including the Le Bonheur

patient DeVincenzo had seen 10 years earlier.

's third biopsy sample went to four new laboratories -- the

St. Jude Children's Research Hospital lab where the boy had been

diagnosed, Methodist University Hospital, the international fungus

testing laboratory at the University of Texas Health Science Center

at San and the Michigan State University laboratory of Dr.

Leonel Mendoza, an authority on the bug DeVincenzo believed was

making sick.

Experimental treatment

With concern mounting that could lose vision in both eyes and

the infection might spread to her brain, DeVincenzo didn't wait for

the lab results. The next day received the first dose of the

two drugs that saved the toddler's life a decade earlier.

Plants, not humans or animals, usually have most to fear from

Pythium. The bug typically lives among the water lilies and grasses

in the warm, moist, swampy regions around the globe. In the Southern

U.S., it is likely as common as mosquitoes or ragweed.

Mendoza, a microbiologist who has studied Pythium for nearly 30

years, said human cases are so rare, people likely are resistant.

He's developed the only therapy licensed to treat the infection, but

it is approved only for use with animals.

Dr. Arnold, an infectious disease specialist and assistant

pediatrics professor at UT Health Science Center, said they'll

likely never know how contracted the infection. There was no

evidence of immune problems.

Confirmation that was battling Pythium insidiosum came Aug.

25 from Mendoza's Michigan lab.

By then the antifungal drugs that saved Le Bonheur's earlier patient

were being supplemented with Mendoza's animal therapy. was

the first person outside Thailand to receive it.

The hope was the purified Pythium protein would spur her immune

system to launch a more organized response, with white blood cells

made specifically to kill the organism.

That meant the therapy would initially make sicker as new

immune components flooded her body.

" We didn't have a choice, " said 's mother, who agreed to the

step, as did the FDA and the hospital committee that monitors

patient research. By then was so sick her mother told

her: " If Jesus wants you to come live with Him, don't worry about

us. We'll be OK. "

But by Aug. 30, Arnold was questioning the decision. Three weeks

into the experimental therapy, there was no evidence of the desired

immune response.

Even worse, the latest chest X-ray showed fluid in 's left

lung.

Within hours she was in the intensive care unit, the experimental

treatment halted. A ventilator was used to help her breathe.

Yarbrough didn't think her daughter would ever leave the ICU.

Arnold had the same fear. " I kept going over it (the decision to try

the immune therapy) in my mind. Was it the right thing to do, " she

said later.

Meanwhile, the doctors moved rapidly to begin a second experimental

therapy using the drug posaconazole. St. Jude investigators are

studying it to combat infections in young cancer patients.

's condition gradually stabilized.

On Sept. 13, after nearly two weeks on the respirator, pulled

out her breathing tube. She left the ICU a few days later.

Going home

It is shortly before 10 a.m. on Wednesday.

After 10 weeks in the hospital, is waiting to leave. She sits

in her hospital bed, leaning against her father, and fiddles with

her MP3 player. She's been up since 2 a.m., singing along with

Underwood and talking to her aunt.

There is still medicine to be collected and paperwork to sign, but

knows waiting for her at home are her brother , sister

and dachshund Brownie.

Tonight her uncle is cooking a roast with carrots and potatoes. She

has a hair appointment Thursday and a party planned for the weekend.

She'll be back at Le Bonheur for weekly visits. The latest round of

biopsies showed no evidence of an active infection, but treatment

will likely continue for more than a year.

Doctors have warned it will probably take that long before the

swelling is gone. After that, she will probably need reconstructive

surgery, including a prosthetic eye.

She has confided to her aunt and mother that she worries people will

laugh at her.

But on this bright September morning, 's more concerned about

catching up with her classmates at Munford Middle School and getting

the hospital wristbands off.

Both her blue eyes remain swollen almost completely shut, but vision

in her right eye remains 20/20. " I'm just glad I have one good eye, "

she says.

" She knows she came very close to dying, " says Arnold, who doesn't

think they will ever know what finally stopped the infection.

hasn't yet asked to see her face. She volunteers that she

only wants to be friends with those who like her for who she is.

Yarbrough says she's not worried. " People will be nice and

understand. She has good friends. "

-- Powers: 529-2383

How to help

Hart's family has established funds to pay expenses not

covered by TennCare and to fund research into the infection.

Donations can be sent to The Hart Fund at any branch of

Patriot Bank or First Tennessee Bank