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d by Immigrants, Leprosy in Heartland Surprises Clinicians

By , North American Correspondent, MedPage Today

Published: December 29, 2008

Reviewed by Jasmer, MD; Associate Clinical Professor of Medicine,

University of California, San Francisco

http://www.medpagetoday.com/InfectiousDisease/PublicHealth/12284

SIOUX CITY, Iowa, Dec. 29 -- The man from Guatemala was admitted to

Mercy Medical Center here with a fever of 109, swollen lymph nodes, and

enlarged liver and spleen.

Initially, his doctors suspected lymphoma or some other form of cancer,

says Bertha Ayi, M.D., an infectious diseases specialist, but they were

ruled out, one after another.

Dr. Ayi was called in to check for tuberculosis, but it took her almost

no time to rule that out as well and determine the correct diagnosis,

one that would not be expected in Iowa.

Action Points

* Explain to interested patients that Hansen's disease -- more

commonly known as leprosy -- is not easily transmissible and is readily

cured with antibiotics if it is diagnosed early.

* Note that the incidence of leprosy in the U.S. is stable, but the

pattern is changing, with new cases now being seen in places such as the

Midwest, where the disease has been rare.

" It didn't take more than five minutes to see the gentleman had Hansen's

disease, " Dr. Ayi said, mainly because of telltale skin lesions.

Bacteriological tests quickly confirmed the diagnosis and the man was

started on antibiotics.

Now, Dr. Ayi said, " he's doing terrific. "

Bu how was the diagnosis missed at presentation? For most U.S. doctors,

leprosy is the last thing on their minds -- particularly in the

heartland of America.

Even the Ghana-born Dr. Ayi -- aware of the disease through both

experience and training -- said she " wasn't expecting to find Hansen's

disease in Iowa. "

Most leprosy in the U.S. is imported and -- until recently -- the

150-odd new cases a year were mainly found among immigrants clustered in

11 port cities, according to Krahenbuhl, Ph.D., director of the

National Hansen's Disease Program (NHDP) in Baton Rouge, La., part of

the federal Health Resources Service Administration.

But the disease is now popping up in states like Iowa, " in places where

we hadn't seen it before, " Dr. Krahenbuhl said. Immigrants -- usually

medically underserved -- " are going where the jobs are " and taking

leprosy with them.

Yet outside of those 11 ports, where there are long-established

surveillance and treatment programs, most doctors in the U.S. have a low

index of suspicion for the disease.

Moschella, M.D., of Lahey Clinic Medical Center in Burlington,

Mass., describes the shifting pattern of leprosy as a " geopolitical

phenomenon " that may catch clinicians by surprise.

" All of our doctors are not familiar with the disease, " said Dr.

Moschella, a former president of the American Academy of Dermatology who

is involved in caring for about 100 active Hansen's disease patients.

That lack of familiarity is why Dr. Krahenbuhl led a symposium on the

topic at the recent meeting of the American Society for Tropical

Medicine and Hygiene in New Orleans.

It was a proactive effort to restore leprosy awareness, said Dr.

Krahenbuhl, in view of the shifting population patterns. As time goes

on, he said, he and other leprosy experts will be giving more and more

talks, seminars, and symposia on Hansen's disease.

Raising the index of suspicion is the first step, according to

Scollard, M.D., chief of the clinical branch at the National Hansen's

Disease Program. " Physicians can easily miss it because they don't think

of it, " he said.

The next step is making the diagnosis, and the " big clue " there is a

reduction or absence of sensation around the suspect lesion, he said.

" Most of the time this can be picked up fairly easily if people think to

test for it, " Dr. Scollard said.

If physicians are not sure about their diagnosis or simply want some

additional guidance, they can take a biopsy and send it to the program's

Baton Rouge headquarters.

" We'll help manage the care, " Dr. Scollard said.

The disease historically -- it's been known since the year 600-- was

feared as highly contagious and devastating. But in fact, it's not

highly transmissible, is now easily treatable, and, with early diagnosis

and treatment, is not disabling.

The Hansen's disease program is the successor to the National

Leprosarium, established in Carville, La., in 1917. The program still

has some elderly in-patients who were admitted to the leprosarium, but

most patients are now followed on an outpatient basis.

More than a century ago, when leprosy was endemic in Hawaii, patients

were forcibly repatriated for life to the remote nearly inaccessible

Kalaupapa colony on Molokai. It was there that Father Damien, the

legendary Belgian priest, held sway from 1873.

Few leprosy patients are at Kalaupapa today, and they are there by

choice. In Louisiana, Dr. Scollard said most patients are treated in

their home towns, but a few -- mainly those who need intensive

management -- will be brought to Baton Rouge for care.

All told, about 7,100 people in the U.S. were reported to have Hansen's

disease over the past 30 years, Dr. Krahenbuhl said, and of those about

6,500 are still alive and some 3,500 are under active management.

It's a chronic granulomatous disease, caused by infection with

Mycobacterium leprae that mainly affects the skin and peripheral nervous

system. Most people are immune to M. leprae.

Treatment recommendations vary depending on the type of disease.

Paucibacillary disease is treated for a year with dapsone at 100 mg a

day and rifampin (Rifadin) at 600 mg a day. Multibacillary disease gets

the same combination, but for two years and with the addition of

clofazimine (Lamprene) at 50 mg daily.

With those drugs, " we can cure the infection, full stop, " Dr. Scollard

said, although some patients will have complications that make the

disease course " more rocky. "

Indeed, from 10% to 20% of patients have hypersensitivity reactions,

characterized by systemic inflammation, that can occur up to a decade

after the end of treatment.

" So we never dismiss any of our patients, " said Dr. Moschella.

Also, Dr. Krahenbuhl said, if treatment is started late, permanent nerve

damage may already have occurred, which will make care more complicated.

Humans are the main reservoir of the disease, although a related strain

found in armadillos is thought by some experts to be a possible source

for some home-grown U.S. cases.

Most home-grown leprosy occurs in southern states -- Louisiana, Texas,

and the Gulf Coast of Florida -- where armadillos are also present --

but " it's tremendously difficult to connect those dots " because the

disease can take years to develop after exposure, Dr. Krahenbuhl said.

New research on the DNA fingerprint of M. Leprae may allow the program's

experts to nail down the armadillo connection, if it is exists, he said.

In the meantime, physician awareness -- leading to early diagnosis and

treatment -- remains key to controlling the disease, he said.

--

ne Holden, MS, RD

" Ask the Parkinson Dietitian " http://www.parkinson.org/

" Eat well, stay well with Parkinson's disease "

" Parkinson's disease: Guidelines for Medical Nutrition Therapy "

http://www.nutritionucanlivewith.com/

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