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Rare disease afflicts Brazilian village


ARARAS, Brazil (AP) - This is a village where the people melt away.

Tucked into the sunbaked rolling hills of Brazil's midwest, Araras is home to what is thought to be the largest single group of people suffering from a rare inherited skin disease known as xeroderma pigmentosum, or "XP."

Those with the disease are extremely sensitive to ultraviolet rays from sunlight and highly susceptible to skin cancers. It robs victims of the ability to repair the damage caused by the sun.

That's a particularly vexing burden in Araras, a tropical farming community where outdoor work is vital for survival.

Rare Disease Afflicts Brazilian Village

"I was always exposed to the sun - working, planting and harvesting rice and caring for the cows," said Djalma Antonio Jardim, 38. "As the years passed my condition got worse."

Agriculture is no longer a real option for Jardim. He survives on a small government pension and meager earnings from an ice cream parlor he runs.

XP shows early signs that it has taken hold of its victims.

Jardim said he was just 9 when a large number of freckles and small lumps started appearing on his face, the tell-tale signs that experts say signal XP is present in children and call for measures to protect them against the sun.

Such precaution wasn't taken for Jardim, who now wears a large straw hat in an effort to protect his face. But it's helped little. He has undergone more than 50 surgeries to remove skin tumors.

In an effort to camouflage how the disease has eaten away the skin on his lips, nose, cheeks and eyes, Jardim wears a rudimentary orange-tinted mask, its stenciled-in right eyebrow not matching his bushy real one that remains.

Beyond skin damage and cancers, about one in five XP patients may also suffer from deafness, spastic muscles, poor coordination or developmental delays, according to the U.S.-based National Cancer Institute.

More than 20 people in this community of about 800 have XP. That's an incidence rate of about one in 40 people - far higher than the one in 1 million people in the United States who have it.

For years, nobody could tell Jardim or the others what was afflicting them.

"The doctors I went to said I had a blood disorder. Others said I had a skin problem. But none said I had a genetic disease," Jardim said. "It was only in 2010 that my disease was properly diagnosed."

Experts say Araras has such a high incidence rate because the village was founded by only a few families and several were carriers of the disease, so it was passed to future generations as villagers intermarried.

For instance, both of Jardim's parents were carriers of the defective gene that causes the disease, largely ensuring he would have it.

Gleice Francisca Machado, a village teacher whose 11-year-old son, Alison, has XP, has studied its history in the area and says she found cases of people having the disease going back 100 years. She has started an association that educates locals about XP and tries to get parents to take extra care for their children, even if they may not have outward signs of the illness themselves.

"The sun is our biggest enemy and those affected must change day for night in order live longer," Machado said. "Unfortunately, that is not possible."

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perorbailey May 06 2014 at 9:01 AM

Ill with the flu, absolutely begged NOT to travel, go anywhere, but tickets had been purchased, and spouse adamant!
One month near the equator in Peru and Brazil, and have been paying for it for 34 years! Dermatologist is my primary doc! If you're blue-eyed, fair skinned, stay away from the equator!

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3 replies
Ashley May 06 2014 at 9:22 AM

:(

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1 reply
erin Ashley May 06 2014 at 12:27 PM

:,(

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Janice May 06 2014 at 9:21 AM

We need to tell our children to start over when young and to tell family members how sad this is,and how to teach them when young to use a hat and and cream that has a high uva/uvb protection. 50 started young.WE need to take care of the children each and every day. I feel good about this,and ck. with a skin Dr.when they are young even as a baby.. Thank you for caring and May God Bless..

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fernandezarthr May 06 2014 at 9:20 AM

...I believe this might have something to do with the Ozone layer not protecting adequately in Equatorial areas,.......or it might just be a Genetic deficiency or alteration that makes them prone to skin problems,........

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1 reply
danyelle69marks fernandezarthr May 06 2014 at 10:12 AM

it IS genetic. read the article!!

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gailstorm54654 May 06 2014 at 9:18 AM

MY Son Was told he has XP when he was 9 yrs. old, hes now 26,[he's a twin,] and out of 6 kids ,he's the only 1 that has it. He had to wear a 'space suit' made by nasa, He knows how important it is to protect himself from sun,and i Thank GOD he has a mild case and sees his skin dr. every 6 months,

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2 replies
jasonbossert_ma gailstorm54654 May 06 2014 at 9:21 AM

The name for that doctor is a Dermatologist!

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4 replies
danyelle69marks gailstorm54654 May 06 2014 at 10:00 AM

one of the advantages of living in this country. your son is fortunate he was diagnosed early.

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Anthony May 06 2014 at 9:23 AM

God help this young man, and hopefully he gets the help he needs. I hope things are being done, and people educated to erradicate this problem? When I think I have it bad I always thnk about people like this that have it worst.

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1 reply
pdbocc Anthony May 06 2014 at 9:31 AM

Are you sure you actually read the article? The article states that "Experts say Araras has such a high incidence rate because the village was founded by only a few families and several were carriers of the disease, so it was passed to future generations as villagers intermarried." Seems the only way to erradicate this problem is to stop breeding unless you are tested first.

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2 replies
Kim Ann pdbocc May 06 2014 at 9:59 AM

Yes stop breeding with your sister, brother and cousins...

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skymett8 pdbocc May 06 2014 at 11:16 AM

EXPERTS sometimes are WRONG. The ROYAL FAMILY HAD BEEN DOING THIS FOR CENTURIES AND NEVER HAD A PROBLEM.

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plenty9plus May 06 2014 at 9:13 AM

I have been reading two books by Dean Koontz, the first called Snowman and the second Seize the Night and the hero has this disease. I had never heard of it until I began reading his books and had not seen anything about it in real life until now. It's so odd that I see this now. I pray for all the people with this disease. It is so difficult to stay out of the sun. it is sad that Mr. Jardim was never diagnosed so that he could take some precautions to help himself. I pray for him and others afflicted with XP. If not for Dean Koontz, I never would have known XP exists. Although his book is fiction the disease is real.

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2 replies
fishingal0926 plenty9plus May 06 2014 at 9:22 AM

I've read those, too...good ones!

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Kim Ann plenty9plus May 06 2014 at 10:02 AM

LOve Koontz. What book. Yes totally off subject at the moment.

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michael.k.goodwin May 06 2014 at 9:23 AM

Nuke that town or render them sterile !

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1 reply
danyelle69marks michael.k.goodwin May 06 2014 at 10:10 AM

we're waiting excitedly for your vasectomy. we don't need any more half wits running around.

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indabody May 06 2014 at 9:06 AM

My take....these people are rendered invisible so I think this is the byproduct of man made toxins. They pollute the world with toxins and call this a disease but the reality I believe is darker then the man- made up story. No one wanted to listen to Ellen Brokovitch when she was telling of all these sudden diseases springing up in her town until she fought hard enough to find the cause linked to industry and chemical waste. They are doing the same to the fertile lands of Brazil. Dumping toxins and exposing the people to volitile chemicals dumped on crops.

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7 replies
wavforce May 06 2014 at 9:23 AM

WAKE UP,This is not made only by sun,there are some crop defensives too !

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1 reply
ransomwright wavforce May 06 2014 at 9:29 AM

Childish.

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