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From: TSS ()
Subject: Fifth woman dies after being diagnosed with CJD
Date: August 14, 2005 at 6:59 am PST

Fifth woman dies after being diagnosed with CJD
By Sandy Miller
Times-News writer

TWIN FALLS -- She was an avid bowler and golfer. She loved helping to organize class reunions.

Then last Sunday, Marjorie Skinner became the fifth south-central Idaho woman since January to die after being diagnosed with Creutzfeldt-Jakob disease, an extremely rare and rapidly progressing fatal neurodegenerative disease. She was 72.

"We have received word that the woman who was ill with CJD died in the last week," said Dr. Christine Hahn, the state epidemiologist with the Idaho Department of Health and Welfare. "It can progress very quickly. We're very sorry for the family. It's a difficult disease for both the person and the family to go through."

Skinner was a Twin Falls native, a member of the Twin Falls High School Class of 1950. She later moved to Buhl, where she and her first husband, Lloyd Adamson, ran the local Arctic Circle restaurant. Lloyd died in 1982 and Marjorie married Jack Skinner in 1984. She was very active in her community and served as the 2003-2004 president of the Clear Lakes Golf Course's Ladies Golf Association.

On Memorial Day weekend, Skinner placed fourth in a golf tournament, said Sue Skinner, a spokeswoman for the family. Less than one month later, "Marge" Skinner had lost her ability to speak due to the aggressive nature of CJD.

"Marge was a vibrant, active woman," Sue Skinner said. "She was a wonderful spouse, mother and friend.

"She will be greatly missed."

All but one of the women diagnosed with CJD were from Twin Falls County. The other woman was from Minidoka County, according to South Central District Health. Brain tissue from at least one of the women has been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland to be studied.

Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, are investigating the five cases -- looking through the women's medical records, documenting family histories and looking for anything these women might have had in common. Becker said they'll compare their findings with studies that have been done in other areas.

"We have our investigation under way right now and we are looking into information on all the cases we have," Becker said earlier in the week.

CJD is carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims typically die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

Health officials believe that all of the women had sporadic CJD and not the variant form of CJD that people get when they eat meat from a cow with bovine spongiform encephalopathy -- commonly known as mad cow disease. All of the women were between the ages of 60 and 83. The average age of people who've died from sporadic CJD is 68. The average age of people who've died from the variant form of CJD is 28, according to the CDC.

However, the unusually high number of cases has drawn attention from state health officials as well as the CDC. Normally, the disease infects just one person per 1 million people worldwide a year. In Idaho, there are about three cases of CJD annually, and in recent years the United States has reported fewer than 300 cases of CJD a year, according to the CDC.

State and local officials are in charge of the investigation. The CDC is only serving an advisory role from its offices in Atlanta. CDC officials have no plans to visit Idaho at this time, said Christine Pearson, public affairs specialist with the CDC.

The investigation into the CJD cases is likely to take months and there are no guarantees a specific cause will be found. Becker has said that in 85 percent of CJD cases, a specific cause is never identified.

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com.

Story published at magicvalley.com on Sunday, August 14, 2005

http://www.magicvalley.com/articles/2005/08/14/news_localstate/news_local_state.2.txt

TSS



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