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  • Second Ashland death from rare brain disease gains attention

  • A second Ashland musician has died of Creuzfeldt-Jakob disease, a rare degenerative brain disorder that affects one in 1 million people per year worldwide.
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      Six cases of Creuzfeldt-Jakob disease have been reported in Jackson County since the Oregon Department of Human Services began keeping track 18 years ago. A previous version of this story contained...
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      Correction
      Six cases of Creuzfeldt-Jakob disease have been reported in Jackson County since the Oregon Department of Human Services began keeping track 18 years ago. A previous version of this story contained an incorrect number.
  • A second Ashland musician has died of Creuzfeldt-Jakob disease, a rare degenerative brain disorder that affects one in 1 million people per year worldwide.
    Jazz pianist Robin Lawson, 70, died Sunday, seven weeks after the disease claimed choir leader Dave Marston.
    Six cases have been reported in Jackson County since the Oregon Department of Human Services began keeping track of Creuzfeldt-Jakob disease 18 years ago, said Ryan Asherin, the agency's Emerging Infection Program coordinator.
    “CJD is considered an emerging infection that we started doing surveillance on in 1991, after there was a scare with CJD in Western Europe from consumption of contaminated meat,” Asherin said in a phone interview, referring to mad cow disease.
    The Centers for Disease Control and Prevention in Atlanta fund the surveillance of emerging infections in each state, but is “not at all concerned” with Jackson County because it's well below the incidence of CJD in the state and nation, said Dr. Emilio Debess, medical epidemiologist with DHS.
    The fact that Ashland had two cases in a short period of time, he said, “could be of concern, but the disease has a long incubation period — seven to 12 years — and it's difficult for anyone to remember what they did or ate seven to 12 years ago.”
    About 250 to 300 cases of CJD are reported in the U.S. each year, with an average age of death at 67, according to the Creutzfeldt-Jakob Disease Foundation (www.cjdfoundation.org). It said 85 percent of cases come from unknown causes, with the other 15 percent from infections during surgery and genetic predisposition. As of August 2005, the foundation said, there are no known cases of ingestion of contaminated beef in the U.S.
    Debess said because of changes in the industry following the mad cow scare of the 1990s, cattle no longer are fed other animals who have died and “transmission of mad cow disease is no longer possible.”
    CJD is characterized by rapidly progressive dementia leading to death, according to the National Institutes of Health. Sufferers initially experience problems with muscular coordination and personality changes such as impaired memory, judgment and thinking. As the illness progresses, their mental impairment becomes severe, and they eventually lose the ability to move and speak and enter into a coma.
    Marston and Lawson were both diagnosed with the disease by specialists at the University of California at San Francisco. But the only way to confirm a diagnosis of CJD is by brain biopsy or autopsy, according to the NIH.
    Debess said an autopsy of Lawson will be performed by Oregon Health & Science University and tested by Case Western Reserve University in Ohio.
    CJD is not communicable between humans, Debess said. It is a “very interesting protein, not a virus, not a bacteria and not a parasite,” he said. “It's produced by everyone, but it can turn into a bad protein.”
    Because CJD is an emerging infection, the Oregon Department of Health requires doctors, clinics and hospitals to report it, said Susan Bizeau, a communicable disease nurse with Jackson County Health Department.
    Many health professionals aren't aware CJD is reportable, but she said she's telling them “anything is reportable as long as it's any uncommon illness of potential public health significance.”
    John Darling is a freelance writer living in Ashland. E-mail him at jdarling@jeffnet.org.
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