From The Press Democrat:

Residents in Sonoma County suffer from a rare and fatal illness related to mad cow disease at a rate that is twice as high as the national average, prompting victims’ families to launch a search for answers.

Creutzfeldt-Jakob Disease, a rapidly progressing and fatal neurodegenerative disorder, on average has taken the life of one Sonoma County resident each year over the past 17 years, according to county data.

But in the past half year, the affliction, which strikes one in a million people in the United States every year, has killed three Sonoma County residents.

Lorraine Collins, a Santa Rosa woman whose husband, Ric, died of CJD in October, has been tracking local deaths and reaching out to families of the afflicted to develop a support network.

“It’s the three in the last six months that’s driving me nuts,” Collins said. “There’s probably a lot more people who have it. And why aren’t we getting that information?”

Scientists at the Centers for Disease Control and Prevention have released a study emphasizing the need for surveillance of public health threats like mad cow disease, at a time when the U.S. government has proposed significant cuts to those monitoring programs.

Creutzfeldt-Jakob disease is one of several “prion diseases” that afflict both humans and animals and that are caused by a misshapen protein called a prion. The most well-known prion disease is bovine spongiform encephalopathy, also known as BSE or mad cow disease, which has been linked to a form of CJD called variant CJD.

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