deer in the woods

National Prion Center Could Lose Funding Just as Concern About CWD Jumping to Humans Rises

The search for rare prion diseases, whether genetic, sporadic, or potentially caused by eating meat from infected animals, could be curtailed beginning next year.

The country's prion disease surveillance center that looks for new brain disorders would lose all of its federal funding and cease operations under President Donald Trump's proposed fiscal 2018 budget.

July 14, 2017 | Source: Milwaukee Journal Sentinel | by John Fauber

When Tim Suroviak developed cognitive problems after coming home following heart surgery last year, one of the first questions doctors asked was whether he was a hunter.

He was not, though his family did eat venison a few times a year.

Suroviak, 63, deteriorated quickly from a rare brain condition known as Creutzfeldt-Jakob. The disease is in the same family as chronic wasting disease, which is endemic in Wisconsin’s deer herd. The Suroviaks live in Wausau.

After his death, Suroviak’s brain was sent to a lab in Ohio, which led to some shocking news for the family: The type of Creutzfeldt-Jakob he had was especially rare, caused by a genetic defect.

That discovery allowed family members to be tested for the disease. So far, no one, including his two daughters, carries the mutation, said Suroviak’s wife, Monica.

But the search for rare prion diseases, whether genetic, sporadic, or potentially caused by eating meat from infected animals, could be curtailed beginning next year.

The country’s prion disease surveillance center that looks for new brain disorders would lose all of its federal funding and cease operations under President Donald Trump’s proposed fiscal 2018 budget.

For 20 years, the brains of people who died of suspected prion diseases, such as Creutzfeldt-Jakob, have been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio.

The center also is the only licensed lab that can test spinal fluid from living people who have suspected Creutzfeldt-Jakob disease. Such tests can allow families to find out if someone has the disease and what type it is, and then plan how to spend the person’s remaining months. The disease is always fatal.

The loss in funding would come at a time of heightened concern over whether chronic wasting disease can infect people. Recent research showed that it could infect monkeys that were fed venison from infected deer.

At the same time, the number of Creutzfeldt-Jakob cases has jumped substantially nationally and especially in Wisconsin, where chronic wasting disease has now been identified in wild deer in 18 counties. For now, officials attribute the increase to better surveillance and an aging population.

“I don’t know why we want to stop surveillance at a time like this,” said Debbie Yobs, president of the Creutzfeldt-Jakob Disease Foundation. “It doesn’t seem logical.”

The foundation is a nonprofit organization that supports families affected by prion diseases, as well as raising awareness and supporting medical research.