While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists

While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists

Mary Van Beusekom, MS Today at 9:15 a.m.

Chronic Wasting Disease BSE CWD zone deer disposal site Lorie Shaull / Flickr cc

Nine months ago, Janie Johnston, 73, left her home in the Chicago suburbs to drive to her doctor's office for routine care. She made it as far as the side of the street opposite the clinic but couldn't figure out how to get there, so she returned home, where she struggled to remember the abbreviation "GPS."

That was the first sign that something was seriously wrong. Soon, the semi-retired geologist couldn't speak in full sentences or feed herself. Within 2 months, the woman who had been reviewing proposals for the National Science Foundation in the weeks leading up to symptom onset was dead of a terrifying neurological disease her family had never heard of: Creutzfeldt-Jakob disease (CJD).

CWD may cause CJD-like disease if it infects people Rather than being genetic or acquired, Johnston's CJD developed when normal prions in her brain spontaneously began misfolding. The abnormal prions accumulated rather than being shed, triggering confusion and fatigue that doctors initially mistook for stroke, meningitis, or alcohol withdrawal. The disease usually occurs in older adults.

Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members. Kristal Enter

While no one is certain, experts think that another always-fatal prion disease—this one currently known to occur only in cervids such as deer, moose, and elk—may behave the same way if it should jump the species barrier and infect people.

Chronic wasting disease (CWD) has been decimating cervid populations throughout North America since it was first diagnosed in a captive Colorado mule deer in 1967. While mitigation measures such as hunting may help slow its spread, it can't be stopped.

This is because cervids are ubiquitous and free ranging, the interval from infection to symptom onset can take years, and prions spread easily from animal to animal and through environmental contamination, which can persist for years.

Johnston's daughter, Kristal Enter, 39, a fundraiser in Boston, is familiar with CWD and its potential implications for human health. "Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members," she told CIDRAP News. "The more we're on top of chronic wasting disease and thinking about it, the better."

But the frightening thing is that, for well over a month during the recent US government shutdown, no one was watching the human disease landscape for CWD, a highly infectious disease with no treatment or cure.

If cases slip by, it will be too late Nine days after the government shutdown began, all four staff members of the Centers for Disease Control and Prevention's (CDC's) National Center for Emerging and Zoonotic Infectious Diseases Prion and Public Health Office were sent home after receiving reduction-in-force (RIF) notices. While the end of the shutdown led all four to be reinstated through at least January, layoffs after that time are possible.

Within the past few months, two other researchers who had been part of the team also had to be let go after their fellowship contracts weren't renewed, per the administration's policy of blocking contract renewals.

The prion unit, which monitors the nation for human prion diseases, is part of the Division of High-Consequence Pathogens and Pathology. It launched in the mid-1990s in response to the outbreak of bovine spongiform encephalopathy (BSE, or "mad cow disease") in UK cattle. BSE prions were inadvertently consumed by people who ate contaminated beef, causing the human form of BSE, variant CJD (vCJD). All infected people—more than 230—died.

The initial goal of the Prion and Public Health Office was to watch for any cases of vCJD in the US population. Since then, its focus has expanded to include advising hospitals on how to prevent and respond to prion contamination of instruments used in neurosurgery (prions are resistant to many usual sterilization methods), as well as working with state health departments on disease surveillance. Unit members also answer questions from the public.

Today, as CWD continues its inexorable march across the landscape, exposing more and more people, the prion unit's priority is conducting surveillance for signs of a CWD species jump into high-risk people such as hunters. Without this expertise, no one will be able to evaluate whether a suspected case of CWD prion transmission to humans is likely from an animal.

The prion unit has launched several epidemiologic studies in collaboration with multiple states to look at whether more hunters are dying of prion diseases than would be expected.

Janie Johnston Janie Johnston / Courtesy of Kristal Enter As an example of the unit's work, last spring, a cluster of CJD cases in Oregon was widely conjectured to be linked to CWD. Such cases require autopsy and an epidemiologic investigation to determine whether CWD was involved and, if so, what kind of public health measures are needed. The prion unit shared ideas and strategy with the Oregon state health department in this investigation, which, thankfully, found no link.

But experts say that without anyone looking for these deviations from normal—particularly given that signs of illness may take years to appear—cases could easily go unnoticed, and it will be too late to implement public health measures that could mitigate some of these consequences.

Lawrence Schonberger, MD, MPH, retired chief of the Prion and Public Health Office, said that, as was the situation with BSE, CWD containment efforts must continue. "Unlike with mad cow disease, however, these efforts to date have not been successful," he said. Surveillance and research "should continue to help people recognize any emerging risk to humans from this agent now and in the future, when this agent's pathogenicity [ability to cause disease] may change."

'Worst time to get rid of such a division' Brian Appleby, MD, is director of the National Prion Disease Pathology Surveillance Center at Case Western University, which conducts diagnostic testing for human prion diseases and conducted Johnston's autopsy. The CDC funds the center, which collaborates with the Prion and Public Health Office on public health efforts and research projects.

When you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space. Brian Appleby, MD

If the CDC prion unit were eliminated, "no one would be looking at prion disease," Appleby said. "We wouldn't be able to tell if we have an increase in cases or where they're going or coming from. And when you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space."

And with the threat of CWD, "this is probably the worst time to get rid of such a division," he added.

While there have been no stated plans to eliminate the unit, history hints that its continued existence may be in jeopardy. In fact, it was removed from President Donald Trump's budget during both of his administrations, before the House of Representatives and Senate reinstated it, Appleby said.

In the first Trump administration, report language stated that human prion surveillance is redundant because cattle are now screened for BSE, and the National Institutes of Health cover research, said Appleby, who refuted the assertions.

"Part of the reason why we are a safe export country for beef is not just the cattle surveillance for BSE, but the human surveillance for variant CJD," he said, adding that monitoring is a separate function from research. "When we were removed from the president's budget this time around, there was no report language, so we have no signal to know why."

Always 'one step behind' Debbie Yobs, president and executive director of the CJD Foundation, a patient-advocacy organization that works with Appleby's center to provide medical lectures, support groups, and other programs, emphasized the importance of sustained surveillance. "You can't have gaps in monitoring a deadly disease like prion disease," she said.

It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up. Debbie Yobs

CJD is devastating for patients and families, said Yobs, whose husband, Patrick, died at age 45 of the less common, genetic form of the disease. "It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up," she added. "There's no definitive diagnosis except through autopsy."

Indeed, Enter called her family's ordeal "unreal," because although Johnston's case was typical of a sporadic case, CJD affects only about 500 to 600 people in the United States each year, per the CDC. At the same time as the family was grieving, they were trying to learn how hospice staff could best manage Johnston's symptoms, which none of them had dealt with before.

"It becomes incumbent upon the family members to become the experts to guide the care," she said. "What they say about CJD is that every day is another new symptom or new complication to have to try to address. And you always feel like you're one step behind."

https://www.cidrap.umn.edu/chronic-wasting-disease/while-no-one-was-watching-tenuous-status-cdc-prion-unit-risk-cwd-people

“While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists”

US BSE testing <25K annually…

Samples Tested by Fiscal Year

Fiscal Year v Total Samples Tested as Valid WOAH Points per Fiscal Year BSurvE Points

2024 22,848 207,808 468,946

2023 22,838 239,695 570,345

2022 21,816 360,553 842,517

2021 23,124 398,508 962,010

2020 21,441 331,561 795,183

2019 18,627 395,732 948,602

2018 21,809 540,960 1,305,585

2017 24,229 577,494 1,383,678

2016 26,564 547,959 1,314,579

2015 40,902 592,353 1,414,630

Updates on the WOAH activities in the field of TSEs

Natalie MOYEN Disease Status Officer

Status Department

May 14 th , 2024

2

Outline 1. Revised BSE standards (Terrestrial Code)

a) b) Transition process: where are we?

Official recognition of BSE risk status

i. Members/zones recognised as having a negligible or controlled BSE risk status

ii. New applications

iii. Annual reconfirmations

2. Revision of Scrapie standards (Terrestrial Code

https://www.eurl.craw.eu/wp-content/uploads/2024/06/07_WOAH_Updates-on-the-WOAH-activities-in-the-field-of-TSEs.pdf

Wednesday, May 24, 2023

***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification

https://wahis.woah.org/#/in-review/5067

SATURDAY, MAY 20, 2023

***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE

https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html

MAY 19, 2023

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;

***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023

''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...

https://www.regulations.gov/comment/APHIS-2023-0027-0002

https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf

Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment

https://www.regulations.gov/comment/FSIS-2022-0027-0002

https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission

https://www.regulations.gov/comment/APHIS-2018-0087-0002

https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed

PUBLIC SUBMISSION Comment from Terry Singeltary Sr.

Posted by the Food and Drug Administration on May 17, 2016

Comment Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

https://www.regulations.gov/comment/FDA-2003-D-0432-0011

https://www.regulations.gov/docket/FDA-2003-D-0432

JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214

February 14, 2001

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Terry S. Singeltary, Sr

Author Affiliations

JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

https://jamanetwork.com/journals/jama/article-abstract/1031186

26 MARCH 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

Terry S. Singeltary, retired (medically)

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6

Prion Scientific Advisors and Consultants Staff Meeting Singeltary Submission Freas Monday, January 08, 2001

Scientific Advisors and Consultants Staff 2001 Advisory Committee TSE PRION Singeltary Submission Freas Monday, January 08, 2001 3:03 PM

FDA Singeltary submission 2001

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version). I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here: fda link is dead in the water;

http://web.archive.org/web/20041212232925/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

https://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html

SUNDAY, MARCH 19, 2023

Abandoned factory ‘undoubtedly’ contains dormant Mad Cow Disease that could threaten humans, Thruxted Mill, Queniborough CJD

https://bseinquiry.blogspot.com/2023/03/abandoned-factory-undoubtedly-contains.html

FRIDAY, NOVEMBER 14, 2025

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2024 UK's National CJD Research & Surveillance Unit (NCJDRSU) ceased to function on March 31, 2025

https://efsaopinionbseanimalprotein.blogspot.com/2025/11/the-european-union-summary-report-on.html

FRIDAY, NOVEMBER 21, 2025

Chronic Wasting Disease CWD TSE Prion Herd Declines

https://chronic-wasting-disease.blogspot.com/2025/11/chronic-wasting-disease-cwd-tse-prion.html

TUESDAY, NOVEMBER 11, 2025

USAHA 128th Annual Meeting October 2024 CWD, TSE, Prion Update

https://chronic-wasting-disease.blogspot.com/2025/11/usaha-128th-annual-meeting-october-2024.html

FRIDAY, OCTOBER 31, 2025

Captive Cervid and the Economic Burden of Chronic Wasting Disease CWD TSE Prion?

The economic burden of ignoring CWD would be far greater, imo, with time, if no cervid were left, or just a select few, if the environment/property was so exposed and saturated with CWD, that you couldn’t sell it, you couldn’t grow crops because of the soil saturation of the CWD, water tables saturated with CWD, saturation of hay, grains, from crops uptake on said property, cervid meat saturated from Cervid CWD, remember, You cannot cook the TSE prion disease out of meat, In fact new data now shows that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification. So, what Do we do, how many humans and animals do we continue to expose, continue to saturate with the CWD TSE Prion, …

https://chronic-wasting-disease.blogspot.com/2025/10/captive-cervid-and-economic-burden-of.html

https://prpsc.proboards.com/thread/183/captive-cervid-economic-burden-prion

THURSDAY, NOVEMBER 20, 2025

Texas 2 cases CWD Confirmed at Tom Green County Facility Linked to “Ghost Deer” Investigation

https://chronic-wasting-disease.blogspot.com/2025/11/texas-2-cases-cwd-confirmed-at-tom.html

WEDNESDAY, OCTOBER 15, 2025

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025

https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html

Terry S. Singeltary Sr.