Creutzfeldt-Jakob Disease Foundation Advocacy Visits
The Creutzfeldt-Jakob Disease (CJD) Foundation is on Capitol Hill today to inform Members of Congress and their staff about Creutzfeldt-Jakob Disease (CJD), a rare,100% fatal, degenerative brain disease that causes rapidly progressive dementia. CJD is transmissible and presently has no treatment or cure. In their lifetime, approximately 1 in 6,000 individuals die from this disease, however, the unreported and undiagnosed number of cases remains unclear.
CJD receives modest support for surveillance through the Centers for Disease Control and Prevention (CDC). We need your support to strengthen and continue the coordination of prion activities and to assure the safety of the American public.
Variant CJD (vCJD), and Bovine Spongiform Encephalopathy (BSE)
One form of this disease in humans, variant CJD (vCJD), is known to be caused by ingesting tissues in beef contaminated with Bovine Spongiform Encephalopathy (BSE), more commonly known as “mad cow” disease. The most recent U.S. case of variant CJD was announced in 2013 and confirmed by the National Prion Disease Pathology Surveillance Center (NPDPSC) in 2014. Limited BSE testing by the USDA adds another layer to the already deepening concerns regarding possible risks to humans. Recently, the USDA has decreased random testing for BSE from 40,000 to 25,000 tests per year (12,719 tests in 6 months, or 1 test per 3,302 live cows). Hence, surveillance of BSE in this country is largely dependent on demonstrating the lack of transmission to humans through human disease surveillance. The vCJD case identified by NPDPSC in 2014 exemplifies the persistent risk for vCJD acquired in unsuspected geographic locations and highlights the need for continuing prion surveillance and awareness to prevent further dissemination of vCJD.
Chronic Wasting Disease (CWD)
Most recently, emerging laboratory data show that Chronic Wasting Disease (CWD) of deer and elk could potentially transmit to humans and other mammals, posing a new threat to public health. Human surveillance through brain tissue examination is the only way to definitely diagnose human prion diseases, determine their origin, and determine whether the spread of CWD found in elk and deer in 24 states in the U.S. and in 2 Canadian provinces has become a human risk. A study in progress has shown that CWD was transmitted to macaques (primates that are genetically similar to humans) by feeding them contaminated deer meat. Unlike the BSE outbreak in cattle, CWD prions are highly infectious and the disease transmits by contact and through contaminated environment, including soil and plants. Additionally, multiple lines of experimental evidence indicate that sheep and cows are susceptible to CWD. Since CWD has been proven to cross the species barriers, this opens up the possibility of oral transmission to humans as well, either directly by eating contaminated venison or indirectly through infected domestic animals. Continued prion disease surveillance, particularly through examination of human brain tissue, will be imperative to evaluate whether CWD has or can spread to humans.
The NPDPSC, funded by the CDC and located at Case Western Reserve University in Cleveland, Ohio, is our line of defense against the possibility of an undetected U.S. human prion disease epidemic as experienced in the United Kingdom.
Key Request 1: We ask for Congressional support in increasing the National Prion Disease Pathology Surveillance Center’s (NPDPSC) appropriation for FY 2020 by $2 million, for a total of $8 million. This would allow the NPDPSC to meet increasing autopsy costs and continue to develop more efficient detection methods while providing an acceptable level of human prion disease surveillance. Reduction of funding to the NPDPSC would eliminate an important safety net to U.S. public health, making the U.S. the only industrialized country lacking prion surveillance, which in turn would jeopardize the export of U.S. beef. The increase in funding would allow the NPDPSC to expand its scope to address the growth in Chronic Wasting Dsease (CWD) among deer and elk, and explore whether CWD could spread to humans.
The NPDPSC is funded entirely by the CDC from funds allocated by Congress. The CDC traditionally keeps approximately half of the appropriation.
Maintaining the 2019 appropriation of $6.0M to the CDC will allow the NPDPSC to persist and continue to develop more efficient detection methods while providing an acceptable level of prion surveillance. Acceptable national prion surveillance would not be possible at a lower level of funding. In addition, adding $2.0M to the appropriation for a total of $8.0M would enable the NPDPSC to increase surveillance, tissue collection, diagnostics and diagnostic test development of prion disease cases from CWD endemic states to determine if CWD is transmissible to humans.
The National Prion Disease Pathology Surveillance Center is the only organization in the U.S. that monitors human prion diseases and is able to determine whether a patient acquired the disease through the consumption of prion contaminated beef (“mad cow” disease) or meat from elk and deer affected by chronic wasting disease (CWD).
The NPDPSC also monitors all cases in which a prion disease might have been acquired by infected blood transfusion, from the use of contaminated surgical instruments or from contaminated human growth hormone. Because standard hospital sterilization procedures do not completely inactivate prions that transmit the disease, these incidents put a number of patients under unnecessary risk and required costly replacement of contaminated surgical equipment.
The NPDPSC also plays a decisive role in resolving suspected cases or clusters of cases of food-acquired prion disease that are often magnified by the media, stirring intense public alarm. To date, the NPDPSC has examined over 6,730 suspected incidents of suspected prion diseases and has definitely confirmed presence and type of prion disease in more than 4,000 cases.
The NPDPSC represents the primary line of defense in safeguarding U.S. public health against prion diseases because the United States ─ unlike other BSE affected countries such as the United Kingdom, the European Union, and Japan ─ does not have a sufficiently robust animal prion surveillance system.
The NPDPSC offers assurances, to countries that import (or are considering importing) meat from the United States, that the U.S is free of indigenous human cases of “mad cow” disease. Recently South Korean and Chinese health officials resumed importation of U.S. beef to their country after a visit to the NPDPSC provided assurances regarding rigorous human prion surveillance.
Key Request 2: The CJD Foundation and families affected by CJD request that the NIH consider prion disease as an Alzheimer's Disease and Related Dementia (ADRD), which would allow NIH funds allocated to these groups of diseases to also be applied to prion diseases.
Some funding for prion disease research comes from the NIH, primarily the National Institute of Neurological Disorders and Stroke (NINDS). However, the CJD Foundation, prion disease researchers, and family members recognize the need to devote more funds to exploring prion disease, to and applying prion disease discoveries to other neurodegenerative diseases.
There are many similarities between ADRD and prion disease. Primarily, all ADRD are due to an abnormal form of normal cellular protein that causes disease (e.g., a-beta in Alzheimer’s disease, alpha-synuclein in Lewy body dementia). These abnormal proteins affect certain areas of the brain causing neurodegeneration and neuropsychiatric symptoms. In the same way, prion disease is due to an abnormal form of the prion protein. ADRD researchers have already exploited what is known from prion disease in order to advance their own fields. As with prion disease, proteins implicated in ADRD can be transmitted between cells. These abnormal proteins can also be amplified using protein amplification techniques initially created to study prion disease. Hence, ADRD have already benefited from prion disease research.
Including prion disease as an ADRD accomplishes a variety of goals. Inclusion of prion disease as an ADRD: 1) allows ADRD researchers to study the prion paradigm in other related conditions which in turn enables the use of more valid animal models as animals naturally develop prion disease, 2) allows technologies originally developed for prion disease to be used in other ADRD without the need for separate funding, 3) allows potential treatment targets to be expanded across ADRD and prion disease, and 4) allows caregiver and health services research of ADRD to be applied to prion disease, which shares many of the same challenges.