Creutzfeldt-Jakob Disease (CJD) Increasing Educational Support for CJD Families and the Nation
Background: Creutzfeldt-Jakob disease (CJD) is an incurable brain disorder that occurs with an incidence of one case per million annually. The majority of patients die within six months of illness onset. The disease causes damage to the brain leaving patients completely dependent on their caregivers for the most basic needs of daily living. In 1996, a variant form of CJD emerged in the United Kingdom, which was causally linked to bovine spongiform encephalopathy (BSE). Over 200 variant CJD cases have been identified worldwide, including three cases in the United States whose BSE exposure is believed to have occurred outside of the United States. Purpose: The purpose of this program is to enhance support to family members of patients with a suspected or diagnosed case of human prion disease as well as enhance support for national surveillancefor CJD and its emerging variants by (1) providing family members of such patients with easy access to consultations with persons experienced and knowledgeable about practical issues related to this disease, 2) facilitating educational and other mutually beneficial interaction of researchers with family members of CJD patients (3) increasing general awareness about CJD and (4) increasing the number of autopsies of suspected CJD cases. This program addresses the “Healthy People 2010” focus area of Infectious Diseases. Increasing awareness about CJD can be achieved by facilitating dialogue among CJD researchers, family members, and health care professionals. Increasing awareness empowers CJD families to make the appropriate decisions about the care of their loved ones. Learning more about prion diseases through autopsy study of CJD cases would assist in the determination of the various types of human prion disease present in the United States and their frequency of occurrence over time. Such study would also assist in the earlier recognition of potentially emerging forms of human prion disease and facilitate the development of improved pre-mortem diagnostic tests or treatments. Increasing autopsy rates is critical because CJD can only be confirmed through neuropathological study of brain tissue. Measurable outcomes of the program will be in alignment with the following Focus Area from Healthy People: Immunization and Infectious Diseases.
Office: CENTERS FOR DISEASE CONTROL
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