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Source:

Nursing2015

February 2005, Volume 35 Number 2 , p 74 - 75

Author

  • BARBARA SHEFF RN, CPH, HNC, MT(ASCP), MA

Abstract

Outline

  • Renegade protein

  • Nursing considerations

  • Patient education

  • SELECTED WEB SITES

  • SELECTED REFERENCE

    ALTHOUGH RARE, Creutzfeldt-Jakob disease (CJD) is the most common type of human transmissible spongiform encephalopathy. A rapidly progressive neurodegenerative disorder, it's characterized by dementia, memory loss, rapid physical and mental deterioration, and death. Most cases occur randomly, for unknown reasons, in people ages 50 to 75.

    Equally deadly, variant Creutzfeldt-Jakob disease (vCJD) was identified in 1996. Because of its link to a similar disease in cattle called bovine spongiform encephalopathy (BSE), vCJD has been popularly but incorrectly termed mad cow disease. It's believed to be transmitted to people when they eat meat from BSE-infected cattle or possibly when they receive blood transfusions from infected donors. Some suspect it can be transferred from contaminated surgical instruments. After an incubation period that may last many years, infected people experience progressive impairment leading inevitably to death.

    Of 156 confirmed or probable cases of vCJD reported worldwide as of April 2004, 146 patients lived in Great Britain, 6 in France, and 1 each in Italy, Ireland, the United States, and Canada. Although the chance of contracting vCJD is extremely small, fear of contracting the disease is widespread.

    Renegade protein

    The infectious agents believed to be responsible for CJD, vCJD, BSE, and related diseases are called prions. Identified in 1992, prions aren't living pathogens, but rather a renegade form of normal protein. For unknown reasons, the normal protein changes shape and becomes infectious, converting adjacent normal protein into the infectious form. This starts a cascade of damage causing the characteristic spongiform appearance of an infected brain.

    Unlike the typical patient with ...

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