Prion diseases have posed a major threat to public health since they were first discovered in the 1920s (Belay & Schonberger, 2005). When these diseases first appeared, no one was sure what was happening or what was causing infected persons to become ill. Because of the potential for prion diseases to be transmitted, it is a major public health concern. All prion diseases are reportable, which means that any incidence in animals or people must be reported to the Centers for Disease Control and Prevention in Atlanta, Georgia.
This section will discuss the extent to which prion diseases currently affect human and animal populations, and the serious threat they pose to the broader realm of public health.
The term prion disease is a broad, overarching classification for different diseases that affect protein folding in the brain and spinal cord (Belay, 1999). All prion diseases have public health implications, but the ones that will be focused on in the following sections are bovine spongiform encephalopathy, variant CJD, and iatrogenic CJD because there are concrete ways to reduce the spread of these forms.
The most frightening aspect of prion diseases is that they are always fatal and currently there are no treatments, vaccines, or cures (Riemer et al., 2008). Death is inevitable for everyone infected. For this reason alone, this classification of diseases is a major risk to public health and must be addressed. In addition to the threat of a widespread prion disease outbreak, these diseases should be addressed because in many cases they are preventable. Although the current incidence rate of all CJD diseases (which are the most common of all of the prion diseases) hovers at about one case per 1 million people per year (Belay, 1999), the threat of this becoming a widespread global issue presents itself as the more serious issue. The issue currently receiving the most focus is transmission of BSE to humans through the consumption of infected cow meat, which leads to the development of vCJD in humans. This transmission can and should be prevented.
Another mode of transmission is through the use of infected surgical tools. Known as iatrogenic CJD, this is typically a risk for those individuals undergoing invasive brain surgeries or procedures such as corneal transplants, human growth hormone treatment, or dura mater grafts (Belay, 1999). Another feature of these diseases is that the infectious agent that causes CJD is very resilient and is not destroyed during traditional disinfection methods. There are current World Health Organization (WHO) recommendations that discuss the best way to deal with contaminated items. These recommendations state that instruments used on a person with or even potentially with a prion disease should only be used once or undergo very specific and thorough disinfecting procedures (WHO, 1999). Iatrogenic transmission is 100% preventable, and the measures needed to make this type of CJD obsolete should be put into place.