Iron out the Folds – Creating a Comprehensive Prion Disease Program

Session Description: While prion diseases have low incidence, they are of high consequence in the healthcare setting. With an estimated incidence of one to two cases per million population worldwide, Creutzfeldt-Jakob disease (CJD) and other prion diseases are sporadic with no consistent pattern of transmission. Symptoms are often nonspecific and not atypical for individuals with advanced age, including but not limited to dementia, vision, and speech difficulties. As a result, identifying suspected and confirmed cases can be a challenge. Development of these progressive, fatal neurodegenerative disorders has been linked with inherence of a mutated prion protein gene, transplantation of contaminated tissue, and a small number of cases have been linked to exposure to contaminated surgical or procedural instruments.

As a result of low incidence of the disease, facilities may not have a robust prion disease infection prevention plan in place. Traditional methods of high-level disinfection and sterilization used at hospitals have been shown to be ineffective at inactivating CJD due to inherent resistance of the mutated prion protein. This places patients and staff at risk for exposure.

In this session we will discuss evidence-based recommendations for handling prion disease tissue and provide an overview of our facility’s creation of a comprehensive prion disease program. We will review components of a successful program, share lessons learned, and provide examples of the tools that we have implemented at our facility. At the end of the session, participants will better understand the risk associated with improper handling of high-risk tissues and will be impowered to establish a comprehensive prion disease program at their facility.