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Precautions for Patients with Known or Suspected Prion-Associated Diseases (PAD) (including Creutzfeldt-Jacob Disease (CJD), variant CJD (vCJD), Gerstmann-Straussler-Scheinker Syndrome (GSS), Kuru, and Fatal Familial Insomnia (FFI) SUMMARY
Updated 11/04

Introduction

Creutzfeldt-Jacob Disease (CJD), variant CJD (vCJD), Gerstmann-Straussler Scheinker Disease (GSS), kuru, and other diseases caused by prions (small, proteinaceous infectious agents) are fatal illnesses characterized by a rapidly progressive dementia, myoclonus, psychiatric changes, often typical EEG changes, and spongiform neuropathologic changes. Prions are resistant to a number of standard sterilization and disinfection procedures.

Most cases are sporadic (about 90%), and most of the rest are inherited. However, in a small number of cases, iatrogenic transmission of CJD has been associated with percutaneous exposure to medical instruments contaminated with prion/CNS (central nervous system) tissue residues. Possible iatrogenic transmission has also been associated with transplantation of CNS and corneal tissues and recipients of human growth hormone and gonadotropin. Recently a case of vCJD has been associated with a blood transfusion. Transmission of CJD has not been associated with environmental contamination or person-to-person skin contact. Although isolated episodes of CJD have occurred in healthcare workers, the incidence of CJD in this group does not exceed what would be expected by chance alone.

This document provides guidance upon which infection control practitioners, healthcare workers, physicians, and those involved in the care of persons suffering from prion disease can base their care and infection control practices. The goal is to prevent transmission of prion disease to another patient, a healthcare worker or a family member.

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Procedures of Note:

The Admitting Physician or his/her designee, at the point of admission of the patient shall call:

  1. the nurse manager/charge nurse on the floor the patient will go to, and
  2. Hospital Epidemiology and Infection Control on call beeper (3-3855) to inform thme that a suspected or confirmed patient has just been admitted and provide the necessary information.
See the Communication Tree (Appendix A) for more detailed information on who to call and when related to PAD patients

Notify the Morgue
If the patient expires with known CJD, Gerstmann-Straussler Disease, Kuru or other disease caused by prions, label the morgue identification tag with the patients diagnosis.

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