Chronic Wasting Disease (CWD)

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1 Blood Safety The American Red Cross (ARC) is denying blood donations from individuals who have spent six months or more in Europe since 1980, as well as that of any blood relative of a CJD victim. Sporadic CJD is not believed to be transmissible through blood, however, the ARC is taking strict precautions. The first case of suspected blood transfusion transmission occurred in the U.K in 2003 through blood donated from a pre-symptomatic variant CJD victim. Even though scientists can t provide positive proof that this case was transfusion-transmitted, in order to protect public health this should be considered the first case. There have been two other cases of suspected transfusion-transmission in the U.K. In 2009, the U.K. authorities also reported possible transmission of vcjd through a treatment for hemophilia produced from blood plasma Other Concerns The public is at risk because the federal surveillance system for monitoring CJD and BSE incidence is poorly funded and fragmented. There is no uniform reporting mechanism in place and each state sets its own requirements. The public is at risk because so little is known about how to detect and treat CJD. Until the gap in that continuum is closed, the public will remain at risk. The linkage of CJD, BSE, the blood supply, and the lack of (continued) knowledge and reporting, unfortunately, combines to fuel those fears. From a public policy perspective, a deeper understanding of CJD - both its causes and the rate of incidence, would provide direction for scientists in their goals to better understand prion diseases and the ultimate goal to find the cure. Chronic Wasting Disease (CWD) Chronic Wasting Disease was first diagnosed in 1967 in Colorado, and it has subsequently been found in captive herds in several other areas. The source of infection for wild and captive deer and elk in new geographical areas is unknown in many instances. While it is not known exactly how CWD is transmitted, lateral spread from animal to animal through shedding of the infectious agent from the digestive tract appears to be important, and indirect transmission through environmental contamination with infective material likely. CWD has not been proven to cross the species barrier. The CJD Foundation, Inc. P.O. Box 5312, Akron, Ohio HelpLine help@cjdfoundation.org HelpLine help@cjdfoundation.org

2 What is CJD? Creutzfeldt-Jakob Disease (CJD) is a rare, fatal brain disorder. The incidence of CJD cases worldwide is one case, per million, per population. In the United States this statistic translates to approximately 300 new cases per year. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present. There are three types of CJD: Sporadic (scjd) Familial (fcjd) Acquired (vcjd or icjd) In the early stages of the disease, CJD patients may exhibit failing memory, behavior changes, impaired coordination and/or visual disturbances. As the illness progresses, mental deterioration becomes more pronounced, and involuntary movements, blindness, weakness of extremities, and, ultimately, coma may occur. scjd usually occurs later in life and typically leads to death within a few weeks or months to one year following the onset of symptoms. HelpLine Sporadic CJD Unknown Causes of CJD Familial CJD Genetic mutation inherited from a parent Iatrogenic CJD Contaminated surgical instruments Contaminated dura mater transplant Contaminated corneal transplant Contaminated human growth hormone Variant CJD Contaminated beef Contaminated blood or blood plasma transfusion The sporadic form of CJD is the most prevalent form of CJD affecting approximately 300 new people in the United States each year. The familial form of CJD accounts for 10-15% of the cases. Acquired CJD, which includes iatrogenic and variant CJD, accounts for less than 1% of all cases. More nformation can be found on As of May 2009, there are no known cases of endemic vcjd in the United States. Diagnosis of CJD is very difficult and is often made from clinical observation and/ or process of elimination of other diseases. The diagnosis of CJD can only be confirmed through a brain biopsy or autopsy. Cerebral spinal fluid testing positive for a or tau protein is often used to confirm a possible diagnosis, this test, however, can be ambiguous. Public Health Concerns CJD is NOT Mad Cow Disease. Bovine Spongiform Encephalopathy (BSE), the technical term for Mad Cow Disease, occurs only in cows. The first documented case of BSE found in the United States occurred in Washington State in December 2003 in a cow imported from Canada. The first endemic case was found in Texas and was announced in Eating infected beef is widely believed to be the cause of the variant form of CJD (vcjd) in humans. vcjd usually affects young people. An endemic case has not yet been documented as originating in the United States. Is the public at risk of exposure to CJD? Although most Americans have never heard of Creutzfeldt-Jakob Disease, they have heard of Mad Cow Disease and fear it. They do not know what it is, but know it is catastrophic. The confirmation of a case of vcjd originating in the U.S. would likely lead to a chain reaction of panic-driven decisions and policymaking.

3 CJD: THE SURGICAL PATIENT Your role as the Primary Nurse

4 UPMC POLICY: HSIC0605 It is the policy of UPMC to identify patients at risk for diseases caused by prions including Creutzfeldt-Jakob Disease (CJD), and to define infection control precautions necessary to prevent transmission of prions to patients and exposure to prions to healthcare workers. Prions are proteinaceous infectious particles made of glycoproteins which are different from viruses because of apparent lack of nucleic acid. Prions collect in the brain tissue as amyloid deposits in patients with CJD and other prion diseases.

5 WHAT IS IT?! CJD is an untreatable, fatal, infectious neurological disorder within the classification called transmissible spongiform encephalopathies (TSE) or prion diseases. Other prion diseases include: GSS, FFI, vcjd and Kuru (infection from ritualistic cannibalism). Prions disrupt brain function by accumulating in the neural cells, which eventually leads to cell death. The incubation period may be months or as long as several decades. Definitive diagnosis can only be made through histological examination of affected brain tissue.

6 WHO IS AT RISK? This policy applies to all domestic patients with a known or suspected prion disease, as well as those who are at high risk for developing prion disease or have risk factors for possible prion disease, including all patients with: Rapidly progressive dementia. Possible Creutzfeldt-Jakob Disease (CJD), Gerstmann-Straussler Scheinker disease (GSS), Fatal Familial Insomnia (FFI) or variant CreutzfeldtJakob Disease (vcjd). Family history of CJD, FFI, or GSS (at least two blood relatives within extended family). Recipients of cadaver extracted pituitary hormones (gonadatropin, human growth hormone) or dura mater grafts. History of living abroad for extended time periods in areas that have been associated with vcjd. History of receiving blood products while living abroad in areas that have been associated with vcjd. (Red Cross) Some prion diseases are genetically transferred. Therefore, patients with a family history of CJD, GSS, or FFI are at increased risk of developing disease.

7 VIII. OPERATING ROOM PROCEDURE Standard Precautions are mandatory for personnel protection. In addition to the usual precautions, the following additional measures will be taken for patients who are identified as high risk for prion transmission: 1. All invasive procedures for high-risk patients with the potential for exposure to infectious material (e.g., open brain biopsy for diagnosis of possible prion disease) will be scheduled as the last procedure of the day. 2. Traffic must be restricted in the operating room. The minimum number of staff needed will be utilized. 3. Equipment/Instruments/Supplies: All unnecessary equipment, instruments, and supplies are removed from the O.R. prior to the beginning of the procedure. Wear protective eye coverings at all times. These can include a mask with face shield, goggles or protective glasses. Wear orthopedic gloves (heavy gauge) or double-glove throughout procedure. Wear shoe covers and impervious gowns. A neutral zone for sharp instruments will be identified and no hand-to-hand passing of these instruments is to be performed. A manual drill will be used to decrease chances of splatter and aerosolization. In the event that a powered instrument must be used, disposable protective covers will be used. Disposable instruments are to be utilized whenever possible.

8 WHAT IS EXPECTED OF YOU: COMPLETE THE CJD ASSESSMENT FORM FOR ALL SURGICAL PATIENTS. THIS IS FOUND AS A TAB IN THE PRE-OP CHECKLIST FORM.

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11 In Summary It is the policy of UPMC to identify patients at risk for diseases caused by prions including Creutzfeldt-Jakob Disease (CJD), and to define infection control precautions necessary to prevent transmission of prions to patients and exposure to prions to healthcare workers. (HSIC0605) CJD is an untreatable, fatal, infectious neurological disorder within the classification called transmissible spongiform encephalopathies (TSE) or prion diseases. It is the responsibility of the RN to screen all surgical patients prior to their procedure for CJD exposure.

12 No One likes a Mad Cow! Don t forget to complete the CJD assessment on ALL surgical patients