Prion Diseases an OVERVIEW for FUNERAL SERVICE PROFESSIONALS

Prion Diseases AN OVERVIEW FOR FUNERAL SERVICE PROFESSIONALS

BY MEGHAN LEWIS, KATIE GLISIC, DANIELLE JORDAN AND DR. BRIAN APPLEBY

Mortuary professionals have encountered Disease, fatal familial insomnia and Gerstmann-Sträus- many potentially fatal and infectious diseases­ sler-Scheinker. and have successfully found ways to both Finally, the remaining cases are acquired through ei- ther ritualistic cannibalism (kuru), ingestion of prions serve families and protect the health of the from cattle infected with bovine spongiform encepha- community. Prion disease is no exception. lopathy (variant CJD, sometimes referred to as the hu- Prion diseases, also referred to as transmissible spongi- man equivalent to mad cow disease) or are secondary form encephalopathies (TSE), occur in humans and an- to receiving contaminated cadaver-derived growth hor- imals and primarily affect the central nervous system. mone, dura mater graft, corneal transplants or through The disease occurs when normal proteins spontaneously neurosurgical contamination. It is important to note that change to abnormal, disease-causing proteins. acquired prion diseases occur under very specific cir- There are three prion disease classifications: sporadic, cumstances. genetic and acquired. The majority are of sporadic etiolo- Prion disease is not transmissible from person to per- gy (85%) and include sporadic Creutzfeldt-Jakob Disease, son through normal contact or through environmental sporadic familial insomnia and variably protease-sensi- contamination. It is not spread through airborne drop- tive prionopathy. Genetic prion diseases account for 5% lets, as is tuberculosis, nor by blood, as are hepatitis and to 10% of cases and comprise familial Creutzfeldt-Jakob human immunodeficiency virus. The average worldwide occurrence of prion disease is CWD or other novel prion diseases can be transmitted roughly one to two cases per million people per year. to people. This number is consistent globally among countries Currently, the only way to confirm a diagnosis of pri- with surveillance centers. Despite being referred to as on disease is through autopsy and the examination and a “one in a million” disease (i.e., the incidence rate), the characterization of formalin-fixed and frozen brain tis- CDC estimates that approximately 1 out of every 6,000 sue. With funding from the CDC, NPDPSC offers a free- deaths in the United States is related to a prion disease of-charge brain-only autopsy to families with loved ones each year. This is due to the fact that age is a risk fac- suffering from suspected prion disease. At this time, the tor for developing the disease and of the variation of ill- center is not aware of any state that requires an autop- ness duration. Given this number, it is highly likely that sy in cases of suspected prion disease/CJD. Enrollment a mortuary professional has served a family whose loved in the program is voluntary and can be withdrawn at one died from a diagnosed or even undiagnosed prion any time prior to autopsy. Families often choose to en- disease. Within the scientific community, prion disease roll their loved one in the program so they can confirm a is known as the perfect “mimicker” of other well-known diagnosis, determine the subtype and learn whether the neurodegenerative diseases, including Alzheimer’s, Par- disease is sporadic or genetic in etiology. The center cov- kinson’s, Lewy Body Dementia, etc., due to the overlap ers all autopsy-related expenses, including but not limit- of symptoms. ed to round-trip transportation between the storage and Prion disease is a rapidly progressive neurodegener- autopsy site (as needed), testing for prion disease and ad- ative disorder. There is currently no cure or treatment, ditional miscellaneous costs. and it is, unfortunately, always fatal. The majority of pa- The center’s network of public and private autopsy pro- tients die within one year of disease onset, with a mean viders across the country works closely with mortuary duration of four to six months. Treatment focuses on professionals to make arrangements and provide educa- minimizing symptoms and maintaining quality of life tion regarding the disease. It is the responsibility of the for both patients and their family/caregivers. contracted mortuary professional to remove a decedent Once a diagnosis of prion disease is made, it is recom- from their place of death. For patients enrolled into our mended that end-of-life concerns be addressed quickly. autopsy program, the center requests that the body is re- Hospice care is usually brought in, and it is recommend- frigerated within four hours of death or as soon as pos- ed that families make pre-arrangements with a funeral sible. A body can be safely and discreetly removed using home, including whether the family wishes for a post- appropriate standard infection control measures, includ- mortem examination before cremation/embalming. ing personal protective equipment. No special precau- tions are required. The National Prion Disease Pathology Surveillance Cen- Patients enrolled in the center’s autopsy program are ter (NPDPSC) was established in 1997 in the Department cared for with dignity. Its staff and licensed mortician of Pathology at Case Western Reserve University. Its pur- take painstaking care to ensure that the level of trans- pose is to acquire tissue samples and clinical information missibility of prion disease is at a minimum. from as many cases of human prion disease occurring in the United States as possible in order to help monitor the Misinformation circulated regarding prion disease can possible occurrence of variant CJD and other emerging be a considerable barrier affecting the care and compas- prion diseases in the country. It also assists clinicians in sion that is extended to families. Education is difficult making a clinical diagnosis of prion disease by offering for numerous reasons. Given that prion disease is rare, it a variety of tests, including cerebrospinal fluid and ge- is not a disease with which many mortuary profession- netic testing. als are familiar. Misinformation about how to handle Although the United States has seen only a few cas- the decedent and the transmissibility of the disease has es of bovine spongiform encephalopathy in our cattle often resulted in families being denied services such as herds, chronic wasting disease (CWD), a prion disease embalming. Per the CDC, embalming bodies of CJD pa- of elk and deer in both free range and captive herds, has tients who have been autopsied can be safely performed been spreading across North America over the past few using standard infection control measures as outlined decades. Since there are an estimated 22 million elk and in the protocol “Infection Control Guidelines for Trans- deer in the United States and a large number of hunters missible Spongiform Encephalopathies,” distributed by who consume elk and deer meat, many humans could be the World Health Organization (WHO). An open-casket potentially exposed to CWD. To date, there have been no viewing should not be prohibited. However, if an autop- reported cases of CWD infection in humans. sy has been performed, it is recommended that family The NPDPSC coordinates with the Centers for Disease members be advised to avoid superficial contact with the Control and Prevention, as well as with individual state body (such as touching or kissing the decedent’s face). health departments to monitor cases from CWD-endem- There are also no special burial or cremation require- ic areas. Furthermore, it is doing experimental research ments. Interment of bodies in closed caskets presents no on CWD transmissibility using animal models. Con- significant environmental contamination risk, and cre- tinued surveillance is necessary to investigate whether mated remains can be considered sterile and safe as the

www.nfda.org June 2019 51 To increase your knowledge about prion LEARN diseases, also check out these resources: has partnered with CJDF to provide education and sup- MORE • cdc.gov/prions/cjd/funeral-directors.html port to mortuary professionals regarding servicing fam- • cjdsurveillance.com ilies with suspected prion disease. Our autopsy team, • cjdfoundation.org along with our licensed funeral director and mortician, has participated in continuing education seminars for the Embalmers Association of Cleveland, the Alabama infection agent does not survive cremation temperatures. Funeral Directors Association, the Funeral Directors As- According to the aforementioned WHO guidelines, any sociation of the Falls Cities and the annual conference of mortuary working surfaces that accidentally become the Oregon Funeral Directors Association. contaminated with a potential CJD agent (e.g., spinal flu- It is our goal that through our various outreach ef- id, brain tissue, etc.) should be flooded with sodium hy- forts and continuing education seminars, we can assist droxide or bleach, left undisturbed for at least one hour, and educate funeral and mortuary professionals in the then (using gloves) mopped up with absorbent dispos- appropriate handling of prion disease patients. We also able rags and surfaces swabbed with sufficient water to aim to clear up any misconceptions or misinformation remove any residual disinfectant solution. The NPDPSC about the disease that may be prohibiting the use of a fa- serves as a resource for funeral homes and embalmers; cility due to a prion disease diagnosis and the family’s its autopsy team is available to answer any questions and wishes of an autopsy to confirm such a diagnosis. can be reached at 216-368-0587. We hope the information presented has enriched your Family advocacy organizations, such as the understanding of prion diseases and invite you to con- Creutzfeldt-Jakob Disease Foundation (CJDF) in the tact our autopsy coordinators at any time for additional United States, are a tremendous resource for families, information or guidance. mortuary professionals and the scientific community. Trained staff and volunteers are available seven days a Meghan Lewis is autopsy specialist, Katie Glisic is week to answer calls from family members, physicians, center coordinator, Danielle Jordan is autopsy program hospice and healthcare providers, mortuary profession- manager and Dr. Brian Appleby is director of the als and others. Over the span of several years, the center National Prion Disease Pathology Surveillance Center.

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52 June 2019 THE DIRECTOR