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Practitioners at funeral homes, cemeteries, and crematories have encountered many potentially fatal and infectious diseases, and yet have found ways to both serve families and protect the health of the public. Creutzfeldt-Jakob Disease (CJD) is no exception.
CJD is a rare brain disease that affects one person per million population each year, and occurs when a normal brain protein spontaneously changes into an infectious abnormal form called "prion" and accumulates in brain cells. Individuals with CJD experience a rapid onset of dementia, and a range of neurological symptoms including walking difficulties, sudden jerky movements, and sometimes, visual disturbances. CJD patients usually die within one year following the onset of symptoms. An autopsy is very important in the diagnosis of CJD because it is the best way to confirm presence of the disease.
CJD is not transmissible from person-to-person by normal contact or through environmental contamination. For example, it is not spread by airborne droplets as are tuberculosis (TB) and influenza or by blood or sexual contact as are hepatitis and human immunodeficiency virus (HIV). CJD transmission can occur during invasive medical procedures involving the central nervous system due to exposure to contaminated brain tissue. This accounts for less than one percent of all CJD cases. The majority of cases occur sporadically, but some individuals can also develop CJD because of an inherited mutation. The average age at death of CJD patients in the United States is 68 years. In New York State, the number of CJD deaths averages 22 per year, which is expected among a population the size of New York State's.
Variant CJD (vCJD), a disease discovered in Europe and more commonly known as the human form of "mad cow disease" (bovine spongiform encephalopathy, or BSE), affects a much younger population. The average age at death of vCJD patients in Europe is 28 years. Only three patients with vCJD have been identified in the United States. These patients presumably acquired the disease while living in Great Britain (2 cases) or Saudi Arabia (1 case).
Standard disinfection procedures and routine embalming solutions are ineffective against "prions"; however, studies show that chemical solutions and physical processes involving bleach, sodium hydroxide, or autoclaving can inactivate the prion. If the bodies of CJD patients have not been autopsied, then transportation, preparation, disinfection, and final disposition can be safely performed when standard precautions are strictly enforced.
Funeral service workers can safely remove the body of a CJD patient from the place of death and transport it to the funeral home preparation room for mortuary procedures using appropriate standard infection control measures, which includes wearing personal protective gear. The World Health Organization (WHO) recommends placing the body in a leak proof pouch prior to moving. The bag should be lined with absorbent material to prevent leakage of body fluids. In instances where there is excess fluid, a double bag can be utilized. After transporting, all surfaces (i.e. stretchers, cots) should be disinfected with bleach.
Preparation & Dressing
Embalming bodies of CJD patients who have not been autopsied can be performed using standard precautions. However, it may be prudent to place the body on a waterproof sheet to collect bodily fluids and use disposable instruments. The bodily fluids should be collected in a suitable container. Incision sites should be closed with super glue, wiped down with bleach and the body washed prior to dressing. Cosmetic restorative work may also be undertaken.
Embalming bodies of CJD patients who have been autopsied can also be safely performed. Adherence to standard infection control measures is paramount when embalming an autopsied body of a suspected or clinically-diagnosed CJD patient. Autopsies on these individuals are often restricted to removal of the brain, therefore, special precautions should be taken including placing a plastic sheet with absorbent wadding and raised edges underneath the head to ensure containment of fluids and prevent any spillage. In instances where sutures do not completely control leaking, the cranial cavity should be packed with absorbent material that has been soaked with bleach, and tightly sutured.
Bodies of autopsied CJD patients should be placed on a waterproof sheet to collect all fluids. It is strongly recommended that disposable instruments, masks, gowns, and puncture resistant gloves be used whenever possible. The entire body should be washed with bleach, rinsed, and sanitized before dressing. Special care should be taken to limit fluid leakage when performing restorative work on a CJD patient. All fluids should be collected in a suitable container.
Casketing & Viewing
Avoid unnecessary manipulation of the body that would force purging of body fluids and risk opening of incision sites. If warranted, the casket can be lined with a leak proof sheet. An open casket for viewing should not be prohibited. However, if an autopsy has been performed, family members of CJD patients should be advised to avoid superficial contact (such as touching or kissing the patient's face) with the body.
Terminal Disinfection & Waste Removal
According to the World Health Organization (WHO) Infection Control Guidelines for Transmissible Spongiform Encephalopathies [External Web Site Policy] , all collected fluids should be disinfected by adding 40 grams of sodium hydroxide pellets per liter of collected fluid. The mixture should be stirred after a few minutes and care should be taken to avoid spillage, as the fluid will be hot. It should then be left undisturbed for at least one hour, after which it can be disposed of like other mortuary waste. Plastic sheets and other disposable items that have been exposed to bodily fluids should be incinerated. Mortuary working surfaces that have accidentally become contaminated should be flooded with sodium hydroxide or bleach, left undisturbed for at least one hour, then (using gloves) mopped up with absorbent disposable rags, and surface swabbed with water sufficient to remove any residual disinfectant solution.
Work surfaces can be disinfected by flooding with undiluted bleach. Although the use of disposable instruments is preferred, reusable instruments and tools can be cleaned and disinfected by using CJD sterilization protocols recommended by the Centers for Disease Control and Prevention. All contaminated solid materials should be disposed of as hazardous waste. Disposing of body fluids and tissues and of hazardous chemicals (formulas) should be handled in accordance with funeral home policy, state, and federal regulations.
Final Disposition – Cremation & Burial
There are no special interment, entombment, inurnment, or cremation requirements for patients with CJD. Interment of bodies in closed caskets does not present a significant risk of environmental contamination and cremated remains can be considered sterile, as the infectious agent does not survive incineration-range temperatures.
Where Can I Get More Information?
Centers for Disease Control and Prevention - Prion Diseases
National Prion Disease Pathology Surveillance Center
World Health Organization - WHO infection control guidelines for transmissible spongiform encephalopathies. Report of a WHO consultation, Geneva, Switzerland, 23-26 March 1999
Creutzfeldt-Jakob Disease Foundation, Inc.
World Health Organization (WHO). Infection Control Manual for Transmissible Spongiform Encephalopathies. WHO, 1998
Brown P, Lamb G. CJD and the Mortuary Profession. The Director March 2000
Centers for Disease Control and Prevention - Questions and Answers: Creutzfeldt-Jakob Disease Infection Control Practices
Metropolitan Funeral Directors Association 1998. Creutzfeldt-Jakob disease: A Practical Guide for the Embalmer.
Crain B. Creutzfeldt-Jakob disease: Safety tips for anatomic studies of possible CJD. College of American Pathologists Today, January 1996