Funeral Directors And
Embalmers Admit Discharge
Of Human Prions
Into Public Sewers
The following report details the dangers and threats morticians face
from CJD and their procedures in dealing with those who have died from
CJD/Mad Cow Disease. -ed
Funeral Directors CJD Warning Report
Wyoming Funeral Directors Association
© 2000, Management Associates, All Rights Reserved
Special Thanks To Halene Shields For Sending This Report
Throughout the 130 plus years of modern embalming, funeral service has faced many infectious and potentially fatal diseases and has still found ways to serve families and protect the public health.
We have survived the emergence of TB, polio, hepatitis, and HIV/AIDS because we have formaldehyde and other embalming chemicals that effectively kill the bacteria and viruses that cause these diseases and we have learned to protect ourselves from infection during the embalming process.
As long as we never encounter an organism that is resistant to formaldehyde and other embalming chemicals, and as long as we have the means to protect ourselves, we will always be able to serve the public with our skills.
But what if, in the future, an organism comes along that we can not destroy through embalming? What then?
Well, the future is now. Such a disease exists.
* There is no diagnostic test for it
* You can have it for up to 25 years and not know it
* There is no vaccine
* There is no cure
* There is no treatment
* It is infectious
* It is always fatal
* Formaldehyde doesn't touch it
* There are no embalming chemicals that will kill it
* Common methods of disinfection and sterilization won't kill it
Its called Creutzfeldt-Jakob Disease.
What is Creutzfeldt-Jakob disease?
Creutzfeldt-Jakob disease, or CJD, is a one of a family of diseases known as Transmissible Spongiform Encephalopathies (TSE's). It was first identified and described by both Creutzfeldt and Jakob in the 1920's.
It is a fatal neurological disease for which there is no treatment and no cure.
There is no vaccine available.
Other TSE's include Kuru (a disease associated with cannibalism in New Guinea), and Gerstmann-Straussler-Scheinker syndrome.1
There are also similar diseases among animals. In sheep and goats it is called scrapie. In cows it is called Bovine Spongiform Encephalopathy (BSE), or "Mad Cow Disease". An outbreak of this disease in Great Britain in 1995 caused worldwide concern over whether or not this disease could be transferred from animal to human through the eating of contaminated meat or other animal products.2
As a result, shipment of cattle or meat products into the United States was halted. No cases of BSE have been found in the United States.
A new variant of CJD called nvCJD has been identified in Britain and has been proven to be associated with BSE. The new variant kills younger people (average age 28) and unlike classic CJD, the incubation period appears to be months (7-24) rather than years in length. Again, none of these cases have occurred in the United States.3 4
Who gets CJD?
Creutzfeldt-Jakob disease affects both men and women worldwide usually between the ages of 50 and 75.
What causes it?
CJD results when abnormal protein accumulates in the brain cells. Scientists do not know what triggers the conversion of protein from the normal to the abnormal form, although a genetic defect has been identified that might provide a clue.5 6 Some believe the conversion is caused by a spontaneous mutation of the normal protein itself, while other scientists believe a virus or virus-like entity may be involved.7
This abnormal protein, believed to be the causative agent of CJD, is known as a prion (pronounced "pry-on"),8 a protein based molecule with no RNA or DNA that is smaller than a virus.9
Actually, because the prion disease we see today differs in many respects from the original description of CJD, some scientists question whether or not the disease we see today is indeed the same disease described by Creutzfeldt and Jakob some 70 years ago.10
What are the symptoms of CJD?
The initial symptoms are subtle and can include insomnia, depression, confusion, personality/behavioral changes, strange physical sensations, balance and coordination disorders, loss of memory, and visual problems.
Rapidly the patient deteriorates with progressive dementia and usually myoclonus (involuntary irregular jerking motions) as the disease progresses. Language, sight, muscular weakness and coordination problems worsen.
The patient finally loses all mental and physical functions. Coma follows and death is usually due to pneumonia precipitated by the bedridden, unconscious state.
The duration of CJD from the onset of symptoms to death is usually one year or less, most commonly 2-6 months.11
How common is it?
The official mortality rate is approximately 1 death per million population worldwide per year.12 CJD is still classified as a rare disease (although the National Center for Infectious Diseases considers it an "emerging infectious disease").13 This figure appears to be understated because CJD is often misdiagnosed. Because of the dementia it causes, it is often confused with Alzheimer's.
One study done by Yale University showed that 13% of Alzheimer's patients were found upon autopsy to actually have CJD.14
Approximately 80% of deaths were among persons age 60 or older. Among this age group, the death rate from CJD is 4.5 per million persons.15
How is it diagnosed?
There is no definitive diagnostic test for CJD. The disease is suspected when a patient develops a rapid dementia and myoclonus. A 14-3-3 spinal test is 95% effective in supporting a clinical diagnosis of CJD. Unfortunately, the only 100% effective diagnostic tool available is an autopsy with appropriate tests.16
The post-mortem finding of a coarse spongy-like surface of the brain (and the underlying microscopic cellular changes) is characteristic of CJD, thus the term, spongiform.
Brain surface of CJD patient on autopsy showing sponge-like appearance
How long does it take for symptoms to develop?
A person can become infected with CJD and have no symptoms for typically 12-25 years, although the disease can run its course and result in death in less than three years from the time of infection.
How do you get it?
Three epidemiologic forms of CJD are well recognized. The familiar (genetic) form (5-10% of cases) results from a genetic mutation.
Approximately 1% of cases are iatrogenic (resulting from a medical procedure) including neurological procedures using contaminated instruments, corneal transplant, from electroencephalographic electrodes, cadaveric dura mater grafts, and pituitary hormone administration.17
Sporadic CJD accounts for 80-85% of all cases. These cases result from the mutation of the protein by some unknown cause.18
So it is infectious?
Yes. Although CJD was first described in the 1920's, it was not considered a transmissible disease until 1966.19
In 1974 a case of CJD transmission as the result of a corneal transplant was reported.20
In 1977 CJD transmission caused by silver electroencephalograph electrodes previously used in the brain of a person with CJD was reported. The transmission occurred despite decontamination of the electrodes between patients.21
In 1985 there were a series of case reports showing that cadaver-extracted pituitary human growth hormone could transmit CJD.22 Shortly thereafter, it was shown that human gonadotropin administered by injection could also transmit CJD from person to person.23
CJD was first reported in a recipient of a dura matter transplant in 1987.24
Is CJD a danger to healthcare and mortuary staff?
As of today, over two dozen cases of CJD exist among healthcare workers including physicians, neurologists, pathologists, and laboratory technicians exposed to CJD. There are as yet no documented cases of transfer of the disease from a deceased patient to mortuary staff.
However, the cause of 80-85% of the cases of CJD is unknown and because it can take up to 25 years for symptoms of CJD to develop, it could be decades before we find out the true number of infections of CJD in healthcare workers exposed to patients with the disease.
What body fluids and organs are infectious?
Since this is a neurological disease, the cerebro-spinal fluid is highly infectious. The transmissible agent has also been shown to be present in the brain, spleen, liver, lymph nodes, lungs, spinal cord, kidneys, cornea and lens, bone, and to a much lesser degree, blood.
It is not found in tears, nasal mucous, saliva, urine, or feces. Therefore, there is little risk involved in becoming infected by casual contact or by living with a person with CJD.25
Can you get CJD from blood?
That is the question being debated and researched at the present time. Although there have been no documented cases of CJD resulting from blood or blood transfusion, consider the following:
The Red Cross will not accept blood from a person with CJD symptoms or family history of CJD. A history of CJD disqualifies a patient from being an organ donor.
Human CJD has been reported to have been transmitted to mice by the injection of infected blood from human patients into mouse brain.26
Studies of experimental CJD in guinea pigs and mice have shown that the infectious agent is present in the blood before clinical disease develops.27
Sufficient evidence of animal transmission suggests that the disease has the potential to be transmitted through blood. Human epidemiological evidence only indicates that if blood transmission occurs, it is likely rare.28
Cases have been found of CJD among persons who have received blood transfusions, but the link between the disease and the transfusion has not been proven. Four Australians have been reported with CJD following transfusion. However, the source of the blood transfusions was undocumented.29 There is a recent report in the western United States of three patients contracting CJD and dying after receiving a transfusion from a person who had CJD. This has not been confirmed.
Several cases of CJD following a blood component (albumin) transfusion have been reported. Two cases have been confirmed to have come from a person who died of CJD.30
The members of the Special Emphasis Panel on Creutzfeldt-Jakob Disease, National Heart, Lung, and Blood Institute, have agreed that, "an unqualified and irreducible risk of exposure to CJD through blood and blood products does exist..."31
Since there is no definitive direct evidence of infection from blood transfusions, there obviously can be no answer to whether or not CJD can be contracted by other blood contact. However, the absence of evidence is not evidence of the absence of transmission of CJD through blood.32
So there is as yet no definitive answer to the question, but the evidence would suggest that extreme caution in exposure to blood is warranted.
Is it airborne? Can you get CJD by breathing it?
The risk of infection from aerosols, droplets, and exposure to intact skin, gastric and mucous membranes is not known.
Nevertheless, the National Institutes of Health strongly cautions laboratory workers to avoid the generation of aerosols and droplets during the manipulation of tissues or fluids known or suspected to be contaminated with CJD.33
And CJD is hard to kill?
Yes, the organism is not destroyed by formaldehyde, phenol, gluteraldehyde, alcohol, dry heat, boiling, hydrogen peroxide, ultraviolet radiation, or standard gravity sterilization.
The only effective sterilization technique is steam pressure sterilization (a process not commonly available to most funeral homes). However, even this method is not foolproof. In 2001 a major hospital in Denver reported the exposure of CJD to 6 neurosurgery patients as a result of the use of CJD contaminated surgical instruments after autoclaving. As a result they have changed their procedure to include disposal of all surgical instruments used on a known or suspected CJD patient. Other medical centers are known to double autoclave these surgical instruments. Incineration, is also effective but obviously destroys equipment along with the organism.
The use of sodium hypochlorite (household bleach) is often mentioned as a potential disinfectant, but its results are inconsistent. It is highly corrosive to metal instruments, gives off irritating fumes, and can not be used as an embalming agent.34 For maximum effectiveness, soaking for two hours is recommended. Wiping floors or countertops with bleach, therefore would not produce this level of disinfection.
Sodium hydroxide (lye) is also mentioned as a disinfectant, but has also shown inconsistent results and is also corrosive. In addition, it is a hazardous substance itself which must be neutralized prior to disposal thus raising OSHA and EPA concerns for handling and disposal. Finally, it deteriorates with age, so it loses whatever effectiveness it may have while in storage.35
Finally, it has been shown that the disease organism has long-term survivability. It is still viable and can be transmitted after an inactive period of a year or more.36
Embalming considerations
First of all, a basic review of some embalming fundamentals. Embalming is defined by the following three terms:
A person who dies from CJD will not present any particular challenges to preservation. The selection of embalming fluid, fluid strength, and fluid volume will be the same as a similar body deceased from any other cause.
Similarly, the appearance of the body will be no different than that of a similar body deceased from any other cause. No special restorative techniques will be necessary.
However, CJD is resistant to formaldehyde and every other embalming fluid component or disinfecting chemical including gluteraldehyde, phenol, and alcohol.
Nothing you can do with the body will render it disinfected.
Therefore, by definition, you can not technically embalm a person who is deceased from CJD.37
You can produce tissue firmness and preservation. You can produce a pleasant cosmetic effect. But you have not really embalmed the body.
When you have completed the arterial injection, the disease organism is just as much alive as it was when you began.
Personal protection measures
While there are no industry-specific standards of protection for mortuary staff when discussing CJD, we can emulate what is done in the medical field.
Most pathology departments have additional guidelines for handling patients deceased from CJD.
Typically this includes wearing two or three pairs of disposable gloves, (rubber or latex, never vinyl) protective eye covering and face shield, mask, cap, jumpsuit, waterproof apron, and shoe coverings. (Hospital guidelines go beyond what is normally considered "standard" universal precautions when dealing with CJD.)
Avoid causing aerosol distribution of contaminants. Avoid contact with all tissues and body fluids.
All solid waste should be placed in a leak-proof container and disposed of by incineration.
Instruments should be disinfected by autoclave or incinerated. They can also be containerized and disposed of as medical waste.
All surfaces should be wiped with sodium hypochlorite. Despite its shortcomings, the use of ordinary household bleach is recommended for general disinfective use in the embalming room and especially when dealing with CJD. Surfaces that may be contaminated should be wiped with a .5% solution (1:10 dilution) of bleach.38 39
Since the organism has also been isolated in several internal organs, aspiration of the body should not be attempted.40 Minute pieces of the internal organs can be aspirated into the trocar, exposing the embalmer to the organism, and the trocar will remain contaminated after the process is completed.
Like other diseases, we are going to be exposed to CJD unknowingly. But when we know or suspect the presence of CJD, we can take steps to protect ourselves. We can not totally eliminate the risk of exposure to any disease, but when we know it is present, special care is warranted.
Finally, you would be well advised to consult with local public health authorities prior to attempting preparation to see if special handling of the case is required. Most states and counties have regulations concerning the handling of infectious disease cases. Whether or not it is specifically named in the regulations, CJD is an infectious disease. There may be restrictions on shipping, public viewing or other exposure to the general public.
Additional concerns following autopsy
The CJD organism is concentrated in the brain and spinal column. While we might assume that exposure to the CJD organism is rather limited when handling the "normal" case, the embalmer is fully exposed to the organism when an autopsy has been performed, when death follows neurosurgery, or when death is due to head trauma.
The embalmer is well advised to request that the body be placed in a body pouch following autopsy to minimize contamination of the cot and removal vehicle or exposure of the removal personnel.
If restoration of the remains is attempted, all instruments, the embalming table, embalming room, and embalming room personnel are exposed to the organism and there are no procedures available that will guarantee rendering the organism harmless. The organism can remain viable for over a year.
Limit the number of people who are exposed to the body by limiting admission to the preparation room during the preparation process. Exposure to others after the body has been repaired should also be limited. This would include other embalmers, hairdressers, cosmetologists, etc.
What should be the final disposition?
There have been no specific tests done to determine if the prion is destroyed during the cremation process. This has led some to speculate that the prion is some type of super-bug that can not be destroyed. Incineration has been shown to destroy the prion at 1000 degrees. Therefore it is reasonable to assume that cremation, which is also essentially incineration, also destroys the prion. Also, since the prion is normally destroyed during autoclaving which is steam under pressure at 130 + degrees, we can safely assume that the 1660 + degree crematory will also destroy it.
Likewise, there have been no specific tests as to what happens to the prion when it is buried. Since we know that the prion can live for a year under normal conditions, there is no reason to believe that it can survive any longer than that underground. Assuming that the body remains intact inside the casket or casket and vault for at least a year, the interred body should pose no threat to the environment or underground water sources.
Should preparation be attempted?
Knowing what we know about CJD (and probably more importantly, what we don't know) the question must be asked.
Should an embalmer attempt to embalm a body with CJD?
Some funeral directors that have been called on to serve a family where CJD is the cause of death have had this question answered for them. The family physician has already told the family that they can not expect to have the body embalmed, have a public viewing, or a funeral. Some families have been told that the body should be cremated immediately upon death.
There is no reason for a family to feel obligated to choose cremation. By use of a body pouch and/or a sealing type casket, there is no reason why an unprepared body with CJD can not have a public funeral and an earth burial.
While we may not agree with the conclusion reached by the physician, we should at least note the seriousness he attaches to this disease.
Whether or not an embalmer agrees to prepare the body is a personal decision that should be weighed carefully.
It is quite different from the questions we faced years ago when AIDS was first identified. Even though we did not know much about AIDS initially, we had an effective embalming technique and the chemicals available to render it harmless if we were careful in our procedures.
While the Americans With Disabilities Act (The ADA) has made AIDS a disability and requires embalmers to embalm AIDS cases or risk civil liability, the refusal to attempt to do what is impossible is an entirely different matter.
Since neither the Department of Justice nor the courts have ruled on the issue of handling CJD. A funeral home refusing to attempt preparation may indeed have to defend their decision if an ADA enforcement action is brought against it.
The case will hinge on whether this risk of CJD can be eliminated by "reasonable modifications" to the embalming process.41
Since no embalming fluid exists that will render the prion harmless, it would be argued that there is no "reasonable modification" to be made.
The threat of defending oneself from an ADA complaint must be weighed against the personal health risks involved.
The other difference that we can note between the history of AIDS and the history of CJD is that within months of its discovery, we knew a lot about AIDS; what it was, how it was transmitted, etc. CJD was identified over 70 years ago and still we know very little about it.
With CJD, the question of preparation can only be answered after carefully considering the facts and weighing the consequences.
* We are being confronted with a 100% fatal disease.
* There is no cure; there is no treatment.
* There is no vaccine.
* The mode of transmission of this disease in humans is largely unknown.42
* There is no effective embalming treatment.
* There is no effective disinfecting technique for instruments, equipment, or the preparation room itself.
* The organism can survive and remain viable for over a year, possibly infecting others during that time.
* You may become infected with this disease and not know it for decades.
It would appear that CJD is not a vicious, rampant, killer organism intent on wiping the human race from the face of the planet. The numbers indicate something far less than a worldwide epidemic, to say the least. There is no need for panic or hysteria. We are not facing Black plague or Ebola.
However, it is a fatal illness and we can not take that lightly. And it is not a disease we can do much with, either by way of protective measures or embalming technique.
Universal precautions coupled with cautious, deliberate work practices and a dose of plain common sense will do much to reduce the exposure risk.
But the risk can not be eliminated and the full extent of that risk is unknown.
Is it worth it?
How much risk are you willing to take?
If you determine that the risk of infection is minimal in the "normal" case and attempt preparation, do you still refuse to attempt preparation of the autopsied case where the highly infectious brain, spinal column, and cerebro-spinal fluid are exposed?
Should you refuse to attempt preparation when there is no concrete evidence that you can be infected with CJD in the preparation process? What happens if it is proven that there is no risk of CJD infection from a dead human body? What happens if it is proven that infection is possible?
If you refuse to prepare the body, will the family choose another funeral home? What effect could this refusal have on your reputation of service within the community? What liability might a funeral home face if they refuse to attempt preparation?
What liability might your funeral home face if it is shown that an employee contracted CJD from a body that they prepared in your funeral home?
What moral obligation do you have to protect your employees versus your moral obligation to serve the public?
Although not specifically addressed by OSHA, the "General Duty Clause" of the Bloodborne Pathogen Standard would require the employer to protect employees from a known hazard. Since CJD is certainly a known hazard, employers would be required to protect their employees from this hazard.
It would appear that CJD would require steps beyond that of "universal precautions" and indeed the question is-- can the employer adequately protect their employees from CJD if preparation is attempted?
Some surgeons have refused to operate on a person known to be infected with CJD. Some pathologists have refused to perform an autopsy on a deceased patient who is known or suspected to harbor the CJD organism. Can you base your own decision on theirs?
Indeed knowledgeable authorities within the medical community flatly advise against embalming, including the National CJD Surveillance Unit in the United Kingdom.43
Can you justify embalming a CJD case for what you charge knowing that equipment may have to be destroyed after use and additional precautions must be taken? (Which might point out the fact that embalming any case might be worth a lot more than what you might presently charge.)
These are questions that funeral home staff members should discuss prior to accepting a person deceased from CJD or suspected CJD.
The embalmer deserves to be fully informed about CJD before they make that decision. That decision should be respected and there should be no thought that there is a shirking of "professional responsibility" by electing not to attempt preparation.
Ideally, there would be a definitive answer. Unfortunately, science has not yet provided them.
Ideally there would be a protocol to follow that would protect the embalmer from this disease while facilitating the embalming process.
Ideally there would be a protocol to follow that would protect the embalmer from this disease while facilitating the embalming process.
Ideally, we would have all of the answers. Unfortunately, such is not the case. We simply do not have definitive answers to many of our questions.
Even knowledgeable people within funeral service have honest points of disagreement. Some see little risk, at least in the non-autopsied case. Some flatly refuse to touch a CJD case, autopsied or not.
Add to that the complications of OSHA obligations to protect employees and ADA requirements to provide services as a "public accommodation" and we can see that the situation becomes even more complicated.
Too many in funeral service know nothing at all about CJD. Too many have never even heard of it.
Some will attempt embalming only because that is what embalmers do. Anything less might appear cowardly, especially when the foe is unseen.
That means that too many are going to be exposed to CJD without ever having considered the long-term effects of their decision to handle or not handle a CJD case.
While we may not have definitive answers, what we do have are cautions.
What we do have are warnings.
Someday we should have more.
Someday this report will be outdated.
© Copyright 2000, Management Associates, All Rights Reserve



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