- The following report details the dangers and threats
- 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
- Special Thanks To Halene Shields For Sending
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- Studies of experimental CJD in guinea pigs and mice have
shown that the infectious agent is present in the blood before clinical
- 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
- 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
- So there is as yet no definitive answer to the question,
but the evidence would suggest that extreme caution in exposure to blood
- 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
- 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
- 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
- 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
- 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
- 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
- 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