Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that can be experimentally transmitted from one animal to another, as well as from human patients to other humans and animals. It affects about one person in every one million people each year worldwide.
The low random incidence of CJD indicates that person-to-person transmission probably does not occur through normal contact. Spouses and other household members of people with sporadic CJD appear to be at no higher risk of contracting the disorder than the general population.
CJD affects both men and women, and most often appears in people aged 50 to 75. The majority of cases occur on a sporadic basis via unknown mechanisms. In a few rare families (7-10% of all cases), the disease is hereditary.
In a smaller number of cases (<1% of all cases) it is transmitted iatrogenically through contaminated surgical instruments (e.g. implanted EEG depth electrodes) and human material (e.g. human growth hormone, dura mater or corneal transplants).
Symptoms: Symptoms of CJD may be similar to those of other progressive dementias such as Alzheimer's disease, but it tends to progress much more rapidly, usually culminating in death over the course of a few months to a year.
In the early stages of CJD, individuals may have failing memory, changes in behavior, lack of coordination, or visual disturbances. As the disease progresses, mental deterioration becomes pronounced; involuntary movements, especially muscle jerks, appear; and the patient may become blind, develop weakness in the arms or legs, and ultimately become comatose.
There is currently no effective treatment for CJD, but clinical trials of potential therapies may begin soon.
NINDS scientists have developed a test for CJD that detects a protein in cerebrospinal fluid that can aid in the diagnosis of the disease. However, the only way to absolutely confirm a diagnosis of CJD is by brain biopsy or autopsy.
Scientists recently found evidence linking an outbreak of a variant form of CJD (vCJD), largely in Britain, to mad cow disease, a deadly brain disease- similar to CJD-that affects cattle. While there is still no definitive evidence for how this transmission took place, it is believed that transmission occurred through the ingestion of beef contaminated by the BSE agent.
Precautions: To protect themselves, health-care professionals should employ universal precautions when handling blood and spinal fluid samples from patients with CJD. When performing medical/surgical procedures and post-mortem examinations, the most important safety rule is to avoid self-induced injury from instruments used in the course of removing and processing tissues for pathological examination. In particular, avoid contact between contaminated material and skin with cuts or abrasions.
Normal sterilization procedures such as boiling or irradiating materials do not prevent transmission of CJD. Whenever possible, contaminated instruments and other materials should be discarded as medical pathological waste or destroyed by incineration.
When this is not possible, special disinfection methods may be employed. Re-used instruments and materials should be kept moist until they can be appropriately decontaminated and cleaned.
Detailed guidelines for infection control during patient care, for disinfection and disposal of CJD-contaminated material, and for performing an autopsy and embalming are available in the document, "WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies."
GUIDELINES FROM THE WORLD HEALTH ORGANIZATION
AFTER DEATH: GUIDELINES FOR HANDLING A DECEASED PATIENT WITH CREUZFELD JAKOB DISEASE
Precautions for handling of the disease patient: On the death of a patient with confirmed or suspected CJD, the removal of the body should be carried out using normal infection control measures. It is recommended the deceased patient be placed in a sealed body bag prior to moving, in line with normal procedures for bodies where there is a known infection risk. Where the skull is open or there is Cerebrospinal fluid leakage, and where sutures do not completely control this leakage, the bag should be lined with materials to absorb any fluid and the body should be moved in a sealed body bag.
Undertakers and embalmers:
General measures. Mortuary procedures may be performed on the bodies of patients who have died from CJD wit a minimum of inconvenience to ensure the safety of personnel and avoid contamination of the workplace. Transportation of the unembalmed body to the mortuary should be in a sealable, impermeable plastic pouch. Ordinary contact or handling of an intact, unautopsied body does not pose a risk, and cosmetic work may be undertaken without any special precautions.
If a body has undergone autopsy, care should be taken to imit contamination of the workplace by leaking bodily fluids, especially from the cranium, when transferring the body from its transport bag to the mortuary table that has been covered with an impermeable sheet. No other precautions are requirex, except for embalming. See the section on Embalming.
Embalming. An intact (unautopsied) body can be safely managed with only minor adjustments to the usual procedures. The body should be placed on an impermeable sheet or body pouch to avoid surface contamination from perfusion drain sites, and all drainage fluids should be collected into a stainless steel container. Perfusion sites should be closed with super glue and then wiped with bleach.
Embalming an autopsied or traumatized body is not encouraged, but may be safely performed when the following precautions are observed. Disposable masks, gowns and gloves should be worn, just as is done by pathologists performing an autopsy. The body should be placed on an impermeable sheet or body pouch so the suture site leakage can be contained and perfusion drain sites should be similarly arranged to avoid surface contamination.
All drainage fluids should be collected into a stainless steel container. Perfusion and autopsy incision sites should be closed with super glue (cyanoacrylates). The entire body should be wiped down with bleach, and special care taken to ensure contact of bleach with perfusion sites and closed autopsy incisions.
At the conclusion of the perfusion procedures, the container of drainage fluids should be decontaminated by adding sodium hydroxide pellets at the rate of 40g per litre of 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 be then left undisturbed for at least one hour, after which it can be disposed of as for any other mortuary waste.
Plastic sheets and other disposable items that have come into contact with 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 an hour, and then -- using gloves -- mopped up with absorbent disposable rags, and the surface swabbed with water sufficient to remove any residual disinfectant solution.
Non-disposable instruments and tools should be also decontaminated. At the conclusion of the decontamination procedures, the instruments should be washed with water to remove residual disinfectant fluid before drying and re-use. Sodium hydroxide or bleach can be disposed of as uninfectious (but corrosive) waste liquid.
Funerals and cremations. Relatives of the deceased may wish to view or have some final contact with the body. Superficial contact, such as touching or kissing the face, need not be discouraged, even if an autopsy has been conducted. Interment in closed coffins does not present any significant risk of environmental contamination, and cremated remains can be considered sterile, as the infectious agents do not survive incineration-range temperatures (1000 degrees C).
Transport and interment are subject to local and national guidelines, and transport overseas is governed by international regulations.
Exhumations. Standard procedures are conducted according to local and national guidelines. The body should be considered as having the same infectivity as at the time of burial and the precautions used for an autopsy should be followed.
Body donation for teaching purposes. Anatomy departments should not accept, for teaching or research purposes, any body or organs of persons confirmed, suspected or at risk for TSE unless they have specific training or research programs for TSEs, including access to specialized equipment, procedures, appropriate containment facilities and training for managing TSE contaminated tissues.
Departments should make inquiries of those responsible for donating the body, and of the medical staff involved in the care of the donor, to insure the rigorous adherence to this recommendation.
Additional Thoughts: (Compiled in 1998 by Curtis D. Rostad, CFSP, a licensed funeral director and embalmer with 30 years experience as an employee, manager and funeral home owner in Colorado).
There is no law that says you have to risk your life by preparing a CJD decedent.
With CJD, the question of whether or not you will accept this case -- and prepare it for burial or cremation -- can only be answered after carefully considering the facts and weighing the consequences.
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? And, how much risk are you willing to take?
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 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?
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?
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.