Creutzfeldt Jacob’s Disease is scary; its cause is seemingly invincible, its incubation can last up to decades, and its mortality is 100%. But it is but a mere prion, and we are humans with the ability to think critically in our duty to give families one last goodbye.
Cover photo By 邱鈺鋒 - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=131098194
Embalmers are funny people; historically we have been known to embalm with our bare hands, answer telephones with bloody gloves, and enjoy a coffee or a smoke while injecting our latest case. We laugh at the police who run from houses emanating the smell of decomposition, and regularly discuss what’s for dinner as we suture up chest cavities. But mention an infection disease such as CJD that little is known about, and boy howdy, even the most steely eyed embalmer will turn white and hide under their bed.
Undoubtedly, the idea of Creutzfeldt Jacob’s Disease is scary; its cause is seemingly invincible, its incubation can last up to decades, and its mortality is 100%. But it is but a mere prion, and we are humans with the ability to think critically in our duty to give families one last goodbye.
What is Creutzfeldt Jacob’s Disease?
CJD is caused by what is known as a prion, which is a type of protein that causes a normal protein to fold abnormally. Proteins in our body are long chains that fold into a more functional shape, and that shape can determine what their function is. There are lots of reasons protein folding errors can occur, and CJD occurs when a prion wraps around a normal protein causing the folding error. There are four types of CJD:
- Sporadic CJD- occurs when the protein spontaneously misfolds. Sporadic CJD accounts for 85% of all cases and is not caused by an infection.
- Genetic CJD- is hereditary and accounts for 15% of cases
- Variant CJD- better known as “Mad Cow Disease” and comes from the consumption of infected brain matter. There has been four recorded cases in the US and they all came from outside the country.
- Iatrogenic (from medical treatment)- comes from a transplant of brain dura matter that has been infected with CJD, and can be transmitted from tools/instruments.
Also of note, the Center for Disease Control (CDC) at one time tracked the occupations of suffers of CJD in order to see if there was causation there, and they were unable to find any so they stopped.
So, what is the danger?
CJD can only be diagnosed correctly through an autopsy, although there is a test known as the Real-time Quaking-Induced Conversion test (RT-QuIC) that can test spinal fluid for the prions that cause CJD. According to Meghan Lewis who is the Autopsy Coordinator at the National Prion Disease Pathology Surveillance Center, while the cerebrospinal fluid is an issue, the real danger comes from the brain matter and specifically the consumption of the brain matter. Since the disease is not blood borne, transmission from a needle stick is highly unlikely and as long as normal universal precautions are taken while handling a suspected case, the chance of transmission is nearly non-existent. If the person is autopsied, the risk is somewhat higher, especially if there is brain matter or a large volume of cerebrospinal fluid present. However, if PPE is disposed of properly and none of the brain matter is consumed, the person handling the deceased is not likely to be infected.
What do we need to know as funeral professionals?
When it comes to embalming a person with CJD the World Health Organization (WHO) offers us the following suggestions that apply to both autopsied and unautopised individuals
- Universal Precautions
- The body placed in an impermeable sheet during embalming to catch any drainage
- All drainage placed in a stainless-steel container and treated with sodium hydroxide
- Incision sites treated with a cynoacrylic glue (an example in your prep room is Aron Alpha) and wiped with bleach
- The entire body washed, and formaldehyde is removed from the surface of the body and table, then the entire body washed with a bleach solution
- Instruments can be disinfected with a autoclave or cleaned with sodium hydroxide. Disposable instruments can also be used
- It is also noted by the WHO that embalming an autopsied CJD body is not encouraged but can be completed using these precautions
Other things embalmers should know:
- The Dodge company makes a product called BRIOHCOL PrP Formula which will kill prions on surfaces. According to the Safety Data Sheets (SDS) for this product, it contains sodium chloride (salt) and hypochlorus acid (a common disinfectant). The formulation of these two ingredients is propriety but the dilutions are present in the SDS.
- Despite its cross-linking action, formaldehyde does not totally destroy prions so extra precautions should be taken.
- CJD is largely undiagnosed or misdiagnosed, and it is possible that embalmers have embalmed someone with CJD and not known it.
- Case analysis concerns for a CJD body are
- Paralysis and muscle contraction resulting in position problems
- Dehydration and malnutrition which may call for a hypotonic and solutions containing humectants
Furthermore, bodies cremated above 1000 degrees Celsius (1800 degrees F) will kill the prion. Light surface contact with a deceased person such as kissing and touching the body need not be discouraged. Further, in the United States we cannot charge extra for embalming a CJD case as it is a violation of the American with Disabilities Act. We also cannot refuse a body because they have CJD if we have the means to care for them, and we cannot dictate form of disposition. Burying an individual with CJD causes no risk to the environment.
CJD is certainly something to be concerned with, however we are regularly at higher risk from diseases transmitted in blood, saliva, and feces on an everyday basis. Instead of living in fear of a disease that is considered to be a ”one in a million disease” we can be more thoughtful in the care of these people and their families by using a scientific mindset to be informed. New information about CJD is coming out all the time, as illustrated by the release of the BRIOHCOL, which only came out as recently as 2017. By continuing to make a decision based on outdated information we are also communicating to the families who lost someone to CJD fear that may not exist with them. Remember, when we encounter a rare disease as cause of death, we are new to the party. Families have already talked to doctors and specialists that handle these cases, and they expect the same from us.
A very special thanks to Kara DeWall, who provided much of the research for this blog post. Her interview with Meghan Lewis can be found here:
Interview with Meghan Lewis: Autopsy Coordinator, Natl Prion Disease & Pathology Surveillance Center
Other Resources
Johns Hopkins on prion diseases
