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CREUTZFELDT-JAKOB

DISEASE (CJD)
CIS Self-Study Lesson Plan
Lesson No. CIS 249 (Instrument Continuing Education - ICE) by Briana Bird
CSR Tech II
Sponsored by: University of Vermont Medical Center
Burlington, VT

C
LEARNING OBJECTIVES REUTZFELDT-JAKOB DISEASE (CJD), A TRANSMISSIBLE SPONGIFORM
1. Define the term “prion” and identify encephalopathy (TSE), is named after the German psychiatrists who first
common transmissible spongiform described the disease in the 1920s. Note: “Encephalopathy” is a general term
encephalopathy diseases, symptoms, relating to brain disease, damage or malfunction. TSEs are more popularly
and diagnosis challenges referred to as prion diseases: a group of rare degenerative brain disorders that can be
2. Review the significant challenges seen as tiny holes that give the tissue a “spongy” appearance when it is viewed under a
involved with processing instruments microscope.
that are or may be contaminated with This lesson provides a foundation of background information helpful to Certified
transmissible spongiform encephalopa- Instrument Specialist (CIS) technicians as they use special procedures to process
thy materials instruments used to treat patients known or suspected to be infected with a TSE.
3. Discuss non-recommended and It provides an overview of CJD, applicable healthcare policy considerations,
potentially useful alternatives for pro- recommended cleaning and sterilization processes, and new technologies to diagnose
cessing instruments known or suspect- the disease.
ed to have been used in Creutzfeldt-
Jakob Disease and transmissible
spongiform encephalopathy surgical OBJECTIVE 1: DEFINE THE TERM
procedures “PRION” AND IDENTIFY COMMON
TRANSMISSIBLE SPONGIFORM
Instrument Continuing Education (ICE) lessons ENCEPHALOPATHY DISEASES,
provide members with ongoing education in
the complex and ever-changing area of surgical
SYMPTOMS, AND DIAGNOSIS
instrument care and handling. These lessons are CHALLENGES
designed for CIS technicians, but can be of value A prion is a protein-containing element
to any CRCST technician who works with surgical
instrumentation.
that is the smallest known infectious
disease-causing agent. It is not a bacteria,
Earn Continuing Education Credits: fungus or virus, and it contains no genetic
Online: Visit www.iahcsmm.org for online
material. For years, many researchers
grading at a nominal fee. believed TSEs were caused by “slow
viruses.” In fact, no viruses could ever be
By mail: For written grading of individual lessons,
send completed 15-question quiz and $15
isolated, and abnormally-folded prions
to: PEC Business Office, Purdue University, Stew- are now thought to be responsible for the
art Center Room 110, 128 Memorial Mall, West family of TSEs in which CJD is the most
Lafayette, IN 47907-2034.
widely known. The Centers for Disease
Scoring: Each 15 question quiz graded online at Control and Prevention (CDC) indicates
www.iahcsmm.org or through Purdue University, there were approximately 400 CJD deaths
with a passing score of 70% or higher, is worth Figure 1 shows an illustration off a prion.
i
two points (2 contact hours) toward your CIS
in the United States in 2010.1
re-certification (6 points) or CRCST re-certification Other TSEs include Fatal Familial
(12 points). Insomnia (FFI), Gerstmann-Straussler- TSEs are progressive and always fatal
More information: IAHCSMM provides online
Scheinker Syndrome (GSS), New Variant diseases. Their incubation time can be
grading service for any of the Lesson Plan Creutzfeldt-Jakob Disease (vCJD), and many years (decades), and fatality is
varieties. Purdue University provides grading kuru. Prions damage specific areas of the almost certain within several months
services solely for CRCST and CIS lessons. Direct
any questions about online grading to IAHCSMM
brain (depending on which TSE causes after initial onset of the symptoms.
at 312.440.0078. Questions about written the infection) and result in the decline of Diagnosis is complicated because of
grading are answered by Purdue University at the victim’s mental abilities. spongiform changes caused by the
800.830.0269.
CIS Self-Study Lesson Plan

Variant CJD has a median age of death of 28 years


and an illness duration period of 13-14 months
(compared to CJD with a median death age of 68
years and an illness of 4-5 months) after observ-
able symptoms. Variant CJD appears to have more
symptoms and is more easily detectable then CJD.
It is initially evidenced by psychiatric and sensory
symptoms, followed by other neurological symp-
toms and progressive cognitive impairment.

specific TSE that can affect different areas products contaminated with BSE. About process TSE-contaminated items, and
of the brain at the same time. that same time, there were also three cases strategies to do so are very difficult
Other challenges arise because of of CJD in the United Kingdom that may because they present particular barriers
astrogliosos, which can be caused by have been caused by an infected blood to most standard processing protocols.
several factors other than a TSE. Note: the donor. Healthcare facilities must plan and
term “astrogliosis” refers to an abnormal Variant CJD has a median age of death implement an applicable policy and
increase in astrocytes (cells that help keep of 28 years and an illness duration period applicable training to manage CJD or
neural tissue healthy by surrounding of 13-14 months (compared to CJD with other TSE cases that arise. These are
and providing support for and insulation a median death age of 68 years and an critical to best ensure that Operating
between neurons in the brain). When illness of 4-5 months) after observable Room (OR) and Central Service (CS)
this condition affects neurons close to symptoms. Variant CJD appears to staff will know what to do before the
the infection site, there is a reduction have more symptoms and is more patient is in the OR.
of inflammatory reaction that makes easily detectable then CJD. It is initially One of the biggest problems with
diagnosis difficult. These degenerations of evidenced by psychiatric and sensory prion-based diseases is that once
brain functions through the loss of healthy symptoms, followed by other neurological instruments are contaminated with the
spongiform and neurons can be seen as symptoms and progressive cognitive TSE, it becomes very difficult to clean
the patient’s mental health decreases. impairment. Magnetic resonance imaging them in a way that ensures the process
Examples of symptoms include possible (MRI) brain scans may enable non- has been effective. There is evidence that
dementia, tremors and spasticity (a muscle invasive opportunities to diagnose the neurosurgical instruments contaminated
control disorder caused by an imbalance of disease; however, the incubation period with affected central nervous system
signals from the central nervous system). for vCJD is still unclear (it might be years tissue from implants or products
TSEs affect animals, as well as or decades). (examples: dura mater and corneal grafts,
humans, and there is some evidence of and pituitary tissue) can transmit CJD if
a link between the two. Consider Mad OBJECTIVE 2: REVIEW THE they are not properly cleaned. Although
Cow Disease [formal name: bovine SIGNIFICANT CHALLENGES transmission of CJD in healthcare
spongiform encephalopathy (BSE)]. INVOLVED WITH PROCESSING settings is exceedingly rare, CS personnel
One may have been responsible for the INSTRUMENTS THAT ARE OR should be aware of the potential for
other in a 1996 European outbreak of MAY BE CONTAMINATED WITH transmission from patient to patient by
vCJD. In that instance, several people TRANSMISSIBLE SPONGIFORM contaminated instruments or equipment
who developed vCJD were believed to ENCEPHALOPATHY MATERIALS or by contaminated tissues.
have been infected by consuming beef It is important to know how to effectively These uncertainties are a primary
CIS SELF-STUDY LESSON PLAN

reason why many people favor using in Australia began requiring the use of A primary instrument
disposable instrumentation in actual or disposable instruments for brain biopsies.
potential cases of CJD and other TSEs, Problems with this policy, however, processing challenge for
and this should be considered as facilities involved the inability to purchase all the prions associated
develop their policies. required instruments in a disposable
Existing loaner policies must also be re- option, and the high cost and relative with TSEs, including
examined. Instruments and sets must be lower quality of some available disposable
properly cleaned and sterilized when they instruments. CJD, is that they are
are received, but what procedures should Due to surgical complications in 2001 unusually resistant
be used before they are returned? Facility when two deaths resulted from surgical
policies frequently require that loaners complications, the facility began using to conventional
leaving facilities be decontaminated high-temperature autoclaving for 18
but not sterilized before being returned minutes as the standard procedure for physical and chemical
to their owners; however, even when neurosurgical instruments although the decontamination
this occurs, it might be preferable to procedure is harsh on instruments.2
decontaminate them once again before methods. Also, the
they are sterilized in the facility where OBJECTIVE 3: DISCUSS NON-
they are used. RECOMMENDED AND POTENTIALLY prions attempt to bind
The review of and changes to existing USEFUL ALTERNATIVES FOR to the surfaces of the
loaner policies provide opportunities to PROCESSING INSTRUMENTS
provide additional training and education KNOWN OR SUSPECTED TO HAVE instruments, making it
to CS, OR and even vendor personnel BEEN USED IN CREUTZFELDT-
about CJD- and TSE-related concerns. JAKOB DISEASE AND even more difficult to
Stakeholders should understand the TRANSMISSIBLE SPONGIFORM remove them.
policies used by the facilities previously ENCEPHALOPATHY SURGICAL
using the instruments and how and why PROCEDURES
the loaners were processed in specific There are ongoing instrument cleaning
ways before their return to the lender. problems and difficulties involving CJD other cases if the quarantine involves
Note: loaners entering the facility are and TSE for which solutions are needed. specialty instruments. There also may be
usually owned by the manufacturer Fortunately, fewer than 1% of CJD cases concerns that quarantined instruments
who, in turn, may desire input to their have resulted from healthcare-associated may be reused if they are returned to the
handling and processing procedures. transmission, and the majority of these facility’s active instrument supply.2
This is reasonable because they currently transmissions have resulted from the use There are numerous alternatives for
provide inservice processing and handling of contaminated tissue or grafts.3 managing instruments potentially or
information to many CS personnel in The Joint Commission (TJC) issued actually exposed to CJD, ranging from
many facilities. an alert in 2001 that referred hospitals autoclaving to soda lye to incinerating
The use of disposable instruments to the WHO guidelines and directed the devices; however, CS professionals
is a tactic to reduce problems with them to establish policies to deal with are committed to zero tolerance
the management of CJD-infected the possibility of CJD transmission. The for ineffective processing, and this
instruments, and information learned alert and guidelines relate to possible philosophy provides the motivation to
in the early 2000s may be useful. cases of patients with unconfirmed CJD continuously seek the best processes
The United Kingdom, along with the and possible tactics that might be used to for managing instruments known or
World Health Organization (WHO), avoid holding instruments in quarantine suspected to have been used on CJD
developed guidelines for infection control until a definitive diagnosis is determined. patients.
management of CJD patients and the Doing so is typically very expensive (a A primary instrument processing
instruments and devices that come into neurosurgical instrument set can cost challenge for the prions associated
contact with them and their tissues. In tens of thousands of dollars), removes with TSEs, including CJD, is that they
response, the Royal Melbourne Hospital instruments from rotation, and can delay are unusually resistant to conventional
CIS Self-Study Lesson Plan

physical and chemical decontamination s %XTENDEDGRAVITYSTEAMSTERILIZATION yield significant improvements in the


methods. Also, the prions attempt to bind cycles for 1 hour at 250° F (121° C) care of patients with CJD and other TSE
to the surfaces of the instruments, making and pre-vacuum cycles for 18 minutes diseases.
it even more difficult to remove them. at 275°F (135° C).
While some enzymes have been shown s .EWERPROCESSESUSINGRADIOFREQUENCY REFERENCES
to be effective, others increase the prions’ gas plasma, and vaporized hydrogen 1. Centers for Disease Control and Prevention.
resistance to subsequent inactivation by peroxide (1.5-2. mg per L). CJD (Creutzfeldt-Jakob Disease, Classic).
steam sterilization. These alkaline and www.cdc.gov/ncidod/dvrd/cjd. Retrieved
enzymatic detergents can reduce protein It would also be helpful to confirm October 24, 2014.
contamination, but are ineffective in whether a patient has CJD or another TSE 2. www.patient.co.uk
eliminating the prions. disease before surgery is performed. Then 3. Infection Control & Hospital Epidemiology Feb
A chemical cleaner containing chlorine OR and CS personnel could determine 2010 Vol. 31 No 2.
and sodium hydroxine (lye) provides the necessary type of instruments 4. www.mdtmag.com/news/2014/08/scientists-
consistent prion inactivation; however, (reusable or disposable, when develop-nasal-test-human-prion-disease
it is corrosive and, therefore, unsuitable appropriate) and processing protocols
for many devices. Researchers continue without waiting for a biopsy result.
their efforts to develop a detergent that Interestingly, a new technology is
can effectively inactivate the prions emerging that may make this possible. IAHCSMM acknowledges the assistance of the
without instrument damage, and its Researchers are developing a nasal test to following two CS professionals who reviewed
discovery would represent a significant diagnose CJD, which would enable results this lesson plan:
breakthrough in processing protocols. to be known much more quickly and less LISA HUBER, BA, CRCST, ACE, FCS; Sterile
One current recommendation is to invasively than a brain tissue biopsy.4 Processing Manager, Anderson Hospital,
ensure instruments are kept damp Maryville, IL
during transport from the OR to time of IN CONCLUSION PAULA VANDIVER, CRCST, CIS; Orthopedic
decontamination, which should occur as There is hope that researchers will Specialist, Anderson Hospital, Maryville, IL
soon as possible. discover more effective ways to clean
Over time, new and updated and sterilize instruments and/or to
contradictions and recommendations make available their cost-effective
emerge. For example, researchers now and appropriate quality disposable
know that some sterilization and high- counterparts. These advancements will
level disinfection processes are ineffective
in deactivating prions. These ineffective
processes include: CIS SELF-STUDY
s 3TANDARDGRAVITYANDPRE VACUUM LESSON PLANS
sterilization cycles of 15 minutes at,
respectively, 25°F (121° C) and 4 TECHNICAL EDITOR WANT TO BE AN AUTHOR?
minutes at 270° F (132° C) CARLA MCDERMOTT, RN, CRCST IAHCSMM is seeking volunteers to write
s $RYHEATSTERILIZATIONCYCLES ETHYLENE Clinical Nurse III or contribute information for our CIS Self-
oxide, earlier forms of Sterrad (Sterrad South Florida Baptist Hospital Study Lessons. Doing so is a great way to
Kos) sterilization, and radiation, micro- Plant City, FL contribute to your own professional devel-
wave, and ultraviolet (UV) treatments. opment, to your Association, and to your
s (IGH LEVELDISINFECTION(,$ BECAUSE SERIES WRITER/EDITOR Central Service department peers.
many HLD solutions are aldehyde- JACK D. NINEMEIER, PH.D. IAHCSMM will provide guidelines and
based and can bind the prions more Michigan State University help you with the lesson to ensure it will be
securely to the instruments. East Lansing, MI an enjoyable process. For more information,
please contact Elizabeth Berrios
In contrast, some processes are effective (elizabeth@iahcsmm.org).
while causing less instrument damag3:
Sponsored by:

CIS Self-Study Lesson Plan Quiz -


Creutzfeldt-Jakob Disease (CJD)
,ESSON.O#)3)NSTRUMENT#ONTINUING%DUCATION )#% s,ESSONEXPIRES-AY

1. Transmissible spongiform encephalopathy 7. Transmission of CJD in healthcare 12. Which process is ineffective in treating
(TSE) diseases are more settings is extremely rare. prions?
popularly referred to as ______ diseases. a. True a. Dry heat sterilization cycles
a. Neurological b. False b. Ethylene oxide
b. Prion c. High-level disinfection
c. Mad Cow 8. Which is a possible disadvantage in d. All the above are ineffective
d. Brain malfunction quarantining instruments potentially
infected with transmissible spongiform 13. Extended gravity steam sterilization
2. A prion is which of the following? encephalopathy (TSE)? cycles may be effective in deactivating
a. Bacteria a. Affected instruments are typically very prions while causing less instrument
b. Fungus expensive damage when operated for _______, at a
c. Virus b. Instruments are taken out of rotation temperature of _______.
d. None of the above c. Affected instruments may accidently a. 1 hour; 250°F (121°C)
be reused b. 1 hour; 275°F (135°C)
3. Transmissible spongiform encephalopathy d. All the above c. 18 minutes; 270°F (132°C)
(TSE) diseases are always fatal. d. 15 minutes; 250°F (121°C)
a. True 9. Which is true about the prions associated
b. False with transmissible spongiform 14. Pre-vacuum steam sterilization cycles
encephalopathy (TSE) including CJD? may be effective in deactivating prions
4. Which is true of astrogliosos? a. They are unusually resistant to while causing less instrument damage
a. It kills red blood cells conventional physical decontamination when operated for __________, at a
b. It spreads prions b. They are unusually resistant to temperature of ___________.
c. It reduces inflammatory reactions conventional chemical decontamination a. 1 hour; 250°F (121°C)
close to the infection site c. They will bind to instrument surfaces b. 1 hour; 275°F (135°C)
d. It quickens the degeneration of neural and become more difficult to remove c. 18 minutes; 275°F (135°C)
tissue d. All the above d. 15 minutes; 250°F (121°C)

5. Which is true of variant CJD (vCJD) 10. Which statement is correct? 15. Which process may be effective in
compared a. All enzymes destroy prions deactivating prions, while causing less
to CJD? b. Alkaline detergents reduce protein instrument damage?
a. It has a lower median death age contamination and eliminate prions a. Radio frequency gas plasma
b. Illness duration is longer after c. Enzymatic detergents are ineffective in b. Vaporized hydrogen peroxide
observable symptoms eliminating prions c. Both A and B above
c. It is more difficult to detect d. None of the above are correct
d. A and B above
e. None of the above 11. Which is true about chemical cleaners
containing chlorine and sodium
6. Which is not an example of central hydroxine (lye)?
nervous system tissue? a. They should be used to process
a. Ganglion tissue instruments suspected of prion
b. Dura matter graft contamination
c. Corneal graft b. These cleaners are corrosive to and
d. Pituitary tissue not suitable for many instruments
c. These cleaners are useful for
instruments removed from quarantine
d. These cleaners are useful if instruments
remain damp after Operating Room use

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