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CONTINUING EDUCATION
Special Needs Populations:
Perioperative Care of the
Patient With Creutzfeldt-Jakob
Disease
MARK KARASIN, BSN, BA, RN
2.6
www.aorn.org/CE
Continuing Education Contact Hours
Accreditation
indicates that continuing education (CE) contact hours are
available for this activity. Earn the CE contact hours by
reading this article, reviewing the purpose/goal and objectives,
and completing the online Examination and Learner Evaluation at http://www.aorn.org/CE. A score of 70% correct on the
examination is required for credit. Participants receive feedback on incorrect answers. Each applicant who successfully
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Commission on Accreditation.
Event: #14540
Session: #0001
Fee: Members $20.80, Nonmembers $41.60
The CE contact hours for this article expire October 31, 2017.
Pricing is subject to change.
Purpose/Goal
To provide the learner with knowledge specific to caring
for the surgical patient who has or is suspected of having
Creutzfeldt-Jakob disease (CJD).
Approvals
This program meets criteria for CNOR and CRNFA recertification, as well as other CE requirements.
AORN is provider-approved by the California Board of
Registered Nursing, Provider Number CEP 13019. Check with
your state board of nursing for acceptance of this activity for
relicensure.
Conflict of Interest Disclosures
Mark Karasin, BSN, BA, RN, has no declared affiliation that
could be perceived as posing a potential conflict of interest in
the publication of this article.
The behavioral objectives for this program were created
by Rebecca Holm, MSN, RN, CNOR, clinical editor, with
consultation from Susan Bakewell, MS, RN-BC, director,
Perioperative Education. Ms Holm and Ms Bakewell have no
declared affiliations that could be perceived as posing potential
conflicts of interest in the publication of this article.
Sponsorship or Commercial Support
No sponsorship or commercial support was received for this
article.
Objectives
1.
2.
3.
4.
5.
Describe prions as they relate to CJD.
Discuss the etiology of the types of CJD.
Discuss the symptoms of CJD.
Compare the different methods for diagnosing CJD.
Describe perioperative considerations for a patient who
has or is suspected of having CJD.
Disclaimer
AORN recognizes these activities as CE for RNs. This recognition does not imply that AORN or the American Nurses
Credentialing Center approves or endorses products mentioned
in the activity.
http://dx.doi.org/10.1016/j.aorn.2014.06.018
390 j AORN Journal October 2014 Vol 100
No 4
Ó AORN, Inc, 2014
Special Needs Populations:
Perioperative Care of the
Patient With Creutzfeldt-Jakob
Disease
2.6
MARK KARASIN, BSN, BA, RN
www.aorn.org/CE
ABSTRACT
Creutzfeldt-Jakob disease (CJD) is a rare but lethal prion disease. The worldwide
mortality rate of CJD is 1.67 per one million people, although 90% of cases will lead
to death within one year of symptom onset. Rapid, progressive dementia is the
cardinal sign of CJD. Other early symptoms include deterioration of muscle coordination, memory, judgment, and vision, as well as personality changes, insomnia,
and depression. It occurs in two types, classic and new variant, and classic CJD can
be further divided into three subtypes: sporadic, familial (ie, genetic), and iatrogenic
(ie, health care associated). Surgery often requires using instruments that come in
contact with high-infectivity tissue, especially during neurosurgical procedures and
diagnostic biopsies. To reduce the risk of disease transmission, infection control
practices should include identification of the risk of infection, implementation of
safety protocols (eg, use of personal protective equipment), proper selection and use
of instrumentation, and adequate disinfection and sterilization practices. Silent
carriers, risks of iatrogenic infection, and the possibility of inadvertent exposure
of healthy patients may carry great liability to public health and hospital
stability. AORN J 100 (October 2014) 391-407. Ó AORN, Inc, 2014. http://
dx.doi.org/10.1016/j.aorn.2014.06.018
Key words: Creutzfeldt-Jakob disease, CJD, prion, neurosurgery, diagnostic biopsy,
infection prevention, disinfection and sterilization.
C
reutzfeldt-Jakob disease (CJD) occurs
in approximately one out of every one
million people in the general worldwide
population every year.1 The rarity of this condition,
however, should not permit health care providers to
underestimate the magnitude of the problems that
CJD poses because of its distinguishing infection
control resistance, modes of transmission, and
invariable lethality. The uncommonness of the
disease and its associated stigma and fears have
created a knowledge gap among health care providers that can lead to inadequate care of patients
http://dx.doi.org/10.1016/j.aorn.2014.06.018
Ó AORN, Inc, 2014
October 2014
Vol 100
No 4 AORN Journal j 391
October 2014 Vol 100
No 4
who are diagnosed with or suspected of having
CJD.2,3 The purpose of this article is to close this
gap by exploring the pathophysiology, varieties,
symptoms, and incidence of CJD as well as focusing on the perioperative implications of caring
for patients with CJD who are undergoing surgery
and examining unique perspectives of infection
control practices specific to CJD.
PATHOPHYSIOLOGY
In 1997, Stanley B. Prusiner received the Nobel
Prize in Physiology or Medicine for his discovery
of prions, which represented a new biological
principle of infection.4 Prusiner coined the term
prion to denote proteinaceous infectious particles
that lack nucleic acid and therefore cannot reproduce, which separates this type of infection from
other pathogens (eg, bacteria, viruses).5 A prion is a
form of protein found throughout the body, with the
highest concentration in the central nervous system.6 Normal cellular configuration of this protein,
referred to as PrPC, does not cause pathology; although its function is not completely understood,
researchers believe that PrPC plays a role in copper
ion management of nerve transmissions and in
protecting the brain from degenerative diseases of
old age, such as dementia.5 When PrPC misfolds
(ie, changes shape) as a result of genetic or external
variables, the prion’s secondary and tertiary structures change into an abnormal form called the
scrapie isoform (PrPSc) of the prion protein.5,7
Instead of reproducing, this malformed prion multiplies by acting as an infectious agent that binds
itself to nearby normal proteins and converting
them into the abnormal form. Accumulation of
these malformed proteins leads to degeneration of
neural tissue. Although researchers do not completely understand this mechanism of neuron
damage, referred to collectively as transmissible
spongiform encephalopathies (TSEs), it causes fatal
prion diseases among animals and humans.7,8
The genetic variability of the prion protein gene
(ie, PRNP) is what leads to diversity in phenotypic
expression that in turn results in a variety of
392 j AORN Journal
KARASIN
abnormal prions and their associated diseases.
Figure 1 provides a diagram of human and animal
prion diseases that are currently known.9 Each of
the listed diseases is clinically important; however,
it is essential to focus on those that health care
providers in the developed world are more likely to
encounter. The sporadic form of the prion diseases
accounts for 90% of all cases of human prion disease.10 Thus, this article focuses on CJD, the most
common form of the sporadic prion diseases.
TYPES OF CJD AND ETIOLOGY
There are two types of CJD: classic or new variant
(vCJD). Together, they account for a worldwide
mortality rate of 1.67 per one million people.11
Table 1 describes the three subtypes of classic CJD:
sporadic (sCJD), familial or genetic (fCJD), and
iatrogenic (iCJD).7,12
sCJD
The majority of CJD cases are sCJD, and the cause
remains unknown. Hans Gerhard Creutzfeldt, MD,
and Alfons Maria Jakob, MD, first recognized the
disease in the 1920s. Since then, there has been no
evidence of human-to-human transmission.13 The
disease is the result of spontaneous conversion of
the normal prion protein into its abnormal form.
The average age at onset of sCJD is between 50 and
70 years, although there are recorded cases of patients as young as 21 years of age and as old as
80 years of age.13-15 Although the disease can be
present but silent for decades, most people with
sCJD die within one year of symptom onset.
fCJD
The familial component of fCJD is misleading
because more than 50% of patients with fCJD
report no family history of the disease. Therefore,
the new term genetic CJD (gCJD) is being used
with this subtype of CJD, although it has yet to
become official.16 The condition arises from a
mutation in the PRNP gene, and its occurrence
is clustered in European countries, Japan, Libya,
Israel, and Chile.17-19 The National Prion Disease
SPECIAL NEEDS POPULATIONS: CJD
www.aornjournal.org
Figure 1. A diagram showing the disease names and abbreviations of spongiform encephalopathies.
Pathology Surveillance Center reports that the
median age at death of people with fCJD depends
largely on the type of mutation but tends to range
from 35 to 55 years. The time frame of the illness is
more extended than that of sCJD, ranging from a
few months to up to five years (e-mail communication, Katie Glisic, executive assistant to Pierluigi
Gambetti, MD, professor and director, National
Prion Disease Pathology Surveillance Center, Case
Western Reserve University, March 25, 2014).
iCJD
Prion resistance to routine sterilization methods
has led to transmission of CJD via medical interventions (ie, health care associated), which is
classified as iCJD. The Centers for Disease Control
and Prevention (CDC) reports that iCJD has affected more than 250 patients worldwide.20 Of
those, only six cases have been linked to contaminated neurosurgical instrumentation and stereotactic electroencephalography depth electrodes. The
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TABLE 1. Subtypes of Classic Creutzfeldt1
Jakob Disease
Subtype Occurrence
Sporadic 85% to 90%
Familial
10% to 15%
Iatrogenic < 1%
Cause/transmission
Idiopathic
Familial (ie, genetic)
Medical interventions
(ie, health care associated)
1. Belay ED, Blase J, Sehulster LM, Maddox RA, Schonberger LB.
Management of neurosurgical instruments and patients exposed
to Creutzfeldt-Jakob disease. Infect Control Hosp Epidemiol.
2013;34(12):1272-1280.
rest were linked to human growth hormone, dura
mater, and corneal graft implants that were derived
from infected cadavers.21 It is also important to
note that anyone with LyoduraÒ dural products
that were implanted before May 1987 may be at
risk for iCJD throughout their life.12 Furthermore,
in the past 15 years, nearly 5,000 patients have
been informed of possible exposure to CJD because
the same surgical instruments that were used in
their procedure were previously used in infected
patients.22-24 Despite such events, there are still
conflicting data about previous surgery being a risk
factor for iCJD, possibly because the incubation
period (ie, time from infection to onset of symptoms) can range from one year to 30 years or
longer.25,26 Adding to this challenge is that iCJD
can strike anyone exposed to contaminated instruments and can vary in its presentation, depending on the source of the original infection and
route of transmission, making it difficult to assess
patients’ survival time frame and their age. It does,
however, currently hold a world record among all
iatrogenic diseases for incubation period, which
can be as long as 42 years.27,28
vCJD
It is important to differentiate classic CJD with its
three subtypes from vCJD, a condition that was first
reported in 1996 and has since claimed the lives of
228 people worldwide.29 This disease develops
394 j AORN Journal
from consumption of meat that is contaminated
with bovine spongiform encephalopathy (BSE), a
cattle form of the prion disease commonly referred
to as “mad cow disease.” The median age at death
of patients with vCJD in the United Kingdom is 28
years.30 Worldwide efforts at curbing the BSE
epidemic of the 1990s led to numerous laws and
regulations that control cattle feed and processing,
which are aimed at eliminating the chance of acquiring vCJD.31 Despite such interventions, one
study has recognized that one in 2,000 people in the
United Kingdom may still be silent carriers of the
disease.32 In the United States, the latest case of
vCJD was confirmed in June 2014 in Texas.33 In
the same month, 4,000 lb of beef were recalled
because of a remote risk of CJD contamination
as a result of paperwork irregularities.34 Furthermore, there are data showing transmission of vCJD
through blood product transfusion.32
Although researchers have developed a prototype blood test for symptomatic vCJD, there are no
such methods for detecting it in asymptomatic
populations.35 This shortcoming has pushed a
global movement to protect the blood supply,
resulting in more rigorous screening practices for
high-risk donors. Some of these include exclusion
of anyone who has lived in or traveled to the United
Kingdom during the BSE epidemic, anyone who
has received pituitary growth hormone or dura
mater transplants, and anyone with a family history
of CJD.36
CLINICAL PRESENTATION
The cardinal sign of CJD is rapidly progressive
dementia. Other early symptoms include deterioration of muscle coordination, memory, judgment,
and vision, which may accompany personality
changes, insomnia, and depression. Late symptoms
include myoclonus, blindness, aphasia, ataxia, and
coma. Different types of CJD may present a variety
of symptoms and survival rates; however, 90% of
cases will lead to death within one year of onset of
symptoms.37
SPECIAL NEEDS POPULATIONS: CJD
DIAGNOSIS
Diagnosis of CJD is not without complexities
because of how closely it may mimic Alzheimer or
Huntington disease.37 When a patient presents with
rapidly progressive dementia, the diagnostic process typically starts with identification of one of
several differential diagnoses. Other reversible
conditions are ruled out first and may include a
wide spectrum of “neurodegenerative, autoimmune, infectious, and toxic-metabolic etiologies.”38(p47) Most important to this process is for
health care professionals to obtain a detailed history from the patient and his or her family members
and to perform a physical examination, because an
inaccurate history, especially relating to the time
frame of symptoms, may lead to misdiagnosis (eg,
viral encephalitis).38 Although tissue biopsy or
autopsy remains the gold standard for definitive
diagnosis, minimally invasive methods with proper
interpretation have been quite effective. In reporting on diagnostic criteria, Zerr et al39 found that
changes in the patient’s magnetic resonance imaging scan were accurate in 81% of patients with
sCJD, identification of the presence of 14-3-3
protein in the patient’s cerebrospinal fluid resulted
in sensitivity of 86%, and the presence of specific
peaks on electroencephalography readings, although
significantly lower, resulted in sensitivity of 38%.
However, combining the three diagnostic results
proved to be 99% sensitive for a correct diagnosis.39
Although studies have described a blood-based
assay test for vCJD, there are no definitive screening
methods for asymptomatic populations.35,37
TREATMENT
There has been a remarkable increase in scientific
contributions to CJD research, yet its treatment
remains elusive and the illness is still invariably
fatal. Doxycycline has proven effective in prolonging CJD survival in vitro and in vivo, but these
results were not found to be reproducible in a
randomized, double-blind, placebo-controlled
study.40 Recently, there has been progress in the
treatment of CJD in mouse models. New bioengineered
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antibodies against prions have shown positive results in the early stages, which provides direction
for future research.41 What makes the fight against
CJD even more challenging is that its prolonged
incubation period can be up to 50 years before
symptoms appear.
INFECTION CONTROL PRACTICES
Considering that the pathology of CJD prions is
always fatal, there is a need for proper infection
control practices.42 The components are identifying
the risk of infection, implementing safety protocols
to include use of personal protective equipment,
proper selection and use of instrumentation, and
adequate disinfection and sterilization practices.
Risk of Infection
In March 1999, the World Health Organization
(WHO) published guidelines for infection control
of TSEs. Although more than a decade has passed
since then, the latest reports on health care practices
relating to patient care, disinfection, and sterilization align for the most part with the WHO guidelines.42-44
One update that calls for closer examination,
however, is the 2006 WHO report on TSE tissue
infectivity ratings. This report categorized tissues
by high infectivity, low infectivity, and no detectable infectivity (Table 2).45
Safety Protocols
Selection of proper personal protective equipment
depends on the type of care being provided and the
kind of tissue and fluid (eg, pituitary gland tissue
versus uterine tissue) that is being handled. According to the WHO, isolation of patients who have
or are suspected of having CJD is not required and
only standard universal precautions have to be
maintained during routine nursing care.43 Special
provisions have to be taken, however, when handling high-infectivity tissues and cerebrospinal
fluid. Therefore, if a patient with CJD or a suspected diagnosis of CJD arrives in the preoperative
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TABLE 2. Levels of Tissue Infectivity in Transmissible Spongiform Encephalopathies
High infectivity
n
n
n
n
n
n
n
n
Brain
Spinal cord
Retina
Optic nerve
Spinal ganglia
Trigeminal ganglia
Pituitary gland
Dura mater
Low infectivity
n
n
n
n
n
n
n
1
No detectable infectivity
Peripheral nervous system
Lymphoreticular tissues
Alimentary tract
Placenta
Other (eg, lung, liver, kidney, pancreas,
bone marrow, skeletal muscle, tongue,
blood vessels, nasal mucosa,
salivary gland, cornea)
Cerebrospinal fluid
Blood
n
n
n
n
Reproductive tissues (ie, except placenta)
Musculoskeletal tissues (ie, except skeletal muscle)
Other (eg, trachea, skin, adipose, thyroid, mammary)
Bodily fluids (ie, except blood, cerebrospinal fluid)
1. WHO Guidelines on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies. Geneva, Switzerland: World Health Organization; 2006.
http://www.who.int/bloodproducts/TSEPUBLISHEDREPORT.pdf. Accessed May 12, 2014.
or postoperative areas with a cranial or lumbar
drain in place, the health care provider should
n
use appropriate personal protective equipment and
n dispose of dressings and linens appropriatelyd
that is, any item that comes in contact with
high-infectivity tissue or cerebrospinal fluid
should be placed in biohazard bags processed
for incineration. No special handling is required,
outside of standard precautions, for items that
come in contact with bodily fluids (except cerebrospinal fluid and blood) or excretions or any
tissue that has no detectable presence of prions.46
It is also important to note that, according to the
WHO, there has not been a single case of CJD
as a result of occupational accident or injury.3
These findings are supported by reports from the
European Creutzfeldt Jakob Disease Surveillance
Network that there is no increased risk of sCJD in
health professionals, and analytical studies do
not show a clear excess risk for health-related
professions.47
Instrumentation, Disinfection, and
Sterilization
Frequently, surgery requires the use of instruments
that come in contact with high-infectivity tissue,
especially during neurosurgical procedures and
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diagnostic biopsies. The Joint Commission encourages administrators at health care facilities to
implement the evidence-based sterilization guidelines as outlined by the CDC, the WHO, the
American National Standards Institute, or the
Association for the Advancement of Medical
Instrumentation.48 In 2000, the WHO reported on
prion decontamination protocols for reusable surgical instruments and surfaces43; the CDC has
since updated these protocols to reflect the most
current evidence (Table 3).44
Recently, there have been exciting developments
in materials used to make surgical instruments that,
similar to nickel, may significantly decrease the
level of protein absorption and residue.49 For example, doped diamond-like carbon coatings may
offer potential antifouling surfaces against microbial and protein attachment, which may reduce the
risk of patients acquiring iCJD and lead to new
instrument manufacturing.49 Until such time, the
most effective approach for minimizing prion residual on surgical instruments before thorough
disinfection and sterilization is to not allow them to
dry for any length of time. Presoaking instruments
has been shown to reduce prion-infected tissue
contamination by up to 96%.50
The challenge with prion decontamination is that
prions are resistant to conventional methods of
SPECIAL NEEDS POPULATIONS: CJD
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TABLE 3. Prion Decontamination Protocols for Reusable Surgical Instruments and Surfaces
1
Three recommended options for decontamination of surgical instruments
Surgical instruments should be
1. immersed in 1N or 2N sodium hydroxide (NaOH), placed in an appropriate container, and heated in a gravity-displacement
autoclave at 121 C (250 F) for 30 minutes. Clean and sterilize by conventional means.
2. immersed in 1N NaOH or sodium hypochlorite (NaOCl) 20,000 parts per million (ppm) for 1 hour and then rinsed with
water, placed in an appropriate container, and heated in a gravity-displacement autoclave at 121 C (250 F) for 1 hour.
Clean and sterilize by conventional means.
3. immersed in 1N NaOH or NaOCl 20,000 ppm for 1 hour, rinsed with water, transferred to an appropriate container,
and heated in a gravity-displacement autoclave at 121 C (250 F) or, if in a porous load, heated at 134 C (274 F) for
1 hour. Clean and sterilize by conventional means.
Recommendations for decontamination of surfaces
Surfaces should be
n reduced in their gross contamination as a first step because the presence of excess organic material will lessen the strength
of either the NaOH or NaOCl solution.
n treated with 2N NaOH or NaOCl 20,000 ppm for 1 hour.
n monitored to ensure they remain wet for the entire duration of the treatment and then rinsed well with water.
Warnings
n Do not autoclave NaOH in aluminum containers or in a container that is in contact with aluminum.
n Be aware that exposure to NaOH solution may corrode some poor-quality stainless steel instruments.
n Be aware that exposure to NaOCl solution corrodes many metal instruments.
n Ensure that autoclave containers have rims and lids designed to allow NaOH condensates to collect and drip back into the pan.
n Allow the NaOH solution to cool down to ambient temperature before handling using appropriate precautions because NaOH
solution is very caustic when hot.
1. Belay ED, Blase J, Sehulster LM, Maddox RA, Schonberger LB. Management of neurosurgical instruments and patients exposed to Creutzfeldt-Jakob
disease. Infect Control Hosp Epidemiol. 2013;34(12):1272-1280.
Table adapted and printed with permission from the Centers for Disease Control and Prevention.
disinfection and sterilization. Such methods are
always scrutinized by researchers51 and often prove
to be ineffective or to contradict previously published modes of disinfection; however, this scrutiny
helps researchers discover new methods of prion
decontamination efficacy. For example, the gas
plasma of hydrogen peroxide and various enzyme
solutions is a recently tested but not yet approved
method of disinfection and sterilization.52-55
Another issue is that methods of decontamination are very extensive and therefore can degrade
the instruments and produce harmful gases in the
process.44 A solution to this may be single-use
instruments. In fact, for optimal management of
CJD procedures, the WHO recommends the use
of disposable surgical instruments that are incinerated postoperatively.43 Although this approach is
the most ensured method of reducing the risk of
transmission of iCJD, it does not come without
challenges. The most common complaint that I
gathered through personal interviews with three
neurosurgeons is that single-use instruments are of
inferior quality (eg, not as sturdy) as nondisposable,
reusable instruments. In addition, automated instruments and equipment (eg, drills) are not available as single-use items. Therefore, surgeons who
wish to perform a procedure on a patient who has
or is suspected of having CJD with single-use instruments must resort to the use of manual controlled drills. These instruments may be difficult
to handle or require additional assembly. Furthermore, data from tonsil and adenoid procedures
show a higher risk of certain complications (eg,
hemorrhage) as a result of using disposable instruments.55,56 However, these barriers are not
enough to discourage health care providers from
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using disposable instruments. Ronald Benitez, MD,
from Atlantic NeuroSurgical Specialists, Morristown,
New Jersey, stated his agreement with this in a
telephone interview (June 20, 2014). He has performed numerous CJD biopsy procedures and has
observed an evolution of instrument sets from
reusable to disposable and states that “you cannot
justify the risk of reusing instruments” for such
procedures. Figure 2 provides a sample list of a
CJD disposable instrument set used at one neuroscience center. Finally, CDC infection control
should involve implementing facility-specific
methods for not mixing neurosurgical instruments
with other specialty instruments and developing a
tracking system for instrument trays used in case of
a delayed postoperative CJD diagnosis.44
PERIOPERATIVE IMPLICATIONS
Caring for a patient with CJD during all three
phases of the perioperative processdpreoperative,
intraoperative, and postanesthesia recoverydis
complex and has specific implications for nursing.
Table 4 describes common CJD symptoms and
provides special nursing considerations for each
phase of patient care relating to each symptom.57
Just as with any patient undergoing surgery, the
perioperative nurse should provide individualized
instruction that is dependent on the patient’s specific manifestations of CJD.
Preoperative and Postoperative
When it comes to perioperative patient-centered
nursing care, it is important to learn from those who
have experience working with terminally ill patients. Clarissa Rentz, MSN, APRN-BC, explored
care of patients with CJD from a hospice and
palliative perspective.57 Some of her findings can
be translated and expanded on for the perioperative
environment, particularly those aimed at patient
safety and comfort by ensuring that the perioperative team is aware of the spectrum of possible
symptoms and ready to manage them.
Although the majority of patients with diagnosed
CJD do not leave the hospital, it is not unrealistic
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for a patient to decide to return home for the final
stages of the disease or for the patient’s family
members to make this decision. In this case, emphasis can be placed on patient and family education of prion infection prevention and standard
precautions as well as the disease trajectory related
to timeline and symptoms.
As with any patient care for which family members are at the patient’s bedside, it is imperative to
take the holistic approach and not discount psychosocial aspects of family involvement. This approach
is even more central to caring for patients with CJD
because of the possibility of a diagnosis of fCJD
or gCJD. This means that children or other family
members of the patient may already know, or will
know, that they themselves can develop the disease
later in life. Their perception of health care provided
to their loved one will form a model of what may be
facing them in the future and potentially affect their
development and coping mechanisms.58
Intraoperative
The WHO43,45 and the CDC8,20 have issued specific and extensive guidelines for decontamination
of prion-infected surfaces. The following are intraoperative recommendations and practices reported by some neurosurgical facilities that were
shown to significantly lessen the time required
for OR personnel to perform proper postoperative
cleaning:
n
Allow only essential items in the room, such
as the anesthesia machine, one suction unit, one
monopolar/bipolar electrosurgical unit, one back
table, one Mayo stand, one sharps container, and
one biohazard garbage bag.59
n Cover all pedals and cords with plastic bags and
secure them in place.59
n Cover all other equipment, the walls, the bed,
and the floor of the working area with sheets
of polyvinyl chloride (Figure 3).59,60 Another
neuroscience center has used disposable liquidrepellent sheets (eg, surgical drapes and heavyduty surgical table covers) to achieve the same
goal.
SPECIAL NEEDS POPULATIONS: CJD
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Figure 2. A sample count list showing a disposable neurosurgical instrument set for treating patients with
Creutzfeldt-Jakob disease.
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TABLE 4. Perioperative Nursing Implications for Patients With Creutzfeldt-Jakob Disease
Symptom
Aphasia and akinetic mutism
Nursing consideration
n
n
Ataxia, abnormal posturing, and rigidity
n
n
n
Cortical blindness
Dementia syndrome
n
n
n
n
n
n
n
Dysphagia
n
Heightened startle reflex, myoclonus,
delusions, hallucinations, paranoia,
and seizures
n
n
n
Incontinence
n
n
Infectivity
n
n
n
n
n
n
n
n
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Speak directly to the patient.
Use simple, verbal, step-by-step commands with prompts and visual cues.
Maintain the patient’s proper body alignment when transferring and positioning
the patient.
Communicate to team members that the patient may not be able to cooperate
because of the feeling of falling.
Sacral dressing for pressure ulcer prevention.
Approach the patient from the front and maintain eye contact.
Maintain patient dignity by using a holistic team approach when providing care
for the patient and his or her family members.
Obtain a detailed history from the patient, including information about the
patient’s level of physical and mental disabilities, symptoms, and when or if a
diagnosis was made.
Include the patient’s family members during assessments to help ensure that the
patient’s information is accurate.
Take into account the psychosocial aspects of family involvement, particularly
because children or other family members of the patient may already know, or
will know, that they themselves can develop the disease later in life.
Obtain test results, including tissue biopsies, magnetic resonance imaging
scans, cerebrospinal fluid tests, and electroencephalography.
Review the patient’s consents, do-not-resuscitate status, living wills, and
powers of attorney.
Assist the anesthesia professional with securing an airway and be prepared for
airway emergencies.
Minimize touching and moving the patient as much as possible.
Decrease stimulation by controlling lights, noise, commotion, and frequency and
limiting the number of interventions.
Maintain patient safety by ensuring that bedside rails are up, providing adequate
padding, and keeping safety straps secure.
Insert an indwelling urinary catheter if clinically indicated.
Use linens and pads as needed.
Obtain the surgeon’s completed risk assessment of the patient.
Identify the level of tissue infectivity (ie, high infectivity, low infectivity, no
detectable infectivity) of the tissue that will be handled during the procedure.
Schedule procedures as the last case of the day to allow adequate time for
terminal cleaning of the OR postoperatively.
Obtain and use the proper personal protective equipment based on the type of
care being provided and the kinds of tissue and fluid that are being handled.
Enforce practice guidelines for governing infection control and prevention and
implementing standard precautions.
Implement prion decontamination protocols for equipment and environmental
surfaces.
Evaluate the pros and cons of using disposable, single-use instruments and
equipment.
Implement neurosurgical protocols and protocols for other specialties when
Creutzfeldt-Jakob disease is suspected or confirmed. If suspected, quarantine
the used surgical instruments until a diagnosis is determined for
n disinfection or disposal of instruments or
SPECIAL NEEDS POPULATIONS: CJD
www.aornjournal.org
TABLE 4. (continued ) Perioperative Nursing Implications for Patients With Creutzfeldt-Jakob
1
Disease
Symptom
Nursing consideration
n
n
n
n
n
n
n
n
n
n
Pyrexia
n
n
n quarantine of reusable equipment for reuse exclusively on the same patient.
Implement instrumentation management algorithms for
n preventiondthe patient is identified preoperatively to be at high risk for
having Creutzfeldt-Jakob disease,
n containmentdthe patient is identified intraoperatively to be at high risk for
having Creutzfeldt-Jakob disease, and
n contingencydthe patient is identified postoperatively to be at high risk for
having Creutzfeldt-Jakob disease.
Dispose of linens, patient clothing, and wound dressings appropriately (ie, any
item that comes in contact with high-infectivity tissue or cerebrospinal fluid
should be placed in biohazard bags and processed for incineration). Other than
standard precautions, no special handling is required for items that come in
contact with bodily fluids (except for blood and cerebrospinal fluid) or excretions
and any tissue with no detectable prion presence.
Provide transfer-of-care reports at every personnel change to communicate the
patient’s diagnosis and care requirements.
Perform a time out, during which team members discuss logistics and infection
control practices, and assign a facilitator who can be a liaison between the OR
suite and other facility departments and address perioperative needs (eg,
anesthesia equipment, sterile surgical supplies).
Assign the minimum number of team members needed to work on the
procedure.
Lessen the time required for OR personnel to perform proper postoperative
cleaning by
n allowing only essential items in the room (eg, the anesthesia machine, one
suction unit, one monopolar/bipolar electrosurgical unit, one back table, one
Mayo stand, one sharps container, and one biohazard garbage bag);
n covering all pedals and cords with plastic bags and securing them in place;
and
n covering all other equipment, the walls, the OR bed, and the floor of the
working area with polyvinyl chloride.2
Obtain and use additional labeling and double bagging to alert those handling
specimen containers of its contents and avoid any potential for spillage (eg, prion
specimen).
Obtain and use disposable laryngoscope blades because of the risk of dormant
Creutzfeldt-Jakob disease and the potential for the presence of infective prions
in the patient’s salivary glands and tonsils.
Provide safe, thorough postoperative care by using all necessary personal
protective equipment and infection control practices.
Provide discharge planning, if appropriate, that accounts for the transmissibility
of infectious prions and provides family or caregivers with education about
providing care that reduces the risk of infection and a possible timeline of the
disease and its associated symptoms.
Monitor room temperature.
Use warming or cooling devices as needed.
1. Rentz C. Nursing care of the person with sporadic Creutzfeldt-Jakob Disease. J Hosp Palliat Nurs. 2008;10(5):272-282.
2. WHO Manual for Surveillance of Human Transmissible Spongiform Encephalopathies Including Variant Creutzfeldt-Jakob Disease. Geneva, Switzerland:
The World Health Organization; 2003. http://whqlibdoc.who.int/publications/2003/9241545887.pdf. Accessed June 27, 2014.
AORN Journal j 401
October 2014 Vol 100
No 4
KARASIN
Figure 3. Sheets of polyvinyl chloride should be used to cover the walls of an OR that is prepped for a procedure
that will be performed on a patient who has or is suspected of having Creutzfeldt-Jakob disease.
These efforts have been shown to facilitate relatively easy disposal of potentially contaminated
material.53
Specimen handling for a CJD biopsy does not
require special considerations and can follow hospital protocols for routine care.59 Additional labeling and double bagging can be used to alert
those handling the container of its contents and
avoid any opportunity for spillage.61 Processing
of the specimen has to be performed by a trained
specialist, usually at the National Prion Disease
Pathology Surveillance Center, a hub for all prionassociated diseases and a great resource for health
care providers (R. Benitez, verbal communication,
June 20, 2014).
Anesthesia
Anesthesia professionals are challenged when
providing anesthesia for a patient who has a diagnosis of CJD or who is suspected of having CJD
because of the variety of recommendations from
professional organizations. The American Society
402 j AORN Journal
of Anesthesiologists recommends using standard
precautions and single-use equipment during procedures on such patients.61 A neurosurgical anesthesia publication supports this and suggests that
modern disposable instruments and equipment (eg,
laryngoscope blades, Magill forceps, dilators,
laryngeal mask airways) are just as effective as
nondisposable items.28 An important development
during the past decade is that ventilators do not
need to be quarantined,7 as previously recommended
by Farling and Smith.62
Health care providers are challenged when
there is a need to use a video laryngoscope, fiberscope, or endoscope during procedures for patients
with or suspected of having CJD, especially for
difficult intubations. Although the WHO recommends processing such items according to heatsensitive instrumentation methods, a more recent
publication from the UK Department of Health
dictates that these materials should be destroyed
after a single use or quarantined for reuse exclusively on the same patient.42,63 Abnormal prions
SPECIAL NEEDS POPULATIONS: CJD
have been found in the olfactory epithelium;
therefore, nasal fiber-optic intubation would fall
into the category of single use.63 Regardless of the
approach taken to administer anesthesia, a variety
of sources suggest having a system in place to track
the use of equipment so that it can be traced in case
of future outbreaks of CJD.28,64,65
FACILITY PROTOCOLS FOR CJD
Although CJD is a rare condition, the unique set of
issues that it poses for infection preventionists and
health care providers requires a close examination
of any neurosurgical facility’s protocols. Silent
carriers, risks of iatrogenic infection, and the possibility of inadvertent exposure of healthy patients
may carry great liability to public health and hospital stability. A review of the literature from
around the world (especially from Europe and
Canada, where vCJD is more prevalent) provides
a set of recommendations that may be helpful in
managing a confirmed or potential case of CJD.
Evaluation of current protocols is of the essence
in ensuring that facilities are up to date with current
evidence-based practices. A Joint Commission
Sentinel Event Alert66 supports this and recommends establishing policies for
n
disinfection or disposal of instruments used in
neurosurgery in general and when CJD is suspected or confirmed and
n quarantine of such surgical instruments until an
unclear diagnosis or biopsy is clarified.
Because of the incubation period and often
ambiguous presentation of CJD, it is not always
possible to know that the surgical patient has the
disease. This brings up the need for contingency
management plans. One Canadian health system
has provided a summary of necessary actions and
instrumentation management algorithms for each
scenario:
n
preventiondthe patient is identified preoperatively to be at high risk for having CJD,
n containmentdthe patient is identified intraoperatively to be at high risk for having CJD, and
www.aornjournal.org
n
contingencydthe patient is identified postoperatively to be at high risk for having CJD.67
When deliberating revision of current policies or
establishing new ones, it is important to customize
them to individual facility needs, but even more
imperative is to take a multidisciplinary approach
in ensuring that all aspects of patient care, operations management, ethical and legal perspectives,
and public health are addressed. This is especially
critical in cases where CJD is diagnosed postoperatively in patients not previously known to be
at risk.
When proper protocols and policies are in place,
orchestrating a successful CJD case is still a team
approach. Ann Poggioli, RN, BA, CRNFA, is a
neurosurgical coordinator at a neuroscience center
in New Jersey with such experience. She claims
that “communication is vital” when it comes to
providing care during a CJD procedure, for which
the biggest challenge is “getting all the players on
the same page” (verbal communication, March 15,
2014). At Poggioli’s facility, the CJD procedure is
not scheduled until the surgeon completes a risk
assessment tool (Figure 4). After this tool is processed, the coordinator handles communication
with personnel from the infection control, environmental services, central processing, anesthesia,
and pathology departments. This aids in effective
implementation of infection control practices
across all departments and throughout the continuum of care.
Preparation of personnel requires special attention. According to the UK Department of Health,
“only trained staff, who are aware of the hazards,
carry out invasive procedures that may lead to
contact with medium- or high-risk tissue.”68 The
WHO has expanded on this concept by advising
that only the minimum number of people needed be
present for the procedure.42 To that end, it may be
prudent to hold a huddle meeting with all team
members involved in the patient’s care; during this
time out, the team should discuss logistics and
infection control practices and assign a facilitator
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October 2014 Vol 100
KARASIN
No 4
Figure 4. The surgeon should complete a Creutzfeldt-Jakob disease (CJD) risk assessment tool before surgery
that is scheduled for a patient who has or is suspected of having CJD.
who can be a liaison between the OR suite and
other facility departments to obtain perioperative
needs (eg, anesthesia equipment, sterile surgical
supplies).58,69,70 Finally, when scheduling the surgery, the perioperative manager should consider the
time required for postoperative terminal cleaning,
which may render the OR unavailable. Thus, it may
be advisable to schedule CJD cases as the last
procedure of the day.70
this article can provide current guidance when
facing this scenario and may pose providerreflecting questions, such as the following:
CONCLUSION
Prion diseases pose complex concerns for all health
care professionals and should be approached with
caution. Despite the rarity of CJD, it is very likely
that members of a neurosurgical team will care for
a patient with CJD at some point in their career.
When that happens, it is critical to adhere to the
most up-to-date, evidence-based practices to ensure
proper infection control, operations management,
and patient-centered care. Information covered in
Perhaps the only clear answer to these questions
is that the scientific community is still in the developmental stages related to aspects of CJD, such as
screening, ease of disinfection, and types of treatment options. Furthermore, future approaches to
surgical procedures are bound to change and will
require consistent reevaluation of up-to-date research
to ensure proper nursing interventions when caring
for a patient diagnosed with or suspected of
having CJD.
404 j AORN Journal
n
Are there systems and policies in place for
managing logistics, instrumentation, disinfection, and exposure to prions?
n Is the specialty team well educated on the disease
process, proper infection control, symptoms, and
accompanying nursing considerations?
SPECIAL NEEDS POPULATIONS: CJD
Acknowledgments: The author thanks Beth
Karasin, BSN, RN, CNOR, RN first assistant, for
providing the topic and the courage to write about
it; Pat Regenberg, librarian at Overlook Medical
Center, Summit, NJ, for her help in gathering
literature; Katie Glisic, National Prion Disease
Pathology Surveillance Center, Cleveland, OH, for
her content input; and Ermias Belay, MD, associate director for epidemiologic science, Division
of High-Consequence Pathogens and Pathology,
National Center for Emerging and Zoonotic
Infectious Diseases, Centers for Disease Control
and Prevention, Atlanta, GA, for publishing the
most current prion guidelines and allowing their
distribution.
Editor’s note: Lyodura is a registered trademark of
B. Braun Melsungen AG, Melsungen, Germany.
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Mark Karasin, BSN, BA, RN, is an RN at
Overlook Medical Center, Summit, NJ, and a
DNP student at Rutgers, The State University of
New Jersey, Piscataway Township. Mr Karasin
has no declared affiliation that could be perceived
as posing a potential conflict of interest in the
publication of this article.
AORN Journal j 407
EXAMINATION
CONTINUING EDUCATION
Special Needs Populations:
Perioperative Care of the Patient
With Creutzfeldt-Jakob Disease
2.6
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PURPOSE/GOAL
To provide the learner with knowledge specific to caring for the surgical patient
who has or is suspected of having Creutzfeldt-Jakob disease (CJD).
OBJECTIVES
1.
2.
3.
4.
5.
Describe prions as they relate to CJD.
Discuss the etiology of the types of CJD.
Discuss the symptoms of CJD.
Compare the different methods for diagnosing CJD.
Describe perioperative considerations for a patient who has or is suspected of
having CJD.
The Examination and Learner Evaluation are printed here for your convenience. To receive continuing education credit, you must complete the online
Examination and Learner Evaluation at http://www.aorn.org/CE.
QUESTIONS
1.
2.
Prions stand out from typical infectious agents
such as bacteria or viruses because they
a. have no nucleic acid and cannot reproduce.
b. are only present in brain tissue.
c. cannot be acquired by consuming contaminated foods.
d. can affect all life forms, including invertebrates
and fish.
The majority of CJD cases are _______, but its
cause remains unknown.
a. familial
b. iatrogenic
c. sporadic
d. new variant
408 j AORN Journal October 2014 Vol 100
No 4
3.
Transmission of CJD via medical interventions is
classified as
a. familial.
b. iatrogenic.
c. sporadic.
d. new variant.
4.
The cardinal sign of CJD is
a. blindness.
b. deterioration of muscle coordination.
c. personality changes.
d. rapidly progressive dementia.
5.
A diagnosis of CJD may be determined by
1. color Doppler ultrasound.
2. magnetic resonance imaging.
Ó AORN, Inc, 2014
CE EXAMINATION
3.
the presence of 14-3-3 protein in the patient’s
cerebrospinal fluid.
4. the presence of specific peaks on electroencephalography readings.
5. tissue biopsy or autopsy.
a. 4 and 5
b. 1, 2, and 3
c. 2, 3, 4, and 5
d. 1, 2, 3, 4, and 5
6.
The incubation period for CJD can be as long as
50 years before symptoms appear.
a. true
b. false
7.
According to the World Health Organization,
______________ have to be maintained when
providing routine nursing care to a patient with
suspected or confirmed CJD.
a. airborne precautions
b. contact precautions
c. reverse precautions
d. only standard precautions
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8.
Certain materials used to make surgical instruments (eg, nickel) may significantly decrease the
level of protein absorption and residue.
a. true
b. false
9.
The CJD prions are susceptible only to conventional methods of disinfection and sterilization.
a. true
b. false
10.
To lessen the time required to perform proper
postoperative cleaning, OR personnel should
1. allow only essential items in the room.
2. allow only one of each instrument to be
placed in the instrument tray.
3. cover all pedals and cords with a plastic bag
and secure them in place.
4. cover equipment, the walls, the bed, and
the floor of the working area with sheets of
polyvinyl chloride.
a. 1 and 3
b. 2 and 4
c. 1, 3, and 4
d. 1, 2, 3, and 4
AORN Journal j 409
LEARNER EVALUATION
CONTINUING EDUCATION PROGRAM
Special Needs Populations:
Perioperative Care of the Patient
With Creutzfeldt-Jakob Disease
T
his evaluation is used to determine the extent to
which this continuing education program met
your learning needs. The evaluation is printed
here for your convenience. To receive continuing
education credit, you must complete the online
Examination and Learner Evaluation at http://www
.aorn.org/CE. Rate the items as described below.
OBJECTIVES
To what extent were the following objectives of this
continuing education program achieved?
1. Describe prions as they relate to Creutzfeldt-Jakob
disease (CJD).
Low 1. 2. 3. 4. 5. High
2. Discuss the etiology of the types of CJD.
Low 1. 2. 3. 4. 5. High
3. Discuss the symptoms of CJD.
Low 1. 2. 3. 4. 5. High
4. Compare the different methods for diagnosing
CJD. Low 1. 2. 3. 4. 5. High
5. Describe perioperative considerations for a patient
who has or is suspected of having CJD.
Low 1. 2. 3. 4. 5. High
CONTENT
6. To what extent did this article increase your
knowledge of the subject matter?
Low 1. 2. 3. 4. 5. High
7. To what extent were your individual objectives
met? Low 1. 2. 3. 4. 5. High
410 j AORN Journal October 2014 Vol 100
No 4
2.6
www.aorn.org/CE
8. Will you be able to use the information from this
article in your work setting? 1. Yes 2. No
9. Will you change your practice as a result of reading
this article? (If yes, answer question #9A. If no,
answer question #9B.)
9A. How will you change your practice? (Select all that
apply)
1. I will provide education to my team regarding
why change is needed.
2. I will work with management to change/
implement a policy and procedure.
3. I will plan an informational meeting with
physicians to seek their input and acceptance
of the need for change.
4. I will implement change and evaluate the
effect of the change at regular intervals until
the change is incorporated as best practice.
5. Other: ________________________________
9B. If you will not change your practice as a result of
reading this article, why? (Select all that apply)
1. The content of the article is not relevant to my
practice.
2. I do not have enough time to teach others
about the purpose of the needed change.
3. I do not have management support to make a
change.
4. Other: ________________________________
10. Our accrediting body requires that we verify
the time you needed to complete the 2.6 continuing education contact hour (156-minute)
program: _______________________________
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