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From: TSS ()
Subject: Re: Creutzfeldt-Jakob Disease in the Obstetric Patient
Date: October 19, 2005 at 11:39 am PST

In Reply to: Creutzfeldt-Jakob Disease in the Obstetric Patient posted by TSS on October 19, 2005 at 9:54 am:

546 JOGNN Volume 34, Number 5
CASE STUDY
Creutzfeldt-Jakob Disease
in the Obstetric Patient
Randa Sperling, Karen Haak, Diane Hesson, and Barbara Blanz Hidde
Recent reports have indicated the presence of
transmissible spongiform encephalopathy or
Creutzfeldt-Jakob disease in the United States. This
disease can occur as a rare, sporadic disease with no
recognizable pattern of transmission or as a familial
disease associated with prion protein gene mutations.
This article discusses the presence of sporadic
Creutzfeldt-Jakob disease in a woman who became
pregnant early in the course of the disease and subsequent
care pre- and postdelivery. JOGNN, 34,
546-550; 2005. DOI: 10.1177/0884217505280277
Keywords: Creutzfeldt-Jakob—Prion—Transmissible
spongiform encephalopathy
Accepted: November 2004
In 1999, the World Health Organization (WHO)
published a report titled “WHO Infection Control
Guidelines for Transmissible Spongiform Encephalopathies.”
This document provided guidance for
medical and infection control practitioners to develop
guidelines for use in caring for patients with
transmissible spongiform encephalopathy or
Creutzfeldt-Jakob disease (CJD), known to the public
as “mad cow disease.” The December 2003 diagnosis
of a case of bovine spongiform encephalopathy
in a dairy cow in Washington State brought this disease
sharply into focus as a significant concern in
protecting the food supply in the United States from
tainted animal products (Centers for Disease Control
and Prevention [CDC], 2004).
Transmissible spongiform encephalopathies in
humans generally occur as rare, sporadic diseases
with no recognizable pattern of transmission or as a
familial disease associated with prion protein gene
mutations. Eighty-five to 90% are classified as sporadic
(sCJD) that affect predominately older age
groups. Five to 10% are familial and are caused by
a gene mutation. Less than 5% are iatrogenic, or
transmitted by contaminated surgical equipment,
corneal or dural transplants, or treatment with
human-derived pituitary growth hormone (CDC,
2003).
Variant (vCJD) affects mainly young people. A
recent outbreak of a new variant form of CJD
(vCJD) has occurred in humans linked with foodborne
transmission of the bovine spongiform
encephalopathy agent. Sporadic CJD (sCJD) has a
worldwide death rate of about 1 case per million
people each year and typically affects people
between 55 and 75 years of age (WHO, 1999).
Prion diseases are a diverse group of neurologic
disorders that occur in familial forms affecting both
animals and humans. This is believed to be caused
Transmissible spongiform
encephalopathies generally occur
as rare, sporadic diseases with no
recognizable pattern of transmission or
as a familial disease associated with
prion protein gene mutations.
September/October 2005 JOGNN 547
by abnormally folded proteins called prions (WHO,
1999). Prions are infectious particles smaller than a virus
and are the only infectious pathogens that lack either
DNA or RNA (Belkin, 2003). Clinical expressions of the
disease include rapidly advancing dementia, progressive
unsteadiness and clumsiness, visual deterioration and
speech abnormalities, myoclonus, and a number of other
neurological signs and symptoms. Nonspecific EEG
anomalies are noted in most patients. Death usually
occurs within 1 year (Crawford, 1998). The final diagnosis
relies on examination of brain tissue obtained by biopsy
or at autopsy. During this examination, characteristic
spongiform changes in the parenchyma are noted.
Similar diseases are noted as occurring naturally in
some animal species. These include scrapie in sheep and
goats, chronic wasting disease in deer and elk, and infections
that occur after exposure of susceptible species to
infected animal tissues (transmissible mink encephalopathy,
bovine spongiform encephalopathy, and spongiform
encephalopathy in domestic cats and a variety of captive
zoo animals) (WHO, 1999).
Transmissible spongiform encephalopathy is not
known to spread from person to person, but transmission
can occur during invasive medical intervention or through
the use of human cadaveric-derived pituitary hormones,
dural and corneal homografts, and contaminated neurosurgical
instruments. Generally speaking, the patient who
is admitted with transmissible spongiform encephalopathy
does not present a risk to health care workers, family,
or community. However, as with all patients, prudent use
of standard precautions is recommended.
Prior to performing a surgical procedure on a patient
with known CJD, the infection control team should be
notified. It is essential that the procedure be carefully
planned including the handling, storage, cleansing, and
decontamination or disposal of instruments. Single-use
items are advisable. Reusable instruments must be
cleaned following specific guidelines as outlined by
WHO. Instruments should be kept moist and cleaned as
soon as possible after use to prevent drying of tissues,
blood, and body fluids onto the item, which renders it
much more difficult to clean. Prions are resistant to a
number of standard disinfection and sterilization procedures
including steam sterilization, exposure to dry heat,
ethylene oxide gas, and chemical disinfection using alcohol,
formaldehyde, or glutaraldehyde. Items that cannot
be thoroughly cleaned must be incinerated. This includes
instruments used during the induction of anesthesia as
well as needles, especially those that contact cerebral
spinal fluid directly, such as those used for saddle blocks
and other segmental anesthetic procedures (Association of
periOperative Registered Nurses, 2002).
During pregnancy and childbirth, no particular precautions
are recommended unless invasive procedures are
performed (WHO, 1999). Method of delivery must be
based on the progression of the disease and patient mentation
at the time of delivery, and a cesarean section may
be required. The type of delivery will be dependent upon
the patient’s mentation, her ability to cooperate with
physicians, and the capability of controlling contact with
blood and amniotic fluid. Precautions must be taken to
reduce the risk of exposure to the placenta and any associated
material. After delivery, standard precautions must
be used consistently to avoid contact with lochia, wound
exudates, and other bodily secretions, including breast
milk. Although these substances are not known to be
vehicles of transmission, caution must be taken until further
studies are completed.
Case Report
Mrs. X is a 41-year-old, gravida 5 para 3012 White
female with a 9-month history of sCJD. She initially
became symptomatic in March 2003. Throughout the
summer, she experienced progressive behavioral regression,
and eventually no longer performed simple activities
of daily living, ate, or slept through the night.
In early April, Mrs. X became pregnant. Because the
disease had not progressed to a point where CJD was suspected,
diagnostic testing to rule out other causes of the
neurological symptoms was performed. She developed
debilitating weight loss, poor sleeping habits, memory
loss, difficulty with concentration and understanding, and
aggressive behavior. As the disease progressed, CJD was
considered as a differential diagnosis. It was deemed
unlikely, as the patient had no history of being out of the
country, having eaten beef in or from Canada or England,
having a blood transfusion or corneal transplant, or
receiving human growth hormone.
During the latter part of July, a brain biopsy was performed
and the diagnosis of spongiform encephalopathy
was confirmed; severe spongiform changes were noted
throughout the layers of the cerebral cortex. A sample of
frozen brain tissue was sent to the National Prion Disease
Pathology Surveillance Center (NPDPSC), and sporadic
CJD was diagnosed. Eighty-five percent of CJD cases are
sporadic with no verified source of transmission. Familial
and iatrogenic cases account for the remainder (Tyler,
2003). The patient was then referred to the maternal-fetal
Prion diseases are neurologic disorders
that affect both animals and humans.
548 JOGNN Volume 34, Number 5
medicine specialists at a university-affiliated medical
center.
Because there had not been another documented case
of pregnancy in a patient with sporadic CJD, there were
no guidelines for prenatal care or delivery. Concern was
immediately expressed not only for fetal well-being but
also for patient management during the remainder of the
pregnancy. Of initial importance was adequate nutrition
to promote fetal growth. Because the patient had stopped
eating independently, the involvement of her husband and
his parents was essential to ensure adequate caloric
intake. Incidentally, weight gain was adequate throughout
the pregnancy.
Concern was raised over possible transmission of the
prion to the fetus. Because there were no prior cases, it
was impossible to predict whether the fetus would develop
the disease owing to prenatal exposure. The NPDPSC
in the Division of Neuropathology at Case Western
Reserve University was contacted, and samples of amniotic
fluid were sent for potential isolation of the prion.
Finally, literature was researched to identify appropriate
precautions to take during delivery of the fetus. Because
the patient had experienced a prior classical cesarean section
and to better control both patient and body fluids, a
repeat cesarean section was scheduled for 36 weeks of
gestation. This would prevent spontaneous labor resulting
in increased risk of exposure to fluids such as blood
and amniotic fluid.
Planning for Delivery
Due to the unusual circumstances, a care conference
was held to discuss antenatal care and plan for the delivery.
Included in the conference were neurology residents,
maternal fetal medicine specialists, residents who would
perform the cesarean section, nursing and surgical staff,
and nursing administration from neonatology.
The conference was held at approximately 35 weeks
into the pregnancy. After a presentation by neurology, discussion
ensued as to how best to handle contaminated
instruments, linens, and drapes; how to prepare the room
for the surgery; how the staff should garb for the procedure;
what labs would be needed during the procedure;
and the location of the biopsy sites for tissue samples as
requested by NPDPSC. The patient’s postpartum course
would require use of restraints and housing in the obstetric
ICU for one-to-one nursing care owing to the level of
dementia. Although the sample of amniotic fluid sent to
the NPDPSC was reported as free of prions by Western
blot analysis, it was decided that the baby would be sent
to the Special Care Nursery specifically due to the availability
of an isolation room.
Delivery
The patient was admitted for the cesarean delivery at
35 weeks gestation. Fixed operating room equipment was
draped with disposable covers to shield the surfaces from
potential splashes of body fluids. The tabletop was
marked for disposal, and a solution of sodium hydroxide
was available for unanticipated spills on structural surfaces
or nondisposable items. All instruments used in the
procedure were to be kept moist, then cleaned, sterilized,
and, finally, discarded. The surgeons were gowned using
plastic aprons, lubri-gowns, double layers of protective
head and footwear, and double elbow-length gloves.
At the time of surgery, the patient was unable to communicate,
although she would follow simple commands
from her husband. Consent for the surgery was obtained
from her husband. She arrived in the operating room in
four-way wrist, ankle, and vest restraints. An intravenous
line was established, and a general anesthetic was administered.
General anesthesia was administered owing to her
aggressive behavior, advancing dementia, and difficulty
with concentration and understanding. Even if the patient
had been cooperative, manipulation of the spinal cord
and exposure to spinal fluid was not recommended owing
to the highly infectious nature of these materials. After
induction of anesthesia, a Foley catheter was placed.
Although family members are not normally allowed in the
surgical suite when patients are given a general anesthetic,
owing to this unique situation, her husband was
allowed to remain in the operating room until the baby
was delivered.
A Pfannenstiel skin incision was started using an electrocautery.
However, because it was not possible to fully
eliminate the smoke from the cautery, this technique was
discarded. Prior to the surgery, physicians had raised concerns
regarding potential transmission of prions through
inhalation of smoke created during tissue vaporization;
therefore, a scalpel was used. Once the uterus was
reached, the newborn’s head was elevated away from the
lower uterine segment and a liter of amniotic fluid was
removed using a 14-gauge needle and 60 cc syringe. The
baby was then delivered through a classical incision with-
Symptoms include rapidly advancing
dementia, progressive unsteadiness and
clumsiness, visual deterioration and speech
abnormalities, myoclonus, and a number of
other neurological signs and symptoms.
September/October 2005 JOGNN 549
out incident. Samples of the amniotic fluid, cord blood,
and placenta, a full thickness biopsy of the uterine wall,
and a biopsy of the uterus in the location of the placental
bed were collected and passed to the neuropathologist for
preservation and research. These samples were later
reported as negative for the CJD prion.
Mrs. X was moved to her hospital bed, restrained,
awakened from the general anesthetic, and extubated. She
was then moved to the recovery room in the care of an
experienced obstetric intensive care nurse. A morphine
patient-controlled analgesia was initiated at a continuous
rate of 2 mg/hr. She was given lorazepam (Ativan) for
periods of agitation. When the morphine patientcontrolled
analgesia was discontinued, pain was controlled
with oxycodone solution.
Postoperatively, Mrs. X. was noted to have increased
respiratory secretions that required frequent suctioning.
She remained in a vest and four-point restraints for periods
of significant agitation, because it would have been
difficult if not impossible to prevent her from both dislodging
her intravenous line and opening the fresh cesarean
incision. Attending staff members carried out frequent
assessments for comfort and restraint requirements, and
fluids were offered at least every hour. Her extremities
were observed for redness or skin breakdown, and personal
needs for warmth, privacy, and comfort were met.
One-to-one nursing care and strict standard precautions
were maintained, and her postpartum recovery was
uneventful. She was discharged from the obstetric ICU to
the care of her husband and family members with a healing
incision, good bowel sounds, and stable vital signs.
After discharge, the curtain surrounding the bed was
removed and discarded, along with the bed linen.
Care of Baby
In preparation for the delivery, the infant warmer was
protected with fluid-resistant drapes that were wrapped
around the mattress to prevent absorption and subsequent
contamination. Upon delivery, the newborn was
dried and bulb suctioned. He experienced mild respiratory
distress. This was in part due to the extended time from
initiation of anesthesia to delivery. He received bag-mask
ventilation at delivery for lack of respiratory effort. Initial
Apgar scores were 5 at 1 min and 7 at 5 min.
In the Special Care Nursery, Baby X was placed in an
isolation room. Anyone who provided care for this baby
wore gloves and observed strict standard precautions. All
wet linens were discarded. Linens that were not wet were
placed in regular biohazard linen bags. Diapers were
placed in regular biohazard trash.
The corrected gestational age of the newborn was 36
+1 weeks as determined by the neonatologist. His weight
was 2.61 kg., length was 45.5 cm, occiput-frontal circumference
34 cm. Crackles were noted bilaterally, so the
newborn was placed on 1 liter of room air per nasal cannula
until resolution at approximately 4 1/2 hr of age.
Due to his mother’s dementia, the baby was to be bottlefed.
He took oral feedings on the day of delivery and continued
to eat well throughout his hospitalization.
Once the newborn was stabilized, a special care nurse
took him to his mother’s room to attempt to facilitate
bonding. The baby’s father and grandparents were present.
There was no change in the level of maternal dementia,
and she ignored the presence of the baby. Normal
bonding was observed with father and grandparents.
Pediatric neurology and infectious disease physicians
were consulted. Neurology reported a normal examination
with intent to follow up in 1 to 2 months. Baby X’s
bilirubin level peaked at 9.7, which did not require phototherapy.
He subsequently passed the newborn hearing
screen and car seat study. His metabolic newborn screen
was normal.
At discharge, Baby X was taking 114 cc/kg/day of 20
cal formula with iron every 3 hr ad lib and was discharged
weighing 2.51 kg. He was not circumcised.
Discharge Planning
Initially Mr. X was interviewed to determine if in-home
help was needed to assist him in providing care for his
wife. He indicated that help was unnecessary at this time,
as his parents cared for the children and assisted in personal
care for Mrs. X, but that it may be required as the
disease and dementia progress. Follow-up appointments
with pediatric neurology were scheduled for Baby X, as
was the initial visit to his pediatrician. It is unknown if
there will be any sequelae from possible perinatal exposure
to prions in utero; however, there are no reported
cases of vertical transmission of CJD.
Mrs. X was closely followed by the neurology department,
and social services was in frequent contact. As the
disease progressed, Mrs. X experienced repeated hospitalizations
due to hypovolemia, anorexia, staph infection,
and repair of open wounds related to a fall. Home care
and later hospice care was initiated, and assistance with
personal care was provided. As of May 2004, Mrs. X was
in the terminal stages of the disease and was hospitalized.
Mr. X felt this was best, as he wished to avoid his wife
dying at home in the presence of their three children. Both
social services and chaplaincy were actively involved in
supporting the family.
Implications for Practice
In retrospect, it is doubtful that the extreme precautions
taken with this patient were necessary, as transmission
usually occurs through exposure to brain, spinal
550 JOGNN Volume 34, Number 5
cord, or other neural tissue. Because prions were not
found in the amniotic fluid, uterine muscle, or placenta,
most likely following strict universal precautions as for
any surgery would have been adequate in this case. However,
because there were no previous studies regarding
obstetric transmission of sCJD and there is no available
treatment to promote the comfort level of all staff, it was
decided that such caution was appropriate.
Diligent nursing care was extremely important, as Mrs.
X was at risk for reopening her incision during episodes
of agitation. Pain was managed by careful assessment and
measurement of vital signs, as she was unable to accurately
report her discomfort level. When she was switched
to oral medications, a regular schedule for administration
was followed to decrease the possibility of unrecognized
pain.
Teamwork, however, was the most important attribute
overall in providing safe, effective care for this patient.
From the onset, all professionals who were or would be
involved in any part of the process were kept informed of
changes and recommendations. When providing care for
a complicated patient, multiprofessional teamwork is
essential. This teamwork is the collective collaborative
effort of all those concerned with the care of the patient
and will lead to the most positive outcomes of care.
REFERENCES
Association of periOperative Registered Nurses. (2002, March).
Recommended practices for cleaning and caring for surgical
instruments and powered equipment. Retrieved October
3, 2003, from the Association of periOperative Registered
Nurses Web site: http://www.aorn.org
Belkin, N. (2003). Creutzfeldt-Jakob disease: Identifying prions
and carriers. Retrieved October 6, 2003, from the Association
of periOperative Registered Nurses Web site:
http://www.aorn.org
Centers for Disease Control and Prevention. (2003). Factsheet:
New variant Creutzfeldt-Jakob disease. Retrieved January
5, 2004, from www.cdc.gov/ncidod/diseases/cjd/cjd_fact
_sheet.htm
Centers for Disease Control and Prevention. (2004). Bovine
spongiform encephalopathy in a dairy cow—Washington
State, 2003. Morbidity and Mortality Weekly Report,
52(53), 3-7.
Crawford, L. M. (1998). Bovine spongiform encephalopathy.
American Journal of Infection Control, 26, 5-7.
Tyler, K. L. (2003). Creutzfeldt-Jakob disease. The New England
Journal of Medicine, 348, 681-682.
World Health Organization Communicable Disease Surveillance
and Control. (1999). WHO infection control guidelines
for transmissible spongiform encephalopathies. Geneva,
Switzerland: Author.
Randa Sperling, RN, BC, MSN, APN, is a clinical nurse specialist
in the Mother-Baby Care Center at Clarian Health Partners
at the Indiana University Medical Center, Indianapolis.
Karen Haak, RN, BSN, RRT, is a clinical manager in the Mother-
Baby Care Center at Clarian Health Partners at the Indiana
University Medical Center, Indianapolis.
Diane Hesson, RN, CM, is a clinical manager in the obstetric
ICU at Clarian Health Partners at the Indiana University Medical
Center, Indianapolis.
Barbara Blanz Hidde, RN, BSN, is a clinical educator in the
Special Care Nursery at Clarian Health Partners at the Indiana
University Medical Center, Indianapolis.
Address for correspondence: Randa Sperling, RN, BC, CNS,
APRN, Indiana University Medical Center, 550 N. University
Blvd., Room 2745, Indianapolis, IN 46202. E-mail: rsperling@
clarian.org.TSS



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