Transcript Infection Control’s Role - Lancaster General Health

Case Study:
Creutzfeldt-Jakob Disease
(CJD)
Jo Ann Miller, BSN, RN, CCRN
Trauma Program Manager
Deb Hess, RN, CIC
Clinical Supervisor, Infection Control
December 19, 2014
Case Presentation
60 y/o Caucasian male
PMH:
 Essential HTN
 Chronic back pain – lumbar region (minor disc bulging)
 Anxiety
 Thyroid CA
PSH:
 Partial thyroidectomy 2011
Social History:
 Married to wife X 30 years
 Two adult sons
 Works as a Controller – Medical LOA
Case Presentation
Medications:
Lisinopril 40 mg daily
Coreg 12.5 mg BID
Amlodipine 10 mg daily
OxyContin 10 mg every 12 hours back pain
Valium 5 mg every 8 hours PRN back spasms
Ativan 1mg every 8 hours PRN anxiety
Oxycodone 5 mg every 4 hours PRN back pain
Case Presentation
December 2013 presented to PCP:
 Chief Complaint
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Confusion – fire alarm in refrigerator, mixed laundry up
Delusional activity – bugs crawling on skin
Difficulty focusing – staring at TV for hours
Memory loss
Back pain with spasms; pain in left buttock extending down left leg
 Wife noted symptoms started in March 2013; slow progression over
past several months with exacerbation over past week.
 Patient was trying to cut back on narcotic and sedative use. Withheld
valium, oxycodone and OxyContin X 11 days.
 CT head – negative
 CMP, CBC – without abnormalities
 Plan: restart oxycodone, OxyContin and valium at lower doses/longer
time intervals.
Case Presentation
December 2013
PCP follow up visit:
 Patient feels as though symptoms improving but wife
states still has confusion and memory loss –
attempted to get into his car, but got into wife’s car
and did not know what to do; could not remember
son’s name.
 Chronic back pain
Plan:
Continue oxycodone, OxyContin, and valium
Referral to Neurology
Case Presentation
January 2014
Neurology Consult:
Assessment:
 HENT, CV, RESP, GI, GU, MS – negative assessment
 Neuro exam:
 MMSE: Alert and oriented, poor concentration and
attention; poor concentration for complex series of
thought; fairly normal memory recall; normal speech and
language
 CN’s 2-12 intact
Diagnosis:
 Memory Loss
 Cognitive Communication Deficit
Case Presentation
Neurology Plan:
 Brain MRI
 EEG
 Lumbar puncture/CSF analysis
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Glucose
Protein
Albumin
Cryptococcal
Lyme
West Nile
Enterovirus
CME
Toxoplasma
TAU Protein
14-3-3 Protein
 Labs:
 PT, PTT
 CC, ESR
 A1C
 ANA
 Thyroid panel
 Protein Electrophoresis
 SSB
 RPR VDRL
 ANCA C
 ANCA P
 Rheumatoid Arthritis Factor
 Angiotensin Converting Enzyme
 Vitamin B12, Folate
 Anti Hu
 Cytology
Case Presentation
Diagnostic Results (February 2014):
EEG: unremarkable; no seizure activity; no slowing
MRI Brain: Extensive confluent white matter disease in both cerebral hemispheres,
nonspecific in appearance. Demyelinating or dysmyelinating processes should be
considered. Vascular processes such as small vessel ischemic change or vasculitis should
also be considered. No mass or abnormal enhancement noted. Prominent cisterna magna
versus small retrocerebellar arachnoid cyst. (unremarkable)
MRI Brain Spectography: Spectra overall fairly unremarkable. There is no significant
choline elevation or NA depression. No findings to suggest a highly metabolic or rapidly
dividing cellular process such as a higher grade malignancy is evident. Demyelinating
pathology can sometimes have choline elevation and again there is no significant choline
elevation throughout the hemispheric white matter abnormality.
CSF: unremarkable except for highly elevated TAU protein of 1929 pg/mL, ambigous 14-33 protein levels, and positive Rt-QuIC test.
Lab work: unremarkable except for an elevated cholesterol level of 217.
Differential Diagnosis
Alzheimer’s Disease
Dementia with Lewy Bodies
Frontotemporal Dementia
Meningoencephalitis
Corticobasal Degeneration
Progressive Supranuclear Palsy
CVA
Seizures
Opioid withdrawal
OR………….
Diagnosis: Creutzfeldt Jakob Disease
(CJD)
(probable)
Neurology follow up visit:
 Probable CJD
 CSF sent to National Prion Disease Pathology Surveillance Center
 TAU Protein highly elevated (neuronal microtubules stabilization)
 Ambiguous 14-3-3 protein (surrogate markers of neuronal damage – not specific for sCJD)
 Positive Rt-QuIC test (specific for sCJD)
 BUT…
 No known exposure base on history
 No blood transfusions
 No transplants
 Lives close to a cattle farm but no exposure to slaughter house
 Diagnosis:
Very early stages of probable CJD
 Definitive diagnosis: BRAIN BIOPSY
 Plan:
 Repeat EEG and MRI in two months
 Follow up Neurology 2 months
Creutzfeldt Jakob Disease
First reported by German Neurologists:
Hans Gerhard Creutzfeldt in 1920
Shortly thereafter by Alfons Maria Jakob
Creutzfeldt Jakob Disease
Transmissible Spongiform Encephalopathy's (TSE’s):
Also known as prion diseases
 Proteinaceous infectious particle – infected protein that folds
 Causes other proteins to fold – damages neurons
Rare degenerative brain disorder
 Characterized by tiny holes that give the brain a spongy appearance:
 CJD is the most well-known of the human TSE’s or prion diseases
 Other Human prion diseases:
 Kuru – found in a isolated tribe in Papua New Guinea (extinct)
 Variant CJD (vCJD)
 Animal prion diseases:
 Bovine spongiform encephalopathy's (BSE’s):
 Mad Cow Disease
 Scrapie – sheep and goats
 Chronic Wasting Disease – elk and deer
Creutzfeldt Jakob Disease
Creutzfeldt Jakob Disease
Creutzfeldt Jakob Disease
Incidence:
World wide 1-2 in 1 million
In US ~300 cases per year
Age of onset: 60
90% die within a year
Diagnosis is difficult
Low index of suspicion
Lack of knowledge of this rare disease
Long incubation period (vCJD)
Creutzfeldt Jakob Disease
Three major categories of CJD:
Sporadic 80%
Hereditary 3-10%
Acquired <1%
most common
Sporadic – occurs for no apparent reason
Inherited or familial – family history / genetic mutation
Acquired r/t Contamination - exposed to contaminated
human tissue
 Cornea/skin transplant/brain surgery
Variant linked to beef infected with BSE (Bovine
Spongiform Encephalopathy)
Creutzfeldt Jakob Disease
Clinical features:
Rapidly progressive dementia
Multifocal neurological findings:
Myoclonus
Visual disturbances
Cerebellar and pyramidal / extrapyramidal signs
Rapid progression of cognitive and functional
impairment toward akinetic mutism and
eventual death within 6 months to 1 year.
Creutzfeldt Jakob Disease
Treatment:
• There is no known cure or effective treatment for
CJD. However, medications can be used to treat
some of the mental changes and personality
abnormalities that occur. Treatment is usually
focused on making patients comfortable and to
help them function safely in their environment.
Case Presentation
• 6 months after dementia type symptoms
noted:
Case Presentation
Self-Inflicted Transtentorial GSW to head:
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Code T
Intubated
To CT
EMR – Electronic Medical Record Documentation
 Brain matter exposure
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First Responders (EMS, Police, Fire)
Pre-hospital equipment
Trauma Bay staff
Trauma Bay equipment
CT scanner
 Infection Control – CDC, NIH, Prion Center
 Decontamination
 Incident Command
 Exposure to CJD
 Exposure to chemicals
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Trauma Bay closed for ~18 hours
CJD: The Role of Infection
Control & Prevention
Deb Hess RN, CIC
Role of Infection Control
Received call from Trauma Coordinator:
• Possible CJD gunshot wound to head
• What do we need to know or do that is
different from our standard of care?
All hands on deck!
• All Infection Control Nurses were given a task
using the “divide and conquer” methodology
Access before you Act
• Paged LGHP-ID physicians (Dr. Riley & Dr. Kontra)
• Promptly identified that this was a true case
– Electronic health record across the Alliance provided
access to outpatient & specialists records because
the providers were part of LG
• Other IC nurses were pulling guidelines and
standards from Centers for Disease Control
(CDC), World Health Organization (WHO), and
American Association for Infection Control &
Epidemiology (APIC)
Identified Key Information
• Brain matter highly contagious; given gunshot wound to
head, needed to protect our employees.
• (Considered blood to be contaminated with brain
tissue)
• Promptly sent one IC Nurse to EMD to assist with
verification that proper PPE was being worn!
Standard Precautions
• Blood or body fluid potential exposure; wear PPE.
• Trauma with excessive bleeding; Impervious gowns
• Potential of splatter; wear mask & eye protection
• Need for containment of all equipment and supplies within
trauma bay to determine appropriate cleaning or disposal
Identified that others needed “to know” & help needed!
• Director of IC & EMD notified
• Director of Quality and Patient Safety notified
• Senior Vice President of Operations notified
• Risk Management notified
• Director Environment Services notified
• Brought expertise & “workers” to scene
Ambulance & Crew
• How to decontaminate?
• Ambulance taken out of service – Instructed not
to leave until further direction provided
CT scan of head performed:
• Confirmed that death would occur
• Family requested life support be continued
Chaplain
until others arrived
Need for containment of all equipment
and supplies taken into CT Room
• Patient admitted to Trauma Neuro Unit (TNU)
Nursing
from CT scan room
Adm.
Trauma
team
Notify TNU of diagnosis; remove unnecessary
supplies within room before patient arrival.
• Another area requiring containment of in room
Safety
supplies & equipment
Officer
Supply & Equipment Management
All equipment that could not be decontaminated with bleach
(1:5 ratio) for 60 minutes would require incineration
•
Identified per CDC, WHO & APIC guidelines
Infection Control Physicians & Nurse Manager did a systematic
approach to the environment:
Is item able to be cleaned with bleach?
• If yes - “one bucket”
• If no – “second bucket for incineration”
• Log items to be incinerated and owner as required
replacement
Surfaces cleaned with Bleach – 1 hour exposure (Close Vents in
area to protect other hospital areas from bleach odor)
Environmental
Services
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Ambulance
Trauma Bay
Hallways
TNU Room
Manager
Patient’s house?
Safety
• HazMat notified and decontaminated room where
incident occurred
Notified
PA DOH
Pa Department of Health
• CJD is reportable disease – Instructed to follow CDC
Guidelines (we were already doing that)
CDC
Family reported to IC that they were previously
told that the CDC “wanted his brain”
IC Manager called CDC - Connected to Emergency Operations transferred to Dr. Ryan Maddox of the Prion Division –
instructed us to call National Prion Disease Pathology
Surveillance Center in Cleveland Ohio for review of case.
Coroner
Notified coroner of planned events
Patient: Support withdrawn the following day and
patient expired soon thereafter.
Nursing
consideration
TNU staff wrapped head in several pads
to prevent pooling of blood within body bag.
Brain Harvest Requested by National Prion Disease Pathology Center
• Autopsy to be done free of charge for confirmation of diagnosis
• LGH refers autopsy requests to Hershey Medical Center (HMC)
– Refused to perform autopsy due to risk
• Called Coroner
– Refused as they legally can only do autopsy when cause of
death unknown
Identified Funeral Home:
Funeral
Director
– Provided funeral director with CDC
guidelines for “Funeral Directors”
PR
Public relations:
• Family concerned about “news coverage” of incident
• Team met and prepared statement and talking points
in the event LGH was contacted to make a statement.
Family’s wishes for privacy honored with no case
specific information / identity to be shared
Emotional Toll of Healthcare Workers
• Two hospital minor exposures – occurred prior to
identification of diagnosis
• All involved felt the emotional impact – we provide
care in a dangerous environment
Financial toll:
• Multiple items were unable to be bleached
• Incinerated items from LEMSA, EMD, and personal
clothing/stethoscopes
• Cost was in the thousands of $$$$
• Debriefing
• Several weeks later, called team together to review
case. Included all involved except family.
• Reviewed what happened
• Reassured all that CDC & Centers for Prion diseases
were involved behind the scenes from the beginning.
• They stated that we went “above and
beyond” to protect everyone
• “We were in unchartered territory”!
Lessons Learned
• Demonstrated the Value of Electronic Medical Record –
diagnosis was identified early in care
• Do we need to place CJD in the header; so it is seen
immediately? Yes
• Reviewed Exposure Policy – currently under revision
• Reinforced need for impervious gown use in trauma
bay
• Need greater awareness of splash potential; use of
goggles/eyewear protection
• Gloves: Switching to higher quality to prevent tears
and better wrist coverage