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Case Study: Creutzfeldt-Jakob Disease (CJD) Jo Ann Miller, BSN, RN, CCRN Trauma Program Manager Deb Hess, RN, CIC Clinical Supervisor, Infection Control.

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Presentation on theme: "Case Study: Creutzfeldt-Jakob Disease (CJD) Jo Ann Miller, BSN, RN, CCRN Trauma Program Manager Deb Hess, RN, CIC Clinical Supervisor, Infection Control."— Presentation transcript:

1 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

2 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

3 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

4 Case Presentation December 2013 presented to PCP:  Chief Complaint  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.

5 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

6 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

7 Case Presentation Neurology Plan:  Brain MRI  EEG  Lumbar puncture/CSF analysis  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

8 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 14-3- 3 protein levels, and positive Rt-QuIC test. Lab work: unremarkable except for an elevated cholesterol level of 217.

9 Differential Diagnosis  Alzheimer’s Disease  Dementia with Lewy Bodies  Frontotemporal Dementia  Meningoencephalitis  Corticobasal Degeneration  Progressive Supranuclear Palsy  CVA  Seizures  Opioid withdrawal OR………….

10 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

11 Creutzfeldt Jakob Disease First reported by German Neurologists:  Hans Gerhard Creutzfeldt in 1920  Shortly thereafter by Alfons Maria Jakob

12 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

13 Creutzfeldt Jakob Disease


15 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)

16 Creutzfeldt Jakob Disease Three major categories of CJD:  Sporadic 80% most common  Hereditary 3-10%  Acquired <1%  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)

17 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.

18 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.

19 Case Presentation 6 months after dementia type symptoms noted:

20 Case Presentation Self-Inflicted Transtentorial GSW to head:  Code T  Intubated  To CT  EMR – Electronic Medical Record Documentation  Brain matter exposure  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  Trauma Bay closed for ~18 hours

21 CJD: The Role of Infection Control & Prevention Deb Hess RN, CIC

22 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

23 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)

24 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

25 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

26 CT scan of head performed: Confirmed that death would occur Family requested life support be continued until others arrived Patient admitted to Trauma Neuro Unit (TNU) from CT scan room Chaplain Need for containment of all equipment and supplies taken into CT Room Notify TNU of diagnosis; remove unnecessary supplies within room before patient arrival. Another area requiring containment of in room supplies & equipment Trauma team Safety Officer Nursing Adm.

27 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) Ambulance Trauma Bay Hallways TNU Room 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 Environ- mental Services

28 Patient’s house? HazMat notified and decontaminated room where incident occurred Manager Safety Pa Department of Health CJD is reportable disease – Instructed to follow CDC Guidelines (we were already doing that) Family reported to IC that they were previously told that the CDC “wanted his brain” Notified PA DOH CDC 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. Notified coroner of planned events Coroner

29 Patient: Support withdrawn the following day and patient expired soon thereafter. 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: – Provided funeral director with CDC guidelines for “Funeral Directors” Funeral Director Nursing consideration

30 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 PR

31 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”!

32 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


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