the nursing challenges of caring for patients with norse syndrome melissa v moreda rn bsn cnrn
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The Nursing Challenges of Caring for Patients with NORSE Syndrome Melissa V Moreda RN BSN CNRN. disclosures. Merz . KS is a 28 yo Korean American female Admitted to OSH flu-like symptoms for 4-5 days. nausea/vomiting and 105.0 Fever.

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case 1 k s
KS is a 28 yo Korean American female

Admitted to OSH

flu-like symptoms for 4-5 days.

nausea/vomiting and 105.0 Fever.

She became disoriented and confused & had a witnessed tonic clonic seizure.

Recurrent seizures continued.

Case #1 K.S.
case 1 k s4
Case #1 K.S.

Transferred to Duke in Status Epilepticus

Workup unremarkable

NO past hx of childhood epilepsy, febrile seizure, no head trauma, no meningitis

NO family hx of sz, no predisposing evidence

case 1 k s5
Case #1 K.S.

Social hx: ANY exposure--- what do they work with? any unusual circumstances or any family/friends with recent sickness?

Habits: drinking/drugs/etc

Allergies: none

Medications (any herbals/supplements)

Extensive negative workup

case 1 k s6
Case #1 K.S.

Initial lines of anticonvulsants started.

Quick escalation of medication dosages and additional anticonvulsants added.

Seizures continued.

Paralytics added, max therapies reached.

case 1 k s hospital course
Case #1 K.S. - Hospital Course

Day 1--Dilantin, Topamax, Pentobarbgtt, Versed

Day 10– Dilantin, Topamax, Pentobarbgtt, Keppra, Zonisamide

Day 20– Lidocaine/Magnesium gtt

Day 30— Dilantin, Topamax, Clonazepam, Keppra,Phenobarb,Valproate, Ketamine

case 1 k s8
Case #1 K.S.

Day 40--Ativan gtt, MgSo4, Keppra, Topamax, Valproate, Phenobarb

Day 50 -- Ativan,Keppra,Topamax, Phenobarb, Memantine, Ketamine gtt

Day 62 -- Status continuing

Uroseptic - death within 1 hour of foley exchange

slide9

Electrical activity of the brain

Neuron

Abnormal electrical signal

What is the difference between

Seizures and Status Epilepticus

what is seizure
What is seizure?

A single (finite) event of abnormal discharge in the brain that results in an abrupt and temporary altered state of cerebral function.

what is status epilepticus
What is Status Epilepticus?
  • Continuous seizures lasting at least 5 minutes.
  • 2 or morediscrete seizures between which there is incomplete recovery of consciousness.

OR

SE=Medical Emergency

slide12

Morbidity & Mortality

SE=Medical Emergency

status epilepticus
Status Epilepticus
  • Repeated partial seizures manifesting as focal motor convulsions, focal sensory symptoms, or focal impairment of function (ie: aphasia not associated with LOC)
  • Tonic-clonic most common type.
  • Convulsive more easily seen clinically. Partial less obvious and more difficult to identify. Subclinical only identifiable on cEEG.
  • Wittman & Hirsch--Neurocritical Care 2005: increasing awareness of nonconvulsive seizures in critically ill
what is refractory se
What is refractory SE?

Failure

of max

doses of

dilantin

Benzos

not

stopping

the

seizures

safety nursing care convulsive vs anticonvulsive
Safety & Nursing Care: Convulsive vs Anticonvulsive

External Safety:

padded rails

suction present

bed in low position

pulse ox/ vitals

staying with the patient

obtaining benzos & AEDS

Family needs education and reassurance

safety nursing care convulsive vs anticonvulsive16
Safety & Nursing Care: Convulsive vs Anticonvulsive

A seizure represents actual danger to brain tissue

Time is BRAIN

Internal Safety:

returning to case study 1k s
Returning to Case Study #1K.S.

What’s going on here???

KS has the flu, spikes a fever, starts seizing and doesn’t stop despite multiple line anticonvulsants. Full life support.

Completely negative workup.

Family, Nurses, MD’s, PRM’s, want to know….

norse
NORSE

New

Onset

Status

Refractory

Epilepticus

what is it
What is it?????

*2005 Wilder-Smith ,Lim, Teoh, Sharma, Tan, Chen, et al documented and claimed this phenomenon in Singapore.

slide21

11

7

15

24

65

92

11

100

0

50

  • Of the 7 identified:
    • Shortest stay: 7days resulting in death

-11 days: 1 patient survived, but in persistent vegetative state with frequent seizures.

      • The other one died.
      • Longest stay: 92 days survived but in persistent vegetative state with frequent seizures as well.
norse eeg relationships
NORSE-EEG Relationships

Initial eeg captured ictal discharges in all.

In 3, eeg showed ictal discharges from bilateral fronto-temporal regions with no side preference.

In 1, continuous parasagital ictal discharge.

In 3, fronto-temporal discharges originating from the right on 2 occasions and once on the left

why use these meds
Why use these meds???
  • Status Epilepticus Clinical Guidelines 2003
    • Benzodiazepines
    • Phenytoin loads + maintenance doses
    • Barbiturates
wilder smith et al
Wilder-Smith et al
  • Treatments included:
    • Benzodiazepines Levetiracetam
    • Phenytoin IVIG
    • Valproate
    • Propofol
    • Thiopental
    • Topiramate
benzos
Benzos

BP

BP

BP

  • Lorazepam (Ativan ):
    • increases action of GABA which inhibits neurotransmission, depressing all levels of CNS

Problem: hypotension, caution in renal or hepatic impairment, MG

ICUadmit*ICUadmit*ICUadmit*ICUadmit*ICUadmit

benzos26
Benzos
  • Diazepam (Valium):
  • Short acting , ½ life 16-90 minutes, the later ½ eliminated slower.
  • High does and accumulation of active diazepam metabolites = respiratory depression and hypotension
  • 5-20mg IV slowly at 1-2 mg/min Repeat 5-10 mg every 5-10 minutes to a maximum of 100mg/24 hours
benzos27
Benzos
  • Midazolam(Versed):
    • Large number of trials reflecting that this medication works
    • Rapid absorption into brain
    • Benign from a hemodynamic standpoint
    • Peds: success of breaking seizures without intubation
    • Load:0.2mg/kg, maintenance: up to 2 mcg/kg/min, most effective as a gtt
    • Problem: tachyphylaxis (wears off), accumulates in critically ill.
phenytoin fosphenytoin
Phenytoin /Fosphenytoin
  • may work in motor cortex, may stop spread of activity
  • Brain stem centers stop tonic phase of grand mal sz
  • 18-20mg/kg, if refractory – may use 30mg/kg
  • Problem: hypotension, purple glove syndrome, fever, cardiac death from widened QRS
refractory status epilepticus
Refractory Status Epilepticus
  • 30 minutes to 1 hour of seizure activity not broken
  • No interval of time between failed treatment and next therapy
  • Start infusions of Benzodiazepines, Propofol, Barbitruates
  • Keep giving bolus doses
barbiturates
Barbiturates
  • Pentobarbital:
    • Extremely effective halting seizures on eeg by inducing coma
    • Load: 5-12 mg/kg, maintenance: 1-10 mg/kg

Problem: high doses suppress cardiac function,difficult to monitor levels, poor chemotaxis of wbc, paralysis of resp cilia, poikilothermia

Dr.Borel

barbiturates31
Barbiturates
  • Phenobarbital:
    • Introduced 1912
    • CNS depressant
    • elevates seizure threshold by decreasing postsynaptic excitation, possibly stimulating postsynaptic GABA inhibitor responses.
    • Sometimes given in combo with dilantin
    • Rapidly absorbed by all routes, 3-4 weeks to reach steady state
medicinal management
Medicinal Management

Traditional goal of therapy: achieve burst suppression on EEG 12-24 hours to control or prevent recurrent seizures.

Termination of the seizures should occur rapidly to minimize neural damage, correction of metabolic defects, and resuscitation

SE=Medical Emergency

slide33

When your clinical guidelines fail

in stopping seizures…

What do you do???

medicinal management of refractory status epilepticus
Medicinal Management of Refractory Status Epilepticus

1st Maximize Phenytoin & Benzodiazepine loads. This usually controls 70% of patients.

2ndMidazolam boluses infusion, Propofol, Barbiturates

3rdTopiramate, phenobarbital, midazolam, pentobarbital, valproate, levetiracetam, lidocaine, ketamine, thiopental, or isoflurane

medicinal management35
Medicinal Management
  • Other treatments we have tried
    • Antivirals upon arrival
    • Magnesium drips
    • Memantine
    • Ketamine drips
    • Prn ativan, phenobarb, pentobarb, versed
    • Giving “holidays” to reintroduce the medications, hopefully to “break through” the seizures.
wilder smith et al36
Wilder-Smith et al

Thiopental: anesthetic that is barbiturate based.

IVIG: ?autoimmune, does not cross the BBB

Propofol: difficult to suppress sz while titrating/ balancing hemodynamics

  • Rosetti et al 2004 burst suppression 31 patients/ 21 successfully suppressed
  • 1 interesting side effect: EPILEPTIFORM ACTIVITY!!!!
alternative therapies
Alternative Therapies

Robakis, Hirch 2006 lit review + expert neurointensivists/ epileptologists

Tx included: ketamine, gammaglobulin, plasmapheresis, steroids, adrenocorticotropic hormone, high dose phenobarb, isoflurane, lidocaine, ect, ketogenic diet, hypothermia, mag

lidocaine
Lidocaine
  • CNS depression with cessation of convulsions
  • Biphasic:
    • Blocks inhibitory CNS pathways resulting in stimulation
    • Blocks inhibitory/excitatory impulses resulting in CNS inhibition (Peralta 2007)
  • Walker, Slovis 1997 effective in peds not responding to barbs
  • Bolus + maintenance dose
  • Toxicity Rare
ketamine
Ketamine
  • Good Stops Seizures through anesthesia: NMDA antagonist with intrinsic sympathomimetic properties
    • 1-4.5 mg/kg
    • Borris et al 2000
  • Bad Neurotoxicity
      • Diffuse cerebellar atrophy
      • 44yo male, tx for status 3 months later- consistent with animal models N-methyl-D-aspartate antagonist –mediated neurotoxicity
      • Ubogu et al2003
      • SE: Pyschotic
using anesthesia for treatment of refractory status epilepticus
Using Anesthesia for Treatment of Refractory Status Epilepticus
  • Rosetti in Epilepsia 2007
    • Each anesthetic has advantages/risks
    • Depends on the protocols regarding duration and depth of sedation
    • The biological background of the patient remains the prognostic determinant of SE
alternative therapies41
Alternative Therapies

Hypothermia (31-35C)

Corry, Dhar, Murphy, Diringer 2006

alternative therapies42
Alternative Therapies
  • ECT: -1 second seizure is induced while anesthetized, wakes 10-15 minutes later
    • 3x week for up to 15 treatments, return to baseline, 1-2 more treatments
alternative therapies43
Alternative Therapies
  • Ketogenic Diet:
    • High fats, low carbs
    • Strict diet requiring medical supervision
    • Body burns fat instead of glucose
    • Kids usually on for 2 years
    • Should we consider this in tube feeds?
nursing considerations
Nursing Considerations
  • Airway/Breathing:vented, full support, possiblity of VAP, need for aggressive pulmonary toileting
  • Circulation: hemodynamically unstable due to AEDs/coma inducing meds requiring vasoactive agents, increased risk of DVTS
  • Disability: (Neuro exam): pupils sometimes work. Can’t see more damage due to coma
nursing considerations45
Nursing Considerations
  • Expose: what does their skin/lines look like? Usually generally edematous, pressure sores common. Med rashes.
  • Fahrenheit: Poikilothermic, immune

system suppressed, may need routine pan cultures

  • Family Finances…Increased anxiety
  • Gadgets: Scds, afo boots, hand splints, various machines for life support, eeg electrodes
nursing considerations46
Nursing Considerations
  • Head to Toe: Assessment of physical appearance. Oh, how I wish I could wash their hair.
  • IV’s: site appearance, correct iv doses, do you have enough access? Do you need to make more pentobarb or levophed? Are you waiting for more depakote or an abx?
  • JP/Drains:hopefully, not needed.
slide47

Nursing Considerations

  • Keep Family Informed: This is the tricky part. I usually want to cry at this point.
  • Labs: Are you therapeutic? Have any of the weirdo labs come back from Mayo or the state?
  • Legal: Have we discussed DNR?
nursing considerations48
Nursing Considerations
  • Meds:Besides the AEDs, anticoagulants, GI motility agents, ABX, acid reduction agents, blood products, SSI… Do you have any more room on your Medication Administration Record?
  • Movement: PROM
  • Nutrition: Are they actually getting any with their gut shut down? High residuals? Have they developed an ileus? Are they on a bowel regimen with daily stimulation?
norse at duke
??? NORSE at DUKE

4

Survivors!

  • In the last 10 years at Duke, we can identify at least 8 patients
    • All young (<35)
    • Previous good health
    • Initially Flu-like symptoms or pyschotic behavior, then status
    • Negative workup
    • 4 AA female, 1 Asian female, 1 Lebanese male, 1 caucasian male and female
diagnosis
Diagnosis:

By exclusion only

When???

workup includes
Workup Includes:

*Radiographic Imagery (ct,mri)

*Continuous eeg

*Serum samples: Heavy Metals, Complex Virus, RMSF, Arbovirus, Rabies, Leptospiral Abs, Autoimmune

*Brain Biopsy

workup includes52
Workup includes:
  • *CSF(culture,gram stains, cryptococcal antigens, herpes PCR polymerase chain reactions- replicate DNA)
  • *Stool (organisms/parasites)
  • *Infection Disease Consult
why does having an actual diagnosis matter
Why does having an actual diagnosis matter?

Merle Mishel PhD, FAAN- UNC Chapel Hill

Uncertainty in Illness Theory

slide56

Seizure

Seizure

Seizure

case study 2 k c
Case Study #2 K.C.
  • 19 yo female with URI and fever 101.0
  • Z pack started
  • 2 days later, in car with family, started seizing and turning blue, incontinent of urine.
  • OSH- tonic clonic sz, intubated
  • Negative primary workup
slide60

Case Study #2 K.C.

  • 2 days later extubated
  • Another sz, meds given, intubated
  • Sz continues, bradycardic and cyanotic
  • Transfer to Duke
  • 3 weeks after initial sz, off pentobarb, and smiling at dad
bibliography
Bibliography
  • Jirsch J. Hirsch LJ. Nonconvulsive seizures: developing a rational approach to the diagnosis and management in the critically ill population. Clin Neurophysiol.2007118(8): 1660-70.
  • Robakis TK, Hirsch LJ. Literature review, case report, and expert discussion of prolonged refractory status epilepticus. Neurocrit Care. 2006. 4(1):35-46.
  • Khaled KJ, Hirsch LJ. Advances in the Management of Seizures and Status Epilepticus in Critically Ill Patients. Crit Care Clin. 2007. (22):637-659.
  • Wilder-Smith EPV, Lim ECH, Teoh HL, Sharma VK, Tan JJH, Chan BPL, Ong BKC. The NORSE (New Onset Refractory Status Epilepticus) Syndrome: Defining a Disease Entity. Ann Acad Med Singapore. 2005, 34:417-20.
  • Hirsch LJ., Kull L. Continuous EEG Monitoring in the Intensive Care Unit. Am. J. END Technol. 2004 (44):137-158.
  • Hickey JV. The Clinical Practice of Neurological and Neurosurgical Nursing.Philadelphia: Lippincott, 1997.
  • Corry JJ, Dhar R, Murphy T, Diringer MN. Hypothermia for Refractory Status Epilepticus. Neurocrit Care 2008: 9(2):189-97.
  • Rosetti AO. Anesthesia for Treatment of Refractory Status Epilepticus. Epilepsia 2007. 48 (8):52-55
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