Transcript The Nursing Challenges of Caring for Patients with NORSE Syndrome

The Nursing Challenges of
Caring for Patients with
NORSE Syndrome
Melissa V Moreda RN BSN CNRN
disclosures
• Merz
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.
• 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.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.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
Day 1--Dilantin, Topamax, Pentobarb gtt,
Versed
Day 10– Dilantin, Topamax, Pentobarb gtt,
Keppra, Zonisamide
Day 20– Lidocaine/Magnesium gtt
Day 30— Dilantin, Topamax, Clonazepam,
Keppra,Phenobarb,Valproate, Ketamine
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
Electrical
activity of the
brain
Neuron
Abnormal
electrical
signal
What is the difference between
Seizures and Status Epilepticus
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?
• Continuous seizures lasting at least 5
minutes.
OR
• 2 or more discrete seizures between
which there is incomplete recovery of
consciousness.
SE=Medical
Emergency
Morbidity &
Mortality
Cellular Respiration &
Glycolysis
Demand ATP 250%
CBF to metabolic demand
Metabolic activity in
skeletal muscles
Oxygen and glucose =
cellular destruction and
death
SE=Medical
Emergency
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?
Failure
of max
doses of
dilantin
Benzos
not
stopping
the
seizures
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 Anticonvulsive
Internal Safety:
A seizure represents
actual danger to
brain tissue
Time is BRAIN
Returning to Case Study #1K.S.
• 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….
What’s going on here???
NORSE
New
Onset
Status
Refractory Epilepticus
What is it?????
• *2005 Wilder-Smith ,Lim, Teoh, Sharma,
Tan, Chen, et al documented and
claimed this phenomenon in Singapore.
11
7
0
11
15
24
65
50
92
100
• Of the 7 identified:
– Shortest stay: 7 days 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
• 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
• Status
Epilepticus
Clinical
Guidelines
2003
– Benzodiazepines
– Phenytoin loads
+ maintenance
doses
– Barbiturates
Why use these
meds???
Wilder-Smith et al
• Treatments included:
–
–
–
–
–
–
Benzodiazepines Levetiracetam
Phenytoin
IVIG
Valproate
Propofol
Thiopental
Topiramate
Benzos
• 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
BP
BP
BP
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
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
– 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
• 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
• 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
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
• 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
What do you do???
Medicinal Management of Refractory
Status Epilepticus
1st Maximize Phenytoin &
Benzodiazepine loads. This
usually controls 70% of patients.
2nd Midazolam boluses infusion,
Propofol, Barbiturates
3rdTopiramate, phenobarbital,
midazolam, pentobarbital,
valproate, levetiracetam,
lidocaine, ketamine,
thiopental, or isoflurane
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 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
• 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
• 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
• 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 Nmethyl-D-aspartate
antagonist –mediated
neurotoxicity
• Ubogu et al2003
• SE: Pyschotic
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 Therapies
• Hypothermia (31-35C)
• Corry, Dhar, Murphy, Diringer 2006
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 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
• 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 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 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.
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 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
• 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
Survivors!
– 4 AA female,
1 Asian female, 1 Lebanese
male, 1 caucasian male and female
4
Diagnosis:
• By exclusion
only
• When???
Workup Includes:
*Radiographic Imagery (ct,mri)
*Continuous eeg
*Serum samples: Heavy Metals,
Complex Virus, RMSF, Arbovirus,
Rabies, Leptospiral Abs,
Autoimmune
*Brain Biopsy
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?
• YOU TELL ME
Difference b/w TBI &NORSE?
Why does having an actual
diagnosis matter?
•
Merle Mishel PhD, FAAN- UNC Chapel Hill
Uncertainty in Illness Theory
Status Epilepticus
Seizure
Seizure
Refractory
Status
Epilepticus
Seizure
NORSE
Ladessa
I’m a NORSE SURVIVOR!!!!
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
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
Another Survivor!
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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
Please contact me with questions or
insight…
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