The Nursing Challenges of Caring for Patients with NORSE SyndromeMelissa 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. She became disoriented and confused & had a witnessed tonic clonic seizure. Recurrent seizures continued. Case #1 K.S.
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, 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.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. • 2 or morediscrete seizures between which there is incomplete recovery of consciousness. OR SE=Medical Emergency
Morbidity & Mortality 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 A seizure represents actual danger to brain tissue Time is BRAIN Internal Safety:
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 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 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 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??? • Status Epilepticus Clinical Guidelines 2003 • Benzodiazepines • Phenytoin loads + maintenance doses • Barbiturates
Wilder-Smith et al • Treatments included: • Benzodiazepines Levetiracetam • Phenytoin IVIG • Valproate • Propofol • Thiopental • Topiramate
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
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
When your clinical guidelines fail in stopping seizures… What do you do???
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 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 N-methyl-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 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: By exclusion only When???