OBJECTIVES
NEURODIAGNOSTICS
Wynne Bird, BSN, RN, CPN, CNRN
Epilepsy Program Coordinator
Children's Hospital Colorado
720-777-6895
0 Recognize different types of seizures
0 Describe common epilepsy syndromes
0 Apply practical skills and interventions
related to seizures
0 Understand medications used for seizure
types
Whether to treat after one
seizure?
Seizure vs Epilepsy
b Anyone can have a seizure under certain
circumstances. For instance, a fever, lack of
oxygen, head trauma, or illness could bring on
a seizure.
0 Diagnosed with epilepsy when they have
seizures that occur more than twice without a
specific cause.
s In most cases — about 1:10 — the cause is
unknown. This type of seizure is called
"idiopathic" or "cryptogenic," meaning that we
don't know what causes them.
WHAT IS EPILEPSY ?
Characterizes by at least 2 or more
unprovoked, recurring seizures. It's also called
a seizure disorder.
h Caused by an uncontrolled electrical discharge
from nerve cells in the cerebral cortex - this is
part of the brain that integrates higher mental
functions, general movement, perception, and
behavioral reactions.
0 Can affect all or part of the brain.
0
Everyone is allowed 2 seizures in their lifetime
since many seizures occur only once and don't
recur
0 Side effect of chronic medication treatment
weighed against benefit
0 If someone has > 2 seizures or if acute reason
for seizures (tumor, stroke, etc.), most
physicians will treat with medications
0
What does intractable
epilepsy mean?
a Seizures that are not controlled with
medications.
0 Failure of 3 or more epilepsy medications
0 A number of different terms may be used:
"uncontrolled," "intractable," "refractory," or
"drug resistant."
1
Etiology
• genetic factors
• metabolic or
chemical
imbalances- Glut-1
deficiency
• fever/infection
• birth trauma
• head/brain trauma
• progressive brain
disease
Stroke
Brain tumors
Cortical dysplasia
Mesial temporal
Sclerosis
Congenital defect hemimegalencephaly
Maternal drug
usage/medications
unknown reasons
Partial Seizures
0
0
0
0
More common than generalized
Occur in one or more specific locations
Can spread to wide regions of the brain
2 types of partial seizures
• Simple
• Complex
Types of Seizures
• Partial seizures - also called focal or localized
E
Generalized Seizures
B
Myoclonic
B
Status Epilepticus
S
Non-epileptic Events (NES)- pseudoseizures
S
Febrile Seizures
Simple Partial Seizure
b No loss of consciousness, but no control
a May experience confusion
a Unilateral uncontrolled movements
• Motor - blinking, tongue movement, facial twitching, jerky
movements
• Emotional - fearful feeling, d6jci vu, feeling something bad is
going to happen
• Sensations- buzzing in ears, voices that aren't there, unpleasant
smells, visual distortions, sudden nausea or odd feeling rising
in the stomach, tingling, numbness
• Usually has temporaiy weakness of certain muscles
after seizure
s May present with aura
B May be able to recall event after it is over
Partial seizures Brain Localization
Complex Partial Seizure (CPS)
s
e
h
0
s
a
Majority of childhood epilepsy
Post-ictal confusion
Used to be called psychomotor seizures.
80% originate in temporal lobe
Staring occurs - but longer than absence seizures and includes
movements
Can have loss of judgment, involuntary or uncontrolled behavior,
or loss of consciousness
• "Oroalimentary" Automatisms - Chewing, lip smacking,
repetitive movements of tongue/lips/jaw
• Hand Automatisms - picking at objects, aimless walking, blank
stare, removing clothing, repeating phrases, rolling, grasping,
searching movements
• Other behaviors : screaming, kicking, disrobing,sexual-like
movements.
0
E
a
s
Temporal
Frontal
Occipital
Parietal
2
Partial seizures - Localization
a Temporal Lobe
• Most common
• 40-80% have automatisms of hands/mouth - lip
smacking, rubbing hands
• Gradual recovery, several minutes of confusion
• Amnesic for seizure but may recall aura
• Language and memory
• aura- feeling deep down/gut feeling, rising up
from the abdomen/chest, emotional feelings fear, anxiety
« Can secondarily generalize
Partial seizures - Localization
s Occipital Lobe
• Visual aura, tunnel vision, image repetition
• Ictal blindness
• Rapid blinking, eyelid fluttering
• Version of head/eyes to opposite side involuntary eye movements, nystagmus
• flashing lights
• Can be mistaken as a migraine
Partial seizures - Localization
s Frontal Lobe
• Motor seizure - tonic extension of limb - abrupt,
bilateral or asymmetric, Todd's paralysis
• Inability to talk - muscle of tongue, mouth
• Motor automatisms - pedaling, sexual movement,
vocalizations
• "Somatosensory" aura - touch aura - pressure,
temp- warm sensation, pain, sensation of muscle
movement, facial expression - (also seen in parietal)
• Can occur in clusters
• Minimal post-ictal confusion
• Can be mistaken as pseudoseizures
Partial seizures - Localization
0 Parietal lobe - rare
• Vestibular hallucinations (vertigo)
• Feeling physical sensations of numbness, tingling,
heat, pressure, and/or pain.
• Distortion of visual perception: objects seem too
close, too far, too large, too small,
• Language dysfunction- difficulty understanding
spoken words or language, difficulty reading or
performing simple math.
• "Somatosensory" aura - touch aura - epigastric
sensations, pressure, temp, pain, sensation of muscle
movement, facial expression - frontal/parietal
• Panic attacks
• Spreads to temporal lobe quickly
Generalized Tonic/Clonic
Seizures (Grand Mal)
Generalized Seizures
s Typically occur in both sides of the brain, or
may start in one area and spread quickly
s Patient safety a priority - airway, injury
a 3 types
• Tonic-clonic (grand mal)
• Absence (petit mal)
• Atonic - "drop attack"
s Tonic phase
«
•
•
•
•
•
First stage
Loss of consciousness
Muscles contract/extension and stiffening of extremities
Loss of balance
Stridor due to muscle contacting in throat - lasts about 30 sec
May have aura
• Clonic Phase
• Muscles alternate between relaxation and rigidity
• Rhythmic movement without stiffening
• Sometimes loss of bowel and bladder
• Post-ictal phase - fatigue., memory loss, headache,
confusion, can be minutes- hours
E Can be scary to watch
3
Absence Seizures (petit mal)
0 Brief losses of consciousness - usually lasting
no longer than 30 sec, can have up to100/day
a Abrupt onset, no aura
s Staring, unresponsive
h Automatisms - chewing, rapid eye blinking, lip
smacking - (atypical absence)
o Mild increase or decrease in muscle tone
h No post-ictal phase
0 Can be provoked by hyperventilation
Atonic or "drop attacks"
Abrupt, Complete or partial loss of muscle tone
a Eyelids may droop, head may nod, may drop
things, body will get limp - may fall or slump
over.
a May need protective headgear and sometimes
a face mask to prevent injury.
0 Beware of the difference- seizures that cause
them to fall rigidly (like a tree trunk) often
have tonic seizures (involving sudden muscle
contraction) rather than atonic seizures
0
Myoclonic Seizures
b Sudden, Brief, involuntary jerk of a muscle or a
e
a
0
a
0
0
group of muscles.
"Myo" means muscle and "clonus" means rapidly
alternating contraction and relaxation — jerking or
twitching —of a muscle,
Lasts only a second or two.
Can be one or a cluster
No loss of consciousness
Mild: only affects one part of body
Severe: may cause child to abruptly fall
Status Epilepticus
s Seizure activity (either a single event or several consecutive
events) lasting longer than 30 minutes
• Tapering/ Adjusting of medications can increase this risk
a 4 types
• Convulsive Status - easy to recognize
• Non-convulsive or Non-Clinical - difficult to recognize
• Complex Partial Status - confusion, lack of
responsiveness, but not loss of awareness, personality
changes, emotional disturbances (fear, anxiety)
* Absence Status - clouding of consciousness, slowed
speech, decreased attention, "stupor", expressionless,
' trance like state"
Febrile Seizures
Non- Epileptic Events
a
s
0
0
0
s
0
No single presentation
Usually occur in kids older than 8 yrs old
Frequently seen in kids that have seizures
Only happens when kids are awake, never in
sleep
Strong emotional content
Pelvic thrusting, non-rhythmic jerking
Eyes closed
0
Onset from 3 months- 6 years
0
Temperature rises rapidly to 102 or higher
0
Not the same as epilepsy as a fever provokes
the seizure and the child is not having
recurrent unprovoked seizures
4
Epilepsy Syndromes
• Defined by a group of features usually occurring
together. These may include:
• Types of seizures
• Age the seizures begin
• Causes of the seizures - genetics
• Part of the brain involved
• Factors that provoke seizures
• How severe and how frequent the seizures are
• A pattern of seizures by lime of day
• Certain patterns on the EEC, during and between seizures
• Brain imaging findings- MRI, CT scan, PET
» Other disorders/problems in addition to seizures
• Prospects for recovery or worsening
Lennox-Gastaut Syndrome
(LGS)
a
2 to 5% of childhood epilepsies.
a
Caused by brain malformations, perinatal asphyxia, severe
head injury, central nervous system infection and inherited
degenerative or metabolic conditions or unknown
a
Typically begins before 4 yr. old
a
Intellectual development is usually impaired,
a
Multiple different types of seizures- particularly tonic
(stiffening), atonic (drop), atypical absence, and myoclonic
seizures.
a
EEG shows a classic pattern of background slowing and
spike-wave bursts at frequencies less than 2.5 per second,
a
Seizures are hard to control - partial relief of seizures may
be obtained by medications, VNS, Dietary, Corpus
Callosotomy
Common Epilepsy Syndromes
a
a
a
a
s
s
a
s
0
0
Landau -Kleffner Syndrome
0
Rare genetic disorder
B
The most common gene mutation is SCN1A.
s
It begins in the first year of life
Rare disorder
a
Usually begins 3-7 years old.
s
Experiences language problems
0
Simple partial seizures, generalized tonic-clonic and atypical
absence seizures.
0
The EEG is often the key. A normal EEG, especially one done
when the child is awake, does not rule out this disorder. Sleep
activates the epilepsy waves in these children, so sleep recordings
are extremely important. The stage of sleep most affected is called
slow-wave sleep.
0
After age 10, only 20% of patients still have seizures.
0
Associated with autism - intense ST helps
Doose - Myoclonic Astatic
Epilepsy (MAE)
Dravet
0
LGS - Lennox-Gastaut Syndrome
Landau -Kleffner Syndrome
Dravet
Doose - Myoclonic Astatic Epilepsy
Infantile (West Syndrome) or Epileptic Spasms
ESES
Childhood Absence Epilepsy
Juvenile Myoclonic Epilepsy
Sturge - Weber
Glut-1 deficiency
a
s
s
s
Most develop some level of developmental disability
0
The first seizure is often associated with a fever and may be a
tonic clonic seizure or a clonic (jerking) seizure on one side of the
body.Myoclonic seizures appear between 1 and 5 years in 85% of
children
a
a
s
Children are very sensitive to infections, slight changes in body
temperature, bright lights, emotional stress or excitement.
s
a
Usually 2 or more seizure medications are needed to treat the
multiple seizure types.
b
Rare syndrome - Possible genetic link but cause unknown
Onset in first 5 years of life.
Children who previously developed normally, and boys are twice as
likely as girls.
Characterized by difficult-to-control generalized seizures - can be daily,
GTC seizures usually occur during thedaytime in this disorder, at least
in the early stages. Failure to suppress the "EEG abnormalities (4- to 7-Hz
rhythms and spike-wave discharges) during therapy and absence of
occipital alpha-rhythm with therapy suggest a poor prognosis
Unfavorable Signs for poor prognosis:
•
G
Nocturnal GTC seizures
Complete seizure control can be achieved in about half of the cases with
medications
Medicines and/or ketogenic diet
5
Infantile (West syndrome) or
Epileptic Spasms
0
s
a
•
E
e
Typically begins 3 -12 months of age and usually stop by 4 years
Consists of a sudden jerk followed by stiffening,
Seizure lasts only a second or two but can occurs in clusters.
Many later develop other kinds of epilepsy.
EEG - unusual pattern called hypsarrhythmia when the
seizures are not occurring. Chaotic, hign-voltage pattern is
often helpful in confirming diagnosis.
Primary approved treatments for children with infantile
spasms include:
» Steroid therapy - adrenocorticotropic hormone [ACTH by
injection] or prednisone
• Sabril (vigabatrin) - associated with damage to the retina of the
eye which can result in permanent loss of peripheral vision
when the drug is used for many months.
Childhood Absence Epilepsy
h Account for 2-8 % with epilepsy,
a Predominantly genetic.
0 Ages 4-8 years, normal neurologic function.
0 Staring spells - not aware or responsive.
0 Lasts about 10 seconds and ends abruptly.
0 Can occur 1 to 50 times per day.
Electrical Status Epilepticus In
Sleep (ESES)
a Usually appears in mid-childhood
0 First sign is the rate of a child's learning appears to
slow or regress significantly.
0 EEG - continuous spike and wave epileptic activity
during sleep (>85% of time) - and especially
during part of the sleep called 'slow wave' sleep.
0 EEG is often abnormal when the child is awake but
may also be normal.
0 Medications - high dose Valium, IVIG, steroids and
clobazam.
a Some children do not respond to any medication.
Juvenile Myoclonic Epilepsy
(JME)
a Most common generalized genetic epilepsy
0 First seizure (absence) starts between 5-16 years
followed by myoclonic jerks about 1-9 years later.
GTC can also appear.
0 The EEG is the most important test in making a
diagnosis. The EEG is typically abnormal with a 3­
6 Hz generalized polyspike and wave discharge.
0 Myoclonic jerks that occur on awakening.
Typically clusters.
b Common precipitating factors include: stress,
emotions, flickering light, missed medications.
Sturge - Weber
Glut-1 deficiency
b Presence of a birthmark ('port wine' stain), usually on
0 Frequent seizures beginning in the first months.
0 SLC2A1 gene - provides instructions for producing a
protein called the glucose transporter protein type 1
(GLUT1). GLUT1 protein is involved in moving
glucose, which is the brain's main energy source, across
the blood-brain barrier. The GLUT1 protein also moves
glucose between cells in the brain called glia, which
protect and maintain nerve cells.
0 SLC2A1 gene mutations reduce/eliminate the function
of the GLUT1 protein. Having less functional GLUT1
rotein reduces the amount of glucose available to
rain cells, which affects brain development and
function.
0 Only treatment to date is the ketogenic diet
one side of the face, associated with an abnormality of
the brain. Caused by abnormal blood vessels on the
surface of the brain.
q Start at birth or in first year of life,
a Usually partial motor seizures involving jerks of one
side of the body only. May become generalized and
evolve into other types of seizures.
0 Learning disabilities are typically present.
0 Seizures are frequent and prolonged.
0 Prognosis depends on the extent to which the brain is
affected. This seems to be closely linked with the
frequency and the severity of the seizures.
g
6
Seizure Management
1st Step to Seizure Safety
• Know type of seizures
0 Seizure Safety
• Know developmental status of patient
0 Rescue medications and administration
• Know triggers for seizures
0 Risks of epilepsy
• Know basic first aid
• Know rescue meds
• Know medications and if meds are being adjusted
Triggers
• Stress
0
0
0
0
S
0
0
0
Sleep - lack of
Lights
Sounds
Fever/illness
Menstrual cycle
Foods- caffeine, sugar, food coloring
Missed medications
OTC or other medication interactions
Airway Management
a Seizures can alter the perception of respiration and
fullness of breath (shortness of breath), respiratory rate
and pattern (tachypnea, hypopnea, apnea), reflexes
(coughing), quality (stridor), and secretions.
• Apnea and cyanosis are common during tonic-clonic
seizures and prolonged tonic seizures. Brief apnea
occurs during many complex partial seizures.
Seizure Safety
0
0
0
0
0
0
0
0
Always stay with the child
Safety and comfort
Do not hold the person down
Do not put anything in the mouth
Airway management
Time the seizure
Stay calm
Seizure Action Plan
Rescue Medications
a Know the medication and dosage
0 Know when to give it
0 Know how to administer it correctly
0 Know how quickly it works
e After the seizure make sure respirations return to
normal
a Know side effects of medication
Rescue Medications
• Rectal diazepam (Diastat)
• Typically if GTC over 5 min or CPS or cluster >10 min or
6 seizures in an hour
• Rectal administration
» Dialed down and locked by pharmacy - set dosage
» Most seizures controlled within 5-15 min
e Intranasal Midazolam
•
•
•
•
•
Typically Older Children
Caregiver has to draw it up - Partial dosage
IV product (5mg/mL) administered nasally
Need atomizer and teaching
Most seizures controlled within 3 minutes
Seizure Safety
a Get Emergency Help:
• If longer than 5-10 minutes (after giving
rescue medications)
« If one seizure after another (except for known
cluster seizure).
• If injured or injury is suspected.
• If the person isn't returning to their baseline.
Risks due to Epilepsy
s Can lead to brain damage or death
0 Developmental delays
E Progressive loss of abilities /Regression
• Injury
• Loss of hope for independence
0 Strong association to depression, psychiatric
disorders, autism, ADHD
a 50,000 annual deaths from status or SUDEP
Rescue Medications
s Buccal midazolam
• IV product administered buccally/orally
• Most seizures controlled within 5 minutes
h Clonazepam or Valium-Oral/GT
• Typically with aura
• Works better with certain types of seizures
• VNS
• Swipe magnet at onset of seizures
• Helps stop or shorter seizure
After Seizure Safety
• Don't leave anyone alone after a seizure until:
• They are breathing normally.
• They can talk/communicate per baseline
• Able to answer the four W's: who, what, when, and
where - developmentally appropriate.
• Able to wake them up if they fall asleep
• They are in a safe environment
a Document seizure
• Date/Time
• Length of seizure
• Description of seizure
• Interventions/Medications given
What is SUDEP?
Sudden Unexpected Death in Epilepsy (SUDEP)
Person with epilepsy dies unexpectedly and was previously
in their usual state of health.
Occurs most often at night or during sleep, leaving many
questions unanswered.
No one knows what causes SUDEP. Possible causes of
SUDEP focuses on problems with breathing, heart rhythm
and brain function that occur with a seizure.
• Breathing: A seizure typically may cause a person to briefly stop
breathing (apnea). If these breathing pauses last too long, tney can
reduce tne amount of oxygen that gets to the heart and tne brain.
Also, a person's airway may sometimes get blocked during a
convulsive seizure, leading to suffocation.
• Heart Rhythm: A seizure may cause a dangerous heart rhythm or
cardiac arrest.
• Brain Function: Seizures may suppress or interfere with the
function of vital areas in the brainstem.
8
Treatment Options
b Medications
s Diet
• Ketogenic
• MAD
0 Surgery
• "curative"
• palliative
Picking a Medication
ta Base it on type of seizure
• Generalized vs. partial: broad spectrum vs. partial
• Syndromes (some medications worsen some
seizures)
• Base it on side effect profile
• Age of patient and sex:
D no valproic acid for teenage girls due to polycystic
ovary disease and potential fetal issues
° No valproic acid for children <2 years old
• If child has liver, kidney issues: avoiding meds
worsening it
• If child has acidosis: no topiramate or zonisamide
Treatment Challenges
s Same challenges for all
« Mixed seizure types
• Partial responders
• Efficacy vs. tolerability
• Drug interactions
• Monitoring blood levels
Choosing the Right Medicine
0 The type of seizure/syndrome
0 The person's age and gender
• Pill/liquid
• Female - childbearing age
a Other medical problems
a Possible side effects from the medicine
a Other medications child is on (interactions)
Response to Anticonvulsants
0 60% of patients will respond to first or second
medication chosen
• 47% responded to first AED
• 13% responded to second
• 1% to third
• 3% controlled with two AED's
b About a third will not respond to any
medication - diet, surgery, palliative care
Treatment Challenges
Why Are Kids So Different?
a Additional challenges in pediatrics
•
•
«
•
•
Lack of research
Metabolism and clearance
Dosing formulations
Ketogenic diet
Rescue meds for school
Super-Metabolizers
Off-Label Treatment/Dosing
• Lack of evidence in pediatrics
a Pharmacoresistant epilepsy syndromes
• Have to move to 3rd and 4th line options
a Examples:
Kids may need higher doses on a mg/kg
basis compared to adults
- Lamotrigine (esp. 2-6 year old, <30kg)
- Oxcarbazepine (esp. 2-4 year old)
• IV levetiracetam is not FDA-approved for use in
patients <16 yo
• Zonisamide not approved for <16 yo or for
infantile spasms
Dosing Formulations
- Phenobarbital (esp. 1-5 year old)
- Phenytoin ("infants and_young children")
- Diastat
Rational for Polytherapy
a Not all are kid-friendly
• Extemporaneous suspensions
0 Clonazepam
Q Lamotrigine
n Topiramate
0 Zonisamide
• Enteral tubes
0 Sprinkle caps (divalproex, topiramate) clog tubes
° DR/ER tabs shouldn't be crushed or broken
28+ Anticonvulsant Choices
a
ACTH
a
a
a
a
Au'taz-oinmidi' (Di.uncix)
Benzodiazepine (Valium)
Bromides
Carbamazepine
{Tegretal,Carbatrol)
Clonazepam (Kionopin)
Clobazam (Onfi)
Eslicarbiizepint'
Ethosuximide (Zaronlin)
Elholoin
Ezogabino (Poliga)
Felbamatc (Felbatrol)
Gabapenlin (Nourontin)
Lacosamide (Vimpat)
Lamotrigine (Lamictal)
B
s
a
a
0
a
a
a
a
a
B
a
a
a
s
a
a
a
0
a
a
0
a
a
a
Levetiracetam (Keppra)
Mi'thsuximidi'
Oxcarbazepine (Trileptal,
Oxtellar XR)
Pcrampaiwl
Phtmyliun (Dilantin)
Phenobarbital (Luminal)
Progabalin (Lyrira)
Primidone (Mysolino)
Rufinamide (Banzel)
Stiripentol
Tiagabine (Babilri!)
Topiramate (Topamax)
Valproic acid (Depakene,
Depakote)
Vigabatrin (sabril)
Zonisamide (Zonegnm)
s Failure of 2 medications
a Target different MOAs
0
Aim for different adverse effect profiles
b Watch drug interactions
a Consider compliance
Broad Spectrum Medications
• Traditional
E
Newer
• Acetazolamide
• Clobazam
» Benzodiazepines Valium, Clonazepam
• Felbamate (LGS)
• Bromides
•
•
•
•
•
•
• Carbamazepine
• Ethosuxamide (absence)
• Ethotoin
• Methsuximide (absence)
• Phenobarbital
• Phenytoin
• Valproic Acid
• Lamotrigine
Levetiracetam
Neurontin
Primidone
Rufinamide
Topiramate
Zonisamide
Newest Medications Out
Since 2008
Partial Medications
E
Traditional
•
•
•
•
Carbamazepine
Eslicarbazepine
Phenytoin
Pregabalin
e Newer
•
•
•
•
»
•
Clobazam
Ezogabine
Oxcarbazepine
Perampanel
Tiagabine
Vigabatrin
a
s
s
0
0
Lacosamide (Vimpat)
Lacosamide
Rufinamide
Vigabatrin
Clobazam
Ezogabine
Rufinamide (Banzel)
0 Approved November 2008
0 MOA: unknown, triazole derivative,
possibly prevents sodium-chartneldependent neuronal firing
h Use: broad spectrum (generalized), LGS(>4
years old)
0 Dose: 5mg/kg BID up to 22.5mg/kg BID
(max 3200mg/ day)
0 AE: dose-dependent QT-shortening,
dizziness, HA, somnolence, vomiting
0 Availability: tablet, suspension
h Other: take with food
0 Approved October 2008
• MOA: Inactivation of slow sodium channels to
stabilize hyperexcitable neuronal membranes
a Use: partial onset seizures >17 years old
• Dose: start l-5mg/kg/day in divided doses;
titrate to 10-20mg/kg/day, max 400600mg/day
s AE: dizziness, ataxia, diplopia, nausea
E Availability: tablets, oral suspension, IV
• Schedule V controlled substance
Vigabatrin (Sabril)
Clobazam (Onfi)
a MOA: irreversible GABA-transaminase inhibitor
a Approved October 2011
a MOA: binds to BZD site (receptor site 5 compared to
a Use: infantile spasms or complex partial seizures
a Dose: 25mg/kg to 75mg/kg BID; max 3g/day
0 Use: LGS (>2 y.o.), ESES
0 Dose: 5 mg daily up to 10-20 mg BID or
a Approved August 2009
(increases circulating GABA)
0 AE: Vision loss (permanent, progressive, increases
w/ cumulative close); anemia, somnolence,
infection, HA, white matter changes
0 SHARE program
• Consent form
• Registered provider/patient/pharmacy
• Availability: 500 mg packets and tablets
other BZDs) on GABA receptors
0.5 -1 mg/kg/day q day or BID
a AE: somnolence, infection, constipation, aggression,
a
tolerance
• Less than other BZD - less drooling and sedation.
• Thought to be more effective seizure control
Availability: tablet (ok to crush)
• Schedule IV controlled substance
11
Ezogabine (Potiga)
a Approved in 2010
E MOA: potassium channel opener
s Use: partial-onset seizures
a Dosage: 200 mg - 400 mg TID
B AE: blue skin discoloration, eye abnormalities
characterized by pigment changes in the retina,
urinary retention
0 Availability: tablets
Specialty Medications
a ACTH - infantile spasms
a High Dose Oral Valium - ESES
a Stiripentol- Dravet syndrome
a CBD or Cannabidiol - medical marijuana
• Schedule V controlled substance
High Dose Valium
ACTH
0
Infantile spasms
0
Ordered from ACTHAR Support - sent via mail
0
Given IM - parents to give
0
High dose Steroid
0
Given daily and weaned every 5-7 days over a month
0
Suggest immunizations be held during treatment as well as
keeping patient away as much as possible from other children due
to potential for immunosuppression
0
Good Hand washing
0
Requires weekly: weight check, blood pressure check, occult stool
lest and dextrosticks due to side effects from ACTH
b Given for ESES - Electrical Status Epilepticus
during Sleep
a ESES can cause learning regression/ delays,
behavior problems, sleep problems
s Given orally at bedtime
h First dose given in hospital to monitor EEG
changes (25% improvement) and respiratory/other
side effects
0 Initial dose 1 mg/kg/ dose then 0.5 mg/kg/dose
0 Weaned off over time (1-3 months plus)
0 Side effects; respiratory depression, irritability,
sleepiness, hyperactivity
CBD
Stiripentol
0
DravetSyndrome- documented SCNla mutation
0
Not available in US - FDA approved provider for compassionate
use
a
Shipped to home
0
Failed 2 or more medications
0
Must be on Clobazam
0
Clinic visit every 3 months mandatory
a
a
Capsule or "sachet" form
AE: loss of appetite, weight loss, difficulty sleeping, sleepiness,
unsteady gait, and low muscle tone, dizziness, low VVBC, liver
problems.
h
0
In addition, this medication will likely cause problems with the
levels of other seizure medications requiring adjustments and
more frequent blood draws for levels of these medications.
Labs need to be obtained at baseline and every 6 months (CBC and
LFT)
a
a
a
s
0
Little objective data about effective treatment
with MCBD - Safety and efficacy of these
products have not yet been determined,
As of April 30, 2014, over 307 patients under
the age of 18 had registered as users of MCBD
Children's Hospital Colorado are not
registered providers for medicinal cannabis
and therefore are not able to prescribe these
products.
Other names/strains: CBD, THCa, THC,
charlotte's web
Forms: liquid, edibles, patches, lotions
Resources
a
www.epilepsv.com - This website is a nice resource for information on
epilepsy and its treatments
s
www,epilepsy foundation.org - This is an advocacy organization that has a
chapter in Colorado that can be a useful resource for answering questions
about epilepsy.
a
www.seiiairetracker.com - Provides a way to track seizures using on-line
tools. Also has an iPlume Application.
•
http://wvvw.cdc.i'ov/epilcpsv - CDC information regarding seizures and
seizure disorders
E
Children's Hospital Colorado Neurology Department
g
QUESTIONS?
THANK YOU FOR YOUR
TIME
Lots of foundations and websites out there
* Dravetfoundation.org
* Hemifoundalion.org
* Charliefoundalion.org - keto diet
13
Diastaf
(diazepam rectal gel)
INSTRUCCIONES DE
ADMINISTRACION A NINOS
Y DESECHO
DiasfafAcuDiar
(diazepam rectal gel)
Cuando tratar. Segun las indicaciones o receta del medico
Consideraciones especiales
Lea antes de usar
Para el encargado de la atencion que usa DIASTAT®:
No administre DIASTAT® hasta:
1. Que haya lei'do cuidadosamente estas instrucciones
2. Que haya revisado los pasos de administration con el medico
3. Comprenda las indicaciones
Para el encargado de la atencion que usa
DIASTAT® AcuDiaP:
No administre DIASTAT® AcuDial™ hasta:
1. Que haya confirmado que:
• La dosis recetada esta a la vista y, si la conoce, es correcta
• La banda verde "ready" esta visible
Confirme que la tlosis y la banda verde "ready" esten vlsibles.
Ventana del visor de la dosis
/ Banda verde
"READY"
DIASTAT® se debe usar con precaution:
• En personas con dificultades respiratorias (por ejemplo, asma o neumonia)
• En ancianos
• En mujeres en edad reproductiva, durante el embarazo o la lactancia
Discuta previamente con el medico los pasos adicionales que puede tener que seguir si hay una
perdida de DIASTAT® o defecation.
La dosis de DIASTAT® del paciente es:
mg
Frecuencia respiratoria del paciente en reposo
Peso actual del paciente
Confirme que el peso actual sea el mismo que cuando se receto DIASTAT®
Verifique la fecha de caducidad y siempre retire la tapa antes de usar. Asegurese de retirar el
perno del sello con la tapa.
TRATAMIENTO 1
Cosas importantes para decirle al medico
Convulsiones antes de DIASTAT®
Hora
Tipo de
Niim. de
convulsiones convulsiones
Fecha:
Convulsiones despues de DIASTAT*
Hora
Tipo de
Num. de
convulsiones convulsiones
2. Que haya lei'do cuidadosamente estas instrucciones
3. Que haya revisado los pasos de administration con el medico
4. Comprenda las indicaciones
Por favor no administre DIASTAT® hasta que se sienta
comodo con el uso de DIASTAT®. El medico le dira
exactamente cuando usar DIASTAT®. Cuando use DIASTAT®
en forma correcta y segura ayudara a controlar las
convulsiones. Asegurese de discutir todos los aspectos de
su funcion con el medico. Si no se siente comodo, discuta
su funcion nuevamente con el medico.
Cosas que se deben hacer despues del tratamiento con DIASTAT® AcuDial™
Permanezca con la persona durante 4 horas y tome nota de lo siguiente:
• Cambios de la frecuencia respiratoria en reposo
• Cambios en el color
Posibles efectos secundarios del tratamiento .
Para ayudar a la persona que tiene convulsiones:
• Usted debera ser capaz de distinguir entre
convulsiones ordinarias y en accesos.
• Debe sentirse comodo y satisfecho de ser capaz
de administrar DIASTAT®.
Debe ponerse de acuerdo con el medico sobre las
condiciones exactas de cuando tratar con DIASTAT®.
y/ Debe saber como y por cuanto tiempo tiene que
vigilar a la persona despues de administrar DIASTAT®.
Saber que respuestas esperar:
• Debe saber que tan pronto deben parar las convulsiones o
disminuir en frecuencia despues de administrar DIASTAT®.
• Necesita saber que hacer si las convulsiones no paran
o si hay cambios en la respiration, la conducta o la
condition de la persona que lo alarmen.
TRATAMIENTO 2
Cosas importantes para decirle al medico
Convulsiones antes de DIASTAT*
Hora
Tipo de
Num. de
convulsiones convulsiones
Fecha:
Convulsiones despues de DIASTAT
Hora
Tipo de
Num. de
convulsiones convulsiones
Cosas que se deben hacer despues del tratamiento con DIASTAT® AcuDial™
Permanezca con la persona durante 4 horas y tome nota de lo siguiente:
• Cambios de la frecuencia respiratoria en reposo
• Cambios en el color
1
Posibles efectos secundarios del tratamiento.
Si tiene dudas o se siente inseguro acerca de usar el
tratamiento, LLAME AL MfeDICO antes de usar DIASTAT®.
INSTRUCCIONES DE DESECHO PARA DIASTAT' AcuDial"
£mbolo -
iDonde puede encontrar mas information y apoyo?
' Tire del 6mbolo
hasta retirarlo por
completo de
la jeringa
' Dirija la punta
al lavabo
o al Inodoro
Para obtener informaci6n sobre DIASTAT®
y DIASTAT® AcuDial™:
1
Vuelva a colocar el
£mbolodentrodela
jeringa empuj3ndolo
suavemente hasta
que sedetenga
1
Halelabombadel
inodoro o enjuague
el lavabo con agua
hasta queya no se
vea el gel
Llame al 1-877-361-2719 o visite www.diastat.com
Recursos adicionales:
Epilepsy Foundation (EF). Puede comunicarse con EF
llamando al 1-800-EFA-1000 o en www.efa.org.
^ VALEANT"
.
© 2008 Valeant Pharmaceuticals North America
Impreso en E.U.A,
4/08
LAVABO 0 INODORO I
Este paso es sdlo para usuados de
DIASTAT® AcuDial™
Alterminarelpaso 14a:
8
Deseche todos los materlales
usados en el botede la basura
DESECHO PARA DIASTAT® 2.5 mg
Alterminarelpaso 13:
* Deseche todos losmateriates usados
en el bote dela basura
diastat.com
* Noreutiiice
* Deseche en unlugar seguro,
fuera del alcance de los nlnos
Instrucciones de
administration
en adultos disponibles
para descargar en
* Deseche en unlugar seguro,
fuera del alcance delos nihos
CHILD ADMINISTRATION INSTRUCTIONS
:•
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* •
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* •*
v
i
I
• II
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y
. s? *•ft>
Put person on their side where they can't fall.
Get medicine.
Get syringe. Note: seal pin is attached to the cap.
Push up with thumb and pull to remove cap
from syringe. Be sure seal pin is removed
with the cap.
Lubricate rectal tip with lubricating jelly.
Turn person on side facing you.
Separate buttocks to expose rectum.
Gently insert syringe tip into rectum.
Note: rim should be snug against rectal opening.
W&mm
Bend upper leg forward to expose rectum.
SLOWLY...
COUNT OUT LOUD TO THREE...1...2...3
Slowly count to 3 while gently pushing plunger
in until it stops.
Slowly count to 3 before removing syringe from
rectum.
Slowly count to 3 while holding buttocks
together to prevent leakage.
CALL FOR HELP IF ANV OF THE FOLLOWING OCCUR
Seizure(s).continues!5 minutes after giving D|ASTA"P or per the doctor's instructions:
•<
*i'i
w.-
—
• . v
1MB—
Keep person
on the side
facing you, note
time given, and
continue to
observe.
•
• Seizurt behavior is different from other episodes
' ,{i You are alarmed by :the frequency or severity of the seizure(s)
;.'t •,YouVre alarmed by the color or breathing qf the person
'? The person is having unusual or serious problems .
•
^•
}/•Local'epnergency number:
•
•
••••v
.' _ JPIease be sure to note if your area has 911)
v
- Doctor's number:
V-'V .jhformation'fdr emergency squad; Time DIASTAT® given:
Dose:.
DIASTAT® Indication
DIASTAT® AcuDial™ (diazepam rectal gel) is a gel formulation of diazepam intended
for rectal administration in the management of selected, refractory patients with
epilepsy, on stable regimens of AEDs, who require intermittent use of diazepam to
control bouts of increased seizure activity, for patients 2 years and older.
Important Safety Information
In clinical trials with DIASTAT®, the most frequent adverse event was somnolence
(23%). Less frequent adverse events reported were dizziness, headache, pain,
vasodilatation, diarrhea, ataxia, euphoria, incoordination, asthma, rash, abdominal
pain, nervousness, and rhinitis (1 %—5%).
D955-0308
Diastaf
(diazepam rectal gel)
DiasfafAcuDiaf
(diazepam rectal gel)
DISPOSAL INSTRUCTIONS ON REVERSE SIDE
I
Children's Hospital Colorado
IN CARE O F KIDS
Home Therapy for Seizures
Using Intranasal Midazolam Therapy
What is intranasal Midazolam therapy and when is
it used?
This therapy is for treating seizures by spraying the
medicine on the skin inside the nose (intranasal
injection). The medicine is injected (sprayed) into the
nose using a needleless syringe.
,.v,
'
What you need to know before using midazolam:
Before you need to use this medicine be sure you
know how much medicine you will give your child.
It is a good idea to keep this handout with the
medicine.
Medicine:
Midazolam
Dose (amount):
How given:
Inject into nose using needleless syringe
Amount in each nostril:
Questions or problems
•
•
Children's Hospital Colorado
Neurology Dept. 720-777-6895
Always store all the supplies in one place so they can be easily used when you need
them.
Supplies needed: syringes or needless vial adapter, atomizer tips, bottle with medicine.
•
•
•
Have your doctor or nurse use a permanent marker to draw a line on the syringe to
show the TOTAL amount of the drug you will use on your child - that way you will not
make a mistake when you are scared or nervous.
Be sure the doctor or nurse includes the small amount of medicine (0.1 ml) that will stay
between the syringe and the atomizer tip (dead space).
o For instance: if your child needs 1.2 ml of medicine, you will need 1.3 ml in the
syringe.
Practice using the equipment! Use a syringe and some water (instead of medicine)
and practice putting the medicine in the syringe and attaching it to the atomizer so you
are comfortable before you really need to do the procedure. Practice as many times as
you need to so you know what to do next.
How to give intranasal midazolam:
plungesB
'.irjfeisai.rfifow-to ,.%\
Step 1:
Pull the plunger of the syringe back
until the black seal is at the mark on
your syringe.
For example if you are
supposed to draw up 1.0 ml of
medication, pull the plunger
back to the 1 ml mark of the
syringe - the syringe will now
have 1 ml of air in it.
jf '-
Pop the protective plastic cap off
the bottle of midazolam.
Pk
Otr
childrenscolorado.org • Anschutz Medical Campus • 13123 East 16th
Avenue •
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1 i\
Pop Cai
Step 2:
Aurora, CO 80045 • 800-624-6553
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v
Unto^otCoEoracio
Anschutz MzcMcM Oa-ftpus
Step 3:
Connect the syringe or needleless
access device by twisting or slipping.
Poke the access device into the
rubber seal of the medicine bottle.
Step 4:
Tip the bottle up-side down so the
syringe is on the bottom and the bottle
rubber seal faces down.
Push the syringe plunger towards the
bottle - injecting (pushing) air into the
bottle.
Step 5:
Pull the plunger back and let the
syringe fill with medicine. (Look inside
the bottle to check that the tip of the
needle is in the liquid drug, otherwise
you will just draw the air into the
syringe). Pull the plunger back so the
medicine fills the syringe to the black
line.
childrenscolorado.org • Arischutz Medical Campus • 13123 East 16th Avenue • Aurora, CO 80045 • 800-624-6553
University d Cokirgcfo
Anschutz
Campus
Step 6:
Disconnect the syringe from the
accessing device or needle by
twisting.
Z
•V
"Xt*
oiff
Twist
discovmeci
\vun*ie
.
;
.
Step 7:
v
y r \
t>rmccr Atomizer
Attach the atomizer tip using the lock
mechanism twists into place.
It is effective as long as the tip is firmly
seated on the syringe tip.
Step 8:
Use your free hand to hold the child's
head still. Place the tip of the
atomizer tightly against the nostril
aiming slightly up and outward
towards the top of the ear.
Inject half o f
drug imo one
nostril
childrenscolorado.org • Anschutz Medical Campus • 13123 East 16th Avenue • Aurora, CO 80045 • 800-624-6553
Step 9:
Quickly push the syringe plunger to
deliver hajf of the medication into the
nostril.
Step 10:
Move the syringe over to the other
nostril and quickly push the rest of the
medicine into that nostril.
/ -
UW
1.
4
V
5
Jennifer Konieczny RN JD CNRN Neuroscience Institute
2013
Approved by Patient/Family Education Committee
©2013 Children's Hospital Colorado, Aurora, CO
childrenscolorado.org • Anschutz Medical Campus « 13123 East 16th Avenue • Aurora, CO 80045 • 800-624-6553
*
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is# Arsschute Mfjwcaf Campus