Epilepsy in Children. School Nurse Curriculum 2009

Transcription

1 Epilepsy in Children School Nurse Curriculum 2009

2 Seizures and Epilepsy Seizure is most common paroxysmal disorder of infancy and childhood 25,000-40,000 children diagnosed with seizures yearly Can be provoked by acute condition (e.g. hypoglycemia, toxic ingestion) or unprovoked with unknown cause

3 The Brain

4 Seizures and Epilepsy Seizure A sudden involuntary electrical discharge of neurons in the brain resulting in alteration in function or behavior Epilepsy From the Greek epilepsia a condition in which an individual is predisposed to recurrent, unprovoked seizures Status Epilepticus More than 30 minutes of continuous seizure activity or sequential seizures without intervening recovery Can be life threatening or permanently disabling (Hosseini, 2008)

5 Morbidity of Status Epilepticus Mortality : Secondary to Status Epilepticus is 30% (initially) now with improved management still 3-10% Lower mortality is associated with responsive ED care Morbidity: Children with chronic neurological problems with low AED levels do well Long term sequelae Epilepsy in 20-40% Encephalopathy in 6-15% Focal neuro deficits in 10% (Hosseini, 2008)

6 Etiology of Status Epilepticus Antiepileptic Medications - Noncompliance, abrupt withdrawal, weaning Trauma Infection - CNS, systemic with CNS Toxins Hypoxic-Ischemic Encephalopathy Cerebrovascular - Stroke, intracranial hemorrhage CNS Mass Lesion Metabolic - Glucose, Na, Ca, Mg, BUN, inborn metabolic disorder Toxin

7 Epilepsy in Children Possible Seizure Etiologies Birth injury Post-natal injury from intracranial hemorrhage or hydrocephalus (seen mostly in preemies) Trauma Genetic predisposition Structural brain abnormalities or lesion

8 Provoked: Seizure Etiology Acute condition Hypoglycemia, ingestion, trauma, infection Unprovoked: Symptomatic Birth or post-natal injury or insult malformation, hemorrhage, hydrocephalus Cryptogenic Idiopathic (genetic) Typical children, normal MRIs, inherited epilepsy e.g. Absence Epilepsy, Benign Rolandic Epilepsy (Hosseini, 2008)

9 Differential Diagnosis Elicit a Detailed History Determine if a seizure occurred Rule out seizure-like non-epileptic events: Breath Holding Syncope (syncopal convulsion) Gastro-esophageal Reflux Pseudoseizure - teens Other: movement disorder (dystonia), behavioral, staring, stereotypies (head banging), parasomnias (sleep myoclonus) (Hosseini, 2008)

10 Description of Symptoms Possible seizure triggers Illness, URI, sleep deprivation, travel, missed medication doses, hormonal changes, weight gain Change in pattern, duration, character from previous episodes Medication compliance Other triggers

11 Seizure Types EPILEPTIC Simple Partial Complex Partial Generalized Rigid body Upper & lower body move the same way Cannot be interrupted Absence is different NONEPILEPTIC Physical Behavioral Body is not rigid Upper & lower body move differently Can be interrupted by touching the child firmly

12 Seizure Types - Partial SIMPLE PARTIAL Conscious, often can talk Aura is common (feeling that a seizure is about to occur) Involves one part of body COMPLEX PARTIAL Not conscious Starts with one part of body, then may spread

13 Seizure Types GENERALIZED SEIZURES Absence (petit mal): very brief pauses (10-30 seconds) Myoclonic: jerking Tonic: stiffening Atonic: drop attacks Clonic: rhythmic jerking, twitching Tonic-clonic: stiffening & jerking

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15 Medical Update EEG helps in determining Seizure type Epilepsy syndrome Risk for recurrence Management decisions MRI required if Post-ictal focal deficit not resolving within 1 hr. Not returning to baseline within several hours after seizure Localized findings on EEG (Hosseini, 2008)

16 Types of Treatment Anti-epileptic Medications (AED s) Surgery to remove part of brain causing seizures Surgery to make seizures smaller and less harmful Implantation of VNS (vagal nerve stimulator) Ketogenic Diet Alternative treatments: none proven

17 Treatment Goals Prevent seizures or keep them short if they happen Prevent injury Improve learning by reducing time spent having & recovering from seizures Increase time spent in school Improve quality of life

18 Medical Update Anti-epileptic medications (AED s) Monotherapy Polytherapy Seizure rescue medications Drug name, formulation, strength, route, dosing schedule, total mg/24 hours, toxicity or side effects Brand vs. generic Recent change in weight

19 Medications Liquid usually must be given three times a day, pills & sprinkles twice/day Sprinkles should be mixed with a teaspoon of sticky food e.g. hot cereal, yogurt, refried beans, applesauce All may cause drowsiness which resolves as child adjusts to the drug

20 Basic Facts about AED s AEDs are more alike than different Old generation AEDs: Phenytoin, phenobarbital, carbamazepine, valproic acid, and ethosuximide All can have multisystem effects: Liver, bone marrow, pancreas (valproic acid), All need routine blood work: Drug levels (trough) CBC, liver function test (ALT/AST), amylase Most affect other drugs: valproic acid increases other drugs, others decrease drug levels (Hosseini, 2008)

21 Basic Facts about AED s AEDs are more alike than different New generation AEDs: Lamotrigine (Lamictal), topiramate (Topamax), levitiracetam (Keppra), zonisamide (Zonegran), oxcarbazepine (Trileptal) No routine blood work No routine drug levels No significant drug interactions Except lamotrigine (Hosseini, 2008)

22 AED Side Effects May occur with oral administration Increase when dose is increased (dose dependent) Generally they are temporary Some AEDs have unique side effects Side effects can be decreased by slow titration If IV load of AED is given, side effects are pronounced and may take days to resolve (Hosseini, 2008)

23 AED Side Effects Side effects common to most AEDS: Sedation, grogginess Difficulty thinking cognitive slowing Off balance, dizzy, ataxic Behavior changes: Worsen: aggressive, hyperactive, irritable Stabilize: less labile Stomach irritation -> abdominal pain

24 AED Side Effects Unique side effects: Weight gain: valproic acid,?oxcarbazepine Weight loss due to decreased appetite: topiramate, zonisimide Weight neutral: Most other AEDs Tremor: valproic acid, lamotrigine Osteoporosis: phenytoin, phenobarbital, carbamazepine Rash: Any AED, but lamotrigine SJS Hypohydrosis and hyperthermia: topiramate, zonisimide Hosseini, 2008)

25 What Guides Choice of AED? Seizure type Focal onset Generalized onset EEG pattern Focal onset or generalized activity Co-morbidities Psychiatric: Labile mood Weight: Overweight vs. underweight Other medical problems (Hosseini, 2008)

26 Narrow Spectrum AEDs Focal Onset Epilepsy: Oxcarbazepine (Trileptal ) Carbamazepine (Tegretol ) Levetiracetam (Keppra ) Phenytoin (Dilantin ) Gabapentin (Neurontin )

27 Oxcarbazepine (Trileptal) Chemically similar to carbamazepine (Tegretol ) Less side effects compared to carbamazepine (Tegretol) & better tolerated: less sedation, dizziness, balance problems Used in infants (2wks of age) -> elderly No routine labs needed Carbamazepine level not checked, oxcarbazepine metabolite level is checked Rare hyponatremia seen in infants Problem: Cost

28 carbamazepine (Tegretol ) Used for partial seizures TID dosing vs. longer acting Tegretol, Tegretol XR, Carbatrol Monitor CBC, platelet, and liver function Drug interaction with erythromycin and INH

29 levetiracetam (Keppra ) Initially used for focal onset seizures Now shown to be effective for generalized epilepsy Used in all ages: Infants -> elderly Behavioral side effects: Aggression, labile mood, can rarely lead to psychotic behavior. Higher risk: Children with history of behavior problems or MR. Lower risk: Typical children generally do not have these problems (Hosseini, 2008)

30 levetiracetam (Keppra ) (continued) Good choice for children who are On multiple medication OR With multi-organ failure No medication interactions Not metabolized or excreted by kidneys No routine labs or drug levels needed

31 zonisamide (Zonegran ) Used from neonatal period -> elderly Broad spectrum medication with once a day dosing, and + weight loss Some behavior problems reported, risk of kidney stones and hyperthermia Used with caution in children who have sulfa drug allergies No routine drug levels or labs needed

32 phenytoin (Dilantin ) Phenytoin (Dilantin ) Partial, secondarily generalized, generalized tonic-clonic, status epilepticus QD or BID dosing Monitor CBC, LFT s, blood levels Side Effects: Hirsutism, gum hyperplasia

33 gabapentin (Neurontin ) gabapentin (Neurontin ) Partial seizures Few drug interactions Renal metabolism Easy to titrate and wean Many other uses Psychiatry, neuropathic pain, other Drug levels not tested

34 Broad Spectrum AEDs Generalized Epilepsy & Focal Onset Epilepsy: ethosuximide (Zarontin ) *Narrow spectrum for absence seizures only

35 Broad Spectrum AEDs Generalized Epilepsy & Focal Onset Epilepsy: lamotrigine (Lamictal ) zonisamide (Zonegran ) topiramate (Topamax ) levetiracetam (Keppra ) valproic acid (Depakote and siblings) ethosuximide (Zarontin ) *Narrow spectrum for absence seizures only

36 Lamotrigine (Lamictal ) Positive Best AED cognitive side effect profile Least sedating and cognitive slowing Good mood stabilizer Negative - Black Box Warning Rash can evolve into Steven Johnson's Syndrome Occurs if lamotrigine (Lamictal ) is titrated too quickly, usually 6-8 week titration (Hosseini, 2008)

37 Lamotrigine (Lamictal ) Valproic acid and lamotrigine combined: Increased risk of SJS Combination of VPA and lamotrigine must be closely monitored If rash discontinue lamotrigine immediately No routine labs or levels needed, but levels can be drawn

38 topiramate (Topamax ) Neonatal period -> elderly Positive Weight loss Also used as anti-migraine agent Generally well tolerated Negative Cognitive slowing, difficulty thinking, fog like feeling (not in all patients) No routine labs (Hosseini, 2008)

39 valproic acid (Depakote ) Positive Effective when other AEDs fail, mood stabilizer, migraine prophylaxis Negative - Older generation drug Causes liver, pancreas, bone marrow problems, routine labs required SE s: Weight gain, polycystic ovaries, hair loss, tremor Increases most medication levels Can cause fulminant liver failure in children <2 years old (Hosseini, 2008)

40 ethosuximide (Zarontin ) Used for Generalized Absence Epilepsy Monitor drug levels, CBC and ALT/AST Take with food to avoid GI upset

41 Other Common AED s Phenobarbital, Primidone + Phenobarbital (Mysoline ) Partial, secondarily generalized, generalized tonic clonic, status epilepticus First choice for infants QD or BID dosing Monitor CBC, LFT s, blood levels Possible effect on development clonazepam (Klonopin ) Adjunct therapy Rescue medication

42 Rarely used AED s felbamate (Felbatol ) Partial seizures, Lennox Gastaut Syndrome Released 1993, FDA warning 1994 SE s Aplastic Anemia, liver toxicity vigabatrin (Sabril ) - now available in US Prolonged seizures, infantile spasms Ongoing studies

43 AED s rescue medications Lorazepam (Ativan ) and Diazepam (Valium, Diastat, Acudial ) Used for prolonged seizures Adjunct or rescue therapy Rectal or oral

44 Tips for AED Use Begin slowly and build up Use divided doses Give largest dose at bedtime Use one drug to toxicity before changing to another Serum levels are just a guide and are not done for all AED s.

45 Other Treatment Options Ketogenic Diet High fat, low CHO and protein Ketotic state may enhance seizure control Can be used with all seizure types All or nothing Patient/family education very important Major lifestyle change for family Close contact with provider, dietician

46 Other Treatment Options Vagal Nerve Stimulation Vagus nerve stimulation (VNS) is a type of treatment in which short bursts of electrical energy are directed into the brain via the vagus nerve, a large nerve in the neck.

47 Process Oriented Epilepsy Assessment for the School Nurse

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49 49 Child Neurology Process-Oriented Triage Model Rosenblum & Sprague-McRae 2009

50 Description of Symptoms What symptom(s) is/are the student demonstrating? Does the child have a diagnosis of epilepsy or other neurologic disorder? What is the classification? Is the child on medication for seizures? Other medications? What is seen at school and under what circumstances?

51 Description of Symptoms Baseline information Typical seizure presentation Full description of the event Activity prior to seizure, length, description of body parts involved, alteration of consciousness, post-ictal behavior, rescue medication used or not Date of prior seizure, circumstances, time

52 Epilepsy Management at School Many school nurses do not feel well prepared to provide seizure first aid. Olympia, Wan, Avner (2005) Surveyed school nurses (n=573) Seizure third most common school emergency 60% were confident managing seizure emergency More confident with cardiac arrest, airway obstruction, bleeding, fracture

53 Epilepsy Management at School Teachers and other school staff believe child with epilepsy is likely to Be a distraction to other students Require more time/attention Create a situation requiring emergency care Require additional staff training to manage Epilepsy and AIDS ranked as highest perceived overall impact in the classroom Olson, Seidler, Goodman (2004)

54 Epilepsy Management at School Increasing Self-Efficacy and Knowledge through a Seizure Education Program for Special Education Teachers (Price, Murphy, Cureton, 2004) Developed by School Nurses Designed to increase pre-school educators knowledge of seizures and seizure management Also measured effect of education program on educators self-efficacy in working effectively with families of children with seizures

55 Epilepsy Management at School Increasing Self-Efficacy and Knowledge through a Seizure Education Program for Special Education Teachers (continued) Findings: Educators had significantly increased scores related to knowledge of seizure management Significantly increased self-efficacy related to coping with seizures and assisting families Support the need for ongoing seizure education programs given by school nurses Price, Murphy, Cureton (2004)

56 Description of Symptoms Possible seizure triggers Illness URI, sleep deprivation, travel, missed medication doses, hormonal changes Change in pattern, duration, character from previous episodes Medication compliance Other triggers

57 Medical Update Anti-epileptic medications (AED s) child takes: Drug name, formulation, strength, route, dosing schedule, total mg/24 hours, toxicity or side effects Brand vs. generic Recent change in weight Compliance Issues affecting accessibility to medication

58 Medical Update Childhood Illnesses and Issues Acute illness can increase the frequency, intensity, and duration of seizures Use PRN medications (in addition to scheduled AED s) until concurrent illness improves and seizure frequency returns to baseline Information about co-morbid issues is helpful as this may impact seizure pattern

59 Medical Update Action Plan Consult with provider re: status of medical co-morbidities, drug side effects or interactions, recent acute illness, lab results, indications for adjusting AED Educate family Seizure etiology Factors that can exacerbate seizures Current medications

60 General Health and Psycho-social Sleep Children with seizures often have poor sleep patterns Nutrition Effect of AED medication Psychiatric co-morbidities ADD/ADHD, anxiety, OCD, other Behavior Communication

61 General Health and Psycho-social Effect of AED s on behavior Some AED s modulate behavior, others make it worse Full behavioral assessment necessary Communication and Social Skills Speech, language, social, emotional Eye contact, peer relationships

62 Family Dynamics and Coping Identify and Describe: Change in family dynamics or structure Provide information on nutritional, financial, insurance issues as indicated Assess parent/patient level of understanding of seizures/epilepsy Assess family support systems Encourage use of community resources

63 Family Dynamics and Coping-Action Plan Review nature of seizures and epilepsy, strategies to address current issues Discuss seizure safety management, i.e. water safety Discuss ongoing follow-up plan, trajectory of management

64 School and Therapy Programs Intervention services Frequently needed, i.e. Early Intervention, preschool, speech therapy, OT/PT Class Placement Mainstream, SDC, special accommodations as indicated Teacher Assess cognitive skills, language, focus, social interaction

65 School and Therapy Action Plan Obtain/verify exchange of information consent is present and up to date Initiate IEP/504 or other plan as appropriate Refer to school liaison, advocacy sources, counseling as needed Keep teachers and other school personnel informed of student status Facilitate special programs, i.e. adaptive PE

66 General Seizure Safety NEVER leave a person with epilepsy alone in a bathtub, not even for a minute Swim with supervision Don t use soft, overstuffed pillows, blankets, or toys in bed Generally do NOT need helmet or head protection Can participate in all school/playground activities to tolerance, avoid heights

67 Seizure Safety Action Plan Goal of Seizure First Aid Protect child from harm Most seizures are self-limiting and will stop on their own Goal of Therapy for Status Epilepticus Stop seizure as rapidly as possible to minimize morbidity and mortality Adhere to predetermined plan of action

68 Seizure Safety Action Plan Seizure First Aid Keep calm Do not hold down or restrict movement Clear the area around the child Turn gently onto one side DO NOT force mouth open or put anything in mouth Stay with child, reassure as consciousness and awareness return

69 Seizure Safety Action Plan Review with family Safety issues Water safety, helmet use Guidelines for 911 call Seizures worsening in frequency, intensity, duration Worsening co-morbid problems Medical, behavioral, psychiatric Interval follow-up with child neurology provider

70 School Emergency Plan School Seizure Emergency Plan Identify individuals responsible for managing emergency Have on file parent contact information, seizure protocol, information regarding usual seizure pattern, monitoring details Identify nearest hospital for transport

71 Seizure at School - Action Plan District Guidelines Epilepsy Foundation Recommendations Health Care Provider preferences Family preferences Templates (forms)

72 Seizure at School - Action Plan Diazepam Rectal Gel (1997) Used for acute treatment of seizures School nurse designated to administer medication Training of others (teacher, aide, principal, etc.) is controversial. Legal concerns Licensed or unlicensed personnel Reasonable accommodation..

73 Seizure at School - Action Plan Quality of Life Reduced disruption of daily activities Feelings of empowerment Acceptance of the Use of Diazepam Rectal Gel in School and Day Care Settings Parents completed questionnaire (n=64) 68% asked school to administer diazepam rectal gel, 81% reported school agreement, 19% reported refusal Refusals due to legal concerns, privacy, training Terry, Paolicchi, Karn (2007)

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75 Myths and Facts About Epilepsy Myth: A person having a seizure can swallow his tongue. Fact: Efforts to hold the tongue down or putting something in the mouth can hurt the tooth or jaw. Myth: People with epilepsy are dangerous or posessed by the devil. Fact: Epilepsy is a neurologic disorder, and it is rare that someone having a seizure will harm another person. Myth: You should hold a person down while he's having a seizure. Fact: You should make sure the area near the person is safe and that there is nothing hard or sharp. Myth: You should perform artificial respiration. Fact: Artificial respiration is only needed if the person does not start breathing after the seizure has stopped.

76 Provider Resources Epilepsy Foundation American Academy of Neurology American Epilepsy Society CDC Epilepsy website National Association of School Nurses