The First time a parent hears the words, Something is wrong. Good Morning, Beautiful, Winning the Battle Over Seizures. Advocates in a foreign land.

Transcription

1 Good Morning, Beautifu, Winning the Batte Over Seizures The First time a parent hears the words, Something is wrong. The ove of a chid carries deep emotion. The onset of seizures changed our word. We had never seen a seizure before. Advocates in a foreign and. Without a medica background reading an EEG was ike trying to read words in a foreign anguage. Learning as much as we coud about seizures and treatments, changed the course of treatment. Advocacy * Finding the right diagnosis is crucia. * The best option for treating epiepsy is at comprehensive epiepsy center. * Work together with the neuroogist and team of doctors. It shoud be a team effort. The mirace of the Ketogenic diet. Since the 192 s The ketogenic diet Has been used as A treatment to Stop seizures. With the introduction of anti-seizure medications in the 5 s, the diet was used ess and ess.

2 The diet paved the way for Brain Surgery Because we were at a comprehensive epiepsy center our daughter received the right care and treatment Advocacy and the team approach Advocate: A person who peads for or in behaf of another; intercessor Dictionary.com unabridged. The greatest success is found in those who don t give up. Surgery at 4 ½ Options for the Treatment of Pediatric Epiepsy Jeffrey Buchhater MD, PhD Comprehensive Pediatric Epiepsy Program Chidren s s Neuroscience Institute Phoenix Chidren s s Hospita Phoenix Chidren s s Hospita Webinar Apri 8, 21 Discosure Fu-time empoyee Phoenix Chidren s Hospita No Financia interest in any pharmaceutica or device manufacturer Consuting reationship with any pharmaceutica or device manufacturer Off-abe useage Learning Objectives Goas of Treatment Understand the options for different types of pediatric epiepsies Seizure-freedom or maxima contro of seizures Understand the roe of parenta advocacy for maximizing effective treatment of pediatric epiepsies No or acceptabe adverse effects from the treatment Optimize the quaity of ife

3 Reasonabe Expectations- What is the ikeihood that my chid wi be seizure free? It Depends Seizure type Seizure number Epiepsy Syndrome Likeihood of Seizure Contro Approximatey 7% of individuas with a first unprovoked seizure wi not have recurrence Of the 3% who have recurrent seizures (epiepsy), 8% wi achieve seizure contro with one or more anti-epieptic drugs 2% of individuas wi have inadequate seizure contro Anti-Epieptic Drugs (AEDs) Which one is my best for my chid? Factors in Seecting an AED Seizure type Epiepsy syndrome Expected efficacy Possibe adverse effects Pharmacoogic profie & interactions Avaiabiity Avaiabe formuations Cost Od & New Anti-epieptic Drugs Prior to 199 Phenobarbita Phenytoin Ethosuximide Carbamazepine Vaproic acid Since 199 Febato Neurontin Lamicta Gabatri Topamax Triepta Zonegran Keppra Lyrica Vimpat Banze Sabri Sounds ike epiepsy contro shoud not be a big probem, except Epiepsy is a very common occurrence, so that 2 % amounts to a ot of peope For exampe..

4 Intractabe Epiepsy in Arizona Intractabe Epiepsy in Phoenix/Scottsdae < 18 years Assume: Popuation Assume: Popuation ~5,5, ~1,43, Assume: Epiepsy 1% prevaence 55, Assume: Epiepsy 1% prevaence 14, Assume: 2% intractabe 11, Assume: 2% intractabe 2,4 Eary Identification of Lack of Response to Anti-Epieptic Drugs Success of AED Use < 18 years od Response to first drug 47% Partia seizures ~ 64% ( 3 AEDs) Response to second drug 13% Catastrophic epiepsies < 2% Response to third or mutipe drugs 4% Spasms Lennox Gastaut Kwan & Brodie, NEJM 2 ESES Bottom-ine Options When AEDs Fai If your chid is sti having seizures after two reasonabe anti-epietpic drugs have been used, he/she has intractabe epiepsy Accept current seizure frequency Try an investigationa AED Ketogenic diet If he/she has partia seizures the ikeihood of seizure freedom is 4% or ess Vagus nerve stimuator (VNS) Epiepsy Surgery If he/she has other seizures types, the prognosis may be worse

5 Ketogenic Diet KD- History Prayer & fasting improved seizures Prayer aone ineffective Dr. Howand, Johns Hopkins Fast- ketones + uric acid Fast- + fat- continued uric acid Dr. Wider, Mayo Cinic high fat, ow carbo & pro- "ketogenic diet 1937 Drugs 199 s- Charie KD- Genera Principes Caoric intake at 75-1% for idea weight Ratio of 3 4 : 1 (fats: protein + carbohydrates) At east 1gm/kg protein for maintenance, not growth Suppement with Ca containing, sugar-free, mutivitamin KD- Seizure Types KD- Age and Inteigence Most commony controed atonic, myoconic, absence Some contro tonic-conic conic Structura disorders microcephay, HIE, strokes, maformations < 12 mos- difficuty with ketosis & hypogycemia Traditionay years od Oder possibe if motivated No known IQ imitations

6 Pediatric Neuroogy 26;35:1-5 Pediatric Neuroogy Initiation of keto diet Mea components- not so bad! Outpatient Patient at home Gradua introduction of ketosis 1:1 1.5:1 2:1 2.5:1 Continues unti urine ketones measure arge Inpatient Hospitaize 5 days Patient fasted unti producing ketones Diet introduced at ½ strength, 4:1 ratio May start with ketonog and advance to foods Discharge when 4:1 Meat or egg = protein Vegetabe or fruit = carbohydrate Butter or mayonnaise = fat Heavy whipping cream = fat Keto meas Ketogenic Diet- Summary Effective for intractabe seizures Requires Knowedgeabe team Dietitian Nurse Physician Motivated famiy & team

7 Epiepsy Surgery Consider Epiepsy Surgery When Seizures not controed with medication Seizures controed, but side effects not toerabe Seizures interfere significanty with ife Not ikey to grow out of seizures Types of Epiepsy Surgery & Seizure-free Rates Tempora obe 6-8% Outside tempora obe 4-6% Hemispherectomy 6-8% Corpus caosotomy 6-8% (drop attacks) Vagus nerve stimuator 6-8% (drop attacks) JRB 29 Epiepsy Surgery- Myths & Reaities 1 Myth: : A chidren grow out of their seizures Reaity: : Ony chidren with intractabe seizures Myth: : Epiepsy surgery is experimenta Reaity: : Epiepsy surgery has known benefits & risks Myth: : Resected tissue resuts in oss of function Reaity: : Resected tissue is frequenty damaged & the pre-operative evauation assess risk Epiepsy Surgery- Myths & Reaities 2 Epiepsy Surgery: Bottom-ine Myth: : Surgery shoud be imited to deayed chidren Myth: : Surgery shoud be imited to norma chidren Reaity: : Different popuations of chidren have different expectations for outcome Concusion: : Epiepsy surgery is appicabe to a heterogeneous popuation of chidren with patient-specific goas If your chid is not seizure-free after being tried on 2 anti-epieptic drugs and Has seizures coming from 1 pace in the brain or Has a brain abnormaity (stroke, tumor, tuber ) that causes seizures Consider evauation for surgery in an experienced pediatric epiepsy surgery program

8 VNS Vagus Nerve Stimuator VNS VNS- How Big is the Device? VNS Programming Wand hed over VNS Settings changed with PDA Intensity of stimuation Frequency of stimuation Vagus Nerve Stimuator FDA approved (1997) as an adjunctive treatment of aduts and adoescents over 12 years of age with medicay refractory partia epiepsy. Aso used off- abe for generaized (esp drop) seizures at younger ages. Efficacy- approximatey 5% of individuas wi achieve a 5% or greater reduction in seizure frequency Advantages Low side effect profie (hoarseness when activated) No cognitive toxicity Disadvantages Requires surgica impantation

9 VNS: Bottom-ine Consider VNS when standard AEDs produce unacceptabe adverse effects, especiay for partia seizures Consider VNS for seizures that resut in drops after evauation for surgery Is there cause for hope? Impantation shoud be done in a comprehensive pediatric epiepsy center Progression in Understanding & Treatment of Epiepsy (Lennox & Lennox 196) Hippocrates Gaen Neurophysioogis ts Berger Wiis Gowers Jackson Boodetting, purging, Powdered human EXORCISM OF sweating, diet, herbs sku, DEMONS Liver of vutures Let out the bad humors Brain surgery Hydantions Ketosis Phenobarbita Bromide Progression in Understanding & Treatment of Epiepsy (Post 196) Knock-out/in modes Human mutations Spontaneous genetic modes VGB Acquired anima modes RNS RUF LAC Ceuar Systems physioogy DBS physioogy Neurochemistry OXC ZON VNS biophysics ME LTG LEV G TPM MR TGB PETMRI SPECT CT S GB Video-EEG FBM P VP CBZ A ET X? Resources Emiy Center Phoenix Chidren s Hospita Epiepsy Foundation of Arizona Phoenix Parent and Ketogenic Diet Support Group, Phoenix, AZ 7: p.m. - 9: p.m. Third Tuesday of the month St. Joseph's Hospita 35 W. Thomas Rd. Dining Room A (Across from Cafeteria entrance) Camp Candeight June 13-19th, 21 Ages 8-15 Heber, Arizona Camp Candeight is a one-week; seep over camp for chidren with epiepsy featuring traditiona camping and outdoor activities. This coming-of-age activity is especiay important for these chidren and teens whom may be acking sef-esteem and the opportunity to participate in activities in which they are not judged. The camp is staffed 24 hours a day by Epieptoogists and Nurses and with counseors who have received epiepsy recognition and response training.

10 Resources P: (62) 546-2CNI ( ) P: (62) Pauette George