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Saturday presentations for LGS Foundation's first conference in the UK
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uk lgs foundation 2017 conference
- 4. WELCOME LGS UK's First Conference 3rd - 4th June, 2017
- 6. Thank You • GW Pharmaceu8cals • LGS Founda8on • All the speakers
- 9. Development
- 10. Age 3
- 11. Now
- 15. •When your healthcare provider talks in clear terms •When you are able to listen actively, absorb the key points, and understand and retain the information •When both parties are able to understand each other without confusion
- 32. LGS Foundation UK 3 Key Areas of Support for Families
- 34. 1. Education
- 35. Education: Children and Families Act – Importance of Section 19 Principles: Section 19 CFA provides that: “In exercising a function under this Part in the case of a child or young person, a local authority… must have regard to… the need to support the child and his or her parent, or the young person, in order to facilitate the development of the child or young person and to help him or her achieve the best possible educational and other outcomes.” Applies to everything done under Children and Families Act
- 37. Education Resources: •Local Offer •IASS - Information Advice and Support Services •Parent Carer Forums
- 38. 2. Health
- 39. EHC Plans – Health Sections • Section C – Health care needs relating to the young person’s SEN • Section G – Health Care provision reasonably required – the Clinical Commissioning Group CCG has veto • If the plan specifies health care provision, the responsible commissioning body must arrange the specified health care provision for the child or young person.
- 40. Health: Multi-Disciplinary Assessment of Needs • Behaviour • Cognition (understanding) • Communication • Psychological/emotional needs • Mobility • Nutrition (food and drink) • Continence • Skin (including wounds and ulcers) • Breathing • Symptom control through drug therapies and medication • Altered states of consciousness • Other significant needs “These needs are then given a weighting marked "priority", "severe", "high", "moderate", "low" or "no needs". “If you have at least one priority need, or severe needs in at least two areas, you should be eligible for NHS continuing healthcare. “You may also be eligible if you have a severe need in one area plus a number of other needs, or a number of high or moderate needs, depending on their nature, intensity, complexity or unpredictability.” www.nhs.uk Eligibility for NHS Continuing Healthcare
- 41. Health and Social Care – joint funding
- 42. 3. Social Care
- 43. EHC Plans – Care Sections • Section D – Social care needs relating to the child’s SEN • Section H – Social care for under 18’s as reasonably required under section 2 of the Chronically Sick and Disabled Persons Act 1970 and under the Care Act 2014 for those over 18 • Any other social care provision reasonably required by the learning difficulties and disabilities which result in the child or young person having special educational needs • LA can take account of resources when deciding whether ‘necessary’ (not just desirable) to provide service • BUT once accepted to be necessary to provide service, must provide sufficient service to meet need
- 45. Quality of Life Matters Happy Talk “Happy talkin’, talkin Happy talk, Talk about things you’d like to do You’ve got to have a dream, If you don’t have a dream, How you gonna have a dream come true?” South Pacific Musical Happy Talk" as written by Richard Rodgers Oscar Hammerstein II
- 48. “Knowledge is Power” – Sir Francis Bacon Education • Children and Families Act 2014 • New SEN regulations and the introduction of Education Health and Care Plans Health • Personal health budgets available from April 2014 • Continuing Healthcare eligibility criteria Guidance for children and young adults updated January 2016 • Health needs included in EHC plans from September 2014 Social Care • Chronically Sick and Disabled Children’s Act 1970 • Care Act 2014
- 52. Lennox Gastaut Syndrome • Age of onset – 1-7 years of age • Seizure types – tonic (mostly nocturnal), atonic, myoclonic, atypical absence, generalized tonic clonic, focal • Associated EEG pa6erns – generalized 1-2hz slow spike and wave, generalized slowing, paroxysmal fast acNvity (recruiNng rhythm) during sleep (see figures) • Common e9ologies – variety of eNologies, proceeded by infanNle spasms in 9-40% of cases • Treatment – felbamate, clobazam, rufinamide, topiramate, zonisamide, ketogenic diet, valproate, leveNracetam, VNS, corpus callosotomy, focal corNcal resecNon (if there is a focus) • Prognosis – moderate to severe intellectual impairment, usually correlates with eNology and seizure control American Epilepsy Society 2015 EEG: Slow Spike and Wave EEG: Paroxysmal Fast AcNvity
- 54. Loscher & Schmidt Epilepsia 2011 ~40 approved epilepsy medicaNons from 1850-2010 • Ketogenic diet • Modified Atkins Diet • Low Glycemic Index Diet • Devices (DBS, VNS, etc.) • Surgery
- 55. LGS Prognosis • Cogni9on – By 5 years from LGS onset, >75% of paNents have cogniNve impairment, >90% will eventually become intellectually disabled • Seizure – >80% will conNnue to have seizures throughout life • Psychiatric– Psychiatric and behavioral disorders are common in LGS • Mortality – 5-10% will die prematurely
- 56. Research Goals • Understand causes of LGS • Encouraging young invesNgators to study LGS • Discover new and beaer understand therapies • Help improve quality of life
- 62. The history of epilepsy geneNcs 1995 1998 2001 2004 2007 2010 CHRNA4 GABRG2 SCN1B SCN1A Copy number variant revoluNon Ion channel disorders > 100 candidate gene studies with negative results Courtesy of Ingo Helbig Next generaNon sequenc bubble
- 63. Gene CDKL5 SCN10A DNM1 RMND1 IQSEC2 WDR45 SCN8A GPR56 CHD2 STXBP1 GABRG3 SCN1A SCN2A DUP15Q ALG13 FOXG1 GABRB3 IDIC15 DNAJC6 DCX FLNA1 GRIN2B LGS associated genes
- 64. Savannah
- 65. • # of Seizures: >40,000 • Diagnosis: 3 years • # Neurologists: 7 • # Treatments: 26 • Cause: Unknown • Hospitalizations: 15 • Surgeries: 5 • Monthly drug cost: $1,640 • Diastat use: $183 / dose • Last stay: $53,475 Savannah’s Odyssey 1995-2011
- 70. Exome idenNfied numerous high impact variants in calcium channel subunits Approximate variant locaNon
- 71. Savannah Today
- 75. Gene Syndrome Change in Management Other Clinical Implica9ons SCN1A Dravet, MigraNng epilepsy of infancy Avoid sodium channel agents, SNripentol Monitor gait, Increased risk of premature death SLC2A1 Glucose transporter deficiency (GLUT1) Ketogenic diet Monitor for movement disorder HLA-A, HLA-B Variants found in certain ethnic groups Avoid Carbamazepine - PRRT2 InfanNle convulsions Consider Carbamazepine Surveillance for other neurological issues TSC1, TSC2 Tuberous sclerosis, InfanNle spasms Consider Vigabatrin, Rapamycin Surveillance for tumors, non-neurological issues ALDH7A1, PNPO Severe early-onset epilepsy Supplement with Pyridoxine (Vitamin B6) - PCDH19 Female epilepsy with intellectual disability Avoid carbamazepine, consider Clobazam - POLG1 Mitochondrial epilepsy disorder Fatal liver toxicity with Valproate - Precision therapeuNcs – now This slide does not represent medical advice Poduri 2017
- 79. Research Goals • Encouraging young invesNgators to study LGS • Grants Program • Travel Awards for scienNsts • Annual Research Round Table • Understand causes of LGS • Grantees: 2015 (Dr. Hunt & Dr. Myers), 2014 (Dr. Grone) • LGS EGI Exome Project (LEEP) • Discover new and beaer understand therapies • Grantees: 2016 (Dr. Poolos), 2014 (Dr. Thiele) • Rare Epilepsy Network (REN) Registry • Help improve quality of life
- 81. Thank you
- 82. Current and Emerging Pharmacological Therapies for LGS (Including Cannabidiol) J Helen Cross UCL-Great Ormond Street Institute of Child Health, London & Young Epilepsy, Lingfield. 4th June 2017
- 83. Treatment in Lennox Gastaut Syndrome • Principles of treatment of epilepsy • Treatments used in Lennox Gastaut Syndrome • What is the evidence? • Is there variability of use? • Newer agents on the horizon
- 84. Principles of Treatment • Choose baseline medication • One change at a time • Start low, go slow • Formulate a plan • Discuss possible side affects at outset • Reassess • Limited role of levels (NB phenytoin, concern re toxicity/compliance)
- 85. RCTs in childhood epilepsy 0 2 4 6 8 10 12 14 16 Partial epilepsy No.ofRCTs GTC LGS Infantile spasms JME Absence Neo- natal SMEI Updated from NICE HTA newer AEDs 2005
- 86. Problems • Trials • Aim to demonstrate safety and efficacy – rarely comparative studies (SANAD) • are predominantly in adult patients • are predominantly in patients with focal seizures • EMEA has relaxed data required for epilepsy where little age difference eg focal seizures • Rarely obtain syndrome specific efficacy • Availability
- 87. ‘Randomised Controlled Trials’ • Randomisation limits the potential for selection bias • Blinding minimises information and investigator bias • Precision determined by sufficient sample size, accurate assessment of outcome endpoints
- 88. • Short term efficacy relative to comparator • Common adverse events • Pharmacokinetic data • Optimal dosage • Benefit relative to other comparators/existing drugs • Long term retention/benefit • Related comorbidity • Seizure aggravation • Synergistic action with other drugs What do the trials tell us? What the trials don’t tell us?
- 89. Objectives in treatment • What are our current treatment strategies in LGS? • What medications have we available? • What can we expect from AED treatment? • Are there alternatives to AEDs – what are the likely outcomes? • How do we monitor effect? • What expectations should we discuss with families?
- 90. First line therapy – expert opinion Wheless et al, J Child Neurol 2005;20s1:s1-56
- 91. Sodium valproate: clinical experience in LGS • No controlled clinical trials • review of 336 patients (38 with LGS) given 400–3000 mg/day • ≥50% improvement in myoclonic astatic seizures seen for 11/11 patients on monotherapy and 10/27 on polytherapy1 • Reviews and opinion • overall 25–30% of patients attain ≥50% improvement2 • reduction in number of atypical absences and myoclonic seizures3,4 • poor efficacy versus tonic seizures, drop attacks and tonic-clonic seizures2,4 • efficacy greater in cryptogenic versus symptomatic LGS5 1. Covanis A. et al. Sodium valproate: monotherapy and polytherapy. Epilepsia 1982;23:693–720 2. Pisani et al. [Lennox-Gestaut syndrome: therapeutic aspects]. Riv Neurol 1989;59:217–9 3. Schmidt D, Bourgeois B. A risk-benefit assessment of therapies for Lennox-Gastaut syndrome. Drug Saf 2000;22:467–77 4. Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol 2003;20:426–41 5. Glauser TA, Morita DA. e medicine 2006. URL: http://www.emedicine.com/neuro/topic186.htm
- 92. Lennox Gastaut Syndrome • Cochrane review 2013 Hancock & Cross • 13 RCTs, 9 available • Cinromide N=56 2-17 yrs (7.5) • Felbamate N=73 4-36 yrs (13) • TRH N=98 ? • Lamotrigine N=169 3-25yrs (9) • Lamotrigine N=20 • Topiramate N=98 2-42yrs (11.2) • Rufinamide N=139 4-30 yrs • Clobazam N=68 • Clobazam N=217 2-60 yrs
- 93. Lennox Gastaut Syndrome • Cochrane review 2013 Hancock & Cross • 13 RCTs, 9 available • Cinromide N=56 2-17 yrs (7.5) • Felbamate N=73 4-36 yrs (13) • TRH N=98 ? • Lamotrigine N=169 3-25yrs (9) • Lamotrigine N=20 • Topiramate N=98 2-42yrs (11.2) • Rufinamide N=139 4-30 yrs • Clobazam N=68 • Clobazam N=217 2-60 yrs
- 94. Conclusions: Cochrane review • Optimum treatment for Lennox-Gastaut syndrome remains uncertain • No study has shown any drug to be highly efficacious in treatment of LGS • Lamotrigine, topiramate, felbamate and rufinamide may be helpful as add on therapy • Evidence base for one therapy over another limited; each patient needs to be considered individually, taking into account potential benefit of each therapy vs risk of adverse effects Hancock E, Cross H. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev 2013;CD003277.
- 95. Clobazam Randomised, phase III study results of clobazam in Lennox Gastaut syndrome Ng et al Neurology 2011 77;1473
- 97. Continued seizures – what next? • Have you the correct diagnosis? • Is it epilepsy? • Have you a syndrome diagnosis? • Have you used an appropriate drug? • What is the likelihood of response? • What drug next? • Should alternative treatments be considered?
- 98. Drugs to avoid Syndromes Juvenile myoclonic epilepsy CBZ, PHT,LTG,VGB, Myoclonic astatic epilepsy CBZ, PHT, VGB, Dravet CBZ, LTG, VGB Lennox Gastaut syndrome LTG, VGB, GBP PME PHT Angelmans syndrome CBZ Wolf Hirschhorn syndrome CBZ Focal seizures CBZ, GBP Delgado-Nunes et al BMJ 2012; 344:e28-35
- 99. Drug Combinations • Assume 10 commonly used drugs How many combinations of 1, 2 or 3 drugs are possible? Is the probability of any particular combination of drugs working independent of previous medications?
- 100. Objective Evidence for Seizure Reduction? Pujar et al 2010
- 101. What is a ‘New’ Treatment? A new medication A new way of management New data on old treatment
- 102. What is the likelihood ‘new’ medication will work? Kwan & Brodie NEJM 2000;342: 314-319 • 525 patients age 9-23 years • 333 (63%) SF during or after treatment • 470 previously untreated • 222 (47%) SF with initial drug • 67 (14%) SF during second or third • If first drug not effective - 11% SF Camfield & Camfield Epilepsia 2007;48:1128-1132 • 692 children followed over 20 years; 80 symptomatic • 17 LGS highest risk of intractability (94%; p<0.001 compared to all others)
- 103. Seizure freedom with additional drugs? 285 drug additions in 155 patients; 16% resulted in seizure freedom (28% SF) Luciano & Shorvon. Ann Neurol 2007 Median 20 months assessment
- 104. How do we measure treatment effect? • Single centre studies likely to have relatively small proportion of LGS • AED effect likely to have to be determined on individual basis • ? Studies require review of alternative outcome measures to seizure frequency
- 105. How do we measure treatment effect? • Seizure frequency • Seizure type? Over what time frame? • Frequency of emergency medication • Admissions to hospital • Attendance in school • Cognitive and behavioural effects • Can they be quantified within a trial situation • Lack of relevant standardised tests • ‘Quality of life’ specific goals different in each child • What is clinically relevant?
- 106. Zonisamide* Japan, pre-registration trials, N=1008 Ohtahara 2006 * Licensed for add on therapy in refractory partial seizures >18 years
- 107. Zonisamide* tolerability & safety Safety Ohtahara & Yamatogi 2004 • 1512 patients – 928 children • 244/928 reported adverse events • 18.9% monotherapy; 30.4% polytherapy • Most common adverse events • Mental/psychiatric 19.4% (includes mental function, motivation or volition) • Gastrointestinal symptoms 8.7% • Neurological symptoms 5.8% * Licensed for add on therapy in refractory partial seizures >18 years
- 108. Cannabis • Cannabis: for the most part, Cannabis sativa. • One of the most widely used recreational and medicinal drugs worldwide. • ~150 million people smoking cannabis daily (WHO) • Likely the first non-food plant cultivated by humans (~8000 BC) • Best known for its psychoactive constituent, Δ9- tetrahydrocannabinol (‘THC’).
- 109. The endocannabinoid system • First described in the late 1980s/early 1990s • Endogenous ligands, receptors, synthetic and degradation enzymes • Cannabinoid receptors: • cell surface receptors • present on a wide variety of cell types. • two CBR types: • CB1: • CB2: • Endocannabinoids: • anandamide • 2-arachidonoyl glycerol • Numerous synthetic ligands for CB1Rs and CB2Rs have also been developed Anandamide 2-arachidonoyl glycerol (2-AG) Courtesy of Dr Ben Whalley
- 110. Summary of historical preclinical evidence • Large preclinical evidence base asserting mixed effects on seizures in animal models 29 Compound Species Number of discrete conditions/models/designs Dose Anticonvulsant No effect Proconvulsant THC 6 31 0.25-200 mg/kg 61% 29% 10%* CBD 2 21 1-400 mg/kg 81% 19% 0% Other plant cannabinoids 2 7 N/A 100% 0% 0% CB1 receptor agonists 2 55 N/A 73% 18% 2% (7% mixed effect) *Includes non-seizure studies where convulsions were reported (see next slide) Whalley (2014) Cannabis and Seizures American Herbal Pharmacopeia
- 111. No reliable conclusions can be drawn at present regarding the efficacy of cannabinoids as a treatment for epilepsy. The dose of 200 to 300 mg daily of cannabidiol was safely administered to small numbers of patients generally for short periods of time, and so the safety of long term cannabidiol treatment cannot be reliably assessed. Four randomized trial reports, 48 patients, each of which used cannabidiol as the treatment agent. One report was an abstract and another was a letter to the editor. Anti-epileptic drugs were continued in all studies. Details of randomisation were not included in any study report. There was no investigation of whether the control and treatment participant groups were the same or different. All the reports were low quality. The four reports only answered the secondary outcome about adverse effects. None of the patients in the treatment groups suffered adverse effects. Cannabinoids for epilepsy D Gloss & B Vickery Cochrane Database of Systematic Reviews 2014, Issue 3. Art.No.: CD009270.
- 112. The British Journal of Psychiatry (2011) 198, 442–447. • 104 chronic cannabis, • 49 early-onset users • 55 late-onset users • 44 controls • No differences in IQ, vocabulary or block design • The early-onset group had • more perseverative errors • completed fewer categories on the WCST and on the Stroop test, poorer performance on the FAB
- 114. Parental survey
- 115. Cannabinoids: GW Pharma • Pure cannabidiol and cannabidivarin; almost insignificant THC • CBD is one of two major cannabinoids in Sativex • Human exposure to pure CBD in clinical trials has been limited
- 116. FDA IND; open label protocol Epidiolex (CBD) Inclusion criteria • Intractable early onset epilepsy • < 3 AEDs (not including VNS or KD) • Non progressive disorder • No significant laboratory abnormalities Protocol • 4 week baseline seizure diary • CBD 5mg/kg/day • Titrated at 2-5mg/kg increments until tolerance or max 25mg/kg/day • Labs for FBC, Liver, kidney function & AED levels 4, 8 and 12 weeks Devinsky, Sullivan, Friedman, Thiele, Marsh, Laux, Hedlund, Tilton, Bruno, Bluvstein, Cilio
- 117. Cannabidiol in childhood epilepsy • 214 patients across 11 sites • safety & tolerability 167 • Adverse events 79% • 137 efficacy analysis Dravet N=32 49% responders, 3% SF LGS N=30 37% responders, 3% SF Lancet Neurology e-pub ahead of print 15th December 2015
- 118. Conclusion: cannabidiol might reduce seizure frequency and might have an adequate safety profile in children and young adults with highly treatment-resistant epilepsy. Lancet Neurology 2016;15:270-8 Safety analysis (N=162) Somnolence 41 (25%) Decreased appetite 31 (19%) Diarrhoea 31 (19%) Fatigue 21 (13%) Convuslsion 18 (11%) Increased appetite 14 (9%) Status epilepticus 13 (8%) Lethargy 12 (7%) Weight increased 12 (7%) Weight decreased 10 (6%)
- 119. AED interaction; clobazam 13/25 children CBD 2— 25mg/kg/day Mass General Hospital, Boston Norclobazam increased in 12/13 Side effects 10/13 • Drowsiness 6 • Ataxia 2 • Irritability 2 • Restless sleep 1 • Urinary retention 1 • Tremor 1 • Loss of appetite 1 Geffrey et al, Epilepsia, 56(8):1246–1251, 2015
- 120. >50% reduction • Clobazam 36/70 (51%) • No clobazam 18/67 (27%) Multiple logistic regression clobazam use only independent predictor of reduction >50% in motor seizures Lancet Neurology 2016;15:270-8
- 121. Hemp oil <0.3% THC CBD 5%, THC 0.2%
- 122. Cannabidiol dose and label accuracy in edible medical cannabis products JAMA June 23/30, 2015 Volume 313, Number 24 August to October 2014; individuals sent to dispensaries in San Francisco, Los Angeles, and Seattle, USA. Entire package contents were assessed
- 123. • Anecdotal, open label and now RCT evidence of benefit - short term tolerability evident • Need for further trial data Efficacy Safety • Long term safety/tolerability & sustained efficacy requires evaluation – pure CBD vs ?THC • Possible wider benefits in comorbidities (eg anxiety disorders, cognition) requires further consideration Where are we now with cannabinoids?
- 124. UCL - Institute of Child Health New and Emerging Therapies in Epilepsy Paediatric Epilepsy Update 9th September 2016 Sophia Varadkar, MRCPI, PhD Consultant Paediatric Neurologist & Honorary Senior Lecturer Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health, London, UK
- 125. UCL - Institute of Child Health Disclosures • I have received educational travel support and honoraria for speaking engagements from LivaNova and UCB with remuneration paid to my department
- 126. UCL - Institute of Child Health Outline 1. Introduction 2. Drug i. Novel targets ii. Re-purposing iii. Re-visiting iv. Concept of precision therapies 3. Non-drug i. Ketogenic diet ii. (Metabolic epilepsies) iii. Stimulation therapies iv. (Epilepsy surgery) v. Immune therapies
- 127. UCL - Institute of Child Health 1. Introduction
- 128. UCL - Institute of Child Health Expectations of treatment and treatment response in epilepsy • Common • Not just one condition • Treatment aim is seizure freedom • Achieved with the first drug in 70% • Achieved with the second drug in a further 10-20%
- 129. UCL - Institute of Child Health ILAE Consensus Definition Failure of: • adequate trials of • two tolerated • appropriately chosen • and used AED schedules (monotherapy or combination) • to achieve seizure freedom
- 130. UCL - Institute of Child Health Quantifying the response to AEDs: Effect of past treatment history Schiller, Y. et al. Neurology 2008;70:54-65 How many drugs should you try?
- 131. UCL - Institute of Child Health AED development 1910 1920 19301940 1950 19601970 19801990 2000 2010 phenobarbitone phenytoin lamotrigine sodium valproate carbamazepine tiagabine zonisamide perampanel clobazam stiripentol lacosamide topiramate gabapentin vigabatrin levetiracetam ketogenic diet Slide courtesy of Prof Helen Cross
- 132. UCL - Institute of Child Health 2. New in treatment - Drug i. Novel targets a. Neuronal synapse b. Other sites ii. Re-purposing drugs used in other diseases iii. Re-visiting drugs used for other epilepsies or abandoned in the past
- 133. UCL - Institute of Child Health Site of action of AEDs at the neuronal synapse Bialer & White; Nature Reviews Drug Discovery 2010: 68-82 • Consider a new drug which acts on a different site • Consider synergism
- 134. UCL - Institute of Child Health New sites of action • Perampanel – post-synaptic AMPA receptors • Cannabadiol – CB1 and CB2 receptor ligand • (Retigabine – potassium channel)
- 135. UCL - Institute of Child Health Perampanel – novel site at the neuronal synapse
- 136. UCL - Institute of Child Health Perampanel - highly selective, non-competitive, post- synaptic AMPA receptor antagonist Redrawn and adapted from [2] and [3].1Hanada et al. Epilepsia 2011;52:1331–1340; 2Rogawski MA, Löscher W. Nat Rev Neurosci 2004;5:553–564; 3Rogawski MA. Epilepsy Currents 2011;11:56–63. 13 Post-synaptic neuron Pre-synaptic neuron Inhibitory interneuron Post-synaptic excitability AMPA receptor Glutamate NMDA receptor
- 137. UCL - Institute of Child Health % of patients achieving ≥50% reduction from baseline in seizure frequency/28 days *P<0.05 vs placebo **P<0.005 vs placeboN=45 N=21 N=13 N=44 N=20 ITT population: studies 304,305 &306, pooled. Double-blind phase 1Data on file, Eisai Inc; 2Rosenfeld et al. 2012 CNS; 3ISE Table 14.2. 2.2 Responder rate 14 Responderrate(%) Overall Population1 N=1478 Adolescents (306)2 N=60 Adolescents (304 & 305)2 N=83 Adolescents (Pooled)3 N=143 N=441 N=180 N=172 N=431 N=254 N=14 N=21 N13 N=12 N=31 N=32 N=20 Adolescents * * * * * Krauss GL et al. Neurology 2012;78(18):1408--1415; French JA et al. Neurology 2012;79:589–596: French JA et al. Epilepsia. Epub 20 Aug 2012; 4Krauss GL et al. Epilepsia. Epub 20 Aug 2012.
- 138. UCL - Institute of Child Health Perampanel – personal view • It works • Even seizure freedom • Even 2mg can be helpful • Prevention of evolution to bilateral convulsive • Long half-life • Introduce very slowly Bad • Aggression can prevent ongoing use • Dizziness to be expected • Can see overshoot • Interaction with other drugs – exacerbates lamotrigine side-effects Good
- 139. UCL - Institute of Child Health Cannabinoids – a (not so) new drug
- 140. UCL - Institute of Child Health Historical use of cannabis in epilepsy • Been around for millenia!
- 141. UCL - Institute of Child Health Cannabinoids Receptors • Endogenous cannabinoid receptors identified in 1988 – CB1 – CB2 • Cannabinoids are ligands at these receptors 3 groups • Endocannabinoids - endogenous • Phytocannabinoids – plant derived Cannabis sativa • Synthetic Cannabinoids
- 142. UCL - Institute of Child Health Two major cannabinoids THC • analgesic, anti- spasmodic, anti-tremor, anti-inflammatory, appetite stimulant and anti-emetic properties CBD • Anti-inflammatory, anti- convulsant, anti-psychotic, anti-oxidant, neuroprotective and immunomodulatory effects. • Lacks the psychotomimetic and psychotropic effects of THC • May alleviate some of the potentially unwanted side- effects of THC.
- 143. UCL - Institute of Child Health What our patients are reading about ‘Charlotte Figi who suffered from Dravet’s syndrome - through treatments using Cannabidiol, her seizure frequency went from hundreds per week, to only a few per month.’
- 144. UCL - Institute of Child Health CBD for epilepsy so far Efficacy • 50% responders, 3% seizure free – Devinsky, Lancet Neurology 2015 – Friedman, NEJM 2015 Emerging experience • Side-effects – Somnolence – Decreased appetite – Diarrhoea – Tremor • Interaction with clobazam – Geffrey, Epilepsia 2015 • Abnormal liver function • Up to 25-50 mg/kg/day • Tolerance
- 145. UCL - Institute of Child Health Tuberose Sclerosis – novel targets away from the neuronal synapse
- 146. UCL - Institute of Child Health Tuberose Sclerosis – novel targets away from the neuronal synapse • mTOR inhibitors – rapamycin/ everolimus • Used for SEGA • Used for renal angiomyolipomata • Ketogenic diet and vigabatrin have effects via the mTOR pathway • Use for epilepsy? – Krueger et al Ann Neurol 2013 – Cardamone et al J Peds 2014 (Sirolimus) – Exist 3 trial
- 147. UCL - Institute of Child Health Repurposing & precision therapies
- 148. UCL - Institute of Child Health Neonatal epileptic encephalopathies 1 month 4 months0 1 year 6 months Epilepsy limited to females with mental retardation PCDH19 Early onset epileptic encephalopathy STXBP1, CDKL5, ARX, PLCB1, SLC25A22, SPTN1, SLC19A3 Early myoclonic encephalopathy Infantile epileptic encephalopathies KCNQ2 encephalopathy Epilepsy in infancy with migrating focal seizures SCN1A, KCNT1, PLCB1 Dravet syndrome SCN1A West syndrome CDKL5 in girls ARX in boys Copy Number Variants 8% Genetic basis of the Early Infantile Epileptic Encephalopathies Slide courtesy of Prof Helen Cross
- 149. UCL - Institute of Child Health Repurposing & precision therapies Loss of function mutations Gain of function mutations • KCNT1 • SCN2A • KCNQ2 • SCN1A
- 150. UCL - Institute of Child Health Repurposing & precision therapies Loss of function mutations Gain of function mutations • KCNT1 • SCN2A • KCNQ2 • SCN1A
- 151. UCL - Institute of Child Health Weckhuysen et al Neurology 2013;1697-703
- 152. UCL - Institute of Child Health Retigabine/Ezogabine • First-in-class K+ channel opener • FDA Drug Safety Communication 2013 – retinal abnormalities and skin discoloration • FDA 2015 – Manage risks • 2016 – production being discontinued Gunthorpe Epilepsia 2012
- 153. UCL - Institute of Child Health KCNT1- precision therapy? Epilepsy of infancy with migrating focal seizures • Gain of function mutation • Therefore use a Na channel blocker from cardiology – Quinidine; reverses gain of function in vitro (Milligan et al) – Mexilitine Quinidine • Favourable response in one patient – Bearden, Ann Neurol 2014 • Our experience- 2 cases treated: 1 no response; 1 no response and severe pulmonary vasculopathy • in vitro response to Quinidine does not always predict clinical response
- 154. UCL - Institute of Child Health Repurposing & precision therapies & re- visiting Loss of function mutations Gain of function mutations • SCN2A and SCN8A • Use Na channel blockers – High dose phenytoin and carbazepine • SCN1A – Avoid Na channel blockers – CBD – Revisting ‘old’ drugs
- 155. UCL - Institute of Child Health SCN1A - re-visiting fenfluramine • High doses used for obesity, heart valve thickening, taken off market • Effective for self-induced/photosensitive seizures – Boel, Neuropediatrics 1996 • Effective in Dravet syndrome – Ceulemans, Epilepsia 2012 • Mechanism uncertain: stimulates serotonin; 5HT2A agonist? • Randomised placebo control trial beginning in Europe and US
- 156. UCL - Institute of Child Health 3. Non-drug
- 157. UCL - Institute of Child Health Ketogenic diet • A high fat diet, designed to mimic the metabolic effects of starvation, used in the treatment of epilepsy • Modified Atkin’s Diet for teenagers and adults
- 158. UCL - Institute of Child Health Ketogenic diet – decanoic acid • Seizure control by decanoic acid through direct AMPA receptor inhibition – Chang, Brain 2015 • ↑DA:OA could make diet better • Seizure control by ketogenic diet- associated medium chain fatty acids • Chang, Neuropharmacology 2013 • Now tolerability trials - ‘Betashot’ with low glycaemic diet • To improve outcome of epilepsy surgery
- 159. UCL - Institute of Child Health Rasmussen Syndrome/Encephalitis (RE) • Progressive, unihemispheric inflammatory disorder. • Cerebral atrophy • Clinical triad: – Intractable focal seizures – Increasing cognitive impairment – Progressive hemiparesis • Presumed autoimmune aetiology.
- 160. UCL - Institute of Child Health Treatment of symptoms • Seizures – Anti-epileptic drugs • limited effect, aim to prevent bilateral convulsive • Topiramate • Perampanel for EPC – Gode J Epileptology 2016 – Botox for EPC Lozsadi Neurology 2004 – Steroids – (VNS, TMS)
- 161. UCL - Institute of Child Health Immune basis to RE • Antibody-mediated CNS degeneration • T-cell cytoxicity • Microglial-induced degeneration – mTOR activation co-localising with microglial activation, Liu Acta Neuropath Comm 2014 • Inflammatory gene expression • Up-regulation of HMGB1 and toll-like receptor in surgical specimens – Luan Epilepsy Research 2016
- 162. UCL - Institute of Child Health Treatment directed against the primary process In 1990s and 2000s • Plasmaphoresis • Immunoglobulin • Steroids • Immunosuppressive therapy Tacrolimus Bien et al 2004 Azathioprine Varadkar et al 2011 Monoclonal antibodies • Rituximab – Laxer Epilepsia 2008 • Natalizumab – Bittner Neurology 2013 • Adalimumab – Lagarde Epilepsia 2016 – Cognitive stabilisation?
- 163. UCL - Institute of Child Health VNS Therapy: next generation device – what’s new?
- 164. UCL - Institute of Child Health What we already knew Benefits of magnet mode stimulation • May abort or decrease severity of seizures1-3 • May improve postictal recovery2 • Offers more control for patients and their families1,2 What about missed treatment opportunities? • By day/night • What if magnet mode didn’t rely on a person? 1. Boon P, et al. J Clin Neurophys. 2001;18:402-407. 2. Fromes GW, et al. Epilepsia. 2000;41(suppl 7):117. 3. Schachter SC and Saper CB. Epilepsia. 1998;39:677-686.
- 165. UCL - Institute of Child Health New Generation VNS Therapy AspireSR What’s new? – (Standard VNS Therapy stimulation with on-demand magnet stimulation) – DNA™ Technology • Seizure detection algorithm based on ictal tachycardia • Automatic stimulation upon seizure detection Detect, No)fy, Act, cardiac-based detec)on system
- 166. UCL - Institute of Child Health 43 Seizure Detec?on Threshold Background Heart Rate Foreground Heart Rate How Does Seizure Detection Based Upon Ictal Tachycardia Work? Detec?on algorithm – Live Example Data on file, Cyberonics Inc . Houston Tx Foreground heart rate = short term average (~10 seconds) reacts quickly to heart rate changes Background heart rate = long term average (~5 minutes) reacts slowly to heart rate changes
- 167. UCL - Institute of Child Health Aspire SR: E36 study • European multi-centre study • Prospective, unblinded • Recorded vEEG and ECG, ictal and non-ictal in the EMU • Looking at performance, safety and efficacy
- 168. UCL - Institute of Child Health Decision at GOSH to implant Aspire SR • Do our children get ictal tachycardia? – At first, we implanted those with confirmed ictal tachycardia on VT • Standard VNS therapy plus – Bigger battery – Auto-stimulation – So, greater dosing • Early impression is of earlier response
- 169. UCL - Institute of Child Health Potentially treatable metabolic epilepsies? NCL2 (Late infantile Battens) Intra-ventricular enzyme- replacement therapy NCL6 (variant of late infantile Battens) Intra-thecal viral vector gene therapy Pyridoxine dependent epilepsy Lysine restriction diets Molybdenum cofactor and sulphite oxidase deficiencies Purified cyclic pyranopterin monophosphate IV
- 170. UCL - Institute of Child Health Summary • Though there has been a slowing of drug- discovery, treatment options for drug-resistant epilepsy continue to expand • Novel sites and modes of action are promising avenues to explore • C10 medium chain fatty acid may be key in the ketogenic diet • VNS Aspire SR device has a cardiac based seizure detection algorithm which may enhance stimulation therapy
- 171. UCL - Institute of Child Health New and Emerging Therapies in Epilepsy Paediatric Epilepsy Update 9th September 2016 Sophia Varadkar, MRCPI, PhD Consultant Paediatric Neurologist & Honorary Senior Lecturer Great Ormond Street Hospital for Children NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health, London, UK
- 172. Ketogenic Diets in the treatment of LGS patients Dr Chris(n Eltze Consultant Paediatric Neurologist Great Ormond Street Hospital for Children
- 173. KD – treatments in LGS • What are ketogenic Diets ? • How does it work ? • What’s the evidence for effec(veness? • When should KD be considered ? • What about side effects ? • How can KD treatments be accessed ? • Other dietary therapies and new research
- 176. History • Fasting suppresses seizure activity: • Conklin (J Am Osteopathic Assoc 1922;26:11-14) Early reports of improving seizure control ‘patient deprived of food……up to 25 days’
- 177. What are ketogenic diets ?Normal Diet Energy from fat rather than carb’s
- 178. MCT Ketogenic Diet Modified Ketogenic Diet (MOD)Classical Ketogenic Diet 4:1 E. Neal, 2012 Dietary Treatment of Epilepsy, Wiley-Blackell
- 179. Classical Ketogenic Diet (CKD): (Long Chain Triglycerides) • Using standard food for composi(on of meals plans: 2:1, 3:1 or 4:1 - fat: (carbohydrate + protein) ra(o • Up to 90% of total calories from fat • Meal/snack recipes, all in correct ra(o
- 181. Medium Chain Triglyceride (MCT) Ketogenic Diet • 40-60% of daily calorie intake as MCT oil / MCT food product (overall 75% fat) • less carbohydrate restricted (15-20% of total calories) • (Possibly) Greater choice of foods
- 182. Modified Ketogenic Diet (MOD) • Carbohydrate restric(on (10-20g/day) • Encourages consump(on of high fat food • No limit on proteins • (No limit on total calories) • Fat : (carbohydrate + fat ) 1:1 ra(o • 70% fat, 25% protein, 5% carbohydrates
- 183. Vitamin supplements • Addi(onal: • Vitamin D3
- 185. How does in work ? • Exact mechanisms unknown - many hypotheses • Effects mediated by polyunsaturated fa_y acids • Ketosis induces shibs in brain amino acid handling favouring GABA produc(on • Suppression of seizures mediated by adenosine ac(ng on adenosine A1 receptors • Reduc(on of toxic waste products in the brain cells • Improve energy produc(on pathways in the cell • Reduce cell death in some animal models • Supresses inflamma(on • Changes ‘biochemical state’ of body • Cells use ketones ( derived from fa_y acids ) as main source of energy • Complex adap(ve processes take place → Enhance suppression of seizure ac(vity Excita(on Inhibi(on Epilep(c seizure Excita(on Inhibi(on
- 186. Neuroprotective effects of KD Maalouf et al 2009 , Brain Research Reviews • Improvement of mitochondrial func(on • Decrease of reac(ve oxygen species – reduc(on of oxida(ve stress • Increased ATP produc(on • Inhibi(on of apoptosis • An(-inflammatory effects Potential role of KD following brain trauma and in neurodegenerative conditions
- 187. What’s the evidence ?
- 188. Borges K, Epilepsia 2008; 49suppl8:64-66 Animal seizure models
- 189. Efficacy in childhood epilepsy D Keene Ped Neurol 2006;35:1-5 A systematic review • 26 studies;14 met criteria for inclusion, mostly retrospec=ve, no control group • Outcome measures degree of seizure control, dura=on pa=ent remained on diet, occurrence of adverse events • Total collec=ve popula=on 972 pa=ents • At 6m • 15.6% (CI 10.4-20.8) seizure free • 33.0% (CI 24-41.8) >50% reduc=on
- 190. Newer studies: comparing KD vs control group (not on KD)Study N Age (y) Type of KD Dura=on (months) > 50% sz reduc=on > 90 sz reduc=on Seizure free Neal et al 2008 1451 2-16 CKD MCT 3 KD: 28/73 (38%) C: 4/72 (6%) KD: 5/72 (7%) C: 0 KD: 0 C:0 Sharma et a 2013 1022 2-14 MOD 3 KD: 26/50 (52%) C: 6/52 (11.5%) KD:15/50 (30%) C: 4/52 (7.7%) KD: 5 (10%) C: 0 Lambrechts et al 2017 483 1-18 MCT, CKD 4 KD: 13/26 (50%) C: 4/22 (18.5%) KD: 3/26 (11.5%) C: 1/22(4.5%) KD: 3 (11.5%) C: 2 (9%) 1 14 (~ 10%) with LGS, 2 47 (46%) with LGS, 31 pa(ent with LGS
- 191. Which KD type works better ? • Classical KD versus MCT • Neal et al , Epilepsia 50(5):1109-1117, 2009 (45 on Classical, 49 on MCT; age 2-16y) • both KD types have comparable efficacy (no significant difference between mean percentage of baseline seizures at 3,6 and 12 months) • Classical KD versus MOD • Kim et al, Epilepsia, 57(1):51-58, 2016,(51 on Classical, 53 on MOD; age 1-18y) • Mean percentage of baseline seizures: • Aber 3 months: Classical KD 38.6%, MOD 47.9% • Aber 6 month: Classical KD 33.8%, MOD 44.6% • Difference not sta(s(cally significant in overall group
- 192. Does KD work in LGS patients ?
- 193. Lennox Gastaut Syndrome • Lemmon M et al 2012, n=71, mean age 3.6 y, classical KD
- 194. Lennox Gastaut Syndrome • Zhang et al, Epilepsy Research, 2016 • Retrospec(ve case not review • Classical KD n=47(age 1-16y) • Aber 3 months • 47% ≥ 50% sz reduc(on • Aber 6 months: • 44.7% ≥ 50% sz reduc(on
- 195. Ketogenic Diet in Adults
- 197. Ketogenic Diet - Side Effects • Gastrointes=nal symptoms: • nausea, vomi(ng (worsening of Gastro-oesophageal Reflux), cons(pa(on • Low blood Sugar (occasionally in ini(a(on phase) • Excess ketosis – acidosis (ini(a(on phase) • Renal stones (3-6%) • Risk factors: young age, hypercalciuria, (tx with carbonic anhydrase inhibitors: Topiramate, Zonisamide) • Preven(on – potassium citrate (alkalinisa(on of urine) reduc(on from 6.7 to 0.9 % (McNally et al, Pediatrics, 2009) • Increased Bruising (Berry-Kravis et al, Ann Neurol 2000) • Weight loss, Inadequate growth • Pancrea((s • Hyperlipidaemia • Decreased bone density – fractures (Long-term treatment)
- 198. When to consider KD treatment • Seizures despite of AED treatment (usually - failure of ≥ 2 AEDs) • Poor tolerance to AEDs • (Rare) Metabolic disorders affec(ng • transport of glucose from blood into brain • Glut 1 transporter deficiency syndrome • Metabolism of glucose • Pyruvate dehydrogenase deficiency
- 199. How can KD treatments be accessed ? • Discuss referral to Ketogenic Diet Service with your Paediatrician, (Paediatric) Neurologist • KD-Team: • Doctor (Paediatric Neurologist) • Die((an • Epilepsy Nurse Specialist ⇒ Detailed assessment • Exclude contraindica(ons (i.e. rare metabolic condi(ons) • Recommend to address feeding difficul(es, ea(ng disorders and swallowing problems before (feeding support may be required) ⇒ Provide more informa(on and discuss which KD type most suitable ⇒ Agree with you treatment goals
- 200. When would be the KD be contraindicated ?• Metabolic condi(ons • Beta-Fa_y oxida(on defects • Organic acidurias • Pyruvate carboxylase deficiency (lac(c acidosis) • Rela(ve contraindica(ons • Feeding difficul(es (food refusal) • Swallowing problems (alterna(ve feeding route: NG tube or PEG) • Severe gastro-oesophageal reflux (frequent vomi(ng)
- 201. Starting on KD • Outpa(ent setng (for all pa(ents > 1 year and well) • Teaching session (Die((an, Nurse Specialist) • Follow up – regular telephone consulta(ons with die((an in ini(al phase • Aber 3 months review by KD team (outpa(ent appointment) • Review progress and treatment goals with pa(ent and family ⇒ Decision to con(nue or stop
- 202. Ketogenic Diet – Duration of Treatment • Aber 3 months assess efficacy • Consensus statement Kossoff et al Epilepsia, 50(2):304–317, 2009 • First effects aber 2 weeks • Dura=on of treatment • Ini(ally up to 2 years (than taper diet) • in sz free pa(ents sz control oben maintained 20% relapsed aber discon(nua(on (Mar(nez et al 2007, Epilepsia) • In Glut 1 deficiency syndrome: • Con(nue into adulthood ? Transi(on to MOD
- 203. Low GI diet • ‘Glycaemic Index’ • Fewer fluctua=ons in glucose lead to effec=ve sz control • Carbohydrate restric=on – 40-60 gm/day • Muzykewicz et al 2009; Epilepsia Boston, Massachuse_s • Retrospec(ve • N=76 (89% had tried >=3 AEDs) • > 50% sz reduc(on • 54% aber 6 month • 66% aber 12 months
- 204. C 10 (decanoic acid) Potential explanation why MCT works Mitochondria – produce energy in cells • Increases number and func(on of mitochondria in cells • Hughes SD et al, J Neurochem, 2014 • Can suppress epilep(form ac(vity by blocking AMPA receptors (receptor for excitatory neurotransmi_er Glutamate) • Chang et al, Brain 2016 On-going first study to evaluate feasibility of new MCT food product (higher percentage of C10 ) Chief Inves(gator: Prof M Walker
- 205. Conclusions • The ketogenic diet is an effec(ve and safe treatment • Consider and discuss dietary treatment op(ons early in the course of LGS • Compliance barriers can be over come with help available online – resources (recipes, cooking demonstra(ons, prac(cal (ps, support groups)
- 206. On-going and future Research • Efficacy of KD in adolescents and adults • Efficacy and Safety of KD in very young children (<2 years) • Inves(ga(ng disease modifying effects of KD • Biomarker – that allow to predict response to KD • Course of epilepsy once KD is discon(nued (?disease modifying effect of KD) • Outcome data should also include development/cogni(on and behaviour
- 212. Moving on: Why • Your teenager is now and adult with adult needs • The paediatric se?ng dose not have the resources and experience to care for adults • Best environment
- 213. Parents anxie>es of Transi>on a teenager with LGS • Can be a complicated process • Consultants have known your teenager a long (me and it is felt they know your teen best • My teenager is “Forever a child” • Scared of the unknown
- 215. Challenges for transi>on for Teenagers with LGS • Behaviour changes/frustra(ons • Learning difficul(es: mild/moderate/severe • Seizures types change from child to adult • Difficult to treat epilepsy • Changes with medica(ons • Stress on parents and family
- 216. Challenges for transi>on for Teenagers with LGS • Fluctua(on with seizures: days when seizures are more severe and affect func(on • Safety issues may change when seizures are more severe. e.g. mobility and alertness affected • Parents can under es(mate what teenagers with the most complex needs can enjoy and par(cipate in • Learning to let go
- 218. • Teenage Talks • Each clinic visit the teenager is given one agreed set goal • Epilepsy informa(on booklet for teenagers • Teenage workshops • Transi(on clinic at the adult hospital Teenagers With Epilepsy At GOSH
- 219. Teenage Talks- topics covered • Feeling posi>ve • Individual goals to help you take responsibility for your medical treatment • Bullying • Sport • Driving • Medica>on management • Noncompliance of medica>ons • Contracep>on • CigareVes, alcohol and drugs, • How can my friends help? • Enjoying yourself when you are out and about • Sudden unexpected death in epilepsy (SUDEP)
- 220. Transi(on Programme
- 221. Epilepsy Transi>onal Stages The beginning stage • Introduce the concept of transi(on • 12- 14 years • Commence Goals epilepsy goals of independence (individualised for many teenagers with LGS) • Gradually gain an understanding of their epilepsy
- 223. Epilepsy Transi>onal Stages My transi>on year • 16 years • Booklet /leQer of what is going to happen over the next year • Final ques(ons with teenager and parents of what will happen next
- 225. Ready Steady Go Transi(on programme Aims: 1. To support young people through transi(on and to encourage them to get involved in the transi(on process 2. To make them feel confident and happier about the change • hQps://youtu.be/30JMnQZz8nk
- 226. How can the parent prepare • Step by step process • Talk to families that have already transi(oned • Make notes and write down ques(ons • Listen to your teenager • Keep a record of past medica(ons • Seizure/behaviour/headache diary
- 227. Aims of Transi>on planning for a teenager with LGS • Awareness of the associated risks of having epilepsy to make healthy lifestyle choices • To be independent and their own advocate • To promote readiness and a smooth transi(on into adult services • Have a sound knowledge of all their health care needs • To prevent being lost in Transi(on
- 228. Outcomes • To be safe and less vulnerable • To reach their full poten(al • To par(cipate and be independent as much as possible
- 231. 1. Remember when you need to take your medicines in the day Reason Ø To keep you safe Ø Medicines help you to have fewer seizures. Ø Having fewer seizures helps you to be able to do the things you want to do and not worry. Tips Ø Remind mum and dad you are due your medicines in the day and evening Ø Set an alarm Ø Use the reminder on the phone app
- 232. 2.Learn how much liquid or many tablets you need to take Reason • This will help you to become more independent Tips • How much syrup or tablets do you take? • Use a syringe and pull the right amount of medicine out • If you're on tablets count the amount you need. • If you have trouble remembering take a photo and keep it on your phone. If you don’t have a phone ask nurse Emma to give you pictures of your medica>ons and you can keep them in a liVle book to show your doctor or nurse when you go and see them.
- 233. 3. Help your parents put your tablets in a medicine wallet for the week Reason • To help you become independent Tips • If you are on tablets use a medicine wallet • Put your medicine in each day for the next week. • Do this on the same day every week. • Perhaps do this every Sunday ready for the start on the Monday
- 234. 4. Learn the names of your medicines Reason • To help you become independent • As you already know how many medica>ons you are on • Now learn the names of your medicines • It easier to learn them one at a >me. • Know what colour the liquid or tablet is of which medicine • Put a photo of medicine packet or boVle on your phone
- 236. 6. Talk to someone when you feel sad or worried and how you feel about growing up Reason Ø So you can live a happy life Tips • Talk to people if you feel angry or stressed about your seizures • If your seizures make you want to cry • If you don’t want to talk to mum or dad, talk to someone you trust: friend, teacher, support worker. • Nurse Emma can also talk to you in clinic or on the phone about how you feel and if you have any worries about your epilepsy. • Lots of teenagers like you who have epilepsy can feel sad and fed up • We all care about you and want to help you feel good
- 237. 7. Tell your friends you have seizures and what happens to you Reason • To keep you safe Tips • Tell your best friend you have epilepsy • Tell your teacher • If you are worried about telling people, tell one friend first
- 238. 8. Help your parents keep your seizure diary up to date. Reason • To see if your seizures are ge?ng worse over a month • To be independent Tips • Use seizure diary • Use App on phone • Your parents can help you do this.
- 239. 9. Learn what 'sets off ' your seizures Reason: • So you can recognise and try and make your seizures stop Things that can make you have seizures • Worried/scared • Periods • Headaches • Being (red • Smells • Noises • Not enough sleep • Not taking your medica(ons on (me or forge?ng to take them • Alcohol and drugs: Don’t drink too much alcohol and dot take illegal drugs.
- 240. Things to help you feel beVer, relaxed and healthy • Going out with your friends • doing things you enjoy: Pain(ng, games, • Exercise: play games and sport: football, swimming • Ea(ng fruit and vegetables • going to sleep on (me • Not ea(ng too much • You must tell your Dr if your seizures are ge?ng worse. You can call nurse Emma if you are worried and she will talk to you and your parents about this.
- 241. 10. Taking more control of yourself as your body is changing Reason: • To keep you safe Tips • It is nice to be clean and healthy • Wash every day: Have a shower as this is safer. • If you have many seizures then you will need a shower chair to keep you safe in case you have a seizure. • Make sure the water is not too hot
- 243. Handover Clinic Na>onal hospital • Adult Neurologist: Prof MaQhias Koepp • Dr Sophia Varadkar • CNS Emma Ninnis
- 244. Thanks • Dr Sophie Varadkar • Dr Sarah AyleQ • Prof MaQhias Koepp • Dr Sophia Erikson • GOSH Adolescent Steering Group
- 245. Thanks for Listening