Epilepsy and the Adolescent

1. Epilepsy and the Adolescent Sandya Tirupathi Epilepsy Fellow Beaumont Hospital

2. Epilepsy in adolescence • Commonest neurological disorder. • Prevalence: 1 in 100 in 14-16yrs old • About 75% of epilepsies- onset before 20yrs. • Epilepsies syndromes • Juvenile myoclonic epilepsy • GTCS on awakening • Symptomatic partial seizures (less frequently)

3. JME “Lots of blanks and jerks; then I had a grand mal… I usually have fits when rushing after getting up; usually does not happen later in the day” ………….An extract from a patient’s diary

4. JME: Clinical Characteristics Peak age of onset: 12-18yrs • Absences (33%), onset:10.5±3.4 yrs • Myoclonic jerks (97%), onset: 15±3.5yrs • GTCS (79%), onset : 16±3.5 yrs • Seizures are usually after 30-60min of awakening • Men and women equally affected • Triggers: sleep deprivation, stress, alcohol or menstruation Represents 2-5% of all patients with epilepsy.

5. JME EEG Characteristics • An EEG showing interictal or ictal 3.5-6hz multispike and wave. • EEG Photosensitivity is common (27%-41%) but 1/10 experience clinical photosensitivity. • HV accentuates the abnormalities in all patients.

6. JME: Asymmetries • Asymmetries are common* • Clinical only (14%) • EEG only (14%) • Both clinical and EEG asymmetries (2%) • Focal abnormalities are common** 54% had focal clinical or EEG or both. * Lancman ME et al, Epilepsia 1994 Mar-Apr;35(2):302-6 **Usui et al, Epilepsia, 2005; 46(10);1668-1676

7. JME: Misdiagnosis (90%) Factors contributing* • Physician failure to inquire about early morning myoclonus. • Misinterpretation of absences as complex partial seizures • Misinterpretation of jerks as partial motor seizures • High prevalence of focal clinical and EEG abnormalities. *Panayiotopoulos CP et al, Epilepsia 1991; 32:672-676

8. Management • Long term AED and avoidance of precipitating factors is essential. • Advice on alcohol and sleep deprivation is mandatory. • Valproate, Lamotrigine, Topiramate, Clonazepam, Levetiracetam, & Zonisamide – potentially effective. • CBZ, Vigabatrin, Tiagabine and Phenytoin are potentially aggravating- avoided • Relapse when AED is withdrawn is >90%

9. Adolescence • Adolescence is a transitional stage involving biological (i.e. pubertal), social, and psychological changes. • WHO:10-20 years • USA: 13-23years End of adolescence and beginning of adulthood varies by country as well by function.

10. Adolescence: time for bodily changes

11. …..Time for constant battles

12. Adolescent with Epilepsy: Challenges: • Uncertainty of seizure timing • Side effects of medications • Associated educational difficulties • Parental tendency toward overprotection • Face restrictions and fight for freedom • Social stigma

13. Adolescent Issues 1. Medical Issues 2. Educational and Employment issues 3. Social issues and restrictions 4. Transition/transfer of care issues.

14. Medical Issues: Establishing diagnosis • Provide a final screen to confirm (or refute) diagnosis of epilepsy, to corroborate, or correctly identify, the epilepsy syndrome and to ensure that the most appropriate AED is being prescribed. • Many paroxysmal events are non epileptic and it is not uncommon to be mistakenly diagnosed as having epilepsy and treated with AEDs. • Patients with TLE associated with Hippocampal sclerosis present often in late childhood or adolescence- appropriate neuroimaging.

15. Medical Issues: Ensuring most appropriate treatment • Review AED regimen and consider all possible side effects. • Delaying Surgery until adult life denies possibility of growing up in a seizure free environment which compromises emotional and employment potentials. • Consider cosmetic side effects as body image and peer acceptance are key issues in their lives. • Consider AED and OCP interactions. • Counsel on the teratogenic effects of AEDs • Folic acid supplementation

16. Medical issues: Compliance • 50% of teenagers with chronic diseases do not comply with care. • 20% - fully complied, 44%- satisfactory compliance and 34% -poor compliance* • Compliance with recommended life-style was poorest. *Compliance with health regimens of adolescents with epilepsy Kyngas, H. Seizure 2000; 9:598-604

17. Predictors of compliance in epilepsy: • Physician support – complied 10.56-fold • Parent support- complied 10.47-fold • Good motivation –complied 9.77 times • Adolescents who did not feel their disease was a threat to social well-being complied 8.38-fold Predictors of good compliance in adolescents with epilepsy. Kyngas, H. Seizure 2001;10:549-553

18. 2. Educational and Employment issues • At risk for failing to reach their goals. • Cognitive dysfunction • Side effects of AEDs • Family stress • Lack of school support • Employer discrimination

19. … Education and Employment • What can/may help: • Educational assessment in elementary school years • Education and counselling of families. • Epilepsy education as part of syllabus of teacher- training curriculum. • Minimise side effects from AEDs and treat comorbidities • Programs like “Training for Success” • Fighting employer discrimination through local epilepsy societies.

20. 3. Social issues: • Social difficulties-Result from: • Cognitive abnormalities • Adverse experiences • Social stigma • Parental psychopathology ( >32% -depression)* • Patient psychopathology (28.6% vs 6.6%)** • Physicians failure to recognise and promote patient autonomy. * Hoare P. Dev Med child Neurol 1984;26:14-9 ** Rutter M, William Heinemann Medical; 1970

21. .. Social Issues: • What can/may help: • Activities promoting friendships • Decision making- to shift to teenagers • Physician to encourage autonomy • Early recognition of parental psychopathology and early intervention of family dynamics by neuropsychologist • Early recognition and treatment of patient psychopathology

22. ..Social: Restrictions

23. ..Social Issues: Restrictions & freedom • Sports • Most are acceptable- builds self esteem • Driving • “rite of passage”. • Early discussion of regulations may promote compliance. • Alcohol • Balanced discussion vs outright proscription- promoting normal peer group socialising. • Recreational drugs • Cocaine, heroin, amphetamine, ecstasy, phencyclidine are proconvulsants.

24. 4.Who should look after the teenager with epilepsy? • Physicians with particular interest in adolescents should ideally treat them • Where specialist involvement is strictly defined by age “Transition clinics” staffed by both Paediatric and Adult specialists may permit to address their special needs

25. Family Concerns Development Growth Growing Independence Adult behaviour Reproduction and Employment Family Concerns Development Growth Patient Autonomy Reproduction Employment Recreation Paediatric Adult Cultures Adolescent

26. Why transition and not transfer • To address inherent problems of the teenage population • To avoid transfers of a haphazard and idiosyncratic fashion. • To prepare the child to take on responsibilities in a graded way. • To instil a sense of security and confidence amongst parents and families.

27. *Obstacles to a smooth transfer • Problems with coming of age • Problems with paediatric professionals • Problems with parents • Problems with receiving adult services • Structural hospital problems *Russel Viner Arch Dis Child 1999;81:271-275 “Transition from Paediatric to adult care- Bridging the gap or passing the buck”

28. Problems with Adolescence • Transition is a life event- losing respected and loved carers and being forced to trust unknown carers. • Non-adherence and oppositional behaviour may prompt abrupt transfers to adult care. • Some make contact with medical profession again in times of emergency, crises that regular contact, health promotion and education might have avoided (pregnancy, suicide attempts)

29. Obstacles from paediatric professionals • Inability of the paediatric professionals to “Let go” • Inability to trust the independence of the adolescent • Inability to trust the skills of the adult services. • This is especially true if there is no contact with target adult services.

30. Obstacles from adult services • Individual rather than family approach of adult physicians can be threatening to young people and families. Families often remain the strongest support and this approach may sabotage effective transition if they feel excluded from all decision making in new setting. • Adult physicians can be tempted to embark on extensive investigations and management reassessments soon after the first meeting – this can be unsettling for families and patients.

31. Obstacles from the hospital system • Poorly established and unreliable communication channels for transfer of old medical records and imaging results between hospitals. • During the transition period neither the paediatric nor adult services may feel fully responsible for patient care resulting in miscommunication, contradictory advice and potential conflict. • If a period of limbo occurs, patient may take the opportunity to opt out of both systems.

32. Worldwide: • N America and Australia: In the past 2 decades lead to rise of “Adolescent Medicine” • In Most parts: specialists treat both children and adults with epilepsy (specialist involvement not defined by age) • UK- “Transition Clinics” – Hand held transfers

33. *Managing the teenager with epilepsy: Paediatric to adult care • 1991 a clinic for teenager with epilepsy. • Clinic: Consultant paediatric neurologist, adult neurologist and a nurse specialist in paediatric epilepsy. (SR in absence of PN or AN but not both) • Referrals from 2 PN, Gen Paeds, rarely GPs. • Each patient was seen by all 3 jointly. *R.E.Appleton, D.Chadwick & A.Sweeney. Seizure 1997;6:27-30

34. ResultsR.E.Appleton, D.Chadwick & A.Sweeney. Seizure 1997;6:27-30 • 120 new patients. 63 males. • Referral age ranged from 12.8 to 21(16.3) • 64% PN, 33% from Gen P, 3% GPs • 116- diagnosis of epilepsy- PNs + Gen Ps • Remaining 4: 2 new onset epilepsy at 16 years and 2 recurrence of szs following stability (GPs) • 58% - PGE including JME (45%), • 22% - LRE (Cryptogenic) • 18% - symp/cyrptogenic gen syndrome • 2%- 2 brothers had PME of unknown cause.

35. ….Did the clinic make a difference? • 18(15%) patients had diagnosis revised. 15 had been ref by gen Ps and 3 by a PN. • Vasovagal in 8, panic attacks in 2, hemiplegic migraine in 1, paroxysmal CA in 1 – all 12 were on AEDs • NES in 1( she also had well controlled epilepsy) • A different syndrome was identified in remaining 6. 4 with JME- all referred by a Gen P.

36. …….Changes to therapy • Following initial consultation 14 patients had meds changed and in 12 gradually stopped. • 85% were on monotherapy ( from 80%) • No one was on more than 2 AEDs in most recent f/u and none on phenytoin.(8 were on 3 AEDs at referral) • 3 were undergoing surgical evaluation.

37. ……Issues and concerns raised by teenagers: • Education and career choices/opportunities (60%) • s/e and possibilities of withdrawal of AEDs (33%) • Driving risks and regulations (30%) • Leisure activities and alcohol use (20%) • Contraception, pregnancy and inheritance (14%) • Epilepsy surgery (2%) Clinic team also offered teenagers opportunity to attend informal meetings outside hospital and at weekends. Forum for sharing ideas, concerns and socialising

38. 2007 survey of the transition clinics in epilepsy in GB and NI • 11 centres: 6 in Adult services and 5 in paediatric unit • 10 staffed with AN and PN. • 1 Nurses led • 6 : ANS + PNS • 2 have ANS only • 2 No nurse support Results presented at ILAE in Southhampton, 2007

39. Survey results… • Frequency of clinics: 3-12 (4-6 mostly) • 2: F/U patients only (6-12) • 2: Only new patients (5-6) • Rest: New + F/u patients (6+6) • New Patients: Min Age 11yrs (Av:15-16) Max Age 20yrs (most<19) • 8 will see all plus 2 have special clinics for severe LD • GP referral accepted in 4/11.

40. What about here?? • Aim for a co-ordinated transfer process • A policy on timing of transfer • A preparation programme and education programme • See teenagers on their own from 13years (with parents invited to join session later) • Uninterrupted and accessible care • Information on how to seek help and how to operate within the medical system. • Hope!… “Adolescent Unit” in “National Epilepsy Centre”…

41. Start at 11-13yrs.(depending on developmental readiness) Review young persons knowledge: know the diagnosis? Discuss AED therapy +side effects + Pubertal changes At 13-14 See them on their own for part of consultation. Discuss professional and recreational restrictions Encourage independence + all of above At 14-15 See them on their own. Discuss sexual health, Folicacid contraception in girls, educational and career options, educate on teratogenic effects of AEDs. Information on how to seek help & operate within the medical system + all of above By 16years: should be seen by the Adult neurologist. If growth not complete ideal to overlap between paediatric and adult services until development satisfactory. Reinforce all above issues.

42. Comments from clinic patients • “Transfer process was incredibly slow and dreadful waiting for 2 years….” • “….not knowing who to turn to when his seizures really were at its worst” • “I was dismissed from the children’s hospital as they felt I was too big for them” ………………………food for thought…..

43. Final Thought .. “Transition years” “These are the years that young people change into whoever they are going to be for the rest of their lives” ….lets get the change right for them..

44. Thank you