The Rocky Road to Adult Health Care for Children with Lennox Gastaut and related disorders

1. The Rocky Road to Adult Health Care for Children with Lennox Gastaut and related disorders Peter Camfield MD Webinar November 2011

2. Who am I? • Professor Emeritus, Department of Pediatrics (Neurology) Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia • My main research interest is the natural history of childhood epilepsy – what happens to children with epilepsy as they grow up. • Main clinical interest – treatment of epilepsy • Main collaborator – Dr. Carol Camfield

3. Disclosures • Honorarium from Biocodex

4. What is Lennox Gastaut Syndrome (lgs)?

5. Nova Scotia Population-Based Childhood Epilepsy Cohort (N = 692): All New Cases (1977-1985) 12% Symptomatic Generalized Epilepsies (SGEs) 74% Partial and Convulsive Epilepsies Camfield CS, et al. Epilepsia. 1996;37:19-23. 14%Absence Epilepsies

6. Lennox-Gastaut Syndrome: A Typical Symptomatic Generalized Syndrome • USA-French connection • Described by Dravet and Gastaut in Marseilles and Lennox in Baltimore • Gastaut – call it Lennox syndrome; • Others suggested Lennox-Gastaut syndrome • Classical triad • Akinetic seizures (drop) (+ many other seizure types; myoclonus, tonic, atypical absence, GTC) • Slow spike-wave on EEG • Many causes, Mental handicap • Refined criterion - Tonic seizures during sleep Arzimanoglou A, et al. Lancet Neurol. 2009;8:82-93.

7. Slow spike and wave Blume WT, et al. Atlas of Pediatric Electroencephalography. 2nd ed. London, Ontario: Lippincott Williams & Wilkins; 1999.

8. Tonic Seizure Blume WT, et al. Atlas of Pediatric Electroencephalography. 2nd ed. London, Ontario: Lippincott Williams & Wilkins; 1999.

9. Lennox-Gastaut Syndrome:A Catastrophic Epilepsy or Epileptic Encephalopathy • Drops cause facial, tooth injury; a helmet with a face mask is the only effective protective gear. • Complete seizure control is very rare. • Development stagnates? Regresses? • Behavior is often difficult. • Family disruption from complex child care needs: difficulty in finding/keeping baby sitter, loss of sleep. • Seizures tend to get “better” with age but persist, social independence unusual Arzimanoglou A, et al. Lancet Neurol. 2009;8:82-93; Camfield P, et al. Epilepsia. 2002;43(suppl 3):27-32.

10. Please Don’t Confuse Me!

11. Related Symptomatic Generalized Epilepsy Syndromes 85% of SGEs • West syndrome – 35% (may evolve to LGS) • Myoclonic astatic epilepsy (MAE) – 10% • Unspecified – 35% • LGS – 5%

12. What Is SGE Unspecified? • Onset : any age • Does not meet the criteria for another syndrome • >1 generalized seizure type; must include 1 of : Myoclonus, Akinetic/atonic, Drop attacks, Tonic, Atypical absence • EEG: generalized spike-wave (irregular or slow) and/or multifocal spikes • Outcome: difficult epilepsy and mental handicap Beaumanoir A, et al. In Roger J, et al, eds. Epileptic Syndromes in Infancy, Childhood and Adolescence. Montrouge, France: John Libbey; 2005:135; Camfield P, et al. Epilepsia. 2007;48:1128-1132.

13. Lets not be too restrictive in this presentation – Transition of LGS and related disorders

14. Symptomatic Generalized Epilepsy (SGE)

15. SGE social outcome if ≥18 yrs at end of follow-up: n=45 Good 18% n= 8: judged able to live independently Fair 22% n=10: cannot live independently but do not require total care Poor 60% n=27: require assistance with all activities of daily living • Only 3 young adult survivors with SGE were intellectually and neurologically normal, seizure-free/off AEDs, financially independent and living on their own with a partner.

16. Erik Erikson – The Life Cycle Infancy 18 Early Childhood Years Play Age School Age Adolescence >60 Young Adulthood YearsAdulthood Old Age

17. SGE in childhood affects Adolescence – identity vs identity confusion FIDELITY

18. SGE in childhood continues to affect Young Adulthood – intimacy vs isolation LOVE

19. SGE in childhood continues to affect Adulthood– generativity vs stagnation CARE

20. SGE in childhood continues to affect Old Age – integrity vs despair WISDOM

21. LGS and Symptomatic Generalized Epilepsy • Many have mental handicap • What is a good social outcome for someone with mental handicap?

22. John Stuart Mill 1806-1878 Hedonistic Philosopher Socrates 469 BC–399 BC Greek Athenian Philosopher

23. What constitutes a good social outcome for someone with mental handicap ? (Miller and Chan 2008) • Social support – large network consisting of socially supportive staff and family and friends • Self-determination/ perceived control • Work/productivity (competitive and supported employment is better than sheltered workshop or home). • Adaptive behaviour – independent living skills, social skills, communication + academic skills

24. How can children with epilepsy find their way to a more successful adulthood?

25. DRUGS SEX ROCK & ROLL Transition to Adult Care

26. What Happens if Transition is not successful? No adult speciality care; problems are complex for the family physician The “please take me back” syndrome Lack of follow-up—no access to newer treatments—“I wouldn’t go back there if you paid me” Lack of management for related conditions (eg, supplemental vitamin D for enzyme inducers)

27. Transition vs Transfer Important Distinction • Transition – a process beginning in childhood to prepare youth with chronic illness and families for “adult’ health care. • Transfer – the formal handing over of care from a pediatric to adult health care system. • A detailed transfer note is an important part of transfer but does not constitute transition

28. Cultural issues: • Pediatric care is family centered • When you go to the physician your mother drives you and accompanies you into the office • The visit is as much about her as it is about you • Adult care is individually centered • When you go to the physician your mother simply does not come • The physician is not interested in your mother • This is all about you

29. Family Comfortable with status quo; attached to pediatric service (we are delightful and attentive) Personal experiences with adult health care With intellectual handicap or intractable epilepsy normal independence is precluded (he or she remains a “child”) Pediatric Service Long-term attachment (we really enjoy working with these families) View adult service, as possibly not competent particularly for psychosocial care Institutional support, time, $ Problems facing our own mortality Problems That Inhibit Transition

30. Young adults with mental handicap present a special problem for the adult health care system • Who (parents or patient) decides what is best? • Difficult behaviour in the office is not tolerated. • The space is inadequate. • There is nothing developmentally appropriate in the office. • Adult physicians are often very uncomfortable with the mentally handicapped. (Gillam, Epi Behav 2009)

31. 2002: Semi-structured interviews with 50 pediatric and adult neurology specialists in 11 major pediatric centers in 7 countries. • There is almost no published peer-reviewed research (lots of editorials, no data). • No centre was satisfied with their transition/transfer process for children with neurologic handicap. • Adult services were perceived to have little interest in the many “non-seizure” problems that accompany LGS – it is not enough just to treat the seizures! • No centre had any data on the success of their program. • 10/11 centers had a transfer process with no formal transition program.

32. Models for transition/transfer No transfer Continued follow-up in a pediatric setting. • The pediatric specialist ages with the patient – puts off transfer until the specialist retires unless the institution is committed to a “life to death” program. • Fosters dependency - everyone deserves to grow up • Logistical problems for medical and social services – what happens if you have a complex epilepsy disorder and need you need your gall bladder out at age 30 in an adult hospital and have seizures post op? No one there knows about your epilepsy problem. • Lack of knowledge about adult diseases – hypertension, type 2 diabetes, COPD

33. Models for transition/transfer • Abandonment(fend for yourself). The family physician takes full responsibility – a physician who has had little/no role during the pediatric years. The youth and family are not attached and doubtful of this physician’s competence. Few are experts at specific chronic diseases. • Adult rehabilitation programThese are oriented towards consultation and short-term intervention. Care is multidisciplinary but long-term follow up is unusual.

34. Models for transfer • Adult neurologist or adult neurology clinic. (may be a consultation model with follow up visits only if re-referred from family physician). Few adult neurologists show interest in long term care for developmental brain disorders. • Transition clinicattended jointly by adult and child neurologists in the adult setting. Families like this idea. Without any objective data, we think this is the best model. It allows family comfort and dialogue between pediatric and adult neurology over several visits.

35. It is not all bleak

36. Managing the teenager with epilepsy: paediatric to adult care.Appleton Seizure 1997;6:27 • Description of 120 “transitioned” patients • Clinic staff – pediatric neurologist, adult neurologist, nurse practitioner • 37% had mental handicap • Most seen for 2 visits • 10% did not have epilepsy, 22% had a change in medication • See also Smith Eur J Neurol. 2002;9:373

37. Solutions: • Transition: Build skills that last for children and for parents. They should know enough about their disease to know when to seek medical care and if the medical care is adequate.

38. Solutions for consideration by physicians • Start a transition program • Learn the realities/practice style of adult neurology and pediatric neurology – spend a few afternoons in each others’ office/clinic. (It takes 2 to tango) • Talk with patients and families often about long-term health issues. Emphasize education, sexuality. • Foster research to know what happens to pediatric neurological disorders in adulthood. • Support, support groups – after your patients leave you, support groups will inform them about new developments – will anyone else?

39. Special Transition issues for parents of a child with LGS • Guardianship – a formal legal process to establish who makes decisions for a mentally handicapped adult • Will – establishes guardians and finances • Trust fund – funds opportunities • Registered Disability Savings Plan • Involvementof younger family members and friends: avoid “well known by no one”, insist on comprehensive care, prevent abuse by demanding accountability

40. A good outcome • Frank is from a tiny town in rural Nova Scotia. • Leukemia age 2, treatment included brain radiation but he was cured. • Development normal until age 3: drop seizures, long absence and rare GTCs. Development stagnated. • Age 3 -12 seizure control was poor (hockey helmet). School: integrated with a TA

41. A good outcome • Socially always very popular. Overall abilities in the mild mentally handicapped range • Age 12-18, fewer seizures, no more drops, no more helmet (hurray) • School – adapted program with community supervised placements in a retail store • Age 19- graduates from high school, living at home, “work” each day (a sheltered workshop). • Epilepsy transition clinic to adult epilepsy service

42. A good outcome • Age 20 – he announces that he wants to live with his friends in a supervised apartment. • Social worker informs family that our Province has a program for supervised apartments. • Family initially very resistant but he is very insistent. They all move to the city. • Apartment and 2 compatible room mates located. Worker comes 1-2/day (7days/week), assists with cooking, cleaning, groceries, checks on medication. • Workshop provides daily transport.

43. A good outcome • Through sheltered work shop he attends Special Olympics with activities 3 times/week (bowling and baseball his favourites) • Visits home for Sunday dinner each week – his sister and family come too. He is very attached to his niece. • Mother attends doctor appointments with him. • Very happy for past 10 years. • Seizures continue but no accidents and no status

44. What constitutes a good social outcome for someone with mental handicap ? (Miller and Chan 2008) • Social support – large network consisting of socially supportive staff and family and friends • Self-determination/ perceived control • Work/productivity (competitive and supported employment is better than sheltered workshop or home). • Adaptive behaviour – independent living skills, social skills, communication + academic skills

45. Resources John Riess MD, University of Florida http. hctransitions.ichp.ufl.edu/resources/html

46. Think a lot about Young Adulthood Adulthood Old Age

47. Thank You