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

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The Rocky Road to Adult Health Care for Children with Lennox Gastaut and related disorders Peter Camfield MD Webinar November 2011 – PowerPoint PPT presentation

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Title: 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)
  • 14 Absence Epilepsies

74 Partial and Convulsive Epilepsies
Camfield CS, et al. Epilepsia. 19963719-23.
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.
2009882-93.
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.
2009882-93 Camfield P, et al. Epilepsia.
200243(suppl 3)27-32.
10
Please Dont 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
  • gt1 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
2005135 Camfield P, et al. Epilepsia.
2007481128-1132.
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 n45
  • Good 18 n 8 judged able to live
    independently
  • Fair 22 n10 cannot live independently but
    do not require total care
  • Poor 60 n27 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
  • gt60 Young Adulthood
  • Years Adulthood
  • 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 BC399 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
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-upno access to newer
    treatments I wouldnt 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
Problems That Inhibit Transition
  • 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

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 physicians competence. Few are
    experts at specific chronic diseases.
  • Adult rehabilitation program These 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 clinic attended 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 1997627
  • 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. 20029373

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
  • Involvement of 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
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