Neuropsych Service

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    Neuropsychology Service in

    Children with SCD

    Dr Kofi A AnieBrent Sickle Cell & Thalassaemia Centre

    Imperial College School of Medicine

    Central Middlesex HospitalLondon

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    Children with SCD

    High Risk of:

    Neurological complicationsCVAs related to severity

    Strokesmost disabling SCD complications

    Silent strokes infarcts common in younger children

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    Neuropsychological Function

    Neuropsychological problems considerable evidence

    Neurological Complications generally lead to neurocognitive decline(Craft et al, 1993; 1994; Cohen et al, 1994; Schatz et al, 1999;

    Boni et al, 2001)

    Educational Risk possible cognitive & intellectual impairment academic underachievement(Brown et al, 1993; Schatz et al, 2001; Schatz, 2004)

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    Overt Strokes

    Neuropsychological Complications associated with brain lesion location & size

    Language & Verbal left hemisphere (Cohen et al, 1994)

    Visual & Motor right hemisphere (Cohen et al, 1994)

    Attention & Executive Function frontal lobe (Craft et al 1994, Schatz et al, 1999)

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    Silent Strokes

    Attention & Executive Function frontal lobe

    more subtle

    (Debaun et al,1998; Brown et al, 2000; Wang et al, 2001)

    Intellectual Functioning lesion size(Schatz et al, 2002)

    Learning eg. reading & maths(Armstrong et al, 1996)

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    Mind the Gap!!

    TCD Screening

    Neurological Examinations

    MRIs

    Neuropsychological Service??

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    Considerations

    Age-appropriateEvidence-based

    ongoing researchSetting

    clinics, referrals?

    Universal routine?

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    Rationale

    Attention & Executive Function fundamentally important in neurocognitive &

    educational performance

    Comprehensive neuropsychologicalassessments in outpatients clinics? time consuming impractical

    Two-tier Approach

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    Screening Assessments

    Paediatric SCD Clinic routine

    All children aged 5 yrs +

    school-going age younger if indicated

    Attention & Executive FunctionEducation

    loss of schooling achievement

    Pain & Quality of Life

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    Full Neuropsych Assessments

    By Appointment

    Attention

    Memory

    Learning

    Executive Function

    Psychomotor

    Intellectual Functioning

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    Educational Liaison

    Neuropsychological Reports

    Schools

    Education Depts

    Educational Psychologists

    Extra Tuition

    Statements

    Special Needs

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    Child AW Demograph & Medical

    Female, HbSSAge = 10yrs, School Yr = 6No History of Stroke, Neuro Complications

    Pain (preceding 12 mons) 10+ episodes 24 hrs 7days

    HSU (preceding 12 mons) 5 hospital admissions 3-7 days

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    Child AW - Psychosocial

    Mood sometimes low did not want to talk about SCD

    Quality of Life physical function moderate social function impeded

    Schooling 30% loss (Sept-Dec) learning concerns (Mum)

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    Child AW Neuropsych Screen

    Attention inattention

    impulsive

    Executive Function some problems

    TCD normal

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    Child AW Full Neuropsych

    Wechsler Norms: Mean = 100, SD = 15Achievement Tests (WIAT)

    Reading Composite = 70

    Maths Composite = 78 Language Composite = 96Writing Composite = 68

    Intelligence Scales (WISC)Performance IQ = 96Verbal IQ = 92Full Scale IQ = 94

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    Child AW Educational

    School Performance not optimal

    School Support

    literacy reading (group 1x weekly)

    writing (group 3x weekly)

    Home Tuition inc. vocabulary, spelling, maths

    Sickle Cell Society Project

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    Child AW Neurology

    MRI silent stroke

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    Conclusion

    Neuropsychological services for children

    with SCD invaluable:

    To complement Neurological Exams, TCDs,MRIs

    To determine Educational Support

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    LETS CLOSE THE GAP!!