Multidisciplinary assessment at National FASD Clinic Dr Alex

Multi-disciplinary assessment at National FASD Clinic Dr Alex Carlisle (Chartered Clinical Psychologist and Neurodevelopmental Specialist Clinician) alexandra. carlisle@sabp. nhs. uk

FASD Clinic

Note of caution…. Due to differences in dose / timing of exposure, genetics & environmental influences: “There is no single neuropsychological measure, nor pattern of neurodevelopmental profiles, that are specific to all individuals with FASD” (Cook et al 2016 Appendix, Canadian Guidelines)

National Clinic – assessment pathway Day 1 FASD overview and photography Wider neurodevelopmental assessment Home with measures Psychology Initial findings Total face to face 12 hrs SALT Indirect evaluation 9 hrs Wider evaluation all measures and full report Day 2

Wider assessment beyond simply confirming FASD: Neurodevelopmental conditions (ASD & ADHD) Sensory profile Behaviour (risk & triggers for dysregulation) Communication profile Neuropsychological Profile (IQ & executive function) Strengths (potential for function and for learning)

Benefits of carrying out wider assessment • Improves ecological validity as direct assessment in 1 -1 structured setting does not always reflect ability in unstructured setting – e. g. EF • There are many secondary conditions and features associated with FASD that are not currently diagnostic e. g. sensory difficulties, sleep • Identification of wider profile of strengths and difficulties guides recommendations and support from services

Hub and Spoke model of service delivery Regional clinic National Clinic Regional Clinic Local Clinic Local Clinic Regional Clinic Local clinic

Local Consultation Model Ø Local CAMHS Team Consultation – supervision of cases within CAMHS to facilitate diagnosis with only complex cases seen at Regional/National Clinic Ø Training of CAMHS FASD CHAMPIONS Ø Invite colleagues to observe clinic to observe qualitative aspects of assessment

Direct and indirect measures used: Facial Dysmorphology: • 4 Digit FASD analysis • 3 -D Facial recognition • Semi structured FASD history Questionnaire Background History: ASD: ADHD: Adaptive Behaviour: Executive Functioning: • SCQ • DISCO • ADHD Screening Questionnaire • Conners III Other: • Developmental Behaviour Checklist • Parent • Teacher • Vineland II • Delis Kaplan Executive Function System Tests (D-KEFS) or Behavioural Assessment of Dysexecutive Syndrome. Child (BADS-C) • Behaviour Rating Index of Executive Functioning (BRIEF) • Short Sensory Profile • Sensory Processing Measure • WAIS IV / WISC V • CELF 4 • Pragmatic Language • Parental Stress Index • Children’s Communication Checklist

Impairment on 3+ Domains/Brain areas Motor Skills Neuroanatomy / neurophysiology Cognition Language Academic Skills Memory Executive functioning Adaptive behaviour, social skills / social communication Focus and Attention Affect Regulation

‘Impairment’ defined as: Direct measure (standardised tool): Indirect assessment • 2 standard deviations (SD) below mean on global score or major subdomain • Discrepancy between major subdomains below 3% of base rate & lower score 1 SD+ below mean • Meet clinical threshold / above clinical cut-off • E. g. DSM V criteria

Where results are limited / inconsistent: Use clinical judgement Gather evidence from multiple convergent sources Observation e. g. in structured vs unstructured settings

Asses as though independent entities Even if theoretically related, do not use a single test score as evidence of deficits in two domains • e. g. impairment on functional communication score on Vineland – adaptive/social communication or language? • e. g. WISC-V Verbal Comprehension Index impairment - language or cognition?

Domains are practical (not theoretical) Not necessary for domains to be theoretically independent; the intent of 3 separate domains is to ensure those receiving diagnosis have pervasive deficits • e. g. microcephaly, low IQ, and low adaptive behaviour may be different indicators of same underlying problem

Benefits and Limitations of Direct and Indirect Assessment of CNS deficits

Direct measures - benefits Standardised and potentially more objective Opportunity to observe qualitative aspects – e. g. more effort required

Direct measures - limitations Experienced / trained clinicians to administer and evaluate scores Test data can be inconsistent - e. g. frontal lobe paradox May lack ecological validity – e. g. hot vs cool EF

Frontal Lobe Paradox George M, Gilbert S. ‘Mental Capacity Act (2005) assessments: Why everyone needs to know about the frontal lobe paradox’ The Neuropsychologist 5; 2018 (May): 59 -66 Patients with prefrontal cortex damage MAY appear proficient within clinical interview/ direct assessments but exhibit marked limitations within adaptive functioning i. e. individuals perform better on externally prompted tasks but have difficulties in non-routine situations, long term rule maintenance, multi-step tasks or tasks, and social cognition

Hot vs Cool Executive Functioning Cool Hot • • • Inhibition/Impulse control Planning and Problem Solving Hyperactivity Working Memory Shifting and Cognitive flexibility + Behaviour EMOTIONAL AROUSAL/ DYSREGULATION Behaviour

Indirect measures - benefits Clinical observation informative especially non-routine / unstructured situations Information from caregivers and teachers can be more ecologically valid than direct assessment

Indirect measures - limitations Need to gather information (with examples) from multiple sources to avoid bias Individuals with FASD may lack self awareness & may confabulate

Measures used to assess CNS / neurodevelopmental areas

Appendix Canadian Guidelines 2016 (App. F) www. cmaj. ca/lookup/suppl/doi: 10. 1503/cmaj. 1 41593/-/DC 1 SIGN – Neurodevelopmental areas of assessment https: //www. sign. ac. uk/assets/neurodevelopm ental_areas_of_assessment_criteria. pdf

Motor Skills Definition Fine motor (manual dexterity, precision), gross motor, grapho-motor, and visualmotor skills Direct Indirect Bruininks Oseretsky Test of Motor Proficiency (gross and fine motor) Tone, reflexes, strength and other abnormal findings on neurological exam or speech articulation / oromotor (in combination with direct assessment) Beery Buktenica Developmental Test of Visual Motor Integration Rey Complex Figure test and Recognition Trial Clinical assessment of balance & coordination as supporting evidence of severe impairment

Neuroanatomy/neurophysiology Definition Orbitofrontal head circumference below the clinical cut-off Seizure disorder not due to postnatal influences Brain imaging of structural brain abnormalities (other aetiologies having been excluded) Direct/Indirect Head Measurement 2 SD below mean (corrected for prematurity) Medical history of seizure MRI

Cognition Definition Full Scale IQ or major subdomain (Verbal Comprehension, Visual Spatial, Fluid Reasoning, and Processing Speed) NB working memory subdomain evidence of EF Direct Wechsler Intelligence Scale for Children(WISC-V) Wechsler Adult Intelligence Scale (WAIS)

Language Definition Receptive and Expressive Language Direct Indirect Clinical Evaluation of Language Fundamentals 4/5 (Core language, receptive language, expressive language) 2 or more higher level language subtests (narrative or complex comprehension) Discrepancy between receptive and expressive Non standardised clinical assessment

Academic Skills Definition Tests of reading, maths & literacy Team must determine if there has been consistent exposure to academic instruction before deficit recorded Direct Wechsler Individual Achievement test, Third Edition (WIAT-III) Composite score below cut-off or discrepancy between cognition and reading, maths & writing Indirect School reports with literacy and numeracy achievement levels can be used as supporting evidence for severe impairment

Memory Definition Direct Global memory and/or verbal memory and/or visual memory Children’s Memory Scale Discrepancy between verbal and nonverbal memory NB not working memory subdomain NEPSY-II Rey Complex Figure test and Recognition Trial Rivermead Behavioural Memory Scale

Focus and Attention Definition Selective attention (focus), Divided attention (2+ stimuli), Alternating attention (switching), Sustained attention (resistance from distractibility) Direct 2+ subtests of Everyday Attention for Children (TEA-Ch) 2+ subtests of Delis-Kaplan Trail Making or Color. Word Interference Indirect Clinical interview, observation, questionnaires e. g. Conners DSM V

Executive functioning Definition Direct Indirect Skills and behaviours involved in meeting goals: – working memory; inhibition/impulse control; hyperactivity; planning and problem solving; or shifting and cognitive flexibility Behavioural Assessment of Dysexecutive Syndrome (BADS) Behaviour Rating Index of Executive Functioning (BRIEF) Delis Kaplan Executive Function System Tests (D-KEFS) Conners – hyperactivity and impulsivity scale

Affect Regulation Definition Indirect Includes mood anxiety disorders evidence of longstanding problem of dysregulation rather than short term response to unfavourable life events or conditions DSM V or ICD 10 criteria e. g. Diagnosis of depression or anxiety Self-report questionnaires such as Child Depression Inventory

Adaptive & social communication Definition Life skills which enable individual to live in safe and socially responsible manner - adaptive behaviour (conceptual, social and practical skills) and/or social communication Direct Indirect Adaptive Behaviour Assessment System-II The Social Language Development Test Vineland Adaptive Behaviour Scales-II Formal assessment of ASD (observation and interview)

In Summary

FASD can be a ‘hidden’ disorder as there a wide variety of profiles and presenting difficulties. Superficial presentation in structured settings / self report / clinic based assessment may not reflect the extent of the level of underlying functional difficulty

Useful to gather information from caregiver and teacher questionnaires / interviews, and observations of unstructured time as well as direct measures to inform diagnosis Direct and indirect assessment can create a unique profile indicating support needs (e. g. scaffolding for deficits as well as reduction of excess/arousal)