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FACE September 9,2003

FACE September 9,2003. Toward defining the behavioural phenotype in children with FASD Presented by Dr. Gail Andrew, medical director, Glenrose FASD Project. Glenrose FASD Project Clinic. Team: Pediatrician - Dr. Gail Andrew Social Worker - Gail Schuller Psychologist - Dr. Kathy Horne

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FACE September 9,2003

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  1. FACE September 9,2003 • Toward defining the behavioural phenotype in children with FASD • Presented by Dr. Gail Andrew, medical director, Glenrose FASD Project

  2. Glenrose FASD Project Clinic Team: Pediatrician - Dr. Gail Andrew Social Worker - Gail Schuller Psychologist - Dr. Kathy Horne Speech-Language Pathologist - Mary Reynolds Occupational Therapist - Lynne Abele-Webster

  3. Glenrose Project Background • Fall 2000 Consultation teams at Glenrose Rehabilitation Hospital, Edmonton and Alberta Children’s Hospital, Calgary • DPN model, Clarren and Astley, Seattle • Funded by Alberta Health and Wellness • Partnerships : Ministries of Children’s Services, Learning and Justice; Community service providers; Caregivers

  4. Glenrose Project Mandate • Clinical: DPN tool for diagnosis Intervention planning • Database: Prevalence Lifespan monitoring Outcomes of intervention • Training: Increase capacity Multidisciplinary teams Mentoring and consultation

  5. FACE September 9, 2003 • Questions: • 1. Is there a neurobehavioral pattern specific to prenatal exposure to alcohol? • 2. How does this pattern change across the lifespan of the individual with FASD? • 3.What measures can be used to assess the disability in FASD? • 4. What are the best interventions/supports?

  6. FACE September 9, 2003 • Concepts: • Behaviour or learning difficulties are presenting symptoms in FASD • Need to determine deficit in brain function leading to that symptom • Need to connect brain dysfunction to etiology of organic brain damage from prenatal exposure to alcohol (maternal hx)

  7. Terminology FASD • F = Fetal: changes in normal development in utero • A = Alcohol: Teratogen causes cell/process changes and damage • S = Spectrum: damage/difficulties present from mild to severe • D = Disorder: difficulty/inability to function/adapt as expected across lifespan

  8. FASDSpectrum of Disability/Dysfunction

  9. Defining FASD : Variables • Organic brain damage in utero: • maternal: drugs, nutrition, metabolism • fetal: susceptibility, protective • alcohol: amount, pattern, timing • genetic factors (maternal, paternal, fetal) • Postnatal: • supportive or increased risk rearing • other brain damage

  10. Defining FASD: Hallmarks • Disordered pattern in development and acquiring expected skills • Out of keeping with measured IQ • Increasing difficulty in functioning with age • May not be evident at early age ( skill not expected) • May be masked by supportive environment

  11. Defining FASD: Hallmarks • Pass tests • Flunk life

  12. Assessment of Brain Dysfunction • No single test - need battery by multidisciplinary team • Test from simple to complex in all domains • Need to sample real life functions (executive, adaptive, socialization) by standardized tests and caregiver reports • Consider secondary disabilities, comorbidities, physical health issues

  13. Assessment Domains • Resources: • DPN model of Clarren and Astley • Glenrose FASD Project • Health Canada National Guidelines • Need for continued research on specificity to FASD phenotype • Need to assess that individual’s pattern to plan interventions

  14. Assessment Domains • Intellectual • Academic achievement • Language and social communication • Attention • Visuospatial • Motor and visuomotor • Sensory processing • Memory • Adaptive and executive functioning

  15. Clinical Reports of FASD • Inattentive, short attention span, not able to regulate responses to environment stimuli, impulsive, act first without thinking • Not learning from experiences or connecting cause and effect • Not able to organize, plan or sequence but live in the now • Slow to learn, need repeated learning, seem to forget especially if overstimulated

  16. Clinical Reports of FASD • Poor and variable memory, not able to generalize to different situation or use stored information • Impaired executive functions such as judgement, reasoning, mental flexibility,adapting, planning • Difficulty with abstract concepts such as math, money, time

  17. Clinical Reports of FASD • Overly talkative, often off topic, interuptive, confabulation, not aware listener is not following, not connecting points or making sense, not communicating • Comprehension of language and nonverbal aspects of communication even more impaired than use of words • Poor social skills, easily victimized, immature

  18. Current Research Data • Memory: short term, encoding, long term, retrieval, working memory, verbal, auditory, nonverbal, visuospatial • Executive function • Attention • Social communication • Relationship to IQ • Correlation to animal research and neuroimaging studies

  19. Memory in FASD • Mattson, Reilly, Roebuck: n = 16, controls • Verbal: slower rate of encoding into short term memory • Continued to learn with repeated trials • Delayed recall accounted for by deficits in initial learning and not in long term memory function or retrieval • Nonverbal: recalled less after delay even when initial learning considered

  20. Memory in FASD • Mattson et al: • Variable rate of learning across trials possibly related to inconsistent attention, not utilizing strategies, more intrusions and preseverations • Carmichael Olson: • Deficits in auditory memory impacted by inattention and language comprehension

  21. Memory in FASD • Uecker and Nadel: (nonverbal memory) • Deficit in immediate but not delayed object recall (implies encoding deficit) • Spatial memory deficit, distorted spatial array • No deficit in facial recognition • Mattson, Reilly etc: n = 22, controls • Subtle differences in interhemispheric transfer in somatosensory domain (corpus callosum) • Distorted temporal processing

  22. Executive Function • Jacobson, Mattson, Coles, Kodituwaku etc: • Deficits in all 4 areas of Delis- Kaplan EF scale not explained by IQ (planning ability, cognitive flexibility, selective inhibition, concept formation and reasoning) • Difficulties in planning, more preservative on incorrect strategies • May be linked to problems using information in working memory

  23. Adaptive Function • Whaley (2001) n = 33, controls • Impaired adaptive function in all areas of Vineland in FASD and children with psychiatric disorders matched for IQ but no significant differences to be a hallmark of FASD • With increasing age FASD showed decline in socialization component

  24. Attention • Review by O’Malley and Nanson (2002) • ADHD pattern common in FASD • Earlier onset, more resistant to stimulant medications, may respond better to Dexedrine (D1 receptor of dopamine in animal model of alcohol damage) compared to ADHD not alcohol exposed • Regulatory difficulties in infancy, hyperactive toddler, later inattentive type

  25. Attention • Kodituawakku et al: • Used Wisconsin Card Sorting Test • More preservative errors suggesting inattention in FASD related to difficulty in shifting responses or attention • More problems in tasks requiring planning and manipulation of information in working memory • No difference on delayed response tasks that required sustained attention

  26. Social Communication • Disordered language pattern in FASD well recognized but communicative social use of language further impaired than even these abilities • Reflected in social failure and victimization across the lifespan

  27. Social Communication • Coggins (Seattle): • Used narrative test that requires ability to make sense of a picture story through inference and perspective taking • FASD failed cohesion (linking logically) and coherence (information) • Theory: failed to encode necessary inferences

  28. Social Communication • Monnet (2002) : n = 43, controls, adults • Impaired affective prosody ( not getting the emotional or attitudinal inferences of what is said) worse in prenatal alcohol exposed • Pattern in FASD different from that in alcoholics and acquired focal right or left brain injury and more like combination • Right parietal cortex dysfunction, also corpus callosum implicated in FASD pattern

  29. Clinical Research in FASD • Koren et al (2002) n = 52, age 4-18 yr. clinic • Profile of neuropsychological characteristics of 21 deficits and 6 assets done by 2 independent raters with threshold for ARND (FASD) Dx • Identified more problems in academic ability, intelligence, language, memory in ARND • Both groups in referred clinic setting had equal ratings on behaviour and social problems

  30. Clinical Research in FASD • Steinhausen (2003) n = 38, controls • Compared IQ, age matched controls with other diagnoses to FAS/FAE Developmental Behavioural Checklist (Einfield, Tonge) • FAS similar to FAE and both more severe than controls in all areas : disruptive, self absorbed, communication disturbance, anxiety • Persistence over time in psychopathology and cognitive function

  31. Glenrose Clinic Experience • Rationale of DPN model - 4 digit code • Characterizes Fetal Alcohol Spectrum Disorder objectively • Documents alcohol exposure without judging causal role • Considers other pre and post natal factors (multifactorial)

  32. Assessment of Brain Function • History and neurological exam • Psychometric testing • Adaptive functioning / daily life • Caregiver interview • Scales • Direct observation

  33. Assessment of Brain Function • Specific tests of executive function • higher order language • social communication • memory • attention • planning, organizing • sensory issues

  34. Glenrose Experience Question 1 • What patterns emerged among the rankings of Growth, Face and Brain Function in children with confirmed prenatal alcohol exposure?

  35. Glenrose Experience:Participants • Question 1 • N = 75 children • 41 male, 34 female • age range 1 year 1 month to 15 years 2 months • All were exposed to alcohol at levels 3 and 4

  36. Glenrose Experience:Participants

  37. Glenrose Experience:Participants

  38. FASD Pilot Project January 2001 to March 2002

  39. Glenrose Experience:Results

  40. Glenrose Experience:Implications • Brain dysfunction can be present without growth deficiency or typical facial features • If only look at face or growth many children with brain dysfunction would be missed

  41. Glenrose Experience Question 2 • What assessed functions most clearly differentiated children ranked as Brain 3 (Static Encephalopathy) from those ranked as Brain 2 (Neurobehavioral Disorder)?

  42. Glenrose Experience:Participants • Question 2 • subgroup of N being n = 34 • 17 Brain 2, 17 Brain 3 • age range 6 years to 15 years 2 months

  43. FASD Pilot Project January 2001 to March 2002 Common Findings in Brain 2 & 3 : • IQ scores variable but considerable scatter • Half of scattered profiles showed statistically significant split but in both directions • Motor skills variable • Discrete measures of basic language usually within average range • < 6 years - 75% variable language delays, expressive better than receptive (early sign)

  44. Glenrose Experience:Results Comparison between Neurobehavioral Disorder (brain 2) and Static Encephalopathy (brain 3) on 8 measures:

  45. Glenrose Experience:Results • Sequencing, social communication, and working memory differentiated severity of brain dysfunction • Attention, behavior and adaptive functioning did not differentiate and present in most • Higher order language, verbosity, and receptive language difficulties indicate some differentiation

  46. Implications of Glenrose Findings • Discrete measures of intelligence and basic language skills alone do not predict or indicate degree of dysfunction / disability • Deficits in executive function characterizes FASD and impacts daily living • Not always evident at young age • May be supported by structured environment • Need to reassess over time

  47. FACE September 9, 2003 • Questions: • 1. Is there a neurobehavioral pattern specific to prenatal exposure to alcohol? • 2. How does this pattern change across the lifespan of the individual with FASD? • 3.What measures can be used to assess the disability in FASD? • 4. What are the best interventions/supports?

  48. Answer to Questions • 1. Clinical reports and emerging research data support a neurobehavioral phenotype in FASD that is complex (small sample nos.) • 2. Increasing difficulty with age and increase in societal expectations • 3. Assessment tools in each domain are identifying specific impairments • 4. Need to systematically apply to interventions and measure outcomes

  49. Future Research • Need larger sample sizes with control groups from normal population, other brain dysfunction , learning disabilities and behaviour disorders; longitudinal follow up • Testing in clinical situation does not necessarily represent how individual functions in day to day life; need to develop tools to assess this • Apply information to intervention strategies

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