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FASD Facial Features and Neurocognitive Outcomes in the Post-Institutionalized Child

FASD Facial Features and Neurocognitive Outcomes in the Post-Institutionalized Child. Judith Eckerle Kang 2/12/2010. “I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this activity.”

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FASD Facial Features and Neurocognitive Outcomes in the Post-Institutionalized Child

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  1. FASD Facial Features and Neurocognitive Outcomes in the Post-Institutionalized Child Judith Eckerle Kang 2/12/2010

  2. “I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this activity.” • I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation.

  3. Goals • FASD- is this a problem for international adoptees? • FASD- brief review of diagnosis • FASD facial features and outcomes in the Growth Endocrine Project • FASD facial features and CANTAB testing

  4. “Of all the substances of abuse including cocaine, heroin, and marijuana, alcohol produces by far the most serious neurobehavioral effects in the fetus resulting in life-long permanent disorders of memory function, impulse control and judgment” —IOM Report to Congress, 1996

  5. FASD in International Adoption • Each year more than 23,000 children are internationally adopted (IA) into the U.S.A. There has also been a shift in demographics such that 80% of IA’s now come from institutional care and from countries with low per capita income and poor nutrition with Eastern Europe as a major participating country in international adoption. • U.S. Department of State. Immigrant Visas Issued to Orphans Coming to the U.S.: Top Countries of Origin: FY2005, 2006. Yearbook of Immigration Statistics.

  6. FASD in International Adoption • Additionally, there was a 48.1% increase in alcoholism among women in the Former Soviet Union (FSU) countries in the 10 years post dissolution of the USSR. Estimates report that eighty to ninety-four percent of girls in FSU ages 15-17 years reportedly drink “sometimes” and 17 percent drank “often” with weekly use by teens up 54%. • Davis, R B. "Drug and alcohol use in the former Soviet Union: selected factors and future considerations." Substance use & misuse 29.3 (1994):303. • United Nations Office for Drug Control and Crime Prevention Statistics. Vienna, Austria: United Nations Publication; 2002

  7. Facts about Fetal Alcohol Spectrum Disorders (FASD) • Most people with FASD have average IQs • Rate of FAS (Syndrome) is 1:500 births • Rate of FASD (spectrum) is 1:100 births

  8. FASD: An Underdiagnosed Spectrum • Data from more than 400 FASD patients in the University of Minnesota’s Fetal Alcohol Clinic database show that the majority of children (68%) with documented prenatal exposure to alcohol do not meet full diagnostic criteria for FAS (unpublished clinical data). • Jeffrey R. Wozniak, Ph.D., Assistant Professor of Psychiatry, University of Minnesota

  9. How is FASD Diagnosed? 4-Digit Diagnostic Code • growth deficiency (height or weight < 10th percentile). (institutional care) • a unique cluster of minor facial anomalies (small eyes, smooth philtrum, thin upper lip). • central nervous system damage (structural, neurological, and/or functional impairment). • prenatal alcohol exposure.

  10. Lip/Philtrum Guides • (displayed with permission, Dr. Susan Astley)

  11. Face of FAS forms during the 3rd week of gestation, most likely days 19-21

  12. Rating of Lip Philtrum • http://depts.washington.edu/fasdpn/htmls/lip-philtrum-guides.htm

  13. FASD Imitators • Cornelia de Lange syndrome (CdLS) • distinctive facial features • growth retardation (<5th % throughout life) • Long smooth philtrum, thin vermillion border of upper lip • Low-set posteriorly rotated and/or hirsute ears with thickened helices

  14. Velocardiofacial Syndrome (VCF or DiGeorge Syndrome) • Cleft lip and/or palate • distinctive facial features • cardiac septal defects • hypernasal speech • Hypotonia • defective thymic development.

  15. Valproate Syndrome • Prenatal exposure to valproic acid (depakene, depakote) during the first trimester • distinctive facial features • Neural tube defects • Congenital heart disease • Cleft lip and/or palate • Genitourinary malformations • Tracheomalacia • Arm/hand defects • Arachnodactyly/overlapping digits • Abdominal wall defects • Intellectual impairment

  16. Attention deficits Memory deficits Hyperactivity Difficulty with abstract concepts Inability to manage money Poor problem solving skills Difficulty learning from consequences Immature social behavior Inappropriately friendly to strangers Lack of control over emotions Poor impulse control Poor judgment FASD is the Invisible Disability

  17. Protective Factors • A diagnosis before 6 years of age • Living in a stable, nurturing home • Not being a victim of violence • Having received developmental disabilities services • Having a diagnosis of FAS rather than FAE • Lower than 70 IQ

  18. Growth Endocrine Project: FASD Facial Features and Cognitive Outcomes • What is the point of the study? • Who was potentially exposed to alcohol? (Face) • Are there other factors that can tell us about who was potentially exposed or that link the children with high risk facial features? (Growth? Cognitive testing?) • Are there times/ages which can best predict those that have been exposed to alcohol and may need extra services?

  19. Methods: • 122 Eastern European children, adopted between 7-59 mo of age on arrival. • Mean age: 20mo • All from institutional care (85% entire life spent in institutional care- rest 0.87)

  20. Measures • Growth (Ht, wt, OFC), Neurocognitive and facial feature information was collected at all 3 time points • Time 1 (arrival): 122 Children • Time 2 (6 mo post): 93 children • Time 3 (30 mo post): 58 children: 8 children in the High Risk Face group (HRF) and 50 in the Low Risk Face group (LRF)

  21. Measures: Cognitive • Cognitive: Time 1 and 2 • The Mullen Scales of Early Learning is a comprehensive measure of cognitive functioning used to assess visual linguistic and motor domains while also distinguishing between receptive and expressive processing. • There are 5 Mullen Scales: Gross Motor, Visual Reception, Fine Motor, Receptive Language, and Expressive Language.

  22. Measures: Cognitive time 3 • The Stanford Binet intelligence scales are used for assessment of individual cognitive abilities. • The entire scale, called the Full Scale IQ, consists of 5 verbal and 5 nonverbal subscales which are compiled for assessment of general intelligence.

  23. Facial Analysis for FASD • Facial features were analyzed on arrival, 6 mo and 30 mo post adoption in person and by photographic software. • 1.0-2.0 was considered low risk facial features (LRF) • 2.5-4.0 were considered high risk facial features (HRF)

  24. Growth Time 1 and 2 were done in collaboration with Dr. Patrick Mason and Christine Narad from Inova Fairfax Hospital for Children, Fairfax Virginia. For the outcomes here, we used all three times points so children are all from U of Minnesota. Time 1 and 2 outcomes are being published Dr. Brad Miller et al; “Auxological evaluation and determinants of growth failure at the time of adoption in Eastern European adoptees” Journal of Pediatric Endocrinology and Metabolism. • We have data on growth but will be focusing on the neurocognitive testing for this presentation.

  25. Time of Arrival: Cognitive • LRF and HRF groups did not differ on the Mullen Scales of Early Learning. -------------------------------

  26. Time of Arrival- Head/OFC • Head circumference on arrival was significantly different but means for both groups were WNL (p<0.004) • LRF= -0.6 SD • HRF= -1.6 SD *

  27. 6 mo Post-Adoption: Cognitive • Trend toward significance in visual reception deficits seen in the children who had evolving high risk facial features. (p<0.072) ------------------------------ *

  28. Visual Reception Scale • This involves intrasensory tasks that focus on visual perceptual ability. • Visual information is presented in a variety of ways so that visual processing skills can be assessed. Visual discrimination is measured in all tasks, while visual memory is assessed in only certain tasks. • Responses are given by manipulations of objects or by pointing to objects and pictures. No vocalization is involved.

  29. 6 mo post adoption: OFC/Head • At 6 mo post adoption there remained significant differences between the 2 groups. • Means were still in the normal range • OFC HRF<LRF (P<0.005)

  30. * *

  31. 30 mo post adoption • Of these children that we saw at 30 mo post adoption, new facial features had evolved in 4 adoptees (50% of the HRF group), that were not evident at earlier evaluations

  32. 30 mo post adoption: Cognitive, Verbal • The HRF group had lower scores for verbal working memory (p<0.05) *

  33. Verbal Working Memory • The verbal working memory subscale measures a child’s ability to transform, store, and retrieve verbal information in short-term memory. The subscale measures the cognitive ability to hold and sort through verbal information. In children, the ability to repeat a sentence measures short-term memory.

  34. 30 mo post adoption: Cognitive, Nonverbal • The HRF group had lower scores for nonverbal fluid reasoning (p<0.02) *

  35. Nonverbal Fluid Reasoning: • This measures a child’s ability to solve novel problems without dependence on academic or cultural information. • Uses visual sequences and analogical patterns to test inductive and deductive reasoning skills. • In children, it measures the ability to identify shapes, colors and sizes in order to identify sequences and patterns.

  36. 30 mo post adoption: IQ • No significant difference was seen in IQ and both groups scored within the normal range. -----------------------------------

  37. Take home points: #1 For kids >1SD below mean for ht/wt/head at 6 mo, alcohol exposure should be considered as part of the differential and assessment of facial features and history should be done.

  38. #2: Facial features evolve over time • IA children should have repeated assessments over time for facial features. Facial features that are borderline at earlier ages should be followed especially closely and consideration should be given for screening of FASD at multiple time points.

  39. Facial features evolve over time • Animal studies- facial features were barely prominent at 1 mo age, most detectable at 6 mo and diminished progressively at 12 and 24 months of age. • Fetal alcohol syndrome: Changes in craniofacial form with age, cognition, and timing of ethanol exposure in the macaque. Susan J. Astley, Shannon I. Magnuson , Lena M. Omnell , Sterling K. Clarren. Teratololgy. Volume 59 Issue 3, Pages 163 - 172. Published Online: 18 Mar 1999.

  40. #3: Consider neuropsych testing • Fluid reasoning and Working Memory • Shown to be deficit in kids with diagnosed FASD • Even children whose IQ’s are in the normal range (and thus do not qualify for school services) may have deficits that prevent them from effective learning.

  41. FASD- Cognitive deficits • This has also been shown in diagnosed children with FASD- attentional and memory tasks, visual-spatial, short-term auditory attention and verbal memory, declarative learning, and cognitive flexibility and planning. Olson HC, Feldman JJ, Streissguth AP, Sampson PD, Bookstein FL. Neuropsychological deficits in adolescents with fetal alcohol syndrome: clinical findings. Alcoholism: Clinical and Experimental Research. 1998;22(9):1998-2012. Verbal Learning and Memory in Children with Fetal Alcohol SyndromeSarah N. Mattson, Edward P. Riley, Dean C. Delis, Catherine Stern, Kenneth Lyons JonesAlcoholism: Clinical and Experimental ResearchVolume 20, Issue 5 , Pages810 - 8161996 The Research Society on Alcoholism

  42. Verbal Learning and Memory in Children with Fetal Alcohol SyndromeSarah N. Mattson, Edward P. Riley, Dean C. Delis, Catherine Stern, Kenneth Lyons JonesAlcoholism: Clinical and Experimental ResearchVolume 20, Issue 5 , Pages810 - 8161996 The Research Society on Alcoholism • Specific Impairments in Self-Regulation in Children Exposed to Alcohol PrenatallyP. W. Kodituwakku, N. S. Handmaker, S. K. Cutler, E. K. Weathersby, S. D. HandmakerAlcoholism: Clinical and Experimental ResearchVolume 19, Issue 6 , Pages1558 - 15641995 The Research Society on Alcoholism • Effects of Prenatal Alcohol Exposure on Attention and Working Memory at 7.5 Years of Age.Alcohol Effects on the Fetus, Brain, Liver, and Other Organ Systems • Alcoholism: Clinical & Experimental Research. 29(3):443-452, March 2005.Burden, Matthew J.; Jacobson, Sandra W.; Sokol, Robert J.; Jacobson, Joseph L.

  43. #4: Gross motor skills are not correlated with other cognitive findings or facial features. • Gross motor skills on this testing were not significantly different between the 2 groups.

  44. #5: Facial features are key • This study supports previous work that suggests facial features are enough to strongly consider or diagnose FASD and can lead to discovery of specific cognitive issues. • Application of the fetal alcohol syndrome facial photographic screening tool in a foster care population.Clinical and Laboratory Observations. Journal of Pediatrics. 141(5):712-717, November 2002.Astley, Susan J. PhD; Stachowiak, Julie RN, MN; Clarren, Sterling K. MD; Clausen, Cherie RN, MN.

  45. What’s Next?

  46. Neurocognitive Functioning in the Internationally Adopted Child with High Risk FASD Facial Features.

  47. CANTAB testing in IA children • In this proposal, we are taking a novel, collaborative approach with Psychology, using the CANTAB (computer game testing) to investigate subtle brain deficits in IA’s who are high risk for FASD.

  48. Specific Aim • Assess the brain functioning of IA children with high risk FASD facial features using computerized testing compared to matched IA children with low risk FASD facial features, half from Eastern Europe and half from Chinese institutional care. • Compare the growth between groups.

  49. Hypothesis • Our overall hypothesis is that children with high risk facial features are at risk for specific deficits in prefrontal lobe brain functioning, similar to kids with diagnosed FAS, that are above and distinct from the baseline risk of transitions and institutional care that is present in international adoption as a whole. Children with high risk facial features will also have decreased growth parameters.

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