Understanding and treating FASD: When things are not as they seem…

1. Understanding and treating FASD: When things are not as they seem… Christopher Boys, PhD, LP Pediatric Neuropsychologist Co-Director, Fetal Alcohol Spectrum Disorders Program Department of Pediatrics

2. Oldest known cause of developmental disability • “Behold now, thou art barren, and barest not,; thou shalt conceive, and bear a son. Now therefore beware, I pay thee, and drink not wine nor strong drink” (Judges 13:3-4) • In the 4th Century, Aristotle associated alcohol with fetal abnormalities (Hett, 1936). • First epidemic of FAS occurred 18th century England • “Gin Epidemic” was reported to cause “weak, feebled and distempered children” (Warner & Rossett, 1975) • Then forgotten until Jones, Smith, & Ulleland (1973) defined the group of physical findings of FAS.

3. Teratogenec Effects • Papara-Nicholson & Telford (1957) • Observed low birth weight, poor locomotion, incoordination, and feeding/suckling difficulties in guinea pigs when exposed to alcohol weekly • Sandor (1968) – Romania • Injected chicken eggs with alcohol and observed malformations and growth deficiencies • Sulik, Johnson, & Webb (1981) • Just two heavy doses of alcohol at day 7 of gestation resulted in notable facial dysmorphology at birth • Clarren, Astley, Gunderson, & Spellman (1992) • “Saturday Night Binge” model of alcohol exposure

4. Physical Features of FASD • Central Nervous System • Microcephaly, or • Neurological Hard Signs (seizure disorder, hemiparesis, etc), or • Mental Retardation, or • Three functional domains affected in neurocognitive areas • Face • Flattened Philtrum and Thinned Vermillion (upper lip) • Palpebral Fissures (<10th%ile) • Growth • Height or Weight in <10th%ile

5. Prenatal alcohol exposure is associated with significant cognitive deficits and behavioral disturbances • Impaired intellectual functioning • Sensory Integration difficulties • Dysregulation of mood and behavior • Poor working memory • Impaired judgment • Impaired language reasoning/processing • Impaired executive functioning • Impaired social adaptive functioning

6. Heavy prenatal exposure is associated with deficits, even in absence of full FAS (Mattson et al., 1997; Schonfeld et al., 2001; Howell et al., 2005) • Overall, the effects of prenatal alcohol exposure are on a continuum – Fetal Alcohol Spectrum Disorders (FASD)

7. Center for Disease Control Criteria • Fetal Alcohol Spectrum Disorder: Fetal Alcohol Syndrome • Fetal Alcohol Spectrum Disorder: Partial Fetal Alcohol Syndrome • Fetal Alcohol Spectrum Disorder: Alcohol Related Neurodevelopmental Disorder • Fetal Alcohol Spectrum Disorder: Alcohol Related Birth Defects

8. DSM-V • Conditions for Further Study • Proposed criteria set are presented for conditions on which future research is encouraged. It is hoped that such research will allow the field to better understand these conditions and will inform decisions about possible placement in forthcoming editions of DSM

9. DSM-V • Neurobehavioral Disorder Associated with Prenatal Alcohol Exposure (ND-PAE) • Proposed Criteria • A) More than minimal exposure to alcohol during gestation, including prior to pregnancy recognition • B) Impaired neurocognitive functioning • IQ<70 • Impaired executive functions, learning, memory, visual spatial reasoning

10. ND-PAE (cont) • C) Impaired self-regulation as manifested by one of the following • Impairment in mood or behavioral regulation • Attention Deficit • Impairment in Impulse Control • D) Impairment in Adaptive Functioning as manifested by 2 or more of the following

11. ND-PAE (cont.) • E) Onset of the disorder occurs in childhood • F) Causes clinically significant distress or impairment in social, academic, or other important areas of functioning • G) Disorder not better explained by the direct physiological effects of substance use, general medical condition, another known teratogen, ort genetic syndrome

12. Domains of Neuropsychological Assessment • Cognitive/Intelligence • Academic Achievement • Attention • Memory • Language/Pragmatic Language • Executive Functions • Emotional/Behavioral • Adaptive Behavior

13. Gross structural abnormalities in FAS(12 year old male subjects) Normal Development Fetal Alcohol Syndrome

14. DTI tractography shows abnormal white matter tracts with partial agenesis of the corpus callosum

15. Functional MRI signal from contralateral ROIs corroborates callosal inefficiency Figure 1. fMRI time-series from one control subject illustrating high correlation between BOLD signal change in right and left medial orbital frontal cortex. Figure 2. fMRI time-series from one FASD subject illustratinglow correlation between BOLD signal change in right and left medial orbital frontal cortex.

16. Most common description in clinic… • The FASD TRIAD • The individual tends to be 1) impulsive, 2) misinterpret the intentions of others, 3) and fail to learn from feedback. • “Can talk to talk but not walk the walk” • Working memory difficulties result in poor planning and a poor ability to follow multiple step directions • Executive function difficulties result in difficulties developing step one to a task

17. Most Common Comorbid Diagnoses • Attention Deficit Hyperactivity Disorder • Conduct Disorder • Aspergers Syndrome • Oppositional Defiant Disorder • Autism Spectrum Disorder

18. Developmental Differences in Clinical Presentation • Newborn and Infancy- • Exhibit “regulatory problems” • Failure to thrive • Delays in development • Motor dysfunction • Otitis media • Cardiac problems

19. Developmental Differences in Clinical Presentation (cont) • Preschool (2 ½ to 6 years)- • Typically exhibiting • Hyperactivity • Language dysfunction • Perceptual problems • Behavioral Disturbances • Sensory Integration problems • If things are calm, can present more typically.

20. Developmental Differences in Clinical Presentation (cont) • School Aged Children (6-13 years) • Unable to sit still in class or pay attention • Difficult to deal with multiple sensory inputs (especially auditory) • Significant difficulties in peer relationships • Reported to have a “lack of remorse” • Fail to learn from mistakes • Lack judgment • Tend to be unusually aggressive

21. Goals of Neuropsychological Assessment • Conceptualization • Assessment should drive interventions • Serial assessment to insure interventions are effective

22. Intervention Focus per Diagnosis • Asperger Syndrome • Social Language • Oppositional Defiant Disorder • Consequence Based Behavior Interventions • Attentional Deficit Hyperactivity Disorder • Sustained attention • Stimulants

23. Interventions for Alcohol-Affected Children • Infants- • Early intervention should focus on language development • Preschool- • The emphasis should be on social skills and behavior training • School-Age • Address academic impairments while providing behavior supports • Direct Instruction for skill deficits in organization and planning • If no academic difficulties, often obtain poor grades due to homework completion • Improved self-monitoring • Young Adulthood • Interventions should focus on supportive employment placement and independent living opportunities.

24. Monitoring/Impulse Control Issues • When children have difficulty monitoring, they get blind-sided by consequences • Do not teach decision making, first must teach identifying the “point of decision making.” • In other words, being more aware that a decision has to be made. • Not ending up knee deep in the muck before you realize the muck is there.

25. Planning/Organization • Children with FASD/executive function difficulties have difficulty locating a starting point and developing an efficient strategy. • When combined with low frustration tolerance, behavior outbursts can result and mask the underlying problem.

26. Planning/Organization • Provide daily practice in use of such things as desk organizers, work folders, an assignment book and a planning calendar. Daily check-ins with teachers or a counselor often ensures that assignments have been recorded accurately and thoroughly. Provide study guides and opportunities for rehearsal for upcoming tests, or provide practice tests prior to unit tests. • Develop a system for keeping track of completed/uncompleted work. Provide detailed checklists to allow self-monitoring of satisfactory completion.

27. Working Memory • Working memory is essentially the ability to hold information in memory and perform a specific manipulation to the information. • Individuals with working memory problems often have difficulty carrying out multistep activities, losing track of what they are doing as they work, or forgetting what they are supposed to retrieve when sent on an errand. • Restaurant Servers

28. Working Memory(cont.) • Provide simple templates for routines that are repeated. • Each day must start fresh, regardless of how the previous day went. • A template lays out the standard steps to complete a repetitive task and can be useful for a variety of home and school tasks. The template can be faded out when the procedure or task becomes automatic. • However, this should be monitored closely so that the template can be brought back if it appears that it was faded too soon. The template can also be used to address problem areas such as homework completion, personal hygiene, time management (get a snack, math worksheet etc.). • IMPORTANT STEP: • May need a reminder/behavior change component to remember to check the list

29. Shifting • Children with FASD must be prepared to receive directions/instructions. • This allows for time to shift to new cognitive set or activity • Notable source of frustration for parents and teachers • This is where the child gets blindsided • Adults are on 3rd command, Child is on first • Deficits in cognitive flexibility also will warrant specific accommodations. Child will need to be reminded to ‘stop and think’ before responding to task demands, and he will probably need cueing to keep him from continuing to respond in ways that are ineffective. Parents/teachers also should monitor child closely to insure that he understands directions for assignments.