Assessment & Management of FASD

1. Assessment & Management of FASD Speakers: Susan Adubato, Ph.D. Denise Aloisio, MD, FAAP MD Champions: Alla Gordina, MD, FAAP Uday Mehta, MD, MPH, FAAP American Academy of Pediatrics, New Jersey Chapter (http://www.aapnj.org/showcontent.aspx?MenuID=999)

2. Disclosure Information: This activity has been jointly sponsored/ co-provided by Health and Research and Education Trust and AAP/NJ & PCORE. Disclosure Information: Neither Denise Aloisio, MD, FAAP, Susan Adubato, PhD nor HRET, AAP/NJ or PCORE has any significant financial interest or relationship with any manufacture(s) of any commercial products(s) discussed in this educational presentation. HRET-NJHA is an approved provider of continuing education by the New Jersey State Nurses Association, an accredited approver by the American Nurses Credentialing Center’s COA. P#131-5/11-14. This activity is approved for 1.25 contact hours. There is no commercial support for this activity. Accredited status does not imply endorsement by the provider or American Nurses Credentialing Center’s COA of any commercial products displayed in conjunction with an activity. Accreditation Statement: This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Medical Society of New Jersey (MSNJ) through the joint sponsorship of Health Research and Educational Trust (HRET) and AAP/NJ & NJ Pediatric Council on Research and Education.  HRET is accredited by MSNJ to provide continuing medical education for physicians. AMA Credit Designation Statement: HRET designates this live activity for a maximum of 1.25 AMA PRA Category 1 CreditsTM. Physicians should only claim credit commensurate with the extent of their participation in this activity.

3. Of all the substances of abuse (including cocaine, heroin and marijuana), alcohol produces, by far, the most serious neurobehavioral effects in the fetus” IOM Report to Congress, 1996

4. Case 1: Bob • Bob presented at the age of 10 years. • He was adopted from a Russian orphanage at the age of 7 months • He likes to play with his trucks and cars. He is social and interactive and is described as having a great personality • He has sleep difficulties, sensory issues and eats small amounts of a limited range of foods.

5. Case 1: continued • He has features of ADHD, a lot of worries and fears, low frustration tolerance, a high degree of reactivity • He has difficulty with problem solving and abstract concepts. • Prenatal is unknown. He was born at 33 weeks gestation with a birth wt. of 4lbs 6oz

6. Case 1: continued • Medical history is unremarkable except for recurrent otitis media requiring tube placement at 18 months. • On physical exam: ht and wt both less than 5th %tile. • Microcephaly with head circumference less than 3rd %tile. • Face- flattened philtrum, thinned upper lip and small eyes.

8. Case 1: continued • IQ testing at 7 yrs with WISC-III Verbal 74 Performance 60 Full Scale IQ 65 • Updated IQ at 10 years with WISC-IV: verbal comprehension 73, perceptual reasoning index 51, working memory 54, processing speed 56, and full scale IQ 50 • Diagnosis: FAS: alcohol exposure unknown • Intellectual Disability • Attention Deficit Hyperactivity Disorder

9. Case 1: continued • Management has included collaboration with school personnel to address difficulties in the classroom and appropriate placement • Medications for ADHD and Anxiety; he has had side effects to many of the stimulants and anti-anxiety medications.

10. Brain Regions Affected by Alcohol

11. Fetal Alcohol Spectrum Disorders is an umbrella term describing the range of effects that can occur in an individual whose mother drank during pregnancy. These effects may include physical, mental, behavioral, and /or learning disabilities with possible lifelong implications. The term FASD is not intended for use as a clinical diagnosis. CDC July 2004 FASD

12. Presentation at different ages-

13. Infants Poor habituation/sleep-wake cycles Irritability/exaggerated startle Failure to thrive (poor weight gain) Chronic ear infections Difficulty nursing/poor sucking response Poor/superficial bonding with caregivers Developmental delays Speech delays; low muscle tone

14. Toddlers Continued developmental delays; potty training Distracted easily Colds, infections, other illness Eating (small appetites or sensitivity to food texture) Fidgeting (meal time or other structured event) Often exhausted/irritable due to poor sleep Danger to self-not grasping cause and effect Usually high maintenance-24/7

15. Pre-Schoolers Delayed speech development Altered motor skills Difficulty following directions Attention deficits/Learning deficits Exaggerated response to sensations (bump into child- she feels she was hit or shoved) Difficulty adapting to changes in environment Caregiver concerns: manipulative, does not understand cause and effect, problems with judgment and memory, disobedience

16. School Age Bedtime Making and keeping friends Difficulties determining body language and expressions Difficulties separating fact from fantasy Boundary issues Attention problems/impulsive Easily frustrated/tantrums Difficulty understanding cause and effect Caregiver concerns: emotionally volatile, manipulative, unpredictable, increased need for stimulation and excitement, disconnected to feelings/limited empathy

17. Adolescents Still need limits and protection due to deficits in reasoning, judgment and memory High risk of being drawn into anti social behavior e.g. stealing, lying, drugs-”thrill seekers” Unable to distinguish between friends/enemies; impaired judgment for decisions; faulty logic Struggle to accept their own disability while trying to prove ability to be independent Often obsessed by primal impulses-sex, fire setting Lacks remorse Negligent of normal hygiene Extremely vulnerable to suggestions in movies, TV High risk for school dropout; academic ceiling reached: usually 4th grade for reading and 3rd grade for math Unable/unwilling to take responsibility for actions; egocentric

18. Adults* Moral chameleons Often exhausted and irritable – poor sleep Vulnerable to anti-social behavior – find structure and supervision in criminal justice system Unlikely to follow safety rules – fire hazards, vehicles, basic life needs Social/sexual/financial exploitation; social isolation Lacks ability to manage money Incapable of taking daily meds Vulnerable to panic, depression, suicide (Huggins, et.al-2008:23%), psychosis Need sheltered environment Think younger- 2/3 chronological age *Chudley, et al(2007): Adults with FASD have higher rates of social problems, executive functioning and psychopathology when compared to general population.

19. Case 1: Ted • Presented for developmental evaluation at the age of 8 years • History of behavioral difficulties • Was irritable as a baby, had sleep problems, didn’t grow well and as a toddler he was very active • He was friendly and social but often impulsive • He was asked to leave three different preschool programs because of difficulties following rules and being disruptive • He was also aggressive at times

20. Case 1: continued • In Kindergarten, he had difficulty learning his letters, he could not sit in group for story time and was disruptive • He threw things when upset and had injured another student on the playground • His pediatrician recommended further assessment

21. More difficulties for Ted • Ted didn’t seem to learn from common discipline techniques, and would repeat the same wrong behaviors over and over • He had no friends and was not allowed to go on the class trip • First grade was even worse and three months into the year he was evaluated by the school team and placed in a smaller class

22. Ted’s Assessment • Ted presented to the Developmental Pediatrician when previous history was obtained • Birth history was obtained and Ted’s mother admitted to drinking some beer regularly during pregnancy, she also smoked cigarettes and was on medication for a respiratory infection • Physical exam revealed some facial features including: small eyes, flat philtrum and thin upper lip. Head circumference was less than the 5%

23. Problem Domains of Individuals with Prenatal Alcohol Exposure Cognition/Intellectual Functioning Activity and Attention (ADHD) Hyperactivity Focusing, encoding, shifting Learning and Memory Auditory, spatial, design, and narrative memory Working memory Intrusion, perseveration, false-positive errors Comprehension, math reasoning

24. Language Social communication Word comprehension, naming ability, articulation Expressive and receptive language skills Motor Abilities Fine and gross motor dysfunction Delayed motor development Speed/precision, grip strength Processing Abilities Spatial memory, processing of visual and auditory information Difficulties in motor control and functioning Problem Domains of Individuals with Prenatal Alcohol Exposure

25. Other Neuropsychological Abilities/Executive Functioning Behavioral and emotional regulation-impulsivity, lability Planning/organization Abstract thinking/judgment Sensorimotor Integration Social Skills and Adaptive Behavior Mental Health Issues Problem Domains of Individuals with Prenatal Alcohol Exposure

27. Case 2: Debbie • Debbie presented at 12 years with a diagnosis of FAS, ADHD and Intellectual Disability • She is rough with the family pets and even killed two of them • She steals items from other children in the family and school • The family has to lock all the doors to rooms in the house

28. Case 2: continued • Medical history significant for being born extremely prematurely at 24 weeks gestation • There was known exposure to alcohol prenatally • She had an Intraventricular hemorrhage and congenital cardiac defect ASD repaired at 4 years. • She has asthma treated with medications • There was a question of seizures but EEG was normal

29. Case 2: continued • On physical exam, height and weight have been consistently below the 3rd %tile. • Head circumference less than 3rd %tile • Facial features consistent with FAS

31. IQ IQ was done at 12 years old with the WISC-IV: verbal comprehension index 59, Perceptual reasoning index 49, working memory index 65, processing speed index 70, Full Scale IQ is 51

32. Case 2: continued Management involves: • Behavioral family services in home • Medications: Strattera, risperdone recently added, Buspar • Family is involved with services through their church.

33. Clinical Implications of Impairments for Individuals with FAS/FASD

34. Clinical Implications of Impairments for Individuals with FAS/FASD • Poor judgment and decision making, which increases susceptibility to being victimized • Attention deficits, which increase distractibility and lack of focus • Arithmetic disability, which leads to difficulty in handling money • Memory impairment, which makes learning from experience difficult • Difficulty abstracting, which makes it difficult to understand the consequences of one’s behavior

35. Clinical Implications of Impairments for Individuals with FAS/FASD Disorientations of time and space, which complicate accurately perceiving social cues, missing appointments Impulsivity and poor self-regulation, which decreases tolerance for frustration, and makes them quick to anger Poor habituation which results in drowning in stimulation, emotional overload, shutting down and behaving irrationally Perseveration which leads to doing the same thing over and over again Difficulty with self reflection which leads to not being able to express ones’ needs and not getting help

36. Secondary Disabilities Resulting from the Primary Disabilities of Individuals with FAS/FASD 60% have trouble with the law 50% will be confined in prison ,mental institutions, and treatment centers 35% have alcohol and/or drug problems -Streissguth 2004

37. Secondary Disabilities Resulting from the Primary Disabilities of Individuals with FAS/FASD • 61% have disrupted school experience • 49% exhibit inappropriate sexual behavior • Other:joblessness, homelessness, inability to demonstrate effective caretaking and parenting, and increase potential for victimization, need for lifelong supervision • Streissguth 2004

38. Universal Protective Factors Early diagnosis Stable, nurturing home environment No violence/victimization Early intervention services DDD services Streissguth, 2004

39. Differential Diagnosis of CNS and Behavioral Feature Found in Fetal Alcohol Syndrome Dan Dubovsky-FASD Center of Excellence, 2011

40. Common Disorders Identified with FASD Anxiety Asperger’sDisorder Attention Deficit Hyperactivity Disorder (ADHD) Autism Borderline Personality Disorder Conduct Disorder Depression Eating Disorders Learning Disability Oppositional-Defiant Disorder Post Traumatic Stress Disorder (PTSD) Reactive Attachment Disorders Receptive-Expressive Language Disorder

41. Similarities Between FASD and Autism • Developmental disabilities that affect normal brain function, development, and social interaction • Difficulty developing peer relationships • Difficulty with the give and take of social interactions • Impairments in the use and understanding of body language to regulate social interaction • Abnormal sensitivity to sensory stimuli, including an over- or under-sensitivity to pain Dan Dubovsky-FASD Center of Excellence, 2011

42. Major Differences Between FASD and Autism FASD Autism Restricted in emotional expression Macrocephaly more common Difficult or impossible to relate to others in a meaningful way • Can express a range of emotion • Microcephaly more common • Superficially social Dan Dubovsky-FASD Center of Excellence, 2011

43. Major Differences Between FASD and Autism FASD Autism Difficulty in both expressive and receptive language Repetitive body movements e.g., hand flapping, and/or abnormal posture e.g., toe walking • Difficulty in verbal receptive language; expressive language is more intact as the person ages • Repetitive body movements not seen; may have fine and gross motor coordination and/or balance problems Dan Dubovsky-FASD Center of Excellence, 2011

44. Possible Misdiagnoses and/or Co-occurring Disorders for Individuals with FASD ADHD Oppositional Defiant Disorder Depression Bipolar Dan Dubovsky-FASD Center of Excellence, 2011

45. Comparing FASD, ADHD, & ODD Dan Dubovsky-FASD Center of Excellence, 2011

46. Comparing FASD, Adolescent Depression and Adolescent Bipolar Disorder Dan Dubovsky-FASD Center of Excellence, 2011

47. Managing Co-existing Disorders • ADHD • Mood Disorders • Oppositional Defiant Disorder • The role of medications • Start low, go slow • Monitor closely • May have opposite effect

48. Reconceptualizing the Behavior of the Individual with FAS Professionals, family members, and caretakers need to reconceptualize how we view the behavior of an individual with FAS/FASD From seeing  To understanding Won’t  Can’t Lazy  Tries hard Lies  Fills in Doesn’t try  Exhausted or can’t start Doesn’t care  Can’t show feelings Refuses to sit still  Over stimulated Fussy, demanding  Oversensitive Resisting  Doesn’t “get it”

49. You Can Make A Difference ! Think: “Stretched Toddler”. Remember: “Individuals with FASD will always need an external brain.” Acknowledge: Interventions must be useful to, and usable by the individual in order to be an intervention. Foster: Inter-dependence. Reflect: Respect. Promote: Self-worth.

50. Support: Self-esteem. Understand: That FASD is not “Chicken Pox.” You can’t catch it and it never goes away. Shift: From a “non-compliance” model to a “non- competence” model. Accept: Individuals with FASD do the best they can with what they’ve got at that time. Believe: You can make a difference. You Can Make A Difference !