Assessment & Management of FASD

Assessment & Management of FASD

Speakers: Susan Adubato, Ph.D.Denise Aloisio, MD, FAAPMD 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)

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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

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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.

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

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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.

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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 unknownIntellectual DisabilityAttention Deficit Hyperactivity Disorder*

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. *

Case 1: continued

Brain Regions Affected by Alcohol

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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

Presentation at different ages-*

Infants

Poor habituation/sleep-wake cyclesIrritability/exaggerated startleFailure to thrive (poor weight gain)Chronic ear infectionsDifficulty nursing/poor sucking responsePoor/superficial bonding with caregiversDevelopmental delaysSpeech delays; low muscle tone

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Toddlers

Continued developmental delays; potty trainingDistracted easilyColds, infections, other illnessEating (small appetites or sensitivity to food texture)Fidgeting (meal time or other structured event)Often exhausted/irritable due to poor sleepDanger to self-not grasping cause and effectUsually high maintenance-24/7

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Pre-SchoolersDelayed speech developmentAltered motor skillsDifficulty following directionsAttention deficits/Learning deficitsExaggerated response to sensations (bump into child- she feels she was hit or shoved)Difficulty adapting to changes in environmentCaregiver concerns: manipulative, does not understand cause and effect, problems with judgment and memory, disobedience

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School Age

BedtimeMaking and keeping friendsDifficulties determining body language and expressionsDifficulties separating fact from fantasyBoundary issuesAttention problems/impulsiveEasily frustrated/tantrumsDifficulty understanding cause and effectCaregiver concerns: emotionally volatile, manipulative, unpredictable, increased need for stimulation and excitement, disconnected to feelings/limited empathy

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AdolescentsStill need limits and protection due to deficits in reasoning, judgment and memoryHigh risk of being drawn into anti social behavior e.g. stealing, lying, drugs-thrill seekersUnable to distinguish between friends/enemies; impaired judgment for decisions; faulty logicStruggle to accept their own disability while trying to prove ability to be independentOften obsessed by primal impulses-sex, fire settingLacks remorseNegligent of normal hygieneExtremely vulnerable to suggestions in movies, TVHigh risk for school dropout; academic ceiling reached: usually 4th grade for reading and 3rd grade for mathUnable/unwilling to take responsibility for actions; egocentric*

Adults*Moral chameleonsOften exhausted and irritable poor sleepVulnerable to anti-social behavior find structure and supervision in criminal justice systemUnlikely to follow safety rules fire hazards, vehicles, basic life needsSocial/sexual/financial exploitation; social isolationLacks ability to manage moneyIncapable of taking daily medsVulnerable to panic, depression, suicide (Huggins, et.al-2008:23%), psychosisNeed sheltered environmentThink 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. *

Case 1: TedPresented for developmental evaluation at the age of 8 years

History of behavioral difficulties

Was irritable as a baby, had sleep problems, didnt 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*

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

Case 1: continued*

More difficulties for TedTed didnt 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*

Teds AssessmentTed presented to the Developmental Pediatrician when previous history was obtained

Birth history was obtained and Teds 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%*

Problem Domains of Individuals with Prenatal Alcohol ExposureCognition/Intellectual Functioning

Activity and Attention (ADHD)HyperactivityFocusing, encoding, shifting

Learning and MemoryAuditory, spatial, design, and narrative memoryWorking memoryIntrusion, perseveration, false-positive errorsComprehension, math reasoning

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LanguageSocial communicationWord comprehension, naming ability, articulationExpressive and receptive language skills Motor Abilities Fine and gross motor dysfunctionDelayed motor developmentSpeed/precision, grip strengthProcessing AbilitiesSpatial memory, processing of visual and auditory informationDifficulties in motor control and functioning

Problem Domains of Individuals with Prenatal Alcohol Exposure*

Other Neuropsychological Abilities/Executive FunctioningBehavioral and emotional regulation-impulsivity, labilityPlanning/organizationAbstract thinking/judgment

Sensorimotor Integration

Social Skills and Adaptive Behavior

Mental Health IssuesProblem Domains of Individuals with Prenatal Alcohol Exposure*

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 *

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 normalCase 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 Case 2: continued *

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

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Management involves:Behavioral family services in home

Medications: Strattera, risperdone recently added, Buspar

Family is involved with services through their church.Case 2: continued *

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 ones behaviorClinical Implications of Impairments for Individuals with FAS/FASD*

Clinical Implications of Impairments for Individuals with FAS/FASDDisorientations 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

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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*

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

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Universal Protective FactorsEarly diagnosis

Stable, nurturing home environment

No violence/victimization

Early intervention services

DDD services Streissguth, 2004*

Differential Diagnosis of CNS and Behavioral Feature Found in Fetal Alcohol Syndrome

Dan Dubovsky-FASD Center of Excellence, 2011

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SyndromeSimilarities to FASDifferences from FASFragile X syndromeAttention problems, hyperactivity, speech deficitsHand flapping, poor eye contact, more severe intellectual disability, autismWilliams syndromeMild prenatal growth deficiency, microcephaly, mild intellectual disability, short palpebral fissures, upturned nose, long philtrumAortic or pulmonary stenosis, hoarse voice, high relative language abilityNoonan syndromeShort stature, mild intellectual disability, ptosis, upturned noseWebbed neck, low posterior hairline, shield chest, pulmonic stenosis, cryptorchidism22q11 deletion syndromeLearning disabilities, IQ range from low normal to mild intellectual disability, speech deficits10% with psychiatric disorders, strong social skills

Common Disorders Identified with FASDAnxietyAspergers DisorderAttention Deficit Hyperactivity Disorder (ADHD)Autism Borderline Personality DisorderConduct DisorderDepression

Eating DisordersLearning DisabilityOppositional-Defiant DisorderPost Traumatic Stress Disorder (PTSD)Reactive Attachment DisordersReceptive-Expressive Language Disorder

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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

FASDCan express a range of emotion

Microcephaly more common

Superficially social

Autism Restricted in emotional expression

Macrocephaly more common

Difficult or impossible to relate to others in a meaningful way

Major Differences Between FASD and Autism *Dan Dubovsky-FASD Center of Excellence, 2011

Major Differences Between FASD and Autism FASDDifficulty 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 Autism Difficulty in both expressive and receptive language

Repetitive body movements e.g., hand flapping, and/or abnormal posture e.g., toe walking

*Dan Dubovsky-FASD Center of Excellence, 2011

Possible Misdiagnoses and/or Co-occurring Disorders for Individuals with FASD ADHD Oppositional Defiant Disorder Depression Bipolar


Comparing FASD, ADHD, & ODD


FASDADHDODD Behavior Underlying cause for the behavior May or may not take in the information

Cannot recall the information when needed

Cannot remember what to do Takes in the information

Can recall the information when needed

Gets distracted Takes in the information

Can recall the information when needed

Chooses not to do what they are told Intervention for the behavior Provide one direction at a timeLimit stimuli and provide cues Provide positive sense of control, limits, and consequences

Comparing FASD, Adolescent Depression and Adolescent Bipolar Disorder


FASD Adolescent Depression Adolescent Bipolar Disorder Acting out, antisocial behavior Acting out, antisocial behavior Acting out, antisocial behavior Misreading social cues; difficulty communicating thoughts and feelings Depression Mania or hypomania

Provide a mentor to model positive behaviors; utilize a lot of role playing Psychotherapy to address issues; protect from harm; medication (antidepressants) with careful monitoring Psychotherapy to address issues; protect from harm; medication (mood stabilizer)

Managing Co-existing Disorders ADHDMood DisordersOppositional Defiant DisorderThe role of medicationsStart low, go slowMonitor closelyMay have opposite effect*

Reconceptualizing the Behavior of the Individual with FASProfessionals, family members, and caretakers need to reconceptualize how we view the behavior of an individual with FAS/FASD From seeing To understanding

Wont Cant Lazy Tries hard Lies Fills in Doesnt try Exhausted or cant start Doesnt care Cant show feelings Refuses to sit still Over stimulated Fussy, demanding Oversensitive Resisting Doesnt get it*

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.*

Support:Self-esteem.

Understand:That FASD is not Chicken Pox. You cant 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 theyve got at that time.

Believe:You can make a difference.You Can Make A Difference !*

Best PracticeOne prevention model contains seven basic components, form the acronym SCREAMS:

Structure: a regular routine with simple rules and concrete, one step instruction, paired with examples

Cues: verbal, visual, or symbolic reminders can counter the memory deficits

Role models: family, friends, TV shows, movies that show healthy behavior and life styles

Environment: minimized chaos, low sensory stimulation, modified to meet individual needs.

Attitude: understanding that behavior problems are primarily due to brain dysfunction

Medications: most often the right combination of meds can increase control over behavior

Supervision: 24/7 monitoring may be needed for life due to poor judgment, impulse control.

Teresa Kellerman, Director of the FAS Community Resource Center, Tucson Arizona*

New Jersey Regional Diagnostic Centers

Six Regional Diagnostic treatment and educational centers were established in New Jersey in 2002. IdentifyScreenDiagnoseCase Management ReferralEducation OutreachBeintheknownj.org

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Comprehensive Assessment and Management of Individuals with FAS/FASDTeam approach:

Multi-disciplinary assessmentPsychosocial historyPhysicianDisciplines (Mental health, speech, OT/PT, LD)Parents/caregiversSocial service agencies (DDD, SS, Child protective, drug treatment centers)

Case managementDiagnosisEarly intervention and trackingStable home environmentMedicationCase manager/mentor in school/home/communitiesSupport services-family community, educational, vocationalSupervised housing/residential facilitySpecial education and vocational rehabilitation *

POLICY STATEMENTS

Since 1966, AMA and APA have recognized alcoholism as disease

AMA, AAP, ACOG, CDC, NIAAA, March of Dimes, and NOFAS all have policies regarding drinking during pregnancy

AMA urges physicians to be alert to possible alcohol related problems in women and to screen all patients for possible alcohol abuse and dependence.

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Be good to me... Stay alcohol free! A few drinks canLast foreverNo safe time. No safe amount. No safe alcohol. Period.NIAAA/NOFAS *

Astley, S., Aylward, E., Carmichael-Olson, H., et. al. (2009). Magnetic resonance imaging outcomes from a comprehensive magnetic resonance study of children with Fetal Alcohol Spectrum Disorders. Alcoholism: Clinical and Experimental research, 33 (10): 1671-1689.

Hellemans, KS, Silwowska, JH, Verma, P., and Weinburg, J. (2010). Prenatal alcohol exposure : fetal programming and later life vulnerability to stress, depression, and anxiety disorders. Neuroscience Biobehavior Review, 34, (6),791-807

Larkby, CA, Goldschmidt, L, Hanusa, BH and Day, N. (2011). Prenatal alcohol exposure is associated with conduct disorder in adolescence: Findings from a birth cohort. Journal of the Academy of Child & Adolescent Psychiatry, 50(3),March: 262-271.

Li, L Coles, CD., Lynch, ME, et al.,(2009). Voxelwise and skeleton-based region of interest analysis of fetal alcohol syndrome and fetal alcohol spectrum disorders in young adults. Human Brain Mapping, PMID: 19278010.

Mattson, S, and Riley, E. (2011). The quest for a neurodevelopmental profile of heavy prenatal alcohol exposure. Research & Health, 34 (1), 51-56.

Wetherill, L and Foroud, T (2011). Understanding the effects of prenatal alcohol exposure using three dimensional Facial Imaging. Alcohol Research & Health, 34 (1),38-42.

Feldman, HS, Jones, KL, Lindsay,S, Slyman,D., Klonoff-Cohen H, Kao,K., Rao, Chambers,C. (2012). Patterns of prenatal alcohol exposure and associated non-characteristic minor structural malformations: A prospective study. Already on-line. To be published: Am J Med Part A 155: 2949-2955 (April)

WHO Factsheet #349 (2011).

References *

American Academy of Pediatrics, New Jersey Chapter: http://www.aapnj.org/

National Organization on Fetal Alcohol Syndrome: http://www.nofas.org/

Fetal Alcohol Spectrum Disorder Center of Excellence: http://www.fasdcenter.samhsa.gov/

Centers for Disease Control National Center on Birth Defects and DDs: http://www.cdc.gov/ncbddd/features/birthdefects-dd-keyfindings.html

Fetal Alcohol Disorders Society: http://www.faslink.org/

Fetal Alcohol Syndrome Consultation, Education and Training Services, Inc.: http://www.fascets.org/

Be In The Know NJ: http://beintheknownj.org/

Article: Researchers quantify the damage of alcohol by timing and exposure during pregnancy http://www.eurekalert.org/pub_releases/2012-01/ace-rqt010812.php

Websites*

Alcohol Research and Health, Volume 34(1), 2011-FASD

Journal of Psychiatry and Law, Volume 38(4), Winter 20120 (one of 2 volumes on FASD)

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Full Journals Books Prenatal alcohol use and FASD: Diagnosis, assessment and new directions in research and multimodal treatment- Bentham Science E book edited by Adubato and Cohen- September, 2011

Fetal Alcohol Spectrum Disorder: Management and Policy Perspectives of FASD (sic) edited by Riley, et.al., 2011 Wiley-Blackwell Publishers

Prevalence of Fetal Alcohol Spectrum Disorders (sic) FASD: Who is Responsible? edited by Clarrin, et.al., 2011; Wiley-Blackwell Publishers

*Contact Information:

Speakers- Susan Adubato, PhD - [email protected] Aloisio, MD, FAAP - [email protected]

MD Champions- Alla Gordina, MD, FAAP- [email protected] Mehta, MD, MPH, FAAP- [email protected]

Thank you!

An evaluation will be sent to all participants on Wednesday, March 21, 2012. Please fill out the entire evaluation for CME/CNE credits. *

****Alcohol is a cardiovascular inhibitor, decreasing the blood delivered to fetus. Fewer sources available to develop cells, which may partially explain growth retardation in FAS

Cerebrum - Largest portion of the brain, including the cerebral hemispheres (cerebral cortex and basal ganglia); Involved in controlling consciousness and voluntary processes.

Corpus Callosum - A bundle of fibers connecting the brains hemispheres.

Hippocampus - Part of the limbic system that is involved in emotional aspects of survival behavior; also plays a role in memory.

Basal Ganglia - A group of structures lying deep in the brain involved in movement and cognition.

Cerebellum - Involved in maintenance of posture, balance and coordination.

Cortex - Outer layer of gray matter covering the survace of the cerebrum and the cerebellum.

Naccortex - Outermost portion of the cerebral cortex that contains the most structurally complex brain tissue.

DiencephalonSeptal Area - related to the limbic system that is involved in emotional aspects of survival behavior.Thalamus - a communication center that relays information to the cerebral cortexHypothalamus - important in maintaining the bodys internal environment, or homeostasis, through the receipt of sensory and chemical input.*****Misunderstood because IQ could be normalEye-hand coordination is poor;balance and gait is offCant process informationCaregivers: no fear;cant follow directions;impulsive;;Shifting problems; poor comprehension of social expectations and rules***Cog: it is more than IQ; look for intra and inter test scatter; LD, learning problemsActivity: more encoding and shifting( Claire Coles);Kieran OMalley: ADHD is earlier onset, inattentive subgroup with co-occurring developmental/psych/medical conditionsSometimes traditional meds dont work be carefulLearning: begins around 3rd grade: problems in math, writing, comprehension**Speech: production and grammar are usually OK as well as syntax; semantics and pragmatics are the problem. They are very concreteCant put things together; talks a lot; inappropriateMotor: more problems with grip strength and speed/precision; writing, drawing; overflow

**Sensory: regulation; hypo or hyper sensitive;shut down or overwhelmedSocial : gullible; nave; innocent delinquent; no stranger anxietyNeeds an external brain 24-7Mental Health: increase in depression and accidental deaths into adulthood; anxiety, conduct disorders, etcNew charts: HL ***Easily influenced, trusting, eager to please manipulatedSensory overloadCant work tho=rough problemsCan talk the talk, cant walk the walk**Hypersensitive to sounds, light, etcCant see cause and effectMiss cues, getures,Therapies: tradition ones dont work use social skills, anger management, alternative (see case management module)****Remember the individual probably is functioning at 2/3 his CANeed help 24-7Strive for independenceLook to their strengths** we must all row with the oar we are given English Proverb**the message is:stop, think, and dont drink when pregnant.

In conclusion, I would like to read a poem.

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Assessment & Management of FASD