behavior, learning, and emotions in dmd smdf conference 2014 stockholm, sweden
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Behavior, Learning, and Emotions in DMD SMDF Conference 2014 Stockholm, Sweden. James Poysky, PhD Clinical Assistant Professor Baylor College of Medicine. Overview. How common are behavior and learning concerns in DMD? Causes of behavior and learning problems in DMD Coping with DMD - PowerPoint PPT PresentationTRANSCRIPT
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James Poysky, PhD
Clinical Assistant Professor
Baylor College of Medicine
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OverviewHow common are behavior and learning concerns in
DMD?Causes of behavior and learning problems in DMDCoping with DMDDMD and brain functioning
Cognitive skills and learning problemsNeurobehavioral disorders
Treatment recommendations
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Behavior Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
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Learning Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
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Potential CausesPsychological
Coping with DMD
Psychosocial FactorsFamily stress/conflictPeer interactionsTeachers/adults
DMD impact on brain functioning
Medical factorsSteroidsFatigue/sleepMedical proceduresBlood sugar
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COPING WITH DMDHow are the boys coping?
Same as boys with other chronic medical conditions Being sad and frustrated at times due to DMD is a normal
reaction Coping gets better with age Ages 8-10 and adolescence might be extra difficult Some boys may become depressed/distressed
Hendriksen, Poysky, Schrans, Shouten, Aldenkamp, Vles, 2008; Fitzpatrick et al 1986; Liebowitz et al 1981
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COPING WITH DMDSome boys not as “independent” as they could be
Big focus on transition to adulthoodLiving independentlyMaking decisions in medical careEmploymentRomantic relationships
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Peer Interactions in DMDSocial Problems: 34%
Hinton, Nereo, Fee, Cyrulnik, 2006
•Immaturity•Overly dependent•Social skills deficits•Social anxiety•Teasing/bullying•Peer inclusion
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Families and DMDFamily Adjustment
Increased rates of parental depression and isolationBehavior problems can be as stressful for parents as
physical aspects of DMDSibling adjustment
Abi Daoud, Dooley, Gordon 2004; Bothwell , Dooley , Gordon , MacAuley, Camfield 2002; Poysky & Kinnett , 2009; Nereo, Fee, Hinton, 2003
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Dystrophin in the BrainFull-length dystrophin
cerebral cortexsub-cortical structuresCerebellum
Smaller isoforms
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Dystrophin in the BrainPossible effects of absent or dysfunctional
dystrophin:Makes neurons less efficient in sending signals
to each otherMakes neurons less ready for new signalsReduced formation of new “connections”
between neurons
Knuesel et al. Eur J Neurosci., 11:4457-62 (1999); Vaillend & Billard, Hippocampus, 12:713-717 (2002); Kueh, Head, Morley, Clin Exp Pharmacol Physiol. 2008 Feb;35(2):207-10
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Neurocognitive Skills Increased risk for weaknesses in:
Language development
Short-term memory
Social reasoning (perspective-taking, social judgment,
“reciprocity”)
Executive functioning (flexibility, planning, organization, insight)
Attention/Impulse Control
Fine Motor Skills and Motor Planning?
Cotton, Voudouris, Greenwood 2001; Hinton, De Vivo, Nereo, Goldstein, Stern 2000; Hinton, De Vivo, Nereo, Goldstein,
Stern 2001; Cyrulnik, Fee, De Vivo, Goldstein, Hinton 2007; Hendriksen, Vles 2006; Hinton, Nereo, Fee, Cyrulnik 2006;
Cotton, Crowe, Voudouris 1998; Wicksell, Kihlgren, Melin, Eeg-Olofsson 2004; Donders, Taneja 2009
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Learning Disorders40% may have a learning disorder
despite normal intelligence.
Dyslexia: Difficulty learning to read
Dyscalculia: Difficulty learning mathematics
Dysgraphia: Difficulty with writing
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Signs of DyslexiaPreschool• Difficulty with:
Rhymingstarting/ending
sounds letter names/sounds
Elementary + Beyond• Difficulty with :
“Sounding-out” words Spelling
•Guessing at words
• Dropping word endings (“slow” instead of “slowly”)
• Slow, laborious, or “dysfluent” reading
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Signs of DyscalculiaDifficulty understanding
Math conceptsMath proceduresMemorizing basic math facts
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Signs of DysgraphiaFine Motor
Writing is hard to read, sloppy
Difficulty staying within space/lines
Slow writing
MechanicsSpelling problemsErrors in grammar
(sentence structure) or syntax (meaning)
Forgets to capitalize, punctuation errors
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Signs of DysgraphiaSequencing/Organization
Difficulty with: thinking of what to write knowing where to start sequencing thoughts
Rambling statements that don’t fit togetherMisses main point
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Neurobehavioral Disorders in DMD
Signs to look for:Impulsive
Blurts things out
Interrupts
Impatient
Fidgets
Easily frustrated
Too loud
Hendriksen & Vles 2008; Poysky & Lotze, 2008; Hinton et al. 2006
Avoids work
Overly focused on fun
Easily distracted
Messy and disorganized
Forgetful
Daydreams
Difficulty following directions
Attention-deficit disorder: 12% – 30% in DMD
(with or without hyperactivity-impulsivity)
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Neurobehavioral Disorders in DMDOppositional, argumentative, & explosive behavior:
52% of boys with DMD?
Hard-headed, gets stuck on things
Rigid expectations
Difficulty adjusting to unexpected outcomes
Difficulty controlling anger
Blames others
Difficulty anticipating consequences
Doesn’t learn from mistakes
Punishment escalates behavior
(Poysky, Hodges, Lotze – unpublished data)
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Neurobehavioral Disorders in DMD“HANGRY” = Hungry + Angry
Angry IrrationalMean/aggressiveEmotionally sensitive/labile
Don’t feel hungry
Rapid return to happy/normal mood
after eating food
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Neurobehavioral Disorders in DMDIncreased risk of:
AnxietyWorriesExcessive fearsAvoids new situations or
peopleFearful of being alone
OCDRituals and excessive
routinesVery particular about things
being even, lined up, etc.Repetitive behaviors Intrusive thoughts/images
Hendriksen & Vles 2007
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Neurobehavioral Disorders in DMDAutism: 3-19% Signs to look for:
Delayed language development
Excessive and unusual interests/obsessions and routines
Impaired understanding of social interactions
Wu et al. 2005; Hendriksen & Vles, 2008; Darke, Bushby, Le Couteur,McConachie, 2006; Hinton et al 2006
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Bushby K, Finkel R, Birnkrant DJ, Case L, Clemens P, Cripe L, Kaul A, Kinnett K, McDonald C, Pandya S, Poysky J, Shapiro F, Tomezsko J, Constantin C, DMD Care Considerations Working Group. The diagnosis and management of Duchenne muscular dystrophy – part 1. Diagnosis, pharmacological and psychosocial management. Lancet Neurology
2010;9(1):77-93.
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Treatment RecommendationsEffective treatment options!
Same interventions as non-DMD kids.
Early interventions work best.
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Treatment RecommendationsRecommended testing/assessments
Neuropsychological/developmental All kids with DMD at diagnosis or prior to starting school
Language and autism If concerns arise
Emotional/coping screening Annually, in clinic
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Treatment RecommendationsPsychotherapy Parental behavior management training
Noncompliance, disruptive behavior, temper meltdowns
Individual therapy Low self-esteem and depression, anxiety, obsessive-compulsive
disorder, coping
Group therapy Social skills deficits
Applied Behavior Analysis Autism
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Treatment RecommendationsSocial interventions
Promoting patient independence and self-advocacy
Talking about DMD: child, peers, teachers, etc.
Developing interests and staying involved! Modified/adapted sports, summer camps, and youth
groups/programs Art groups, equestrian, and aqua therapies, use of service
dogs, nature programs, and internet/chat rooms, etc.
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Treatment RecommendationsEducational interventions
Learning needsBehavior needsModifying potentially harmful activities (physical
education)Saving energySafety (e.g., climbing on playground)
Private tutoring
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Treatment RecommendationsPsychiatric MedicationFor moderate to severe problems
Stimulants for ADHDSSRI’s for anxiety/depression
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Treatment RecommendationsFamily interventions Parent mental health
Counseling Involvement in DMD community Support network Marital support
Sibling mental health Individual attention Connect with other siblings Promote own identity
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Treatment RecommendationsOther therapies
Speech/Language Therapy Developmental language delays, articulation problems
Occupational Therapy Independent living skills, assistive technology, writing problems
DietMore frequent, smaller meals (need to be healthy)Consult with doctor/dietician
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SUMMARY Increased risk of behavior problems in DMD
Multiple factors may contribute to behavior problems Coping/adjusting to DMD Brain functioning Family stress/adjustment Peer/social issues
Interventions can help Multiple interventions may be needed Earlier is better
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TOMORROWTopics for discussion
Behavior problems.Talking to kids
about DMD.Learning problems.Parent relationshipsOther?
Private conversations OK
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