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

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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

Behavior, Learning, and Emotions in DMDSMDF Conference 2014Stockholm, Sweden

James Poysky, PhD

Clinical Assistant Professor

Baylor College of Medicine


Overview

Overview

  • How common are behavior and learning concerns in DMD?

  • Causes of behavior and learning problems in DMD

  • Coping with DMD

  • DMD and brain functioning

    • Cognitive skills and learning problems

    • Neurobehavioral disorders

  • Treatment recommendations


How common are behavior and learning concerns in dmd

How Common are Behavior and Learning Concerns in DMD?


Behavior concerns in dmd

Behavior Concerns in DMD

DuchenneConnect (preliminary data that may change before publication)


Learning concerns in dmd

Learning Concerns in DMD

DuchenneConnect (preliminary data that may change before publication)


Causes of behavior and learning problems in dmd

Causes of Behavior and Learning Problems in DMD


Potential causes

Potential Causes

  • Psychological

    • Coping with DMD

  • Psychosocial Factors

    • Family stress/conflict

    • Peer interactions

    • Teachers/adults

  • DMD impact on brain functioning

  • Medical factors

    • Steroids

    • Fatigue/sleep

    • Medical procedures

    • Blood sugar


Coping and psychosocial factors in dmd

Coping and Psychosocial Factors in DMD


Coping with dmd

COPING WITH DMD

How 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


Coping with dmd1

COPING WITH DMD

  • Some boys not as “independent” as they could be

  • Big focus on transition to adulthood

    • Living independently

    • Making decisions in medical care

    • Employment

    • Romantic relationships


  • Peer interactions in dmd

    Peer Interactions in DMD

    Social Problems: 34%

    • Immaturity

    • Overly dependent

    • Social skills deficits

    • Social anxiety

    • Teasing/bullying

    • Peer inclusion

    Hinton, Nereo, Fee, Cyrulnik, 2006


    Families and dmd

    Families and DMD

    • Family Adjustment

      • Increased rates of parental depression and isolation

      • Behavior problems can be as stressful for parents as physical aspects of DMD

      • Sibling adjustment

        Abi Daoud, Dooley, Gordon 2004; Bothwell , Dooley , Gordon , MacAuley, Camfield 2002; Poysky & Kinnett , 2009; Nereo, Fee, Hinton, 2003


    Dmd and brain functioning

    DMD and Brain Functioning


    Dystrophin in the brain

    Dystrophin in the Brain

    • Full-length dystrophin

      • cerebral cortex

      • sub-cortical structures

      • Cerebellum

    • Smaller isoforms


    Dystrophin in the brain1

    Dystrophin in the Brain

    • Possible effects of absent or dysfunctional dystrophin:

      • Makes neurons less efficient in sending signals to each other

      • Makes neurons less ready for new signals

      • Reduced 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


    Neurocognitive skills

    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


    Learning disorders

    Learning Disorders

    40% may have a learning disorder despite normal intelligence.

    Dyslexia: Difficulty learning to read

    Dyscalculia: Difficulty learning mathematics

    Dysgraphia: Difficulty with writing


    Signs of dyslexia

    Signs of Dyslexia

    Preschool

    • Difficulty with:

    • Rhyming

    • starting/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


    Signs of dyscalculia

    Signs of Dyscalculia

    • Difficulty understanding

      • Math concepts

      • Math procedures

      • Memorizing basic math facts


    Signs of dysgraphia

    Signs of Dysgraphia

    • Fine Motor

      • Writing is hard to read, sloppy

      • Difficulty staying within space/lines

      • Slow writing

    • Mechanics

      • Spelling problems

      • Errors in grammar (sentence structure) or syntax (meaning)

      • Forgets to capitalize, punctuation errors


    Signs of dysgraphia1

    Signs of Dysgraphia

    • Sequencing/Organization

      • Difficulty with:

        • thinking of what to write

        • knowing where to start

        • sequencing thoughts

      • Rambling statements that don’t fit together

      • Misses main point


    Neurobehavioral disorders in dmd

    Neurobehavioral Disorders in DMD


    Neurobehavioral disorders in dmd1

    Neurobehavioral Disorders in DMD

    Signs to look for:

    Attention-deficit disorder: 12% – 30% in DMD

    (with or without hyperactivity-impulsivity)

    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


    Neurobehavioral disorders in dmd2

    Neurobehavioral Disorders in DMD

    Oppositional, 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)


    Neurobehavioral disorders in dmd3

    Neurobehavioral Disorders in DMD

    “HANGRY” = Hungry + Angry

    • Angry

    • Irrational

    • Mean/aggressive

    • Emotionally sensitive/labile

    • Don’t feel hungry

    • Rapid return to happy/normal mood

      after eating food


    Neurobehavioral disorders in dmd4

    Neurobehavioral Disorders in DMD

    Increased risk of:

    • Anxiety

      • Worries

      • Excessive fears

      • Avoids new situations or people

      • Fearful of being alone

    • OCD

      • Rituals and excessive routines

      • Very particular about things being even, lined up, etc.

      • Repetitive behaviors

      • Intrusive thoughts/images

        Hendriksen & Vles 2007


    Neurobehavioral disorders in dmd5

    Neurobehavioral Disorders in DMD

    Autism: 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


    Treatment recommendations

    Treatment Recommendations

    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.


    Treatment recommendations1

    Treatment Recommendations

    • Effective treatment options!

    • Same interventions as non-DMD kids.

    • Early interventions work best.


    Treatment recommendations2

    Treatment Recommendations

    Recommended 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


    Treatment recommendations3

    Treatment Recommendations

    Psychotherapy

    • 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


    Treatment recommendations4

    Treatment Recommendations

    Social 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.


    Treatment recommendations5

    Treatment Recommendations

    Educational interventions

    • Learning needs

    • Behavior needs

    • Modifying potentially harmful activities (physical education)

    • Saving energy

    • Safety (e.g., climbing on playground)

  • Private tutoring


  • Treatment recommendations6

    Treatment Recommendations

    Psychiatric Medication

    • For moderate to severe problems

      • Stimulants for ADHD

      • SSRI’s for anxiety/depression


    Treatment recommendations7

    Treatment Recommendations

    Family 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


    Treatment recommendations8

    Treatment Recommendations

    Other therapies

    • Speech/Language Therapy

      • Developmental language delays, articulation problems

    • Occupational Therapy

      • Independent living skills, assistive technology, writing problems

        Diet

    • More frequent, smaller meals (need to be healthy)

    • Consult with doctor/dietician


    Summary

    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


    Tomorrow

    TOMORROW

    • Topics for discussion

      • Behavior problems.

      • Talking to kids about DMD.

      • Learning problems.

      • Parent relationships

      • Other?

    • Private conversations OK


    Behavior learning and emotions in dmd smdf conference 2014 stockholm sweden

    Thank youSMDF and boys/young men with DMD!treat-nmd.euTREAT NMD: Family Care Guidelines parentprojectmd.orgEducation Matters: Learning and Behavior Guide (Poysky)Psychology of Duchenne (Hendriksen)


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