Poysky Boston 2011

James Poysky, PhDClinical Assistant ProfessorBaylor College of Medicine

Overview How common are behavior and learning concerns in DMD? Causes of behavior and learning problems in DMD Behavior concerns in DMD  Coping with DMD  Neurobehavioral disorders Learning concerns in DMD Strategies/Interventions  General  3 highlighted areas

Behavior Concerns in DMD Total = 1284 Families No concerns (150) 12% Dont know Yes, No Diagnosis(267) 21% Yes, Diagnosis(30) 2% (836) 65%DuchenneConnect (preliminary data that may change before publication)

Learning Concerns in DMD Total = 1264 Families No Concerns Dont Know (182) 15% Yes, No Diagnosis Yes, Diagnosis (267) 21% (764) 61% (37) 3%DuchenneConnect (preliminary data that may change before publication)

Potential Causes DMD impact on brain  Social functioning  Family stress/conflict  Peer interactions Medical factors  Teachers/adults  Steroids  Fatigue  Economic resources  Medical procedures  Behavior and Cognitive Psychological interactions  Coping skills  Disposition

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 might be extra difficult Hendriksen, Poysky, Schrans, Shouten, Aldenkamp, Vles, 2008; Fitzpatrick et al 1986; Liebowitz et al 1981

COPING WITH 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

Neurobehavioral Disorders in DMD Attention-deficit disorder: 12% – 30% in DMD (with or without hyperactivity-impulsivity)Signs to look for:Impulsive Avoids workBlurts things out Overly focused on funInterrupts Easily distractedImpatient Messy and disorganizedFidgets ForgetfulEasily frustrated DaydreamsToo loud Difficulty following directionsHendriksen & Vles 2008; Poysky & Lotze, 2008; Hinton et al. 2006

Neurobehavioral Disorders in DMDOppositional, argumentative, & explosive behavior:52% of boys with DMD?Hard-headed, gets stuck on thingsRigid expectationsDifficulty adjusting to unexpected /unfavorable outcomesDifficulty controlling angerBlames othersDifficulty anticipating consequencesDoesn’t learn from mistakesPunishment escalates behavior (Poysky, Hodges, Lotze – unpublished data)

Neurobehavioral Disorders in DMDIncreased risk of: Anxiety  OCD  Worries  Rituals and excessive  Excessive fears routines  Avoids new situations or  Very particular about things people being even, lined up, etc.  Fearful of being alone  Repetitive behaviors  Intrusive thoughts/images Hendriksen & Vles 2007

Neurobehavioral Disorders in DMDAutism: 3-19% Signs to look for:  Delayed language development  Excessive and unusual interests/obsessions and routines  Impaired understanding of social interactionsWu et al. 2005; Hendriksen & Vles, 2008; Darke, Bushby, Le Couteur,McConachie, 2006; Hinton et al 2006

Neurobehavioral Disorders in DMDSocial Problems: 34% Signs to look for: Immaturity Overly dependent Social skills deficits Hinton, Nereo, Fee, Cyrulnik, 2006

Cognitive Skills  Increased risk for weaknesses in:  IQ  Language development  Short-term memory  Social reasoning (perspective-taking, social judgment, “reciprocity”)  Executive functioning (flexibility, planning, organization, insight)  Attention/Impulse Control  Fine Motor Skills? 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 Disorders40% may have a learning disorder despite normal intelligence.Dyslexia: Difficulty learning to readDyscalculia: Difficulty learning mathematicsDysgraphia: Difficulty with writing

Dyslexia Dyslexia is NOT a visual problem!!!!!!  Deficit in the “sound” aspect of reading (phonemes).  Problems are in sound-segmentation and sound- blending when trying to decode words  English language compounds the problem, because it is not consistent.

Signs of DyslexiaPreschool Elementary + Beyond• Difficulty with rhyming • Difficulty “sounding out” written• Problems identifying the wordsstarting/ending sounds •Looking at the first or last soundof spoken words in a word and guessing at the rest• Difficult or inconsistent • Dropping word endings whileidentification of letter reading (“slow” instead of “slowly”)names/sounds • Difficulty with spelling • Slow, laborious, or “dysfluent” reading • Hates reading

Dyscalculia Problems in 3 main areas  Math concepts (e.g., “number sense”, numerical relationships, symbolic representation, values manipulating each other)  Math operations (remembering the steps for a math problem, internal “number line” and mental arithmetic)  Math fluency (memorizing basic math facts)

Signs of Dyscalculia Concepts Difficulty understanding  money ($1 is 100 pennies OR 10 dimes)  “number’s place” (40 means 4 tens)  number families (7+3=10, 3+7=10, 10-7=3, 10-3=7)  part-to-whole relationships and fractions  estimating value

Signs of Dyscalculia Procedures  Can’t sequence or re-organize word problems  Difficulty remembering steps in multi-step problems  Difficulty doing mental arithmetic Fluency  Counting on fingers  Slow on timed math drills

Dysgraphia Can have problems in three main areas:  Fine motor (letter formation/spacing, “fluidity”)  Writing mechanics (punctuation, spelling, capitalization)  Sequencing/organization (knowing how to get started, organizing/sequencing ideas, tying it all together at the end)

Signs of Dysgraphia Fine Motor  Mechanics  Writing is hard to read,  Spelling problems sloppy  Errors in grammar  Difficulty staying within (sentence structure ) or space/lines syntax (meaning)  Slow, laborious writing  Forgets to capitalize,  Can sometimes make punctuation errors writing look OK, but can’t sustain this  Writes bare minimum

Signs of Dysgraphia Sequencing/Organization  Hard time thinking of what to write about  Problems knowing where to start  Difficulty sequencing thoughts  Rambling statements with no cohesion  Misses main point

Bushby K, Finkel R, Birnkrant DJ, Case L, Clemens P, Cripe L, Kaul A, KinnettK, McDonald C, Pandya S, Poysky J, Shapiro F, Tomezsko J, ConstantinC, DMD Care Considerations Working Group. The diagnosis andmanagement of Duchenne muscular dystrophy – part 1.Diagnosis, pharmacological and psychosocial management. LancetNeurology 2010;9(1):77-93.

Treatment Recommendations Effective treatment options! Same interventions as non-DMD kids. Early interventions work best.

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

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 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.

Treatment RecommendationsEducational interventions  Learning needs  Behavior needs  Modifying potentially harmful activities (physical education)  Saving energy  Safety (e.g., climbing on playground) Private tutoring

Treatment RecommendationsPsychiatric Medication For moderate to severe problems  Stimulants for ADHD  SSRI’s for anxiety/depression

Special Focus Areas

Special Focus 1: Dyslexia  Phonological awareness  Intensive synthetic phonics program  Teaches left-to-right sound-segmentation and sound-blending  Repetitive (5x/wk for 20 min better than 1x/wk for an hour)  Systematic (sequenced to reinforce previously learned skills)  Minimize “rules” that child has to memorize (not good for boys with DMD who have short-term memory weaknesses)  Repetitive reading of short passages  Improves fluency

Special Focus 2: Arguing/Temper Learning Model of Behavior (Traditional)  Child engages in negative behavior because he has learned that he can get what he wants if he acts a certain way.  Behavior is willful and intentional (manipulative)  Consistent discipline results in behavior changes.

Special Focus 2: Arguing/Temper Cognitive Deficit Model of Behavior  Child has specific weaknesses:  Cognitive flexibility  Frustration tolerance and delaying gratification  Problem solving  Anticipating consequences  Expressive language  Social skills  Insight  Problems occur when demands of situation exceed child’s skills in these areas  Punishment escalates behavior, patterns develop

Special Focus 2: Arguing/Temper Avoid escalation (remain calm) Focus on connecting with child vs. fighting child Active Listening  Validate feelings  Paraphrase content  Ask questions (avoid “Why?”)  Don’t give advice or point out how they are wrong Promote compromise and problem-solving Emphasize positive communication

Special Focus 2: Arguing/Temper Save discipline for major infractions Ignore small things Connect/problem-solve for things that are routinely difficult for child Provide extra incentive  Contingency-based strategy  Reward-based strategy

Special Focus 3: Talking about DMD Parents want to prevent distress in child  (more likely to avoid their own distress) Goal of parenting is not to protect child from life, but to cope with life Silence about DMD promotes:  keeping secrets  Ignoring/suppressing negative feelings  child should protect parents  false assumptions/misinformation  child does not learn to advocate for self  resentment when older

Special Focus 3: Talking about DMD Start early Don’t wait for child to initiate conversation Ongoing process, not just a “one time event” Model appropriate coping Involve them in medical discussions/decisions

Special Focus 3: Talking about DMD Process:  Start by asking questions about what the child knows  Correct any misinformation  Use simple explanations  Connect explanations with child’s every-day experiences  Do not ignore difficult questions  Be honest but hopeful  Reassure child that it is not their fault  Allow them to express frustration/deny problem

Special Focus 3: Talking about DMD Educate peers  More likely to protect and include child with DMD  Less likely to tease/bully child with DMD Involve child with DMD if they want, or allow them to be absent Simple explanations Reinforce that it is not contagious Utilize physical/visual examples Review ways peers can help

Poysky Boston 2011

by Christine McSherry on Nov 23, 2011

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