Behavior, Learning, and Emotions in DMD SMDF Conference 2014 Stockholm, Sweden

1. Behavior, Learning, and Emotions in DMDSMDF Conference 2014Stockholm, Sweden James Poysky, PhD Clinical Assistant Professor Baylor College of Medicine

2. 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

3. How Common are Behavior and Learning Concerns in DMD?

4. Behavior Concerns in DMD DuchenneConnect (preliminary data that may change before publication)

5. Learning Concerns in DMD DuchenneConnect (preliminary data that may change before publication)

6. Causes of Behavior and Learning Problems in DMD

7. 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

8. Coping and Psychosocial Factors in DMD

9. 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

10. 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

11. Peer Interactions in DMD Social Problems: 34% • Immaturity • Overly dependent • Social skills deficits • Social anxiety • Teasing/bullying • Peer inclusion Hinton, Nereo, Fee, Cyrulnik, 2006

12. 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

13. DMD and Brain Functioning

14. Dystrophin in the Brain • Full-length dystrophin • cerebral cortex • sub-cortical structures • Cerebellum • Smaller isoforms

15. 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

16. 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

17. Learning Disorders 40% may have a learning disorder despite normal intelligence. Dyslexia: Difficulty learning to read Dyscalculia: Difficulty learning mathematics Dysgraphia: Difficulty with writing

18. 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

19. Signs of Dyscalculia • Difficulty understanding • Math concepts • Math procedures • Memorizing basic math facts

20. 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

21. 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

22. Neurobehavioral Disorders in DMD

23. 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

24. 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)

25. 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

26. 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

27. 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

28. 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.

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

30. 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

31. 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

32. 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.

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

34. Treatment Recommendations Psychiatric Medication • For moderate to severe problems • Stimulants for ADHD • SSRI’s for anxiety/depression

35. 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

36. 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

37. 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

38. TOMORROW • Topics for discussion • Behavior problems. • Talking to kids about DMD. • Learning problems. • Parent relationships • Other? • Private conversations OK

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