Behavior and Duchenne Muscular Dystrophy
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Transcript Behavior and Duchenne Muscular Dystrophy
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
DMD and brain functioning
Cognitive skills and learning problems
Neurobehavioral disorders
Treatment recommendations
Behavior Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
Learning Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
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 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 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
Social Problems: 34%
•Immaturity
•Overly dependent
•Social skills deficits
•Social anxiety
•Teasing/bullying
•Peer inclusion
Hinton, Nereo, Fee, Cyrulnik, 2006
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
Dystrophin in the Brain
Full-length dystrophin
cerebral cortex
sub-cortical structures
Cerebellum
Smaller isoforms
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
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
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
Preschool
Elementary + Beyond
• Difficulty with:
• Difficulty with :
Rhyming
starting/ending
sounds
letter names/sounds
“Sounding-out” words
Spelling
•Guessing at words
• Dropping word endings (“slow”
instead of “slowly”)
• Slow, laborious, or “dysfluent”
reading
Signs of Dyscalculia
Difficulty understanding
Math concepts
Math procedures
Memorizing basic math facts
Signs of Dysgraphia
Fine Motor
Mechanics
Writing is hard to read,
Spelling problems
sloppy
Difficulty staying within
space/lines
Slow writing
Errors in grammar
(sentence structure) or
syntax (meaning)
Forgets to capitalize,
punctuation errors
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
Attention-deficit disorder: 12% – 30% in DMD
(with or without hyperactivity-impulsivity)
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
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 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 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 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
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 Recommendations
Effective treatment options!
Same interventions as non-DMD kids.
Early interventions work best.
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 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 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 Recommendations
Educational interventions
Learning needs
Behavior needs
Modifying potentially harmful activities (physical
education)
Saving energy
Safety (e.g., climbing on playground)
Private tutoring
Treatment Recommendations
Psychiatric Medication
For moderate to severe problems
Stimulants for ADHD
SSRI’s for anxiety/depression
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 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
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
Topics for discussion
Behavior problems.
Talking to kids about
DMD.
Learning problems.
Parent relationships
Other?
Private conversations OK