Behavior in Cornelia de Lange Syndrome

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Behavior in Cornelia de Lange Syndrome

• Eileen P Ahearn MD PhD
• CdLS Conference 2004

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Behavior in CdLS

• Children with CdLS are warm and lovable. They are
  also tempermental, and sometimes have rapid
  changes in mood. They can have strong reactions
  to things like noise, excessive stimulation and
  even normal events. What may not bother them on
  one occasion, they may find intolerable on the
  next occasion. They can get obsessive about
  things, have unusual preoccupations, and they
  hate change in their routine. They are wonderful
  and exasperating all at the same time.

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Factors that can lead to anxiety and aggression
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Consequent behavior
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How to Approach Behavior Problems

• 1. Prevention
• 2. Behavioral intervention
• 3. Medication treatment

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The Philosophy of Prevention

• Begin building life skills
• communication
• academic
• activities of daily living
• social skills

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The Philosophy of Prevention Structure

• Setting up the physical environment
• Daily schedule
• Quiet work area for homework/ activities
• Minimize distractions, reduce stimulation

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Schedule

• What activities will occur
• When activities will occur- sequence
• With whom activities will occur

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Daniels schedule

• eat breakfast
• get dressed
• brush teeth
• go to school
• snack
• toilet
• homework
• go for a walk with Kristin
• eat dinner
• jump on trampoline with daddy
• go to bed

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Activities

• Find a way to let children to know the following
• What should I do?
• How much should I do?
• When will I be finished?
• What happens next?

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The Philosophy of Prevention Exercise

• Fast walking, running, treadmill
• Swimming
• Roller-skating, aerobics, biking

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The Philosophy of Prevention Calming Activities

• Engagement
• Focus
• Lower anxiety

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Calming Activities

• Listening to soft music
• Lying in a hammock
• Holding a special doll or stuffed animal
• Looking at a favorite book
• Muscle tension and relaxation (squeezing a ball)
• Deep breathing (Show a picture of this and
  demonstrate. Get child to practice when they are
  calm).

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Social Stories

• Used for difficult behaviors/anxiety provoking
  situations
• Write a story about the event
• Practice the story
• Let the child tell the story using pictures or
  words.

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Example of a Social Story

• DANIEL GOES SWIMMING WITH KRISTIN
  
  Every
  Wednesday, Daniel goes to the YMCA with Kristin.
  First they go into Kristins gray car. Daniel
  likes to ride in Kristins car. Then they get to
  the YMCA and Daniel puts on his orange bathing
  suit. This is his favorite bathing suit. They
  get ready to go into the water. Daniel is
  scared, but Kristin helps him. Daniel gets into
  the water. He is happy because the water is
  warm. He has a lot of fun swimming. Good job,
  Daniel!

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Intervention Strategies

• Develop a communication system
• Create a place to go (e.g. bean bag chair,
  special chair, etc)
• Add calming activities

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Intervention Strategies
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Intervention Strategies Continued

• Provide a clear beginning and end (egg timer may
  be helpful)
  
• Develop release strategies (alternative
  strategies if the calming activity does not work
  ex. Punching a bean bag, squeezing something,
  biting a plastic toy)

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Behavioral ManagementWhat to ask when problems
occur

• Is there a pattern to behavioral problems? Is
  this situational?
• Does this happen at a certain time or place, or
  with the same person?

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Behavioral Management

• What preventative strategies can I use to
  decrease the frequency of this behavior?
  (communication, structure, exercise, calming
  activities)
• What intervention can I use to help my child
  control their challenging behavior?
  (communication system, place to go, calming
  activities or release strategies)

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What to do if intervention fails

• Sometimes it takes multiple tries or fine
  tuning of strategy
• Consider revising your intervention strategy
• Remember, as a child matures you may need to
  change strategies
• Practice calming activities
• Seek professional advice (school specialist,
  developmental psychologist, psychiatrist)

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The medication Issue

• Prior to using medication, look for physical
  problems
• Is my child in pain?
• Is a medical problem causing my child to become
  irritable?
• Does my child have reflux?

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The medication issue

• There is a lot that we dont know about
  psychiatric medications, and few systematic
  studies have been done for children with
  disabilities.
• All categories of psychiatric medications have
  been used for CdLS. It is sort of hit or miss
  as to whether medications will work and keep
  working.

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Antidepressants

• usually very safe
• helpful for depression, agitation, anxiety, and
  obsessions/compulsions
• can be used long term, safe, may wear off over
  time
• Prozac, Paxil, Zoloft, Luvox, Celexa, Lexapro,
  Remeron, Wellbutrin

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Antipsychotics

• Medications developed for Schizophrenia, not
  specific for developmental disabilities
• can by used for aggressive/self-injurious
  behavior
• the old ones haldol, prolixin, navane, mellaril-
  not used much anymore
• the new ones (atypical antipsychotics) Zyprexa,
  Geodon, Risperdal, Seroquel, Abilify.

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Other medications

• Anticonvulsants (seizure medications) Depakote,
  Tegretol, Lamictal, Gabapentin, Gabitril. Used
  for bipolar disorder, can have calming/mood
  stabilizing effect.
• Clonidine used for restlessness, aggression,
  hyperactivity
• Stimulants Used for attentional symptoms
• Opiod antagonists Revia, has been used for
  difficult to treat self injury , mixed results

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Summary of Behavior Management

• Building life skills, providing a structured
  environment, ensuring regular exercise for your
  child, and teaching them calming strategies to
  use when they are upset, will help to minimize
  the difficulties they may face. Medication may
  also be helpful when these strategies do not work.

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Summary of Behavior Management

• It is difficult to anticipate behavioral problems
  that may arise in a childs life. Even the best
  parents struggle with these issues. Because
  children with CdLS often have cognitive and
  communication limitations, they are are at higher
  risk for developing behavioral problems. These
  problems are treatable.