1. Posture and Movement in CHARGE Syndrome Texas CHARGERS November 6 th - 8 th 2015 David Brown Deaf-blind Educational Specialist

2. The uniqueness and complexity of CHARGE syndrome

3. Working with children with CHARGE teaches you that everything is much more complicated than it seems.

5. “Why Aren’t You Paying Attention? The Interaction of Posture & the Executive Function of Attention” Three-part webinar by Dinah Reilly http://www.idahotc.com/Webinars/ tabid/218/categoryid/4/5.aspx

6. P Hodges, et al (1991) Postural activity of the diaphragm is reduced in humans when respiratory demand increases Journal of Physiology 537.3: 999 1008 Every muscle in the trunk is both respiratory and postural muscle, especially the diaphragm. If breathing is compromised, postural muscle activation is reduced to focus on immediate needs of respiration and thus posture is de- stabilized. L Yardley, et al (1999) Effect of articulatory and mental tasks on postural control NeuroReport 10:215- 219 Articulation increases postural sway in stance in adults.

7. We must always remember that everything joins up! Self determination + Sensory perception + Self image + Emotional competence + The attitude & behavior of others + Self regulation + Executive function + Availability for learning + Previous experience + Expectations & Motivators + Communication & language

8. CHARGE syndrome involves many more senses than just vision & hearing, and it is not enough only to consider the tactile sense as a compensatory channel.

9. The Senses Distance Senses Vision Hearing Smell Near Senses Taste Touch Vestibular Proprioception 9

11. “The Forgotten Senses” PROPRIOCEPTION The receptors are in the muscles and joints throughout the body Tells us about the position of our body and all of our limbs, and if anything is moving VESTIBULAR The receptors are in the Inner Ears Tells us about head position & the pull of gravity, detects motion, and it has very close links with the eyes and vision

12. “The only function of the body is to carry the brain around” Thomas Edison 12

13. Whose perception counts? “The brain, the organ that is responsible for your conscious experience, is an eternal prisoner in the solitary confinement of the skull…and must rely on information smuggled into it from the senses…the world is what your brain tells you it is, and the limitations of your senses set the boundaries of your conscious experience.” Coren, Porac & Ward “Sensation & Perception” (1984, p2)

14. Communication Communication Communication

15. *communication with one’s own body *communication with one’s immediate environment *communication with the wider world Deaf-blind Communication

16. Most children with CHARGE are not in touch with/do not feel their bodies very well

17. Assessment Questions D Brown, “Follow the Child” (2001) How do you feel? What do you like? What do you want? What do you do?

18. Most people focus on the child’s disabilities, but close attention to their abilities can reveal more about the difficulties they face as well as the strategies they use to function effectively.

19. Everything that children with CHARGE do has meaning, and the first obligation on the teacher is to ascertain that meaning (or at least to come up with a really good guess).

20. “After air to breathe, postural security is our next most urgent priority.” Jean Ayres

21. [Self-regulation]… “is defined as the capacity to manage one’s thoughts, feelings and actions in adaptive and flexible ways across a range of contexts” Jude Nicholas, CHARGE Accounts, Summer 2007 21

22. The 9 levels of arousal (Carolina Record of Individual Behavior) Uncontrollable agitation Mild agitation Fussy awake Active awake Quiet awake Drowsy Active sleep Quiet sleep Deep sleep 22

23. Yes, I believe that posture should be included as a “self- stimulation” and/or a “self- regulation” behavior (especially for people with CHARGE syndrome)

24. Observing how and when a child self-stimulates will offer invaluable insights into who they are and how they work, for assessment, teaching, behavior management, and relationship building

25. Why is walking a big problem? Bone malformations Loose joints Visual impairment Other sensory issues (Proprioceptive & Vestibular!!!) Breathing problems Cerebral palsy? Stress, depression, fear Medication Distractibility (one thing at a time) Low expectations & over-protection Too many faster, safer ways of moving!

26. Movement Patterns Roll Side-winding 5-point crawl Back scoot Bum shuffle Bare foot, flat foot, stamp/slide Bare foot, tippy-toe, knees bent Rolling gait (drunken sailor)

27. 27 Vision and Balance 27

28. 28 Vestibulo-ocular Reflex (VOR) Normal head rotation: eyes move in opposite direction of head to stabilize retinal image (VOR) Conflicting sensory information from visual and vestibular senses is a problem 28

29. Where’s my head? Head weaving Head binding (hat, sweatband, scarf, string) Head holding/ tapping Head pressing Jaw clenching Teeth grinding Biting/ chewing

30. What helps? Physical jerks Deep pressure Binding Good physical support Regular movement Controlled environment Self-taught and taught strategies Appropriate Vocabulary

31. So….????? *Getting the brain in better contact with the body/postural security *Preparation for attending and learning *Self-regulating *The importance of sensory inputs, positioning, & posture/movement