Posture and Movement in CHARGE Syndrome

Texas CHARGERS

November 6th - 8th 2015

David Brown

Deaf-blind Educational Specialist

The uniqueness and complexity of CHARGE

syndrome

Working with children with CHARGE teaches you that everything is much more

complicated than it seems.

“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

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.

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

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.

The Senses

Distance Senses

• Vision

• Hearing

• Smell

Near Senses

• Taste• Touch• Vestibular• Proprioception

9

“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

“The only function of the body is to carry the brain around”

Thomas Edison

12

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)

Communication

Communication

Communication

*communication with one’s own body

*communication with one’s immediate environment

*communication with the wider world

Deaf-blind Communication

Most children with CHARGE are not in touch

with/do not feel their bodies very well

Assessment Questions D Brown, “Follow the Child” (2001)

• How do you feel?• What do you like?• What do you want?• What do you do?

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.

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).

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

Jean Ayres

[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

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

Yes, I believe that posture should be included as a “self-

stimulation” and/or a “self-regulation” behavior

(especially for people with CHARGE syndrome)

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

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!

Movement PatternsRoll

Side-winding5-point crawlBack scootBum shuffle

Bare foot, flat foot, stamp/slideBare foot, tippy-toe, knees bent

Rolling gait (drunken sailor)

27

Vision and Balance

27

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

Where’s my head?Head weavingHead binding (hat, sweatband, scarf, string)Head holding/ tappingHead pressingJaw clenchingTeeth grindingBiting/ chewing

What helps?

Physical jerks

Deep pressure

Binding

Good physical support

Regular movement

Controlled environment

Self-taught and taught strategies

Appropriate Vocabulary

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