Rett’s syndrome is a genetic disorder that affects only girls and although there is no cure, activities for Rett syndromes may help maintain or slow down loss of motor and communication skills and prevent contractures.
Rett’s Syndrome Symptoms and Challenges
Rett’s syndrome is considered one type of pervasive developmental disorder (PDD) where the child presents with autistic-like symptoms such as repetitive self-stimulatory movements (i.e. hand flapping, wringing and mouthing) and apraxia (poor coordination). Children with Rett’s syndrome appear to develop normally during the first six months of life and thereafter lose skills previously learned. They may develop spasticity or resistance to movement, seizures, swallowing/chewing impairment and hyperventilation. Children with the syndrome are typically nonverbal and have severe cognitive delays. In addition, poor balance and motor coordination cause children to walk with an unsteady, wide-based, stiff legged gait.
Activities for Rett's Syndrome: Positioning and Splinting
Children with Rett’s syndrome should be positioned to prevent contractures and to promote function with feet, knees and hips at 90 degree angles. Chairs should have arm rests for support and sized so that the child can position his or her feet flat on the floor. Wheelchairs may be adapted to fit within a circular table or a tray may be attached to the chair so that work materials can be stabilized on the tray and easily reached. An occupational therapist can determine the best height or angle of activities (such as rubbing various textured objects or activating switches) so that they can be most easily viewed and manipulated. Children with Rett’s syndrome have limited abilities to manipulate objects but they can be assisted in grasping them inside their palms during functional tasks such as:
- Brushing hair
- Brushing teeth
- Bringing a spoon to the mouth
- Mixing ingredients in a bowl
- Painting with paint brushes or a sponge attached to a handle
All of these activities can be adapted with built up handles that make grasping easier. Hand-over-hand assistance to perform these tasks provides passive range-of-motion, and grasping large objects opens up the hand, helping to prevent fisting and contractures. At the same time, grasping weighted items such as wrist weights or objects that vibrate (i.e., electric toothbrushes) provides sensory stimulation that may promote attention to tasks.
It is beneficial for students with Rett's syndrome to spend time positioned so that hips, arms and legs are stretched in extension and they can bear weight on hands. Suggested positions include prone over a wedge, bolster or therapy ball small enough to enable reaching the floor. Slowly rock the child over the equipment and/or provide objects to push down on with palms such as switches, textured pictures or water play mats (sold for babies).
Activating Switches to Teach Causality
Students with severe cognitive delays often enjoy activating switches after they learn that pushing, squeezing or rolling the switch activates a desirable electronic device such as a radio, fan or vibrating snake. These activities teach causality, the understanding that an action causes a specific result. At the same time, these activities for Rett syndromes promote functional hand use.
Promoting Communication Skills
Higher functioning students may communicate by touching pictures in a communication board. Students who lack adequate motor control to activate switches may be able to communicate using eye gaze technology instead. Axel F. Bang describes in his article “Girls With Rett Syndrome find Their ‘voice' in Eye-Gaze Technology" how five-year-old Maisy identified animals by name by using a combination of pointing her finger and eye gaze technology that tracked her eye movements.
Students with Rett’s syndrome may enjoy a variety of sensory activities that promote hand use and environmental awareness. Try one of the following:
- Rubbing hands in whip or shaving cream
- Squeezing toys and sponges in a basin filled with soapy water
- Feeling dry beans, lentils or pasta in a container. Try adding a vibrating toy that shakes the container.
In addition, if the student is able to safely chew and swallow small food pieces, spread them on a tray to reach, grasp and eat. Provide one piece at a time if the student tends to overfill his or her mouth. Self-feeding is a great sensory motor activity as well as a self-care skill. The most effective activities for Rett syndromes will take into consideration the student’s cognitive and motor abilities and sensory preferences.
Rett Syndrome Fact Sheet, 2009, National Institute of Neurological Disorders and Stroke
Girls with Rett Syndrome find Their "voice" In Eye-Gaze Technology
Smith, B., The Recycling Occupational Therapist; Austin, Texas: Pro-Ed., Inc.; 1997.
Richard, G. and Hoge, D.; The Source for Syndromes; East Moline, Illinois: Linguisystems; 1999.
Smith, J, Allen, A and O'Brien, J. ; Occupational Therapy for Children; Boston, Massachusetts: Mosby; 2009.