Rett Syndrome and Sarah: A Comprehensive Guide To Having A Child With Rett Syndrome In The School Setting
All material Copyright© 2010 Girl Power 2 Cure
Reprinted with the express permission of Girl Power 2 Cure as originally published on their website.
Research finally proves:
- Rett girls are capable of learning!
- No longer considered a degenerative disorder, there is no brain damage
- Brain organization in tact: however, the brain stem is immature due to the gene mutation and there is arrested development in cortex due to brain stem immaturity and inactivity (physical and mental).
CONTINUOUS THERAPY IS KEY TO GROWTH AND MATURATION
In both the brain stem and cortex
PART ONE: IMMATURE BRAIN STEM
Immature brain stem affects autonomic function:
SPECIFIC TO SARAH in the School setting:
(none are life-threatening, unless obvious emergency distress arises, which it has not to date)
Digestion (impaired digestion and bowel movement)
Problems to watch for: reflux, constipation, bloating, belching
Remedy: special diet, core strength, physical activity
Need from school: follow diet, keep physically active, communicate bowel movements, etc.
Breathing (random patterns of breath holding, forceful and shallow breathing)
Problems to watch for: when the above breathing patterns interfere with concentration or activity (this is rare and should not be an issue for the majority of the day)
- Breath holding: a simple touch with verbal reminder to breath out
- Forceful breathing: massage upper back and stroke hair to calm
- Shallow breathing: very hard to tell when this is happening!
Need from school: Remember, overall, breathing regulates many other functions to include blood pressure. If Sarah is having sporadic breathing, she will feel very dizzy and faint and may need time to rest. If she is being uncooperative during therapy, this can be a cause.
Oral dysfunction (Tongue thrusting, drooling, hand mouthing)
Problems to watch for: when tongue protrusion, drooling and hand mouthing interfere with activity or are socially uncomfortable for the other children.
Remedy: these issues improve with oral motor therapy, an arm brace can be used to stop hand mouthing.
Need from school: follow SLP recommendations for oral motor strengthening daily to prevent this.
watch for: delayed reaction to verbal communication
Remedy: Music is proven to increase response time. Even just singing the question can help! Also, sensory activity increases response time as it helps regulate the nervous system.
Need from school: Lots of patience! It may take Sarah at least 30 seconds to respond at times. Follow recommended sensory protocol (ie deep pressure, spinning, weight bearing)
PART TWO: ARRESTED DEVELOPMENT OF CEREBRAL CORTEX
Growth and learning potential is huge when two key things are in place:
- Autonomic functions are encouraged and controlled
- Sarah is kept physically and mentally engaged
“Therapy” needs to continue throughout each day, incorporating both the therapists IEP goals as well as constant attention to opportunities for mental and physical activity.
Sarah is capable of following the Illinois State Learning Standards for Kindergarten through an adapted curriculum. Specifically, Sarah is capable of learning to read and write with the use of a device.
Transitions: sit to stand, floor to all fours, all fours to stand
Walking with support of walker or aide
Hand, arm and shoulder flexibility and strength
joint compressions and muscle stretching
increased hand use for self-feeding, communication and expression through art and music
Use of AAC device
Eye gaze communication
Facilitation of all of the above
Stimulate cortex maturation in auditory processing
Peer interaction, group involvement.
Increase self-control, appropriate verbal and physical behavior
* While there may not be huge strides in short amounts of time, all of this therapy is important for maintaining Sarah’s overall health and well-being. We want to avoid physical problems like scoliosis, muscle atrophy, shortened tendons, joint contractures, foot deformity, and gastrointestinal distress. We also need to avoid mental/behavioral problems such as depression and anxiety.
SPECIFIC TO SARAH IN SCHOOL SETTING:
Problem: cannot independently move in classroom / throughout school
Remedy: Sarah needs minimal assist to “walk” between activities in room (no chair needed). For longer excursions to library, etc, Sarah can be pushed by aide or student in her chair.
Need from school: Help Sarah move as the other children move. Do not leave her in one area. If they are lining up to get a drink, help Sarah walk out to do the same. Sarah should participate in Phys Ed as much as possible. Obviously she cannot jump rope, but someone can help her hold the rope. She can go on the scooters, kick a ball from her chair, and be pushed in chair for running activities. While she will not experience the physical aspect of many activities, she will experience the social and sensory: ie wind, speed, noise, teamwork, etc.
Problem: Sarah cannot speak or sign.
Remedy: Adapt verbal activity and social communication for use with her AAC device or other communication means (photos, choosing from objects, eye gaze, etc)
Need from school: encourage eye gaze, consistent use of AAC device. This is her voice and must be accessible to her at all times. Remember she is learning this time and there will be a lot of trial and error, miss hits, etc which will lessen with time, but will take years for her to master.
FINE MOTOR SKILLS:
Problem: self feeding
Remedy: must assist with all feeding, including hand over hand assist
Need from school: please understand that Sarah’s hand function is a great source of stress for her. While there may be small intermittent gain, there is little hope at this time that she will ever have useful hand function.
Problem: Drawing, writing, Glue, paint, staple, cut, etc.
Remedy: all activities using these skills must be adapted.
Drawing ideas: large marker/crayons with assist, use AAC device or hold up colors for her to choose (aide does coloring). Writing can be done using large letter stamps or choice cards for aide to actually write the letters selected. Craft skill ideas: use glue stick or paint brush in cup of glue; finger paint, large paintbrush handle, switch activated scissors. Also, these commands can be programmed into her device to give a peer or aide the instructions.
Need from school: again, this is a great source of stress. These activities must remain fun and give Sarah freedom to express herself, regardless of accuracy.
Problem: accurately measuring competence
Remedy: anything you can think of to check for understanding!
Need from school: I am hoping for high expectations with low stress. Aide and teacher must assume competence. Whether or not you get the necessary feedback, after several exposures, you must assume she has processed the information.
Problem: Sarah puts her hands in her mouth a lot.
Remedy: Use arm bands when hand mouthing is incessant
Wash hands 3-4 times/day
When sitting on the floor she needs to be on a clean mat.
Need from school: patience! This is a behavior that Sarah cannot directly control
Problem: Sarah is not toilet trained.
Remedy: Change diapers when soiled or wet. We will work towards training with a voice output communication button she can wear on her wrist.
Need from school: private area for changing
An extensive body of research tells us that children with and without disabilities learn academic, social, and communication skills, as well as character traits of empathy and appreciation of individual differences when they are educated together(Wolery & Wilbers 1994; Staub & Peck 1995; Hestenes & Carroll 2000).
PART THREE: SAMPLE IEP GOALS
GOAL: Sarah will be presented with curriculum adapted for her to meet the Illinois State Learning Standards for Kindergarten
GOAL: Sarah will read preprimary/kindergarten sight words
- Given choice of sight words, Sarah will be able to identify correct word with 80% accuracy on 4/5 days.
- Given two vocabulary words, Sarah will match a picture/sign to the correct word with 90% accuracy.
GOAL: Sarah will show comprehension of literature
- Given pictures, Sarah will identify beginning, middle and end of a story with 80% accuracy
- Given picture choices, Sarah will predict what happens in the story next with 80% accuracy
- Sarah will listen to a story, then answer simple questions about the story by choosing the correct answer from a field of two or three, with 70% accuracy.
GOAL: Sarah will show comprehension of basic math
- Sarah will demonstrate knowledge of ordinal numbers 1-100 with 80% accuracy.
- Sarah will demonstrate knowledge of the terms “more” and “less” with 90% accuracy.
GOAL: Sarah will exercise increased self-control. Increasing appropriate verbal and physical behaviors with fading prompts/models.
- Given a class situation when Sarah is evidencing inappropriate behavior, she will indicate, with modeling or prompts (ie are you OK?” “What’s the matter?” or “hit your switch if you need help”), her feelings (frustration, discomfort, fear), and a more appropriate behavior and language (ie “help me”, “it’s my turn”, or “I need a rest”) 80% of the time 4/5 days.
Goal: Sarah will demonstrate improved social skills.
- Sarah will look at the person speaking with minimal prompt 60% of the time.
- Sarah will participate in turn taking with minimal assistance 80% of the time
- Sarah will engage in direct communication/activity with a peer/peers in class throughout the day (ie students will help Sarah with activities whenever possible. The aide should facilitate/accept to have a student model an activity, ask Sarah questions, take peer suggestions, accept peer volunteerism, etc.)
Sarah will have access to her AAC device at all times
Sarah will get the sensory information she needs during the school day to help her focus and organize herself.
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