Finding Play Within the Whispers:

Adapting Activity for the Silent World of Rett Syndrome

General/History: Rett Syndrome is a unique neurological disorder that is often misdiagnosed as Autism and Pervasive Developmental Disorder. The disorder was first noted by its namesake, Dr. Andreas Rett in 1964, but it became most well known in 1983 after a first English publication by Dr. Bengt Hagberg.

Occurrence: Rett syndrome is now known to occur in anywhere from 1:10,000 to 1:23,000 female births.  Approximately 99.5% of such births occur only once in a given family. Rett involves a genetic defect that is carried on the x chromosome, which is why it is almost always exclusive to females (who have an extra x chromosome ?male fetuses cannot compensate without the additional chromosome and therefore don't usually come to term). The disorder occurs across all racial and ethnic groups.

Appearance/attributes of girls with the syndrome: Girls with Rett appear to develop quite normally until anywhere from 6 to 18 months of age (although a decrease in head circumference may be noted as early as 2 months). At 18 months children with Rett begin to experience a loss of acquired speech and fine motor skills, a loss of or difficulty with mobility, and the presence of repetitive hand movements (often hand washing, hand wringing, hand shaking, and mouthing of the hand, etc.). There may be a general period of stabilization around 10 years of age that may or may not be followed by a further loss of motor skills later in life. Although complications such as seizure activity, hyperventilation and/or apnea, teeth grinding and curvature of the spine (scoliosis) can occur, most females with Rett syndrome are expected to survive into adulthood.

Adapted Activity and Retts Syndrome: Rett syndrome offers A.P.E. professionals, parents, caregivers, and any other personnel a unique opportunity for creatively adapting activity.  There are numerous aspects to adapted activity that can be considered to include:

  Decreased language:  Adapted physical educators may want to provide services in a language rich environment in hopes of slowing the progression of lost language skills. Verbalizing activities before, during, and after completion of a given task is an important way of doing this. Adapted physical educators should also be aware of any interventions by speech professionals or family members to encourage non-verbal communication (picture boards, gestures or signs, eye gaze communication, etc.) so that this may be incorporated into physical activities. Music is also an excellent way to encourage verbal skills.

  Music:  Music appears to be a common denominator for many children with Rett Syndrome. Girls with the syndrome appear to be very motivated by music and may sometimes respond to it more than other interventions. Adapted physical educators, should, therefore make attempts to incorporate music into as many activities as possible. In inclusion settings, the adapted professional may even wish to provide shakers, drums, and other musical instruments and encourage the child to set a pace for any locomotor activities in which she can not readily engage (with it being a given that as much activity and movement as possible should always be encouraged). Even the use of rhythmical, "sing-song" instructions of an activity may prove more effective than regular intonations.

  Decreased Mobility and Motor Skills: Adapted physical education professionals should focus on activities that will assist in delaying the loss of motor and mobility skills. Stretching should be emphasized at the beginning and end of activities as a result of a risk for decreased range of motion. While a child with Rett is still able to be upright and mobile, activities that challenge balance may be helpful (walking on uneven surfaces, water activities using buoyancy to challenge balance, etc.). Activities that strengthen the hip stabilizer muscles -- the adductors and the abductors can be very helpful.  For example, to strengthen the hip adductors the child can be asked to squeeze her straight legs together, while sitting, to try to break a balloon.  To strengthen the hip abductors, the child can be asked to squeeze her straight legs, while sitting, against the feet of the teacher, trying to force the teacher's legs apart.  Additionally, a wide variety of mobility devices (gait trainers, mobile standers, etc.) can be used during physical activities. Interdisciplinary efforts are very useful in incorporating such mobility equipment into a variety of settings.

Water Activities: The water is another effective element for individuals with Rett syndrome. This may be explained by the many sensory properties that can be offered through and aquatic environment (neutral warmth, gentle compression, increased mobility, muted noise levels, etc.). More information about the sensory benefit of aquatic activities can be accessed at the sensory intervention and aquatics page.

Repetitive Hand Movements: The repetitive hand movements of Rett syndrome may vary from child to child. Adapted physical educators may want to be aware of any management strategies that parents and other professionals have developed to assist with this. Frequently sensory input such as deep pressure, weight bearing on the hands, two handed activities, and even vestibular (fast moving rotary or stop-start) activity can assist with decreasing repetitive hand movements.

HOOKING UP WITH USEFUL LINKS

Below are some excellent web pages to provide information about Rett Syndrome:

Family Village Rett Page

http://www.familyvillage.wisc.edu/lib_rett.htm#pers

International Rett Syndrome Association (IRSA)

http://www.rettsyndrome.org/

Living with Rett Syndrome Page (parents page)

http://members.aol.com/Rsmother/sitemap.html

Rett Syndrome Research Foundation

http://www.rsrf.org/

Unnamed General Website with Multiple Links

http://www.isn.net/~jypsy/rett.htm

Final Thoughts

Rett syndrome offers both challenge and opportunities for children, families, and professionals alike. Anyone who has had the privilege of knowing an individual with Rett Syndrome can attest to the fact that the journey into finding what makes a difference for that individual is always one that is worth making.

Information Sheet Home

Information on this sheet contains only suggested guidelines. Each person must be considered individually, and in many cases, a physician's written consent should be obtained.

This webpage created by Amy McBride-Conner, doctoral student in adapted physical education at Texas Woman's University. Please reprint with permission from author at lilrar22@hotmail.com