What is the outlook for persons with Rett syndrome?
Most individuals with Rett syndrome (RTT) are dependent on others to assist them with the activities of daily life. However, that is not to say that some independent skills associated with eating, drinking, toileting and/or dressing, movement, recreation and communication, cannot be learnt.
Communication is particularly important as there is strong desire on their part to interact with those around them, despite their lack of expressive language and impaired hand use. This desire is evident by their eye gaze, facial expressions, and/or body gestures. Females with Rett syndrome have greater receptive language (i.e., what they hear and understand) than expressive language (speech). It is important for them that the activity and/or content of a conversation involves something that they find very stimulating.
Communication methods vary considerably among the Rett syndrome population. Methods can include one or more of the following, eye pointing and blinking, use of yes/no, flash cards, pictures, switches, facilitation, communication boards, head pointers, voice output devices, computers (with touch windows, Intellikeys keyboard, switch activated mouse or eye gaze technology such as Tobii), and more recently, the Ipad. Abilities can also be influenced by such things as the extent of hand use, degree of mobility and/or stiffness, state of posture, level of intelligence and the listener’s appreciation of communication techniques to use, to name just a few.
Education and learning
‘……… Think about Rett syndrome (RTT) as a ladder with a bottom and a top and a lot of rungs in between. Since we don’t know the basic nature or extent of the brain impairment in RTT, why can’t there be varied levels of ability? We see it all the time in other disabilities. We see it in children who learn more typically. Why shouldn’t there be individuals with RTT who have varied abilities?
Some with RTT get a bigger dose (of it) than others and will likely be more severely affected. Some may not make much progress, but some will. It probably has more to do with the “dose” of RTT from the beginning. But how do we know? Can others do the same if we expect them to? If we believed that they could do it? If we looked for evidence of higher thought? In the absence of evidence to the contrary, what if we assume that they can and will make progress rather than assuming progress does not occur? And if we never tried to teach them, how could they ever learn?………’ (Source: Kathy Hunter, The Rett Syndrome Handbook, 2007 page 316).
One group whose mission is to empower parents, educators and therapists with knowledge and strategies to help RTT students reach their highest potential, is Rett University, an introduction to which can be found by clicking on http://rettuniversity.org/ Despite their difficulties, persons with RTT can continue to learn and enjoy family and friends, well into middle age and beyond.
Survival data for the disorder has to be treated with caution as statistics on those actually affected by the condition and the number who die, do not include all such cases from around the world. The Australian Rett Syndrome Study has reported that 98% of Australian Rett syndrome girls born from 1976 onwards, would still be alive at 10 years of age and 75% at 25 years. Life expectancy data concerning an American Rett syndrome population was presented at the 7th World Congress on Rett Syndrome in New Orleans in June 2012 and stated that the odds of living for 10 years was the same as that of the general population of the United States living for 10 years. As for longer terms of survival, it was calculated that 90% of their Rett syndrome population would still be alive at age 20, 75% at age 30, 65% at age 40, and 50% at age 50 years.