Rett Syndrome
Rett syndrome is a disorder of the nervous system that leads to developmental reversals, especially in the areas of expressive language and hand use. It is characterised by normal early development followed by loss of purposeful use of the hands, distinctive hand movements, slowed brain and head growth, gait abnormalities, seizures and mental retardation. It affects females almost exclusively.
The disorder was diagnosed by Dr. Andreas Rett in 1966. It was not until a second article about the disorder was published in 1983 that the condition was generally recognised. The condition is caused by mutations, structural alterations or defects in the MECP2 gene, found on the X chromosome. This gene was identified in 1999 and contains instructions for the synthesis of a protein that acts as one of the many biochemical switches that tells other genes when to turn off and stop producing their own, unique proteins during the child’s developmental stages. Although Rett Syndrome is a genetic disorder, less than 1% of recorded cases are inherited or passed from one generation to the next.
What are the effects of Rett Syndrome?
There are four stages of Rett Syndrome. Early onset, the first stage of the condition, usually occurs in infants between six to 18 months of age. The child may show fewer signs of eye contact and have a delay in the development of motor skills such as sitting or crawling. Stage two, known somewhat alarmingly as the rapid destructive stage, usually manifests between the ages of one and four years. This may be either rapid or gradual as purposeful hand skills and language skills are lost. Some girls may also display autistic-like symptoms such as a loss of social interaction and communication skills. Slowing of head growth is more pronounced and noticeable at this stage.
Stage three is known as the plateau or pseudo-stationary stage and is apparent between the ages of two and 10 years. Although there may be an increase in motor problems and seizures at this stage, there may also be an improvement in behaviour, with less irritability, crying or autistic-like symptoms. At this stage, the individual may show more interest in their surroundings and their alertness, attention span and communication skills may improve. It is at this stage where intervention using Verbal Behaviour is of most benefit. If Verbal Behaviour is used prior to this stage, we are able to teach family members and children to develop effective behaviour, communication and learning patterns which are able to be built on, thus enhancing progress.
The last stage of the condition, called the late motor deterioration stage, may last for years and is characterised amongst other symptoms by reduced mobility, muscle weakness, stiffness and curvature of the spine. However, there is no general decline in cognitive ability, communication or hand skills.
How can Verbal Behaviour help?
Because of the direct effect on language and communication skills suffered by those with Rett Syndrome, VB can help by minimising the loss of communication skills suffered during stage three in particular. At this point, a therapist can use the higher abilities in alertness, attention span and communication skills to develop the learner’s language skills at a comparative rate to a child with no disabilities. This maintains the child’s levels of communication, ensuring that they do not become anxious or frustrated through the loss of the ability to effectively make themselves understood. As with all language difficulties, a degeneration of communication skills may lead to feelings of isolation within social peer groups, alienation and anxiety. If these can be avoided by ensuring that the child can communicate with family and friends effectively, the impact of the development of further psychological issues can be minimised.
At VBC, we understand how frustrating it can be for children with limited linguistic skills to communicate effectively and how this can impact on other forms of behaviour. By incorporating a balanced Verbal Behaviour programme, often together with other recommended therapies, those barriers can be removed and many children show remarkable progress in a very short space of time, achieving their potential and often excelling expectations. If you would like to know more about our ground-breaking therapy treatment plans and the network of support we put into place for the families of children with Rett Syndrome,
contact us in confidence for further details.