Rett syndrome is a severely limiting condition but carefully planned activities and sensory experiences can greatly enhance achievement. Carolyn O'Grady sees the principle in action
Mary looks at you with an expression full of interest and a gaze that is firm. So despite the fact that she is in a standing frame to keep her upright because she cannot stand by herself, and she is fed through a tube, it is hard to believe her comprehension is severely limited and she cannot speak.
Mary, who is 11 years old, has Rett syndrome. She is a pupil at St Margaret's School in Tadworth, Surrey, which is fast acquiring a reputation for expertise in helping young people with this particularly difficult and complicated condition found only in girls.
An independent residential school which takes 40 pupils aged between eight and 19 mainly from the south-east of England, St Margaret's is part of the Children's Trust and is based on the Trust's site in Tadworth. Apart from the school, the charity's other services include intensive rehabilitation for children with acquired brain injury; respite care, and an outreach service for those who live at home. Pupils at St Margaret's are funded by different LEAs, but capital developments are mainly paid for through fund raising.
All the pupils have profound and multiple learning difficulties and often degenerative conditions and complex medical needs. Because of this, they all require a particularly sensitive approach to their emotional, physical and medical needs. Twenty-five per cent have Rett syndrome. In their case comprehension is more developed than their ability to express themselves, which can lead to frustration and anger; feeding can be difficult and a lot of physical therapy is required to combat increasing stiffness and curvature of the spine.
"We want to keep them on their feet as long as possible," says the curriculum manager, Geraldine Northway, pointing out the numerous standing frames and other special devices arrayed around the school. These enable the girls to remain comfortably standing while doing a variety of activities such as cooking, listening to music, eating or communicating with teachers.
Jan Cunningham vigorously defends the school's policy of disapplying all children from the national curriculum: "We believe that the children are entitled to something designed for them individually, not a curriculum into which we have to slot them. The emphasis is on an integrated 24-hour curriculum which embraces all aspects of development rather than subject areas." Therapies and education are delivered to six children in each classroom by multidisciplinary teams, which include a member of the care staff from the residential houses to make sure that the methods used in the school are continued there. "We aim for a seamless approach," says Jan Cunningham. Teams work to each child's Individual Education Plan, which details simple and achievable targets. Left to their own devices girls with Rett syndrome can be very passive. "You have to bring experiences to them. In a cookery session, for example, the children will be put into the right position for that activity and encouraged to look, listen, to hold on to something, to taste and smell.
"We're not teaching them to cook or paint," says Geraldine Northway. "We're giving them experience of these things and within those activities working on their IEP." Sensorycognitive development, communication development, social development, motor skills and life skills development will all be monitored and tasks set to help progress along.
Physiotherapists, music therapists and other specialists work with the children in the classrooms and residential houses. So a target set for an individual child in the classroom will also be pursued in the residential houses. Jane, for example, is being encouraged to use her hands more purposefully, rather than engaging in the constant hand wringing and patting characteristic of girls with Rett syndrome. In the classroom in water play she is helped to splash the water and use her hand in other ways, and this might be developed later in her bath.
"Consistency, routine and attention to detail are our strengths," says Geraldine Northway, drawing attention to the system of "touch cues" used throughout the school and in the residential homes. These prepare the child for the fact that something is about to happen - children with Rett syndrome and other disorders often do not like things to happen suddenly and unexpectedly. A touch next to the eye, for example, is a signal to look; a touch next to the mouth prepares them for receiving a drink.
Most of the girls with Rett syndrome and many of the other pupils at St Margaret's will go on to residential adult placements when they leave the school. "Most parents say they want a replica of St Margaret's," says Geraldine Northway, "but it's not easy to find the same sort of intensive therapy and unfortunately any skills they haven't learned by the time they leave here they probably won't learn. We hope the skills they have learned will be maintained for the rest of their lives."
* Further information can be obtained from Jan Cunningham, St Margaret's School, Tadworth Court, Tadworth, Surrey KT20 5RU. Tel: 01737 365811. E-mail: firstname.lastname@example.org Web: www.thechildrenstrust.org.uk
Rett syndrome, first described by Andreas Rett in 1966, is a profoundly disabling neurological disorder which may affect one in 10,000 females in the population. The syndrome has variants, but typically the girls appear normal at birth and in their early months. At about age one their development slows, and at roughly one to three years of age they lose speech and to some extent the use of their hands. They also withdraw socially, begin to have great difficulty in co-ordinating movement and show a jerky gait, involuntary movements, poor balance and tremors. Only about half of those affected learn to walk independently. Eating problems are common, including difficulties in chewing and swallowing. Other symptoms include repetitive hand movements; grinding of teeth and breathing abnormalities such as breath-holding and swallowing of air. Epilepsy is common.
After the initial period of rapid loss of skills the situation stabilises at around eight years of age and the girls may regain some of their original skills. There may also be better eye contact and improved memorising and non-verbal communication.
In the third stage physical disabilities tend to increase, with muscle wasting, foot deformities, curvature of the spine (by the age of 18, 70 per cent of the girls have some sort of spinal deformity) and increasing stiffness. Previous walkers may now find themselves unable to walk. Many affected girls survive well into adulthood.
Further information: Rett Syndrome Association UK, 113 Friern Barnet Road, London N11 3EU. Tel: 020 8361 5161. E-mail: email@example.com