July 6, 2001

Keeping In Touch

BYLINE: Carolyn O'Grady

SECTION: RETT SYNDROME; No.4436; Pg.10

LENGTH: 1165 words

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." Sensory/cognitive 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: c-trust@netcomuk.co.uk Web: www.thechildrenstrust.org.uk

RETT SYNDROME

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: rettassoc@aol.com

COMPANY: ST MARGARET'S SCHOOL (81%);

ORGANIZATION: ST MARGARET'S SCHOOL (81%);

SUBJECT: EDUCATION (75%); BRAIN (73%); LEARNING DISABILITIES (70%); FUNDRAISING (70%);

PERSON: GERALDINE NORTHWAY (75%);

LOAD-DATE: July 7, 2001

LANGUAGE: English

PUB-TYPE: Magazine

Copyright 2001 TSL Education Limited SECTION: RETT SYNDROME; No.4436; Pg.11 LENGTH: 738 words Dionne, a 13-year-old pupil at Davenport School in Houghton-le-Spring near Sunderland, has Rett's syndrome. Girls with this inherited disorder often make repetitive writhing movements, but Dionne is calm. She sits in a high wheelchair in the centre of the room, gazing without expression at nothing in particular. Suddenly the room reverberates to a burst of electronic sound from a couple of speakers. Dionne moves her head to one side. The pattern of sound changes. Dionne moves again. Again the sound alters. She begins to smile. She has done this before. She knows that her movements affect the sound; the more she moves, the more it will change. She is in control.

The system responsible for the fluctuating sound is called a Soundbeam, and the man who devised this way of using it is Phil Ellis, professor of performance arts at the University of Sunderland. The key component of the system is a small ultrasound transmitter and receiver mounted on a stand behind Dionne. This device directs a beam of ultrasound at the back of her head, then detects the reflections from it. Each time she moves the reflections change, and these changes are used to modulate the output of an electronic sound synthesiser.

Phil Ellis trained in classical music, taught in a comprehensive school, then moved into higher education to work on electro-acoustic music. He became interested in children with special needs 10 years ago while working in Warwick University's education department. "For a lot of these young people it's the only time in their lives they're in control of anything. Normally they may have no self-control, little self-awareness and no self-help skills at all. Everything has to be done for them. Some have no communication skills. They may be blind or hearing impaired or have epilepsy or cerebral palsy." Although he speaks of what he does as sound "therapy", Phil Ellis admits he only uses the word for want of something more appropriate. "The essence of the approach I've developed is very much non-interventionist." The children are doing it for themselves.

Some children, for example, are plagued by involuntary movements. Once they have learned to associate these movements with changes to the sounds they are hearing, they have an incentive to control the movements.

In a related project Phil Ellis uses sensors attached directly to the body; even tiny movements of the child's muscles affect what is coming through the speakers. "I remember one boy who'd had months of physiotherapy but wouldn't move at all. The first time we put a sensor on his arm he started to move it. After about two months he was using his arm in day-to-day living."

Emotional development is another aspect. Phil Ellis talks of "reaching" pupils, of establishing relationships with them, of seeing them smile for the first time. "Just magic," he says. Cognitive improvement is less dramatic, but he claims to detect signs of greater concentration and engagement. "Time and again you find children who might be able to concentrate on something for just 10 or 15 seconds. They come to sound therapy and after that they're regularly engaged for 10 or 15 minutes."

Class teacher Jackie Magee sees changes in Dionne when she comes back from a session. "She's calmer, less likely to hyperventilate, more aware of what's happening. Like the other children she seems to get a better sense of herself."

The aim is to help able pupils to develop their potential. With the variety of disorders and disabilities at a school such as Davenport, this covers a wide range. Sceptics might wonder whether the benefits of the Soundbeam are as specific as Phil Ellis believes: whether the more general effect of the extra attention paid to the children might not be just as important. He offers two arguments. First, some of the children have already failed to respond to any other form of help; second, most of what goes on is an interaction between the child and the sound, not between the child and the therapist or teacher.

Young people using the Soundbeam normally have weekly sessions lasting from five to 30 minutes. The cost of the set-up is around Pounds 2,700 but the training required is minimal. "One of my aims is to make it possible for anyone to operate this system," says Phil Ellis.

* Soundbeam, e-mail: phil.ellis@sunderland.ac.uk Geoff Watts presents Radio 4's science programme 'Leading Edge'