Prader-Willi Syndrome
Prader-Willi Syndrome (PWS) was first described in 1956 by Swiss doctors Prof. A. Prader and Dr H. Willi, who recognised the condition as having unique and clearly definable features. These features include hypotonia or weak muscle tone, immature development of sexual organs and central nervous system and endocrine gland dysfunction. This causes varying degrees of learning disability, short stature and poor social and emotional development.
Many people with PWS also exhibit certain facial characteristics and have poor large muscle strength, which is often coupled with poor co-ordination and balance. This can be improved with the appropriate physical therapy and exercise. The common factor amongst all those with PWS is borderline or moderate learning difficulties, with the average IQ being around 70 with poorly developed social and emotional skills. Reading and writing skills are usually considerably better than analytical problem solving skills such as Mathematics and abstract thinking processes.
What causes Prader-Willi?
PWS is caused by an abnormality on chromosome 15, with the majority of cases caused by a deletion on the chromosome 15 inherited from the father. A small minority of people have a translocation or imprinting irregularity involving chromosome 15. The condition is not an inherited one and is currently thought to be a purely accidental occurrence. PWS-like symptoms may also be ‘acquired’ by damage to the hypothalamus during a person’s life or from a dysfunction of the hypothalamus. This may be the result of a head injury or from a dysfunction from birth. In these cases, sufferers will not show the physical effects of the condition but may acquire some or all of the behavioural problems associated with the syndrome. One of the most common symptoms is an obsession with food. If this is not to lead to obesity, a strict control needs to be maintained on the sufferer’s food intake, as because of a reduced requirement for calorie intake due to weak muscle tone, overeating can quickly lead to further health problems.
The symptoms of Prader-Willi in children
In early childhood, children with Prader-Willi Syndrome will develop an extreme interest in food and in severe cases develop what can appear to be an insatiable appetite. This can manifest in how the child plays, with a preoccupation with food in play and talk. Children with PWS are generally placid and friendly but may begin to display stubborn or obsessive behaviour and outbursts of temper if they cannot get their own way or are denied access to food. This can be controlled using a strict dietary regime. Cognitive Behavioural therapy and Verbal Behaviour can direct the child’s attention onto more constructive pursuits. Children with PWS often excel at sedentary activities such as jigsaw puzzles, drawing and colouring and by refocusing the child’s attention in these areas, obsessive-compulsive eating patterns can be controlled more easily.
How is PWS treated?
Because of the effect on the physical development of the child, growth hormone replacement therapy is commonly used to improve stature, muscle tone and strength. As yet, no drug therapy has been effective as an appetite suppressant, but this can be controlled using good dietary management and exercise programmes. The prevention of further complications in behavioural patterns (such as obsessive compulsive behaviour, anxiety, aggression and communication skills) can be addressed at an early stage through the use of Verbal Behaviour. This encourages the learner to express themselves in a more socially interactive way and minimising the possibility of aggressive outbursts.
At VBC, we have a team of experts dedicated to the development of effective social and communicative skills using both Verbal Behaviour and Cognitive Behaviour therapies. If started at an early stage in the child’s development, these can encourage a developmental process that is comparable with children of typical capabilities. If you would like to know more about how we can help both you and your child,
contact us in confidence for further details.