Prader-Willi Syndrome: A Primer for Teachers

Prader-Willi syndrome is a genetic disorder. Specifically it is caused by the lack of expression of genes at 15q11–q13 (Haig and Wharton 2003). It was first noted in 1956 by Prader, Willi, Labhart, Ziegler and Fanconi.

The characteristics of Prader-Willi syndrome include hypotonia, obesity, hypogonadism, short stature, small hands and feet, mental deficiency and a characteristic face (Butler 2005).

The PWSA (UK) states that:

Babies with PWS are very floppy at birth, and the ability to suck is weak or absent. Tube-feeding may be required for the first few days or weeks of life; breast-feeding is rarely initially successful. Babies show little interest in feeding during the first few months of their lives, have a very weak cry, and sleep for most of the time during the early weeks. Male babies may have noticeably underdeveloped genital organs. Female babies may also have underdeveloped genitalia, but this is much harder to detect. Developmental milestones such as sitting, standing, walking and talking are generally delayed, but most children with PWS are able to attain all these abilities by the time they are about 5 years old. Infants with PWS are very lovable and placid, and seem to draw admiration wherever they go.

Some time between the ages of one and four years, children with PWS begin to show a heightened interest in food and in severe cases develop what appears to be an insatiable appetite, so that they will try to obtain food by any means possible. The degree to which this occurs varies considerably between individuals, but there is always a preoccupation with food in play and talk. This can be helped by good management, dietary control and educating the child about his or her diet. However, if energy intake has not been carefully monitored, weight gain is rapid; hence, a lower calorie diet (energy controlled regime) is essential throughout the person's life. An appropriate exercise programme helps to keep weight levels down and also improves muscle strength. If weight is not controlled, fat accumulates in a characteristic way on the buttocks, stomach, lower trunk and thighs. Children with PWS are generally placid and friendly, but may begin to exhibit stubborn or obsessive behaviour, and outbursts of temper if they cannot get their own way or are denied access to food.

The video posted below, developed by the PWSA (USA), gives some useful tips for teachers:

References:

Butler, M. (1990). Prader-Willi syndrome: Current understanding of cause and diagnosis American Journal of Medical Genetics, 35 (3), 319-332 DOI: 10.1002/ajmg.1320350306



Haig D, & Wharton R (2003). Prader-Willi syndrome and the evolution of human childhood. American journal of human biology : the official journal of the Human Biology Council, 15 (3), 320-9 PMID: 12704708