Like many parents, Patsy Lecont had not heard of Prader-Willi syndrome (PWS) until her daughter, Millie, was diagnosed with the genetic condition which affects one in 25,000 live births.
Millie is now 12 years old, but Ms Lecont still recalls the anxiety of her starting nursery at two and a half years old, as well as the invaluable support that she received from Prader-Willi Syndrome Association (PWSA) UK and an early learning foundation worker who liaised with nursery staff and explained Millie’s needs.
‘Starting nursery is a big deal for any parent, but when it is a child with special needs, it can be really overwhelming and frightening. There is a lot of reassurance to parents if staff know or gain an understanding of the syndrome,’ she says.
PWS is a complex developmental disability that causes a malfunction in the hypothalamus. This area of the brain controls aspects including appetite, temperature and emotion. PWS can be characterised by:
- hyperphagia (uncontrollable hunger)
- hypotonia (low muscle tone)
- hypogonadism (under-developed sex organs)
- cognitive impairment
- challenging behaviour.
These impairments can result in children being delayed in reaching developmental milestones such as sitting unaided, walking, toilet training and speech and language skills. Between the ages of two and seven years old, children with PWS often develop a heightened interest in food which can lead to food-seeking behaviours, being preoccupied by food or taking food without permission. A child with the syndrome can eat three to six times more than other children of the same age and still feel hungry.
Over-eating and low muscle tone can result in obesity, type 2 diabetes, heart failure and respiratory difficulties, so one of the most important parts of caring for a child with PWS is to try to maintain a normal weight. An increased appetite can also bring a heightened risk of choking on food, so carers are advised to practise how to respond to choking incidents.
Children with PWS can tire easily as a result of disturbed sleep patterns, so they may need to have a quiet place to rest or take a nap. They may also have difficulty feeling whether they are hot or cold, so it is advised to ensure that they are dressed appropriately for the weather or activity.
Recently, an increasing number of children with PWS are also being diagnosed on the autistic spectrum.
BUILDING TRUST
‘It is important that nursery staff gain the trust of parents and communicate well with them. They need to let them know the wonderful things that their child is doing as well as what they are finding difficult,’ says Ms Lecont, who is now the support and training team manager at PWSA.
‘In the right environment, many children with PWS are very caring, thoughtful and loving children. Pre-school children may be non-verbal and not very mobile, so it is important to be mindful of their additional needs. It is unusual for children with PWS to demonstrate challenging behaviour at nursery age, but this does come along later. A lot of children with PWS do not fully develop emotionally and socially and their reactions are often childlike, which can start to show more when they move to Reception.’
While Ms Lecont had a very positive experience of Millie attending mainstream nursery, there were some ‘teething problems’, particularly concerning the specialist equipment that Millie required for her poor motor control. She was a late walker and could not sit unaided when she started nursery.
‘The equipment was bulky and took up room,’ she says. ‘I felt that staff were unwilling to accommodate it until the impact of its usefulness was made clear.’ Another issue was snack time, when food was easily accessible and staff had to put boundaries in place to stop Millie from eating too much.
‘Staff have to be very mindful of food and what the child is being given in nursery – don’t think that it’s “just a biscuit” or “just a glass of milk”, because children with PWS need 60 per cent fewer calories than children without and are often on calorie-controlled diets, so one biscuit with 50 calories can take up a lot of their allowance,’ explains Ms Lecont.
She advises that staff tell parents in advance when special events are being held that involve food, such as birthday celebrations or cooking activities, so that they can prepare for them by bringing in alternative treats or adjusting their calorie intake.
Ideally, snacks should be fruit or vegetables and staff should seek advice from parents on portion sizes. Where possible, children with PWS should be supervised around food to prevent food-seeking and promote positive eating habits.
TRANSITIONS
It is recommended that parents of children with PWS apply as early as possible for an Education, Health and Care Plan (EHCP) so that it is hopefully in place for when the child starts school. Moving from nursery to a mainstream primary school can often be when problems arise because of the lower ratios combined with increased needs as the child gets older. Ms Lecont says that it is greatly appreciated if nursery staff are able to support parents through the process because there is often little help available.
‘I advise nursery staff to ask parents how they feel PWS affects their child. This gives them an opportunity to voice their concerns and help paint a picture of how their child functions on a day-to-day basis,’ says Ms Lecont.
‘Millie still remembers her time at nursery and the friendships that she made, especially the children who would push her around in her special needs buggy.’
Case Study: Liam
Liam* started attending nursery when he was 11 months old and was diagnosed with Prader-Willi syndrome. Staff had no previous experience of working with a child with this genetic disorder, so they looked to his parents for advice.
They found Liam to be a very loving boy who made strong bonds with the staff team and enjoyed visiting the different rooms for cuddles. It was when he was two years old that staff noticed that he was particularly drawn to food and would eat all the fruit in the bowl if they did not intervene. At mealtimes, he would grab food from other children’s plates and eat it along with his own.
Liam’s keyperson discussed his hyperphagia with his parents and health professionals. It was decided that although his excessive food consumption was not yet adversely affecting his weight, it was best to limit the amount of food that he had access to both at home and in nursery while still enabling him to have food choices.
The fruit bowl was moved out of reach so that Liam had to ask for it, which also encouraged his delayed communication skills. Staff introduced picture cards at snack times so that he could point to the fruit that he would like. He learned that once he had eaten what was on his plate, it was all gone.
The table that Liam sat at for mealtimes was extended so that he could still socialise with other children while they all ate together but not reach their plates. Staff used positive interaction and distraction with him and encouraged him to leave the table and go to play when he had finished his food.
Liam is a tall and active child so although he ate a lot, his weight remained steady. Physical activities incorporated into the nursery’s daily routine for all children, including ‘Wake up, shake up’ sessions with dancing and action songs, supported Liam to manage his weight and increase his muscle tone.
When it came time to start school, Liam’s parents were anxious about his move to Reception, so his keyperson eased the transition by having visits and transition meetings with the new teaching staff.
*not his real name
MORE INFORMATION
Prader-Willi Syndrome Association UK provides advice, information and training to parents and carers, including nursery and school staff, on supporting children with PWS. Visit www.pwsa.co.uk or call 01332 365676
