Introduction

All people are different and develop at different rates. You will notice individual personalities and strengths in each person. People living with Prader-Willi syndrome (PWS) are no acceptation. However, PWS is complex. It is often described as a developmental disability that results from changes to chromosome 15. There are changes to the hypothalamus in the brain which controls appetite, temperature, and emotion.

People living with PWS can experience the following:

• Hypotonia (low muscle tone)
• Hypogonadism (underdeveloped sex organs)
• Hyperphagia (uncontrollable hunger)
• Cognitive impairment
• Challenging behaviours

Health professionals that may be involved with this child include:

• Dietician
• Endocrinologist
• Geneticist
• Occupational Therapist
• Ophthalmologist
• Orthopaedic nurse or surgeon
• Orthoptist
• Orthotist
• Paediatrician
• Physiotherapist
• Speech therapist

You may need to seek advice from the above in addition to reading this leaflet.

A child living with PWS is joining your preschool, kindergarten or day program

This article contains information which is appropriate to the age of this child and will help you to support their needs, maintain a safe environment and facilitate learning.

But do remember, if you have any queries, Prader-Willi Syndrome Australia (PWSA) is here to help. We are only a phone call or email away.

Getting to know this child?

Most children living with PWS have an affectionate and friendly disposition. They respond well to humour and are often able to make others laugh. Although they are likely to have some level of learning disability, children with PWS are often skilled when it comes to creative work and puzzles. The child may have limited language but are likely to be socially aware and enjoy the company of other children and adults.

What to expect from this child and how you can best support them

Developmental Milestones

Developing the skill of walking is usually later than we might expect to see in others. It varies from child to child, but usually takes place between 18 months to 3 years of age. The average age for sitting unaided is 12 months. Some infants benefit from the short-term use of a posterior or reverse walking frame. These frames are designed to enable the child to eventually walk out of the device, hence they are reverse designed to most walking frames.

Although not all children have difficulty with speech and language, delayed speech development is common. Vocabulary may be minimal, and the child may stick to certain topics, saying or asking the same thing over and over. The child may receive speech therapy, although it can also be useful to develop strategies to help the child express their needs. This could be in the form of signing such as Key Word Signing Australia or with the use of pictures, symbols, or actions. See PWSA’s article ‘Speech and language’ for further detail. [Add link when available]

Toilet training can be a long and challenging task, and it is important that you do not assume that the child is able to toilet independently. If the child is no longer in nappies, it is likely they will still require support with personal care and hygiene.

Some babies are tube fed due to their low muscle tone. Between the ages of 2 and 7 years the children may develop a heightened interest in food (hyperphagia). This may manifest in food seeking behaviours, taking food without consent or a preoccupation with food. Many children living with PWS enjoy talking about food and need reassurance regarding meals and meal times. See the PWSA article ‘Healthy eating in PWS 2-5 years’. [add link when available]

How can you best support a child with weak muscle tone?

Hypotonia, or weak muscle tone, is present in all children with PWS. Some children are now commencing growth hormone treatment at a very young age which has been shown to improve muscle tone over time. It is important that activities which focus on gross motor skills are used as this can help with core stability and muscle tone.

It is useful if you can incorporate physical activities into your daily routines to manage weight and improve muscle tone. This could be a gentle ‘wake up, shake up’ session or just general play. Be aware that the child may require special equipment such as a standing frame or specialist seating and tire easily.

Information to be gained from Parents/carers

Before the child joins your service, it is important that you gain information from parents/carers about the individual needs of this child. This may include:

• How the child sits and stands – any specialist equipment?
• Does the child have input from a physiotherapist?
• Are they crawling or walking?
• Are they able to grasp objects?
• Does the child have trouble with speech, vision, or hearing?
• Information about diet, as recommended by allied health
• Which professionals does this child see? (Be sure to gain contact details)
• Does the child tolerate touch, messiness on their hands, noise, etc?

We would also advise that you ask parents/carers to describe how they think living with PWS affects their child. This will give them an opportunity to voice their concerns and help to paint a picture of how this child functions on a day-to-day basis.

Adapting routines and environments

You may have some routines in place that would be unsuitable for a child living with PWS, such as rolling snacks or self-service lunches.

They should not have unlimited access to food and will find the availability of this food distracting and at times distressing. We would always suggest that food is only available at set times and is easily portioned.

Ideally, snacks should be in the form of fruit or vegetables or other low-calorie snacks. If you are unsure about portion sizes or food items, always seek advice from parents. Where possible, the child should be supervised around food to prevent food seeking and promote positive eating habits. In young children living with PWS, a verbal reminder is often enough to discourage negative behaviours.

A child with PWS is likely to grow tired throughout the day and may require rest breaks or naps. It is a good idea to ensure you have a quiet space available for this.

Inclusion

Including a young child living with PWS is relatively easy, given that they are sociable and affectionate in nature. As with all children, you should check that activities are appropriate to their ability and check that instructions have been understood. You may need to repeat Instructions, or use signs, or demonstrate. Asking the individual to repeat the instruction back to you can be useful in some instances.

It can sometimes be useful to educate the other children on the needs of this child, with the consent of his/her parents. This may mean using social stories or persona dolls. In doing this, you can reduce the chances of other children sharing food and can create a nurturing, safe environment.

An increasing number of children are receiving dual diagnosis of PWS and autism. If the child is also on the autistic spectrum, they are likely to need extra support in social situations and in play.

Medicines

It is likely that the child will take medication, usually in the form of a growth hormone (GH) injection. GH has positive effects in terms of muscle tone and height and is to be taken daily. It is more than likely that this will be administered in the home, but if you are asked to keep medication on site, please seek advice from the child’s doctor and check the Government policy in your State. Victorian policy can be found here:  https://www2.education.vic.gov.au/pal/medication/policy

Special Medical concerns

There can be health complications in PWS, so it is important to be aware of warning signs that something is wrong:

• If this child vomits or complains of stomach pains, it is imperative that this is taken seriously. Children with PWS are unlikely to vomit at all and doing so may be a sign that something is wrong.
• Due to their high pain threshold, any complaints of pain or discomfort should be taken seriously. Most children living with PWS will only complain of this if the pain is extreme, and even then, the person may only describe it as an ache or discomfort.
• It is important that staff are aware that bruising can occur easily, even resulting from a slight knock or bump. Documenting even small bumps can be a good practice.
• Children with PWS also have poor body thermostats so this child may need extra support regarding appropriate dress and regulating temperature. Many individuals may live with a ‘normal’ body temperature that is slightly below their peers, therefore, even a slight rise in temperature may be a sign of something more serious.
• Some children will skin pick, which is usually a sign of anxiety. Skin picking requires careful monitoring to avoid infection and sores.
• Sticky saliva is also common (a crusting around the mouth) and drinking plenty of fluids can help with the discomfort as well as the difficulties caused by this when swallowing food. Try to avoid sweet drinks (sugar or sweeteners).
• Scoliosis is a common problem which causes a sideways curve to the spine. This can be very mild, requiring little or no treatment or, when more severe it can require bracing or surgery in later years. If the child is wearing a brace, it is important that the back is always kept as straight as possible.

Further information

The PWSA website has articles about positive behaviour support practices, healthcare, care for the eyes, and other advice that might be relevant to your setting.

A PWSA UK video gives more information about children with PWS in a childcare/ kindergarten setting  www.youtube.com/watch?v=wEpfpl6O9xo (30 mins)

Thank you ….

to PWSA UK for sharing their original source material.