This article contains information appropriate to secondary school age students living with Prader-Willi syndrome (PWS). It will help you to manage and support their needs, maintain a safe environment, and facilitate learning. But do remember, if you have any queries, we are here to help. We are only a phone call or email away. Training is available.

Introduction

A student living with PWS is joining your school. Each person is an individual and will develop at their own pace, however, understanding some of the common characteristics of the syndrome may assist you in teaching and supporting your new student.

Please remember that PWS is just a part of your pupil. They will have similarities and differences, as all other children do. Many people with PWS have specific and valuable attributes and skills. Many have excellent reading and writing skills as well as fine motor skills and are particularly clever with jigsaw puzzles and fine handiwork. They often have excellent long-term memory and visual processing skills.

PWS is a complex developmental disability that results from genetic variations on chromosome 15. It causes changes in an area of the brain called the hypothalamus, which controls aspects such as appetite, temperature, and emotion.

Other characteristics can be:

• Hypotonia (low muscle tone), if not receiving growth hormone treatment
• Hypogonadism (underdeveloped sex organs)
• Hyperphagia (uncontrollable hunger)
• Cognitive impairment or delay
• Challenging behaviours
• Anxiety

Health professionals that may be involved with this student include:

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

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

Quick tips for behavioural support

• Use positive language
• Keep verbal responses to a minimum during behaviour incidents
• Use humour where possible
• Appear disinterested in negative behaviour
• Be consistent with your responses as a whole staff team
• Use a structured discipline procedure
• Work collaboratively with parents/carers

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

Learning challenges

Many people living with PWS experience some level of learning disability. The level of which varies from one person to the next. Please do be aware that even if the student presents as able, there may be underlying issues regarding comprehension and cognitive ability. It is common for children and teenagers living with PWS to face challenges in expressive language use and short-term memory.

What can you do to help?

Ensure instructions are repeated and ask the child to repeat instructions back to you. Check understanding and include ‘refreshers’, revisit previous work regularly.

Dietary support and food seeking behaviours

Food seeking behaviours are common in people living with PWS and the pupil is likely to have lots of anxiety surrounding food. These types of behaviour are a result of the damage to the hypothalamus, meaning the child is unable to experience the sensation of fullness, known as hyperphagia. It is very unlikely that your student with PWS would be able to make informed or safe decisions around food, particularly when left unsupported.

What can you do to help?

It is important that all staff are aware and familiar with the person’s dietary needs and food security practices. It can sometimes be helpful to make other pupils aware of this too, providing you have parental permission. Anxiety can be reduced if food is kept out of sight and some form of food ‘security’ or food ‘barriers’ in place.

• Food security is a state of mind – the pupil known what food they will be getting, when they will eat, and that they will not be getting any extra food. It is advised that you discuss any food within the classroom with the pupil’s parents/carers beforehand and that any food consumption for special occasions is arranged with them in advance. This can allow parents to ‘borrow’ calories from their evening meal. The pupil will need reassurance of his/her food routine which should include fixed times.
• Food barriers might include keeping food storage areas locked, clearing empty plates away immediately after eating or ensuring lunch boxes are not left unattended.

Emotional support

Some people living with PWS have difficulty in controlling and understanding their emotions. You may find that emotional outbursts occur when the student feels anxious, frustrated, confused or that the situation is out of their control.

What can you do to help?

Ensure staff work together to reduce anxiety and to create positive and safe working environments. This means consistently implementing any plans that the student may have in place, for example from a psychologist. Reassure the student where possible and focus on achievements and goals with lots of positive reaffirmation.

Anxiety

Most individuals experience some form of anxiety a lot of the time. Anxiety levels can increase further when the student is faced with changes to their routine or when they are unable to predict the near future. At times, you may be able to see the anxiety growing with behaviours such as skin picking or fidgeting.

What can you do to help?

Ensure lines of communication between other staff and parents/carers are open to provide consistency and reassurance. It is important that the student has been kept informed and knows what to expect. Don’t assume that they have absorbed class instructions like others.

Be clear about rules and routines and try not to deviate from these. If a change occurs that is unavoidable, inform the student as early as possible and be prepared to confirm these changes as often as needed.

Perseveration or repeated questioning

Repeated questioning is a common feature, which usually occurs when the person requires reassurance or if they have been unable to process the information given. On occasions, the student may use repeated questioning to gain 1:1 attention and verbal responses.

What can you do to help?

Agree to answer the question just 3 times. On the 3rd occasion, ask them to repeat your answer back and confirm they have understood your response. If repetition persists, try reducing verbal responses by shaking/nodding your head, or using visual reminders which they can refer to, e.g., if the question is “what time is lunch?” try drawing a clock face on a paper plate to indicate your answer.

Time management

Some individuals struggle to move on from one activity to another. You may find they are reluctant to move on until the task is completed. Most can focus more effectively on several consecutive smaller tasks, as opposed to one large, on-going project.

What can you do to help?

Provide short activities and be clear on time frames or when you expect it to be completed. Ensure a gentle reminder is given regarding how much time is remaining.

If the student insists, they cannot move on until the task is complete, try issuing an “extension card”, enabling them to borrow time which they can cash in later to complete the task.

Day time sleepiness

Disturbed night-time sleeping patterns are common. It is likely that this student may grow tired during the day and may require rest breaks.

What can you do to help?

Ensure you have a quiet space available where the person can go to rest. It may be that the individual requires a short nap (approx. 10 mins). With some students, this may be required more than once a day.

Rewards

Positive behaviour should always be recognised or rewarded as people with PWS respond well to praise. They are also likely to enjoy responsibility and being told they’ve “done a good job with that ….”. Doing this can boost their self-esteem and confidence.

What can you do to help?

Most importantly, food should never be used as a reward for a person living with PWS. For the younger student, it can be useful to record achievements and praise on a graph or star chart. You can make this available for the person to refer to and the visual representation will make this accessible to them. When rewarding the whole class with food items, ensure you have consulted parents beforehand. They may provide a low-calorie alternative or plan for the calories to be borrowed from their evening meal.

What can make behaviour worse?

Several factors can make these behaviours worse, such as:

• Major changes in daily routine or environment
• Changes in diet or meal routine
• Conflicts of opinion amongst others in close relationship to the person with PWS
• Unexpected happenings or upsets
• Major life stresses such as bullying, teasing, bereavement, moving residence, changing schools etc
• Low mood

Daily, very minor occurrences can affect behaviour. For example:

• Proximity to meal times
• Tiredness
• Minor change to routine
• Change in room or air temperature
• Mild teasing
• The teenager being unable to get their own way
• “Trigger” words or situations which are individual to the person

The right approach

The attitudes of other people are very important.

Behaviours likely to have a negative effect on a teenager living with PWS are:

• Aggressive attitudes and tones of voice
• A confrontational approach
• Being overly sympathetic
• Displaying dislike of the person
• Not talking to the person
• Talking to the person too much
• Conflicting with another person in authority – both giving different rules to the teenager with PWS or arguing in front of him or her.
• Saying “Don’t” or “No” as a means of trying to stop him doing something.

Likely to have a positive effect are:

• Firm, but loving and caring attitudes
• Ability to keep a sense of humour, and to see and exploit the funny side of a situation
• Ability to give clear guidelines and boundaries and stick to them
• Praise, and stressing the positive aspects of the person
• Saying “Do’s” as a way of showing him how to do something correctly, e.g., instead of saying “Don’t leave the door open”, say “Please shut the door”.

Remember: Let the student know what’s expected of them in a situation, beforehand, even it seems obvious.

Your school environment

Many potential outbursts can be avoided by providing the right environment to support your pupils needs. This might include:

• A structured day, with plenty of advance warning if changes are planned
• Unpressurised tasks or expectations. For example, don’t expect them to be ready to go out in five minutes if it normally takes them 20 minutes – give them plenty of warning, possibly using a “countdown” system, with a reminder at 15 minutes, then at 10 minutes, and so on. This is particularly important for those with higher intellectual levels who may be placed under greater pressure because more is expected of them, but which they are often unable to deliver in the time space allowed. This includes educational tasks.
• Ensuring that all who encounter the pupil are giving the same message about what they should expect in the way of behaviour.

Change Ahead

• Help them to learn techniques to keep calm such as deep breathing, listening to music, using worry beads or stress balls
• “Now” and “Then” visuals can be useful
• Put up notices and pictures to remind them to “keep calm” and/or timetabling what happens when during the day
• It may be useful to discuss in advance with him or her situations they find difficult and how to cope with the situation
• If the pupil finds it difficult to be around food (most, but not all, do), remove food from sight, do not allow others to eat in front of him or her and, if necessary, lock food away.

Finding the right level of independence

Most teenagers living with PWS respond well to responsibility and the concept of independence. It is likely that your pupil will be keen to learn independent living skills.

However, it is worth bearing in mind that very few will have the capacity to live independently in adult life (most living at home with their family or in supported independent living homes.

Finding a level of independence whilst maintaining a safe environment can be a difficult balance, particularly around food. If the pupil takes part in a Life Skills course, try to focus the food preparation modules on Healthy Eating, or even Living with PWS. Speak to parents about their plans for the future, or what they hope their son/daughter might achieve. This can avoid setting unrealistic expectations or setting the pupil up for disappointment later in life.

Supporting your pupil

Most people living with PWS will have a National Disability Insurance Scheme (NDIS) Plan. The Plan has funding to purchase ‘reasonable and necessary’ supports to pursue their goals. In addition to the supports that may be possible through their NDIS Plan, we would always recommend some form of support during social times and when around food to avoid high levels of anxiety or food seeking behaviours.

Students with PWS respond well to 1:1 support. If you already have support in place, bear in mind that it can be very easy for the student to become attached to this person, particularly if they are together for the entire school day. You may notice some obsessive behaviours surrounding this person. Whilst the consistency and reassurance of a familiar face can be beneficial, it is important to avoid the pupil taking ‘ownership’ of their support worker or teacher’s assistant (TA). This can be easily overcome by the TA working with various pupils in the classroom, showing that he/she is not there solely for the pupil.

It is common for these teens to be fond of adult company and the person may need some support in socialising with peers. Encouraging them to take part in school clubs or activities is beneficial in aiding their social development. You may find that the student will still require the support of a TA during these times, and it can often be useful to provide a quiet, ‘safe’ place if they become overwhelmed.

Sometimes the assistance of a Speech Pathologist or the development of a Positive Behavioural Support programme by a Behavioural Support Practitioner with knowledge of PWS, can help with social skills and relationship skills. You may notice the pupil struggles with conversational skills such as turn taking, waiting for others to finish speaking or listening to others. In cases such as these, it can be useful to play turn taking games such as board games or role-playing games.

It is important to listen carefully to the parent(s) of the student as they will understand their individual needs and idiosyncrasies better than anyone else. It may be appropriate to have more regular meetings with the parents/carers than with other parents. A diary for the teacher to record notable events throughout the day may also benefit both parent and teacher.

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 your pupil vomits it is imperative that this is taken seriously. People 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 also be taken seriously. Most people 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 all staff are aware that bruising can occur easily, even as a result of a slight knock or bump.
• Research is indicating that most people living with PWS have a normal temperature a little below their peers. They also have an altered body thermostat, so this child may need extra support regarding appropriate dress and to regulate their temperature.
• Some children with PWS 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 and a crusting around the mouth may be visible. Drinking plenty of fluids can help with the discomfort as well as the difficulties caused by this when swallowing food.
• Scoliosis may be present, which causes a sideways curve to the spine. This can be very mild, requiring little or no treatment or, when more severe, can require bracing or surgery. If the child is wearing a brace, it is important that the back is always kept as straight as possible.
• Please do not assume that the student is able to attend to their own personal care or toileting. If you are unsure, his/her parents/carer will be able to direct you.
• It is possible that the person may take regular medication. If you are asked to keep medication on site, please seek advice from the person’s doctor or check the requirements in your State, e.g., Victoria has a set of procedures around medication at school: https://www2.education.vic.gov.au/pal/medication/policy

Inclusion

Including a student is relatively easy, given that they are generally sociable in nature. As with all students, you should ensure activities are appropriate to their ability and check that instructions have been understood. You may need to repeat instructions, demonstrate activities, or use signs or pictures to reinforce.

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

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

Other Resources

Students living with Prader-Willi Syndrome (PWS) like to do their best. They work well with educators, but have special needs in their learning style. You will have a keen and happy student if the strategies and hints below are adopted.

• Tips For Educators of Students with Prader-Willi Syndrome, leaflet about behaviour
• Learning with Prader-Willi Syndrome (65 mins) Australian video by a school teacher about educational techniques for students with PWS.
• Key Components for Success with a Child with Prader-Willi syndrome in a School Setting
• Teaching students with PWS (video) (27 mins) Helpful information and practical strategies for teachers who have students with Prader-Willi syndrome, in their classroom (USA).
• Prader-Willi Syndrome in the classroom NZ booklet – addresses the unique needs of the student with PWS, and gives practical management strategies for teacher and student.
• PWSA resource for the staff room ‘Medical Alert checklist for schools’. [add link when available]
• Presentation for Secondary School Staff (youtube.com) (35 mins) PWSA UK video

Thank you ….

to PWSA UK for sharing their original source material.