People with PWS are extremely stress sensitive and live with high levels of anxiety which can lead to obsessive or compulsive thoughts and behaviours, rigidity, stubbornness, argumentativeness and temper outbursts. This is thought to be due in part to differences in the production of hormones in the brain that regulate emotions. In addition, most individuals have decreased intellectual and executive functioning skills, impaired sensory integration, and slower processing speed – all of which can make understanding the environment more difficult and raise the level of anxiety.
However, each person with PWS is unique and not all people with PWS experience behavioural problems. Behaviour also fluctuates throughout a person’s life and challenging behaviours are more likely to occur during adolescence and early adulthood. The impact on caregivers is also known to be higher at these times. Remember that individuals with PWS are usually friendly, loving and eager to please, but due to the extraordinary challenges they face, they need us to support them by managing their environment and using proactive rather than reactive behaviour management strategies.
Behaviour Management Strategies
1. REMEMBER THE 4C’S: BE CALM, CONSISTENT AND CLEAR – AND AVOID CONFRONTATION
Create a calm environment: People with PWS can be ‘hyper-reactive’. The best environment for someone with PWS may be one where everyone responds calmly and one that is not too busy or noisy. (It is known that some people with PWS may have heightened sensitivities to sound, smell or touch.) Always speak calmly – your tone of voice is often more critical than the words used. Slow down your instructions – remember many people with PWS have a processing delay. Allow plenty of time to transition from one activity to the next.
Use consistent and clear rules and routines: People with PWS have a preference for routine. Visual supports and schedules are very helpful because predictability reduces anxiety. In general, people with PWS are also rule followers. Rules provide understanding for what is expected and how to behave. Rules and routines lower anxiety. If changes to rules and routines are necessary, it is important to provide advance warning and allow extra time for processing these. If the person with PWS has concerns, be sympathetic and check their understanding, but be consistent and do not give in to demands.
People with PWS can appear to have a greater level of understanding than they actually do. Always use clear, literal language and ask questions to check on their understanding about what is happening next and what is required of them. An increase in repetitive questions or perseverating on a topic are signs that anxiety levels are raised, perhaps due to uncertainty or confusion. Answer questions specifically to provide reassurance, but once understanding is checked, limit questions and redirect thoughts.
Avoid confrontation: Try to avoid using “no” and rephrase in the positive instead, e.g. “We can go to the park tomorrow,” rather than, “No, we can’t go to the park now.” When disappointment, anxiety or frustration lead to outbursts or non-compliant behaviour, don’t tell them to calm down or attempt to talk them out of it. Instead, show empathy for their feelings and try to meet them halfway, e.g.“You’re upset. I can see how you might feel it’s unfair.” Offering the person a limited number of choices may also help them to recover a sense of control. But do not engage in a power struggle. Just keep restating and explaining what needs to happen in a clear, low key, matter of fact way, and allow the individual lots of time and space to process and work through their initial oppositional reaction. Ignoring unwanted behaviours as much as possible and responding to escalation in a calm, indifferent manner is helpful because this helps keep anxiety low. This approach takes considerable time and effort on the part of the carer, and may seem as though you’re letting the person get away with behaving poorly at times. It’s worth keeping in mind that people with PWS cannot simply learn to manage their emotions, and most individuals will always need external supports in times of stress.
2. USE POSITIVE BEHAVIOUR MANAGEMENT
People with PWS usually respond well to positive reinforcement of desired behaviour. This may be as simple as verbal praise, or scheduling a less preferred activity and asking for it to be completed prior to a preferred one. It may involve earning rewards using a token system. Punishment does not serve to teach a person with PWS what behaviour is required, and instead usually escalates unwanted behaviours and creates a meltdown.
3. USE THE PRINCIPLES OF FOOD SECURITY
The quality and type of food a person with PWS eats can affect their behaviour. In general, a diet that is low in sugar and processed foods is best. Sweet foods have been shown to be highly rewarding for people with PWS, even more so than a typical person, and may drive obsessive thoughts and behaviours more than other foods. Many individuals do better on a lower carbohydrate diet. However, even with an excellent diet, most people with PWS will always be hungry and a preoccupation with accessing food can lead to anxiety and behavioural issues.
In order to reduce anxiety about how and when food will be obtained, people with PWS need ‘Food Security’. Food Security is a concept coined by PWS specialists Linda Gourash, MD and Janice Forster, MD, from the Pittsburgh Partnership. It consists of the following 3 principles:
NO DOUBT that appropriate foods will be served at the appropriate time
+ NO HOPE or chance that unauthorised foods can be obtained or unplanned extras will be given = NO DISAPPOINTMENT, which means fewer behavioural problems.
Never give in to demands for additional food as this creates hope that it may happen again and will lead to disappointment. Sometimes locking cupboards and the fridge will be required, and some adults with PWS report that they prefer it when food is locked away. Food security is not just about securing access to food. When you have achieved food security, the thoughts of food are also locked away and this frees the person with PWS to think about other things and relax.
Please refer to our Dietary Management page for more information.
A study of the characteristics of autism spectrum disorders in PWS found that many people with PWS have some degree of impairment in sustaining conversations and in the quality and amount of their reciprocal social interactions. In PWS, the ability to form and maintain social connections is affected by the impact of cognitive and processing limitations upon insight, social perception and social skills. It can be additionally compounded by speech and language difficulties, untypical behaviours and a reduced ability to ‘self-monitor’.
However, most people with PWS want and need social interaction. It is common for children with PWS to gravitate toward adults because they will patiently listen and engage with them. Due to their need to make sense of their world and manage their anxieties, people with PWS can present as egocentric in their interests and views, but when you engage with someone with PWS, you will find that their interests are not usually restricted and topics can be extended. Support them by teaching and reinforcing social skills, such as not interrupting, listening, turn-taking, reading non-verbal communication and maintaining an acceptable distance. They may need support to interpret the views, feelings and behaviour of others. Also, it cannot be assumed they will be aware of the expected response in certain situations or understand social appropriateness, so these need to be taught – role play and social stories are useful tools.
Coping with Challenging Behaviour
Sometimes, changes to routine, a miscommunication or other external stressors are unavoidable and people with PWS will experience a ‘meltdown’ where they are unable to control their emotions. Rarely, this will involve aggressive behaviour. Difficulties with expressing feelings can mean that these situations sometimes seem to occur ‘out of the blue’ – they may be the result of anxious thoughts which have been building for some time or over a seemingly insignificant recent event. When a meltdown happens, it’s best to give the individual some space, and remove yourself and others from harm until the meltdown passes. When it does, try to avoid discussing what happened and instead move on with the day. The next day it can be useful to discuss what happened, what the triggers were and what coping strategies could be used next time.
Although behaviour can escalate very quickly, parents and carers will begin to understand what makes an individual anxious and can often recognise the signs of an impending meltdown. In these early stages, preventative steps can be taken and redirection may be possible. Whilst acknowledging their feelings, it is also useful to help them gain a wider understanding of the situation and see other viewpoints. Help them to problem solve. It is important that individuals are taught to recognise their own feelings and use coping strategies, such as slow breathing and counting, using stress relief objects or retreating to a quiet, calm space for rest.
When nothing is working…
It is very common for parents or carers to have a bad patch where they are struggling with their child or client’s behaviour and nothing seems to be working. Thankfully these type of crises are usually temporary, but can be very difficult and exhausting to deal with at the time. Here are some suggestions:
- Give the PWSA(NZ) a call on 0800 4 PWS HELP. It can help to chat to someone else who really gets it. We can just listen or offer advice if asked. We can also visit in person.
- Get professional help. Visit the GP or paediatrician for advice. Ask your NASC about a referral to Explore Specialist Advice service. You can receive one to one professional help to support you in managing behaviour – it doesn’t need to be extreme to get a referral. The waiting list can be long, so don’t delay.
- Consider the use of medications. Making a decision on whether a person with PWS requires medication in order to manage their behaviour is very difficult. This video “When and Why to Consider Psych Meds in PWS” by Dr Elizabeth Roof is an excellent resource. More information on the types of medications used can be found here.
- Are the behaviours very out of character? If this is the case, other causes should be investigated, such as: constipation, tooth pain, recent food binges or accidents. People with PWS have a high pain threshold and often cannot localise pain. People with PWS are also more prone to mental health issues, so it’s important that unusual behaviours be properly evaluated rather than automatically putting them down to ‘PWS behaviour’.
Working through behavioural issues can be exhausting and overwhelming. It’s important for carers to consider their own needs. Talking to someone or taking a break might help. Most families will qualify for carer support and respite via their NASC. Support for carers can also be found through organisations such as Carers NZ and Care Matters.
- Behaviour Management for PWS – PWSA(NZ)
- Anxiety and PWS – PWSA(NZ)
- Food Security – PWSA(NZ) extract from ‘Managing Diet and the Food Environment’
Unique Behavioural Features of Prader-Willi Syndrome
Elizabeth Roof, by PWS Australia, 2017
Food Security The TRAIN Model
Dr J Forster, 3rd Asia-Pacific PWS Conference, 2015
Improving Social Skills in PWS
Elizabeth Roof, by FPWR 2017 (read a summary here)
- Behavioural Features of PWS: Consensus Paper – PWS Clinical Trials Consortium, 2021. Click here for printable summary infographic >
- How Does a Person With PWS Think? – PWSA(USA) from The Gathered View (ISSN 1077-9965), 2014
- The Noncompliant, Oppositional and Hyper-RE-active Child – Drs Gourash and Forster, The Pittsburgh Partnership, 2015
- Behaviour Management Skills 101 – Dr Linda Gourash, Pittsburgh Partnership, 2013
- Principles of PWS Behaviour Management – K Beaver, L Graziano, PWSA(USA) 2012
- Strength in Boundaries – Famcare, a project of IPWSO
- Coping with Change – Famcare, a project of IPWSO
- PWS Behaviour Management Strategies Tool Kit – Prader-Willi Calfornia Foundation
- Strategies for Improving Behaviour in Persons with Prader-Willi Syndrome – Do’s and Do Not’s – PWCF
- Supporting Challenging Behaviours in Persons with Prader-Willi Syndrome – Paul White, Behaviour Specialist, for PWCF
- Managing a Meltdown – Famcare, a project of IPWSO
- Anatomy of a Tantrum – Pittsburgh Partnership, 2008
- Aggressive or Violent Behaviour in PWS – PWSA(UK)
- Skin Picking – click to find information under S in our Medical A-Z
- Confabulation (fabricated, distorted, or misinterpreted memories) – The IPWSO Blog, Sept 2014
- Food Security Checklists – The Pittsburgh Partnership
- What’s Wrong with Food Rewards in PWS? – Drs Gourash and Forster, Pittsburgh Partnership, 2015
- Examples of Tools for Monitoring and Managing Feelings, plus Examples of Calm Down Strategies and Tools, i.e. what to put in a calm down box (Note: the food suggestions are not recommended) – PWSA(NZ) Pinterest page
- Sensory Integration / Sensory Motor Issues in PWS – VIDEO: Dr J Forster, 3rd Asia Pacific PWS Conference, 2015
- Anxiety – VIDEO: Elizabeth Roof, FPWR Canada Conference 2013
- Behaviour and Mental Health in Prader-Willi Syndrome – VIDEO by PWSAI & IPWSO for families featuring Professor Tony Holland, 2014