1. Anxiety in adolescents and adults with Williams Syndrome
Rachel Royston
Dr Jane Waite, Prof. Patricia Howlin & Prof. Chris Oliver
Cerebra Centre for Neurodevelopmental Disorders (CCND)

2. Contents Meet the team What is anxiety?
Why is anxiety more likely in people with WS?
How to spot signs of anxiety
Strategies

3. The team

4. Williams Syndrome Research at the CCND
Three year research project:
Interview study (n=13, mean age=21.9, 8 female)
Online survey study focusing on mental health (n=34, mean age=25.5, 14 male)
Direct assessment anxiety study (n=35, mean age=30.0,
range=13-58, 13 male)
Anxiety in adolescents and adults

5. What is anxiety or worry?
“a feeling of worry, nervousness, or unease about something”
When is it ‘normal’?
Situation that is threatening/difficult
Example: public speaking
Anxiety stops during or after the event
When is it a problem?
Constant anxiety all the time
No reason
Out of proportion to threat of situation

6. General Population
Royston et al., 2016

7. Intellectual disability
General Population
Royston et al., 2016

8. Rare genetic syndromes (e. g
Rare genetic syndromes (e.g. Williams, fragile-X or Cornelia de Lange syndromes
Royston et al., 2016

9. Anxiety in WS – what we know from existing research
48% of individuals with WS may experience clinical levels of anxiety X
Royston et al., 2016 X
Age
Papaeliou et al., 2012
Stinton et al., 2010
Woodruff-Borden et al., 2010

10. Royston et al., 2016 %

11. Royston et al., 2016 %

12. Why do people with WS experience more anxiety?
Genetic vulnerability?
But not everyone with WS experiences anxiety…

13. Risk factors for mental health problems
Age
Health
Sensory processing
Intolerance of uncertainty

14. x Age General anxiety In our sample of 34 adolescents and adults:
Low mood
General anxiety
Hyperactivity
Social difficulties
Obsessive compulsive behaviours
Total mental health difficulties
Intensity of mental health difficulties

15. Health Type of health problem Percentage Dental problems 83%
Stomach difficulties
80%
Cardiovascular problems
Ear problems
60%
Bowel problems
57%

16. Health General anxiety In our sample of 34 adolescents and adults:
Low mood
General anxiety
Hyperactivity
Social difficulties
Obsessive compulsive behaviours
Total mental health difficulties
Intensity of mental health difficulties

17. Sensory processing
Hyperacusis: sensitivity to specific frequencies and volumes of sound
Loud sounds
Every day sounds
Experienced by up to 80% of individuals
(Levitin et al., 2005)

18. Sensory processing General anxiety
In our sample of 34 adolescents and adults:
Low mood
General anxiety
Hyperactivity
Social difficulties
Obsessive compulsive behaviours
Total mental health difficulties
Intensity of mental health difficulties

19. Anxiety triggers – specific phobias
Noise
82.4%
Unpredictable situations
50.0%
Storms
64.7%
Routine changes
55.9%
Upcoming events 52.9%
New or unknown
situations
70.6%

20. Intolerance of uncertainty
Cognitive bias where individuals interpret ambiguous of uncertain situations negatively (Dugas et al., 2005)
Association between an intolerance of uncertainty and anxiety has been identified in autism spectrum disorder(Hodgson, Freeston, Honey & Rodgers, 2017; Wigham, Rodgers, South, McConachie & Freeston, 2015)

21. Intolerance of uncertainty
Individuals with a higher intolerance of uncertainty scored higher on the anxiety scale

22. How can we best manage anxiety?

23. What to look for… seeking proximity more repetitive behaviours
Many signs of anxiety overlap with signs of physical health difficulties.

24. The vicious cycle of anxiety

25. Understanding the triggers for anxiety
Knowledge of the type of anxiety and triggers associated are important
Date/Time
What happened before? Immediate Trigger
How long did it last?
What did the person worry about?
What happened afterwards? How did the person calm down?

26. Overview: Strategies Interventions
Practice labelling emotions and increasing communication
Increase coping
Make life more predictable
Addressing sensory issues
Not all strategies work for everyone

27. Labelling emotions and increasing communication
Knowing you have a way to let people know that something is difficult can reduce anxiety
Card or picture cue to ‘take a break’ or ‘leave the situation’
Avoidance = more likely to experience anxiety in the future: pair this strategy with gradually increasing the amount of time the person spends in feared situation
Having an allocated “worry time”

28. Increasing the person’s coping skills
Self-calming strategies
Listening to calming sounds or music
(Blomberg et al., 2006; Dykens et al., 2005)
Deep breathing
Stress ball
Strategies should be used to help reduce anxiety in difficult situations rather than avoiding the situation

29. Making life more predictable
Ensure predictable pattern to the day
Visual schedules
Social stories
Once the person has a predictable routine it may be possible to gradually introduce more flexibility so that the person can begin to tolerate more variety.
Slowly! Monitor progress.
Use a cue to indicate a change is coming
Not too early as this anticipation can increase anxiety

30. Addressing sensory issues
Example: hypersensitivity to noise, ear pain
Treat physical health problem (i.e. an ear infection)
Strategies: ear defenders for loud noises

31. In summary Higher levels of anxiety are associated with WS
Health problems and sensory processing difficulties may increase the risk of developing mental health problems
There are several things that we can do to try to reduce anxiety
There is still a lot for us to learn!

32. Thank you for listening
Rachel Royston: