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)

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

The team

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 - 12+

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

General Population Royston et al., 2016

Intellectual disability General Population Royston et al., 2016

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

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

Royston et al., 2016 %

Royston et al., 2016 %

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

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

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

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

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

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)

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

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%

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)

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

How can we best manage anxiety?

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

The vicious cycle of anxiety

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?

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

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”

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

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

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

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!

Thank you for listening Rachel Royston: rxr180@bham.ac.uk