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Strategies for Managing Behavior in PWS [2021 CONFERENCE VIDEO]

Dr. Tony Holland discusses strategies for effective behavior management and recent research that shows promise in improving the behavior profile of PWS.

In this one hour and 17‑minute video, Dr. Tony Holland, Emeritus Professor of Psychology, Cambridge University, discusses strategies for effective behavior management in PWS. The session includes Q&A from participants in the 2021 FPWR Virtual Conference.

Click below to watch the video. If you're short on time, scroll down for timestamps to find the portions you're most interested in.

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Presentation Summary With Timestamps

0:48 Dr. Tony Holland presents

Strategies for Managing Anxiety and Behavior in PWS

  • Dr. Holland is a distinguished psychologist with expertise in the area of developmental and intellectual disabilities.
  • He has published extensively in academic and practice‑based journals.
  • In 2020, he was elected a Fellow of the UK Academy of Medical Sciences.
  • Since 2015 he has held an Emeritus position at the University of Cambridge.

2:00 Behavior Management for Families and the Results from the VNS Study

  • The problem with any talk on behavior is it’s complicated, and there are no straightforward solutions and interventions.
  • Interventions may be environmental, psychological or medical, and these interventions are often not directly with the person with PWS, but with those who support them.
  • Dr. Holland offers a conceptual framework for how you might understand the problem.
  • PWS parents and caregivers are the real experts when it comes to your own son or daughter.

3:56 Behavior is a Top Concern for PWS Parents

  • The International Prader‑Willi Syndrome Organization has set up a special mental health initiative, bringing people together from all over the world to try to look at this issue from many different perspectives.

4:33: People with PWS Have Many Strengths

  • Need to keep that in mind because some of the behaviors he discusses will seem negative.
  • Life expectancy and opportunities for a baby born with PWS today is better.
  • Information is key in supporting people with PWS.
  • A young man in our vagus nerve stimulation trial lives in a good, caring environment that understands the needs of people with PWS.
  • His behavior has actually gotten much better since he has had vagus nerve stimulation. 
  • We are making progress and have made progress.

6:18 Terminology and Definitions

  • Mental health and behavior should not be separated. Important to remember that behavior is often how mental health problems can present.
  • What are some of the reasons that classical behaviors such as temper outbursts affect people?
  • Behaviors are maintained as a consequence of an interaction between biological vulnerabilities (brain development) and environmental circumstances.

8:49 World Health Organization Definition of Mental Health

  • More than the “absence of  mental disorder.” It includes our emotional, psychological, and social wellbeing.
    • A dynamic internal state or equilibrium
    • The ability to maintain harmony in line with universal values
    • The ability to recognize, express, and modulate emotions
    • The ability to cope with adverse events and function socially
  • Analogous to physical health: being able to be robust in the things we do.
  • People with PWS do have real problems with some of these things.

9:24 Mental Health and Mental Ill Health

  • The point for understanding the behavioral difficulties experienced by people with PWS is the context in which people live, grow up, develop, and become independent.

10:40 Mental Health and Subjective Wellbeing: Key Dimensions Specific to People with PWS

  • Individual strengths of the person with PWS
  • Healthy weight, physically active—these things matter
  • Living environment and opportunities specific to the needs and wishes of a person with PWS
  • Past and present family, cultural, and social environments
  • Dimensions of mental ill‑health
  • We cannot affect some biological factors, but we can affect environmental
  • Hyperphagia and emotional outbursts and skin picking are dimensional; they are not categorical. It may vary in intensity in the same person over time, and it certainly varies in intensity across groups of people with PWS.

12:49 Dimensions of Mental Ill‑Health Specific to People with PWS

  • Delayed and atypical brain development: genetic subtype differences
  • Cortical and subcortical structures: hypothalamus and the neural and neuroendocrine pathways. 
  • Cognitive, Communication, and Social Functioning (measured)
    • Impairments in intellectual and executive functioning, social cognition, and communication
    • Cognitive inflexibility/rigidity
    • Intellectual Development Disabilities
    • Autistic Spectrum Conditions (ASC)
  • Behaviors of Concern (observed)
    • Hyperphagia
    • Emotional (temper) outbursts
    • Body‑focused self‑harming behaviors (skin picking, etc.)
    • Repetitive and ritualistic behaviors
    • Rigidity and perseveration
  • Abnormalities of Mental State (assessed)
    • Mood instability
    • Situational anxiety
    • Affective disorder
    • Psychotic illness (mUPD>deletion)
  • One of the challenges is to be aware that someone has a problem because they may choose not to tell you or be reluctant to tell you, and that’s where you as a family become very important.
  • You may be the people who can help psychiatrists get inside the head of someone with PWS to get a better understanding of what’s going on.

16:24 Cognition in People with PWS

  • Intellectual functioning distribution of scores around a mean of 60. Del‑UPD differences.
    • Executive functioning
    • Theory of Mind
    • Recognition of emotional expressions
    • Working memory
    • Planning
    • Attention (task) switching
    • Concrete thinking
    • Concept of time
  • Educational attainments
    • Numerical skills
    • Reading comprehension
    • Expressive<receptive language
  • BUT cohort effects are studies from earlier generations.
  • Avg. IQ of general population is 100, Mean IQ in PWS population is 60.
  • One has to be very careful to assume that someone with PWS will have cognitive problems, but it varies from person to person.
  • Some higher order cognitive impairments are hidden, may only be apparent in certain social situations.
  • That’s why formal assessment can be really important.

20:42 Impact of Cognitive Impairments

  • Impacts on learning and level of educational attainments and their maintenance over time.
  • Ability to reliably interpret and make sense of the physical and social environment (study by Friston, et. al). 
  • Affects ability to respond to and make decisions in an efficient and effective way and to respond to change.
  • Particularly in adult life, people with PWS will struggle with this.

22:56 Atypical and Delayed Brain Development in PWS

  • Impaired development of cognitive skills that help them gain an accurate understanding of the world and the ability to respond effectively.
    • Impaired ability to regulate (homeostasis)
      • Food intake to match energy expenditure (hyperphagia)
      • Response to ‘threat’/unexpected situations (emotional outbursts)
      • Mood in response to life circumstances (affective disorders)
      • Temperature in response to environmental change

26:43 Neuropsychiatric Phenotype of PWS (Dimensions of mental‑ill health)

  • Emergence of hyperphagia in early childhood (100%)
  • Repetitive and ritualistic behaviors (50‑60%)
  • Emotional (temper) outbursts (60‑80%)
  • Skin picking (40‑60%)
  • Non‑psychotic mood disorders—Depression, mania, hypermania (15‑20%)
  • Psychotic illness (predominantly in those with mUPD‑60%)

27:50 What Does Research Tell Us? Skin Picking

  • Biological 
    • Impaired perception of bodily sensations (sensory hunger)
    • Increased pain threshold
  • Environmental
    • Particular environmental conditions (lack of activity)
    • Functional analysis‑behavior becomes a way of communicating need
  • Functional analysis showed that people who were ignored or alone were more likely to engage in skin picking.
  • Shows that activity can address skin picking. 
  • When there is a problem, we tend to ask if there is a medicine, but sometimes something in the environment can help it.
  • Skin picking is best understood as an interaction between a biological vulnerability, physical and psychological factors and environmental circumstances.
    • Functional analysis‑behavioral interventions
    • Treatment of co‑morbidity (like depression or mood disorder)
    • Environmental changes
  • Topical treatments to skin to reduce itch
  • Medication to modify glutamatergic pathways in the brain (Jennifer Miller study)
  • Need blind clinical trials to determine if medications are effective

33:25 Abnormal Mental State in 18 Year Old with PWS Due to mUPD

  • Sudden (over hours) deterioration in mental state at a group home
  • Confusion (bewildered)
  • Anxious 
  • Unable to talk coherently‑referring to “blackmail”
    • Seen in A/E‑Diagnosis confusional state (often related to infection) given IV antibiotics
    • Using unrelated words
    • Staring at his hands, grabbing things, hitting, saying strange things
  • Discharged after some improvement, relapsed within hours
  • Diagnosed with atypical psychotic illness and started aripiprazole
  • Mental state improved over 4 days, 2 months later remains on medication
  • This mental illness appears to be more common with mUPD than deletion
  • Suggests causation isn’t related directly to PWS

36:56 Systematic Review of Psychosis in PWS/Mental Illness in People with PWS

  • Presents with a deterioration in behavior and/or the onset of new, bizarre behavior
  • Onset usually acute, but can also be gradual
  • Associated with abnormal mood state and the development of abnormal mental experiences (confusion, hallucinations, delusions)
  • Interventions
    • Medications (carefully given) based on diagnosis
    • Reduce demands
    • Consistent informed support
    • Prevent harm
  • It is treatable

38:38 Severity and Impact of Temper Outbursts

  • Characteristics
    • Can be frequent and severe
    • May result in harm to self and others
    • Impact on welfare of family and caregivers
    • Decreased quality of life and wellbeing
    • Increased financial costs
    • Can involve criminal justice
  • Interventions
    • Applied Behavior Analysis (ABA) (positive behavioral support)
    • Food security
    • Anti‑anxiety, antipsychotic medications
  • Challenges
    • Limited understanding of causation
    • Maintenance of behavioral interventions
    • No evidence for medication — may be a role, but may do harm
    • No effective approved treatment

39:20 Temper Outbursts in PWS

  • Survey of 101 Families in Australia
    • Behavior worse in adolescence
    • Triggered by goal blockage, social injustice (perceived and real)
    • Difficulty dealing with change
  • Interventions
    • Giving space and distraction is the only effective intervention.
    • Medications were being used but families thought they were of limited value.
  • Switching attention, for someone with PWS, is a greater challenge.
  • Temper outbursts often related to unexpected change.
  • Sometimes repetitive questions are used to “buy time,” but usually it has to run its course.
  • Question is can you help people with PWS better cope with change?

41:24 Vagus Nerve and Its Stimulation

  • Autonomic nervous system
    • Sympathetic
    • Parasympathetic (vagus nerve)
  • Vagus nerve‑main link between the brain and the viscera (gut)
    • Visceral function
    • Satiety cascade
    • Impacts immunological function
    • Moderates response to stress
  • Vagus nerve stimulation (VNS) has been used (implanted and transcutaneous devices) in epilepsy, depression, and other disorders.
  • VNS may improve problem behaviors, but in a study of 3 people with PWS it did not affect weight: 1 person gained weight, another showed modest weight loss, and another showed relatively stable weight.
  • Parents and participants did say VNS was transforming behavior.

46:00 Video: Parents and Adults with PWS Reporting on Behavior Changes with VNS

48:26 Transcutaneous Vagus Nerve Stimulation (t‑VNS)

  • In 4 people rates of outbursts decreased, controlled mood, flexibility, opportunity for intervention
  • “Before you couldn’t challenge him on certain things, but now he’s prepared to sit and listen.”—parent
  • Emotional dysregulation (interventions modify autonomic nervous system response to “threat,” eg. psychological, VNS)

51:00 Conceptual Model

  • Biological interventions, like VNS
  • Environmental interventions—optimize understanding, reduce uncertainty
    • Structured timetables
    • Visual support
    • Use of language (avoid irony, etc.)
    • Food security
  • Psychological—manage change, limit outbursts, reduce skin picking
    • Applied Behavioral Analysis (predispose, precipitate, maintain)
    • Early intervention
    • Specific training (preparing for change, managing outbursts)
  • Medical — treat physical and psychiatric co‑morbidities known to respond to treatment
    • Treatment of physical illness like sleep apnea
    • Treatment of psychiatric illness like mood disorders, psychosis

53:06 Beware! 

  • Difficult behaviors may occur for different reasons.
  • When a behavior happens, ask yourself “Is this what we’ve seen before, or is it different?"
  • If it is new or has become worse, ask why?
  • Is there any evidence that the person with PWS might have developed a physical or mental illness?
    • Abnormal mental experiences
    • Altered mood
    • Physical symptoms like vomiting, etc.

53:25 Interventions: The Future‑Behavior and Mental Health

  • Social support
    • Delivery of support in place (family, social support services)
    • Informed and well‑resourced social care
    • Education and opportunities in adult life (UN CRPD)
  • Potential new treatments for hyperphagia, behavior, social functioning (like oxytocin)
  • Personalized medicine, including pharmacogenomics (guide dose of medication) and biomarkers for responders vs. non‑responders
  • Application of neuroscience to PWS
    • Better understanding of atypical brain development
    • Mechanisms underpinning behaviors, cognition, etc.

54: 38 Thanks and Acknowledgment of Funders and Supporters

55:06 Q&A

FPWR Enewsletter

Topics: Research

Susan Hedstrom


Susan Hedstrom is the Executive Director for the Foundation for Prader-Willi Research. Passionate about finding treatments for PWS, Susan joined FPWR in 2009 shortly after her son, Jayden, was diagnosed with Prader-Willi Syndrome. Rather than accepting PWS as it has been defined, Susan has chosen to work with a team of pro-active and tireless individuals to accelerate PWS research in order to change the future of PWS. Inspired by her first FPWR conference and the team of researchers that were working to find answers for the syndrome, she joined the FPWR team in 2010 and led the development of the One SMALL Step walk program. Under Susan’s leadership, over $15 million has been raised for PWS related research.