Rigidity in routines and the development of resistance to change in individuals with Prader–Willi syndrome

Haig, Elaine; Woodcock, Kate

doi:10.1111/jir.12368

Cited by 8 publications

(4 citation statements)

References 58 publications

(90 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Parents and clinical experts report that people with PWS often have rigid thinking styles and behaviors that interfere with daily functioning. Individuals with PWS commonly show a strong resistance to change [ 17 ]. This difficulty with change of routine and inflexibility in PWS may be linked to a cognitive deficit in task switching [ 56 , 57 ].…”

Section: Rigidity In Pwsmentioning

confidence: 99%

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Schwartz

Caixàs

Dimitropoulos

et al. 2021

J Neurodevelop Disord

View full text Add to dashboard Cite

Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC “Behavior Outcomes Working Group” sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive–compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS.

show abstract

Section: Rigidity In Pwsmentioning

confidence: 99%

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Schwartz

Caixàs

Dimitropoulos

et al. 2021

J Neurodevelop Disord

View full text Add to dashboard Cite

show abstract

“…On the other hand, when inappropriate behavior was noted, the staff empathized with the patient's emotions rather than pointing out the inappropriate behavior, and she was provided with repeated, easy-to-understand explanations on appropriate behavior and sufficient praise when she improved her behavior. In this regard, Haig and Woodcock advised that even if the patient engaged in repeated inappropriate behavior, the staff should take care to continuously display a unified stance [31].…”

Section: Discussionmentioning

confidence: 99%

Behavior Modification Maintenance with Long-Term Blood Glucose and Weight Management in Prader–Willi Syndrome Complicated with Diabetes: Team Management Approach Combined with Pharmacological Treatment

Kato¹,

Kimura

Okuda³

et al. 2019

Case Reports in Medicine

View full text Add to dashboard Cite

The patient was a 40-year-old woman, who had been diagnosed with Prader–Willi syndrome (PWS) at 1 year of age and type 2 diabetes at 27 years of age. At 34 years of age, she was hospitalized to start insulin therapy and receive guidance on treatment. During the next 6 months and through regular once-monthly outpatient clinic visits, the blood glucose level was relatively stabilized although body weight gradually increased. Two years following discharge, the blood glucose level became unstable, and she was hospitalized again to receive guidance on treatment. A team medicine-based approach was established upon hospitalization. The basic treatment was unchanged (insulin, diet, and exercise). The approach taken by the team included understanding the characteristics of PWS by all team members, clear definition of treatment goals, positive evaluation of the patient, and maintenance of the patient’s motivation for treatment. Anxiety and stress related to mother’s illness dampened motivation and adherence to treatment, but the addition of appropriate pharmacological treatment helped in rapid recovery of motivation to adhere to the treatment protocol. At 3 years after discharge, HbA1c is maintained at around 6%, and body weight continues to fall. Our protocol of the combination of a team medicine approach with appropriately timed pharmacological intervention could probably be applied to not only type 2 diabetes in PWS but also the management of patients with poorly controlled type 2 diabetes.

show abstract

“…Furthermore, exposure to more varied environments has been linked to better cognitive exibility in early childhood (Barker et al, 2014). And more rigid routines at this age have been associated with more resistance to change later in life (Haig & Woodcock, 2017). Thus, an approach that supports families to expose children to su cient exibility in routines and environments may constitute an effective early intervention for resistance to change.…”

Section: Introductionmentioning

confidence: 99%

Understanding the Psycho-Social Context for a New Early intervention for Resistance to Change that Aims to Strike a Beneficial Balance Between Structure and Flexibility

Blackwell

Zylberberg

Scerif

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Emotional and behavioural problems linked to changes to expectations – resistance to change – are linked to disability in neurodevelopmental disorders, including autism spectrum disorder (ASD), Prader-Willi (PWS) and fragile X syndromes (FXS). Structuring routines is best practice for minimising current resistance to change. But complete structure is impractical and flexibility in early life may actually reduce later resistance by supporting cognitive development. We aimed to examine the psycho-social context of families with children at risk of developing resistance to change so as to identify design requirements for an intervention that strikes a beneficial balance between structure and flexibility. Methods: Thirty-six caregivers of children aged 4-12 years (17 ASD, 15 PWS, and 4 FXS) took part in an interview designed collaboratively with 12 professional stakeholders. Results: Children need to feel like they are in control of flexibility but they also need support in choice making, understanding plans (using individually tailored visuals) and anxiety reduction. Caregivers need an accessible approach that they have full control over, and which they can tailor for their child. Caregivers also need clear guidance, education and support around structure and flexibility. Conclusions: We propose a digital approach which addresses the needs identified. It tackles the most perplexing challenge by presenting flexibility to children in the context of a game that children can feel they have full control over, whilst caregivers can maintain control in reality. Furthermore, individualised support for children and caregivers would be enabled.

show abstract

Rigidity in routines and the development of resistance to change in individuals with Prader–Willi syndrome

Cited by 8 publications

References 58 publications

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium

Behavior Modification Maintenance with Long-Term Blood Glucose and Weight Management in Prader–Willi Syndrome Complicated with Diabetes: Team Management Approach Combined with Pharmacological Treatment

Understanding the Psycho-Social Context for a New Early intervention for Resistance to Change that Aims to Strike a Beneficial Balance Between Structure and Flexibility

Contact Info

Product

Resources

About