Is food restriction in client with Prader-Willi syndrome considered abuse?

By

ACKNOWLEDGEMENT

I would take this opportunity to thank my research supervisor, family and friends for their support and guidance without which this research would not have been possible.

DECLARATION

I, [type your full first names and surname here], declare that the contents of this dissertation/thesis represent my own unaided work, and that the dissertation/thesis has not previously been submitted for academic examination towards any qualification. Furthermore, it represents my own opinions and not necessarily those of the University.

Signed __________________ Date _________________

ABSTRACT

This paper aims to explore the management of a rare chromosome disorder, PradereWilli syndrome (PWS), within families. It is particularly concerned with developing an understanding of the management of diet and other everyday practices affecting the body. People with PWS tend to experience poor muscle tone combined with food obsession. The level of control over diet needed to prevent obesity and related health complications is often lacking as individuals also experience various forms of learning disability, autistic spectrum disorders and behavioral problems. The findings are based on data from twenty qualitative case studies of English families with a young person with PWS. Analysis of management strategies highlights the centrality of embodied agency in shaping everyday practices and interactions. The significance and influence of biology within this process is particularly evident, as people with the genetic condition PWS experience embodiment and emotion in distinct ways and differently from non-PWS family members in the research sample. Focusing on the multidimensional nature of processes surrounding body management, the paper highlights three key management practices and explores how these practices are influenced by people with PWS and interpreted by family members. Three key practices are identified as: restricting access to food, keeping occupied, and use of routine. The study represents the first UK empirical sociological study of PWS and primarily adds an insight of family management of PWS to a medically dominated literature around the disorder. The findings can sensitize health and social care professionals to some potential issues for families managing PWS, and guide and develop appropriate interventions to support young people with PWS and their careers.

TABLE OF CONTENTS

ACKNOWLEDGEMENTII

DECLARATIONIII

ABSTRACTIV

CHAPTER 1: INTRODUCTION1

Introduction1

Background1

Research Aims and Objectives2

What is Prader-Willi Syndrome?3

Physical and Development Characteristics3

Contentious debates4

CHAPTER 2: SELECTION AND IDENTIFICATION OF THE EVIDENCE5

Evidence Based Practice5

Embodiment and the role of biology8

Search Methodologies10

Over-eating behavior14

Body composition and obesity16

CHAPTER 3: CRITICAL APPRAISAL OF SELECTED EVIDENCE18

'Keeping occupied' practices22

'Use of routine' practices25

Embodied interaction28

Abuse29

Using CASP tool33

Artcles used33

CHAPTER 4: CONCLUSION AND IMPLICATIONS35

Medical Challenges37

CHAPTER 5: RECOMMENDATION AND PLAN FOR IMPLEMENTATION39

Parent coping and support40

Support Needs of Children with PWS42

Medical, health-related, and developmental support needs42

Educational support needs43

Family support needs43

CHAPTER 6: CONCLUSION45

REFERENCES46

CHAPTER 1: INTRODUCTION

Introduction

PradereWilli syndrome (PWS) is a genetic condition with an estimated prevalence of 1:52,000, based on research from one UK health region (Whittington et al., 2001). PWS is a complex, multisystem disorder that has among its key features an intense interest in food, which often develops into an obsession. Body composition is also affected within the disorder. Muscle to fat ratio is poor, resulting in the requirement of a low daily energy ...