In most Western countries in the world there are residential options for adult people with Prader-Willi Syndrome. The International PWS Organisation (IPWSO) has undertaken to provide through its Professional Providers Caregivers Board (PPCB) a set of Best Practice Guidelines constructed from the input of many countries. In order to put these Guidelines into practice, each country must assess its needs, its cultural responsibilities and its own set of best practices. All guidelines should take into account the assessment of the individual to assure health and safety to promote quality of life.
This is a comprehensive and full Guide, available from IPWSO for EUR30 in the form of a CD. Topics are similar to those following, but are reviewed and added to after each PPCB conference.
The Pam Eisen Memorial Lecture (by invited speakers)
Environmental structure for PWS living
Communication with persons with PWS
Neurological, psychological and cognitive aspects of behavior
Fitness, sports and motivation
Training for teachers in PWS
Communication between parents and caretakers
Environmental structure for the work situation
Aspects of psychological work with PWS
Environmental Structure of Living Best Practices
Appendix: Medical Checklist
All guidelines should take into account the assessment of the individual to assure health and safety to promote quality of life.
From Environmental structure of living: Across cultural lines all participants agreed that in order for an individual with PWS to succeed and live a healthy productive quality of life that a base line level of supports conducive to promoting safety and personal growth, must be in place prior to providing care. It was determined that environmental supports were non-negotiable, including but not limited to; food security, daily schedules, personal growth and development plans, trained caregivers, and a continuum of care throughout their day. It was also agreed upon, that it will be imperative that as care providers, that we continue to explore this cohesive collaboration of efforts through on-going education, training, and awareness.
From Communicating with persons with PWS: Caregivers should be trained in active and diagnostic listening. This means they must discern the person’s ability to communicate and to assess their emotional state. They should be aware that there can be a large discrepancy between a PWS client’s apparent understanding and their actual grasp of the big picture, or long term goals.
From Neuropsychocognitive: When people with PWS have food security they are able to pursue other interests for a meaningful life. People with PWS have unique neuropsychological strengths and vulnerabilities. Strengths include good long-term memory, visual processing, shape discrimination, attention and perseverance for preferred activities. The environment has to consider that people with PWS have short-term memory deficits, deficits in dichotic listening, fine motor deficits, sensory perception and integration problems, deficits in simultaneous and sequential processing, poor time sense, cognitive inflexibility and rigidity, and they have poor coping strategies.
From Fitness, sports and motivation: The importance of exercise in the life of an individual with PWS was universally endorsed. The difficulty in motivating individuals with PWS to begin or maintain a lifestyle which includes effective exercise as a matter of routine was also identified by all group members. All clients with PWS will require motivation to do something that is new and may require some effort but once the activity is routine and consistent the ongoing motivating factor can be that of good health and success.
From Training the Teachers: Families of children with PWS find it quite disheartening to see their children enter a school system that is ill-prepared to teach them. Students with PWS can be quite challenging in an educational environment; yet those challenges can be addressed when there is a shared knowledge and understanding of PWS and how it is manifested by a particular student.
From Nutrition: People with PWS need a special, lifelong diet. The calorie needs are based upon their body composition, BMI, and their daily physical activity. Even though most adults with PWS need a low calorie diet administered by others, there are considerable individual variations in calorie needs. The importance of food security must be stressed. An individualized diet and ongoing education of all people involved in the care of the person with PWS is required.
From Crisis Intervention: While certain characteristics are attributed to persons with PWS, an in-depth knowledge of the particular individual being served is of equal or greater importance in identifying specific triggers to potential crisis-inducing situations. Personality style, coping skills, temperament, self-esteem, and past experiences vary from person to person and all contribute toward a person’s tendency to experience a crisis.
A crisis impacts the life of a person with PWS in significant ways. To begin with, a crisis calls into question whether or not their world is secure and their caregivers are trustworthy. If those questions are not answered affirmatively through the effective resolution of a crisis, then the increased anxiety of the person with PWS will likely lead to even more crisis situations. Conversely, if the crisis situation is handled appropriately and effectively, then proper crisis management can strengthen the bond between the person with PWS and their caregivers, leading to a more crisis-free environment in the future.
From Communication between Caregivers and Families: The flow of communication between a caregiver and the family of the individual receiving care is a crucial component to creating synergy in a person’s life.
From Structure of Work: “Every person has dreams and wishes for their future. How do we manage so their (the persons with PWS) dreams can come true?”
…a PWS supported work environment…must include safety and security, skill development, choice, an ability to promote positive self image, and allow them the personal experience of being a productive member within their community.
From Aspects of Psychological Work: The psychologist plays a primary role when providing direct supportive counseling or therapy to the person with PWS...a secondary role in providing emotional support and guidance to caregivers and family members. The psychologist can play a key role in facilitating communication among all stakeholders, including the person with PWS.
From Interpersonal Relationships: Among persons with PWS, relationships of any sort are constrained by the “genetically and neurologically” determined incapacity for reciprocal relationships. The capacity for emotional understanding is further limited by a lack of opportunity and experience. So relationships on all levels need to be externally managed and supported to reduce stress.
From Behavior Management: Every person who is involved with the care and management of a person with Prader-Willi syndrome will eventually experience a crisis situation. And each crisis experience offers an opportunity to learn how to better manage a crisis in the future. First, caregivers must understand the crisis. Second, caregivers must know what to do when a crisis occurs.
Third, after the crisis is resolved caregivers should take time to reflect with the client involved. Fourth, caregivers should use information and insight gathered in the three previous steps to create prevention strategies.
The behavior management plan should reflect the preferences, opinions and emotions of the individual. The individual should help create the plan, accept the final version of the plan, and perhaps sign the plan to demonstrate their agreement. The plan should be tailored to each individual’s mental level, and have clear rules and limits for appropriate behaviors while offering structure in the form of limited choices. While focusing on prevention of behavioral crises, the appropriate consequences of maladaptive behaviors should be explained. It is important to take into account that not all individuals with PWS will be able to comprehend the consequences of their behaviors.
Best Practice Guidelines for Residential Care
> Medical Care & Research
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