Best Practice Guidelines for Residential Care

Key considerations

All guidelines should take into account the assessment of the individual to assure health and safety to promote quality of life. A person-centred approach should consider the person’s preferences and goals, with the understanding that all people have rights to personal safety, health, meaningful relationships, dignity and respect. 

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 (managed by others or learned by the person), 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.

Communicating with people 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. It is important to build a relationship with the person through positive interactions, taking the time to find interests, and participating in preferred activities and encouraging trying new activities. Using patience and empathy will help build trust and open communication. 

Neuropsychocognitive: When people with PWS have food security (managed by others or learned by the person) 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 can 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, poor coping strategies. deficits by learning from mistakes, they need support perceiving the emotions of others, changing their emotional perspective, building transformation chains, focusing on details, and with executive functioning skills. The use of pictures or written schedules, the use of social stories and “first, then” language can be helpful.

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. The goal is to come to an intrinsic motivation by the person with PWS. Building natural exercise throughout the day is important.

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. Further guidance for parents can be found here. 

Nutrition: People with PWS should be taught from when they are very young, about food and food management so that they can have a high chance of independence and self-regulation around their own food/nutrition. People with PWS need a lower number of calories because of the lower muscle mass. Calorie needs are based upon their body composition, BMI, and their daily physical activity. In many countries, this is directed by a physician and/or dietician. 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 (managed by others or learned by the person) must be stressed.  An individualised daily caloric intake and ongoing education of all people involved in the care of the person with PWS is required.

Crisis intervention: While certain characteristics are attributed to people 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. Understanding the behaviour of the person with PWS is a prerequisite for good support during the 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.

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. 

Structure of work: Every person has dreams and wishes for their future. How do we manage so the dreams of the person with PWS 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, by experiencing their own self-efficacy. 

Aspects of psychological work: The focus of psychology is understand the human experience and behaviour, and so the psychologist plays a key role in support of understanding behaviour in PWS. The psychologist plays a primary role when providing direct supportive counseling or therapy to the person with PWS and 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.

Interpersonal relationships: Among people with PWS, relationships of any sort are constrained by the “genetically and neurologically” reduced competence for reciprocal relationships, and challenges in shifting emotional perspective. The capacity for emotional understanding is further limited by a lack of opportunity and experience.  So a focus must be to support people with PWS to overcome this deficit and be more and more able to handle relationships. Ongoing modeling and training in the areas of positive/healthy relationships is a great support. 

Behaviour management: Every person who is involved with the care and management of a person with Prader-Willi syndrome will eventually experience a crisis situation. 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 the information and insight gathered in the three previous steps to create prevention strategies. 

A behaviour 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 behaviours while offering structure in the form of limited choices. While focusing on prevention of behavioural crises, the appropriate consequences of maladaptive behaviours 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 behaviours.