Family Care for Prader-Willi Syndrome
Family Care (Famcare) is a project of IPWSO's designed to support families whose adult son or daughter lives at home. We know that this can sometimes be difficult and that for living arrangements to work successfully there often have to be many compromises made by parents and other family members. The support of extended family members and friends is also most valuable to the well-being of the person with PWS and his or her parents.
Where possible, community support can also play a significant and important role. Providing an appropriate environment for someone with PWS in all areas of their life will promote good health and longevity.
If you have an older son or daughter - someone who has left school, but is still living at home - and need our help, please answer our questionnaire. (Spanish ) Our Famcare team will do its best to come up with some supportive answers.
Famcare will be producing leaflets on topics connected with the care and management of the syndrome, compiled with your help so that the advice you will receive will come from other parents, as well as professionals. We hope that this will be helpful to you. As each leaflet is written and published, it will be made available on this website.
A Guide to the Basics of a Healthy Adult Life
An early childhood diagnosis of Prader-Willi syndrome (PWS) usually provides parents with the opportunity to learn strategies to manage potentially difficult behaviour and obesity. Despite variable intellectual and physical abilities, it must be remembered that a cognitive disability, hunger and reduced satiety remain with the person with PWS throughout their lives. The ability to make appropriate independent decisions regarding energy intake and choices of life rarely eventuates, even as the child with PWS becomes an adult... (Read more in the following languages)
English - Spanish - Italian - German - Dutch - Arabic - Russian
Mandarin
A Guide for a Health Checklist
The basics of a healthy adult life for someone with Prader-Willi Syndrome (PWS) include a healthy, appropriate eating plan and regular, effective physical activity to avoid obesity, to keep the body fit and to maintain good mental health. Before any of these can be achieved the people working with the person who has PWS must have a good understanding of the complexities of the syndrome.
This “checklist” is to assist you in maintaining good health for your person with PWS. It is to be shared with other family members, professionals and caregivers who are involved with your person with PWS. (Read more in the following languages)
English - Spanish - Italian - German - Dutch - Arabic - Russian
IPWSO GUIDES
A Guide to Confabulation (story-telling in PWS)
What exactly is ‘confabulation’? It is defined by dictionaries as: a memory disturbance, defined as the production of fabricated, distorted or misinterpreted memories about oneself or the world, without the conscious intention to deceive.
Confabulation is different from lying as the person is fabricating stories that he or she genuinely believes in, and believes that you, too, should also believe what they are saying. They are often coherent, consistant, and the stories are relatively ‘normal’. The information produced in these stories is generally false, or made up from incorrect memories, either of the person’s own, or from what they may have heard or read or seen. Confabulation can range from subtle alterations of a story, to quite bizarre fabrications. (Read more in the following languages)
A Guide to Coping with Change
Most people with Prader-Willi syndrome (PWS) have difficulty coping with change. This varies from person to person and can be a real problem for those families affected. Change can be anything from a substitute teacher at school, to a different route being taken in a car trip or a new cup being used. Change can also occur suddenly and unexpectedly, as in an electrical blackout. A bad reaction to change may result in the person with PWS refusing to comply with requests, routines or plans and can quickly escalate into perseveration (repeated questions or comments), arguments and aggression. “Shutdown” is another typical response to the anxiety associated with change. These responses can be stressful for families as they often occur at the most inconvenient times. (Read more in the following languages)
English - Spanish - Italian - German - Dutch
A Guide for when your child states: "I want the same!"
How do you answer your person with PWS, who asks why they can’t have the same or do “the same” as other people? The “same” includes many more things than just food. People with PWS want to travel independently as do other adolescents and adults. Once someone with PWS learns how to reach a destination they can usually repeat the journey alone, without getting lost and are, consequently, considered to be good at “travel training” - so what’s the problem? (Read more in the following languages)
English - Spanish - Italian - Dutch
Hindi
Guide to Managing a Meltdown
Most people with Prader-Willi Syndrome (PWS) have meltdowns from time to time. This varies from person to person and is a serious problem for those families affected. The levels of anxiety seen in people with PWS cannot be understated. Most of the difficult, anti social behaviour demonstrated by people with PWS can be related to their inability to express and/or deal with anxiety or emotions they are feeling. They can be upset by something we would just take for granted, or it could be over something that occurred yesterday or last week, as well as something that may have just occurred but has not yet even acknowledged by others. It is often “either all or nothing”. They may become highly anxious about a particular meal being served slightly differently and they may show minimal emotion when a family member dies. (Read more in the following languages)
English - Spanish - Italian - German - Dutch - French
A Guide to PWS Awareness for Professionals
People with Prader-Willi Syndrome (PWS) often present as cooperative, knowledgeable and highly capable of making their own decisions. They appreciate responsibility and respect. Adults with PWS, who are intellectually able, usually resent the fact that parents or other family members want to accompany them to appointments with professionals invovled in their health care or life matters.
This can create much frustration for parents and families as well as the professionals. It can also lead to misleading information being received and incorrect action being taken on the part of the professional.... (read more in the following languages)
A Guide to managing Skin-picking
Skin picking or scratching is a common behaviour seen in people with Prader-Willi Syndrome (PWS). The intensity and duration of this behaviour varies from person to person and episode to episode. It may occur in the form of scratching an insect bite until it bleeds and becomes an infected sore or it may manifest in the form of nose picking or anal picking. When this behaviour continues for a prolonged period it can cause unsightly and distressing open wounds. 75% of the people surveyed by FamCare reported skin picking in their relative with PWS. A USA study reported anal picking in 9.5 % of people who skin picked. (Read more in the following languages)
English - Spanish - Italian - German - Dutch
A Guide to Setting Boundaries
People with PWS have a heightened anxiety and need others to set consistent and consistently enforced guidelines or boundaries for them to feel safe and respond appropriately to what is going on around them. Boundaries include positive behaviour strategies through the encouragement of desired behaviour rather than the punishment of undesired behaviour. Boundaries teach the person with PWS what is desired by you and society and how to communicate effectively. Anything taught must be simply stated, understandable and reinforced in a consistent manner to minimise anxiety. (Read more in the following languages)
A Guide to your Duty of Care
When you have a son or daughter with Prader-Willi Syndrome(PWS) who continues to live in the family home, your parental duties do not reduce as your person with PWS progresses through the different stages of life. This can be very tiring and impact on you and your whole family. Caring for an adult with PWS means you rarely have time to relax and feel that all is well. People with disabilities are as much a part of society as anyone else and you, as parents or family members, have the right to expect and advocate for their needs to be met. This means seeking the best possible workplace, respite care or
day support for your family member. A major difficulty with most service providers is their keenness to promote independence for individuals will often include options that are inappropriate for people with PWS, despite the fact that the person with PWS may try to convince those supporting him/her otherwise. (Read more)
A Guide to Exercise
People with PWS are born with reduced muscle tone (which affects muscle contraction) less muscle mass and especially in childhood, weaker muscles than non-affected people. Strong muscles are important for good movement and the protection of joints, ligaments and tendons. Most children with PWS, develop a greater percentage of fat tissue than non-affected people, from infancy. Even when the person has a healthy weight for height (BMI) the percentage fat will be greater than in the non-affected person. This means it is much easier for them to gain fat and harder to “burn fat” through daily energy expenditure. The more muscle a person has the greater their potential for more “fat-burning” or energy expenditure, as muscle tissue burns energy (calories) first. When people are on restricted energy intake menus, as needed by people with PWS to lose weight, there is a risk that their muscle mass will reduce as well as fat. People with PWS need to protect
against muscle loss and this is only done through movement and exercise. (Read more)
A Guide to Personal Care
Personal care is an important part of everybody's daily routine. However some people with Prader-Willi Syndrome
(PWS) have a reluctance, or a reduced ability to complete personal care routines properly. Factors such as obesity or
lack of coordination can make it difficult for a person with PWS to reach all areas of their own body, and a lack of
motivation or time can impact the care and thoroughness of cleaning routines. Poor sequential processing or
difficulty in completing multi-stepped tasks, often inhibits the thoroughness of washing. For example: the person
with PWS may just stand under a shower rather than wash themselves. (Read more)
A Guide to Promoting Positive Behaviour
Some people with Prader-Willi Syndrome (PWS) engage in inappropriate behaviour from time to time. This varies
from person to person and can cause problems for the person with PWS, their families and the community. It also has the potential to limit opportunities to socialise with friends and isolate or cause embarrassment for members of the family. (read more)
English
A Guide to the Transitional Years
People with Prader-Willi syndrome (PWS) have great difficulty coping with change! Planning for change is
important to help reduce possible reactive, fearful and oppositional behaviour that can develop as a result of
impending change, not just sudden change. Periods of transition can be some of the most stressful and difficult
times for people with PWS. (Read more)
A Guide to Nutrition
"Need to Know Nutrition" is a published booklet (available online) and written by Professor Peter SW Davies,
The Children’s Nutrition Research Centre, The University of Queensland and Helen d’Emden, Mater Health Services, Co-author Brigid Knight, Mater Health Services. Although food groups will differ greatly from country to country the basics of nutrition for those with PWS is clearly laid out in this little booklet.
A Guide to Rituals and Obsessions
Many people with Prader-Willi Syndrome (PWS) engage in repetitive behaviours and rituals. Sometimes these behaviours can be beneficial and are often used by parents and carers to implement a predictable routine. For example, having set times and routines involving exercise and meals is often the cornerstone of a healthy lifestyle for an adult living with PWS. A predictable routine can also help to alleviate anxiety. A routine reduces idol time and the possibility of unscheduled opportunities for additional food. (Read more)