Each individual who is admitted to the service is assessed prior to being offered a placement at Chesfield House. The assessment is carried out under consultation with the service user, their family or carer, the care manager or community nurse and any other people who are felt to be important to the process.
The Assesment Will Include
• Mental Capacity
• Health and medication checks
• Mobility and transport needs
• Personal care
• Mental health support
• Behavioural needs
• Dietary needs
• Social activities
• Daytime Activities
The continued involvement of family members and friends is supported and encouraged, and each individual is cared for with respect by each and every member of staff.
What is Prader-Willi Syndrome?
It is a complex medical condition affecting both boys and girls from the moment they are born and continues to affect them throughout their lives.
People with PWS have multiple disabilities which include:
- An obsession with food and eating from about the age of 2 years.
- Poor muscle tone
- Learning disabilities, varying from between mild and severe
- Lack of normal sexual development
- Emotional instability and lack of maturity in social skills
- Short stature
Why do people have Prader-Willi Syndrome?
People do not ‘catch it’, nor can they grow out of it. It is not very common and you will find that few doctors know much about it. It is a genetic condition caused by an abnormality in the person’s chromosomes (chromosome 15). It is rarely inherited, usually being caused by an accidental occurrence at the time of conception: neither parent being to blame.
It can therefore occur in any family and normally only one child is affected. Brothers and sisters rarely pass on the condition to their own children. An epidemiological study carried out in one health region in the UK in 2000 estimated birth incidence to be in the region of 1:22,000, with a lower bound for population prevalence of 1:52,000. Previous estimates have estimated a birth incidence between 1:10,000 and 1:25,000. Much of the uncertainty with regard to the statistics for incidence and prevalence is due to the fact that PWS may still go undiagnosed in some people (PWSA,UK,2010).
Stages of Development
However, as awareness and knowledge grows, more and more children are now being diagnosed at or shortly after birth.
What is it like to have Prader-Willi Syndrome?
Babies have difficulty with sucking and feeding and may not thrive well in the first few months of life. Walking and talking milestones usually occur later than average.
Between the ages of 2 and 4, appetite increases in the vast majority of cases, results in an obsession with food and eating. This continual hunger is probably due to a disorder of the hypothalamus within the brain.
People with PWS have poor muscle tone and therefore use up less energy than most other people. This combined with the effects of overeating means that if a person’s food intake is not controlled, gross obesity results which can become life threatening from an early age.
With proper dietary management people with PWS are able to keep within reasonable weight levels.
Most people with PWS have learning disabilities and although in many cases this is mild, people can and do have uncontrollable temper tantrums or rages and may have obsessional or stubborn traits in speech and behaviour.
What treatment is available?
There are currently no drugs or treatments which will cure all aspects of the condition. Sexual maturity and growth can be helped with hormone treatment.
Conventional appetite suppressants do not work and drugs to control behaviour may have unusual side effects.
Therefore, at present the only treatment for the eating and behavioural aspects of the condition is to establish a stable, understanding and supportive lifestyle which includes the following features:
- Strict dietary control
- Physiotherapy and exercise programmes
- Specialised education for children of school age
- Special supported living or specialised residential care and support.
Inspiration Care and Supporting an individual with PWS
With better management and understanding people with PWS are now living well into middle age and beyond and can bring many skills and good qualities to the community in which they live. We have one such individual who is supported in attending a job at a local shop.
We look at providing a holistic approach to care for each individual, helping to promote their well being, self esteem and image is just as an important part of support as is ensuring the support around managing their healthy living style and dietary requirements.
It is important that an individual with PWS is supported by staff with an excellent knowledge of:
- Managing Behaviour
- Temper outbursts
- Managing the environment
- Stealing food
- Stubbornness and resistance to change, argumentativeness
- Perseveration (asking the same question, or reverting to the same subject over and over again)
- Obsessive-compulsive behaviour
- Attention- seeking and manipulative behaviour
- Skin picking and spot picking
- Lying and blame-shifting
- The use of medication in severely challenging behaviour
- Mental health problems
For more information about PWS - you may find this website useful.... http://pwsa.co.uk/