Adaptive Behaviour Skills in Angelman Syndrome
Adaptive behaviour skills are everyday living skills such as washing, dressing and communication.
An overview of skills in Angelman syndrome is provided below. You can also visit our interactive database to ask a question about the development of skills and behaviour. The database will give you an answer based on data collected during a large scale research study with people with Angelman syndrome.
Self-help skills in Angelman syndrome vary widely. Skills such as washing, dressing and feeding require fine motor control and planning, and many children and adults will need help with these daily tasks. Fine motor skills are often compromised in Angelman syndrome. Children will reach for an object and miss and then over-correct the movement. This is often observed as jerky hand movements. The development of self-help skills may be delayed among children with Angelman syndrome but some do achieve a degree of independence in this area. Adults with Angelman syndrome will need continued support with day to day living.
A child with Angelman syndrome will start walking between 2 and a half and six years of age. However, around 3 out of 10 children with Angelman syndrome do not learn to walk independently.
Research has shown that there are differences in mobility between the different genetic causes of Angelman syndrome. Individuals with the deletion genetic cause are the most severely affected with 50% not being able to walk by the age of 5. In contrast around 95% of individuals with Angelman syndrome not caused by deletion are able to walk by the age of 5.
Communication skills in Angelman syndrome are often severely impaired and the majority of individuals with Angelman syndrome have an absence of expressive speech. Although expressive speech is usually limited, there are some individuals with Angelman syndrome that can use one or two word phrasing. Expressive communication skills (ability to express oneself) are usually more impaired relative to receptive language skills (ability to understand others). Difficulties with other areas of expressive communication have also been reported including gesture, signing and the use of alternative communication devices. Individuals with Angelman syndrome often use a number of non-verbal strategies to communicate including, for example, approach, touch and pushing a person's hand away. Some older children may be able to sign or use communication boards whereby they point to what they want.
In this short film a family discuss how they have used the iPad to help improve communication.
Communication Skills and Behaviours That Challenge
Poor expressive communication is not unique to Angelman syndrome (it is also seen in both Cri du Chat and Cornelia de Lange syndromes for example) but it may have a particular relevance to behavioural difficulties. If individuals with Angelman syndrome are unable to express themselves using formal methods of communication that can be well understood by others, this may lead to frustration and behaviours that challenge.
Some parents may find it helpful to produce a communication passport for the individual they care for. This helps to keep everybody who works with the individual informed about how the person prefers to communicate and may reduce the individual's frustration and behaviours that challenge. To learn more about communication passports and view one parent's example and experience, please click here.
Socialisation skills are reported to be a relative strength in Angelman syndrome and a key characteristic of children and adults.
Grieco et al. (2014) - research shown minocycline resulted in improvements of adaptive behaviours in children with AS. Further investigation needed as optimal dosage and long-term effects needs to be determined.