Many people with Angelman syndrome are not able to communicate that they are experiencing pain and discomfort when this occurs. Consequently, they may find other ways to let you know that they are in pain.
In this section, you will see that we focus primarily on self-injurious behaviour related to pain and discomfort. This is because we think that when pain and discomfort arethe cause of behaviours that challenge, self-injurious behaviour is the type of behaviourthat challengesthat is most likely to be influenced by this factor. However, this does not mean that other forms of behaviours that challengeare not influenced by pain and discomfort. For any sudden outbursts of any form of behaviours that challengethat appear 'out of the blue' pain and discomfort should be taken into consideration when trying to identify a cause.
There is some evidence that challenging behaviour, in particular self-injurious behaviour can occur in response to painful medical conditionsand discomfort. Examples of medical conditions which have been found to lead to self-injurious behaviour in children and adults who have intellectual disability include otitis media (middle ear infections), skin infections and irritation and gastrointestinal reflux.
In the 1960’s, a researcher called De Lissovoy studied head banging in children and found that the children in his study who were head banging were significantly more likely to have had otitis media (middle ear infection).
Self-injurious behaviour is sometimes directed towards areas of the body which might indicate that the person is experiencing pain in that area. For example, banging or rubbing around the ear in response to earache or banging around the jaw in response to toothache. You can see an example of pain related behaviourin the re-enactment by a role play actor at the top of this page.
At other times, the self-injury may be directed towards another part of the body that does not appear to be related. For example, we have observed children who pull their own hair in response to chronic pain in the stomach. Signs of pain are not always obvious . The section below describes why we might observe self-injury in response to pain and discomfort.
How does self-injurious behaviour relieve pain and discomfort?
It seems odd that someone would engage in self-injurious behaviour if they were already experiencing pain elsewhere. Pain gate theory suggests that an acute pain (sharp and intense pain such as that caused by self-injurious behaviour) can block (or gate) the sensation of chronic pain (long lasting pain such as that caused by a painful medical condition).
Pain gate theory applies not only to individuals who have an intellectual disability but to everyone. Have you ever banged your head and rubbed it better? This is an example of how you are able to block one type of sensation (pain) by creating another.
For more information on identifying pain in children with severe intellectual and/or communication difficulties please visit the Cerebra webpage.
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FAQs
Self-injurious behaviour is sometimes directed towards areas of the body which might indicate that the person is experiencing pain in that area. For example, banging or rubbing around the ear in response to earache or banging around the jaw in response to toothache.
What happens to the body when you have Angelman syndrome? ›
Most people with the syndrome will have learning disability and limited speech throughout their life. In adults, some mobility may be lost and joints may stiffen. People with Angelman syndrome usually have good general health and are often able to improve their communication and acquire new skills.
How do people with Angelman syndrome feel? ›
Children with Angelman syndrome typically have a happy, excitable demeanor with frequent smiling, laughter, and hand-flapping movements. Hyperactivity and a short attention span are common. Most affected children also have difficulty sleeping and need less sleep than usual.
What are the challenging behaviors of Angelman syndrome? ›
Behaviours that Challenge in Angelman Syndrome
They include self-injury, aggression, shouting, screaming and others. The term 'behaviours that challenge' does not mean that there is something about the individual themselves that is challenging.
What are the most significant symptoms of Angelman syndrome? ›
Angelman syndrome symptoms include:
- Developmental delays, including no crawling or babbling at 6 to 12 months.
- Mental disability, also called intellectual disability.
- No speech or little speech.
- Trouble walking, moving or balancing.
- Smiling and laughing often and seeming happy.
- Being easy to excite.
Seizures often begin between 2 and 3 years of age. Speech impairment is pronounced, with little to no use of words. Individuals with this syndrome often display hyperactivity, small head size, sleep disorders, and movement and balance disorders that can cause severe functional deficits.
What famous person has Angelman syndrome? ›
James Padraig Farrell. Farrell and model Kim Bordenave welcomed the actor's first child, James, on Sept. 12, 2003. In 2007, Farrell shared that his son, now 19, was diagnosed with Angelman syndrome, per People.
Does Angelman syndrome give super strength? ›
Frequently our Angelman superheroes have normal, or better than normal, strength. A huge part of gross motor skill learning is protecting yourself from falling, or saving reactions.
What are the strengths of Angelman syndrome? ›
In general, Angelman syndrome individuals have relative strengths in nonverbal reasoning skills and with social interactions that are based on non-‐‑verbal events.
Do people with Angelman syndrome like water? ›
Angelman syndrome is characterized by mental retardation, seizures, ataxia, inappropriate laughter, lack of speech, a particular facial appearance, and generally a chromosome 15q11–q13 deletion. Recently, a fascination with water and water-related activities has been reported in individuals with the syndrome.
Around 14% of children and 18% of adults with Angelman syndrome show levels of positive emotion that may be considered 'excessively high'. In the research study that looked at this, no individuals showed negative emotion, however this is not to say that it does not occur in a few individuals.
What is Angelman syndrome mouthing? ›
Pica and Object Mouthing
Many individuals with Angelman syndrome will place objects into their mouths and chew them. Pica (the eating of non-edible materials) is also sometimes reported.
How do people with Angelman syndrome act? ›
Children with Angelman syndrome typically have distinctive behaviors (in addition to the characteristic symptoms), including: A happy, excitable attitude. Frequent smiling and laughing. Frequent hand-flapping motions.
How smart are people with Angelman syndrome? ›
Developmental Characteristics
As the child gets older, the impairment will become more apparent. While the scope of impairment can vary, children and adults with Angelman syndrome will almost invariably have severe to profound intellectual disability.
Do people with Angelman syndrome understand things? ›
Communication difficulties
Notably, individuals with Angelman usually are able to understand things that are communicated to them to a greater extent than they are able to communicate for themselves.
Can kids with Angelman syndrome cry? ›
In Angelman patients, anxiety is exhibited by clinginess, inability to relax, nervousness, irritability, restlessness, and crying.
Why do people with Angelman syndrome like water? ›
Research has also found that individuals with Angelman syndrome show this preference for water-related objects/activities relative to other activities and individuals without Angelman syndrome. This may be related to sensory processing in the syndrome.
What is the quality of life for someone with Angelman syndrome? ›
Prognosis and Life Expectancy
Some symptoms of Angelman syndrome improve as individuals get older. Sleep issues and seizures tend to become less severe or infrequent. Because of mobility issues, obesity and scoliosis can develop in adolescence. The life expectancy of people with Angelman syndrome is normal.
