The Effects of Fragile X Syndrome on Learning

Fragile X Syndrome (FXS) is wide ranging in its effects, which can range from subtle learning problems with an average IQ, to severe intellectual disability. Although there are many symptoms of FXS that have educational implications for FXS children and their teachers, it is important to remember that any one child may present only a few of the problems or may present a range of symptoms but in a relatively mild form.

Speech and Language
Speech and language are almost always affected, and indeed, delayed development here is often one of the earliest presenting features. Litany-like repetition of words and phrases with up and down swings of pitch is common, as are repetitions of the last word or phrase spoken to them (echolalia), repetitions of words or phrases they have themselves spoken (palilalia), and a tendency to skip rapidly from topic to topic while in conversation. Speech is inclined to be rapid with poor control of rhythm (dysrhythmia), and words seem to fall over each other with pauses often in the wrong places making speech difficult to follow. Comprehension of the spoken word often represents significant problems for the FXS child.

The advice and guidance of speech therapy services can prove helpful in these areas.

Information Overload
FXS children frequently find it difficult to make sense of and react appropriately to the welter of information coming to them via their senses. In ‘busy’ environments they can become overwhelmed by excessive stimulation,
sometimes responding with tantrums, withdrawal, hyperactivity, perseverations or other confused behaviours. Adults need to be sensitive to the situations that may provide such reactions, but when they do occur, a calm and comforting response should be aimed for.

Behavioural Aspects
Perhaps the most debilitating clinical feature noted in individuals with FXS is that of behaviour. Behavioural features may include obsessive-compulsive tendencies, hyperactivity, attention deficit, mood swings and autism. There is a suggestion that people with FXS are more prone to the following:

Over-activity/Hyperactivity – always rushing about, never able to sit still
Impulsivity – not inclined to wait for anything, wanting things straight away, doing first and thinking later
Inattention – marked concentration problems, inability to stick to one game or task for any length of time
Restlessness – always up and down, out of one’s seat

All of the above may improve as the child grows up. When these four handicaps occur simultaneously in a variety of settings and with many different people they are known as ‘hyperactivity’. Some children may have received a diagnosis of attention deficit hyperactivity disorder (ADHD).

Other characteristic patterns of behaviour which tend to moderate as the child grows older are:

Social Anxiety – a reluctance to give eye contact, avoidance of other people’s gaze, particularly in situations the child perceives as stressful
Mimicry – tendency to mimic humorous or slang phraseology and bad language
Memory Retention – generally short, but can be excellent over the long term in a topic where the child’s interest has been engaged
Liking for Routines – children can become upset when established routines are broken
Repetitive Behaviours – such as hand-flapping and hand-biting

Many people with FXS relate well to others, but a significant number do have problems getting on with others. These problems show themselves in the following ways:

People affected by FXS may have difficulty communicating to others what they want, how they feel etc. They probably also have difficulties in understanding what other people are trying to get across to them, whether they use words, gestures or other ways of communicating. In addition they may be reluctant to look other people in the eye, having to avert their gaze if they are looked at. Socially, people with these problems may tend to be loners. They may find it difficult to get on with other people. They may prefer to be on their own or be extremely shy or anxious in company.

They may be obsessional in their behaviour. They may have their own routines and habits which they insist on sticking to and any unexpected changes often result in tantrums and other behavioural problems, since they have difficulty understanding what will happen next. They may like everything to be the same and find changes hard to cope with. They may persistently flap or wave their hands, in particular in response to anxiety or excitement.

When these disabilities are severe and occur simultaneously, the condition is described as autism, and may be associated with any degree of intelligence. Minorities of individuals with FXS have autism. Many more have some of the above features. Particularly common in FXS (and much less so in other conditions) is the combination of a likable, happy, friendly personality with a limited number of autistic-like features such as hand flapping, finding direct eye contact unpleasant, and some speech and language problems.

Generally speaking the problems experienced by girls and women with FXS are similar to those of boys and men. Girls and women with more average intellectual functioning may still have large discrepancies between different ability areas and may show similar concentration problems with impulsiveness, distractibility and difficulty sticking to tasks even if they are not overactive. Shyness and anxiety in social situations can occur. There can also be difficulty in organising thoughts, planning ahead, shifting readily from one piece of thought to another and going through tasks in one’s mind.

By and large, children with FXS are not what are commonly understood by the term ‘badly behaved’. They can, nevertheless, present teachers with management problems, to do with, say, getting up on impulse and meandering around the classroom, or reacting vigorously and inappropriately to events around them, or by appearing uncooperative at times.

While we might expect such behaviours from a child with FXS that does not mean we have to accept them. Teachers need have no worries about insisting on appropriate behaviour. That is part of the learning process. As with other curriculum areas however, long-term repetition will probably be required and perhaps, even a permanent behaviour management strategy.