Leaflet nº2. Behavioral disorders in FXS

Leaflet 2

BEHAVIORAL DISORDERS IN

W ith the suppor t of:

Departament d'Educaci贸 i Universitats de la Generalitat de Catalunya

FXS

Leaflet 2

INDE X

1. INTRODUCTION

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2 . WHAT IS BEHAVIOR ?

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3. WHAT CHARACTERISTICS

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MAY BE AFFECTING BEHAVIOR ?

Direction: Mercè Bellavista y Carles Fauró

Language

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Anxiety

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Sensory aspects

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Attention deficit and impulsivity

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4 . BEHAVIOURAL INTERVENTION

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5 . SOME PRACTICAL ADVICE

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6 . WHAT IS THE ROLE PHARMACOLOGICAL

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Contents: Katy Garcia y Eugenia Rigau Illustrations and English translation: Marc Isamat Graphic Design: Vargas*comunicació Associació Catalana Síndrome X Fràgil, 2006 Plaça del Nord,14 (Col.legi La Salle Gràcia) 08024 Barcelona Depósito Legal: B - 8844 - 2007

TREATMENT IN BEHAVIOR ?

1. INTRODUCTION Not every kid with Fragile X Syndrome (FXS) has behavioral problems, but it is nonetheless a fairly common trait that appears at some point during the child’s development. Some disruptive behaviors, though not all, may be related to FXS. The aim of this leaflet is to inform on what we know about motives behind behavioral disorders in these kids. Some basic

2. WHAT IS BEHAVIOR? This leaflet uses the term behavior to describe everything that a child does or says. Behavior can be observed and felt, and in this sense it is different from what the kid might be actually thinking or feeling in that precise moment.

concepts, which may help improve such behaviors, may be obtained from this knowledge.

When a child is moving about, he or she is performing a motor behavior. If the kid is talking, then it is a verbal behavior. If the child is crying or laughing, then we’ll speak of emotional behavior. A person is constantly performing behaviors.

Being able to know and observe behavioral characteristics will be of great help for the understanding of behaviors that may otherwise seem isolated or nonsensical. The popular approach stems from the premise that a child’s inadequate behavior is due to inconsistent parental

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practices. Very often parents express

The use of disciplinary measures, like

their embarrassment when their child

threatening, reminding the consequences

throws a tantrum in public or has a fit of

of a behavior, punishment or spanking,

temper. People, unfortunately, tend to

that have been somewhat successful in

have thoughts of the sort of “...what feeble

other children with no neurocognitive

parents, what the kid needs is a good

disorders, may not be useful or may even

spank.” But there is a different inter-

be detrimental for developmentally

pretation of these types of behavior,

challenged children. It is very important

which involves the understanding that

to listen and get advice from FXS

such fits are due to the child’s lack of the

specialists and make sure that we use

skills required to generate adequate

the appropriate techniques to manage

behavior. So parents may not always be

behavioral disorders.

to blame for their child’s bad behavior.

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behavioral disorders.

When we learn why they react in a specific way, the strategies that we can adopt to improve their behavior become c l e a r e r. S o m e t i m e s , s i m p l y understanding the child’s difficulties can alone improve the adult-child relationship.

3.WHAT CHARACTERISTICS MAY BE AFFECTING BEHAVIOR ?

For instance, when a child feels

There are many explanations for the

frustration with a particular school task

motives behind behavioral problems in

and he fails to recognize exactly where

FXS. The most common cause is anxiety,

the difficulty lies, the reaction will be

but there are also other factors that may

inadequately manifested by ripping the

be playing an important role in inadequate

piece of paper or stripping the book, so

behaviors, such as:

A 7-year-old boy may react by throwing himself on the ground in a fit of temper to tell us that he doesn’t want to go somewhere. If we fail to react before this situation we will not recognize what he wants, but if we react in an exaggerated manner the boy will very likely remain on the ground.

frustration of these children we can offer alternatives or strategies to help them not fall so easily into frustration, and in so doing we will be avoiding the need for punishment. In these cases punishing or telling off will not only be useless but will generate even more frustration and further blockage of the child’s skills.

behavior. Kids may develop inappropriate behaviors to try and express what they want or feel, because they are unable to express themselves verbally.

this point uncontrolled reactions such as shouting, anger, throwing oneself onto the ground, hand flapping, and repetitive routine breaks may contribute to high anxiety levels. There are studies demonstrating that FXS associated anxiety is more rapidly generated and remains for longer periods of time than in children with no FXS.

Language limitations, or lack of language, is a predisposing factor to bad

anxiety and are unable to control it. At

language, may appear. Changes or

that he will evade performing that task.

If we acknowledge the low tolerance to

Anxiet y. They occasionally suffer from

An 8-year-old boy in his classroom goes and stands silently by the door to indicate that he wants to go out. The same boy shouts and flaps his hands when a routine, or a thing he is used to, changes in the school. We must therefore foresee those changes that can cause anxiety in him.

A 9-year-old goes every Saturday to a leisure center, but when Saturday morning comes he does not want to go. He has difficulties before certain situations. Once he gets to the leisure center, as every Saturday, he enjoys himself a lot and even wants to stay there when it is time to leave.

If these children had the correct skills to communicate their feelings and had no anxiety generated by unforeseen changes, they would not manifest such behaviors. It is completely absurd to think that they enjoy behaving in these ways.

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Sensory aspects. Our brain helps us analyze, classify and respond to the sensory inputs that we get from our immediate surroundings. There are children whose ability to integrate this information is underdeveloped and may thus react inadequately to certain sensory stimuli.

Different sensory aspects like the texture of certain foods, noises, smells or touch may give rise to behavioral reactions.

Often if somebody touches them they may try to avoid physical contact or react in an exaggerated way.

A 10-year-old had all his clothes’ labels cut because they were a cause of distress to him. Significant behavior problems in a 7-year-old during lunch hour in the school canteen were finally explained as an over-reaction to loud noises. It is also commonly found that they deeply dislike having their hair cut.

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Social aspects. FXS kids normally suffer from high levels of social anxiety and difficulties in their use of language, which makes interacting with others even harder. Their avoidance of eye contact together together with their inability to sustain a conversation affects their sociability deeply.

Attention deficit and impulsivity.

These traits hinder learning abilities and also affect behavior. They make them react without thinking, never stop fidgeting, may want to touch everything and cannot control certain impulses. It is important to remember that, sometimes, they can be more active than other kids.

Very often we hear parents explaining than when their child meets someone for the first time they throw themselves on the floor or just start running away.

If we know this may happen, it may be wise under certain circumstances not to insist on having our child greet the new person as would be expected or asking to look at his face.

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4. BEHAVIORAL INTERVENTION

There just isn’t one specific behavioral

ques and programs that may help improve

program for kids with FXS. Professional

the behavior of these kids. One behavioral

therapists that have dealt with these

program which has been successful in

childresn point to the need of using

FXS kids is the ABC Model [A (antece-

strategies from different programs in a

dents) - B (Behavior) - C (consequence)].

non-mutually exclusive manner.

This perspective stems from the fact that

Intervention must be carried out by a

a specific behavior does not happen in

specialist person who knows about the

an isolated way, but that it is the result of

FXS behavioral phenotype.

a complex interaction between the kid

Each case will need a personalized

and its immediate environment. Modifying

intervention program, given that every

antecedents and working on the

child has particular characteristics of its

consequences we can adjust and

own.This leaflet will offer some techni-

straighten many behaviors (Table 1).

Antecedent: the kid is hungry.

Behavior: The kid cries because he cannot express verbally that he is hungry.

Antecedent

Behavior

Consequence

A

B

C

Consequence: We give the kid something to eat and the crying stops.

The kid quickly learns that every time he or she wants something to eat, crying is a good way to get it, and we may be reinforcing this behavior by providing food. We can anticipate when the kid will be hungry and offer some food before the crying starts.

WHAT?

WHAT DOES THE KID DO?

HOW DO WE REACT?

Other techniques that have been successful are:

TABLE 1

MODEL A-B-C

By getting rid of, or modifying ANTECEDENTS we may decrease or remove altogether a certain behaviour.

The CONSEQUENCE of a certain behaviour will help us identify a way to modify it.

It is the CONSEQUENCE that keeps the behavioural cycle repeating itself.

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For instance:

Positive reinforcement: often we notice only bad behavior. It is important to notice what the kid does well and letting the kid

know, so that he or she may do it again in other situations.

Time outside: this technique has given good results in many occasions. It consists of sending the kid to the bedroom or some other pre-established place for a few minutes, when the situation gets difficult, until the kid calms down.

Token economy: This is an exchange system. It consists on rewarding with a token the performance of certain behaviors that have been previously agreed with the kid. The tokens can then be exchanged for something, which had been agreed. For instance, if the child helps clearing the table he gets one token every day, and with 7 tokens he may go and buy a packet of the stickers he is collecting. These are classical behavioral intervention techniques.

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Another model that should be considered

From this viewpoint, the parents may

is that known as CPS (Collaborative

negatively look upon the child’s behavior

Problem Solving) developed by Greene

and contribute in this way to increase

Ross Greene. The explosive child. Harper

inadaptive behaviors. Taking into account

Ed.. It stems from the premise that

that certain basic skills are needed to

a kid’s behavior responds to deve-

modulate behavior, then it is

lopmental delays in certain cognitive

understandable that behavioral problems

skills necessary for adequate behavior

may arise under certain situations when

(Table 2).

these skills are not present.

This approach focuses more on cognition than on behavior and enables the adult to understand that bad behavior may not necessarily be intentional or pretended blackmail. Certain behaviors may require skills that the kid may not have yet developed.

How some of the abovementioned

I Independently from what this

dysfunctions may modulate behavior can

situation may turn into, we can attempt to think what John may be trying to tell us if he didn’t have difficulties to express his feelings and sensations:

be illustrated by John’s case: Dad: Stop watching TV and let’s go to the party.

frustration, and problem solving abilities

John: No! I’m watching TV .

Dad: Turn that TV off and come here, we HAVE TO GO NOW

are not developed independently in the

John: I can’t

Control of emotions, tolerance to

child. Moreover, these skills are dependent on the ways and on the different models that adults may choose to try and teach them.

This model states that the result of a behavior depends on the degree of compatibility between the adult’s and the child’s characteristics.

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Ross Greene. The explosive child. Harper Ed.

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Dad: What do you mean, you can’t? We are all here by the door waiting for you, we’re leaving, COME HERE NOW! John: Shut up you!

Dad: Can you please stop talking to me like that? Why do you always answer me back in that tone?

John: I DON’T KNOW !!!!!!!!!!!!!!

“Look Dad, I have a problem. You keep on asking me to stop doing something that I like and to go to a party instead. I don’t like going to parties, they are a source of anxiety to me. When you ask me to make changes I feel anxious and get blocked. I stop thinking clearly, then you start shouting and I say things I don’t want to say. Then you get angry, sometimes I get punished or you just suddenly turn off the TV, and that makes me very angry. I know you don’t like any of this, but I don’t like it either”. it either”.

When frustration and mental block appear is time for therapeutic intervention. Once we learn what types of situations are disruptive for the child we must interrogate which are the skills that are underdeveloped or lacking in the kid. Bad behavior normally starts when the cognitive demands overwhelm the kid’s capacity to react in an adaptive manner. We must know what triggers the reaction and respond in an understanding way offering as much help to the kid as possible. We can provide the child with strategies to solve similar situations in the future. In addition to understanding disorders and shortcomings, the CPS forces us to classify each behavior into threedefined categories, called coves A, B and C. defined categories, called coves A, B and C.

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TABLE 2

COGNITIVE SKILLS RELATED TO BEHAVIOR

In cove A, the adult must retain a position of authority. Not negotiable.

No quarreling, no shouting, simply do as told by the adult. Cove A must only be used in situations where the kid’s integrity is in danger, or when his behavior may be dangerous to himself or to others, or may result in breaking furniture or important things.

Cove C includes certain behaviors that we temporarily ignore. The adult does not interfere, like walking barefooted in the house, laughing when he is told something serious, use of swear-words, being fidgety, etc these behaviors can be overlooked. They are unimportant aspects of his behavior and we have noticed that even after telling him lots of times, these behaviors continue.

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Executive functions.

Difficulties adapting to new situations.

Disorganised.

To think of the consequences of an action.

Problem solving.

Cognitive flexibility.

When the kid is in the classroom and unable to go to the playground because it is raining, he needs cognitive flexibility to adapt to the change of routine.

Language processing skills Difficulty expressing thoughts in words.

Cove B includes all behaviors that may help improve the skills needed to eventually acquire good conducts.

The aim is to have the child regulate his own behavior. In order for this to happen the adult must express empathy, define the problem and help the child find a mutually satisfactory solution for both adult and child, in which both will have some level of compromising. Empathy will help both the child and the adult to remain calm.

Defining the problem enables the child to understand what is happening and then work together to find a solution that the child may be able to apply in other situations.

Explain how they feel in words.

Very often they bite themselves and shout, have fits on the ground or flap their hands when they experience an emotional state which they cannot express in words.

Emotional control skills Irritability.

Frustration/anxiety.

A low frustration tolerance may cause an agressive reaction when something does not work for him in class.

If a sticker is normally inside a snack packet, and on that day the snack doesn’t come with a sticker he may get very angry and be mentally blocked without the sticker.

Social skills Low consciousness of how the others see him.

To know how to act in each situation.

Understand and attend to social clues.

Take others’ viewpoints into account.

Poor eye contact and the difficulty in sustaining a conversation often negatively affects the kid’s sociability.

He also finds it hard to know what others think and what to expect from us.

It is important to know that a child’s behaviour is due to a delayed development of these specific cognitive skills.

Behavioral problems must be dealt with just like anyother learning disability.

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5. SOME PRACTICAL ADVICE Create a favorable environment and act before the behavior manifests itself.

When things start to improve, the kid will gradually become more receptive.

Be receptive to warning signals that things are starting to go wrong or that the situation is overwhelming for the child, so that we can react in time to avoid or smooth out the problem.

Changes trigger behavior problems. We can anticipate changes, but sometimes this may backfire as the child will become increasingly nervous asking insistently when the change is going to occur. It is useful to provide an objective or reward related to the change. Providing an objective makes the change understandable and decreases the anxiety generated by not knowing what is happening or the reason for the change.

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For instance, if we have to go to a birthday party and the kid is scared or he just doesn’t want to go, we can explain to him that he is in charge of handing the gift to the birthday girl, and that’s why he has to go.

For instance, Or, if a child doesn’t want to come out of the car when he gets to school and he starts shouting, hitting or biting, we may explain to him in advance that he must do something important when he gets to the classroom. This important thing can be something like putting some coins that mum provides into a box in the classroom. The money will then be used to buy things for him and his classmates at the end of the week.

We must know WHEN, WHERE and HOW OFTEN certain behaviors arise.

All behaviors are the result of something.

We must understand the Antecedents, the Behavior and the Consequence.

Modifying some of these aspects can improve the child’s behavior.

Modify the environment or the situation whenever possible reduces the probability of triggering a certain behavior. It is often easier to change the environment or the structure of a certain routine than the behavior itself.

For example, very often when a FXS kid goes into a department store he is bombed with all sorts of stimuli, and his behavior worsens. Once we have analyzed this situation we must

understand that the cause of that undesired behavior is the kid’s hypersensitivity, the consequence of our negative response, probably after incorporating extra stimuli like shouting at him, holding his hand more firmly, etc....

In this situation, a possible solution would be not to take the kid with us to the department store, and in this way avoid the conflict altogether, or go sometime when we know the store will not be so busy. Changing the structure of a routine, may be as simple as changing the routine each day.

For example, allowing the kid to get dressed after breakfast, instead of before, if he feels better about it and generates less conflict first thing in the morning.

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Remember that holidays may alter the

Be consistent. This aspect may be difficult

If in the middle of a fit of temper, it is best

kid. He may need a few days to adjust

to apply. Coherence and predictability of

to forget to try and reason with the child.

to the routine. Keep the crucial points of

routines are important in all children but

He wouldn’t want to hear you even if he

your routine unaltered when possible.

particularly for those with FXS. The latter

could.

Waking hours, eating, bathing and slee-

have problems interpreting the subtleties of situations.

ping times, may be good references.

It is important to try and talk positively to the child. Instead of always telling him

Take toys, pacifiers or familiar things with you to help them get used to the new situation.

Offer behaviors that may substitute

what you don’t want, try and change the

undesired ones. We must never get rid of

sentence around. Instead of saying: “stop

a certain behavior without providing an

doing that once and for all, we have to

alternative conduct.

tidy up”, you may say: “look, help me tidy this up and then we can watch TV”.

For instance, he can wear headphones

For example, instead of crying when they

and listen to music if the environmental

are finished eating, show them that they

noise is too high, like in school buses,

can sing. If before certain situations he

cafeterias, etc. If we fail to provide an

starts hitting or hand biting, or hand flapping,

alternative conduct that can help them

it may be useful to give him an object with

channel their anxiety they will very probably refuse to listen to us and the behavior will systematically be reproduced.

which he can substitute such behaviors.

Biting aids, bracelets or necklaces have been useful in preventing self-biting or hair pulling fits.

Offer the child alternatives before certain

Use visual clues with all the steps to be

Stay calm. Choleric responses will worsen

situations, which may be a source of

followed when approaching a difficult

the situation. If we cannot guarantee that

stress or anxiety, or trigger an undesired

task. Offering pictograms may help

you will not loose your temper it is best to

communicate needs, as sometimes,

withdraw from the situation.

behavior.

these are kids with language difficulties and we can prevent frustration in this way.

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Do not use hurting language or

Take note of the most difficult hours of

Don’t try to change from one activity to

When you are playing with the child, do

disqualifying adjectives. Make it clear

the day. Giving the child his supper half

another too quickly. Warn him in advance

so at his level. If he’s playing sitting on

that it is the child’s behavior that you

an hour earlier is better than putting up

so he may have time to prepare for the

the ground, then you should also be on

dislike, not the child.

with half an hour of shouting and crying

change.

the ground, it is important to be at the

only because he is hungry.

same level in order to communicate.

Sometimes it is not the taste of a certain

Reinforce positive behavior. Identify those

food, but rather its texture, that the child

Apply the same measures to all the kid’s

efforts that are useful for the kid, knowing

dislikes. We can try and change the way in

environments.

that every kid is a different world. The

which that particular food item is

most efficient reinforcement is the natural

administered, and see if the child accepts

Behavioral intervention is very important.

one, give the child positive attention, offer

it. Some kids don’t like raw carrots but will

But fining time to play, laugh, joke,

eat them if they are cooked. This is not the

abandoning the limits and expectations

most important, try and not overreact. Leave

is also very important. Try and create

the kid eat what he wants from what remains

positive moments and have fun with your

him immediate and adequate responses.

Even then, we must take into account that sometimes FXS kids can be autistic

on his plate without making a fuss over what

and will therefore not respond to social

he hasn’t eaten.

child.

reinforcement. In these cases they may improve with external reinforcement like

If every bath-time is a crisis and alters

It is important to let the child take the initiative

little toys, more TV watching, etc.

the bedtime routine, find a different time

at certain moments during a game. This is

in the afternoon for the bath. It may be

not easy, try doing it without falling into

still a problem but it is good decide when

gearing or attempting to improve the game.

we can better tolerate certain crises.

It is difficult for the child to relax and stay still immediately after having played very actively. We must understand that he must lower his activity gradually.

Let him tell you what he wants you to do.

Imitate what he does with the building blocks or with whatever you are playing with. This is a way of telling him that you like his ideas and that’s why you are copying them.

Sometimes we will have to help him calm down.

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WHAT IS THE ROLE PHARMACOLOGICAL TREATMENT IN BEHAVIOR?

CONCLUSIONS Lots of kids may have behavioral problems at some point in their development, and they may be able to improve or overcome these traits. Sometimes the behavior improves with time and as the child grows up and deveMedication may play a very important

correct use of the skills that are being or

role when we are trying to help the child

have been learnt. A certain amount of

acquire adequate behavior.

aggressiveness among adolescents has

specialist to see improvements. Understan-

been observed, and pharmacological

ding why the child reacts with a certain beha-

lops, but often parents will need help from a

Pharmacological treatment may be

intervention in these cases should be

necessary in cases of attention deficit,

considered.

vior is very important to guide future interven-

Neuropediatricians or psychiatrists

tions. We must remember that there is always

anxiety or depression disorders. Anxiety is a common component among FSX children and often requires treatment.

Medication for attention deficits can also improve learning.

evaluate the need for pharmacological treatment of these symptoms in each child. It is important to remember that pharmacological treatments are not an

Anxiety driven pharmacological

alternative but a complement to

intervention can help the child gain

behavioral intervention.

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a cause behind every type of behavior.

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