Integration at school
As a parent, you can often be concerned about your child’s development, school skills and behaviour. As a parent of a Duchenne child, that concern can become your number one priority. You can see so many obstacles in your child’s way and you want to be proactive in making their school experience as positive as possible.
As a teacher, care coordinator or school manager you may also have concerns about having a child with Duchenne in the class. The fact that you are looking for information is a good start and shows that you are open to offering all children, no matter who, opportunities to develop at both at school and socially. As parents, we want to say a big thank you! The support we get from schools in integrating our children into society means the world to us.
In this section, we want to provide information about that difficulties that arise in regards to school abilities and behaviour in general. The key word here is “ability”. Like all children, children with DMD can be very different and every child is unique. Therefore, what follows is a general guide and may not be applicable to all children with DMD.
It is our hope that we can contribute to the awareness about possible problems that can occur so that they can be recognized and corrected as needed. Clinical research and experience have shown that learning and behavioural problems are more common in DMD boys than the general population. We therefore want to address these issues and how to approach them.
In order to provide a child with DMD the best chance for a positive school career and social integration it is very important to have a good and constructive cooperation with all parties involved; teacher, parents, care coordinators, and GON and CLB counselling. Open and honest communication is essential
School and Duchenne
School plays a very important role for children with Duchenne, maybe even more than for other children. The activities that that they will perform for most of their lives are learned here: reading, counting and working with computers.
In addition, school plays a huge role in their social development. Making friends, being accepted in society influences how they deal with their illness and how they build a happy life. Their school time moulds their future. The choice of school and type of education is therefore crucial.
As far as possible we strive towards integrating children with DMD into main-stream education. This is not only good for the boy but can also enrich the class and the school. In main-stream education the boys are immersed in society and learns how it functions. He learns to deal with physically healthy peers while conversely the other children can learn how to deal with others who do not have the same opportunities as themselves.
Of course, the motor function limitations demand a particular social and dynamic attitude from the school. For example, children with DMD cannot be in a classroom where there are many stairs or where there are no wheelchair accessible toilets. There are helpful tools (such as sturdy chairs with armrests to support posture) the can make accommodating these children easier. With creativity and effort, the motor restrictions should not be too problematic, particularly in kindergarten and primary education. Children with DMD are also entitled to GON counselling and this supervisor can also help with school integration. While the vast majority of Duchenne students attend regular main-stream schools there can be reasons for transferring a student to special education, for example when intelligence, learning difficulties or behaviour problems give rise to developmental stagnation.
Dystrofin in the brain
Recently it has been discovered that dystrophin (the deficient protein in Duchenne) is also found in a number of areas in the brain. Dystrophin is found in the hippocampus, that plays a role in memory, in the cerebellum, that controls coordination, and in the prefrontal cortex that controls planning and organisation.
In the brain, dystrophin is important for synaptic information transfer. Without dystrophin, the above-named functions do not appear to work as efficiently as normal.
Intelligence and Duchenne
Intelligence is the ability to act purposefully, think rationally and deal effectively with the environment. An Intelligence Quotient (IQ) test is a way to determine a value for intelligence. Such tests can be administered by the CLB. The average IQ in the population is 100 with a standard deviation of 15. This means that approximately 70% of the population has an IQ between 85 and 115. A score of 90 – 110 is considered average intelligence.
A large-scale review of all Duchenne intelligence studies over the past 40 years included over 1200 boys showed that the average IQ was 80 points. This is considered to be low to average. Thirty percent of all DMD boys have an IQ score of 70 or lower indicating that there is a significant intellectual disability. There appears to be a wide variation in IQ among DMD boys and scores can vary from 14 to 134 points. Therefore, there are some boys with DMD with very high to very high intelligence scores.
The total IQ score is subdivided into verbal IQ (VIQ) and performing IQ (PIQ). The VIQ covers language and reasoning in terms of language (like matching words e.g. piano and trumpet are similar). It is given orally through questions and answers and determines part of the intelligence score. The PIQ covers visual thinking and motor skills. It is the deliberate action part of intelligence.
Contrary to the decline in muscle strength, the intelligence of boys with DMD remains stable. The VIQ score is significantly lower in boys with DMD younger than 9 but after the age of 9 it increases until it matches the score of the PIQ. Language is more problematic at a younger age than an older age. Language-related problems tend to decrease was DMD boys grow older.
Cognitive profile of DMD boys
While not all children with DMD are faced with cognitive problems, it has been shown that specific cognitive issues can be associated with DMD. While every child is unique, there is a certain neuropsychological and cognitive profile seen that is independent of their IQ and degree of motor skill limitation. There are three major areas where problems can occur. It is possible to deviate from the profile below this is a risk of underestimating or overestimating the child with DMD.
Late to speaking
Having difficulties finding words
Having a hard time expressing themselves
Having difficulties making fluent sentences
Difficulties speaking words with three or more syllables.
These signs indicate a specific language developmental disorder that could lead to reading problems later on.
Short-term memory should briefly store information that you do not need permanently. The memory is also used to understand language and plan behaviour. DMD boys can struggle remembering both what they see and what they hear. For example, when a complex assignment is given, much of the message is lost and will not be executed (E.g. take your reading book and your calendar and record your task for tomorrow in your calendar).
In internal study of the Duchenne Parent Project showed that more than 18% of DMD boys suffer from concentration problems compared with 8% of the general population. This can affect school performance. It is important to realize that boys with DMD have a wide range of health problems ranging from fatigue to pain. The fatigue itself can also result in concentration problems.
Boys with DMD also have their strong points. It is known that they often function better in visual and spatial tasks that a more normally developing child. They are therefore often strong in visual thinking and drawing skills.
The first common problem is that of language. The child will find difficulties speaking in addition to the weakness of the speech muscles. Slow speech development is often diagnosed before Duchenne.
Language, memory and concentration issues can make it difficult for the boys to:
Finish: they do not complete their tasks
Start: they do not know how to start properly.
Transitions: they find it hard to switch from one activity to another.
Time-management: they cannot estimate time very well.
Flexibility: the cannot adapt easily to changes.
How do we cope with these issues?
First of all, it is important to understand that these problems are related to the functioning of the brain, and is largely beyond the control of the child. When a child with DMD does not respond to a complex verbal assignment which they perform only partially or when they have trouble performing or stopping an action on command, it is often seen as a lack of motivation. It can appear as rude or that the child is disinterested when they do not respond to what was said. This can happen particularly when the child is already in the middle of a task. These problems all relate back to the three main areas of language, short-term memory and concentration.
It is important to encourage and assist the boys in finding strategies that work for them personally.
A few general tips:
Ask the child to look at you before you give instructions.
Use short sentences and not too difficult words. Share more complex information or instructions in shorter pieces.
Provide visual support (for example, show the book at the instruction of a reading command)
Verify that the command has been understood by asking them to repeat.
Merge important information and repeat the core elements.
Share information and repeat. That's essential, especially when learning new things.
Provide additional time for assignments if necessary.
Provide more frequent but shorter work periods instead of one long working period.
Provide a quiet work space without distraction.
Teach the child to make "to do lists"
Help them to make a daily schedule and learn to work with an agenda
Learning disabilities and Duchenne
The lack of dystrophin appears to give and increased chance of learning disabilities. Learning disabilities are seen when a person with normal IQ with sufficient instruction still has problems with reading (dyslexia), counting (dyscalculia), spelling and writing (dysgraphia). It is related to difficulties or failure to automate functions, presumably related to the lack of dystrophin in the cerebellum. Children with DMD may also suffer from poor coordination that is not directly related to the muscle weakness. A learning disability is not something the child will grow out of but it is possible to learn how to cope with the problem.
Practice helps. Repeating and practicing skills for example 10 minutes a day, 7 days a week is better than 35 minutes twice a week. The school team (CLB, learning support and GON teacher) play an important role in recognizing the learning needs and providing guidance.
Dyslexia and Duchenne
In the total population dyslexia occurs at approximately 3% in primary schools, but in children with Duchenne the prevalence increases to 40%. Of these, 20% have a moderate disability, 20% have a more serious reading disability. The problem in dyslexia is learning to associate the sound and letter. Sound awareness is the most important building block for learning and can be trained early. From childhood, you can play rhyming games, listening games, stories, poems and playing with sentences and words. Reading and learning moments should be fun. Involve the child in the choice of the book.
For children with DMD, reading is a very important skill. As the muscles weaken, the child will depend more and more on the computer and therefore written language. It is therefore important to plan and invest time in this. Reading problems must therefore be detected and addressed as quickly as possible. Further information can be found here:
Less is known about dyscalculia but it is estimated to be present in 2-4% of the general population. According to the Duchenne Parent Project in the Netherlands, it is thought to be present in 10% of children with DMD.
In children with dyscalculia there is some insight and understanding. The problem lies in integrating counting skills. Exercise helps here too! Particularly if practiced briefly and regularly. Visualizing mathematical concepts can also be a great help. This can also be done with a work plan. Tools like a calculator can be permitted in higher years of education but it is best to teach only one solution strategy instead of multiple, to avoid confusion. Further information can be found here:
Since most Duchenne boys quickly transition to computer due to their motor problems, little is known about their possible writing issues.
Duchenne and behaviour
There are no specific personality traits associated with DMD. As mentioned earlier, each child is unique and, like the other students, there is a wide variety in personalities. Some are quiet and reserved in nature, others are open and exuberant. However, there are a number of features that appear more pronounced than normal. Parents often notice some inflexibility in thinking and a rigid attitude. From early on, frustration plays a big part in their lives. Being unable to keep pace with their fellow students in the playground, in sports and games, and possible confrontation with learning disorders, can make their school life difficult and have a major influence in their self-esteem. These frustrations can make the boys irritable and prone to anger. Fatigue and the side effects of the medications (particularly corticosteroids such as calcort or prednisolone) can also play a role in this. The rigid attitude could be a form of defence against a world beyond their control.
Students with DMD, just like other students, must comply with school and home rules. It is very important that they, just like everyone else, be held accountable for their actions. That is a key part of a healthy social education. However, a very strict approach to discipline can have an adverse effect and can escalate bad behaviour earlier.Try to keep in mind that what looks like bad behaviour (e.g. ignoring an instruction) may result from a cognitive weakness (see cognitive profile) or may be a reaction to frustration and adaptation problems to the consequences of disability.
As a teacher you, together with parents, play an important role in helping adaptation to, and acceptance of, Duchenne's disease (psychosocial adaptation). A good psychosocial adjustment is extremely important in preventing behavioural and emotional problems and safeguarding the happiness of the child. By being an understanding teacher, and providing support and consistency in word and deed you can play an important role in psychosocial adaptation. Through a practical problem-solving approach to difficulties, you achieve better results than with a strict, punitive approach.
Psychosocial adaptation for boys with Duchenne
Psychosocial adjustment is the ability to adapt to the difficult and stressful events such as having a disease like DMD. A patient questionnaire however showed that the vast majority, 83%, were well adjusted to the effects of their illness. In addition, DMD boys score better in psychosocial adjustment as they grow older. They learn how to deal with their disabilities, even though they are progressive! Between 8 to 10 years old are associated with the lowest adjustment score. At this age, boys begin to realize the effects of their illness. Walking becomes increasingly difficult and falling occurs more frequently.
Six functional domains play a major role in psychosocial adaptation. These are: relationships with friends, dependence, hostility, productivity, fear / depression and withdrawal. Studies have shown that relationships with friends become worse as the boys grow older. Here it is very important that parents and teachers explicitly promote and stimulate friendships. The scores associated with fear, depression, and withdrawal remain stable as they grow up. In fact, the scores for dependence, hostility and productivity improve with age.
When people feel unable to control their environment and what is happening to them, it can give rise to what is known as "learned helplessness." A degenerative disease in particular can have this effect. Understanding the mechanism behind this is an important aspect in dealing with boys with DMD.
If a certain action results in noticeable positive feedback, this can lead to learned helplessness. Despite the many improvements in physiotherapy, a boy with DMD will feel his motor capabilities decrease. This can lead him to lose his courage and give up. The learned helplessness can result in emotional reactions (apathy, depression,) and / or behavioural problems (passivity, lethargy,).
Parents, teachers and therapists can try to prevent this by using a conscious strategy in dealing with boys with DMD. They can try to choose activities that the boys themselves can perform where they get an immediate positive feedback. This strengthens their sense of control and motivates them to continue to develop in different ways.
Some boys may have problems with their mental flexibility. This means that they can be rigid in their thinking. They are often of one mind and do not deviate. This also means that they are often struggling with transitions in activities, changes in expectations, and that they can be quickly frustrated if things go in a way other than they expected.
The follow tips can help:
Make it clear that the rules apply to them consistently.
Set clear limits and expectations in behaviour.
Prepare for new events: Explain what will happen and what is expected of them.
Do not use vague terms like “be good”.
Be positive and appreciative when they do well.
Prepare a warning that there is going to be an activity change (eg: in 5-minutes we will brush our teeth and sleep or if when the timer goes off, we will clean up.)
Emotional problems are a normal part of growing up. In addition to normal stress situations, DMD young people are also prone to illness-related stress.
Really listen to the boy. Try to see it through their eyes.
Help the boy learn to recognize and name his emotions. By naming them they can become more manageable.
Solve problems together. Search for a solution together or find a few options and let him chose.
Set limits to his behaviour. Make clear that his feelings are not a problem, but some behaviours based on those feelings are not acceptable.
Duchenne behavioural disorders
Attention deficiency and hyperactivity disorder (ADHD), autism spectrum disorders and obsessive-compulsive disorders appear to occur in Duchenne muscle dystrophy slightly more frequently than in the general population. Nevertheless, one should not lose sight of the fact that most of the DMD children do not have these conditions. Be careful not to jump to premature conclusions. If in doubt, the child can be tested at a specialized centre. These concerns can also be discussed with the treating clinicians of the NMRC, which may be referred to for testing.
Autism spectrum disorder (ASD)
ASD occurs in 3% of DMD boys compared with 1% of normal population. ASD causes disruption in three areas particularly:
1) social interactions
2) verbal and non-verbal communication
Typical possible behaviours include:
• Limited interests and repertoire of activities
• Repeating the same behaviour (stereotyped behaviour)
• Difficulty with change
• Focused on details
• Focused on themselves
• Cannot move or move others
ASD is neither preventable and nor is it curable. The problems persist throughout life. An important point is to adapt the environment to the person with ASD. Treatment is primarily aimed at preventing exacerbations.
Further information can be found here: http://www.eurekaonderwijs.be/leerstoornissen/autisme
Attention deficiency with hyperactivity disorder (ADHD)
A study showed that 11.8% of boys with DMD receive ADHD diagnosis versus 3% to 6% in the normal population. ADHD is also the most common behavioural disorder in children. There are 3 types, namely a hyperactivity disorder, an attention disorder (ADD) and a combined type.
In ADHD, there are problems with:
• Emotional regulation (self-control)
• Stop single-use behavior
• Flexible thinking
• Planning and troubleshooting
• Learning own mistakes
Further information can be found here:
Obsessive-Compulsive Disorder (OCD)
Obsessive compulsive disorder is categorised as one of the anxiety disorders. It is characterized by compulsive thoughts and forced acts designed to relieve fear and worries. This may explain why OCS is more common in boys with progressive disease like Duchenne. In the normal population, OCD occurs in 2.3%. In DMD this is about twice as frequently. But this means that it occurs only in a small percentage (4.8%).
Talking about Duchenne at school
For both parents and children with Duchenne, confidentiality is very important! For parents, it is often very difficult to figure out what exactly they tell their son about his illness. Do not just assume that the boy knows everything about his diagnosis and what it means. A meeting before the start of the school year is important to discuss with the parents how to deal with the condition in the classroom and what is told to the other students. For older students, it is also important that they agree with what is told to the class. They must be left free and should not experience any pressure to say anything. In addition, they must be able to choose whether to be present at this conversation or not.
A general discussion about diversity can be a good reason to address this topic. In younger children, it can be said that everyone has their own needs. Some need glasses to see properly, others need extra help to learn to read or write, some need a wheelchair because their muscles are not strong enough. Young children do not have to receive too many details and certainly no future references.
Children who understand what is going on, can help and defend their classmate rather than bullying. An open, honest communication within the confines of the parents agreed confidentiality and sensitivity is therefore essential! It may also be possible to assign a buddy that helps open up heavy doors, carry book bags, ... This can easily be made into a kind of job.
Addressing practical challenges in the classroom
Since Duchenne is a progressive disease, needs will change over the years. Therefore, we divide these issues out by age. The progression of the disease is not uniform and can be faster or slower in some boys than others.
Ages 4 to 7:
Pulling themselves up into a sitting position
When needed, make sure your student can grab something to support them when they try to sit up. This is particularly important in toilets. A Certainly in toilets this is not an unnecessary luxury. A large toilet space can also be handy. Maintaining balance and sitting up are much more difficult for a child with DMD.
Standing up from the ground
Standing up from the ground is very difficult and tiring for them. The best support is a sturdy, well-supporting chair with armrests. Even so, the number of activities they are asked to do on the floor is best limited. When there is an activity on the ground, try not to isolate the boy by placing him alone on a chair.
Lifting objects, opening heavy doors, carrying bookbags, ...
A student with DMD is already experiencing muscle weakness and can easily lose his balance. A buddy can help him if needed. Try to make the 'buddy' job something fun and special so it will be a job and not a chore. It is also important to listen to whether the child asks for help. Sometimes they will want to do certain things themselves, which is important for their own self-worth.
Walking long distances
Try not to force a student with DMD to walk long distances. Keep in mind that 'long' has a different meaning here than for a healthy person. A pupil with Duchenne uses up his energy much faster than a healthy person. Their energy should be stored for playing and participating with the other children in class. A wheelchair can be used for covering distance, even when the student is still able to take steps. Most children want to play so much that they often go beyond their limits. If they have to exert themselves even more, for example to travel a short distance from a playground to a car, this may not be possible. They may then suddenly complain about pain in their legs. This may appear that he is trying to exploit the situation but this is not the case. The desire to play drove them too far and when that motivation goes away, they can suddenly "feel" that their energy is gone. A child with Duchenne may want to move but cannot force his muscles because they do not recover well.
At every age, stairs should be avoided for children with Duchenne. Stairs increase stress on the muscles, causing muscle damage to accelerate. Make use of elevators or sloping surfaces wherever possible.
Play time and physical education
Maintaining balance and climbing is difficult for children with DMD. Parents, counsellors and physiotherapists can often give advice on what can be done and what cannot. Often the child himself with have a good assessment of what he can do. They tend to fall a lot and often become cautious. Let children play but do not force them. Falling presents greater risk to them than for other children.
Special hand grips for pencils
These can be a great help for writing
Ages 7 to 11:
Changes in posture
During this time, the pelvic muscles become weaker. As a result, balance becomes increasingly difficult. The boys will compensate for this by adjusting their center of gravity. As a result, his posture changes when he walks and he will look for support from the wall. This often seems unusual to other students, which can lead to questions and comments. A sensitive conversation between student and classmates help ease this tension.
Getting up out of a chair
This also becomes more difficult and the boys will often need of something to hold when getting of a chair. In the toilet, this is particularly important.
Getting up off of the ground
This is now really difficult for him and is important to keep in mind. Do not try to isolate the student, but think about an activity in which everyone can participate equally.
It's important to provide age-appropriate information to your students so that understanding, inclusion and friendships are encouraged. Keep the confidentiality agreement with the parents in mind though.
Because of the increasing muscle weakness and the disturbed balance, it may not be possible to participate in a normal way. It might be more useful to schedule a physical or occupational therapy session during this time. It is difficult to think of other ways to include them in these sessions.
Difficulties with long note-taking sessions
The student may have to use a computer in the classroom.
Slower in setting up for class
Getting and preparing books or closing books needs more time because of the muscle weakness. Keep this in mind. A buddy can also help with this.
Decreased breathing function
Breathing becomes more difficult, and at night there may be oxygen shortage. This can result in symptoms such as headache, concentration disorders and fatigue. If you notice this, please report it to the parents in case they have not noticed them. It may be necessary to intervene with night breathing support. Be aware that colds and flu, due to the reduced respiratory muscle strength, can lead to pneumonia. A DMD student therefore will stay at home sooner to take care of these diseases.
Try to provide adequate space so that a wheelchair or scooter can enter the classroom easily. Hallways can be very busy, so you can consider letting him in and out of the class before the rest of the students. If you go on a class trip, try to plan a trip that is wheelchair accessible.
Stairs should be avoided. Sometimes it is difficult for boys to accept help at this age even though they need it. If he still persists in trying to use a staircase, for safety reasons he should be accompanied by an adult.