Wednesday, 19 January 2011

CORNELIA DELANG SYNDROME


A rare syndrome - possibly as rare as 1 in 30,000 children.  The child has microcephaly (small brain) with its associated  central processing difficulties. They also have  severe high myopia and nystagmus, so their vision is always pretty poor. You can look up the symptoms on the web but I have been working in a class with a teacher who has a child with CdLS this past week. I have a couple of thoughts for the teachers and anyone else who works with these children. So I am sharing my comments for anyone who is interested. Feel free to contact me about my comments.


Harriette (a pseudonym) is about 15 years old and she was initially lying down in a corner on a cushion and rug. when I came in. She had just been changed and was a bit uncooperative no doubt thinking, ‘leave me alone’. CdLS children tend to be reactive. That is they have some distinctive somewhat aggressive or anti-social behaviours that may be provoked by something happening to them. In this case it was having her private space invaded and it left her wanting to lie down and withdraw into herself. Once the circumstance is over however they can get back to normal.


There was a good attempt to get Harriette up and walking or marching for a PE lesson to the tune of ‘I will walk 500 miles’. A great song by the way and made me feel like marching. She seemed quite engaged for a little while, though it looked an effort to hold her as she walked. She is hard to keep sustained on a task. Her attention span is limited of course. 


The first thing I would say is that my comments are not criticism. There is no right or wrong way and the teachers and TAs are the experts because they know the kids better than I do. But what I have found in the past is that as I talk with TAs and teachers I am a catalyst for their own adaptations. I bounce ideas off them and make suggestions and then they go away and think about how to incorporate that into their work. Working with children like Harriette is not a precise science but also it’s not exactly ‘rocket science’. There are basic principles and a lot of it is about understanding how the brain works and how brain damage affects learning. Once you get hold of how different the way they process sensory information is then it will have an impact on whatever the teacher does.  A lot of it is common sense once you have an idea of the problem.

The commonest part of the brain that gets affected in these kids is a part called the parietal lobes. That’s because it is the furthest part of the brain away from the heart and blood takes the longest to get there. So anytime the heart stops in and around birth oxygen fails to get here and cells die. The cerebral cortex has four pairs of lobes each with different functions. The occipital lobes (vision - visual cortex) are at the back and the parietal lobes are over the top. The parietal lobes deal with making sense of all the sensory information that the brain receives. It is the sensory integration region. It also integrates the senses with motor skills and functions. So it is possible and common that a child with damage here will not be able to coordinate movement and vision. So visually guided movement is difficult. A prime example is cerebral palsy. Reaching using vision alone is challenging for these kids. They will look and turn away and use their hands to find things.


Back to Harriette. If sensory integration is challenging then it is hard to make sense of complicated sights and sounds or sensory info coming at her simultaneously. So what I would say is sensory info should be simplified and sequential. That is things should be slowed down dramatically and presented to her one at a time. She cannot process things simultaneously just we would have a problem listening to two people at once.


The two key things for this week for teachers to remember

1.     Slow things down – speech, movement, events. Try slowing your speech down; it may sound funny at first but with some kids it can have a dramatic effect. Anyway even if you don’t see a response there can be something going on inside.
2.     Events to be one at a time or one after the other. Say something and wait for her to respond, or show her something, then say something again. People should not speak at the same time near her.



That’s it for today. Have a great day!

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