3P25 Deletion Syndrome.

3P25 Deletion Syndrome. - Today we welcomed back a two year old little girl for her second
assessment. She has an exceptionally rare genetic disorder known as 3p25 deletion syndrome. This is so rare that since the first person was reported in the medical literature in 1978, only around 50 people with a 3p25 deletion involving no other chromosome(s) have been described in the medical literature. Today after 10 months on the Snowdrop programme she had made some very significant developmental gains. Her visual ability has moved from the 4 month level to the 12 month level. Her auditory development has moved from the 8 month level to the 18 month level. Gross motor skills have improved from not even sitting, to standing against furniture, - she is well on the way to developing a standing balance. Language has moved from the 7 month level of babbling, to the 14 month level of possessing two words of speech, - 'dada' and 'go.' Socialisation has moved from the 8 month level to the 18 month level. It just shows that genetic expression can be fought against. Well done to mum and dad who have brought about this dramatic change!

Autism, Sensory processing and Social Communication.

One of the defining attributes of what the world likes to call 'autism' is problems with sensory processing.  I have yet to meet a youngster with autism who does not have some sort of sensory processing issue, be it visual, auditory, tactile, etc, or indeed all the senses.  This is so important because it is these sensory input channels to the brain which dictate the way in which we see, hear and feel the world around us.  This in turn then influence what a child produces by way of the output pathways of gross and fine motor behaviour, vestibular behaviour, fine motor function, language, social communication and emotional behaviour. 

Today we welcomed a four year old little boy and his family from eastern Europe for their first assessment. The little on has a diagnosis of autism, but his major problems lie in the areas of sensory processing and social communication. Of course these two areas are linked.  Because he experiences auditory hypersensitivity, he doesn't like to interact with people he doesn't know, in particular with children.  Children are unpredictable and produce many sounds at the frequencies to which he is sensitive.  His visual magnocellular pathway is under-active, (this pathway helps us to notice movement and aids with visual accommodation) which is why he is focussed on objects which move, in particular objects which spin, (he is unwittingly trying to activate that pathway by creating more and more movement).  Some children with problems here will sit and wave their hands in front of their eyes in an attempt to stimulate this pathway.  This is also why he holds objects close to his eyes in order to observe them.  He spins himself around and spends time upside down in a clear attempt to stimulate the vestibular pathways, which he needs because his balance is very poor, (his brain innately understands this, hence the self - stimulation. - The brain shows us what it needs, we just need to observe)!

He was a delightful little chap who has lots of possibilities. Our first steps, as always are to try to improve sensory processing, because as I say, if the input channels which feed information into the brain are not working correctly, then neither will the output channels because they are operating on faulty information. Let's see how he fares on the Snowdrop programme.

Snowdrop. - Who are we and what do we do?

Our story begins 29 years ago with the birth of our own son, Daniel.  My wife's pregnancy had been described as being a "textbook pregnancy," but unfortunately it was not a textbook birth and Daniel had been delivered dead by emergency caesarean section having suffered severe oxygen starvation during labour.  It took the medical staff 20 minutes to resuscitate him and by the time they did, the brain injuries which he had sustained during a mismanaged labour had been compounded.  As they rushed him to the neonatal intensive care unit he was already suffering multiple seizures and we were later informed that his injuries were so severe that he was not expected to survive 24 hours.  An EEG then confirmed his brain activity as being a 'pre-terminal event.'  I remember every minute of those 48 hours as though they happened yesterday.  I remember a little baby boy who fought dearly to live.  As he did throughout his life, he fought everything, sometimes even beneficial things and so it was that every time they put a line into him, he ripped it out.  It was sheer determination that he survived those bleak hours and a neonatal medical and nursing team to whom I owe a great deal.

The next few days were very traumatic but Daniel did survive and eventually we were allowed to take him home.  We were told that he would be very unlikely to have escaped his ordeal without significant disability.  In fact he turned out to be blind, deaf and quadriplegic.  We were told that this was a situation which would never change, - a statement we refused to accept and we set out on a journey to find answers to his problems, - a journey which would take us all over the world.

Daniel hardly slept and this was gradually wearing Janet, my wife down to the ground.  We decided that she needed help and that if we were really going to solve Daniel's problems, that I should give up my career, so that I was available at home to take some of the strain. We also decided that I should enroll at university to study psychology and child development so that I was learning more and more about Daniel's problems.  The degree I enrolled on was full time, so at least we would be in receipt of a student grant, but it had the advantage that my lectures were so spaced out, that I could be at home much more than previously.

Somehow over the next 3 years, despite a profound lack of sleep, I managed to get a degree based in psychology and child development and this combined with other things we were doing meant that we had been able to bring back Daniel's sight and hearing, much to the initial shock of the medical profession, however this shock soon changed to the position, agreed amongst themselves that "it would have happened anyway."  Yes of course if we had done nothing, it would have happened anyway.

So, over the years we managed to give Daniel a good quality of life, using help from various therapies and our own increasing knowledge.  However, at age 13, Daniel suddenly developed an unsafe swallow.  This was a setback because his weight was always judged to be on the 'light side' and so the decision was made to insert a naso-gastric tube.  This did have the desired effect of him putting on weight, however another more sinister complication arose, - he started to display signs that he was entering puberty.  This obviously had the effect of releasing various hormones into his system and the poor little mite just could not cope.  He began to experience brainstem seizures which despite all their efforts his doctors were unable to control.  Then when he was 15 he suffered a brainstem stroke.  Over the next weeks he began to decline and a couple more of these episodes ensured that there would be no recovery.  On the morning of the 21st of December 2003, I saw his eyes close for the final time and at 4-40am we lost him.

As you may imagine, over the next few months, we were devastated, (in fact we still are, losing a child is something you never get over). We wanted nothing to do with the 'special needs world' we totally rejected it.  However, eventually I decided to finish my studies and returned to that world to supplement my degree with post graduate qualifications in 'language and communications impairments in children' and ultimately a degree based in neuroscience and child development.  In August 2008 Snowdrop was born.  We called it Snowdrop because on the morning of Daniel's passing, Janet went into the garden and saw the new life of the snowdrop bulbs just beginning to break through the soil.  We later discovered that the Snowdrop is an international symbol of hope through adversity, - a fitting metaphor.  Initially we had one child on our programme, but today we are bursting at the seams with hundreds of families using our programme from all over the world. We have children on our programme with a wide variety of developmental problems, ranging from 'specific language impairment, through to sever cerebral palsy, autism, ADHD, sensory processing disorder and much more.  We also treat a wide variety of sometimes rare genetic disorders.

Our success has outstripped my wildest dreams.  We have children seeing, hearing, feeling, walking, speaking and much more, who previously were unable to.  This is not just empty talk of success which cannot be substantiated, I can actually introduce you to the families whose children have been helped.   When I was an 18 year young laboratory technician starting out in the world, I never thought my life would take this turn.   If you want to learn more about us, simply visit our website at http://www.snowdrop.cc or you could visit our Facebook page or simply email us at info@snowdrop.cc

ADHD and Sensory Processing Disorder.

Today, to end a busy week, we met another lovely new family to the programme with a little boy who is just much too physically active and just cannot stop.  He has extreme ADHD.  He also has huge sensory processing and attentional issues. underneath it all is a beautiful, intelligent little boy. We shall endeavour to lead him out of the world in which he is trapped into a calmer, organised world of normal sensory experience.  We have the techniques to slow him down, so that he can then develop and use his attentional abilities properly.  Only then will he join 'our world' and begin to learn the way he should.  Watch this space!

The Reticular Formation and Sensory Processing.

We are constantly taking in information from the environment through our senses. It is something we cannot help but do and we use this sensory information to each construct our version of reality. But what is reality?

None of us really have any idea what reality is actually like: all we have is a limited sensory system, which interprets visual, auditory and tactile information and relays it to our conscious awareness. But people can only iterpret a small part of reality, being unable to detect, for example, radiation or broad colors on the light spectrum.

This is one reason why there is folly in totally accepting the world your senses provide you with. But there is another reason, one that you have more direct control over: the sensitisation of your reticular system and what it means for how you experience life on a daily basis.

The general rule of your reticular system is that whatever dominates your thoughts - both conscious and unconscious - will also dominate your attention, whether you like it or not. Ever had a toothache and then noticed that there seem to be an awful lot of adverts on TV about toothpaste and dentists? This is your reticular system at work. When a mother has a baby, she becomes acutely aware, even in sleep, of every noise her baby makes. - This is her reticular system at work, - tuning attention to what is dominating her thought processes.

Now let's consider what happens when the functioning of the reticular system is not as it should be. Many children suffer from sensory oversensitivity, whether it be visual, auditory or tactile; - or all three! This might present itself as a general oversensitivity in the affected modality, or a more specific oversensitivity, such as being oversensitive to specific sights, sounds and / or sensations. This is again the work of the reticular system, (inconjunction with the thalamus) Because of a dysfunction within the brain, whether caused by genetics or brain injury, the reticular system of the child becomes sensitised to particular stimulus, whether visual or auditory, etc and works in conjunction with the thalamus to excite the cortex so that the stimulus is processed. However, because of the dysfunctional reticular system, the cortex becomes over-excited and the child, not understanding why the stimulus is triggering this reaction in his system, reacts wildly. Here we have the basis for sensory oversensitivity in many types of developmental disability, including cerebral palsy, autism and Asperger's syndrome. or any other type of brain injury.

Fortunately, these neurological structures can be re-tuned, as they constantly are in uninjured human being, as our awareness and attention are constantly redirected to salient features of our environment. Snowdrop has developed techniques to help children who suffer from this type of difficulty to re-tune the dysfunctional reticular formation, thus allowing the opportunity for normal developmental processes to resume.

If you would like more information about Snowdrop's treatment programmes for brain injury, visit http://www.snowdrop.cc

Dyslexia; - A failure of sensory attention?

I see this inability to 'tune out' extraneous stimuli from the environment in order to focus on a specific task or feature in children with many types of developmental disability. I talk about it in my book, where I call it 'wide spectrum tuning,' but I have never seen it in anyone with dyslexia. An interesting article apart from them going on about dyslexia having one cause! We already know of two!, - injury to part of the cerebellum and injury to parts of the 'magnocellular system.' Snowdrop provides therapy which helps to retune these attentional systems to more normal levels.

With thanks to Medical News Today. 

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Dyslexia affects up to 17.5% of the population, but its cause remains somewhat unknown. A report published in the online journal PLoS ONE supports the hypothesis that the symptoms of dyslexia, including difficulties in reading, are at least partly due to difficulty excluding excess background information like noise. 

In the study of 37 undergraduate students, the researchers, led by Rachel Beattie of the University of Southern California, found that the poor readers performed significantly worse than the control group only when there were high levels of background noise. 

The two groups performed comparably at the prescribed task when there was no background noise and when the stimulus set size was varied, either a large or a small set size. 

According to Dr. Beattie, "these findings support a relatively new theory, namely that dyslexic individuals do not completely filter out irrelevant information when attending to letters and sounds. This external noise exclusion deficit could lead to the creation of inaccurate representations of words and phonemes and ultimately, to the characteristic reading and phonological awareness impairments observed in dyslexia." 

Sensory Processing Difficulties.

Although the sensory system is very complex and its correct development is vital, this post is only able to provide a brief guide. I will however highlight the major problems, which children who suffer brain injury face in the developmental areas of vision, hearing and touch.

We take in information about our environment through our senses. This is something, which we cannot help but do. The amount of sensory information our brains are processing at any one moment is phenomenal. As I sit here typing for instance, I am aware of several sensory stimuli. Visually I see the computer keyboard, with the letters printed on the keys; I can see my hands, the desk, the computer screen and more. In my peripheral vision, I am aware of the window, my dog, the sofa and other items in the room. Auditorially (hearing), I can hear the kettle beginning to boil, I hear my fingers tapping on the keys of the keyboard and I hear my cat mewing. In terms of touch (tactility), I can feel the keys of the keyboard; feel the wooden floor beneath my feet etc. These are merely the things of which I am aware and these sensory stimuli are all being processed simultaneously, in a fraction of a second.

As an example of this processing, consider the complexity of my typing this text, which hopefully you are reading with enjoyment! The front part of my brain (the frontal cortex) is sending out messages to the motor parts of my brain (the motor cortex), which control my hands,

instructing it which keys on the keyboard I need to hit next in order for the written words on this page to make sense. The motor cortex then instructs the hands to move in order to hit those keys. Parts of the brain known as the basal ganglia and cerebellum then become involved in

order to attempt to execute the necessary movements of the hands in a fluent and accurate manner. When the movements have been executed, feedback signals are then sent back to the frontal cortex, via the ‘cerebellum’ and ‘basal ganglia’ to inform it how successful the hands

were at hitting the correct keys on the keyboard and whether the movements were accurate and fluent. If necessary, the frontal cortex then issues new instructions, to correct any errors.

In a healthy, uninjured brain, this grossly oversimplified description of events all takes place within a fraction of a second whilst the brain simultaneously takes care of many other complex tasks. It is a phenomenal feat.

Also, consider how the brain decodes the various sounds we call language and how it regulates its own attention. Imagine you are sitting in your lounge holding a conversation with a visiting friend. There you are, happily chatting away; - you are attending to your friend’s voice

so that your auditory system is able to process the constant stream of noise, which we call speech. Your brain is able to take this constant stream of sound, break it down into recognisable chunks and attribute meaning to it so that you understand what it is your friend is saying. At

the same time, your brain is tuning out extraneous sounds in the background, such as traffic passing outside your window, so that you are able to focus on the task at hand. Your brain does all of this and much more, (this again is actually a gross oversimplification), with the

minimum of effort, without you even being conscious of the processes involved.

Now consider a brain, which is not healthy; - a brain, which has suffered injury and try to imagine the chaos, which might ensue for a child whose sensory processing system has been impaired. Imagine this child’s ability to ‘tune out’ noises, which he does not wish to pay attention to, has been impaired. What havoc would that child experience?

I believe that the sensory problems, which are faced by brain-injured children can be assigned to five categories, which we shall discuss later. Fundamentally it seems to me that many brain-injured children experience difficulties in correctly modulating incoming sensory information, their sensory system processing incoming stimuli in a distorted manner.’ I have applied these theories (which are all supported by evidence), to differing patterns of brain-injury: - And there are many patterns of brain-injury to which they can be applied! A particular pattern rarely has absolute, identifiable boundaries and symptoms from another. There is often a great deal of ‘overlap’ in the symptoms which differing patterns of brain-injury display. Allow me to explain.

A good example is my proposition that cerebral palsy, autism and ADHD are not distinct separable conditions, but are a continuum; they are overlapping expressions of brain–injury and consequently it is possible to have some symptoms of autism, or ADHD within what is termed cerebral palsy. It is also possible to have some symptoms of cerebral palsy within what is known as autism or ADHD.

I can already hear the howls of indignation over the fact that I have referred to autism as brain-injury! Have you not seen all the recent evidence, they will say, which points to the cause of autism as being genetic? Yes, I have seen this evidence and I accept that some forms

of autism have genetic causes. However, I have seen too many children with brain-injuries, who display amongst their repertoire of symptoms, many autistic qualities. Thus, I cannot ignore the fact that some forms of autism also have environmental causes. In other words, they are produced by brain-injury. I am also aware that genes can only express themselves in the correct environmental conditions!

It was Delacato in the 1970’s, who first claimed that children suffer distortions of sensory processing, separating them into the categories of ‘hyper-sensory,’ hypo-sensory’ and white noise. I have managed to identify five categories of sensory difficulties, which children display,

(other researchers may find more!) and I see these as symptoms of a malfunctioning ‘tuning mechanism’ in the brain. This ‘tuning’ mechanism is the structure which enables us to ‘tune out’ background interference when we wish to selectively attend to something in particular; it also enables us to ‘tune in’ to another stimulus when we are attending to something completely different. It is the same mechanism of the brain, which allows us to listen to what our friend is saying to us, even when we are standing in the midst of heavy traffic on a busy road. It is this mechanism that allows us, even though we are in conversation in a crowded room, to hear our name being spoken by someone else across that room. It is this mechanism, which allows a mother to sleep though various loud, night-time noises such as her husband snoring, or an aeroplane passing overhead and yet the instant her new baby stirs, she is woken. It is a remarkable feature of the human brain and it seems to be the responsibility of three structures operating cooperatively; - these are the ascending reticular activating formation, the thalamus and the limbic system.

Having made such a bold claim, allow me to furnish you with the evidence to support it. The three structures just mentioned receive sensory information from the sense organs and relay the information to specific areas of the cortex. The thalamus in particular is responsible

for controlling the general excitability of the cortex (whether that excitability tunes the cortex up to be overexcited, tunes it down to be under excited, or tunes it inwardly to selectively attend to it’s own internal sensory world.) (Carlson, 2007). The performance of these neurological structures, or in the case of our children, their distorted performance seems to be at the root of the sensory problems our children face.

I would label the five categories of sensory distortion, which I have witnessed in brain-injured children as follows: -

1. Sensory over-amplification. The particular sensory modality, (vision, hearing, touch, etc) can become oversensitive to stimuli from the environment. It is my belief that in this case, the thalamus, limbic system and reticular formation, which are acting as the brain’s ‘tuning

system’ are malfunctioning and are not effectively regulating the level of incoming sensory stimuli. Indeed, in this case they would appear to be acting to over-excite the cortex, which would have the effect of amplifying the sensory stimuli. This could possibly cause the child to

overreact, or to withdraw into himself as a defensive strategy from a world, which in sensory terms is simply overwhelming.

2. Sensory under-amplification. The particular sensory modality can appear to become under sensitive to incoming stimuli from the environment. In this case, I believe the thalamus and other two brain structures, acting as the tuning system, are acting to under-excite the cortex, which is having the effect of appearing to dampen down incoming sensory stimuli. This could influence the child to act as though he cannot see, hear or feel; - he may be deficient in this way, in one or more sensory modalities.

3. Internally focussed sensory tuning. In this case, the particular sensory modality appears to be ‘inwardly tuned.’. In this case the three brain structures, acting as the brain’s tuning system are exciting the cortex to attend to sensory information of the sensory system’s own making, or from within the child’s own body. Consequently, the child may have difficulties perceiving the ‘outside’ sensory world through this haze of internal stimulation. We see this effect ourselves in the visual aura of a migraine, or when we have 'pins and needles.'

4. Wide spectrum tuning. In this case, the three neurological structures are exciting the cortex and attempting to tune its attention to many incoming stimuli simultaneously. They seem unable to filter out background noise, sights, etc in order to allow the child to focus on one aspect of the environment. For this child, the world is absolute chaos and again, he often withdraws into himself.

5. Narrow spectrum tuning. In this case, the neurological structures are only exciting the cortex selectively, allowing the cortex to attend to limited, isolated sensory stimuli. This child may often seem ‘over-focussed’ on one particular aspect of his environment. He can for instance, become intensely interested with a spinning top or the particular features of one toy and will not play with anything else, to the point of seeming obsession. For this child, it appears his sensory tuning system is focussed too narrowly and he cannot spread his attention to incorporate several features of his environment simultaneously.

Can these problems be addressed? Yes they can. As part of its programme for children with sensory processing issues, Snowdrop creates an individually tailored 'adapted sensory environment' for the child to encourage his sensory system to begin to process stimuli on a much more normal level. Are we experiencing success with children who have sensory processing problems? Yes we are.

Anyone requiring more information should contact info@snowdrop.cc

References.

Beck, A. T., and Guthrie, T. (1956). Psychological significance of visual auras: Study of three cases with brain damage and seizures. Psychosomatic Medicin, Vol XVIII, no 2,

Carlson, N. R. (2007). Physiology of Behavior. London. Allyn and Bacon.

Haist, F., Adamo, M., Westerfield, W., Courchesne,E., and Townsend, J., (2005). The functional neuroanatomy of spatial attention in autistic spectrum disorder. Developmental Neuropsychology, 27, 3, 425-458.

Mulleners, W. M., Chronicle, E. P., Palmer, J, E., Koehler, P. J., and Vredeveld, J. W. (2001), Suppression of perception in migraine: Evidence for reduced inhibition in the visual cortex, Neurology, January 23, 2001; 56(2): 178 - 183.

Yang, T., and Maunsell, J. H. R.. (2004) The effect of perceptual learning on neuronal responses in monkey visual area V4. Journal of Neuroscience, 24, 1617 – 1626.

Snowdrop and 'The Listening Programme.'

Snowdrop is pleased to announce that in conjunction with US neurotechnology company ‘Advanced Brain Technologies,’ we are now able to offer our clients ‘The Listening Programme.’ (TLP).

The Listening Programme is a music listening therapy which provides engaging brain stimulation to help ameliorate a range of problems which our children face.

Systematic training is provided through listening to psychoacoustically modified classical music which trains the brain to process sound more efficiently. This can lead to improvements in:

• Learning
• Attention
• Communication
• Reading

• Listening
• Sensory Processing
• Social Engagement
• Behaviour

• Self Regulation
• Musical Ability 

Children, teens and adults can use the programme in the home, classroom or workplace. Completely portable and easy to use, The Listening Programme fits easily into anyone's schedule and only requires 15 to 30 minutes of daily listening.

The Listening Programme is a fusion of beautiful art and sound science. The masterful performances of the award-winning players of the Arcangelos Chamber Ensemble are skillfully crafted using advanced audio technologies to provide an unrivalled listening experience.

How can something that seems so simple - listening to psychoacoustically modified classical music - actually impact a wide range of abilities, such as sensory processing, reading, communication, learning and memory?

Numerous studies worldwide, including research at the University of Sheffield, provide empirical evidence that substantiate The Listening Programme’s efficacy and credibility.

The Listening Programme’s psychoacoustically modified music and production techniques are designed to stimulate or “exercise” the different functions of the auditory processing system. This enables the brain to better receive, process, store and utilise the valuable information provided through the varied soundscapes in our lives such as music, language and the environment in which we live.

THE EAR BRAIN CONNECTION

So the question becomes...how can we overcome auditory processing challenges - moving from a “disorganised” to an “organised” system?

Certain classical music, like that of Mozart, Haydn and Vivaldi, has specific structure, producing sound waves in organized patterns. Within these patterns are vital elements including time, frequency and volume. When listening to music, the ear is receiving the musical sound waves - waves that arrive in different frequencies, measured in Hertz (Hz). These frequencies stimulate the brain, and thus affect different functions of the mind and body.

SPECIFIC FREQUENCIES FOR SPECIFIC FUNCTIONS

The Listening Programme combines decades of clinical research in several fields, including neurology, physiology, psycho-acoustics, auditory processing, music theory and more. The method builds on the work of respected leaders in these fields, such as ear, nose and throat (ENT) physician Alfred A. Tomatis, M.D. (1920-2001). Among other discoveries, Dr. Tomatis helped identify the relationship between certain sound frequencies and their effect on their functions of the mind and body. A simplified explanation of Dr. Tomatis' findings shows that certain brands, or zones, of sound frequencies affect different abilities.

TLP is designed to address these zones, systematically providing auditory stimulation that, when customized for listeners by ABT Providers, can help improve their ability to function in a number of ways.

THE BASIC BUILDING BLOCKS OF AUDITORY PROCESSING ARE TRAINED USING THE FUNDAMENTALS OF MUSIC - FREQUENCY, VOLUME AND TIMING.

How much does 'The Listening Programme' cost?

Unfortunately, the cost is out of Snowdrop's control, but anyone who knows me knows that I would not be offering something unless there was evidence to demonstrate it's effectiveness.  That effectiveness has been demonstrated by my own university the University of Sheffield.

The cost of the 10 psychoacoustically modified CD's which make up the programme is £378.  The additional cost of Snowdrop controlling the programme on a fortnightly basis is an additional £15 per fortnightly consultation. 

The Listening Programme for Schools. 

The cost of TLP for use in a school is £600 plus fortnightly monitoring of the programme by Snowdrop at a cost of £50 per fortnightly consultation.