If you have children, (or you are part of a family), imagine
that you have celebrated the birth of your beautiful child,
and you have phoned all your family members to tell them of your joy!
As time goes on you have showered love and adoration on this delightful little person and watched them flourish and grow and learn, and then...
a few years down the track, you are hit with a diagnosis that means you will not get to see your child's graduation, or their wedding, or the birth of their children.
Your student is the precious child of parents who are dealing with this nightmare. In order to honour them, and to support your student, here are some things which you need to know:
Batten Disease is a disorder of the nervous system that begins in childhood. Early symptoms of this disease usually appear between the ages of 5 and 10, when parents or doctors may notice a previously normal child has begun to develop vision problems or seizures. In some cases the early signs are subtle, taking the form of personality and behavior changes, apparent learning disorders or motor difficulties.
As time passes, affected children begin to show signs of
intellectual disability, increasing seizure activity, progressive vision loss and impairment of motor skills. Eventually, children with Batten disease become blind, immobile and affected by dementia-like symptoms.
Unfortunately, at this time, Batten Disease is a fatal condition.
It's named after the British pediatrician who first described it in 1903. It is also called Neuronal Ceroid Lipofuscinoses (or NCLS). Batten disease is not contagious and, at this time, no cure is available.
How does the disease progress?
The student with Batten Disease usually begins to show some signs of motor difficulties, problems at school and
progressive vision loss between the ages of five to nine years. Within a few years, vision is completely lost and, for some time, this may be the only obvious problem. Over time, however, understanding and concentration become increasingly difficult and behavioural disturbances may become evident.
Epileptic seizures may begin at around ten years and, also at this age, erratic and challenging behaviour may manifest. This is due both to the disease process, and the
frustration, anger and confusion the student feels about their loss of previously-held abilities. The course of the disease can vary quite considerably and the student may seem quite stable for long periods but gradually, throughout the teenage years, physical and mental skills diminish, until he or she is unaware of his or her surroundings and requires an intensive level of care and support.
As painful as this is to witness, reports suggest that at this stage, the condition is not a painful one and your student will be unaware of what is happening in the later stages of the disease. Eventually the combination of the diseased brain and physical weakness becomes too great to sustain life and death usually occurs between the late teens and early thirties. As a parent, teacher or carer, you will be aware of your child’s increasing frailty, and death is usually relatively peaceful and expected when the time comes.
Some strategies which we found useful within the school environment:
1. An important transitional object or toy will often alleviate the anxiety of leaving for school each morning. This can be included as a strategy in the student's Individualised Learning Plan. A familiar item has been found to ease the student's anxiety and increase their comfort in making this transition each day.
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3. Utilise their Interest Areas:
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It is vitally important to your student's sense of self esteem, that they feel that they are achieving and meeting challenges. They clearly remember this feeling, and it is so important to their sense of self and their personality.
And of course, utilise these favoured activities as a reward, after completing work tasks.
4. Try spacing Braille with one space between every letter. As your student's tactile perception skills diminish, they may still be able to decode Braille more easily as each cell can be interpreted separately.
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5. Try using Audio Description Settings on favourite DVDs:
6. De-escalate potentially difficult situations by responding in the first instance with conversational topics which your student finds soothing or interesting. You know your student by now. You know how to engage their interest and comfort them. Don't push the boundaries of their tolerance levels. They are dealing with enough already!!
7. Allow a Retreat space:
When things become overwhelming, ensure your student has a quiet space in which to retreat, with favourite items. A Multi Sensory approach is recommended depending on the student's interests. You will know and understand those activities which your student finds pleasurable. (If not, PLEASE consult their parents: they are the major stakeholders in your Learning Support Team and can provide you with so much valuable information!)
Some students love to go out and throw a ball around. Others will need the kinesthetic stimulation of a trampoline or an exercise bike. Others will prefer a beanbag with their favourite movie. Build this into their Individualised Learning Plan, with their parents' approval.
8. Most importantly, be aware that your student's needs are constantly changing!
Appropriate activities and strategies are completely
dependent on the stage and progression of your student's abilities. It is vitally important to stay in clear communication with their parents, in regard to their current and ever-changing level of medication, their overall well-being, any additional stress in their life and the overall progression of this disease.
Your program will need to change frequently to meet your student's current needs.
9. Develop a Tactile Communication Program:
It is recommended that this program is developed and introduced to the student before the need is apparent, to ensure that they are familiar with the items and their meaning. This is a sample of the kit we developed for our students, using a sheet of black perspex and object reference symbols, which were attached using silicone - a visit to your local hardware store will supply these, or you can contact us to arrange a long-term loan.
A small felt indicator in the top left-hand corner indicates the orientation of the tile, and Braille was included, with a space between each letter to assist the student's deteriorating skills of tactile perception, while they could still decode Braille. (We made these using a hole-punch and some adhesive suede contact).
We also recommend displaying only 3 Tactile Choices at one time. We presented ours in a wooden tray to delineate the options available to the student.
It is important that real, meaningful objects, related to the student's experience are used, for example, we used a real toilet button to indicate our student's desire to go to the toilet. (Not shown as a student is currently using this to indicate his needs). Depending on your student's level, a piece of absorbent undergarment [nappy or diaper] could be used. Real life objects might be used to represent activities for your student also, such as a plastic shopping bag and cane, to indicate an outing.
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Please be alert to the following symptoms:
Epilepsy
In a normal ‘healthy brain’ there are many thousands of messages being transferred electrically by pathways to and from various centres that operate thought processes, movements and reactions on both an involuntary and voluntary level, consciously or subconsciously.
With Batten Disease, brain cells gradually deteriorate, which prevents normal nerve pathways from working by blocking routes on the path. The message then has to find different ways around the route and eventually the brain cells send more and more signals leading to lots of random energy being released in lots of different directions at once.
When this happens, periodically, the person will have seizures. Sometimes these will be clustered together or be lots of different types and the person will have ‘brainstorms’ that may last for several weeks, and be typified by increased seizures, hallucinations and agitation. During these ‘brainstorms’ quite large areas of the brain will be damaged and when they eventually settle, the person will have lost some of their abilities or skills – often the most apparent symptoms are impaired mobility and/or speech.
It is very important that all seizures are recorded accurately. This will be helpful in indicating how the disease process is working and whether the medication is effective or needs reviewing.
Hallucinations
The student who has Batten Disease will, from time to time, suffer periods of hallucinations. It appears that these hallucinations are closely related to the overall epileptic pattern of the individual.
We have supported students who were convinced that there was a gaping, dangerous hole in the floor, and another who was certain that spiders represented a real and present danger. Unfortunately, hallucinations are invariably frightening and very realistic. Although hallucinations, like seizures, will never be completely prevented, there are some general ‘management’ guidelines that have ameliorated these hallucinations from being too severe and prolonged.
Management Guidelines When People Are Experiencing Hallucinations:
Always record each hallucination. State clearly where they were and what they were doing immediately prior to the hallucination; what form the hallucination takes (particularly body language and facial expressions); how carers interact with the person during the hallucinations and how long the hallucination goes on.
The person’s hallucinations may worsen as they lose skills and abilities, particularly their ability to communicate and concentrate, so environmental factors may well antagonise hallucinations (e.g. noise, crowds and prolonged periods of the same activity).
If the student is experiencing an hallucination, we would recommend the following:
- 1. In our experience, it is more reassuring to appear to take the student's concerns seriously and let them know you are "dealing with the problem".
- It is much more difficult, and far less reassuring to tell them that it is all in their imagination. They are completely convinced that this situation represents a clear and present threat to their well-being, so it is simpler to reassure them that you are managing this alarming situation and that they will be fine.
- 2. Ensure the person is in a quiet environment with which they are familiar.
- 3. Ensure there is only one person talking to them during an hallucination. If other people are present they should remain silent as extra voices will mean more confusion.
- 4. Within your school team, identify how you will approach the person during hallucinations and adhere to this approach to maintain continuity. Speak to the student in a quiet, calm and reassuring voice and repeat the same messages rather than trying to engage the person in conversation. They may not be able to reply due to their anxiety and deterioration in their overall condition.
- 5. If, for their own safety, the student needs to be physically assisted during the hallucinations e.g. from one room to another, act quietly and apply team strategies, as above. Physical manoeuvres are best done quickly, with the person being quietly informed as to what is going on around them.
- 6. It is important that the person is not physically restrained during an hallucination as this will reinforce their fears. If the person’s safety is in doubt they should be allowed to be on the floor where they would be least at risk. Physical contact and reassurance should again be restricted to the main carer.
Recent developments:
In 2015, Charlotte and Gwyneth Gray, the daughters of Hollywood Producer Gordon Gray, were diagnosed with this rare terminal disease. As a consequence of this high profile family's diagnosis, many famous Hollywood stars have begun a campaign to heighten awareness of Batten Disease and are raising impressive amounts of funding for further research.
Some useful resources:
Understanding and Managing Behavioural Changes:
Understanding and Managing Cognitive Changes: