tag:blogger.com,1999:blog-65558158089455678402023-11-15T10:12:01.443-08:00Diary of a Duchenne MumThis blog charts my learning and reflection as the parent of a son with Duchenne muscular dystrophy. Duchenne is complex and this is a diary, comment, and resource on the many issues we deal with as parents.Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.comBlogger5125tag:blogger.com,1999:blog-6555815808945567840.post-54987759935578103442015-07-01T02:58:00.000-07:002015-07-01T04:42:48.097-07:00Tom's Adventures in Mainstream: eight tips for better school inclusion of pupils with special educational needs and disabilities<!--[if gte mso 9]><xml>
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<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">When Tom was nine he left his mainstream school and started at a special school.<span style="mso-spacerun: yes;"> </span>While
we’re delighted that Tom is now in what is a more enabling
environment for him, we’re reflecting on how Tom’s experience in mainstream
could have been more inclusive.<span style="mso-spacerun: yes;"> </span>The
mainstream school was keen to be inclusive:<span style="mso-spacerun: yes;">
</span>the Head teacher had a wonderful attitude and knew and loved all the
children in the school.<span style="mso-spacerun: yes;"> </span>Socially,
ethnically, and on behaviour issues the school did seem to be very
inclusive:<span style="mso-spacerun: yes;"> </span>what could they do to include
children with Special Educational Needs and Disabilities (SEND) more
successfully?</span></div>
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<br /></div>
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<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Before listing what I think are the
top eight tips for more successful inclusion, first a bit about Tom
himself.<span style="mso-spacerun: yes;"> </span>Tom's condition,
Duchenne muscular dystrophy (Duchenne or DMD for short), means he has weak muscles, poor balance,
and poor coordination – he can’t run and writing by hand is laborious and
difficult for him.<span style="mso-spacerun: yes;"> </span>He needs various adaptations
to help him sit and write, including a keyboard.<span style="mso-spacerun: yes;"> </span></span></div>
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<br /></div>
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<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Many children with DMD also have
cognitive difficulties, summarised by difficulty processing information.<span style="mso-spacerun: yes;"> </span>Like water on clay soil, information takes
longer to absorb and too much information at once is overload.<span style="mso-spacerun: yes;"> </span>Anything which needs a lot of process – like joining up letters to read words – is difficult for Tom, thought
he can tell you loads about many subjects and has a lively curiosity.<span style="mso-spacerun: yes;"> </span>You can appreciate the challenges for a child
like Tom in mainstream:<span style="mso-spacerun: yes;"> </span>taking in
teachers’ instructions, recalling vocabulary, other children’s fast-moving
games and social interaction, dealing with transitions from one task to another
and the hustle and bustle of the school day, and most significantly how Tom
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<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">What can be done to address better
the needs of children like Tom in mainstream?<span style="mso-spacerun: yes;">
</span>Well, an education revolution so all classes and schools are much
smaller, with a higher ratio of teachers to pupils, would help, though that
would not in itself be sufficient.<span style="mso-spacerun: yes;"> </span>In my
view, eight tweaks even to the current system would effect significant
improvements.</span></div>
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<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">1.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Enough Special Needs
Co-ordinator (SENCo) time</span></b></div>
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<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">In my son’s mainstream school there
were over 400 pupils and a more than average proportion of pupils with special
educational needs, including pupils with Down’s Syndrome or on the autistic
spectrum.<span style="mso-spacerun: yes;"> </span>But the Special Needs
Co-ordinator (SENCo) was there only two days a week.<span style="mso-spacerun: yes;"> </span>There needs to be enough time for the SENCo
to work with teachers and teaching assistants (TAs), time for the SENCO to
follow up and evaluate the success of teaching strategies, time for the SENCo
to go into classrooms to observe pupils, time for the SENCO to get the advice
that they may need, time for the SENCo to liaise with outside agencies.<span style="mso-spacerun: yes;"> </span>In our case none of these things were
happening to the extent that they needed to happen.<span style="mso-spacerun: yes;"> </span>For example, it was me, not the SENCo, who
met with the local authority SEN ICT advisory teacher to get some ICT resources
for Tom’s computer, resources which could also help other pupils.<span style="mso-spacerun: yes;"> </span></span></div>
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<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">2.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Time for teachers and teaching
assistants (TAs) to plan together</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Tom’s TA would have about thirty
seconds between hearing the teacher’s instructions for the class and starting
work with him, to take in the learning objective and task, decide how much of the
task Tom could engage with, how to break the task down into more manageable
chunks, and what, if any, alternative strategies or resources to use.<span style="mso-spacerun: yes;"> </span>Without planning ahead of time it was
extremely difficult for Tom to do differentiated tasks with other pupils.<span style="mso-spacerun: yes;"> </span>As you’ve probably guessed, I have a teaching
background and the year I learned most was when I planned together with a
TA.<span style="mso-spacerun: yes;"> </span>We both worked part time and had our
planning meetings in unpaid time.<span style="mso-spacerun: yes;"> </span>We
should not have had to do that:<span style="mso-spacerun: yes;">
</span>contractual time should be scheduled for such meetings.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">3.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Adequate training for class
teachers to support pupils with SEND</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">It is crucial for classroom teachers
to have adequate special needs training, because in the current system the
usual chain of command is that the classroom teacher tells the teaching
assistants on a daily basis what they need to be doing with their pupils with
special needs.<span style="mso-spacerun: yes;"> </span>The SENCo is there to
support, to offer ideas, and to help evaluate strategies (though in our case
there was little evidence that this was happening). </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Teachers need to be more trained in
concepts such as task analysis, backward chaining, and differentiation.<span style="mso-spacerun: yes;"> </span>For example, Tom’s class had to do ‘free
writing’.<span style="mso-spacerun: yes;"> </span>So Tom was given a pencil and
paper and ‘freedom’.<span style="mso-spacerun: yes;"> </span>His teacher told me
afterwards that he ‘made some marks and wrote the word ‘the’.<span style="mso-spacerun: yes;"> </span>Tom would certainly have had plenty in his
mind to write about, so why couldn’t his free writing have been more supported?<span style="mso-spacerun: yes;"> </span>.<span style="mso-spacerun: yes;"> </span>Why
could he not have used a keyboard?<span style="mso-spacerun: yes;"> </span>And
an ICT programme to prompt him with the sight words he knew?<span style="mso-spacerun: yes;"> </span>Both of these resources were sitting in the
stock cupboard.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">If the teacher had been adequately
trained she would have seen that what she had done here was differentiation by
outcome – in other words, throw the children in at the deep end and those who
succeed are those who make it to the shallow end without drowning.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">4.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Adequate training for special
needs teaching assistants (TAs)</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Teaching assistants need to have
adequate training to carry out the tasks they are expected to do. They need to
be trained in the pedagogical concepts which underpin their work with pupils
with SEND, because they are the ones who work with pupils day to day, the ones
who on a daily basis are fine-tuning teaching strategies, evaluating them and
looking at next steps at the micro-level.<span style="mso-spacerun: yes;">
</span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Though the chain of command is from
class teacher to TAs, the remit of the class teacher is the whole class.<span style="mso-spacerun: yes;"> </span>While the teacher may have the big picture,
in our experience class teachers don’t have the time to plan and evaluate in
detail the work for pupils with special needs – it’s the TAs who have the day
to day picture and the teacher needs the feedback from them. </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">When teaching assistants don’t have
sufficient grasp of their work, it is letting down our children with special
needs and also the teaching assistants themselves.<span style="mso-spacerun: yes;"> </span>Many TAs are parents.<span style="mso-spacerun: yes;"> </span>They feel strongly the importance of giving
children the best possible start in life; they want to help provide that for
their pupils.<span style="mso-spacerun: yes;"> </span>How can they undertake
complex teaching tasks to support the learning of pupils with special
educational needs if they don’t have adequate training?</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">5.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Information and computer
technology</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Have computers and programmes more
readily available in classrooms and ensure that SENCos, teachers, and TAs have
working knowledge of SEND-supportive programmes.<span style="mso-spacerun: yes;"> </span>By SEND-supportive programmes I mean ‘shell’
type programmes which are designed to be tailored to individual pupils’
learning and physical needs, not ready-made activities you can just put pupils
in front of.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">There was a long saga in trying to
get a computer for Tom at his mainstream school.<span style="mso-spacerun: yes;"> </span>Finally my brother stepped in and got us a
laptop.<span style="mso-spacerun: yes;"> </span>We bought the Clicker and
Clicker Paint programmes for it.<span style="mso-spacerun: yes;"> </span>Then
there was another long saga in making sure the teacher and TAs were confident
to use the computer, with me going in several times to talk them through it.<span style="mso-spacerun: yes;"> </span>The local authority
Physical Disabilities Support Service was great, going into school numerous
times to train and support. </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">6.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">A curriculum which allows for
a variety of learning styles</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">When Tom was in Year One his
mainstream school had poor results in literacy.<span style="mso-spacerun: yes;">
</span>So the following year they instituted a school-wide phonics
programme.<span style="mso-spacerun: yes;"> </span>This was an improvement – a systematic
approach to literacy which mapped the territory.<span style="mso-spacerun: yes;"> </span>But it was inflexible.<span style="mso-spacerun: yes;"> </span>And Tom’s Year Two teacher was
inflexible.<span style="mso-spacerun: yes;"> </span>At the start of the year she
told me that to learn to read ‘you go from letters to words to sentences’.<span style="mso-spacerun: yes;"> </span><span style="mso-spacerun: yes;"></span></span><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;"><span style="mso-spacerun: yes;">Readers do need to learn phonics at some point, but it doesn't have to be the initial approach to reading and it doesn't have to be the only approach to reading. Phonics is process-intensive, which is Tom's cognitive weakness, and it doesn't use his strengths, such as visual memory. </span></span>Progress was
extremely slow.<span style="mso-spacerun: yes;"> </span>To address this the SENCo prescribed an
extra fifteen minutes per week of phonics.<span style="mso-spacerun: yes;">
</span>Not analysis of Tom’s difficulties and lateral thinking about solutions,
just more of the problem.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">The children learned phonics through
worksheets.<span style="mso-spacerun: yes;"> </span>In Year Two Tom spent at
least two terms doing handwritten worksheets overlearning letter-sound
correspondence.<span style="mso-spacerun: yes;"> </span>He began to hate school.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Though the endless worksheets were
heart-sinking, Tom did end up knowing his letter sounds.<span style="mso-spacerun: yes;"> </span>At home we started teaching him sight words
– ‘word of the week’ – and with this and his knowledge of letter sounds Tom
began to read.<span style="mso-spacerun: yes;"> </span>Excitedly he would ask
‘what is the word of the week this week?’ and suggest words that he would like
to learn to read.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">While phonics was taught in each
classroom, outside in the corridor, by coincidence right outside Tom’s
classroom, was the makeshift base (made of display boards) of the Reading Recovery teacher.<span style="mso-spacerun: yes;"> </span>Reading Recovery is a one-to-one
multi-approach pupil-centred reading programme, designed to accelerate learning
for pupils experiencing literacy difficulties in the mainstream classroom.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">In Year One I asked the SENCo if Tom
could join the pupils on the Reading Recovery programme.<span style="mso-spacerun: yes;"> </span>No, she said, he’s too old.<span style="mso-spacerun: yes;"> </span>Couldn’t the TAs have some training in
Reading Recovery?<span style="mso-spacerun: yes;"> </span>No, it’s a year-long
intensive training course.<span style="mso-spacerun: yes;"> </span>She did later
try to get Tom on the programme, but didn’t chase it up and nothing came of
it.<span style="mso-spacerun: yes;"> </span>In the meantime I went to the local
university’s education library and after the equivalent of about three working
days with the key book had a summary of Reading Recovery – enough to cover its
basic principles.<span style="mso-spacerun: yes;"> </span>With the support of
Tom’s Year One class teacher I gave this to his TAs.<span style="mso-spacerun: yes;"> </span>It was difficult for the class teacher to
give input to Tom’s learning because she was incredibly busy, having just been
made Key Stage One co-ordinator and PE Co-ordinator.<span style="mso-spacerun: yes;"> </span>Tom’s TAs had little training, no time to
plan, and apparently no SENCo support and in those circumstances it was difficult
for them to put the Reading Recovery approach into action.<span style="mso-spacerun: yes;"> </span>In Year Two the class teacher was slave to
phonics alone (though towards the end of the year she did begin to acknowledge
the sight word approach).</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">7.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Appropriate accommodation for
pupils with SEN</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">In Tom’s school, pupils with SEN often
worked with their TAs out in the corridor, with the attendant noise and
distraction.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoListParagraph" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-family: Arial; mso-bidi-font-size: 12.0pt; mso-fareast-font-family: Arial;"><span style="mso-list: Ignore;">8.<span style="font: 7.0pt "Times New Roman";"> </span></span></span></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Challenging an unthinking
culture of discrimination</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">At a meeting of the school’s
disability equality working group I raised the point about the accommodation
for pupils with SEND.<span style="mso-spacerun: yes;"> </span>One of the
school’s governors said ‘Accommodation is always a problem – always has been
and always will be’.<span style="mso-spacerun: yes;"> </span>I said ‘Maybe
you’re right – and if it’s a problem, how about the top groups learn in the
corridor and pupils with special needs learn in quiet classrooms?’<span style="mso-spacerun: yes;"> </span>She looked stunned at this suggestion.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">At one point I went in to observe for
a morning in Tom’s classroom.<span style="mso-spacerun: yes;"> </span>When the
class split for a task into lower and higher attainment groups, it was the
class teacher who took the higher attainment group, while the TA – without
teacher training and paid less – took the children who were more vulnerable and
who had more complex and challenging learning needs.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Tom’s class had an overnight school
trip to an outdoor activity centre – and the letter from the school said that
they would like to take all the children.<span style="mso-spacerun: yes;">
</span>Tom’s teacher did not book accommodation for him, accommodation which
included an overnight carer and which met health and safety regulations for a
pupil with reduced mobility.<span style="mso-spacerun: yes;"> </span>Was this
just forgetting, or an assumption that Tom, being disabled, wouldn’t take up
his curriculum entitlement or share the aspirations of his peers?</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Conclusion</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">In our experience, these are eight
issues which need to be addressed, eight tweaks which could be made in the
current mainstream system to improve inclusion for pupils with special
educational needs and disabilities<span style="mso-spacerun: yes;"></span>.<span style="mso-spacerun: yes;"> </span>In Tom’s mainstream school they were the key
ways to translate into inclusion the wonderful, genuine, and laudable love
which Tom’s Headteacher had for the children in the school.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">Some of these tweaks are about
money:<span style="mso-spacerun: yes;"> </span>paying for SENCO, teacher, and TA
time and training.<span style="mso-spacerun: yes;"> </span>Some of them are
about good pedagogical practice:<span style="mso-spacerun: yes;"> </span>having
the concepts to support pupils with special needs and the curriculum to nurture
a range of learning styles and enable pupils to use their strengths to overcome
their difficulties.<span style="mso-spacerun: yes;"> </span>Some of them need funded local authority support to provide continuing professional development
and specific training.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">But the really key issue, the
foundation of all inclusion, the driver of all practical measures, is a school
culture which recognises the equal aspiration and entitlement of all pupils and
is committed to solving or working round any problems which may arise.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-ansi-language: EN-GB; mso-bidi-font-size: 12.0pt;">And a final note: on Tom's final day at that school, he brought all his accumulated phonics worksheets home, a great big telephone directory sized pile of them. He put them in our wood burner. I helped him light the match and he burned them all. </span></div>
Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.com0tag:blogger.com,1999:blog-6555815808945567840.post-24133873936875133132015-07-01T02:05:00.001-07:002015-07-01T05:31:58.321-07:00DMD life expectancy, and talking about it with a child living with DMD A week ago we told eleven year old Tom about the life-shortening aspect of Duchenne muscular dystrophy. This was a conversation we had been anticipating and dreading ever since he was diagnosed with DMD aged four.<br />
<br />
It was on Saturday morning, after we had had a nice time looking at a book together, and before breakfast. Tom and I were sitting on the sofa. I said, 'There is some more you need to know about Duchenne, now that you are getting into the teenage years. Most people with Duchenne live to around thirty years old. But the scientists and doctors are working hard to find effective treatments, so people with Duchenne can in future live longer lives.' Pause, while he took this in. Then: 'It's important that you have accurate information, because other children might look up things about Duchenne and say things to you that may not be right. You can say to them, 'Most people live to thirty and scientists and doctors are working hard to find effective treatments, so people with Duchenne will be able to live long lives'.'<br />
<br />
Tom said 'So most people with Duchenne live to thirty...' and I finished off '...and scientist and doctors are working hard to find effective treatments, so in future people with Duchenne will be able to live long lives.'<br />
<br />
<br />
Tom then said 'You don't have to rub my feet when we're talking about Duchenne'. I realised that I had been mechanically massaging Tom's foot as we had been speaking. Then he said 'Can we have breakfast now?'<br />
<br />
It was clear that Tom had taken in the basic message, but also clear that it was something he would absorb over time.<br />
<br />
There were quite a few things we considered in the background to this conversation.<br />
<br />
One was the principle: tell the truth, but be positive. What we told Tom is the most positive spin on the current situation. Thirty is to my knowledge the average life expectancy for people in the UK getting good standards of care. And there is a lot of research going on; the message is one of hope.<br />
<br />
Another was the timing and who would tell him. It needed to be a quiet time, when Tom would be fresh, and when he would have some time after the conversation to take it in. Tom's dad and I decided that it would be one of us, not both, who had the initial conversation, to keep things low-key.<br />
<br />
Another consideration was the fact that we had given Tom information about DMD over years preceding this, in a 'drip-feed' way, responding to his own questions and observations. He already knows about the things he has observed and he has asked questions about DNA and dystrophin. However, this conversation was different. It was different because it did not arise naturally from something he himself had observed (such as older boys using wheelchairs full time) and it was always - obviously - going to be outside our comfort zone as parents. How far it is outside Tom's comfort zone I don't know. I don't think being thirty - or dying - is something that he can envisage at the moment (though it will be as he gets older). He has met lots of scientists at the Action Duchenne conference and he is on a clinical trial, so the current work on effective treatments is within his existing experience.<br />
<br />
It is very difficult to give this information to kids so young. What made us have the conversation now is that Tom is in contact with other children who can look up DMD on the web. We needed to tell him before he heard from other, possibly inaccurate or hurtful, sources. We are lucky in a way that we were able to wait until he was eleven. Tom's DMD-related information-processing difficulties mean that his literacy is still too limited for him to look up DMD himself; also he attends a special needs school where most of the other kids are unlikely to look it up either. But just this year his social horizons are expanding and he is additionally spending time with other children in mainstream settings.<br />
<br />
A further consideration for us was the impact on the rest of the family. Before we had the conversation with Tom we had to think about how we as parents are handling these huge and difficult issues; what are our different attitudes, how can we communicate about them, how can we agree a way forward? What is the impact on our emotional health of dealing with the fallout of information? We didn't know how Tom would respond; would he be traumatised and how would we as parents cope with that and best support him? Clearly we need to keep listening and supporting Tom in whatever way is helpful to him as he absorbs the information. As Tom is an only child we didn't also have to deal with the possible impact on siblings. But grandparents and other people close to Tom will need to be in the picture about what he knows and about ways to support him. And these are of course huge issues for them to take on too.<br />
<br />
Over the past year or so we have consulted the children's psychology service at our local hospital, who have given us a lot of help and support to handle these issues.<br />
<br />
<br />
Another background consideration is having plenty of positive, affirming, self-esteem building things going on for Tom as well as this difficult information. To make sure good things are happening, things where Duchenne is not invited. Some examples: Tom has fun at an art club, at the moment he's loving mixing colours and experimenting with fabric dyes. He loves acting too, making up stories, putting on funny voices, being different characters, and hanging out with other kids who love all that stuff too. Recently we've got a dog; she's a wonderful friend and it's satisfying and good fun training her. We make photo albums of all these good things Tom does. We are upfront about his qualities as a people person, a creative person, and some one who enjoys getting out and about and finding out about the world.<br />
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It's a relief to have all the information about DMD in the open, and not feel that there is something we fear Tom finding out. But it was also traumatic to have the conversation. The afternoon of that Saturday we went to a party. I was in a bit of a daze. Looking around at those other people there I just wanted to shout 'This morning I told my son his condition is life-shortening, oh god, oh god, oh god'.<br />
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<br />Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.com0tag:blogger.com,1999:blog-6555815808945567840.post-21972870472847576542015-01-28T08:07:00.000-08:002015-07-03T14:22:30.747-07:00Steroids and Duchenne muscular dystrophy - our family's experienceTen days on and ten days off or full-time? Prednisolone or Deflazacort? What are the issues with weight, height, puberty, growth spurts, scoliosis, bone density, behaviour management, diet??? These are the questions and variables concerning steroids which face parents of children living with DMD. Here I would like to share our experience of steroids, for the light it may cast on these questions. It is a layperson's account, hopefully giving some idea of one family's lived experience of using steroids. Anyone looking for advice on steroids for DMD should consult their specialist neuromuscular consultant.<br />
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When our son Tom was five he went on to Prednisolone ten days on, twenty days off. We soon upped that to ten days on ten days off. There would often be behaviour issues towards the end of the ten days on. Tom is now on daily Prednisolone and the behaviour issues are more frequent and more easily provoked.<br />
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The behaviour issues were - and are - heightened reactions to frustration and uncertainty. There are outbursts: Tom will cry and shriek and not respond to reason at all. These outbursts can last a couple of minutes or half an hour depending on how tired he is. In Tom's case the steroid reaction is on top of difficulties with mental flexibility common to some children with DMD. Tom finds it difficult to hold two thoughts in his mind at once or switch from one to another quickly - his thinking tends to be 'black and white' and any kind of uncertainty means that he is trying to juggle two things in his mind and as a result he heads into meltdown.<br />
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We deal with this by having very strong routines and trying to keep things as calm as possible. However, that can't solve it all. The most simple-seeming conversation can get into meltdown territory. For example, recently Tom was talking about where pancakes come from, and that there are French pancakes, American pancakes, Chinese pancakes. He asked me whether English pancakes come from France. As I wasn't able to give an immediate and categorical answer he got increasingly frustrated, ending up shouting at me, 'Do English pancakes come from France YES OR NO???'<br />
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In September 2012 when Tom was eight his mobility started to decline more rapidly. In the November we found out that the final clinical trial for Translarna (Ataluren as it is on the trial) was due to start some time in 2013 - a drug for which Tom was eligible. If Tom was to have a chance of being recruited to the trial he would have to be ambulant. We were scared stiff he would lose ambulation before the trial start date.<br />
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So we decided to increase to daily steroids to try and prolong walking as long as possible. When I say 'we decided': it was a massively stressful process with inadequate support from specialists. Our consultant was extremely busy and unavailable to consult with us between our scheduled six-monthly visits. So awaiting an email response from our specialist we consulted a paediatrician. As a non-specialist he was frankly horrified at the idea of such a high steroid dose and went through all the possible dreadful side effects - fractures, diabetes, cataracts, stomach ulcers etc etc. The meeting with him was so stressful that I had the experience of my mind detaching and looking down at myself sitting in the consulting room.<br />
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Afterwards on the internet I looked up what the leading Duchenne specialists say about daily steroids. The most helpful was <a href="http://www.newcastle-hospitals.org.uk/services/northern-genetics_treatment-and-medication_steroids-and-duchenne-muscular-dystrophy.aspx">www.newcastle-hospitals.org.uk/services/northern-genetics_treatment-and-medication_steroids-and-duchenne-muscular-dystrophy.aspx</a>. I gleaned that the jury is out on the best dose regime, that there is roughly a 50% split between clinicians on prescribing daily or intermittent doses, that a research study was at that time about to start on the question of steroid dosage, and that the mean age of loss of ambulation for children with DMD who took daily steroids from age 5 was 14.5 years, as opposed to 12 on intermittent and 10 without steroids. One phrase from a clinician stuck in my mind: 'I hate steroids, but I hate DMD more'.<br />
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When our consultant eventually replied to our email he said that he favoured the intermittent regime as it had far fewer side effects, but if we wanted to go on to daily he would go with that. Nobody told us at that point it would be difficult to go back from a daily to an intermittent regime.<br />
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We consulted Tom. This built on years of drip-feed information we had given him about DMD. He knew his muscles were getting weaker. He was also aware that boys with DMD tend to start needing a wheelchair full time in Year 7 or Year 8 at school. At nine years old he had the maturity and information to be able to think about what daily steroids would mean. He was able to understand both that the steroids caused the shrieky behaviour and that he would have to move from a low-calorie diet to a lower-calorie one to control weight gain. He also understood that being on the Ataluren clinical trial might slow the muscle wasting and help him keep walking. Given the choices, Tom opted for daily steroids.<br />
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This was in April 2013. A weight check in July showed a rapid weight gain - from 30 to 33 kilos on what we considered to be a healthy low calorie diet. The diet then got even lower calorie, Weight-Watchers gone mad. It was do-able, just. Result: after a further two months a visit to the dietician revealed that Tom had lost three pounds and the dietician was happy with the diet from a nutrition point of view. We were able to ease up a tiny bit.<br />
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It has been challenging to maintain the very low calorie diet in the long term. However, Tom's rigid thinking has been an advantage here. Just brainwash him that junk food is dreadful, and that he is making positive choices to stay slim and healthy, and he is on-side, railing against the evils of junk food. Tom's Dad and I have more difficulty. On holiday each of us slopes off sometimes to 'browse in the bookshop' - code for going off for cake, ice cream, or fish and chips. Tom does have a day off every couple of months to have whatever he wants.<br />
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Fractures are also a worry. We take Tom swimming a lot and try to avoid exercise where there is more of a risk of falls. Tom takes vitamin D and calcium supplements (Fultium 800mg and Calcichew D3).<br />
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In terms of growth, there was almost no increase in Tom's height from September 2013 to October 2014. He has grown a bit since then, but the trajectory is that from an average baseline he will be close to the bottom centiles on height by the time he is fourteen if he stays on daily steroids. <br />
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We hoped that once past the trial, Tom would be able to revert to the intermittent dose regime. But the advice to us now is that to do that Tom would have to go down to zero steroids, then build up again. With him entering the growth-spurt years, loss of mobility now could lead more easily to scoliosis. However, what can be done is keep the steroid dose steady, so as Tom grows the dose will get proportionally less. We brace ourselves to continue to ride the steroid outbursts; to try to balance Tom's need for exercise with protecting him from fractures; to keep on the diet.<br />
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In my experience of steroids, what I have found most helpful is: getting Tom used to healthy eating from the word go (and fighting the battle with well-meaning givers of sweets); being able to consult a dietitician who understood the challenges of steroids; and behaviour management techniques including getting enough sleep myself in order to stay calm, and the technique of 'collaborative problem solving' from psychologist (and dad of a boy with DMD) James Poysky (for a useful summary of this approach google Opposition and Aggression Parent Project Muscular Dystrophy). <br />
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<br />Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.com0tag:blogger.com,1999:blog-6555815808945567840.post-51499879775730542602014-03-11T06:31:00.000-07:002015-07-03T14:27:12.650-07:00Adaptations to make our kitchen wheelchair-accessibleTwo major things happening this week. One is huge in the big scheme of things: Tom is taking part in a clinical trial. Tomorrow he has his first appointment since being recruited. The whole issue of taking part in a clinical trial will be the subject of a number of forthcoming posts. Today I want to focus on the second thing happening this week, which is possibly lesser in the big scheme of things, but very preoccupying right now: next Monday all hell breaks loose as the builders arrive to adapt our kitchen to be wheelchair accessible.<br />
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In this post I'll outline the key things we took into account when planning the kitchen, and the support we're getting from the council.<br />
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In a future post I'll write about the much bigger adaptation we have already done, to put in a downstairs bedroom and bathroom. I'll also publish the talk I was asked to give to our local team of Occupational Therapists, on a parents' perspective on Occupational Therapy. This focused primarily on adaptations in the home. <br />
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First a quick diary update. Still haven't rebooked Tom's appointment with the neuromuscular consultant. The school meeting went well - things not completely sorted but better than they were. The negotiations with Tom about pants reached a compromise: Tom is in long johns. I think he is finding it more difficult to manage when he goes to the toilet. He doesn't want anyone to go with him. Long johns are easier to manage than pants. All part of Tom quietly getting his head around decreasing mobility (he is ten and a quarter).<br />
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The kitchen:<br />
The deal with the kitchen is that Tom needs to be able to prepare, or help prepare, simple meals, and generally be able to access the kitchen and its appliances.<br />
Therefore we need enough space for the wheelchair to get in and out of, and around, the kitchen. 1500mm will give enough space to back and manouevre the wheelchair. 1700mm would be a full wheelchair turning circle.<br />
We need worktops, a hob, and a sink unit with space underneath, so a wheelchair user can use the worktop. Ideally we need an L-shaped worktop, so Tom's arms can rest on each side. We need an oven at a height Tom can see into, with a pull-out shelf below.<br />
At this point in time, when Tom is still ambulant but in danger of falling, we need a super-non-slip floor.<br />
Accessible wall units we just gave up on. Tom won't have the arm strength to utilise these in the future.<br />
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To get the wheelchair space we are taking out one kitchen cabinet, and replacing it with a 300mm wide shelf.<br />
The worktops, hob, and sink unit we are getting from Howden's, who have branches around the country. The oven also comes from Howden's. <br />
We've thought long and hard about whether to get a height adjustable sink and hob, and have decided yes. It's essential so that both Tom and his dad, B, who doesn't use a wheelchair and is around 5'10", can both use the kitchen. However, it'll add about £2000+ onto the bill.<br />
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The floor is from the Altro range. We also looked at the Tarkett range (formerly known as Marley I believe). Both do very non-slip floors. The colour I want is called 'Tree Frog'. B said it looked like frog vomit. I said that was fine, as when he looked at it and threw up, the vomit wouldn't show. We left it there. (B did say he would stop noticing it after a while.) I've ordered Tree Frog.<br />
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The local council is paying for anything that Tom needs for access and to use the kitchen to prepare or help prepare simple meals. So they are paying for some doors to be widened, for the worktops (but not the rise and fall mechanism, or the hob as Tom is still a child), and for the flooring. They are also paying for the cabinet to be taken out, and for the washing machine to be moved to another room.<br />
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I think it helps that we are doing this work right at the end of the financial year. For some reason, incredible to me in this time of austerity, it seems the council have money they need to spend before the end of the financial year.<br />
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The key player in the kitchen adaptations is our Occupational Therapist. She has advised us throughout and also advised the council. <br />
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Two very useful resources are the Muscular Dystrophy Campaign's house adaptation manual, and also Portsmouth City Council Occupational Therapy service, who have produced a very useful guide. Google 'portsmouth.gov.uk design guide for wheelchair accessible housing'.<br />
<br />Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.com0tag:blogger.com,1999:blog-6555815808945567840.post-71834475601340215892014-03-03T03:25:00.000-08:002015-07-03T14:29:00.615-07:00Welcome, and a beginner's guide to DMDWelcome to Diary of a Duchenne Mum!<br />
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I could plunge in the deep end, and tell you about the multiple things I'm dealing with today: chasing up the neuromuscular consultant to rebook an appointment for T; replying to the contractor who has just sent us an estimate for making adaptations to our kitchen to make it wheelchair accessible; preparing for the meeting with T's special school tomorrow; negotiating with T on whether he wears pants today.... These are all posts in their own right, and I'll get to them all in turn in the next days and weeks.<br />
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But first, to introduce us: I'm in my late forties and live in the UK with my husband B and our ten year old son. Let's call him Tom. I'm keeping the blog anonymous, partly to protect our son, but also because I want to be able to be completely honest and tell it like I experience it. Tom was diagnosed with Duchenne muscular dystrophy five and a half years ago, when he was four and a half. We have been on a Duchenne journey ever since. Of course, having Duchenne is just one thing about Tom. He's also a sparky, imaginative, sociable, loving kid who's got loads of ideas and interest in the world, and is out there living life to the full.<br />
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Over the years of the Duchenne journey so far, I've written a number of things about Duchenne for a wider audience. To kick off this blog, here is the one that sets the scene of what Duchenne muscular dystrophy is: a beginner's guide to Duchenne, its causes and potential treatments. <br />
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<div align="center" class="MsoNormal" style="text-align: center;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="font-size: 16.0pt; line-height: 115%;">Duchenne
Muscular Dystrophy:<span style="mso-spacerun: yes;"> </span></span></b></div>
<div align="center" class="MsoNormal" style="text-align: center;">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="font-size: 16.0pt; line-height: 115%;">a beginner’s
guide to its cause and potential treatments</span></b></div>
<div class="MsoNormal" style="margin-left: 180.0pt; text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><br /></span></div>
<div align="center" class="MsoNormal" style="text-align: center;">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">What is Duchenne muscular
dystrophy?</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Duchenne muscular dystrophy (DMD) is a progressive muscle
wasting condition.<span style="mso-spacerun: yes;"> </span>It is relatively rare
- roughly one in seven thousand births – and affects mainly boys.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">People with DMD gradually lose strength in the skeletal
muscles.<span style="mso-spacerun: yes;"> </span>Later the heart and respiratory
muscles are involved.<span style="mso-spacerun: yes;"> </span>DMD does not
affect continence, speech, or eye movements.<span style="mso-spacerun: yes;">
</span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">On average boys with DMD begin to need a wheelchair full time
between the ages of eight and twelve. </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Life expectancy when DMD is untreated is around nineteen
years.<span style="mso-spacerun: yes;"> </span>With specialist care management
life expectancy can rise into the thirties and even forties.<span style="mso-spacerun: yes;"> </span>Work being done on potential new treatments gives
realistic hope of even longer lives for the current generation of boys growing
up with DMD – the hope that DMD might become a long-term manageable condition.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">What causes Duchenne
muscular dystrophy?</span></b></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Lack
of a crucial protein called dystrophin</span></i></b><b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">: </span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">To work, our muscle cells need a protein called
dystrophin.<span style="mso-spacerun: yes;"> </span>Dystrophin is crucial for
holding muscle cell walls together.<span style="mso-spacerun: yes;"> </span>It
is like scaffolding for the cell wall.<span style="mso-spacerun: yes;">
</span>It holds in place a number of other proteins which all help the cell
wall work properly.<span style="mso-spacerun: yes;"> </span>Dystrophin is also
like a shock absorber.<span style="mso-spacerun: yes;"> </span>It is
springy.<span style="mso-spacerun: yes;"> </span>This enables muscle cells to
absorb impact without getting damaged.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">People with DMD do not have any dystrophin in their muscle
cells.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Why
do people with DMD lack dystrophin?</span></i></b><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"> </span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">In every cell of the body there is an instruction book called
DNA.<span style="mso-spacerun: yes;"> </span>It is made up of chemicals and
those chemicals act just like letters and words.<span style="mso-spacerun: yes;"> </span>DNA contains instructions for the body to
make everything it needs to grow and to maintain itself, including dystrophin
and thousands of other proteins.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">DNA has twenty two chapters plus a chapter called X.<span style="mso-spacerun: yes;"> </span>The scientific name for these chapters is
chromosomes.<span style="mso-spacerun: yes;"> </span>In chapter X there is a
paragraph giving the instruction for dystrophin.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Imagine that you are in a muscle cell.<span style="mso-spacerun: yes;"> </span>In this imaginary cell you are going to use
the DNA instruction to make dystrophin out of Lego bricks.<span style="mso-spacerun: yes;"> </span>You turn to the paragraph in Chapter X and
you read this instruction:</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>Start with
blue brick then green brick then blue brick then </span></div>
<div class="MsoNormal" style="margin-left: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">red brick yellow brick red
brick yellow brick red brick yellow brick </span></div>
<div class="MsoNormal" style="margin-left: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">red brick yellow brick red
brick yellow brick red brick yellow brick </span></div>
<div class="MsoNormal" style="margin-left: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">red brick yellow brick red
brick yellow brick red brick yellow brick</span></div>
<div class="MsoNormal" style="margin-left: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">blue brick green brick blue
brick.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Fine:<span style="mso-spacerun: yes;"> </span>you make
dystrophin.<span style="mso-spacerun: yes;"> </span>But imagine that you turn to
the dystrophin paragraph and instead get this instruction:</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>Start with
blue brick then green brick then blue brick then</span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>rbr ickye
llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickre </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">dbr ickye llowbr ickre dbr
ickye llowbr ickre dbr ickye llowbr ickre </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">dbr<span style="mso-spacerun: yes;"> </span>ickye llowbr ickre dbr ickye llowbr ickre dbr
ickye llowbr ickbl </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">uebr ickgr eenbr ickbl uebr
ick.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">The instruction is unreadable.<span style="mso-spacerun: yes;"> </span>You can’t make dystrophin.<span style="mso-spacerun: yes;"> </span>Two letters have been missed out.<span style="mso-spacerun: yes;"> </span>The words are all of the right length.<span style="mso-spacerun: yes;"> </span>The other letters have shunted up, in the
place of the missing letters.<span style="mso-spacerun: yes;"> </span>The result
is gobbledegook.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Now imagine that you turn to the dystrophin paragraph and instead
of the previous examples you get this instruction:</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>Start with blue
brick then green brick then blue brick then</span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>red.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">There is a full stop before the instruction has finished.<span style="mso-spacerun: yes;"> </span>You can’t make dystrophin.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">If the body can’t make dystrophin, the muscle cell dies.<span style="mso-spacerun: yes;"> </span>The long term effect of this is that muscle
fibre degenerates and is replaced by fatty tissue.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-spacerun: yes;"> </span>What I have outlined
here are the two kinds of mistake that can occur in DNA instructions.<span style="mso-spacerun: yes;"> </span>In about 85 – 90% of DNA mistakes, letters are
missed out or added.<span style="mso-spacerun: yes;"> </span>In about 10 – 15%
of mistakes, full stops are put in before the end of the instruction.<span style="mso-spacerun: yes;"> </span>When these mistakes happen, the body can’t
follow the instruction and can’t make the protein.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">These two kinds of mistake can happen anywhere in the DNA
instruction book.<span style="mso-spacerun: yes;"> </span>Depending on where
they are they cause different conditions.<span style="mso-spacerun: yes;">
</span>Haemophilia, cystic fibrosis, some forms of sight loss, and predisposition
to certain cancers are all examples of conditions caused by DNA mistakes.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">The body does have a back-up plan.<span style="mso-spacerun: yes;"> </span>Girls get a full copy of the twenty-two
chapters plus chapter X.<span style="mso-spacerun: yes;"> </span>Every cell
contains two full copies of DNA.<span style="mso-spacerun: yes;"> </span>If
there is a mistake on one copy, the body can use the other copy.<span style="mso-spacerun: yes;"> </span>Boys get a copy of the twenty-two chapters,
but instead of a second chapter X they get chapter Y.<span style="mso-spacerun: yes;"> </span>Chapter Y contains instructions for making
testicles, but it doesn’t contain the instructions that are in chapter X for
making dystrophin and some other crucial proteins.<span style="mso-spacerun: yes;"> </span>That is why it is usually only boys who get
Duchenne muscular dystrophy.<span style="mso-spacerun: yes;"> </span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">How are scientists tackling
Duchenne muscular dystrophy?</span></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Scientists are exploring a number of ways to tackle Duchenne
muscular dystrophy and turn it into a manageable condition.<span style="mso-spacerun: yes;"> </span>The main approaches are listed below.<span style="mso-spacerun: yes;"> </span>Different research projects are at different
stages of development.<span style="mso-spacerun: yes;"> </span>We are looking at
a time scale of five to ten years for research to produce the first treatments.
</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Target
the DNA mistake</span></i></b></div>
<div class="MsoNormal">
<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Exon skipping</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"> targets DNA instructions which have been turned into gobbledegook.<span style="mso-spacerun: yes;"> </span>It cuts letters out of the faulty instruction
so that the letters get back in the right place.<span style="mso-spacerun: yes;"> </span>In the example here, you could cut the
underlined letters to restore a readable instruction: </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Start with blue brick then
green brick then blue brick then</span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"><span style="mso-tab-count: 1;"> </span>r<u>br ickye
llowbr ickr</u>e dbr ickye llowbr ickre dbr ickye llowbr ickre </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">dbr ickye llowbr ickre dbr
ickye llowbr ickre dbr ickye llowbr ickre </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">dbr<span style="mso-spacerun: yes;"> </span>ickye llowbr ickre dbr ickye llowbr ickre dbr
ickye llowbr ickbl </span></div>
<div class="MsoNormal" style="text-indent: 36.0pt;">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">uebr ickgr eenbr ickbl uebr
ick.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">You would not get full dystrophin but it would work.<span style="mso-spacerun: yes;"> </span>Duchenne muscular dystrophy would become a
milder condition called Becker muscular dystrophy:<span style="mso-spacerun: yes;"> </span>still muscle degeneration, but usually much
less severe and life-shortening than DMD. <span style="mso-spacerun: yes;"> </span>A number of scientists are working on exon
skipping, including Matthew Wood at Oxford University in the UK and Steve
Wilton in Perth, Western Australia. </span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Ataluren</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"> is a drug which targets premature full stops.<span style="mso-spacerun: yes;"> </span>It aims to enable the body to ‘read through’
the premature full stop.<span style="mso-spacerun: yes;"> </span>In cases of
premature full stops, the rest of the instruction is there, but the body does
not read it because it stops at the full stop.<span style="mso-spacerun: yes;">
</span>Ataluren could potentially restore full-length dystrophin. <span style="mso-spacerun: yes;"> </span>It is being trialled by an American
pharmaceutical company called PTC Therapeutics.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Bring
in dystrophin from outside the body</span></i></b></div>
<div class="MsoNormal">
<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Adeno-associated virus vectors (AAV)</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">:<span style="mso-spacerun: yes;"> </span>this approach uses empty virus ‘shells’ to
bring dystrophin into the body and transport it to every cell.<span style="mso-spacerun: yes;"> </span>There are two main difficulties which need to
be overcome here.<span style="mso-spacerun: yes;"> </span>The first is that
dystrophin is very big, as you would expect of a protein which is a cell wall
scaffolding and shock absorber – too big to fit into viruses without being
modified.<span style="mso-spacerun: yes;"> </span>The second is the problem of
immune responses by the body to incoming viruses.<span style="mso-spacerun: yes;"> </span>AAV is being explored by Keith Foster at
Reading University in the UK.</span></div>
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<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Replace
dystrophin with another protein</span></i></b></div>
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<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Utrophin</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"> is a protein which does the same job as dystrophin at
the foetal stage and shortly after birth.<span style="mso-spacerun: yes;">
</span>It is then switched off.<span style="mso-spacerun: yes;"> </span>Kaye
Davies and her team at Oxford University are working to develop a drug which
would keep utrophin switched on and boosted up to replace dystrophin.</span></div>
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<b style="mso-bidi-font-weight: normal;"><i style="mso-bidi-font-style: normal;"><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Build
up muscle to compensate </span></i></b><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"></span></div>
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<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Steroids</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;"> are currently used to help prolong muscle
strength.<span style="mso-spacerun: yes;"> </span>While they do have some effect,
they can’t boost up muscle strength to anywhere near the level needed and they
have a lot of side effects.</span></div>
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<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Myostatin inhibitors</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">:<span style="mso-spacerun: yes;"> </span>myostatin is a substance in the body which
stops it from making too much muscle.<span style="mso-spacerun: yes;"> </span>If
myostatin could be switched off or inhibited, the body would make more muscle, potentially
enough to compensate for the continuous muscle loss in DMD.<span style="mso-spacerun: yes;"> </span>George Dickson at Royal Holloway College,
University of London, is working on this approach.</span></div>
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<u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">Stem cells</span></u><span lang="EN-GB" style="line-height: 115%; mso-bidi-font-size: 12.0pt;">:<span style="mso-spacerun: yes;"> </span>these are
cells in the body with the potential to turn into any kind of cell.<span style="mso-spacerun: yes;"> </span>It might be possible to direct stem cells to
turn into muscle cells.<span style="mso-spacerun: yes;"> </span>Research into
stem cell use for Duchenne muscular dystrophy is being done by Jenny Morgan at
University College, University of London.</span></div>
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Duchenne mumhttp://www.blogger.com/profile/00203996279144968411noreply@blogger.com0