Monkey World

I have an anger management issue.  I am asking myself where I can put these feelings of anger.

To cut a long story short, it's not Duchenne that sets me off.  It's dealing with a world that doesn't get it.  In the last three weeks, it's been: the officious volunteer at the Chiltern Open Air Museum, a venue that actually has good access, greeting us with a list of what Tom would not be able to access as a wheelchair user; it's my mum being charged £25 for a £10 taxi journey because we had a wheelchair accessible vehicle; it's feeling 'told off' by a restaurant owner who got very flustered when we arrived with wheelchair and assistance dog;  it's the unnecessary step from plane to corridor when we came back to Heathrow from our holiday; it's the 'wheelchair space' on the Heathrow - Oxford bus, precarious to get up to and sit in, too small, secured only at the back, without seatbelt, and taking twenty minutes to put together by the (very nice) driver who needed to consult the instruction manual.  It's the accumulated effect of examples like this going back ten years.  When we are about to celebrate the 2016 Paralympics, why is accessibility still not routine in much of the UK?

So how can I deal with my anger?  To carry it around eats me up, and fighting every battle is obviously not possible.  Cognitive behavioural therapy had me writing a diary of every time I got angry.  A 'mindfulness for carers' course had me observing my breath and sitting by a river watching the feelings flow by.  But I still want to thump someone.

'Let go and let God' is another option.  I'm not a believer but I have a good imagination.  What kind of god could I give my anger to?  Maybe the Narnia route, sitting by a stream in Aslan's country?

Then the image came into my mind of a tourist attraction in Dorset that I went to a couple of years ago:  Monkey World.  It's a refuge for primates rescued from abuse.  A wonderful place, with big enclosures and deeply caring staff.  I remember the gibbons silhouetted in their high tree top home, and how the children's playground was a lot like the chimpanzee enclosure.  The traumatised residents of Monkey World were accepted there as themselves, calmed, reassured, and eventually brought back to physical and mental health, their natural energy and grace.  I imagine such enclosures and such care for my own anger, fear, anxiety, frustration, and grief.  The God to whom I could give my anger would be like a keeper at Monkey World.

Direct Payments for Carers - my experience

Direct Payments - receive money from your local authority to choose and employ your own carer.  What's not to like, what could possibly go wrong?

We had a carer for our son Tom for 18 months, using the Direct Payment scheme.  Recently she left and we wanted to employ a new carer.  Here is the story of sorting out the employment of the new carer.

Having sorted through the job description, health and safety notice, and information on disciplinary and grievance procedures, and having updated the risk assessment, insurance certificate, employee registration form (not to be confused with the employee registration and liability form), and P46 for our son's new carer; having photocopied three forms of ID from our carer as specified in lists 1, 2, and 2a, and having decided that the carer personal information form was probably not necessary, the information being reproduced elsewhere, I realised that we needed electronic, or at the least new paper copies, of the contract and the employee registration and liability form.

I also wasn't sure if I still needed to complete the forms for routes 1, 3, and 6 of the DBS checking system and send them to the local authority, or how I could find the online DBS form our new carer had already done for another employer.

The original organisation who had supplied the pro-forma contract 18 months previously was no longer contracted by the local authority. I tried to phone the local authority's direct payments helpline, as given on my original 18 month old paperwork.  The number was no longer available.  The email address of the person who had at that time been my point of contact in the local authority was also not working.  So I phoned the number of the new outsourced direct payments organisation.  They told me they don't do contracts, told me to contact the local authority directly, and gave me the generic social services number.  That number put me through to the children's disability service, who put me through to my son's social worker, who we had seen the previous day.

All this took so long and was so frustrating that I neglected Tom, who was downstairs watching telly.  I got tea ready late and then tried to hurry Tom at bathtime; he started having a meltdown and I blew up and shouted at him.  And he's the one whose care and wellbeing this is all about.

The moral of this story:  policymakers, always keep your focus on the realities of life for service users.  Parent carers like me sorting direct payments are by definition in need of help with care.  They will be juggling the paperwork with the needs of their child/children and their jobs and household tasks.  Please keep it simple.  And please make sure there is one-stop-shop admin back-up for direct payments that can supply parent employers with the paperwork and admin advice they need.  Oh, and please make sure that the Direct Payments scheme is flagged up to parents via school or the health service - we found out about it through the grapevine, talking to other parents.

A Parent's Perspective on Occupational Therapy in Treating and Managing Duchenne Muscular Dystrophy

A Parent’s Perspective on Occupational Therapy in Treating/Managing Duchenne Muscular Dystrophy (DMD)

                                                                                   

This is a summary from my experience as a mum of a son with Duchenne muscular dystrophy, of what families/people living with DMD are dealing with and what we need from our Occupational Therapists.

What we are dealing with

A relatively rare, complex, severe, progressive, life shortening, and currently incurable condition

·         Diagnosis can be difficult to get and late

·         The emotional impact of diagnosis – families are traumatised

·         A plateau stage (phoney war) followed by rapid deterioration – parents can be unprepared and in denial

·         Time is of the essence

·         A lack of integrated multi-disciplinary specialist care and advice in many areas

·         A raft of different issues which all need to be addressed:  physical, learning, social interaction, behavioural, psychological.

·         Families working with many professionals – currently fourteen for our son

·         Families facing demands of time and loss of income in co-ordinating and managing care

·         Families getting their heads around house adaptations – which are complicated and expensive

·         Getting our heads around current research and whether/how our children/young people may be eligible for clinical trials

What we need from OTs

Understanding of the condition:

·         Knowing the diagnostic symptoms of DMD

·         Knowing the clinical progression of DMD

At-a-glance information for parents, given at the stage when they need it:

·         An A4 sheet summarising equipment and house adaptation needs and where we can get help and more info

·         An easy-to-follow sheet of daily hand strengthening exercises

OTs quick and easy to reach:

·         A phone number or email address where we can reach the OT quickly

·         Quick response times from OTs

·         OTs proactive in getting equipment for boys in good time for when they need it

·         A publicly funded and publicly accountable one-stop OT service

·         Both adult and paediatric OT service

OTs training and working with educators:

·         OTs planning occupational therapy in school together with the SENCo, class teacher, and TA (and parents).

Tom's Adventures in Mainstream

Tom’s Adventures in Mainstream

A parent’s top eight tips for better inclusion of pupils with

special educational needs and disabilities

                                                                  

My nine year old son – let’s call him Tom – left his mainstream school last summer at the end of Year Two and started in September at a special school.  While we’re delighted that Tom has finally come to what is a more enabling environment for him, we’re reflecting on how Tom’s experience in mainstream could have been more inclusive.  The mainstream school was keen to be inclusive:  the Head teacher had a wonderful attitude and knew and loved all the children in the school.  Socially, ethnically, and on behaviour issues the school did seem to be very inclusive:  what could they do to include children with Special Educational Needs and Disabilities (SEND) more successfully?

Before listing what I think are the top eight tips for more successful inclusion, first a bit about Tom himself.  Tom has a condition called Duchenne muscular dystrophy (Duchenne or DMD for short).  This means he has weak muscles, poor balance, and poor coordination – he can’t run and writing by hand is laborious and difficult for him.  He needs various adaptations to help him sit and write, including a keyboard. 

Many children with DMD also have cognitive difficulties, summarised by difficulty processing information.  Like water on clay soil, information takes longer to absorb and too much information at once is overload.  Anything which needs a lot of process – like doing maths or joining up letters to read words – is difficult for Tom, thought he can tell you loads about many subjects and has a lively curiosity.  You can appreciate the challenges for a child like Tom in mainstream:  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 learns and the pace of his learning.

What can be done to address better the needs of children like Tom in mainstream?  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.  In my view, eight tweaks even to the current system would effect significant improvements.

1.    Enough Special Needs Co-ordinator (SENCo) time

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.  But the Special Needs Co-ordinator (SENCo) was there only two days a week.  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.  In our case none of these things were happening to the extent that they needed to happen.  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. 

2.    Time for teachers and teaching assistants (TAs) to plan together

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.  Without planning ahead of time it was extremely difficult for Tom to do differentiated tasks with other pupils.  As you’ve probably guessed, I have a teaching background and the year I learned most was when I planned together with a TA.  We both worked part time and had our planning meetings in unpaid time.  We should not have had to do that:  contractual time should be scheduled for such meetings.

3.    Adequate training for class teachers to support pupils with SEND

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.  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).

Teachers need to be more trained in concepts such as task analysis, backward chaining, and differentiation.  For example, Tom’s class had to do ‘free writing’.  So Tom was given a pencil and paper and ‘freedom’.  His teacher told me afterwards that he ‘made some marks and wrote the word ‘the’.  Tom would certainly have had plenty in his mind to write about, so why couldn’t his free writing have been more supported?  .  Why could he not have used a keyboard?  And an ICT programme to prompt him with the sight words he knew?   Both of these resources were sitting in the stock cupboard.

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.

4.    Adequate training for special needs teaching assistants (TAs)

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. 

Though the chain of command is from class teacher to TAs, the remit of the class teacher is the whole class.  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.

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.  Many TAs are parents.  They feel strongly the importance of giving children the best possible start in life; they want to help provide that for their pupils.  How can they undertake complex teaching tasks to support the learning of pupils with special educational needs if they don’t have adequate training?

5.    Information and computer technology

Have computers and programmes more readily available in classrooms and ensure that SENCos, teachers, and TAs have working knowledge of SEND-supportive programmes.  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.

There was a long saga in trying to get a computer for Tom at his mainstream school.  Finally my brother stepped in and got us a laptop.  We bought the Clicker and Clicker Paint programmes for it.  Then there was another long saga in making sure the teacher and TAs were confident to use the computer.  The local authority Physical Disabilities Support Service was great, going into school numerous times to train and support. I also went in a number of times.

6.    A curriculum which allows for a variety of learning styles

When Tom was in Year One his mainstream school had poor results in literacy.  So the following year they instituted a school-wide phonics programme.  This was an improvement – a systematic approach to literacy which mapped the territory.  But it was inflexible.  And Tom’s Year Two teacher was inflexible.  At the start of the year she told me that to learn to read ‘you go from letters to words to sentences’.  This approach is process-intensive – Tom’s cognitive weakness.  Progress was extremely slow.  The SENCo prescribed an extra fifteen minutes per week of phonics.  Not analysis of Tom’s difficulties and lateral thinking about solutions, just more of the problem. 

The children learned phonics through worksheets.  In Year Two Tom spent at least two terms doing handwritten worksheets overlearning letter-sound correspondence.  He began to hate school.

Though the endless worksheets were heart-sinking, Tom did end up knowing his letter sounds.   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.  Excitedly he would ask ‘what is the word of the week this week?’ and suggest words that he would like to learn to read.

While phonics was taught in each classroom, outside in the corridor, by coincidence right outside Tom’s classroom, was the makeshift hovel of the Reading Recovery teacher.  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. 

In Year One I asked the SENCo if Tom could join the pupils on the Reading Recovery programme.  No, she said, he’s too old.  Couldn’t the TAs have some training in Reading Recovery?  No, it’s a year-long intensive training course.  She did later try to get Tom on the programme, but didn’t chase it up and nothing came of it.  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.  With the support of Tom’s Year One class teacher I gave this to his TAs.  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.  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.  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).

7.    Appropriate accommodation for pupils with SEN

In Tom’s school, pupils with SEN often worked with their TAs out in the corridor, with the attendant noise and distraction.

8.    Challenging an unthinking culture of discrimination

At a meeting of the school’s disability equality working group I raised the point about the accommodation for pupils with SEND.  One of the school’s governors said ‘Accommodation is always a problem – always has been and always will be’.  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?’  She looked stunned at this suggestion. 

At one point I went in to observe for a morning in Tom’s classroom.  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.

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.  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.  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?

Conclusion

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.  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. 

Some of these tweaks are about money:  paying for SENCO, teacher, and TA time and training.  Some of them are about good pedagogical practice:  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.  Some of them take well-funded local authority support to provide continuing professional development and specific training. 

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.

A Beginners Guide to Duchenne Muscular Dystrophy

Duchenne Muscular Dystrophy: 

a beginner’s guide to its cause and potential treatments

What is Duchenne muscular dystrophy?

Duchenne muscular dystrophy (DMD) is a progressive muscle wasting condition.  It is relatively rare - roughly one in seven thousand births – and affects mainly boys. 

People with DMD gradually lose strength in the skeletal muscles.  Later the heart and respiratory muscles are involved.  DMD does not affect continence, speech, or eye movements. 

On average boys with DMD begin to need a wheelchair full time between the ages of eight and twelve.

Life expectancy when DMD is untreated is around nineteen years.  With specialist care management life expectancy can rise into the thirties and even forties.  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.

What causes Duchenne muscular dystrophy?

Lack of a crucial protein called dystrophin:

In order to work, our muscle cells need a protein called dystrophin. 

Dystrophin has two functions: 

Firstly, it is like scaffolding for the cell wall.  It holds in place a number of other proteins which all help the cell wall work properly.  Without dystrophin the cell wall disintegrates.  Secondly, dystrophin is like a shock absorber.  It is long and springy.  This enables muscle cells to absorb impact without getting damaged.

People with DMD do not have any dystrophin in their muscle cells.

  

Why do people with DMD lack dystrophin?

In every cell of the body there is a chemical ‘instruction book’ called DNA.  The chemicals of DNA act just like letters and words.  DNA contains instructions for the body to make everything it needs to grow and to maintain itself, including dystrophin and thousands of other proteins.  Each DNA ‘word’ is three ‘letters’ or chemicals long.

DNA has twenty two ‘chapters’, or chromosomes.  Each person has two copies of each of these chromosomes in every cell.  There are also two further chromosomes, called X and Y.  Women have two copies of the X chromosome and no Y chromosome.  Men have one X chromosome and one Y chromosome. 

The DNA instruction for dystrophin is in the X chromosome.

The instruction for making dystrophin (or any other protein) is a bit like the instruction for building a tower out of Lego bricks.  You can imagine it like this:

            Start with blue brick then green brick then blue brick then

red brick yellow brick red brick yellow brick red brick yellow brick

red brick yellow brick red brick yellow brick red brick yellow brick

red brick yellow brick red brick yellow brick red brick yellow brick

blue brick green brick blue brick.

No problem.  But sometimes there is a ‘mistake’ or mutation, and the instruction is unreadable.  Letters or words can be repeated, or missed out, as in this example:

            Start with blue brick then green brick then blue brick then

            rbr ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickre

dbr ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickre

dbr  ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickbl

uebr ickgr eenbr ickbl uebr ick.

Here, two letters have been missed out.  The other letters have shunted up, in the place of the missing letters.  The result is gobbledegook.

Sometimes there can be a full stop before the instruction has finished, as in this example:

            Start with blue brick then green brick then blue brick then

            red.

 The body reads up to the full stop, and the result is a truncated protein which won’t work.

If the body can’t read the instruction and can’t make dystrophin, the muscle cell dies.  Over time that means progressive loss of muscle tissue.  Muscle is replaced by scarring and fatty tissue.

 What I have outlined here are the kinds of mutation that can occur in DNA instructions.  In about 85 – 90% of DNA mutations, letters are missed out or added.  In about 10 – 15% of mistakes, full stops are put in before the end of the instruction.  .

Mutations can happen anywhere in the DNA instruction book.  Depending on where they are they cause different conditions.  Haemophilia, cystic fibrosis, some forms of sight loss, and predisposition to certain cancers are all examples of conditions caused by DNA mutations.

The body does have a back-up plan.  Every cell contains two full copies of 22 chromosomes.  Girls also get two X chromosomes.  If there is a mutation on one, the body can use the other.  However, boys only get one copy of the X chromosome.  The Y chromosome 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.  That is why it is usually only boys who get Duchenne muscular dystrophy. 

How are scientists tackling Duchenne muscular dystrophy?

Scientists are exploring a number of ways to treat Duchenne muscular dystrophy and turn it into a manageable condition.  There are four main approaches to tackling the condition.  The four approaches are outlined here, along with the main lines of research which use each approach.  The examples are not exhaustive

Correct the body’s reading of the DNA mutation

Exon skipping:  this targets DNA instructions which have been turned into gobbledegook.  It cuts letters out of the faulty instruction so that the letters get back in the right place.  In the example given earlier, you could cut the underlined letters to restore a readable instruction:

Start with blue brick then green brick then blue brick then

            rbr ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickre

dbr ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickre

dbr  ickye llowbr ickre dbr ickye llowbr ickre dbr ickye llowbr ickbl

uebr ickgr eenbr ickbl uebr ick.

If you cut these letters you would not get full dystrophin, but it would work.  Duchenne muscular dystrophy would become a milder condition called Becker muscular dystrophy:  there would still be some loss of muscle, but Becker is usually much less severe and life-shortening than DMD.  

Translarna:  this is a drug which targets premature full stops.  It aims to enable the body to ‘read through’ the premature full stop.  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.  Translarna could potentially restore full-length dystrophin.  

Bring in dystrophin from outside the body

Adeno-associated virus vectors (AAV):  this approach uses empty virus ‘shells’ to bring dystrophin into the body and transport it to every muscle cell. 

A difficulty here is that dystrophin is very big indeed, 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.  So researchers are looking at two solutions: dividing the dystrophin into three parts, each transported by a virus; and using ‘micro-dystrophin’ – finding the optimal version of dystrophin which has all the essential parts but some of the middle ‘springy’ bits left out, a version which would produce the mildest possible symptoms of Becker muscular dystrophy.

Replace dystrophin with another protein/ways to hold the cell wall together

Utrophin is a protein which does the same job as dystrophin at the foetal stage and shortly after birth.  The body then switches it off.   Researchers are developing a drug to enable the body to keep utrophin switched on and boosted up, to replace the missing dystrophin.

Ways to prolong strength or build up muscle to compensate for muscle loss

Steroids are currently used to help prolong muscle strength.  They do have some effect, on average prolonging ambulation by an average of 2 to 4.5 years depending on when they are started and the dose.  However, they can’t boost up muscle strength to anywhere near the level needed and they have a lot of adverse side effects.

Myostatin inhibitors:  myostatin is a substance in the body which stops it from making too much muscle.  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. 

Stem cells:  these are cells in the body with the potential to turn into any kind of cell.  It might be possible to direct stem cells to turn into muscle cells.