obstaclemudrunner.co.uk
ForEliteAtheletes toFunRunners
40
My Amber by Jennifer Shackleton.
My niece Amber was diagnosed with Infantile
Batten Disease at only 2 years old. Amber grew up
a seemingly healthy vibrant child, reaching all her
milestones but at 17 months, she began to regress.
My sister and her husband took her to the doctor as
she stopped holding her cup, and had “unusual”
behaviour. They were told there was nothing
wrong and she was just “being lazy”. She began to
deteriorate some more and soon stopped walking,
talking and holding objects. She continued to regress
at a rapid rate. Amber’s parents decided they wanted
a private diagnosis as even they knew this was not
normal.
She could no longer sit up, was diagnosed with
epilepsy, had severe mental impairment, chronic
constipation, could not chew or swallow,
lost her sight, suffered uncontrollable
seizures and lost all abilities. This
disease took over extremely rapidly and
Amber sadly lost her fight in February
2016.
About Batten Disease.
Batten Disease is the common name
for a group of diseases called the
Neuronal Ceroid Lipofuscinoses (NCLs).
They are rare, genetic, progressive
neurodegenerative diseases that occur in children
and adults worldwide. Batten Disease is the main
paediatric neuro-degenerative disorder in the UK.
It affects 1:30,000 births and there are approximately
200 affected children and young adults in the UK.
They are born apparently healthy but deteriorate
rapidly. Expected life expectancy is 5-30 years.
There are 3 main types of Batten Disease, affecting
children at different ages depending on which
defective gene has been inherited. Infantile, Late
Infantile and Juvenile Batten Disease. There is also
a congenital form and other variants of the disease.
There is much research into all types, but no cure at
present.
Over the past year I have been running races to raise
awareness and fundraise for the charity. I knew a
cure would be too late for Amber but I still couldn’t
accept it. There are many more children and families
that still have some hope.
I’d like to thank a few people whose support has
been quite overwhelming at times and I’m humbled
to be able to call these people my friends
and my family.
Race directors and runners alike have
shown support and for that I’m grateful.
Judgement Day turned Bordon orange
(the charity colours) in memory of my
niece one week after she died.
The team at Mudstacle really pulled
together and helped to raise over £150
by painting faces orange.
I’ve had some extremely tough days where I just
want to cry as I had felt like I had failed my niece,
that there was no point in me racing anymore as my
inspiration had passed away but then I remembered
all the other children that still have hope and I have
to carry on.
I’ve had some
extremely tough
days where
I just want to
cry as I had felt
like I had failed
my niece
INTERVIEW
Jennifer Shackleton on turning orange...
If you’d like to know more
or how to help with fundraising
you can email me at
or contact the charity Batten
Disease directly via the website
When the OCR
world turned orange
You might remember back in February, our usually muddy world went orange.
Here’s why…
During the time I’ve been doing this, I was also selected as one of 50 Britain’s toughest
mums and got to run Tough Mudder with Ben Shepherd on good morning Britain which
raised massive awareness. I’ll never give up hope of finding a cure.
