Amanda and son James family portraitJames and sister




My son James was born perfectly healthy in March 2008 and as time went on he met all of his important milestones.

He was walking at 18 months while holding one of our fingers, and he could say the odd word such as “Dada”, and “Ozza” (Oscar our cat).

He was into everything–pinching my lunch, pulling the cat’s tail and helping us to decorate by putting his full arm into the paint tin! All with the cutest smile.

I was pregnant with my second child and life was good.

Then the seizures started. Short, quick jerks that left James floored. After many hospital tests he was started on medication.

When our daughter Ruby was born two months later it was clear that something was very wrong with James. He had stopped crawling, walking, standing and was struggling to sit.

He was a different boy – like something had been switched off.

But when the seizures changed, we had a glimmer of hope: hospital staff said it might just be epilepsy that needed controlling. We felt such relief.

No words

When we were asked to meet James’ hospital consultant in January 2010 we thought it was to discuss these new seizures.

I hated going to the outpatients department because it was always so busy but this time it was…empty. Two nurses were waiting for us. Alarm bells started to ring.

The consultant called us in and gave us a diagnosis: Infantile Batten Disease. There was no treatment, no cure.

Our son James, now nearly two-years-old, would not live beyond his fifth birthday.

There are no words to describe what it feels like to be told this. “Shocked”, “devastated”? Not even close.

We learned that Infantile Batten Disease is a neurological degenerative disease–causing James’ brain cells to die.

We were handed a sheet that read: ‘over time affected children suffer mental impairment, worsening seizures and progressive loss of sight and motor skills. Eventually, children with Batten Disease become blind, bedridden and demented. It is always fatal.’

Because it’s a genetic condition we had to have Ruby tested as well. We waited a week for her results and when they came back clear the relief was also indescribable.

Two choices

We had two choices: to let this diagnosis ruin our family or dust ourselves down and live.

We never expected to be faced with this but we owed it to our children to give them the best life possible, however short that may be for James.

So we pledged to make the days count. Bravely or stupidly, we also decided to have another baby. A roll of the dice…

Fortunately, early pregnancy testing is available and we learned that James and Ruby’s new sister Isabel would be clear of the disease. Our family life took on a new dimension.

Loving life

James is proving the doctors wrong. Yes, he is severely visually impaired, immobile, tube-fed and cannot speak but he has outrun that fifth birthday milestone and he is loving life.

He goes to school where he has friends. He loves hydrotherapy and the sensory room. He smiles to show us he’s happy. He has started opening his eyes wide to communicate with us and if we’re really lucky we get a laugh or giggle.

He loves cuddles and having his legs and arms wiggled. His sisters are so caring and thoughtful with him.

James needs 24/7 care and it’s exhausting – it’s the hardest job I’ve ever done (and I was deputy head of an inner city primary school!) but I love every minute.

Respite is key to staying upbeat and positive

We stay at The Donna Louise Children’s Hospice as a family and I get a complete rest as James is so well cared for.

There we become ‘normal’. I chat to other mums about feeding pumps and suction machines (other mums without special needs children would have looks of horror on their faces!).

There’s counselling, sibling groups and help with equipment. During our stay I can be a mother to all of my children and not a nurse. I leave the hospice a new woman.

This respite is key to staying upbeat and positive.

We’re not sure what the future holds for James but we do know it would be a lot harder without the hospice’s care and support.

Another of my favourite quotes is: ‘Life is not measured by the breaths we take but by the moments that take our breath away’. James provides us with so many of those.