Batten Disease Awareness Day – Matthew’s story

Batten Disease is an extremely rare, genetic, neurological condition, a form of dementia which affects just 150 children and young people in the UK

Matthew’s mum, Mel, tells the story of their families’ journey with Batten disease and how they were supported by their local children’s hospice

Mel and Matthew

I guess we would always describe ourselves as a “normal” happy go lucky family, very much part of our local community. Matthew was born a perfectly healthy, whopping 10llbs. Our family was complete.

Matthew thrived as normal toddlers do up to when he was just 3. He was a typical 2nd child; I had my hands well and truly full! He was a character, a blessing and a bundle of complete and utter trouble!

It was February 2006. Matthew turned blue in the back of my car. Panic stricken we dialled 999. After being blue lighted to hospital, little did i know this was the beginning of a rollercoaster ride of extreme stress and worry.

Over time it became apparent that things were desperately wrong and a battle was ahead of us to find out what exactly was wrong with our little blue-eyed boy. Matthew’s milestones were slowly slipping away from us. What was going on?

After hundreds of hospital appointments from Cheltenham General Hospital to Great Ormond Street, being airlifted by the air ambulance twice, dialling 999 weekly, sometimes daily, mad dashes to the nearest A&E department, countless blood tests, MRI brain scans, lumbar punctures and EEGs, we eventually got the diagnosis we had been fighting for.

We received the utterly devastating news that our little man had Late Infantile Battens Disease. Our world was turned upside down, we were completely devastated.

It’s a condition that begins between ages two and four. The typical early signs are loss of muscle coordination and seizures along with progressive mental deterioration.

This form progresses rapidly and ends in death between ages 8 and 12. As few as 5 or 6 children are diagnosed with the condition, every year.

How do you deal with this? How are you supposed to come to terms with the fact that your child is one of 8 in the country. Your child is going to die.

Matthew was a healthy, normal child. We had become a family being torn apart by genetics!

This is where a special relationship began. We were introduced to Acorns, a very very special place and dear to our hearts.

What can I say about Acorns? I will admit to you when we were invited to visit, to have a look around, myself and my husband Chris argued all the way up the M5 with the stress of the thought of visiting “a hospice.”

I was terrified and completely petrified of what was ahead of me and what it was going to be like. A hospice is for those that are dying right?! I didn’t plan this trip when we planned children, this shouldn’t be part of my family’s future. This was just all so very wrong.

We arrived at Acorns. My, how very wrong we were!

Acorns isn’t a sad place, it’s a happy place, full of smiles, laughter and fun! It’s bright, vibrant. If I could take every one of you there, I would be honoured to.

Matthew went to Acorns for valuable respite so me and my family could take some time out and try to have some normality in our life. Acorns gave us that much needed time out.

Matthew’s health took a dramatic turn for the worse on his 7th birthday. We cancelled his party and in a whirlwind we found ourselves watching over Matthew on life support at Bristol Children’s Hospital.

Acorns were at our side with our wonderful community nurse firmly supporting us through this horrendous time. If I needed someone to hold my hand, it was there in an instant.

Mel and Matthew

This was a very alien experience to me and I wasn’t coping well on the inside. In good old Mel fashion I coped and seemed to be doing remarkably well on the outside. I fooled many, even my Mum, but Chris (our nurse) knew what really was going on and made us feel like we were the only people that mattered in the world. We were sleep deprived, confused and needed someone to say: ‘I’m here and not going anywhere. We will get through this and you will be ok.

After 10 days on life support we knew that Matthew’s short little life had ended and we had to let him go. We will never be able to thank Chris enough for the role she played at this time, so much love and support, making sure that we were given the privacy so we could cope with what we needed to do as a family. She never left our side but always seemed to know when we needed time on our own. A very special lady.

Matthew was moved from Bristol Children’s Hospital to Acorns with Chris’s planning, and the funeral directors. My wishes were for him to be at Acorns as soon as he passed away and never to be on his own. We wanted him to be in a place where we felt comfortable; Acorns Special Bedroom.

The care and compassion to my family and extended family was just amazing. If i didn’t feel like cooking, they gave us food. If i felt like crying, they provided the tissues. If I wanted silence, they respected that.

The one thing I will never forget, is how Matthew’s brother, Tom, was taken care of. That’s something I always worried about – how will Tom cope? They knew he loved football so before you knew it, a football was found and he was loving it, playing football until he had had enough. He forgot the real reason we were there. There were times if I couldn’t find Tom you could guarantee I would find him in the games room with other children playing on the Wii!

It’s been 11 years since our treasured Matthew passed away. Matthew was the most amazing little boy who we think of every day. He was and is adored by so many.

Acorns are very much still part of our journey. I am now lucky enough to be part of their fundraising team. It gives me great comfort to spend time where Matthew not only loved but also where he last was, and to be able to raise awareness of Acorns every day.

There have been wobbly times, which is to be expected – to know there’s someone who we can talk to is invaluable. This relationship will always be a given from Acorns.

I promised Matthew that I would always keep raising awareness of Acorns, for him, and all the other children and families they care for.