How CLN2 Batten disease changed our life: “A Mother’s Love”

16 May 2024

My name is Gail Rich and our two daughters were both diagnosed with CLN2 Batten disease in late 2016. We also have a son, Louis, who is 13 years old.

In October 2023, we very sadly lost our precious daughter Nicole. Nicole was 11 years old. We are absolutely heartbroken and are just taking things day by day and keeping strong for our other children.

Jessica continues her Batten journey alone but everything she does, her big sister Nicole will be with her.

My husband Matthew and I are passionate advocates for Batten disease and rare disease and we write on ourFacebookpage about our experiences.

The poem I am sharing here is called “A Mother’s Love”. It is about how our life changed with a rare disease and my love for my children. It was read at our darling Nicole’s funeral.

A Mother’s Love

The moment you lay on my chest I just knew,

I had found my life’s calling when I looked at you.

The whole world stood still, our heartbeats – the noise,

As I kissed you and felt overcome with the joys.

Of knowing we made you and you grew in my tummy,

The miracle of life, I felt blessed as your mummy.

New feelings of pride and protection then rose,

Breathing you in from your head to your toes.

Overwhelming, instinctive, like nothing I’d known,

No amount of preparing could ever be shown.

For it comes from inside and is truly surreal,

To be filled fit to burst with the love that you feel.

My life had new meaning and you were my soul,

I was blessed with the gift of my new mother role.

Your fierce protector, shielding harm from your path,

Should anyone hurt you they shall feel my wrath.

I will love you and guide you, encourage and cheer,

Wherever you are I will always be near.

You are my everything and my love will endure,

Unconditional, unwavering, wholesome and pure.

The ups and the downs and the challenges we face,

Shall be met with the unity of a beloved embrace.

Staying buoyant beside you, your Daddy and I,

From newborns to now in the blink of an eye.

There is no crueller hand than the one you’ve been given,

Yet you face it will grace and through love you are driven.

Thank you, my darlings, for all that you are,

Each one of you sweethearts, a unique guiding star.

You give me the strength when I feel I have none,

Unaware of your power to rouse what was gone.

Though tinged with a darkness from which we can’t hide.

As we walk through together, I am there by your side.

How can I fall when you endure far more?

Giving in to the weight and reduced to the floor.

It is not going to happen, I will stay strong for you,

Our family, our world – we will always pull through.

Every moment is special, every day is a gift,

Health is a blessing and life brings a shift.

What a privilege to watch you flourish and form

Establishing a life far removed from the norm.

Role reversal through the passage of time,

From carefree and able to a subtle decline.

Opportunities gone, limitations creep in,

Yet adaptive and positive, a new path you begin.

Resilience personified, is the most perfect way,

To describe our sweet Coley, who brightens our day.

Despite all the challenges you faced every day,

You were paving a path, you were leading the way.

A way filled with love, sprinkling hope all around,

Bringing light to the darkness so deeply profound.

Your resilience and bravery are traits to admire,

Unknown to yourselves, you are ones who inspire.

My role is evolving, adding strings to my bow,

Strings I would never have wanted to know.

But each one essential in this rare disease world,

Not knowing their existence until health needs unfurled.

Being your Mammy is a blessing beyond measures,

I am devoted to you, my three little treasures.

Unconditional and boundless, a love like no other,

Infinite and pure is the love of a Mother.

About CLN2

The gene called CLN2 lies on chromosome 11. CLN2 disease is inherited as an autosomal recessive disorder, which means that both chromosomes carry mutations in the CLN2 gene, and both parents are unaffected carriers…

CLN2 normally directs production of a lysosomal enzyme called tripeptidyl peptidase1 or TPP1. A deficiency of TPP1 results in abnormal storage of proteins and lipids in neurons and other cells and impaired cellular function. The cells cannot function as they should and symptoms develop.”

Source:h ttp://www.bdfa-uk.org.uk/cln2-disease-late-infantile /