Monday, May 14, 2012


On Sunday it was six months since Estella passed away. People were very kind as always and we had thousands of messages and photos and balloons and candles. We spent the whole day keeping up to date with all of the very kind support.

How different to the dark November Sunday evening when we sat by her bed. She had died that afternoon and we had bathed her and dressed her for the final time. If I had to choose one moment from the whole of my experience with Estella that will live with me forever it was watching Maria bathing her little body and preparing her for her final journey. I have never seen anything as heart rendering and sad. I would give the world not to have had to see that.

The reason I am trying to smash SMA is because I do not want other parents to be there in that room holding their child for the last time. Watching the water wash over legs that can no longer splash and eyes that can no longer see. The dearest person I have ever known silenced and stayed by the most evil and damned of diseases.

I sat by her bed that night in that cold cold room and read her her story book and sang her her songs and then I got down on my knees and I apologised. I apologised to my little Tinker that there was nothing I could do to have saved her and there was nothing I could do to protect her and comfort her. There was nothing I could do.

As a Daddy that makes me feel weak. As a Daddy that means I failed. I failed because the most hideous monster that could exist came to take my daughter away and nothing I could do could win that fight. I know I tried but I was nothing compared to Maria and the amazing 24/7 care and love that she gave Estella. I could not save her. And as I knelt I promised her that i would try my very best to make sure that people heard about this disease and I would not rest until it was something that people no longer needed to hear about.

Six months on and awareness has been raised. We are putting politicians and celebrities and scientists in the same room and moving towards solutions.

That did not matter last week though.

Last week we went to the park in Spain where half of Estella's ashes sleep in a lovely fountain. We took her toys and sweeties and a balloon and sat and had a few words. A family came up, as always, and took the gifts away for another child. For another time. For another place.That felt good.

Sat there feeling warmth on my skin and cool breeze in my hair I thought of Estella and how she had always seemed to be so wise and so knowing. I looked at other children and for a moment I wondered why she could not have had more time, more experience, more life. I know the philosophical debate that she lived a full life and one without pain and that she was very loved but she deserved more- she deserved so much more.

Six months on and life is starting again. The shadows of the night are not always laced with fear and there are still rainbows and there are still moments of beauty and peace. The world is an amazing place and I was gifted for a short, too short, while with the most perfect little daughter. Her little body did not work. Her little mouth could not speak. Her little legs could not run


Once when I was sat by her side at three in the morning - trying to think of reasons to go on I sat with my head bowed. I looked up and saw that she was awake and I remember one thing.

Her little eyes could cry.

A single tear ran down her cheek and she looked at me with such love and such care and such concern. She was crying because I was sad. She was crying because I was crying. She was longing to cuddle me and make it all right.

Last week when we sat by her fountain Maria said to me that it was OK to cry but she tried her best not to because if Estella was watching she may think that we were crying because we were annoyed at her or diasppointed that she left us - and that broke my heart.

Wherever she is - six months on - wherever she sleeps - wherever she dreams she has to know one thing ... My darling little Tinker , I could not have been more proud of who you were and who you are and who you will be. No Daddy ever had a daughter so beautiful, so wise, so perfect and so capable of love.You came here for a reason little monkey and if that reason was to bring SMA to an end one day sooner and to ensure that other babies live and that other Daddies dont cry then all is fine and all is fair and all is peace.

Adn if I tell myself that then I feel Ok
And if I tell myself that I dont cry
And if I tell myself that then I will SMASH SMA

Even though its never - never- never fair.

I miss you with every heartbeat and every breath. I love you with everything I am.

I could not be prouder of the changes you are making to this world.

Maybe I failed but I know one thing. Tinker didnt think so. She forgives me

You wonderful people are helping to keep her spirit alive.


Six months on. What do I think we have achieved ?


Just love

And I would give anything I own
Give up my life, my heart, my home I would give everything I own
Just to have you back again
You taught me how to love
What it's of, what it's of
You never said too much but still you showed the way
And I knew from watching you
Nobody else could ever know
The part of me that can't let go
And I would give anything I own
Would give up my life, my heart, my home
I would give everything I own
Just to have you back again
Is there someone you know?
You're loving them so
But taking them all for granted
You may lose them one day
Someone takes them away
And they don't hear the words you long to say
And I would give anything I own
Will give up my life, my heart, my home
I would give everything I own
Just to have you back again
Just to touch you once again
Just to speak to you - words again


Somebody asked me the other day if I really think we can SMASH SMA or if its just a campaign to raise attention about the disease that is the number one genetic killer of children - a disease that is carried by 1 in 40 of us.

I hope this blog gives the answer

Those of you who are regular readers will be familiar with the work of Prof. Gillingwater and his team in Edinburgh. They are leading research on finding solutions to SMA and we are backing them by encouraging awareness and funding for them.

I had the following mail from Tom today

Hi Tom & Maria,
I hope you are both keeping well.
I wanted to update you on some very exciting progress in our SMA research that has come through over the last couple of weeks:
- First, we have had a huge number of high-quality applications for the Estella Star SMA Scholarship. We are going to be interviewing over the next couple of weeks and will let you know who we have appointed ASAP. We will ask them to write a short report for you when they finish their project to let you know how they got on.
- Second, we have made some really exciting progress with identifying a potential new drug for SMA. We are going to use the remainder of the funds you have helped raise to undertake pre-clinical testing in SMA mice. Any additional funds that come in will also be used for this trial (we are also hoping to approach some of the large funding bodies in an attempt to raise some serious cash for this...we need at least £50k!). If the results of this trial are positive, and show a beneficial effect of the drug in the SMA model mice, we would hope to try and move the drug towards human clinical trials as quickly as possible.
Both of these developments would not have been possible without your incredible advocacy and support. I can't begin to thank you both enough. I hope that you feel that these developments are moving things in the right direction and that together we can SMASH SMA!
Very best wishes,

They have made some really exciting progress with identifying a potential new drug for SMA and they are going to use the funds you donated to trial this on mice.

The really exciting news though is that a donation of £ 50,000 will enable them to move the drug towards human clinical trials.

That is earth shattering

There are millions of pounds donated to charities and pharmacutical firms to help solve SMA and here is the team who may have the solution and they are based in the UK. That has to be worth a 50k gamble.

We will now dedicate the fund rasing side of SMASHSMA to trying to raise the 50k that Tom and his team need. Every donation counts.

Here is the web link to make a donation if you would be so kind.

The idea of there being a scholorship in Estella's name is moving beyond words.

Am i serious about SMASHING SMA ?

It's what I promised her.

We are going to cure this disease.