Sunday, December 30, 2012

NEW DAWN, NEW DAY / FEELING GOOD

Stunning News - For the first time, EVER, SMA is going to be reviewed by the Govt with a view to pre-conception screening.



We have received a letter from Norman Lamb MP, who is the Minister of State for Care and Support at the Department of Health.

It is a lengthy letter, that is copied in to the Deputy Prime Minister  but here is a summary

Dear Tom and Maria

I wanted to write to congratulate you on the success of your evening reception on September 18 , and to commend your work to raise awareness of spinal muscular atrophy (SMA) through the SMASHSMA campaign. I know many MPs and supporters ( myself included ) were moved to hear the story of your daughter Estella. I was also impressed to read your blog setting out the successes of your campaign, and wish you every success in the future on campaigning on what is an important issue.

I am the Minister responsible for policy relating to long term conditions , and following your reception, I asked my officials to investigate the possibility of setting up a screening programme for those who might want to be tested for SMA

The letter then goes on to say that

  • The UK National Screening Committee ( UK NSC) are responsible for advising ministers on all aspects of screening policy
  • UK NSC will now review SMA screening programmes including pre-conception, antenatal and newborn screening for SMA.
  • This is the FIRST time the UK NSC have considered SMA for screening
  • The review will take the form of a PUBLIC CONSULTATION in the new year. More details of the consultation will come through next year. In the meantime any person or organisation wanting more details can look at www.screening.nhs.uk
Once again congratulations on the success of your reception , and I wish you the very best in your future campaigning endeavours

NORMAN LAMB

I will be responding personally to Mr Lamb and Nick Clegg this week but what does this mean for SMA

1) There is now no need to get 100,000 sigs to bring SMA to the Government's attention. This has now been done.
2) For the first time ever, screening for SMA is being consulted on
3) When the consultation starts we will be calling upon SMASHSMA followers to be vocal about the need for screening and SMA research. We will be asking stakeholders to register for the consultation and to make their views known.
4) Above all it means that as 2012 ends we have achieved the first goal of the SMASHSMA campaign. We have brought SMA right to the front of government and medical screening attention. Through your efforts SMA has been raised from a disease that nobody had heard of to one that is now firmly on the government and DOH radar. By any stretch of the imagination that is a major achievement. Thankyou to every person who has followed the campaign this year. Well done - you wonderful people
5) Well done to Mr Lamb and Mr Clegg too for taking this matter seriously and for moving SMA up the political and research agenda.



So the year ends on a high as far as SMA and the possibility of screening is concerned.

What matters now is to make sure that the consultation is done correctly and that the spirit and the drive of this cause gets through - in the end this is all very, very simple. We are talking about a disease that is carried by 1 in 40 people. If those people are unlucky and have a baby born with SMA type 1 then there is no cure. That baby will die. It would be good if those 1 in 40 people knew who they were so they can take informed decisions and have choices.

What parent would disagree with that.


Estella's life was short and free from pain. That said we had to kiss her goodbye when she was only eight months old. If her legacy is that other parents don't have to go through that pain then I am sure she would be very happy. I have told you before , my little girl had wise eyes and an old soul in a little body that didn't work too well. She would very much like the idea of sparing sadness, she would very much like the idea of helping others.

Thankyou to everybody who has given so generously of their time.

Put your feet up for a couple of days. We#re going to need you again soon.

It's a new dawn , it's a new day - and we're feeling good.

Friday, December 28, 2012

STOP PRESS

Two days after I posted a blog regarding disappointment at a lack of follow up from the reception at the House of Commons I am told a letter has arrived at home from the Minister for Health giving details of a consultation.

I would still argue that this is not what was suggested at the time but will wait to read the letter before commenting. In the meantime please keep this in mind when reading the blog entitled The Good, The Bad and the Pretty.

I would very much like to believe that the support shown in September was genuine and remain open minded.

Tuesday, December 25, 2012

AND TO EACH A GOOD NIGHT

Christmas Day

It's an entirely different feel to last year. We are in England rather than Spain - Cristina is due in 8 weeks or so which means we stayed here and had Maria's parents over for a few days. I know she is  a wee bit sad but we have had a good day and there are a fair few presents flying about ( literally in the shape of my remote controlled helicopter )

We had a trip down to Estella's tree this morning. I had never noticed before that it is an evergreen and that it was the only one by the pond still to be green and brimming with life. As always as soon as we touched the wood time melted away and we were back with Estella. Heart wrenching tears from Maria and that feeling I get as if my soul had died with her. Anybody who saw us would not know whether to laugh or call the Funny Farm as we hug a tree from both sides and talk to it as we cry.

We always take a present along. Today we wanted her to have her first ever orange and so peeled it and left the flesh and skin to decompose into the ground but we also took a Gingerbread house. An inspired present choice from Maria. Usually we leave the present there in the hope that a family comes by and takes it but today it soon became clear that the birds were interested. We took off the wrapper and set the house down in front of Estella's tree so she could have a good view.


Birds are daft

They flocked around the house but did not realise that they could eat it. It was like watching the apes in 2001 circling the monolith until one of them develops the courage and intelligence to touch it. Eventually after breaking pieces off they worked out it was edible and started to feast. Our tears had gone and we were laughing at the birds and the Christmas morning show.


Things are better - we know that

But they will never be the same. All that happens is that the heartbreaks creep up on you sometimes when you are least expecting them. The nice thing is that the happy memories also suddenly slide into focus at unusual moments. I still feel Estella in the air and hear her in the night. I still miss her with more pain than I ever thought possible.

I know that it's a special day to day but I have been so annoyed with myself for not updating you all on what is happening. We have a little girl arriving in February - today Maria held her bump against Estella's tree for her to meet with Cristina. They will have a lot in common. We know that Cristina will be her own person though but one day when we can we will tell her about the little diamond who paved the way for her. The little girl who showed more bravery in her few short months than I have seen in a lifetime.

We will tell Cristina about her big sister - and we will tell her with smiles and wonder

And we will never forget her and the life she gave us

Happy Christmas everybody - cuddle your loved ones and sleep tight

And to each a good night .



THE GOOD , THE BAD and the PRETTY



It's been a while.

There has been a lot going on. It started out that I did not want to write up details of the trip to London for the SMASHSMA reception with Nick Clegg as I wanted to be able to report on outcomes. I wanted to give time for things to settle to evaluate how succesful the event had been. For the background please see earlier posts.

So much has happened I hope you will forgive me just using bullet points


  • The reception in London was a massive success. We had the Deputy Prime Minister, Nick Clegg, and the Health Minister, Norman Lamb, there as well as numerous MPs and other guests. At the time we were delighted with the session which went on for two hours. The Gillingwater SMA team from Edinburgh were on form revealing some groundbreaking SMA research that had been directly funded by your contributions. An amazing evening.
  • Mr Clegg made a speech and instructed Norman Lamb to look into the possibility of testing for the gene that is carried by 1 in 40. He took our details and said that as he had just come into the post it would take 3 to 4 weeks to contact us to discuss in more detail how he could help. Mr Clegg had already asked for a consultation into SMA and we were awaiting contact on this.
  • The London reception could not have happened without Andrew Roddison and Morrisons Facility Services who sponsored the reception and made sure that we had a wonderful memorable day in London. They have been great supporters and tremendous advocates for smashing SMA funding sponsorship at Rotherham United and paying for every penny at the reception.
  • We had support from some of our most vocal tweeters and that was one of the main joys of the day - meeting some of you in the flesh. It was great to see people who had been so vocal on Twitter having their time in the sun too. Maria did point out that they were all female and all pretty but I put that down to sheer random luck :)
  • Natalie Cassidy came down and was a great help on the day.She played the room and knew her stuff and gave a touch of celebrity and glamour to the event.The support of Natalie has been a cornerstone of the campaign. She is a true star.
  • We were told by the Health Minister that he was very moved by our case and that he would be in touch in three or four weeks - that never happened. To be honest that has been the first major set back of our campaign. The Government showed interest on the evening but did not go through with their promise to stay in touch . Everyone tells me I was politically naive to trust them and I apologise to everybody who has followed our campaign for doing so. 
  •  It has now been almost a year to the day that we first met with Nick Clegg. I was convinced on the night that our presentation had moved him and it would have been a perfect way for Mr Lamb to make a splash right at the start of his new position. My old Headmaster always said that people should be judged not by their words but by their actions and as nothing has happened we have almost given up hope of true political support. I MUST point out that this is not a political decision. It was never about parties - It was about our little girl Estella and we trusted Nick Clegg because he is a Husband and a Father. There are probably good reasons why we have not been contacted but it would have been nice to update us with progress.
  • For clarity - the Gillingwater campaign needs £ 50,000 to keep going strong for 2 years. It is our belief that the breakthroughs they are having will make a MAJOR difference to the SMA experience for thousands of families. Tom Gillingwater forbids me from using the word cure ever - but he knows what I think :)
  • We have continued to try and raise money for Tom Gillingwater - this will increase soon due to the kind donation of 1000 SMASHSMA bracelets and envellopes by twitter followers. We will be passing over the first £ 300 or so in January and there will be a monthly donation beyond that.
  • We have moved house into an amazing place that has a lovely nursery for Cristina ( note new Spanish spelling ) 
  • We have had Maria's parents over for a pre-Christmas Christmas. Great success and those of you who remember last Christmas will be pleased to see them looking so genuinly happy. They were our greatest rock during our short time with Estella and its now time for them to have the happy years they deserve.
  • As I said there has been a lot going on and this is only a brief catch up. All goes well with Cristina - probably looking at mid February for her birth. Maria is doing an impression of the Goodyear Blimp and all tests are looking good.
  • The Twitter account now has 41,500 followers and interest is as strong as ever. We are as grateful as ever for the support. We still get daily messages asking what SMA is - and that pleases me - my theory is that every person we tell can possibly become an advocate and there are some amazing people out there.


Sunday, September 9, 2012

STARS and BRAS

As we get ready for the serious business of the SMASHSMA reception with Nick Clegg at the House of Commons there is a bit of other business going on that may raise the profile of Spinal Muscular Atrophy even higher.

We decided early on in this campaign that if we were going to alert people to this dreadful disease not everything could be about the disease itself. To capture attention , imagination and inspiration you sometimes have to appeal to senses other than empathy.

We could not have a better example than the wonderful celebrity art bra auction that will take place later this year.

First of all let's introduce you to the splendiferous Linda Kirkbride @lindakirkbride1

LINDA and HER ART BRAS






Linda came up with the idea of creating a bra that could be auctioned to raise money for the official SMASHSMA charity at Edinburgh University.

SMASHSMA CHARITY DONATIONS PAGE


She had made the bras before themed around the charity that she was supporting. Straight away it was suggested that the theme would be STARS - Linda set to work getting her materials and we started to think about how to get the highest possible donation.

As you know we have had amazing support ( no pun intended ) from celebrities ever since Estella died last November. Just this week Tom Cruise tweeted her details to millions of people. It made sense then that if we could get a celebrity to model the bra then this would raise the profile of SMA and at the same time increase the bids that we would get towards research.

So off we went to Natalie Cassidy who has been amazing since her first tweet about Estella.




 I asked if she knew anybody who may model the bra ( secretky hoping that she would say that she would ) - Natalie came back though with another suggestion - and that's how we met Nicola Mclean.



Nicola has been fantastic and straight away said taht she would be the model for the charity art bra. One of her bras is currently winging its way to us so that Linda can start work with the arty side of the project. Linda has not only been so supportive and enthusiastic she has also managed to get us hundreds of followers with her retweets. She has also spread the word among other celebrities who are also retweeting for our little Tinker, Estella. An amazing lady - please follow her on Twitter @mrsnicolamclean

So there we have it. Sometime before the end of the year Nicola will be modelling the bra and we will be holding a charity auction. There is talk of Linda making more bras for more celebrities as well and we are being followed by two excellent candidates in Natasha Giggs and Heather Bianchi



Two more celbrities who mention Estella a lot.

I can never say how gratteful I am for all of this enthusiasm and commitment from celebrities who are going the extra mile to alert people to the number ONE genetic killer of babies. There are some superb people about and I know that Estella would have had a cheeky smile for all of them.

OK - anybody want to start the bidding for a CELEBRITY SMASHSMA BRA ?

Thanks Nat - You are unbleievable.

and Nicola - you're an Angel






Friday, August 24, 2012

LONDON CALLING

September 18th will be an important date in the fight against Spinal Muscular Atrophy.

We have a confirmed House of Commons reception with Nick Clegg, the Deputy Prime Minister from 6 pm - 8 pm that evening. This follows a constructive meeting we had with the Deputy PM earlier this year.

This is an opportunity to


  • Explain what SMA is
  • Explain that 1 in 40 of people carry the disease
  • Highlight the support and research that surrounds SMA in the UK
  • Stress the need for awareness and how screening could improve the options available to parents
Morrison Facilities Services have been tremendous friends to the SMASH SMA cause and they will be sponsoring the reception. Natalie Cassidy will attend to add celebrity weight to the message.

The most important thing now is to ensure that as many MPs as possible attend the reception and that is where you come in. 

It would be terrific if you would email the Member for your area to ask if they would attend the reception. I have put the suggested wording below.


Dear .............


Spinal Muscular Atrophy Awareness Reception

I am writing to inform you that a reception is being held in the Macmillan Room of Portcullis House on Tuesday 18th September from 6 pm - 8 pm. The reception is being hosted by the Deputy Prime Minister, Nick Clegg.. The reception is to raise awareness of Spinal Muscular Atrophy (SMA). 

  • SMA is the number one genetic killer of babies in the UK
  • 1 in 40 adults are SMA carriers
  • If both parents are SMA carriers then their baby stands a 1 in 4 chance of having SMA
  • A simple genetic test would alert parents if they were carriers

The evening has been organised by Nick Clegg following a meeting with two parents who approached him following the death fo their daughter in November 2011. Estella Sweetman-Lopez was 8 months old when she died from SMA



Estella's parents have worked tirelessly to raise awareness of this disease. They have 

  • 35,000 followers on Twitter and Facebook
  • Raised over £ 40,000 towards SMA research
  • Had national newspaper coverage for their SMASH SMA campaign
I have followed and supported their campaign closely and would be very grateful if you would attend this reception on behalf of myself and the parents who have been affected by this disease from within your constituency. Research suggests that the cure for SMA may be very close - and it is the UK that stands a very good chance of unlocking the genetic solution. How wonderful if this campaign resulted in a cure being found for the number one genetic killer of babies.

For more details of the background to Estella please look at these two links. The first one is a moving video that has been seen by over 50,000 people. The second gives details of Estella's life. . 



I know that if you look at the information you will understand the importance of supporting this tremendous cause. May I thank you in anticipation for your support in raising awareness of a disease that could affect any one of your constituents


YOUR NAME XXXXXXXXXXXXXXXXXX




Please take a moment to send that to your MP

 You can find their details here




Last thing for this blog.

I mentioned Morrison and their support of the SMASH SMA campaign. Just look at this tremendous sign that they have put in at Rotherham United's new stadium for the forthcoming season. They have been tremendous supporters and could not have been kinder.



Wednesday, August 15, 2012

KATIE FUTURE STAR



As part of our support of the Gillingwater-Parson research work we were delighted when a scholarship was set up in Estella's name. We were even more delighted to receive this report from Katie Hoban detailing her work within this innovative environment. This is Katie's report 

Estella Star Scholarship Report

Having just completed my BSc degree in Neuroscience and looking forward to beginning my training to become a medical doctor at the University of Edinburgh, I was keen to spend time in a scientific research laboratory. I am particularly interested in the way in which basic research will inform my future clinical practice and when I saw the advert for the Estella Star Scholarship I immediately applied. I was delighted upon hearing I had been awarded the scholarship and the chance to work in a leading SMA research laboratory. My project was to be supervised by Dr Simon Parson and entitled “The role of the vascular system in Spinal Muscular Atrophy’.
You may be surprised that I was not to work on motor neurons, but instead the vascular system.  This is because it is becoming increasingly apparent there are other key targets, such as the heart and blood vessels, in the disease as well as the characteristic muscle atrophy. Previous research in the laboratory shown the amount of blood vessels in muscle is dramatically reduced in mouse models of severe SMA. My project was to investigate the blood supply to the spinal cord (where motor neurones are found) and nerve (which connect the motor neurons to muscle). First we carried out a detailed analysis of normal tissue upon which to base future SMA studies. I learnt new techniques to isolate specific tissues, prepare them for microscopy, stain them to highlight blood vessels and then quantify the amount of blood vessels present. This was challenging at first, but with help from the other students in the laboratory I soon became proficient and was able to collect data. We found that the newborn spinal cord was sparse of blood vessels, but steadily increased over the first few weeks of life. We specifically noted changes in the part of the spinal cord where motor neurons are found. Peripheral nerves were much better supplied with blood vessels at birth. This is important as defects in blood supply to either of these regions could have catastrophic consequences for motor neurons. I presented this data at a recent symposium on Motor Neurones and Diseases of Motor Neurones, and hope to submit it for publication later this year. Finally we began to assess tissue from mice which model severe SMA and found some potentially very interesting results, but these are too preliminary for us to publicize yet, so watch this space!
Working in a hospital environment in recent months has highlighted the fact there are patients in urgent need of therapies for currently incurable diseases. To be given the opportunity by your incredible Estella Star fundraising efforts, allowed me, in a small way, to add to current research. When previously speaking to you both during your visit to the lab, I was struck by your profound dedication and personal commitment to heighten the profile of SMA and I vowed that by the end of my placement, I would be able to report new developments which would provide a platform of information to help further understand the disease pathology of SMA.
I will make it my utmost effort to follow the progress of the Gillingwater-Parson lab because I sincerely believe that they are at the cutting edge of scientific research and with the advances to date, we are en-route to answering many of the unresolved questions in SMA and together with your fantastic fundraising efforts, are another step closer to SMASH SMA.
I can only thank you for this unique opportunity and have nothing but respect for your dedication to help us find treatments to the fatal disease that is SMA.
Katie Hoban

Thursday, August 9, 2012

Saturday, August 4, 2012

STRAWBERRIES, CHERRIES and AN ANGEL'S KISS IN SPRING

For everything there is a season and a time for every purpose... under heaven


Following the death of our daughter , Estella, last November I knew two things. I knew that I had to do what I could to alert the world about SMA and I knew that Maria had to one day become a Mummy again.

I'm OK at this Daddy lark. I have my moments but Maria is a natural. She cared for Estella with such love and devotion and ever since we had to say goodbye to her I have seen a Mummy who has had her soul and heart ripped away. It was essential that this devotion should have a child to be bestowed on.

We had made our peace with a number of things during those sad, beautiful, tortured, memorable days that Estella was dying. I had sat by her bed and explained to her that although we would want another child they would never replace my Tinker in my heart. I asked her permission to let life carry on and asked her to give signs that she was OK. Of course there was guilt with wanting another child - of course there have been moments when I have asked if it is fair. Of course Estella was my cheeky monkey.

We were originally going down the IVF route. By doing that we could guarantee that our next child would not be cursed with SMA. After a couple of months of discussion though we decided that the odds were low and that the route had its own problems. So in May we decided to go for the natural method and to hope ...

And that's what we did.

The odds were in our favour but still amazingly scary.

By the natural method there would be a 1 in 4 chance that a new baby would have SMA
There would also be a 2 in 4 chance that the baby would be a carrier of SMA

Then things got worse

Maria was told that her reserves were low. On the score system they use she needed to be 16 and above to be fertile. She was a 2.

Maybe all options had gone

Within three weeks of trying Maria told me one morning that she was pregnant.

And then the waiting began

At first the wait was OK. We knew that the odds were probably in our favour and rested on that but as time went on we started to worry more. Maria told me every morning how big the baby was a pea, a bean, a cherry, a strawberry and she was also being incredibly sick.

This was becoming real

No test can be done on the placenta DNA until the 11th week. When that test is done the result takes about another week. We were clear - if this was SMA then we would have very difficult decisions to make. If we chose termination it would be done within a day or so.

The problem was though that we had already seen the little bump twice on a scan.


The shape was very clear. The hands , the legs, the head and the beating heart.

Last Tuesday we went to do the examination that would decide the future. While they took their sample through a very big needle poked into Maria's tummy the bump was kicking its legs and waving. I sat watching and saw it wave. I thought it was waving goodbye.

We knew that we had to wait until the following Tuesday for the result.
Yet this Friday morning when we woke up ( yesterday ) we knew that this would be the day.

Maria received the phone call at work and rang me.

It was healthy and it was a girl.

And in that moment the rooms and the conversations and the tubes and the held hands and the smiles and the heart stopping moments and the bluebells and the songs and the rhymes and the tears and the goodbyes and the decisions and the revisions and the nights and the bent knee prayers and the cuddles and the dreams and the dragons and the lights and the dawn and the last gentle touch of her gorgeous hand - in that moment all the moments became a  moment.

and I felt nothing but calm.

We are going to have  a baby girl next February. The baby will arrive on , or around , Estella's birthday.- if there's anything that tells me that Estella is cool with that then that is the fact.

When Estella was very ill Maria made her a very fanciful promise. She said that we were sorry that we had made her a body that was so little and beautiful but did not work. She said that we would go away and build her a little body that did work.

and we're not stupid and we're not deranged we know that this is a new baby and we know it's got its own personality and its own ways - we know that it's not Estella - but I tell you this - when she raises an eyebrow at my singing or smiles when I cry then maybe , just maybe the world will stop for a second and we can all remember our greatest human achievement - the capacity for wonder.

We are happy beyond words

For clarity though

The fight against SMA was never about us, or Estella

It was about the generations yet to come.

This baby has not got SMA - we got lucky

But as long as there are babies who are born with this horrendous disease I'm gonna keep telling people about it. I'm going to complete wish number two.

Wish number one - Maria will become a Mum again ( box almost ticked )
Wish number two - SMASH SMA - still working on it

Last thoughts

I'm about to go on a great adventure. At the end of the rainbow I become a Daddy again. Tomorrow morning though I am going to see Tinker and I am going to thank her

and I am going to take her a toy

and I am going to tell her that I love her more than anything in the whole of creation

Because I have to take care of her

Because she takes care of me

Fair





PS

There are a couple of people reading this that I want to say sorry to. One of the hardest parts of the last 9 weeks has not being able to tell them what was happening or ask their advice or seek their support. I hope they understand.

Thursday, July 19, 2012

SWEET TWEET FEAT is NEAT

This isn't a post as such it's just an explanation of something that is bothering me. recently I have posted a number of tweets that say something like - " 95 % of Twitter users do not retweet for @estellastar1 - but the 5 % who do are amazing."

This tweet has had the result of losing many followers and I have had a number of people saying that they don't like it.

I totally understand that.

But

A normal tweet has on average 30 people retweeting it and picks up about 6 new followers.

The tweet above has been retweeted 2,588 times and has picked up 1,844 new followers.

I know that is simplistic and that a quality follower is worth 500 temporary ones but my message on Twitter is to spread awareness about SMA - doing it this was means that the 6 tweets I have done this way have been seen by around 10,000 people.

It's not right maybe - but it's certainly not wrong

Hope that makes sense.

Major post coming soon


Thanks for reading

Tom

xxx

Sunday, July 1, 2012

CELEBRITY FORTUNES

This is a blog I have been thinking about writing for a while.

It's not a sad one - it's not particularly about Estella

Well, in a way it is.

One of the things I have noticed on Twitter is how annoyed some people get when they can not get celebrities retweeting them.It usually starts with tweets asking for retweets and then ends with an outburst about how celebrities could not care less.

Let's think about this a minute.

We seem to have this concept that celebrities are all rich and famous and should be conscious all the time that they are 'lucky' to be in the public eye - as such they should be tweeting about good causes all day long.

Why should they ?
How could they ?

They are all people and all very different people.

We have 26,000 followers on Twitter and a lot of that was down to the fact that one very nice American lady knew Stephen Fry and he tweeted about Estella. The time line went crazy and I remember being in tears as hundreds of people got to hear about Estella. It was amazing.

Stephen Fry himself said that seeing one tweet out of the thousands that he gets every day is like seeing a snowflake in a blizzard.

So there's the first answer - quite often the celebrity does not even see the tweets.

To get round this to start with we had snowstorm hours - calls to action to tweet a celeb all at the same time. I remember Mia Farrow doing us a retweet when we shut down her time line - effective but not the best way to win friends and influence people. I think she was rather cross :)

Anton Ferdinand came across us and retweeted and we picked up hundreds. One night he asked his brother ( the man who should still be England captain ) Rio to tweet and it went crazy. We received 2,000 new followers in the space of an hour. It was an amazing evening.


Just lately we have been lucky enough to meet with Natalie Cassidy. Natalie saw Estella's video because her neice rang her and told her she should watch it. The support we have had since is for one reason only - Natalie is a Mum , and an extrordinairily good one , she was touched by Estella and has taken SMA under her wing with regular tweets, a Sunday People article and more care and love than you could ever believe.

Maybe that's the answer. A lot of the celebrities who have supported us are Mums or ones who have had their own experiences in life that have taught them the value of support and thought.





We have had dozens of celebrity retweets about Estella. As soon as they see the video ( which has now been viewed by over 45,000 people ) they get it and they are so wonderful.


Last week we had several retweets from another actress. The difference this time is that she is actually a star in the adult film industry in America. I had two tweets asking if it was such a good idea to be retweeted by her. All I can say is that she wanted to spread the word about SMA and she was touched by Estella's story - so the answer is obvious. She's a very caring person and we are proud that she gets Estella - so thanks for so much support to the amazing Sophie Dee.





Last Sunday we also had terrific support in Direct Messages from the ever wonderful Gail Porter. She retweeted us and won us so many new followers.




So that's my thoughts this fine Sunday morning. No tears today. No calls to action just a thankyou to the celebrities who have been touched by Estella and her little life.

I'm biased - I know that Estella herself wins them over with her eyes and her spirit and her charm. If you are here reading this then you already know that.


There are a couple that we just have not been able to get Ed Sheeran and Jessie J must have had a million tweets but remember about the snowflake in the blizzard - one day a smile from Estella will land on their radar and BOOM - SMA will get another kicking.


I want to end with just one more thought.


I am sure a lot of celebrities don't like the word. They are very much normal people who are also touched by sorrow and pity and tears and regret.

By the same token there are so many of you following Estella who would never think of yourselves as celebrities. Never think of yourself as important or famous.


I tell you this


We are going to put an end to this disease that took my daughter. We are going to pick away at it. We are going to shout at it. We are going to kick it.

We are going to smash it


And when we do there will be no more babies born who already have their days numbered in months rather than decades

And when we do there are going to be no more Muppet Daddies struggling to read nanite stories through tear soaked eyes

And when we do there will be no more families who have to sit in that room holding the cold hand of their little treasure and kissing her forehead one final time before lifting and carrying her to her final sleep.


and when that happens every tweet, every retweet, every donation, every promise , every smile, every wish, every shout out , every Follow Friday , every tear will all be worth it. Small random acts of kindness

Like snowflakes

That make a blizzard

That make you all celebrities


Crying in the snow for one little snowflake who never saw her Winter


Let it snow
Let it snow
Let it snow


You didn't really think I was going to let you get away without a little sob did you :)




Sunday, June 17, 2012

FATHER'S DAY - A LETTER TO ESTELLA

Estella

Good Morning Tinker.

Muppet Daddy here.

Don't worry I'm not going to sing Puff the Magic Dragon, The Runaway Train or Twinkle Twinkle Little Star. I'm not here to tickle you and I won't pull any of those silly faces . I'm not even going to make you listen to The Cat in the Hat complete with overacting and silly voices.

I'm here for a purely selfish reason today.

I wanted to tell you how I'm doing.

I say selfish because I tell you how Mummy is doing every night - this morning though I thought I'd fill you in on how I'm feeling.

You know that I miss you more than I can ever say. I know I try not to cry because if I do and you are watching me then you may think that Daddy is sad or that he is lonely. I know youi do see me cry but sometimes they are happy tears because I remember something silly about you. Sometimes I cry because I miss you, you know that but sometimes I cry because some trees look lovely or I hear something about naughty SMA getting a kicking or I want to tell you about something that I can not.

There is not a day goes by that I don't think about how things could have been and I cry then too. That's selfish though I know. Your poor little body wasn't very well and I know you had started to realise that and that you would have become very upset if you had stayed much longer.

I know that.

Sometimes I cry because it was the right thing for you to go when you went. I know that's selfish too because the tears are just my way of saying that I wish things could have been different.I wish I could have held you for longer or sang you a few more songs. I wish I could have kissed that illness out of you and had it in me. God I wish I could.

The one thing that I never want to be different though is the effect that you had on this world. In your eight months here you managed to move so many people to care, to care about their lives, their children, their hopes- and to care about you.








So how am I doing

No point pretending is there - I know you know

When you first said goodbye I thought that I would never laugh or smile again. You soon showed me that was wrong. You sent me so many signs. That's why days like today aren't too bad. It's not been Christmas or New Year of Father's Day when I have missed you the most. It's at random unexpected moments. It happens a lot when I am driving or showering or waking up. If you see me crying then you have to forgive me please. It's just time without you.

It's just time passing by,

The nice people are still here. They are doing all sorts to try and make it that other babies don't have the naughty disease you had. When that happens then I know you will be very happy. Imagine that because of you living other babies don't have to have those tubes and machines and those rooms and those dreadful moments that we thought we were losing you.

Anyway enough about all that

It's Father's Day

I think there will be a bacon butty somewhere along the way. Better had be

but

It's Father's Day and you are supposed to get me a present.

There are 364 days a year when Daddies have the right to spoil their little girls and buy all sorts that they can be told off for but today is my day and I shall be expecting  my present later. I hope it's a big one

What am I hoping for ?

I don't want a tie
I don't want socks
I don't want a World's Ugliest Daddy Mug
I don't want a trophy
I don't want a card that you have made by painting with your fingers
( Although that would be very nice)
I don't want an electronic thingy machine


I just want a moment in the day - an unexpected moment. I just want a breeze across my cheek. I just want a rainbow in the kitchen. I just want a song on the radio. I just want to have one moment when a memory of you comes floating by. I just want a white butterfyl to land on a bush. I just want a memory of something you did.

I just want a heart rending, awe-inspiring , inexplicable moment when I remember something about you that I have already forgotten.

It may be something like the way you raised your eyebrows or squeezed my useless finger or smiled when I put you in the big bed and told you to hush so we did not wake Mummy. We talked for hours in teh big bed with Mr Giraffe- all our plans - all our dreams.

All I want is to know that you are there

All I want is to know that I have not lost you

All I want is sunshine on my face or a gentle rain or some natural beautiful moment that creeps up and suprises me and reminds me


Of you

Of my Estella

Now go and get that sorted.

I shall be expecting a miracle - not a diddy one - a big whap you in the belly with a fish miracle

Not much to ask for

Not much for a Muppet Daddy

On Father's Day



Monday, May 14, 2012

EVERYTHING I OWN



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

but

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.

Thankyou

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

Love

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

SERIOUS SMA SMASHING

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,
Tom


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.


https://edinburghuni.workwithus.org/Fundraising/Donate.aspx?page=7182

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.







Wednesday, April 18, 2012

ONE FINE DAY

By any stretch of the imagination an amazing day.

A TV company is paying for a memorial garden at Bluebell Wood. We met with the companytwice and they will be filming Maria in June.

Tom Gillingwater contacted me today from Edinburgh University . You may remember his team are working on an SMA cure. They have decided to introduce a scholarship at the University for a student. The scholorship is called the Estella Star SMA memorial scholorship and will allow a student to be trained to help find a solution to SMA. As you can imagine an over emotional Mupet Daddy found this idea to be more than wonderful. Weeping like a fool.


I thought nothing could beat that.

Then we got home and there was a letter from The Deputy Prime Minister.
He had sent a response last month from the secretary of state. I did not write about it at the time because it was not very positive. I wrote back with a few comments and asked for more.

Today it came

Are you ready for this ?

The Parliamentary Under Secretary for Public Health , Anne Milton MP has agreed that SMA will be considered for preconception and carrier screening by the UK National Screening Committee (UK NSC). They are going to look at the viability of screening for SMA - and here is the important bit - for the very first time ! They have never considered SMA screening before.

I have been asked to contact the Projects Manager who will be responsible for the consultation and reviews process. In other words there will be a public consultation before they decide whether to screen for SMA.

When I last held Estella's hand and looked into her eyes I promised the little Tinker that we would SMASH SMA for her.

Five months on the UK Government has asked for a detailed enquiry and consultation to decide if SMA should be screened for. 1 in 40 of you reading this article carry |SMA. It is the number one genetic killer of babies.

In the coming months I will need you.

I will need you to be part of the consultation and the review. I will need the strength and support and love that you have shown to our Tinker

because in the next 5 months

We are going to take awareness of this disease into the House of Commons
We are going to have a reception hosted by the Deputy Prime Minister
We are going to have research and consultation into genetic screening for SMA
We are going to have a major celebrity raising the profile of SMA nationally
We are going to have over 20,000 social network followers
We are going to fund SMA research at the University of Edinburgh
We are going to raise thousands of pounds for the Jennifer Trust

When I last held her hand I promised her that we would SMASH SMA. I never dreamed that so many of you would walk this path with us. But you have.

Tonight I am very happy

but

What I would rather be doing is bathing Estella and watching her splash and play.
What I woulod rather be doing is drying her with a bif towel and holding her close
What I would rather be doing is singing her a song and telling her a tale
What I would rather be doing is tucking her in and kissing her cheek

Nite Nite. Sleep Tight. Don't let the bed bugs bite.

And watching her breathe and sleep with stars shining through her window.

Muppet Daddy can't do that

I was her Dad for eight months. She gave me the happiest days of my life.

Now ?

I'm going to SMASH SMA into so many little brittle pieces.
I'm going to SMASH SMA until no babies have to kiss goodbye to their Mummy and Daddy
I'm going to SMAS SMA and then I'm going to sit by Tinker's Fountain and I'm going to sing her a song and tell her a tale and tell her that there will be babies that will laugh and breathe and run and laugh and love and live  .... because of her.


Because of her

I'm going to SMASH SMA - and you are going to scatter that disease to the winds and the rain and the rain and the skies.

They are going to consider screening for SMA
For the first time ever

Because of a little Tinker
Because of you

Thankyou

FOOTNOTE and on the same day Pfizer have announced that they are going to sellall of their baby care products to Nestle. An ideal opportunity to change the name of their SMA milk.

A garden at Bluebell Wood
A scholorship at a University in her name
A major drug company shamed into selling its interests in SMA milk

and

A government that heard every tweet , every message you wonderful people posted.

Not a bad day Tinker

Not a bad day




i love you

Thursday, April 5, 2012

THE BIGGEST WISH

The most common question we get asked when people hear Estella's tale is , " What can I do to help?" It's a wonderful reaction , it's a very human reaction and it leaves me genuinely humbled every time somebody says it.

What they do not realise is that simply asking the question is helping. It shows they care.

I know there was something very special about my daughter. I am biased but time and time again I see people moved and willing to do anything they can to tell her story. I know when that happens that the magic and the mystery that was Estella is still alive and I am so so grateful.

BUT

This blog is not about Muppet Daddy - it's about practicalities. People ask how they can help and so I wanted to write a  blog with genuine hard factual ways that they can contribute towards helping us. The only thing that I will say again is that I am so moved by the fact that people actually want to do anything . I take nothing for granted. I expect nothing. All you do for us , for SMA awareness , for Tinker is gold dust. Thankyou, with all my heart , Thankyou.

If you are here then I don't need to retell Estella's story. It is documented well enough elsewhere in the blog. I will just concentrate on things that can be done to make this world a better place.




OK. That's Estella. She is why we are here
What can you do to help ?


ENCOURAGE SOCIAL NETWORKING

We have over 16,000 followers on Twitter and 1,300 on Facebook. They are very powerful tools. I spend three hours every day keeping on top of things and trying to answer every message in person.
This is one of the main areas where you can help. Some people are there just to follow, some are there to support and some seem to work all night trying to spread the word. There will be different responses but overall you can help by

  • Retweeting when you find something interesting
  • Encouraging your followers to follow Estellastar1 on Twitter or Estella Meansstar on Facebook
  • Joining in with the silly photo competitions. They raise awareness
  • Trying to get a celebrity retweet. Stephen Fry brought 1,000 new followers and Rio Ferdinand added 2,500 in 3 hours. Think carefully about what you ask them to tweet. Will it grab attention ? Don't complain if they don't help. They get hundreds of requests but they are very kind and many do respond.
FOLLOW WORTHY CAUSES THAT HELPED ESTELLA

Every now and then I will ask that you follow another cause. Usually these are people that made Estella's little life better. Wonderful people like THE JENNIFER TRUST or BLUEBELL WOOD. Sometimes though I see a family out there struggling to raise awareness and I like to think that we can help them. Every time I do that I think of Estella smiling at helping a family or a baby.

CONTRIBUTE TO SMA RESEARCH FUNDING

We decided very early on that we were not going to set up a SMASHSMA charity. We are not rich but we are not poor and we don't want any money at all for anything. The promise that was made to Estella was that we would do everything we could do to do three things

1) Raise awareness of SMA so that people know that this disease is the number one genetic killer of babies
2) Help any family who came our way by telling them of our experiences and listening and being there
3) We would do everything we could do to genuinely get rid of this disease.

Number 3 is the one that confuses people. I think when we talk of smashing SMA some people see us as grieving parents who are trying to do the impossible. It's not impossible. There are many institutions with hundreds of thousands of pounds trying to come up with a cure. There are genuine people out there and I know they are coming up with major breakthroughs every day.

So

We suggest three different kinds of charitable donation

1) BLUEBELL WOOD - This is the hospice that gave Estella her childhood. Any donation to them makes us very happy indeed. That's a personal response.

2) JENNIFER TRUST - They are superb at helping parents with newly diagnosed children. They give practical and real advice and they make a daily difference.That's a response that helps parents of children with SMA

3) EDINBURGH UNIVERSITY - This is our big one. If the team at the University can get £100,000 I am convinced that they will make massive contributions to ending this disease. I wrote at length about them in the last blog - they are doing amazing things and they will make a difference to so many lives. Trust me please. I live and breathe SMA and research and developments . Tom Gillingwater and his team are the real deal. If you want to SMASHSMA give them a tenner. If you're a celeb reading this make a donation of a hundred grand and let's get rid of this disease !

Please donate by following the link below






SIGN THE PETITION



We have had terrific all party support for the raising of SMA awareness and there are going to be remarkable things happening in the coming months. We have met with The Deputy Prime Minister, Nick Clegg and he has remained in contact.

We need to have SMA discussed in Parliament - this will happen soon one way or another but we also have a petition to raise awareness of the need for screening for the disease. Even though we will have the matter discussed in the House of Commons it would still be good to have as much support as possible for this important petition. If you have not signed it already then please do so. Every person will count.







THINK OUTSIDE THE BOX



Don't be afraid of having a brilliant idea. We have had people writing SMASHSMA on their backs and taking photos, we have had people diving out of planes, we have had people writing SMASHSMA in the sand, we have had people painting portraits of Estella, we have had a star named after her, we have had school assemblies about SMA and people writing to their MP, we have had blogs, lit candles , made cakes and biscuits , painted SMASHSMA on their kids, written poems , - not only do all these things raise awareness they make Muppet Daddy and Cuddly Mummy so proud of the effect that their little Tinker is having on the world.

Do daft things - we love them !




THE BIGGEST WISH




What would I ask of you if I could only ask one thing though ?

What is more important than the money and the awareness and the retweets?


How can you really make a difference ?


This one is the simplest answer of all.


If you have children. If you have the amazing luck to have children that are there with you every night. If you have children in your life. If you are a Mummy or a Daddy or a Grandparent then do one thing


Cuddle them so very closely.
Read to them even when you are tired
Listen to them even when they don't seem to be making any sense.


I have had happy moments since Estella said goodbye. Some really happy moments. Those moments have all been when something happens to remind me of how she felt, and how she looked and how she smiled. I was amazed that I still had any capacity to laugh or joke or smile or taste happiness.

The fact that I can is only because I was so lucky. I was lucky enough to be a  Daddy for eight months, I was lucky enough to have some impossibly beautiful moments and I was lucky enough to see eyes looking at me with more love than I ever imagined existed.

I was lucky enough to love and to be loved by a little Tinker who has never left my heart, who has never left my soul.


So what I ask of you is very simple.


When you tuck your kids in at night. When you kiss them goodnight and walk wearily down the stairs to the heaven of tea and armchair - every now and then remember Estella and when you do remember that you are so blessed to be so loved by your children and you are so blessed that they will be there in the morning - causing a commotion.

And if enough people do that then she is still here.
If enough people do that then she remains real
If enough people do that then there is a star that will always burn bright.


This Easter - buy more eggs than they need.

and do one more thing

If you take them to the park and you watch them play and watch them run 

Do one more thing

When you've had enough and you want to go home

Do one more thing

It's the biggest thing of all. If you read on you have to do this ...

Look at your watch and give them an extra 30 minutes beyond the time when you think you've had enough Sit and watch them and just for that thirty minutes imagine what riches, what fortunes, what gold I would give to have 30 more minutes with my Tinker. 30 little minutes watching her play. 30 more minutes to tell her what she means - what she will always mean.

Imagine what that 30 minutes would mean to me

Enjoy what that 30 minutes become to you

And if you can do that I promise you - you will have the happiest half hour of your life.  






Go on , let them play a little longer and let them stay up to watch that programme that they want to watch. Cuddled up with their Mummy. Nestled into their Daddy


Have a lovely Easter

Thankyou for being there