What is a guardian angel?

Out there there are people working from dawn to dust, they dont know us, not our children, nor the times we had to fight to get the oxygen flowing to their lungs, nor the times we cried secretly with anger, feeling defeated because we were fed up of going to a war we had no chance to win...
They still go to work everyday, like that elfs of the shoemaker's tale, winning battles while we sleep, keeping us in bussiness with the hope of a cure for our kids.
After a lot of hard work they did amazing discoveries, like how SMA affects the muscles, and why our babies got worse from the 2nd month of life. Why many babies are misdiagnosed and die before their time as nobody heard of the disease, and the most important of all, they have transformed the most severe form of the disease to type 2 in mice which now have passed from living  1 week only to several months, that translated to humans is from living months to years with a much better quality of life, giving more quality time to families, and time to find the cure also. They are offering us hope in a better future
This people are becoming my guardian angel, the ones i pray and trust, not with my daughter's life, i can't do that anymore, but with the lives of the ones to come, with the future, with a victory in this war that has taken so many lives.
How much a dream cost?
£100,000 a year, only to keep the lab working. Is not much if we think about the expenses that NHS has to pay for SMA collateral damages, is nothing compared to the heartbreak of our families. They struggle to find the money, they end covered of grant forms to fill in to keep on going, they sell their wings to heartless farmaceutical companies just not to kill the dream, our dream, of a childhood for Estella, Alfie, Theo, Maddy.... And so many others, too many, like poppies in remembrance day.
Why for once dont we help the guardian angel to help us? why can't us help Proffessor Gillingwater's team? by doing so we will be empowering ourselves, we will give the babies that left and the ones that are still fighting a second chance

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

THE ART OF MAKING THE PERFECT SOUFFLE

As many of you know Estella’s birthday was last Friday, she would have been one, but you already know that too. We got thousands on messages for the Tink, some people even baked cakes and blew candles and let balloon fly free in the February night, for all that demonstrations, thank you so much.

We promised Estella a few things, Smash Sma is one of them, and although still we are far from see this wish coming true we get a bit closer everyday. Another thing her dad and myself promised was keeping on living, maybe this is the hardest, keeping on the faith and the smile no matter what, but a promise is a promise.

When Estella was here I saw life as a rollercoaster, you never knew, you lived for today, if she had a good day you took it with both hands making it the best, making memories for the bad days to come. There was no future nor past only NOW. You lived for the day, you smelt her hair and tried to remember every note of her sweet fragrance. Every second precious and intense, today was what mattered…

Then she died, and from that day life became a Marathon. It didn’t matter how fast you run, what mattered was to keep in the race, to think of the future as a goal. Today didn’t matter and you only wished the days past fast to that day where you life began again. We promised her to keep on living, and to make her live again in a certain way by giving her a sibling, at least part of the genetic inheritance that made her would make new life… make it to that first appointment when all possibilities start again with the hope on a better future.

This transition is hard, life trained us for the moment and now we have to learn to be patient and hang on for tomorrow.

Last Thursday, at last, we went to our first appointment with the Geneticists… making a baby in those premises is like having the recipe for the perfect soufflé. If you like cooking as I do you know is maybe the most difficult recipe because it can go ploff at any moment, so the recipe to bake a healthy baby… it goes like this:

First let the mum not be too fat, not too slim, not a smoker, not a drinker, not too old. Then they switch off her ovaries to a chemical menopause so to be able to control her periods, then they will over stimulate the hen, pardon the mum to give as many eggs as reasonable, at this point too much or too little is bad. They will extract them latter by using a syringe with a very long scary needle.

Then take the dad to a quiet place with a specimen bottle and a copy of penthouse magazine to do his business. Once you have the basic ingredients they will make the embryos ant Guys in London. At this point only negative or carriers of SMA embryos can be used and only if they are healthy enough to survive the trip back to the womb. If we get any embryo (sometimes they don’t have any left and they have to start from scrap) mum and dad will go back to Guys and the embryo will be implanted, again with the scary long needle and a little bit of tender loving anaesthetics. A few weeks later, pregnancy test, if positive scan at two weeks, if all ok appointment with GP and all the ups and downs of a natural pregnancy… and then concentrate on a positive outcome ( here comes my mantra: baby in the crib, baby in the crib, baby in the crib)

With this procedure we have some less than 30% of a baby in the crib… I know that it doesn’t sound much, but is still much more that my chances of baking the perfect soufflé or being a size 8 or giving up chocolate for good.

Thank you from this humble blog to that clever people at Children’s, Jessops and Guys that can make the miracle happen, I’m already wishing on my own personal star.

We are still running the marathon… Let’s the Games begging.

HOW DID IT HAPPEN?

This week very soon, we have two very important meetings, one with SMA milk and the other with Mr. Nick Clegg, and I still wondered, how did it happen?

I started blogging when Estella was diagnosed, as a therapy sometimes, as a denial of her condition others. When Estella was diagnosed (it seems so far away now)  we had a plan, she was going to be a type II whose life expectancy is considerably longer and in the meantime a cure would be found and we would live happily everafter. That is what the first part of the blog was about.
Then one afternoon in June Estella went into respiratory arrest and I had to call 999. It was so sudden than when it happened I was Skyping with my dad who was in Spain, he was the one who told me to call the paramedics, because he is a doctor and he could see how his only grandchild was getting worse in a question of seconds. I describe that day as the worst of my life and the beggining of the "journey".
We made it through hospital somehow, she was a fighter, and although her body was getting weaker she had such a will to live... from that period I just wrote one blog to thank staff at the Children's for giving us more time with her.
I didn't know then but we barely had 3 months left, the last two we had to spend at Bluebell because Estella was too fragile. There was when we really started the SMASH SMA campaing. 10 days before she died Tom started writing in twiter and I followed in facebook, the day before she died Estephen Fry retwited. When Estella passed away in my arms I remember telling her "Twinklebell, the clapping is for you" because Tom's phone wouldn't stop ringing, one bell, one follower...
And then, you know the rest, more than 12,000 followers in both social networks, and everytime we get a new follower our Tink smiles, I know she does.
So how did it happen? I took for granted that getting meetings with multinationals and the Deputy Prime Minister was easy... aparently is not only difficult but almost impossible. So how did it happen?
 I think that even when Estella has very stubborn parents who won't give up because they don't have nothing to lose (just the smile and nobody can't take that from us), and the Tink's eyes are magical and make people melt and help her and feel her as part of their own family (we have been fostered by many of you). I think that all this happened because of you, the more than 12,000 people and counting that want to kick SMA where I'm not allowed to write. All this Mayhem, all this hope, all this opportunities to change things that we seem to keep on having is thanks to you all

Thank you for making it happen x

RYANAIR, MUFFINS AND SQUIRRELS

We are flying over the North of Spain now, heading towards Manchester, the steward won’t keep on speaking about some raffle tickets. No need to say we are flying with Ryanair, I could say for the same matter I’m flying on a coach, because that is what low cost is, a bus with wings. No wrong with that, it allows me to visit the family as often as I have holidays so even when is annoying and I can’t listen to music with the noise of the raffle is not so bad if I look at the prize.
Funny enough I’m listening to Muse “New Born”, that is how how I feel more or less .
I gave myself time, time to live with Estella’s condition, time to come to terms and enjoy what we had left, time to let her go with dignity and time to grief afterwards.
Today is 8 weeks since she died, I had gone through days not wanting to go out of bed and others in which I needed to control everything because otherwise the world would follow apart over our heads. I also cried a lot and I laughed, because not all has been despair, there have been good moments too when Tom and I got amazed at people’s generosity and we achieved signatures for SMASH SMA, money for JTSMA and Bluebell Wood, and awareness of this not so rare disease so more families can ask for the tests. We also shared some very nice times with the family and at our own by the fountain telling Estella what is going on and leaving helium balloons in the free air.
Anyway, since Estella was diagnose till now life has been on hold and now I feel I run out of excuses to stay still. I feel I keep on looking at the world as an spectator but not really taking  part of it, as If this bus was too fast, or better said, as if I kept missing the bus just because I don’t feel like getting in it.
I’m running out of excuses not to live, and that is not fair on our daughter because she didn’t have the chance of experiencing life.
So the rest of my life starts as soon as this plane lands, I own it to her and to me. Somebody told me that, even when there are situations we can’t control, most of the time people make their own luck, well I’m going to make good luck for me and my own as If I were making muffins (Tom laughs every time I say muffin because I pronounce it /muh’fin/ also when I say squirrel /skuihrel/). By the way my muffins are quite good because I put lots of chocolate and nice calories in them… same recipe for life.

First of all is the IVF project, baking a nice baby for Tom and me so we can eat him/her with lots of kisses. Estella will be the responsible of this new SMA free healthy life and this new life will give us happiness to us as well as our family, this baby will be a new hope (like in Starwars… gosh that is geeky) and will have his/her own guardian angel looking after him/her… I bet Estella can make a very good eldest sister (she was always very bossy, as me and I’m the eldest too).
Also we are going to get those 100,000 signatures and convince SMA milk to help us with the campaign. We are going to make more noise than this Ryanair steward (he’s leaving me deaf… but I may buy a ticket for the raffle after all).
In a smaller scale I’m going to lose a stone, I’m going to start using all the wii games I have to make exercise (I have them all, zumba, wii fit, wii fit plus, my personal trainer…), I’m going to be the positive me that started working at Learning Disabilities a few years ago and I‘m going to stop saying why me and change it for a why not.
Above all I’m going to be happy again. 2012 starts as soon as we land…my diet maybe a few days later J

Whatever it takes to be happy

I have to trap this feeling before is gone, a feeling of redemption and hope bigger than me, bigger than life itself.
It's half past one in the afternoon. We are by the fountain, Estella's ashes still there, and floating in it's waters, the little toy Tom and I bought for her .
I am wondering how different today would have been if we had her, 3 Wise Men morning.
I sing Cabaret for her when a White dove approaches listening from a nearby branch.
Suddenly I see a gorgeous little boy, no more than 2 years old with his Mum running after a ball. The ball gets in the fountain and he reaches for it, and laughs and plays and I know he is playing with Estella.
Just before leaving he stops on his tiny feet for a second to look back at the fountain, he nods with a serious expression, is he saying goodbye to her?
Right after  an Irish Setter (exactly as the one I had as a child) comes and runs to the fountain, he is playing with Estella too...
and the birds sing, and the sky is blue and the air is warm and my Daughter is whispering in my ear, "Don't be sad, don't you see how happy I am?"

 Miracles are around the corner if you listen and look with your heart. Hand made miracles to suit your needs. Everyday stuff for some, signs for others. As my Dad told me once, whatever it takes to be happy.

MOVE LIKE JAGGER

First time I listen to this since Estella died, curiously starts saying
Just shoot for the stars
If it feels right

 If you like maroon five and it’s awesome singer you know already I mean Jagger. While I’m writing this I’m crying, writing, singing, laughing and trying to be quiet for Tom’s sake because he gets very emotional every time he listens to anything it meant a moment with Estella, and we have plenty as we both, better said, we three love music and we sang and danced anything and everything to her, from Puff the magic dragon to cabaret to jose merce… you name it.
You say I'm a kid
My ego is big　
Estella always since the day she was born, knew very well what she liked, for instance she never liked Michael Jackson, not even the motown period. She would frown in despise. She loved lady Gaga and Beyonce though.

Since she was diagnosed we spent an awful time with physiotherapy, mainly my mum and me… she would sing funny songs she made up on the go and that Estella found amusing sometimes annoying others. I would use the chart as I always said I had a teenager trapped in a baby’s body.

Physio as all the mums with a child with a neuromuscular disease know, is crucial, it helps coordination and allows abilities to be lost at a slower pace, also helps bonding and something I discovered makes the child and the mum feel “normal”.

I treasure every moment I “danced” with Estella, I used to move her arms and legs for her while I sang to her. Not long ago I visited some good friends at Bluebell Wood who still remember my off tune version of “All the single ladies”… God bless their souls and their ears. It was moments of pure happiness, my baby wasn’t going anywhere then, there was no masks, vents, ng tubes. Only Estella and crazy mummy laughing their heads off.

Estella had a rare complication of SMA, bradicardia, that is her heart rate could go downhill very fast, but when we danced she always was great, oxygen 100 % heart rate 140... Perfect healthy baby. Music is a powerful thing.

When she died, last thing I sang to her was Cabaret… would it be inappropriate to say she was the happiest corpse I have ever seen?. Happy, happy little tinker surrounded by love, laughter and songs.
Maybe it's hard
When you feel like you're broken and scarred
Nothing feels right
But when you're with me
I'll make you believe
That I've got the key
Now is me who needs healing and feel normal, and for the first time I am laughting and dancing with Jagger, she is with me smiling at my impersonation of Mick, blowing kisses in the air so she can catch them.
You wanna know how to make me smile
Dance like nobody is watching, sing like there are no neighbours, live like there is something amazing around the corner, because there is always something. And when is over just be like Elsie in Cabaret, like a Queen. We are the salt and the earth. WE ARE THE MOTHERS OF THE ANGELS, OF THE TINKERS and SMA cant take that away from us x

We’ve got the moves like Jagger