A whole new world

Tuesday 01 September 2015

New School

In December 2012, when she was not yet 2 years old, we were told that our precious Little S had only 'weeks or months' to live.

Now, two years and eight months later, Little S, our amazing stubborn wonder, is about to start school.

Tomorrow morning she begins a new chapter of her life and ours.

A proper grown-up little girl at Immanuel Prep.

She'll have to navigate through her full school day both emotionally and physically - getting used to life in 'big school' and all of the new challenges that brings: playing, learning to read and write, making her own friends, finding out how the world works, and being independent and creative.

But also learning to cope with walking to and from the canteen, up and down the stairs to the music room, across the yard to the tennis courts and school fields, and doing PE.

Things that most 4 year olds would likely manage with ease.

For Little S they're likely to be quite a struggle.

She'll probably need her reserves of stamina and perseverance  to tackle some of these challenges but she'll also need to develop her self-awareness and talking skills to realise when she's done enough and needs help.

And although we're only a phone call away and we're there at the end of the day, she'll mostly have to do this herself.

Which is massive.

But I think she'll manage.

Maybe it will be tough to start with but I think she'll work it out.

She'll cope.

And I don't even think she'll be bothered that she's being treated a bit differently to the other kids because the school won't make big deal of it and because actually she'll probably quite enjoy being a bit 'special'!

New Year

A new era for Little S, and a new Jewish year with Rosh Hashanah less than two weeks away.

This year, as ever, there's a lot to think about and a lot to be grateful for but I think, inevitably, I'll be focussing on Little S.

Praying that she stays healthy over the next year and beyond

Praying that someone comes up with a plan to help her over the years ahead

Praying that science can find a way to fix her up once and for all

Praying that she and the rest of our family can continue to lead 'normal' lives: living, loving and learning together and figuring out how to become better people

Praying that the ever present black cloud on the horizon doesn't get any closer

Praying for magic

New Experiences

Talking of magic, Little S had a wonderful time over the summer: meeting and chatting with Snow White, waving as Anna and Elsa rode past, spinning around in Lightning McQueen's cars and the Mad Hatter's tea cups, and watching Mickey and Minnie and a herd of buffalo in the wonderful Wild West show - we went to Disneyland Paris!

Little S really enjoyed three full-on days and seemed to have boundless energy!

It helped a lot that, due to Little S's heart condition, we were granted a priority pass that allowed us to jump most of the queues! (as long Little S was with us on the ride!). It felt like we were on a first class visit to Disney - a truly magical place and a very special experience for Little S - one that she hopefully won't forget in a hurry.

Life is about making memorable memories, and whatever happens in the future we'll have Little S's smiles at Disney.

New Procedure

So, what's next?

Well, Little S was supposed to have a procedure last month - a catheterization to dilate or widen parts of her pulmonary arteries to enable more oxygen to flow between her heart and lungs.

It's a similar procedure to the one that she had last November when Dr D dilated her stent - the GoreTex tube that was surgically implemented in her heart to be the valve that she never had between her heart and lungs.

Last November we were led to believe that it was a pretty risky procedure but having done it once, the doctors are much more confident that Little S has the resilience to cope with a similar cath - they believe in Little S!

Having said that, she still needs to stay overnight as it's a general anaesthetic and they have to have an intensive care bed on stand-by just in case they need it.

So we were all prepared emotionally and logistically until GOSH phoned the day before to tell us that they had a spate of emergency operations scheduled and there were no beds available for Little S - the procedure was cancelled and moved back to mid October.

Which shouldn’t be a problem but we don’t really want to get cancelled again - Little S really should get on and have the procedure, especially with the added pressure she'll be facing once she gets to big school.

And that starts tomorrow!

87%!

Thursday 21 May 2015

We have a community nurse come over every couple of weeks to check Little S’s sats and make sure that she’s generally looking ok.

Last week Little S got 87%!

87%! I don’t remember her ever getting 87% before.

Most people would panic if their sats went below about 95% but during her bad spell in GOSH Little S went below 60% more than once, which was terrifying.

Before the procedure in November she was hovering at just over 70% and since the procedure has been more like 79/80%.

So 87% is amazing!

Now I don’t for a minute expect her to get 87% again any time soon but it is one of a number of indicators over the past couple of months that seem to show Little S is continuing to build up more resilience and strength rather than, as I had feared, getting weaker with every passing month.

For one thing we’ve now given back two of the three oxygen machines that we had in our lounge for the previous 28 months, along with two of the large oxygen canisters that were in our garage.

It’s taken a long time to get to this point – and it’s only partly because we were worried that Little S might still need them: the process of getting oxygen machines removed is very, very strict (or cumbersome, depending on your point of view).

I can understand why – we get the machines from a private company and they want to make sure that every possible liability is removed before they make any changes to the ‘prescribed’ set of equipment that they provide and service.

When you combine that with the fact that there doesn’t seem to be any standard way of writing an oxygen ‘prescription’ and it ends up being quite difficult to convince the company that their machines are better off with them than us.

The reason that we had three machines in the first place was that Little S was a baby when we got them and wouldn’t countenance putting on an oxygen mask so we had to treble the power of the oxygen and waft it around her room in the hope that that had some sort of effect.

But we’ve still got one machine just in case – the hope is that if she ever needs to she would use a cannula to gently blow oxygen into her nose.

Until then it will gather dust in our lounge and the kind folk from the oxygen company will come and check that it works every few months.

Just in case.

We’re also now only going to check-ups at GOSH every four months (plus occasional visits to top up with her meds).

Little S is also generally a bit more active.

That was certainly true over Pesach where she was staying up really late, singing away with everyone and bossing everyone around.

And at her fourth birthday party and J’s fortieth she was boogieing away on the dance floor, although admittedly at J’s party she was asleep before the real dancing got going.

Then at her nursery, she was in a dance show – dancing to For The First Time in Forever from Frozen and giving as good as any of the other kids there – I went along to watch and it astounded me to see her joining in: bending down, moving around – to see her behaving just like everyone else was wonderful - I was so proud and amazed and very close to tears.

Earlier this year we were asked by the local children’s hospice if we could remove Little S from their list – she’d been on their books for as long as we’d had the oxygen machines.

So we said yes – we hadn’t used any of their services (which don’t just revolve around end-of-life care but include respite for parents and siblings) and every time they called we would tell them that “she’s doing fine at the moment – let’s speak again in a couple of months”.

Little Miss Stubborn

So all of this sounds really positive – and it is – Little S is genuinely doing very well.

She’s growing up, developing into an beautiful and articulate and extremely stubborn little girl.

And thank god for her stubbornness!

Whenever she says ‘NO!’ or argues or shouts or screams I try and remember that it’s probably a personality manifestation of the same part of her that is fighting every minute, every hour and every day to stay well; to pump enough oxygen around herself to enable her to do everything that everyone else does, because why should she miss out? to refuse to give in; to never give up.

To me, the fact that being stubborn is so much part of her personality indicates that it’s also deeply ingrained in the way her physical self works and reacts.

I’m fairly sure that this stubborn streak is inherited – we’re blessed with a long line of strong women in our family: my sister, my Mum, my Nana and back further into the Vigons (that’s their family name, not the name an alien race that we’re all descended from!)

Stay stubborn Little S!

What next?

The next stage is going to be another procedure to further widen her pulmonary arteries and let more oxygen flow between her heart and lungs, which should improve her wellbeing further over the next year or two.

Given that she’s gone through a catheterisation once since she had heart failure, and it went relatively well, the risks should be lower to do it again as we know she is pretty resilient.

We’re currently waiting for a date for that procedure.

Alongside that I’m still waiting for Dr B in Petach Tivka, Israel to ask Dr R in Stanford, California (he’s the more friendly colleague of Dr H in Stanford, California that Dr B knows) for his thoughts on the results of the November cath. But Dr B is waiting for Dr D in London, England to provide him with some extra data without which he doesn’t think Dr B can really help. And I’m trying to chivvy them all along without appearing to be too much of a pain!

I forgot the good news!

Tuesday 20 January 2015

Not sure how I forgot to let everyone know but the catheterisation that we were so worried about back in November, over two months ago, went really well.

Dr D’s happy – the procedure went as well as he could have expected.

Little S is happy – she’s got a bit more breath.

We’re happy (although we weren’t happy on the day of the procedure – more about that in a bit) – Little S is fine and doing more stuff than she did before the procedure.

And I still believe in magic!

The day itself however was fraught – we were genuinely worried about what might happen, which wasn’t helped by at least two doctors going through all of the risks involved in all of their gory detail. 

Now I know that that’s part of their job nowadays in the litigious culture that we live in – I guess that there is also something about being transparent and ensuring that patients are properly informed in advance of any decision – 

but we’d already made the decision! 

and sat through all of the details on risks! 

and they went through all of this information with Little S sitting on the bed right next to them – not the sort of thing that you want a three year old to be hearing an hour before her cath.

Taking Little S down to the cath lab and handing her over to the doctors was about as horrible and traumatic as we’d expected, we'd done the same thing a couple of years ago and knew that it was not going to be pleasant.

The nurses did their best, getting Little S a bit drunk on liquid anaesthetic so that she was pretty dozy by the time we got down from the ward to the cath lab.

But no one had counted on Little S’s reserves of strength and sheer stubbornness – as the nurses tried to put on her mask to send her to sleep, she pushed through her stupor like a wild elephant trying desperately to escape from the poachers after being hit with three tranquiliser darts – eventually even Little S went down, but not before many long seconds where we were trying to reassure her that it will all be fine and yet wanting to tear her away and take her home to a happier place.

Two long hours passed spent wandering the streets of Bloomsbury and Russell Square and hanging in Pret trying to eat some lunch, trying to not ask each other the questions we didn’t want to answer ourselves.

As we were walking back to GOSH, the nurses called but rather than saying that everything was fine, they simply said that we had to go straight to the cath lab!

If the preceding two hours were long, the next ten minutes were interminable.

Hearts ... in ... mouths.

We ... went ... to ... the ... ward.

And ... then ... to ... the ... lab.

Where we were greeted by smiling nurses, a happy Dr D and, best of all, loud cries of anger and confusion (but not pain) from Little S.

She was fine!

Why the hospital didn’t tell us that as soon as they spoke to us, god only knows.

Such relief!

We spent the rest of the day on the ward and both J and Little S stayed overnight, just to keep an eye on her.

But she recovered quickly and by lunchtime the next day we were on our way home.

And the memories that Little S took with her were of playing with Lego and ponies and having a sleepover with mummy rather than strange lights, nasty doctors and painful surgery.

Amazingly, after a couple of days at home Little S was back to normal, in fact better than normal as her sats went up from around 73% before the procedure to around 80% afterwards - fantastic!

During the procedure, Dr D was able to get good imaging of her pulmonary arteries and to fit a stent which has widened the conduit that sits where her pulmonary valve should be from just under 5mm to over 7mm. It might not sound like a big deal but that doubles the surface area through which blood can flow between her heart and lungs.

Little S actually seems pinker now, and her nursery teachers say that she has more energy than before the procedure - she's certainly trying to climb stairs a lot more often - so it's all good really!

Dr D also saw that there are one or two other parts of her reconstructed pulmonary arteries that are a bit pinched and suggested that he might be able to widen them with a similar procedure, which could give her even more puff. He's since suggested that this might happen during the spring, in the next few months, and following that, she might be good for months or even a few years.

So the black cloud has receded somewhat and the horizon is clearer than it has been for a while.

Little S is happy - and I believe in magic! 

(and in the quite brilliant team at GOSH that stuck a tiny catheter tube into my daughter's heart, located the bit of Gore-Tex conduit that they put there two years ago to make up for the valve she didn't have, expanded a small metal mesh to widen the conduit and increase the blood flow between her heart and lungs and give her a bit more puff, videoed the whole procedure and got out again without any side effects - wow!)