There are no words

Shani

Named after the ever-loved and wonderfully single-minded Grandma Janice, Shoshana Jan (or Shani) was always stubborn.

Stubbornness runs deep in both sides of her female lineage and thank God that it does.

As most of you know, Shani had a congenital heart defect called pulmonary atresia, which meant that she was born with a large hole in her heart and without the valve that links the heart to the pulmonary artery that takes blood to the lungs to be oxygenated.

And so, when she was in vitro, incredibly she found a way to grow and connect two other arteries to her lungs to provide them with blood.

When Shani was a baby, she needed to have an operation to link up these two arteries and fix them to a tiny Gore Tex tube implanted in her heart where the valve should have been.

Having seemed to be making good progress, at 20 months old Shani suffered from heart failure and spent two weeks in hospital trying to recover.

We were told at that point that she wouldn’t make it, and yet over the next four years she grew into the beautiful little girl that we all know and love, full of spirit and tenacity, living and loving life and bringing joy to the people close to her.

She had a radiant smile that lit up every room, a gorgeous giggle and a cuddle that melted your heart.

Shani was never able to do quite as much as those around her but she didn’t seem to see this in any way as a disability. Instead it was more like a super power. She had a Magic Heart that enabled her to overcome impossible odds to make the world a brighter place whilst shrugging off injuries that would incapacitate the vast majority of people. In fact, one of her doctors recently told us that only a trained Sherpa carrying baggage up Mount Everest would cope with the low oxygen levels that Shani had throughout her life.

Shani was in many ways just like any other five year old girl. She would play for hours with her Barbies, her Disney princesses and her Lego Friends. She would trundle around inside on her little scooter and she loved being pushed on playground swings. She loved dancing and showing off her dress at Joel's barmitzvah. When I got home in the evening, if she wasn’t already playing in the bath, she would toddle into my arms with a big kiss and a cry of Daddy! And she loved very much to spend time with Joel and Tammy, with her wonderful grandparents, with her aunties and uncle and with her gorgeous cousins Maia and Lev.

She loved going to school at Immanuel Prep and had made some really close friends in her class that took good care of her: understanding when she had to stop playing and sit at the side of the playground and then sitting and chatting with her rather than leaving her ignored. I say that she loved going to school – she loved being in school, getting her to go into school sometimes ended in a screaming fit with Jules or I often walking away torn between admiration for her fighting spirit and concern that she was going to do herself a damage!

Last Summer, Shani took part in school Sports Day. She was in the pick-up-the-vegetables-as-you-run-along race, and though admittedly she was given a head start on the other girls, our wonderful girl managed to finish in the medals, in third place! Might be worth pointing out that there were only three runners in the race … but that’s not what matters – Shani gave it a go, ran the whole race, and was smiling about it all day, thrilled that she’d taken part and that everyone was so proud of her!

Despite all of this life, two weeks ago, Shani had to go into hospital for a necessary but difficult operation. She had been slowly deteriorating, her oxygenation and energy levels dropping and becoming visibly bluer. The operation would replace her tiny Gore Tex tube with a proper heart valve, which was risky, but it was also very uncertain how her weak heart would react to the ‘large insult’ it was given (as one of her nurses put it).

Many of you will know that there were big complications both during surgery and in the two weeks following. Initially we thought and believed that Shani was going to fight her way out of this one as well but it turned out that we were praying for one miracle too many.

Shani’s heart eventually gave up the fight and let her leave this world at peace, free of discomfort, pain and distress.

During that time, Shani was cared for in hospital by some incredible nurses and doctors, and at the same time we were immensely strengthened by the support we received from our wonderful friends, family and community.

On leaving Great Ormond Street yesterday for the final time, I saw a rainbow over the hospital. At the same time in Rehov Shani in the Israeli town of Modiin another rainbow shone in the sky. Perhaps a parting gift from our wonderful little girl, like a rainbow always with us but nowhere we can hold and kiss, a light shining in the heavens and a constant reminder of the daily miracles in our world.

Now I Am Six, by A A Milne, slightly amended. 

For Shani, aged five and three quarters

When I was one I had just begun

When I was two I was slightly blue

When I was three I was really me

When I was four I was so much more

When I was five I was most alive

Now I am (nearly) six I’m as clever as clever

So I think I’ll be (nearly) six for ever and ever

Three more sleeps

Sat 28 Jan 2017

Last month, during each of the eight evenings of the Jewish festival of Chanukah, we lit the celebratory candles and, as a family, sang the traditional song of Mo'atzur.

It's a song with a tune that sounds not unlike a Christmas Carol but each verse describes a different episode of Jewish history in which, as the saying goes, 'they tried to kill, we survived, let's eat'.

The final verse picks up the story of Chanukah and, referring to the part of the story where the Jewish people survived against the much more numerous Greek army and managed to keep the Temple Menorah alight for eight days instead of the expected one day, contains the line 'who performed miracles for his beloved' which translates from the Hebrew 'na'aseh nes lashoshanim'.

Given that Little S's name is Shoshana, for us these words take on a much more poignant reading, that God 'performs miracles for Shoshanas'.

Little S knows that her name is in the words of Mo'atzur and gets excited whenever we get to the final verse, smiling and dabbing as her name is sung out!

With the operation postponed from early December until this coming Tuesday, we were given eight opportunities that we would not otherwise have had to sing for miracles for Little S.

And it feels like we might need all of the miracles we can get - I'm scared.

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This evening I watched Avengers Age of Ultron with Little S's brother J - a movie about superheroes overcoming impossible odds to save themselves and the world whilst shrugging off injuries that would incapacitate or kill the vast majority of people.

Sound familiar?

Little S has been surviving and thriving against the odds for almost six years now - where Captain America has his vibranium shield and Thor has his hammer Mjolnir, Little S has her magic heart.

Well she's about to face a stern test over the coming days and weeks - I have hope and confidence that her courage, fortitude and sheer bloody mindedness will carry her through and that she and her magic heart will be stronger as a result.

But it's not going to be easy.

And even if everything goes as well as it possibly could, she not going to like it one bit.

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Assuming that the operation doesn't get cancelled, the days and weeks ahead are going to be tough for all of us, especially but not just for Little S.

As much as we could we've tried to just get on with life and be as normal as possible - it's always seemed to be a good strategy - it kept us and the kids on an even keel and stops you thinking too much about what could go wrong.

However, this week we're not going to be able to just carry on as normal, much as we might like to.

It's at times like this that we know that we'll be leaning heavily on our wonderful family and friends for support, both logistically and emotionally.

The logistics are important, of course they are, and the kids couldn't get on with their lives without everyone's help.

But the emotional support is incredible - to know that so many wonderful people are rooting for and praying for Little S provides a mental safety net and makes us remember that we are not on our own.

As Peter Gabriel and Kate Bush put it: 'when times get rough you can fall back on us - don't give up, don't give up'.

Emotional roller coaster

Tuesday 20 Dec 2016

This time last week Little S was scheduled to be in the Cardiac Intensive Care Unit (CICU), recovering from open heart surgery that would have provided her with a bigger pulmonary valve and given her more puff, more energy and make her less blue.

Today or tomorrow she would have been scheduled to leave hospital and come home for a 4 or 5 week recovery period.

Instead, on the morning of the day that we were due to go in, GOSH called off the operation because Little S had picked up a cold virus.

She often struggles with a cold at the best of times, so giving her the challenge of recovering from major surgery whilst feeling a bit under the weather? No, I don’t think so. That would not be a good idea.

GOSH knew that she had a virus because they’d tested some snot that, on the previous Friday, they’d sucked out of Little S’s nose!

On that Friday, we spent the best part of the day (did I say ‘best’?) convincing, conniving and cajoling Little S though various tests – bizarrely, she was quite content during the hour and twenty minutes that she spent having an ultrasound (probably due to the well-placed TV above her head showing CBeebies the whole time), the ECG wasn’t too bad, and even taking the bloods was no worse than usual, but she screamed through height, weight, SATs and mouth swabs like they were the start of the actual operation!

Apart from Little S being in the main brilliant for most of the day, I think we also owe a huge debt to the wretchedly addictive iPad app Colour Switch (I’ll make it past Level 4 next time!) for helping us pass the time. 

We would have made it home sooner but we were asked to wait for quite a while for Mr K, the surgeon that was going to do the operation, and the same surgeon that operated on Little S five years ago - he was in surgery for most of the day. 

We felt that it was important that we met him though. Despite the fact that he reminded us of the some of the big risks involved in the operation, there is a reassurance that comes with having met and spoken to the man that is going to hold your baby's life in his hands, especially as he also reminded us of the real need for the operation (but not so urgent that we shouldn't wait until Little S's virus passes) and the intimate knowledge that he has of the condition of Little S's heart and arteries.

Whilst we waited for Mr K, we (including Little S) were shown around the CICU - it's changed a lot since we saw it last five years ago: much newer, quieter, more spacious and more modern - a lot less frightening. We even told Little S that this is where she would be after her operation and she didn't seem too freaked out.  

She was also with us when we spoke to Mr K - not sure that that was our smartest move, although it didn't seem to have much effect - Little S just carried on with her Colour Switch moves and didn't take much notice of our conversation but I bet she picked up on at least the odd word and phrase.

Over the next couple of days, during which we weren't sure if the operation was going ahead or not Little S got very upset before going to bed a couple of times, crying that she didn't want to have the operation, although it made us feel better to some extent when we got her to explain why she was worried and the worst fear she had seemed to be that she wouldn't be able to sleep as they would keep waking her up during the night!

The upshot was that having had all of the emotional preparation for a major operation, and worrying big time about the possible things that could go wrong, and assuming that we would effectively have to cancel Christmas this year, we've ended up with our holidays back and we wait for the date of the rescheduled operation. 

Monday 26 December

It's both Boxing Day and the third night of Chanukah tonight and we're able to make sure that Little S has a wonderful holiday and to forget for a while about the operation. 

We've been told that it will be on 31 Jan with Little S going into hospital the night before. No need to do pre-ops again. 

This afternoon Little S asked, apropos of nothing, whether she will be able to do more stuff after her operation. 

We said that she'll probably still need to use her wheelchair but that she'll have more puff and should be able to walk or run a bit further, climb a few more stairs and feel a bit less tired. And it should prevent her ending up with less puff, which she might do if she doesn't have the op. 

It's a bit of a hard sell keeping her feeling positive about the operation whilst not worrying her too much about the consequences of not having it. 

Meanwhile she's having a lovely time in Brighton, and as long as she's smiling and happy, so are we. 

So whilst the sun is out and the skies are blue, Merry Xmas! Happy Chanukah! And a very happy and healthy new year to one and all!

The Chapel

Tuesday 27 September 2016

There have been times when the sun disappeared, when all we could see were black clouds, and when we hadn’t known if we’d see the day again.

There have been times when Little S could not be comforted or cuddled or sometimes even touched, and when there was nothing we could do to help her other than pray and try to stay strong.

In those times, she is in the hands of some amazing medical professionals – it is their skill and judgement, her stubbornness and fight, and the Divine decree that decides her fate.

And we just watch … and wait … and cry … and, if we have the strength, talk.

Talking is so important, and yet it can be so difficult. Because once you verbalize a thought you make it real.

Sometimes it’s the first step in helping you deal with it – it’s no longer a demon preying on you – you’ve given it form and shape and boundaries – and you’ve remembered that you’re not alone – and that is a source of such strength.

But it always feels like making it real is such a horrendous risk to take – maybe if I don’t talk about it, it will go away? What if I’m not strong enough to bear the words? Making it real might break me.

Sometimes it helps to be alone.

There is a room in Great Ormond Street Hospital that offers a place to do just that – it’s called The Chapel.

It’s quite a small room – a bit like a church, with biblical inscriptions on the walls and a statue of Jesus at the front, but unlike any church I’ve been in, it’s stuffed full of teddy bears!

And it’s a quiet room – one that encourages reflection and stillness – a place to gather oneself.

In the run up to Rosh Hashanah, the Jewish New Year, we are supposed to step back and think about our priorities in the world – what is it that is truly important and worthwhile to us – where should we be putting our energy over the coming year.

It’s a time to remember to seize the opportunities inherent in every day – to remember that most of our problems are not really problems, they are just noise, a distraction from focussing on the real meaning in our lives: our children, celebrating joyous occasions together, drinking in the wonder of the universe around us, making the world a better place.

It’s something that I often do not remember, even when I’m pushing Little S to synagogue in a wheelchair – we might be a bit late, she might have been a bit naughty, it might be raining – all too easy to forget.

But Dr D is giving us a big wake-up call – we received a letter from GOSH to say that Little S’s next major op is being scheduled and will probably be sometime in the next few months. When you open a letter like that it halts you in your tracks – the world carries on around you as you stare at the black typed words on the page or screen, feeling like the black cloud is rushing towards you out of the corner of your eye.

And Dr D is not the only one giving us a wake-up call – as with four years ago, we’ve been agog watching the Paralympians – champions over adversity, destroyers of limitation, superhumans – racing down the track or through the water with what we would normally think of as a disability but what they’ve turned into an opportunity - inspirational.

Little S knows that she has a ‘magic heart’. She knows that she needs a wheelchair to get around any distances more than about fifty yards or so. She doesn’t call this a disability. It’s just how life is.

The things that she does complain about are the same things as any 5 years might: shouting that she wants to watch one more episode of Spongebob Squarepants, screaming that she doesn’t want to go into to her new Year 1 class at school, refusing to brush her teeth.

Strangely, whilst I have to keep reminding myself to not complain about the little things, I’m quite happy to be reminded that Little S has the strength to kick up a fuss about something so trivial!

So the black cloud is looming but for the moment I have an awful lot of simcha and joy to look forward to: it’s the Jewish New Year, it’s Little S’s older brother’s Bar Mitzvah, it’s a time of many opportunities to celebrate with family and friends, all within the next month.

Black clouds are going to have to wait.

Shana Tova U’mtuka (have a happy and sweet new year)

Where there's a wheel there's a way

Until a few weeks ago, if we walked anywhere more then a hundred yards or so with Little S, we would need to push her in a child's buggy. Given that she's now over five years old it was getting to be a little bit silly - should a five year old really still be in a buggy?

Now she's much better off thanks to the NHS and their provision of a child's wheelchair.
It took about four or five months from applying to getting hold of the chair but they came through and, as with all of the amazing facilities that we make use of within the NHS, it is provided for free.

It lets her sit up and see more of the world around her and makes it a lot easier for us to talk to her as she's moving - it also means that people walking past realise that there is good reason that she is not walking - which I suspect will become increasingly important to us and her as she grows up.

Of course, being a five year old girl, we've decorated the chair in pink and purple ribbon - wouldn't you?!

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Little S has started talking to her heart, or more accurately, has received some messages from her heart.

She’s starting to become a lot more aware of her ‘magic heart’ and sometimes gets told by her heart that she shouldn’t walk any further or that she should slow down, or perhaps instead that she should keep playing on the climbing frame.

She's also mentioned that she sometimes has to ignore the messages from her heart as it's hard to concentrate at school sometimes if she doesn't!

She will still say sometimes that she’s ‘not got any breath’ or just ‘too tired’ but I think she’s starting to learn the difference between being tired in the way that everyone gets tired sometimes, and genuinely not having enough breath because her heart is struggling with the amount of oxygen that it gets.

I think this is part of her becoming more aware of her heart and the implications. And we’re certainly also talking to her more about how hearts work and what it is about her heart that is a little bit different to most!

Despite becoming more aware of her differences she doesn’t seem to be at all frightened at the prospect of having a heart that doesn’t work as well as other children’s hearts. We haven’t mentioned any of the potential dark clouds on her horizon and pointed out any of the downsides because it's too terrifying a prospect and doesn't feel like it would help her at all right now. 

I suspect though in time as she develops more awareness she might either start asking, or just worrying, about that sort of thing. So important to start having little ‘heart’ conversations now to ensure that we’re all able to talk about more difficult subjects should we need to.

In the main though, the last few months have been fairly steady in terms of her weekly sats check from the community nurse – Little S is normally about 76% which, to put in context, is a little above the danger zone of low 70’s / high 60’s in which, should she be there regularly, Dr D would want to get on with surgical plans.

Things are not always steady of course – during one week recently, the community nurse arrived during one of Little S’s screaming hissy fits!

Little S wanted something really important to her (like a biscuit or a TV programme) and we had decided that we weren’t giving in as she had been quite demanding and rude in the way that she had asked for it.

It’s always difficult to balance the desire as a parent to let your child just scream on the naughty step versus wanting them to be quiet and just let them have what they wanted -for us, we also have to include our fear that by letting her scream she’s going to cause herself to be sick or worse, as she puts additional stress on her heart.

She’s certainly made herself sick before and ended up pretty drained as a result.

This time we managed to calm her down, eventually, and the community nurse took it in her stride, but her sats we're not so good that week :(

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Sports Day this week and Little S wasn’t going to be left out!

She took part in the pick-up-the-vegetables-as-you-run-along race and she was given a head start on the other girls - our wonderful girl managed to finish in the medals, in third place!

Might be worth pointing out that there were only three runners in the race … but then again that’s not what matters here – Little S gave it a go, wasn’t going to be left out, actually run the whole race, and was smiling about it all day, thrilled that she’d taken part and that everyone was so proud of her!

Surprisingly high sats

Wednesday 24 February 2016

Little S had surprisingly high sats this week – 78% when the community nurse came over on Monday and 84% when Little S went to Barnet Hospital today. 

Although she is no stranger to hospitals, she wasn’t supposed to be in hospital today – this wasn’t a planned visit – she was only there because she fell off a space hopper at school, bashed her head and had a big lump appearing by the time J arrived to pick her up! 

However, once they reached the hospital, with big sis T in tow, Little S was feeling much better and quite enjoying the attention! And with sats of 84%, J wasn’t too concerned either. But the hospital staff were worried as Little S is a bit complicated and red-flagged on her notes as a child to be particularly careful about. 

So, after an hour of waiting for a consultant and with Little S clearly fine, J took the executive decision to discharge her herself and headed back home. 

Even her sats earlier in the week had no right to be as high as they were (note the relative use of the word ‘high’ and remember that most of us would be dead with sats of 78%) given that she was off school that day as she’d been sick and under the weather that morning.

Seeing sats in the high 70’s (and over 80 at hospital) was particularly encouraging, with the reading in Barnet Hospital corroborating the community nurse’s figure earlier in the week, as her sats had been stubbornly low (around 72%) for about three months now, and it had looked like the procedure that she had done in October was not having the effect that we’d hoped for.

Back in October her sats had increased to around 80% but only for the week or so following the catheterisation performed by Dr D that dilated her pseudo pulmonary arteries in four places in order to improve the flow of deoxygenated blood from her heart to her lungs and thus increase her overall oxygen levels – 

I say ‘pseudo’ pulmonary arteries because, as you may well remember, they were constructed during major surgery by combining the extra arteries that Little S had created in utero from her left subclavian artery (from her shoulder) and her right coronary artery (from her heart) before the amazing GOSH surgeons attached them to a small GoreTex tube that took the place of her missing pulmonary valve.

The cath procedure was a lot less risky than major heart surgery but still involved general anaesthetic and had the potential to cause a number of nasty complications if things didn’t go to plan. 

The good news was that it was a lot smoother from our point of view than the previous one in Nov ’14 – 

waiting on the day ward with familiar, friendly nurses that have got to know Little S over the years; 

better, calmer ‘bedside manner’ during the pre-brief which put us more at ease; 

a better anaesthetist, able to slowly, slowly send Little S to sleep without any plaintive cries for help; 

and, most importantly, a phone call of reassurance when the procedure was over letting us know that everything was fine and not (you’ll remember from the year before) creating panic, fear and uncertainty for the longest ten minutes until we saw our gorgeous bundle waking up with her teddy beside her and wishing mummy was there.

Like the previous year it only took an overnight stay for Little S to be ready to head for home – indeed, by the time I’d arrived back at GOSH the following morning, Little S was out of bed, and her and J were playing on the floor, packed and waiting to ‘check-out’: my Dad had come up to London to spend some time with them, bless him, and I suspect hadn’t banked on them leaving the hospital so shortly after he’s arrived! But it takes more than a general anaesthetic and 3 hours of someone fiddling with your arteries to stop Little S getting back home!

Since the cath, Dr D has been talking about a plan for the next stage which would involve more major surgery to replace the GoreTex tube with a proper valve that could be resized as required to grow with her over the next few years and potentially into adulthood. 

To have a plan like this now is amazing. 

Since December 2013 we’ve not been able to think for anything more than a few months ahead as Dr D had not been convinced of Little S’s resilience to invasive surgery. But with the success of the last two caths, he and the other surgeons at GOSH are prepared to take the next necessary step, although not until Little S’s sats show a further drop below 70%, which would indicate that she’d outgrown the GoreTex tube and needed something bigger to provide her body with sufficient oxygen.

We’ve also been asking Dr B’s opinion in Israel. He also thinks we should get on with the valve replacement, and sooner rather than later. But he has a disagreement with Dr D.

Dr D thinks that if and when we do the surgery we should not also close the VSD (the hole in  her heart) whereas Dr B thinks we should. I’d like to understand more about why they think different things before we get to the actual surgery – hopefully the two docs will discuss and come to a consensus on what the right way forward is: if they don’t then I’m not quite sure where that leaves us.

But the great thing is that there is a way forward for Little S, one fraught with great risk, but a way forward nonetheless – hope, again.

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!)

Do you believe in magic?

Sunday 09 November 2014

And if you were to ask me
After all that we've been through
Still believe in magic?
Yes, I do
Of course I do

Coldplay, Magic 2014

Nearly twenty three months ago Little S went into hospital undergoing heart failure.

During those dark times we didn't think she was going to make it to her second birthday, and yet through a combination of medication and sheer stubbornness, she is not only surviving but thriving. Magic? It's certainly a kind of magic!

A couple of weeks ago my son J and I went to Israel for a short holiday to see family and friends and Jerusalem and also to meet up with Dr B who I've been in touch with since the dark times and who gave us our first and probably only really useful second opinion so far. 

He chatted to me and J for about 25 minutes and then decided he would take us on a tour of the hospital, the Schneider Institute. We saw the incredible facilities there and even met a tiny baby with pulmonary stenosis (narrow pulmonary valve) for whom Dr B was going to do a catheterisation (one hour later) to widen her valve - a similar procedure to what Little S is having on Monday.

He told us that Little S's heart failure wasn't great but it wasn't too bad either and that:

- the cath coming up was really important both as an opportunity to expand her conduit and pulmonary arteries and also as a way of obtaining lots of information about how well or otherwise her arteries have been growing and therefore what opportunities there might be for follow up procedures and surgery.

- he would forward the scan from Monday's catheterisation to the world's best pediatric cardiology surgeons, in Stanford California, (Drs H and R) for their opinion on what might be done next

- he thought there may be a couple of options going forward, although even doing nothing may well be viable for a while

- one possibility is further heart surgery to partially close the hole in her heart with a patch (which means, amazingly, that they would be able to then go in at a later date with a catheterization and fully close the hole - it's like a zip!) and to replace her conduit with a homograph which acts like a proper heart valve. 

- the other even riskier option is to start considering a heart-lung transplant.

So hope, but hope reached by a long road full of ifs and buts, risks and dangers.

............

And now it's the day before the catheterisation.

Fear and apprehension fill my thoughts.

It's the right thing to do, of course it is, but the risks are there and they're not negligible.

Prayers have been said, friends and family have offered support.

It will soon be time to leave it up to Dr D's expert hands, Little S's strength of will and whatever help we can get from up above.

Believe in magic? Of course I do

Summer's gone

Sunday 14 September 2014

Summer's gone, day's spent with the grass and sun 
...
Wake up it's a beautiful morning,
The sun shining for your eyes.
Wake up it's so beautiful,
For what could be the very last time.
The Boo Radleys – Wake Up Boo

It's been a glorious Summer - 
lazy days, holidays, rock pools, 
sunshine, cycling, swimming, playing, 
on the beach, in the pool, 
seaside, countryside, 
laughs, giraffes, 
friends, family, walking, talking ... fishing! 
and wishing it would never end - but it does.

Little S has been amazing throughout the summer - 
loving being outdoors, swimming lots and generally having a great time!

She's laughed, scooted, danced and grown - 
she's a big girl now, going into the 'rising fours', the oldest kids in the nursery.

But this is a time of year that is always a time of transition - 
a new school year begins, 
the holidays come to an end and wonderful experiences become memories, 
the Jewish new year approaches with its focus on reflection on the past year and resolutions for the year ahead, 
work somehow becomes a bit more serious as everyone tries to knuckle down and get things done, 
the evening skies start to darken and the weather becomes more inclement. 
It all feels like the fun and colour of summer is changing to darker autumn days.

It certainly feels that way with Little S.

After months of being able to not worry too much about her condition and just loving spending time together, we now have to turn our mind to the prospect of a catherisation procedure in the next month or two - needed to improve the flow of oxygen to her heart whilst she is still in a generally healthy state.

Normally a catheter would be a low risk job - 
keyhole incision,
insert some dye,take some detailed pictures,
insert a balloon, inflate an artery or a conduit added during previous surgery,

but with Little S the stakes are higher - 
her heart's not doing so well,
so if things don't go according to plan,
she might have a hard time recovering.

But it needs to happen - 
without it her sats are going to get worse,
whilst with it they may get a bit better for a while
and could possibly put her in a place where she could undergo further, much needed, surgery.

So darker days - 
but Autumn is not Winter

In Autumn, greens turn to reds and golds
In Autumn, nature builds up her strength to prepare for long Winter months
In Autumn, leaves fall so that trees can bloom again

Monday 15 September

A visit to GOSH: sats 72%, cath Oct 14

We're going to need to talk to Little S about what's going to happen to her.

Over the Summer she asked me:
'Did you have meds when you were three?'

I replied:
'Sometimes, and I take meds nowadays as well'

Because all three year olds take meds - otherwise, why wouldn't older brother J and older sister T be also taking meds every day?

That's bound to be a much easier question to answer than something like: 'What's going to happen to me today?'