40 and the miracle of 8

Saturday 30 November 2013

Life began yesterday.

What a great couple of days – who knew that turning 40 could be so much fun!

Little S has been on form throughout – I spent the morning of my birthday watching her Chanukah show at nursery where, surprise, surprise, as part of ‘8 doughnuts in the baker’s shop’ she toddled across the room to take a doughnut from her teacher right on cue.

Then lunch with her and J, followed by a bike ride in the afternoon and a l’chaim of whiskey and doughnuts at the synagogue on Friday evening.

And, on Saturday night, a cocktail bar (and barman) in the lounge, an 80’s playlist that took me right back to student days and a house packed full of wonderful friends and family!

Little S was so excited!

Although she was asleep by the time the party kicked off, and she missed the sushi and more doughnuts, she was dancing around the lounge whilst we were putting up decorations and grinning from ear to ear – gorgeous!

But of course it’s not just my birthday, it’s also Chanukah – 8 days of stories, lights, hope and miracles.

Rabbi Jonathan Sacks teaches that one of the miracles of Chanukah – that time in history where the Jewish people fought and won against the mighty Greek empire in order to retain their freedom and way of life; when the oil for the menorah that was left in the desecrated Temple in Jerusalem, which was only enough for one day, lasted for the eight days it took to replenish the oil from the olive trees of the Galilee – one of the miracles was that, in the depths of despair, the Jewish people did not lose hope. Not then, and not in the thousands of years since, to the extent that Hatikvah (the hope) is the national anthem of Israel.

As Little S’s nursery song goes One little candle, one little candle, one little candle burning bright!

Sometimes one candle can be enough.

Na’aseh Nes La’Shoshanim.

65 and 74

Monday 11 November 2013

Little S was feeling a bit under the weather, suffering from a bad cold for the previous week or so, more lethargic than usual and not as smiley as normal, so we had a feeling that when we went to GOSH her sats might be a bit down and she might not quite be her usual sparkly self.

Well her sats were definitely down, down to 65% which isn’t good even for Little S.

Dr D was concerned.

He began discussing the shunt connecting her heart to her reconstructed pulmonary artery - highlighting the possibility that it may no longer be delivering enough oxygen.

If so, they may need to use a balloon to expand the artery as part of a catheterisation procedure.

He was fairly sure that this would need to happen at some point but was hoping, I think, that it might not be for a little while yet as any procedure carries risks, especially whilst undergoing heart failure.

We weren’t convinced.

He seemed to think that her sats had been in decline for a few months but we were pretty sure that they’d been stable at about 70% since the spring.

So we decided to get a community nurse to visit on a weekly basis to take Little S’s sats and check whether they would recover once she gets over her bug (J also though that they may have not measured sats properly at GOSH this time).

Little S also had to have a blood test which she wasn’t best pleased about although the problem was much more about putting on the anesthetic cream and plaster than actually sticking a needle in and taking the blood! The nurses ended up using cling film instead of a plaster which seemed to do the trick.

Before the community nurse turned up we thought we’d get hold of our own sats monitor so that we could check ourselves.

So I ordered one from Amazon – seemed to have good reviews and be suitable for toddlers but when we tried it out, although it worked fine for J and me it went haywire when trying to measure Little S!

Thankfully the community nurse had better luck and a week after going to GOSH, Little S had recorded 73%.

She’d pulled it off again – another recovery.

And next week she got 74%, her best score since leaving hospital last New Year’s Eve!

The black cloud had scuttled away again, settling back onto the horizon.

Bend not break

Tuesday 29 October 2013

Today J took Little S to see the physiotherapist – we’ve been concerned that she’s not able to climb stairs or walk for that long (Little S rather than J!) and wanted to check whether this was just due to her heart condition or whether there was anything else involved that we might be able to help her with.

Well, it turns out that Little S also has hypermobility – joints that stretch farther than normal, sometimes known as being 'double jointed'.

It’s not necessarily a problem but can mean that joints can be a bit too bendy and so muscles need to be a bit stronger to get joints to work properly. Can potentially also cause problems in child birth – but that’s a long way away for Little S to worry about!

Hypermobility is found in about 5% of the population, but that percentage seems to be much higher for ballet dancers and in my family – for instance, my dad goes to yoga and I can ‘walk through my hands’: hold my hands and then take them 360 degrees around myself without letting go. Oh, and my sister has also been diagnosed as hypermobile, so there probably is some connection there.

The upshot is that Little S has some exercises to add into her daily regime – I say exercises but they’re really just games she needs to play every day – like standing up to put something in a box that’s just out of reach or throwing a ball or swimming or doing more walking up and down stairs – all of which she thinks is great fun!

The good thing is that all this means that it’s not just her heart that’s holding her back and that we can do some things to help her improve her mobility.

In other news, the community nurses called to ask if we wanted to be discharged as they hadn’t spoken to us in six months – we thought that a better question would be to ask if we wanted them to come and see us! But actually it’s probably better being ‘on their books’ and not discharged just in case we need to get in touch quickly.

That’s all for now - keep smiling!

Succot – how fragile we are

24 September 2013

We’re in the middle of the Jewish festival of Succot when we build a succah (a booth or hut) in our gardens that we eat (and sometimes even sleep) in to remember the forty years of journeying of the children of Israel in the desert following their Exodus from Egypt.

But the succah is not just a historical commemoration, recreating the dwelling places of the Israelites. The story goes that throughout those many years in the wilderness, the children of Israel were protected by divine clouds of glory, shielding them from extremes of heat and cold and warding off their enemies.

And so the succah is a way of connecting that idea to our lives today – somehow being able to have faith in the face of insecurity (as R. Jonathan Sacks puts it), living life to the full in an uncertain and dangerous world.

It’s supposed to make us focus on the important things in life – not the gizmos and gadgets that fill our houses but the family and friends that fill our homes: we step out of our houses, reflect on how lucky we really are and express joy and gratitude for the good things we have.

For me, Succot encapsulates everything about Little S and her future – faith and fragility, wonder and worry, all rolled together.

We even sing the Hoshana prayer – a cry for a divine rescue from the perils of real life – and I can’t help but add Little S in as we sing: Hoshana Shoshana, protect her, save her.

And the strange thing is, at the moment she doesn’t even know.

Chag Sameach

Growing up fast

Sunday 25 August 2013

Having talked about it over and over whilst we were on holiday, within a couple of hours of getting home, Little S’s bed was fixed up and that evening she was no longer sleeping in her cot – instead she was in her bed like a big girl!

And two days later, I’ve taken down her cot – so there’s no way back now :)

And in two and a half weeks, Little S starts nursery – now that’s a big big step … for us anyway.

She’ll be fine but we’ll be on shpilkers (it’s Yiddish – I think) – what if she falls over? Or gets pushed by another child? Or goes blue? Or gets breathless and needs oxygen?

Well, <takes a deep breath> she’ll be fine … won’t she?

I know all Jewish parents have these worries when they let their little darlings into the big wide world (ok, it’s only nursery but you know what I mean), and we certainly did with big brother J and big sister T, but this feels different. No. This IS different.

But it’s also the same – Little S WILL be fine, unless something happens, as with all kids. It’s just that the something that happens might be bad. And so, if something happens, they’ll call us and we’ll deal with it.

What else are we going to do?

A wonderful week

Friday 23 August 2013

We’ve been staying in the Cotswolds for the past week, in a gorgeous nature reserve, just outside of Cirencester.

Our cottage looks directly onto the quiet of an English lake, a view from the balcony of ducks, trees and water – of serenity, sunsets and swans.

We’ve had a great time – swimming in the spa swimming pools, playing tennis, cycling, visiting Bourton-on-the-Water and a Roman Villa.

And Little S has loved it – couldn’t get enough of the water, of walking around, of freedom, of spending time with the rest of the family, of playing, of smiling.

We took oxygen with – a couple of canisters – but didn’t use it all .

And it’s all felt so normal :)

I like hopistal

Monday 12 August 2013

This time we were at GOSH with both Little S and her big sister T.

Little S was great, almost enjoying herself – not even complaining about the ECG and the Echo that she has to ensure – there was no blood test so that probably put her in a better mood!

And having T around was also fun, especially since the waiting area has a Wii with Mario Kart on it!

Little S weighed in at 12 kg and managed to come up with 74% for her sats, higher than most previous visits.

We were seen by a different consultant to normal – hopefully another sign that Dr D sees that there is not much to worry about at the moment – although Dr D did pop in to say hello and to let us know that the previous blood tests yielded a BNP of 13,000, much lower than the 30,000 that Little S recorded at the start of the year. Dr D doesn’t get particularly excited about BNP levels but it was another small chink of light, another glimmer of hope.

And they’ve upped one of the medicines now (Captopril) from 0.5 ml slowly up to 1.0 ml, basically because Little S is growing up and getting bigger!

So we come back in three months now – daring to hope in the meantime that Little S stays as healthy and happy as she currently is.

Petrified

Friday 9 August

At first, I was afraid, I was petrified / Kept thinking, I could never live without you by my side

Whilst on holiday in Brighton, we decided to go swimming in the local swimming pool - a not uncommon activity for us on our visits to Brighton, but one that we hadn't done since Little S had been in hospital in December. In fact, earlier in the year we had been told that swimming wasn't a great idea for her at all as she may not be able to cope with the cold water, or the colder air especially when getting out of the pool.

More recently we were told that we could give it a go, but as we entered the pool with a very excited Little S, I was petrified - really worried that any second she was going to go blue or get breathless or collapse or all of the above.

Of course, being our little miracle, she was fine - in fact she was a lot more fine than I was!

Saturday 17 August

Buoyed by this experience, we tried again when on holiday in the Cotswolds - in an indoor swimming pool, an outdoor swimming pool and even at an inland beach (the largest in the country apparently).

And each time, although I was worried stiff (maybe a bit less after the third or fourth time), Little S loved it, and wanted to get on with swimming on her own, without armbands!

So it seems, unlike her older two siblings who hated going into the water at her age, that Little S is a bit of a water baby - maybe I should be a bit less worried and a bit more amazed!

A scare in the morning

Wed 17 July 2013

So I was in Luton airport preparing to board a plane to Edinburgh for an overnight business trip.

J calls to say that Little S was looking rough and lethargic and droopy and not waking up properly – what should we do?

We gave it ten minutes to see if Little S looked any better, by which time I was at the flight gate!

With no change in Little S's condition, I turned around and J took Little S to Barnet Hospital (via the kids’ schools) – she sounded pretty similar to how she was just before December’s nightmare of two weeks in GOSH and the terrible news that went with it.

Figuring out how to get back through security was fun but I managed (along with about 15 people who had actually missed their flights!) and made it back home, whilst J was waiting to be seen in Barnet.

And it turned out that Little S had given us a bit of a scare but that she was actually fine – probably just a bit exhausted from the heat (isn’t it really hot?!). Must admit that we were pretty worried that evening as we thought it might happen again, and possibly worse this time, but we gave Little S a more powerful fan and she slept in just a nappy – that seemed to do the trick and she was in good form the next day!

Bizarrely, the same thing then happened to our car – suffering big time in the heat, took it to the garage, they checked it over and said it was fine and it’s been OK since!

Take care of your little ones in the heat – the moral of my story today!

Every silver lining has a cloud

Wed 10 June

No change – Little S continues to amaze and be amazed, to look pretty good on the outside and, day by wonderful day, to carry on turning into a gorgeous toddler.

But every so often there’s a thought at the back of my mind:
 – What happens next?
 - What if ...?
 - How would I cope?
 - Don’t let it happen.

It’s like a clear blue sky with a worrying black storm cloud hovering on the horizon, stubbornly refusing to go away or even to shrink a little bit.

And it’s not even there unless you look in a particular direction.

You almost need to remember to forget about it in order not to look.

And that's not easy to do, so, every so often, it ends up trickling back into your mind and the worries begin again.

And maybe that’s no bad thing – to worry occasionally - as long as that cloud stays on the horizon and doesn't get any closer.

 

Na’aseh Nes L’Shoshanim

It’s been a while but we’re back again

Mon 17 June 2013

A visit with Little S to GOSH – we hadn’t been for 7 weeks and this was our first visit since last October to the ‘standard’ clinic rather than the day patients ward.

Well, as part of all the building improvements going on in the hospital since we were last there, the clinic had moved to where the old day ward was and so there was plenty of space and toys and tellies (for the obligatory beebies!) – a much better experience all round.

When we arrived, Little S came out with ‘love hopital’, so that felt like a good omen and, in the weeks before, she’d been started saying ‘Dr D pleased’ when she takes her meds!

She had all her tests: height (85 cm), weight (11.6 kg), blood pressure (68 / 40), sats (72%), blood test and echo and was great throughout.

Her weight puts her just below the 50^th percentile for her age, which is better than we thought she might be – room for improvement but at least she seems to be getting a fair amount of the calories that she needs.

Her sats were taken by Dr D after Little S had toddled across the ward from his office to the sats machine – normally we’d expect them to be a bit low after some ‘exercise’ but 72% is the highest I’ve seen her for a while (Dr D got 95% and I then bested him with 96%!)

For the blood test we were initially told to go to the next door hospital which would have been a right pain and probably taken hours. Then Dr D suggested that we pop down to Walrus (the day ward), phoning to check that they weren’t too busy. So we wandered down and found two of the nurses J and R waiting for us with open arms and massive smiles, well waiting for Little S really: they were so excited to see her again!

Little S got the red carpet treatment with nurses fussing over her and we were so pleased that we hadn’t gone elsewhere for the blood test!

We had a good chat with Dr D – he’s very pleased with how Little S is doing in terms of her outward demeanour, behaviour and mobility.

He talked about her being able to fly at some point (probably next year now - in a plane - she doesn't actually have super powers) which he seemed to rule out before – apparently there’s a room in GOSH where they can test a child’s reaction to aircraft cabin pressures for an hour or two.

He also suggested that we probably didn’t really need to take oxygen machines and large oxygen tanks with us on holiday over the summer.

So lots of good stuff.

But Little S’s heart seems to be just as weak as it was back in January – all of her improvements are symptomatic and, presumably, down to her medication, her miraculous tenacity and the positive, normal atmosphere that we encourage around her.

Dr D used her echo scan pictures to show us the part of her heart wall that is affected by heart failure – whilst it should be moving almost a third of the way across the heart chamber and back as part of each heartbeat, Little S’s was still not really moving much at all.

So the good news is the Little S looks like she’s doing really well and is in really good spirits and on course to actually start nursery in September! Who would have ever thought that that was possible – she’s already been along for a couple of hours to check out the toys and seemed to have had a good fun time. The teachers seemed pretty clued in to her needs so we’ll give it a go.

The bad news is that there is no change inside. Dr D also felt that there was no point doing a speculative procedure, like an angiogram, to look in more detail at her coronary arteries (they might be the cause of all her troubles – and world expert, Dr H at Stanford seemed to think that an investigation might yield some useful information) as she’s doing fine right now and a procedure could put all of that at risk.

 

So it’s just try and keep up the good work until our next appointment in August.

Expert opinions - finally!

Wednesday 24 April

 

We've finally, after what feels like moths of waiting, had responses from Dr Frank Hanley at Stamford in the USA (world expert in pulmonary atresia) and from Dr William Brawn at Birmingham Children's Hospital in the UK (top semi-retired pediatric cardiological surgeon in the UK). Both said that there are probably things that can be done to improve her pulmonary atresia but only if and when she is no longer suffering from heart failure.

Dr Hanley suggested trying an angiogram of her coronary arteries to try to determine if problems within those arteries were the cause of the heart failure, in the hope that it may be something that is reversible. It's not clear what he might be hoping to find and Dr D was not too keen to subject Shoshana to an angiogram for something that is probably quite speculative, at least for the next few months anyway.

So I think we've now got the opinions of the all the experts in the UK and most of the top experts globally.

 

And our summary of their collective opinions is something like: keep going with the medication, make sure she's eating well, live life as normally as possible, celebrate and enjoy that she's doing so well, and pray - because things could still get a lot worse.

 

A good day at the hospital

Wednesday 24 April

Dr D was pretty happy with Little S today.

We had to wait quite a while to see him and when we did it was all a bit rushed as the free parking voucher that we got from GOSH had almost run out so I had to go and get a new one and missed most of what he told J by the time I got back.

And he got on a bit of a mission when we asked him about whether Little S should have a chicken pox vaccine, so that we didn't have to find a friendly pediatric A&E doctor next time Little S plays with a child with pox.

Another doctor just happened to be passing that had got hold of a pox vaccination for his daughter and so knew what to do - and Dr D pretty much ran off to write a letter, by hand, to ask our GP and local pharmacy to prescribe, dispense and deliver the vaccine.

We've not quite figured out yet how to do that but that's another story.

But Dr D was really pleased with Little S. In fact we subsequently got a letter from him in which he said that 'it was fantastic to see' Little S and that 'she is really doing very nicely'.

And, he doesn't want to see her for another six weeks!

 

Brighton (Hove actually!)

Friday 19 April

We'd been in Brighton (well, Hove actually) for a week and it had been a really lovely few days.

It was the first time that we'd stayed in the new family flat - right next door to my Mum, so right next door that they share the same front door and we could open up both flats and let the kids just wander from one to the other.

And Dr D had signed off a request to our oxygen company (not really our company, it's the company that supplies us with oxygen, well with extra oxygen anyway) to supply oxygen canisters to our flat in Hove - so this meant that we didn't need to install three oxygen machines in Hove in the same way as we have at home, and this is because we don't really use the oxygen machines at home, well only when everyone is asleep and even then we're pretty sure that Little S doesn't actually need them. But it's precautionary, it's risk mitigation and no one is brave or stupid enough to stop turning them on.

Anyway, lo and behold, we arrived in Hove and there were a set of large oxygen canisters left outside the flat! Just on the path outside the front door. I say large - each metal canister was about as big as bar stool.

Little S had a great time in Hove - as did we all (although I was at work in Leatherhead for a few days but even then it was great to come home to the seaside in the evening!).

On our first day in Hove we got a message from one of J's cousins who we had seen the day before to say that her little girl had developed chicken pox - in case we were worried about Little S - nice of them to tell us. We were a bit nonchalant about the whole thing (we really should have learned by now) until another cousin of J got in touch to recommend the exact medication that they thought we should be giving Little S to protect her from chicken pox!

So we got in touch with Dr D and, just before Shabbat, we got an email from him to say that yes we really should get hold of the medication, from our GP. However, our GP was a two hour drive away and closing from the weekend. On the off-chance, we got in touch with the pediatric accident & emergency department at Sussex County Hospital in Brighton and a wonderful doctor on the other end of the phone told me that they had the exact medication right there and that I could pick it up straight away. And within an hour of getting Dr D's email, we had the medication and I had a renewed respect for the NHS, and the fact that mountains can be moved if you really need them to be.

Two

Friday 5 April 2013

Four months ago it didn't seem possible, but Little S turned two today!

Of course we made a fuss of her in the way that all parents do when their baby has their second birthday: we showered her with presents, we put up happy birthday banners and balloons, we helped her open her cards from friends and family, we made sure that she was having lots of fun and getting loads of attention.

But there was something else going on with Little S's second birthday, something that made it feel like we had something to celebrate beyond a family birthday - a milestone reached that we didn't think we'd get to just a months before, a celebration that perhaps might never have happened.

And so, 
After the presents, 
And after the wonderful party we held two days later in which so many of Little S's friends came to have fun and make our house buzz with joy and sunshine, 
And after we'd all sung children's songs together with a sing-along mummy and a guitar-playing daddy, 
And after Little S blew out the candles on her birthday cake (handmade as a giraffe looking like the number two) with a blow that looked as good as any toddler's should be, 
And after Little S had said goodbye to our wonderful friends as they headed home, 
The family stayed behind (to help clear up, to have a cup of tea or just because we lived there!) and, with a lump in my throat and a tear in my eye I opened a bottle of champagne and proposed a toast: 
To our little miracle girl, who's made it back from the brink and given us such great and wondrous cause for celebration and who we love so very, very much.

And yet, the funny thing is, that although the toast was poignant and memorable and moving, it was the weekend of celebration that preceded it that was so much more important.

Because as much as we might stop to think about how far Little S has come and how miraculous she is (which we should),
And as much as we might keep praying for her recovery from heart failure and for her to not suffer any further deterioration (which we must),
The thing we really, really have to do is to celebrate every single day with her, to live life to the full, to drink in every moment and to not miss a thing.
Because for all of us, when it comes down to it, living and loving life together, with the people that you truly love, is the only thing that really matters.

Na'aseh Nes L'Shoshanim 

Why is this night different?

Monday 25 March
First night of Passover: Seder night

Why is this night different from all other nights?

Every year at the Passover Seder table we ask this question.

And every year we respond that Seder night is different because we eat matza and bitter herbs, we recline as we drink our wine, and we dip parsley into salt water, all to remember the Exodus of the Children of Israel from slavery in Egypt over 3,000 years ago.

This year we had a extra answer - this was the first time that the family had gathered together to celebrate a holiday since Little S began her personal Exodus from hospitalization during the dark days of December.

We know that she has far to go before she reaches anything like the promised land, but it took the Children of Israel 40 years to get from Egypt to the land of milk and honey with many trials and tribulations along the way - but they made it.

Jewish mysticism claims that the first night of Passover is endowed with an intense spirituality and strength - it is called 'leil shimorim', the night of guardianship, the night that the Children of Israel were guarded as the angel of death struck down the Egyptian firstborn with the tenth plague thus triggering the Exodus, a night that we today can call on to guard us from ills and from danger.

Now I don't know how much I can count on the claims of Jewish mysticism but, being both a pragmatist and a romantic, both a scientist and a spiritually observant Jew, I'd like to think that our prayers on Seder night have provided Little S with some form of extra protection over the coming months and years from whatever it is that she will inevitably have to deal with.

Thursday 28 March
Third day of Passover

So we went back to GOSH again this week and, whether or not this was anything to do with Little S's leil shomorim, we did receive some positive news.

Dr F was there and he was pleased with how Little S was looking.

He said that they have been measuring her Brain Natriuretic Peptide (BNP) as part of her blood tests. BNP (which consists of 32 amino acids) levels increase markedly with left ventricular dysfunction and with the severity of heart failure symptoms.

Sometime in January, Little S had a BNP level of 30,000 (not sure of the units here).
Now she has improved and is down to 22,000, which is the positive news, but she has to make it to just 600, so there is a long way to go!

But progress is progress - cautious progress as Dr F put it.

And not long now until Little S's birthday!

Na'aseh Nes L'Shoshanim ...

A week of downs and ups

Worries in Barnet (9 Mar)

Last Saturday we were worried.

Little S had picked up another kiddie bug a couple of days before and her symptoms were getting similar to how she was in December - very lethargic, not walking, smiling, playing or eating. She didn't have swollen feet or hands but she was far from herself.

And we weren't going to wait until she did this time.

So Saturday morning we were off to Barnet Hospital and within 1.5 hours we'd seen the nurse, the doctor, had a chest x-ray (to figure out if she had a chest infection) and were just waiting for the registrar to take a look and decide whether to prescribe anti-biotics.

So J headed off for lunch at some friends (her sister had already taken the kids there) and I was left to munch on a chicken leg and a challah roll - she'd then come back to pick me up once we were done.

Five hours later we were still waiting!

The silver lining of Little S being so lethargic was that I could just sit and sit and read my book! But it wasn't exactly how I'd intended to spend Shabbat.

On J's return I went in search of the registrar and talked my way back into the pediatric A&E (we'd been moved to a ward hours before). I convinced them to find the registrar and then explained that we were leaving in 30 minutes as Little S had to get home for her evening heart meds. To be fair, she apologised - a whole load of emergency cases had turned up during the afternoon and she was the only pediatric registrar in the entire hospital! So, although she would much rather have taken the time to find and examine Little S, she ended up just writing a prescription there and then and the A&E nurse dispensed the anti-biotics from the cupboard next to her!

Better in GOSH (14 Mar)

After a couple of days of anti-biotics, Little S had started to improve. 

By Thursday we were back in GOSH and she was pretty much back to 'normal' again. (Annoying to have to put normal in quotes but then that was why we were back in GOSH wasn't it?).

They were really pleased to see her again on Walrus ward. Dr D didn't even recognize her as she had had her first haircut since our last visit!

But he was very happy with how she'd been doing - he increased her beta-blocker to the full dose and reduced both of the diaretics to once a day instead of twice.

We mentioned that we're intending to head down to Brighton for a few days during the Easter holidays and he said that he didn't think that we need to arrange for oxygen machines whilst we're there (none of us think that they're really doing anything for her) - just taking a couple of emergency cannisters of oxygen should be enough. Brilliant!

Oh, and it's Little S's second birthday in under three weeks - now that's something to celebrate!

Na'aseh Nes L'Shoshanim ...

A good heart these days is hard to find ... in Japan

So it appears that a big reason that Dr S has been doing his pioneering operations to combat heart failure using heart stem cells is because there are so few heart transplants performed in Japan.

The first heart transplant in Japan for 30 years was performed in February 1999 following a change in the law to allow organs to be donated on being declared brain-dead rather than only once the heart had stopped as was the case previously.
http://news.bbc.co.uk/1/hi/health/287880.stm

However, further legislation was necessary in 2009 to make transplants more widely available as, during the previous year, only 11 heart transplants had been performed in Japan (compared to 2,000 in the USA) and about 400 Japanese would die each year as they were unable to get a heart transplant.
http://www.bloomberg.com/apps/news?pid=newsarchive&sid=ajgBoFtYJFKI

A lot of drugs, but they're working

Wednesday 6 Feb

Little S started on another drug - this one is a beta-blocker and the last of the bundle of medicines normally given treat heart failure - it's the one we've been building up to but probably won't have much effect until the dosage increased.

First time though and Little S had no bad side effects, so we were allowed to go home again!

Before we left, we talked to Dr D a bit more about Japanese Dr S. Dr D feels that this pioneering work will eventually become routine and will replace a lot of heart transplants, but that's not going to happen for at least 5 - 10 years. The procedure involves taking some stem cells from a healthy part of the heart and introducing them to the failing part of the heart - and 6, 9, 12 months later Dr S finds significant improvements in heart function from the half dozen kids that he has treated so far.

So we're going to ask Dr S for his opinion about Little S - her condition may mean that she is not suitable or that he is not allowed to treat her for ethical or legal reasons - maybe she has to be Japanese! And anyway, even if she was suitable and allowed there are so many hurdles, not least that we would have to get her to Japan when Dr D has told us that long-haul flying is not a good idea at the moment!

But there is no harm in asking ;-)

And then there is the question of whether we would want the operation done anyway given the risks involved in major surgery - it obviously depends on the risks in not doing it. And on whether there are any other alternatives: at the moment it's just the drugs. But the drugs are working.

So how do you weigh up drugs versus high-risk potentially life saving procedure? You dodge the question and take the pragmatic route for the moment of just trying to find out more.
Thursday 14 Feb

It was Dr F today as Dr D was away.

This turned out to be a good thing - Dr F provides an extra perspective that feels a bit more positive than Dr D is sometimes. Maybe it's because Dr F wasn't the one that had to look doom in the face and give us the worst possible prognosis back in December. In any case he seems pleased with Little S - he hadn't seen her for a few weeks and so noticed a big improvement.

He also let us know that he has seen a number of infants and small children suffer from heart failure (although not necessarily with Little S's extra complications as well), have the same course of medication as Little S and, over two or three years, actually recover heart function - little kids are so amazing that they can even get their heart working again, given a bit of medicinal help (no, not whiskey!).

Thursday 21 Feb

J went without me today - a good sign, both because it means that we have more confidence in Little S going to hospital without needing the comfort of both her parents there (admittedly this was a simple check-up rather any blood tests or scans) and it also means that J felt more confident about getting there and back again in the car!

Thursday 28 Feb

Little S has had a cold for the past few days.

Given that it was probably a winter bug virus that brought her down and triggered all of the problems she had in December, getting another bug has been a bit of worry over the past month or two.

But then when it happens, you just have to deal with it - and it turns out that there hasn't been a great deal to deal with - it's just a cold, like any other toddler's cold.

So to me this is big news - she seems to have developed enough resilience to fight a cold bug without becoming overly lethargic or starting to lose further heart function.

Little S versus a cold - the poor bug never stood a chance!

Anyway, saw Dr D again today and Little S had both a blood test and an echo scan.

Dr D was pleased - the echo scan showed no deterioration in her heart.
He also said that there has been no improvement in heart function - we took that as a positive comment too - it implies that there must at least be the possibility for improvement!

And he's given us next week off - two whole weeks without hospital - hooray!

In the mean time, we wait to hear from Dr B in Birmingham (UK's top pediatric cardiological surgeon), Dr F in Stanford (world expert on pulmonary atresia) or Dr S in Japan (ground breaking stem cell procedures).

Two months home

Two months ago Little S had just arrived home: one hour before the start of January 2013.

At that point, we didn't know if we'd all make it to March, and we couldn't see past the next day.

Now we are thinking about planning Little S's second birthday at the start of April, and even what we should think about doing over the Summer.

And Little S continues to make good progress - she's now moving around like a toddler should: smiling, laughing, playing and saying 'No!'.

We know that it might change at any time, but for the moment, the medication combined with her stubborn refusal to let anything beat her seems to be doing the trick.

Toddling Again! or Three Thursdays Third

Thursday 24 January

Another echo and some more blood tests and a chance to have a think about whether the new drug was working and, if so, whether to increase the dosage.

During the previous week, Little S has been doing rather well - a bit of optimism and progress amidst all of the doom and darkness - on a few occasions she was actually toddling around our house (or someone else's house) - not climbing stairs (or anything really), but then she never did, but certainly back to how she was around the beginning of December, before the worrying symptoms began that led to her being admitted to hospital.

And Dr D seemed to think she was making further progress as well - maybe this new drug is having an effect?!

He has told us to increase the dose a little bit - and hopefully over the next few weeks we'll be able to increase it again, as the more we can give her without side effects the better the effect should be on her heart function.

However, we need to temper all of this hope and happiness with a little bit of realism - we are still in a situation where Little S's heart is failing and no one knows why; it is currently stable but potentially could get worse again, either because of the unknown underlying condition or if she picks up a virus or a bug.

Having said that, we're still waiting for Birmingham and Stamford to get back to us and Dr D mentioned today that he and Dr B (remember him - he's the one from Israel) were both at the same conference this week (in Miami!) where they heard a talk by a Dr S from Japan describing an experimental technique that, although involving major surgery and perhaps not directly applicable to Little S's condition, could significantly increase the heart function of a child with heart failure. I'll know more when we get sent some of the details from Dr B.

So for now we have to get on with trying to be normal - living with Little S on a knife edge, managing a heart poised at DEFCON 2, sidestepping the 'how are you?' question from work mates that don't know, and seizing the day but only as long as we've got our oxygen with us.

Most importantly, enjoying the smiles and laughter of Little S and those around her.

Na'aseh Nes l'Shoshanim

Shabbat Shalom.

A New Drug, or Three Thursdays Second

Thursday 17 January

We were back - this time to try out a new medicine for Little S, an ACE inhibitor that was supposed to relax her arteries and allow blood to flow around her body more easily.

However, they had to check that her blood pressure didn't fall too much, which can happen with this sort of drug.

So we were in for most of the day, with a lot of the time spent checking that the new drug didn't have any bad side effects - and it seemed to be fine, which means that Little S is now on five different medicines every day, plus we also have a whole cocktail of super drugs to be used in emergencies in case anything goes wrong.

Little S also had to undergo the trial that is the blood test - for some reason the nurse looking after her that day refused to believe that she would find it difficult to get blood (even though we reminded her that Little S was notoriously tricky) and it was only after two attempts failed - very uncomfortable, loud and tearful for Little S - that she agreed to call in the doctor that has been so successful on the previous occasion: Dr J.

Needless to say, Dr J saved the day.

Meanwhile, we let Dr D know that we'd sent Little S's echo and angio images to Boston Children's Hospital and that a Dr T would hopefully be in contact soon with their opinion. And we were also trying to get the same images to the world renowned Dr H at Stamford -this was proving to be a bit trickier. We also talked about a famous surgeon in Birmingham, Dr W, and Dr D agreed to ask him as well (although he said that he'd be surprised if he said anything different to GOSH).

But you've got to try every avenue - never give up.

At Least They Haven't Given Up, or Three Thursdays First

Thursday 10 January

Back in GOSH for the first time since Little S was discharged on New Year's Eve.

We had been set up with a special appointment with Dr D rather than having to sit in the normal clinic for hours.

So we went straight into an echo scan, supervised both by Dr D and by Dr F - a specialist in pediatric heart and lung transplants - and so they were both able to see the scan as it was taking place (rather than having to wait for the results) and to chat to us about various possibilities for the next few weeks.

It was a very calm atmosphere, helped by Little S being brilliant - watching 'beebies' and letting everyone just get on with their work around her.

Dr F was there to give us a better insight into why the transplant option had been ruled out by GOSH - and by Newcastle Freeman hospital, the other main centre for pediatric heart transplants - and why it was also so unlikely to work at any other hospital.

Because we weren't now just relying on GOSH - we'd started to contact other experts and centres of excellence, initially in the UK but increasingly around the world - anyone that might be able to provide us with hope for Little S. 

Before we left on New Year's Eve we'd managed to get a copy of Little S's echo and angiogram images on CD from GOSH. Then, with the help of some wonderful friends and family, we sent the disks via Hove, Luton, Ben Gurion Airport, Modiin and Zichron Yaacov to Dr B in Israel. Dr B was rather amazing and just a few hours after the disks had been delivered he had analyzed and discussed the images and called me directly to talk about Little S!

He suggested that there may be a couple of medicines that could help Little S to better manage her heart failure - and he also sent that information to GOSH.

So when we talked to Dr D and Dr F they knew that we'd been busy trying to find some hope - and although a transplant increasingly looked like a lost cause (they mentioned that GOSH hadn't even done a pediatric heart-lung transplant in the last two or three years, and that a heart transplant wouldn't work as Little S's body was used to the blood pressure it receives from a heart with a hole in it - maybe a heart transplant might work if we could find a heart with a hole in!) they now appeared to have not given up either, agreeing with Dr B's suggestion of medication, and alluding to possible 'other options' if Little S got stronger.

And, although they didn't want us to get our hopes up, they were more than willing to help us to get opinions from other experts around the world.

On the previous occasion, we'd left GOSH with the feeling that they'd done all they could and that was it - this time there was definately a different feeling: we were still on the watchlist but at least they hadn't given up.

Thank you so much

All the stars are coming out tonight, they're lighting up the sky tonight, for you, for you.
Take That

Don't give up, 'cos you have friends.
Peter Gabriel

The last few weeks have been really, really difficult.
But without the incredible support that we have received from our wonderful friends and family, they would have been nigh on impossible.

We've received so many messages of love, prayers and strength and it's really helped us to bear the strain - we know we're not alone in this.

And because of the crazy way that communication works these days we've been getting messages from FaceBook, text, Wordfeud, What's App, voice mail, email (work and personal), to both me and Jules - a virtual stream of love coming at us from everywhere.

We even got a couple of actual get well soon cards!

And it is from all over - not just our amazing close friends and family (you know who you are and how much your amazingness means to us both now and as the future unfolds) but also the friends, friends of friends, and friends of friends of friends - often people that we don't know and that had never heard of us before and are now praying for Little S - our synagogue community, our friends' communities - in London, Manchester, Brighton, Israel, America - sending us messages of support, parcels of food and prayers and hopes for better news.

• Tehillim groups here, in Israel and around the world.
• Synagogues saying special prayers for Little S.
• Delivery after delivery of food, sustaining us for weeks to come.
• Psalms and prayers said by friends before their regular poker game - guys you made me so proud to be called your friend.
• Messages from school mates that we haven't seen for a long, long time.

A friend of my mum told her the other day that she was praying for a very poorly little girl with a failing heart and it turned out that she was praying for Little S without even realising!

All so so amazing.

All genuinely making it a little bit easier to cope.

Bless you all.

Oxygen

Our dining room is now a bit noisier than it used to be - there are three oxygen machines in there along with three emergency canisters of oxygen just in case the machines stop working!

Little S isn't on oxygen all day every day - we only really need to give her oxygen if she looks like she is having difficulty breathing.

But because she isn't hooked up to a machine to monitor her oxygen saturation level (sats), we have to be able to see her to tell if she needs the extra O2.

So at night, when we can't see her as we're asleep (hopefully), or when she has a nap in the afternoon, we turn on the machines and pump oxygen up to her room through a dainty plastic pipe running up the stairs.

And as she won't wear an oxygen mask, we need three machines to ensure that when the oxygen wafts across her cot she'll end up breathing in enough of it.

The community nurse came over this week and said that in twenty years she had never seen three oxygen machines used in a single house!

Well, we've got used to the idea now of Little S being a little different to other kids!
But then isn't every child different in their own way?

Home

Little S is home and that means that world to us.

She got back an hour before midnight on New Years Eve which meant we just managed to enjoy seeing our friends on FaceTime to see in the new year.

After a first rather fraught night, Little S has settled down much more and seems to be enjoying being at home and in her own cot much more than being in the hospital!

We've even managed to get her out of the house a couple of times - have oxygen will travel!

And she smiles a bit, laughs a bit, plays a bit, watches cbeebies a lot, eats a bit more and sleeps quite well (at the moment).

So although the future still looks just as bleak, the present has become much better.

And the more we can ensure that Little S is good on the outside, the longer we have together as a family and the longer we have to find a miracle.

Na'aseh Nes l'Shoshanim

Simon

Your song

Sometimes a beautiful song can suddenly mean so much more than it did before.

Take That, Rule The World

You light the skies up above me
A star, so bright you blind me
Don't close your eyes
Don't fade away
Don't fade away

Yeah you and me we can ride on a star
If you stay with me girl, we can rule the world
Yeah you and me we can light up the sky
If you stay by my side, we can rule the world.

If walls break down, I will comfort you
If angels cry, oh I'll be there for you
You've saved my soul
Don't leave me now
Don't leave me now

Yeah you and me we can ride on a star
If you stay with me girl, we can rule the world
Yeah you and me we can light up the sky
If you stay by my side, we can rule the world.

All the stars are coming out tonight
They're lighting up the sky tonight
For you
For you
All the stars are coming out tonight
They're lighting up the sky tonight
For you
For you,

Yeah you and me we can ride on a star
If you stay with me girl, we can rule the world
Yeah you and me we can light up the sky
If you stay by my side, we can rule the world.

All the stars are coming out tonight
They're lighting up the sky tonight
For you
For you
All the stars are coming out tonight
They're lighting up the sky tonight
For you
For you