Memories are made of this

Sunday 6 July

Sometimes you have to ensure that you make your own memories – in fact in some ways, life is all about making as many positive memories as possible.

This week, we decided, in a slightly crazy way, to take all three kids to a wedding of one of J’s cousins in Crewe – and then drive home again on the same day, which meant leaving Crewe at 11pm and getting to bed at 2:30am (we hit a traffic jam on the M6 ‘cos I thought I knew better than Google, but that’s another story).

Well it was definitely worth it … and not just due to the strange sight, during the beautiful outdoor wedding ceremony, of cows from the next door field clambering onto one another, perhaps feeding off the innate romance of the day.

But the real reason was the way that Little S enjoyed herself so much – chatting with other 3 year old girls and telling us that they were ‘talking about dresses’!

And then dancing together at the end of the evening, along with her older sister T and of course my gorgeous wife J.

Dancing with your kids at a family wedding is, I think, one of life’s great joys, something to treasure, and Little S was certainly enjoying it.

She twirled, she toddled, she laughed, she asked to be carried …

Must do more dancing!

Wednesday 9 July

Memories and milestones often coincide – Little S took part in her first nursery sports day: a little relay race, a balance-a-rubber-ring-on-your-head race and a step-in-the-hoops-as-you-run race.

She was superb! I’m gutted that I wasn’t there in person but it seems as though you can make memories out of videos – I now almost feel like I saw it all first hand, recalling the massive smile on Little S’s face on completing her ‘balance’ race.

There was little or nothing to imply that Little S was taking part in any way ‘different’ to any of the other kids – even leading the exercises at one point (think she was doing lunges!).

It’s just wonderful that she continues to find ways to live life to the full – and that we can create long lasting glorious memories as a result.

Monday 14 July

This month’s visit to GOSH – sats were just about ok at 72%, possibly slightly lower than previous visits but possibly just low because Little S was a bit knackered from the past week.

School’s out for summer so we had older sis T with us, which was great when we needed someone to help distract Little S like when the nurse tried to put anaesthetic cream on her hand – she hates that, even though it doesn’t hurt – I guess because she kind of knows that it’s going to lead to an injection later on, for blood tests. So between T and Peppa Pig we managed – both on hand again for the actual injection.

Dr D was on good form – even though he had a gaggle of three female medical students in his office he still managed to pay a lot of attention to Little S and to us.

He was genuinely interested and pleased to see the sports day video (the wonder of mobile phones) and, later, played a bit of catch with T.

Based on some of her recent sats scores, and the fact that Little S is doing fairly well at the moment, Dr D suggested that he thinks it’s probably time to schedule a catheterization procedure to try and insert a stent into Little S’s reconstructed pulmonary artery to inflate and widen the conduit a little bit – the conduit is a small 6mm goretex tube that was inserted when Little S had surgery to connect the reconstructed artery to her heart – essentially it’s what she has instead of a fourth heart valve.

I think he said that it might be able to be inflated to around 7mm, which should improve the blood flow to her lungs and the amount of oxygenated blood flowing around her body and, consequently, her sats. Dr D said that the idea would be to do this without placing undue stress on her heart, either as a result of the procedure and accompanying general anaesthetic or as a result of the extra blood that her heart would need to be pumped around.

Dr D needs to chat this idea through with colleagues but we could be looking at something happening before the end of the year.

I also met with Dr B – a cardiologist at GOSH with particular experience in cardiac regenerative stem cell therapy. 

We had a good chat – he implied that some success has been reported from people injecting stem cells into adults or kids hearts to try and improve heart function, but that the results were far from conclusive – studies have either not used randomization and placebo trials (so you can’t tell whether it’s definitely the stem cells that are causing the effect) or they’ve been performed on adults following heart attacks rather than kids with congenital heart defects (which means that they’ve not needed as big a biopsy to garner the stem cells because a heart attack leaves a localised scar on heart tissue whereas CHDs tend to affect larger parts of the heart – the left ventricle in Little S’s case) or they’ve taken cells from bone marrow rather than the heart itself which seems to have a weaker effect but perhaps is less risky.

Overall Dr B said that technically they could do something with Little S but the chance of it having much effect was pretty small and the risk of something bad happening as a result of the surgery was much higher.

And he hadn’t heard of Dr K (who I’d been trying to talk to in Maryland) but said we should try and get hold of their ‘protocol’ so that Dr B and Dr D could form more of an opinion about how likely is it to be effective.

So in summary, not right now – but in the future, who knows?

And in terms of memories? Well we took in a pizza afterwards that made the day seems a lot brighter for all of us.

Tuesday 15 July

Today we went to the Warner Bros Harry Potter Studio Tour in Leavesden – a genuine tourist attraction just a few miles from home!

Absolutely brilliant and great fun (although not cheap!) - it reminded me (because we got to see them!) of Albus Dumbledore’s memory viewing pensive and his intriguing collection of small, glass, ornate, memory storage bottles – each containing an incredible memory.

Even better than a mobile phone :)

Birthdays and everything after

Monday 19 May 2014

Went to GOSH today and it felt relatively positive.

The hospital has a new reception area which looks good: a rainbow frontage and a reception desk that apparently looks like a boat - not quite as exciting as we’d thought it might be given how long they’ve taken to build it, but fun nonetheless, and at least there’s a fake digital pond on the floor containing ‘water’ that ripples when you walk on it – so Little S had a fun time with that.

However, the cardiology clinic is now hidden away – in via a different entrance and then through a corridor marked as Staff Only!

Having said that, we did eventually find it and everything went fairly smoothly, even though Dr D was not actually there having been delayed ‘in the laboratory’ – not quite sure what he was actually doing there and didn’t really want to ask.

So we got to see Dr R the younger registrar instead, who we hadn’t seen before – she was fine, but much more clinical than Dr D, more interested in the stats and the technical details than in finding out and talking about the person.

She said though that Little S looked much better that she’d been expecting given her notes – she thought that a much more poorly child would be coming through the door, and then Little S had appeared, toddling in, laughing and chatting and looking anything but poorly!

Dr D did arrive at the end of the consultation and Little S seemed pleased to see him – he had a quick look at her, checked her echo (and said that her right ventricle seemed to be doing pretty good – although her left ventricle is still doing badly) and told us to come back in a couple of months.

One thing that they both said is that we need to make sure that Little S is drinking lots: she suffers more than ‘normal’ kids would from dehydration as it makes the blood thicker and therefore even harder for her heart to pump around her body. Unfortunately, drinking water is not Little S’s favourite pastime and so I suspect that there may be few heated debates (or tantrums as they are also known) before the Summer is over.

Dr D had previously agreed that there was no need for blood tests today, so we were done pretty quickly and took the opportunity to grab a couple of ice creams and meander through one of those lovely little square parks that you get in central London – there’s one just across the street from GOSH.

Little S had a few little chats with a couple of the park’s resident pigeons and then we made our way back home.

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I’m finding it a little frustrating that, whilst Little S is in this stable period where her condition is not deteriorating, I don’t seem to be able to make any progress in searching for alternative ways forward or for additional glimmers of medical light and hope in an otherwise bleak future.

Dr D did follow up on the couple of attempts that I’d made at contacting experts in paediatric cardiac regenerative therapies, in Japan and in Maryland, but to no avail.

Like me, they sent him no reply at all.

So I think I now need to go more direct and just pick up the phone.

My other course of action is to try and meet up with Dr B in Israel when I go there in the Summer – I’m hoping to furnish him with the latest Little S data from GOSH and a video of some of her antics and smiles, and then have a chat about his thoughts on what we could possibly do next and whether there is anything that could be done in Israel that wouldn’t be done in GOSH.

Whilst on the topic of paediatric cardiac regenerative therapies, although it’s almost certainly going to take too long to help Little S, one astounding story in the papers caught my eye recently about Professor Williams from the Cardiovascular Innovation Institute in Kentucky who is attempting to use 3D printing to create a functioning human heart from a person’s own cells!

Mindblowing stuff and many years away yet but it at the very least adds another glimmer of light and enlarges the scope of the possible.

Maybe, just maybe …

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Last month was Little S’s third birthday – we had a great day in the sun, outside at the in-laws.

Little S was laughing and dancing and really enjoying herself, and why not – she’s three!

I’ve stopped making speeches now at every family gathering saying how wonderful it is that we’ve reached this milestone in the life of Little S and that we never thought that we’d get there in the dark days at the end of 2012.

It’s not because I’m not constantly reflecting on where we were back then and where we might be in the future, but more because I’d rather just enjoy the happiness of wonderful events like Little S’s birthday, or Seder night at Passover where Little S was in full flow bossing everyone around: where they should sit and what they should do – as a result, I find that I’m confining my more morose thoughts to places like this blog, to conversations with close family and friends and to my own mind.

I’m not sure what others focus on when they see or talk about Little S but I find myself slipping back and forth between the world of a wonderful little girl living and loving life to the full and the dark horizon threatening to invade the normal family life that we’ve created.

“I’m a bit puffed out” she’ll say or “I’ve lost my breath” or just “I’m tired” or she’ll cry in her sleep or look a little under the weather and me or J will comfort her and anxiousness will surface in my mind, rising from the deep where it’s been waiting, biding its time through a period of carefree happiness. Is she ok? Will we need to go to the hospital? Sometimes even: is this it?

But I have to keep telling myself that she just needs a minute to get her puff back, or a couple of hours to chill out and wake up properly, or a day or two to get over a little bug – this is most definitely not it.

Sometimes you have to fight a bit to get what you want out of life – Little S does that a lot – and she keeps winning.

Checkup and How the Heart Works Part 1

Monday 10 March 2014

Back at GOSH again with Little S – time goes so quickly – feels like just a few days since we were last here.

Building work continues on the reception area but things were a bit better this time: toilets and toys were working for a start!

More importantly Dr D was working, in the sense that he paid us a lot more attention, presumably because he had no students sitting in his office that he could ask to prod and check Little S.

He seemed to be in a better mood as well – silly but it can make all of the difference.

Even though the message was pretty much the same as last time (no change on the inside but she’s stable on the outside and we still don’t really know what to do if she deteriorates) we came home thinking more positively simply because Dr D had been smile-ier.

Little S was on good form, toddling around and laughing as she does – Dr D was genuinely and happily stumped about how our miracle girl is remaining so ‘well’.

We looked at the scan of the right and left chambers of her heart and saw how the left side was doing so badly whilst the right side was a lot better (although not functioning normally either).

In a normal heart the left chamber receives newly oxygenated blood from the lungs and pumps it around the body, at which point it returns, deoxygenated, to the right hand side of the heart – the right chamber then pumps it back to the lungs to be oxygenated via the pulmonary arteries and around we go again.

However, Little S has a hole in her heart between the left and right hands sides so the oxygenated and deoxygenated blood mix together to some degree and when one side pumps it affects the other.

Because of this hole, it seems that the right side of Little S’s heart has been able to help the left hand side do its job of pumping good blood around her body whilst also managing to pump bad blood back to her lungs – nice to know that it had some usefulness after all!

Then there’s the conduit that’s been put in to her heart as part of her reconstructed pulmonary arteries – it’s a fixed size, 6mm, so as she grows, it’s might get harder to push enough bad blood through to her lungs to get re-oxygenated – she’d ‘go bluer’ – so there was more talk of a possible catheterisation at some point to stretch her artery and get her ‘pinker’ again, and then a discussion of the attendant risks in doing so – could her weak heart cope with ‘better’ blood?!

We also talked about Dr S and the possibility of cardiac regenerative therapy – injecting stem cells into the heart to improve the way that it functions - Dr D agreed to contact Dr S and Dr K (another doctor doing similar work but in Maryland USA) and see if that gets a better response from them than my response - which has got little better than nowhere so far.

However, just to make sure that we weren't having too much of a warm feeling, he let us know that he thought that it might be decades before stem cells could do the trick for Shani - but let's not lose hope!

By the time we'd done Little S's blood test - in another building like last time - it was nearly time for supper - so we figured let's make this day a bit nicer for Little S and took her to Pizza Express - think we might do that more often now - it makes the day a bit more pleasant for her and means we're not rushing back to sort out some food for her - and we get a pizza as well, so everyone's winner!

We overdosed our daughter - but she's fine

Saturday 8 February 2014

So on Saturday afternoon at about quarter to four, J gave Little S her afternoon heart meds, Captopril, and went to have a nap.

About half an hour later I thought to myself that it was time for Little S to have her afternoon meds and promptly did the same. :(

It was another half hour later, when J woke up again, before I twigged that I should have probably checked with J first, and we found out what we'd done - oops.

We checked with GOSH and they told us to take Little S to Barnet Hospital to make sure that her blood pressure hadn't dropped too much - Little S wasn't exhibiting any worrying symptoms but we were getting pretty anxious. 

I drove Little S to the hospital - she thought it was a great adventure - it's so dark outside!

We got fast tracked to see the pediatric A&E nurse - and we had Peppa Pig videos to keep us company - Little S seemed to be fine - sats were good and, if anything, her blood pressure was a little high rather than low.

The doctor checked her over and said he thought Little S was doing great - he let us know that a double dose of any of her medicines still leaves her below any toxic levels so we shouldn't feel too guilty or get too worried.

Little S came home, happy with her night out.

First GOSH of the year - not great

Monday 13 January 2014

Back at GOSH for the first time this year - the place seems to have gone to pot a little bit.

The building work to upgrade the reception area has overrun, and the cardiac day ward has been closed so we're all squashed into the clinic waiting area that we used to use a year or so ago. As a result, most of the toilets have stopped working and some of the toys as well :(

Then to make things worse, the nurses that usually take Little S's blood were away so we had to go another building to queue up for a blood test, Dr D seemed more interested in using Little S as an educational tool for the two students that were in his room with us than talking to us about his latest pessimistic prognosis, and the heavens weren't happy either dumping hailstones on us as we were about to leave at the end of the day.

The prognosis was that, although Little S's BNP had now come down from 30,000 to about 6,000, her ventricular function was still as bad as it was a year ago. And at some point in the not-too-distant-future her sats are bound to start dropping again as she outgrows the small conduit that was placed in her heart back in November 2011 in order to connect to her reconstructed pulmonary artery linking her heart and lungs. When this happens, she might have to have a catherterisation to inflate her artery or her conduit to allow more oxygen through - but this carries it's own risks: such a procedure is risky anyway given her current condition, and providing more oxygen to her heart may put undue stress on a heart that's having trouble functioning properly anyway.

Meanwhile, Little S was literally dancing around Dr D's room and singing - I think it was a Wiggles song!

We came home a bit deflated but clinging to the silver lining that her BNP was still falling and this may still indicate that things could improve, and also to the incontrovertible fact that Little S is a bit of a miracle worker. ;)

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).