Do you believe in magic?

Sunday 09 November 2014

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

Coldplay, Magic 2014

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

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

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

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

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

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

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

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

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

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

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

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

And now it's the day before the catheterisation.

Fear and apprehension fill my thoughts.

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

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

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

Believe in magic? Of course I do

Summer's gone

Sunday 14 September 2014

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

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

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

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

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

It certainly feels that way with Little S.

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

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

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

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

So darker days - 
but Autumn is not Winter

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

Monday 15 September

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

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

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

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

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

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

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.

--------------------------

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 …

-------------------------

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. ;)