Tuesday, August 21, 2007

August 21, 2007

Hello again. I've only just realised that I've never really put any details about when Charlie was diagnosed with HLHS, and what happened when he was born. Although I know a lot of people who read his blog already know his story, we've picked up a lot of new friends along the way who don't! So here it is....
We were thrilled and terrified to discover we were expecting our third child. I had miscarried our first baby at 12 weeks, and our second son, Will, died fifteen minutes after he was born because of a lethal genetic condition.
Will’s condition is autosomal recessive, which means that every child my husband and I conceive has a 25% chance of inheriting this fatal condition. Needless to say, when I became pregnant again, we were full of dread that this child would also be doomed to leave us within minutes of birth.
We were told at the 14-week scan that all was looking very good, and the doctors were cautiously optimistic that we might have escaped MGS this time. We were told that the 16-week scan would be 'crunch time' - at which the baby would be well-enough developed for the doctors to confirm whether or not we had passed on Meckel-Gruber syndrome to this child too.
As I lay on the scanning table at 16 weeks, the radiographer and doctor - with whom we were already on first-name terms having already had a 'unusual' pregnancy not too long before - told us that our child was clear of the kidney and brain problems which would have indicated MGS.
However, we had barely let out a whoop of delight, when the radiographer told us to 'Hang on minute, I don't think the heart is normal. It looks too small.' We were horrified.
As well as having MGS, Will had been diagnosed with a major congenital heart defect called Hypoplastic Left Heart Syndrome - a condition where the left side of the heart does not develop. It had been assumed by all the medics at the time that this was linked in with his MGS, as cardiac abnormalities had been reported previously in other cases of the syndrome.
I asked the radiographer if it was the same heart condition as Will had - and she said she was virtually certain it was.
The cardiac consultant was called to the room and confirmed that it was true. Our child had HLHS.
We were blown away. Rather than being upset, I was furious, and ranting at everyone in the room. How could this be? How could something ELSE be wrong with one of our children? How many more potentially fatal conditions would we be forced to deal with? We had braced ourselves for miscarriage or Meckel-Gruber syndrome, but not this - something new and horrible.
We wanted to know whether we were having a boy or a girl, but the baby wouldn't co-operate to let us find out.
We were taken to one of the 'bad news rooms' - we knew it well, as it was where we'd been taken after Will was diagnosed.
The cardiologist, with the aid of many, many scribbled diagrams and notes and statistics, explained to us what the future held.
Without treatment, the child would definitely die within a few days of birth. We could opt for termination, compassionate care - taking the child home and leaving nature to take its course, or three-stage, high-risk palliative surgery.
The baby would need at least three major, open-heart surgeries before it started school. These operations could not cure the condition - merely enable the heart to continue functioning. The odds of getting it through these operations successfully to age five were around 60-65%.
There were many other issues that could crop up over the course of the child's life, and there was a strong possibility that the single ventricle the child did have would begin to fail in late adolescence or early adulthood, and that transplant, with all its attendant problems, would then be the only option available. There were no guarantees.
Treatment for the condition was relatively new, and the oldest child in the country was only around 13 years old at the time.
I think the medical team knew what was coming as we had already opted to take a pregnancy to term knowing that the baby was 'incompatible with life'. My husband, Pete, and I looked at each other. When we were having Will, we had wished and wished that there was even a 1% chance of him surviving, but we knew there was no hope at all.
This child, despite having a major heart defect, had a chance - 60-65% chance in fact - and we grabbed it with both hands.
Two weeks later when we returned for our next scan, our baby was in a better position and we were told that it was a 'he'! We named him Charlie William - his middle name after his big brother.
As my pregnancy progressed, we were stunned by the support we received from everywhere. We received letters and cards from people we had never even met, telling us how they were thinking of us and praying for us. Everyone was resolutely positive - and so were we.
I had to deliver Charlie at a hospital 150miles from home, so he could be near to the children's hospital which performed the high-risk surgery.
He finally arrived two weeks late on November 5, 2003. He came into the world shouting, weighing 8lbs 13oz. He was pink and feisty, and his apgar scores were nine and nine. We couldn't have asked for more.
His first surgery was performed at two days old. We were calm and resolute as we watched him anaesthetised, and the six-and-a-half hour wait while he was in theatre seemed to go on for ever.
Pete and I, and my parents were sitting in a little waiting room drinking bad coffee when the nurse who had been caring for Charlie before he went to theatre came running in and shouted 'He's fine! He's in ITU!'. En masse we burst into tears of relief. Everything went smoothly with Charlie's recovery and we were home a mere 12 days post-op.
His second-stage surgery took place - uneventfully - when he was five months old, and we were home only six days later.
Since then, Charlie has been remarkably robust and well, and seems to be pretty much unaffected by his heart problem. He runs about, rides his bike, goes to playschool and is way ahead of all his developmental milestones. He is ahead of his peers as far as reading and writing is concerned. People are always telling us that you would never know there was anything wrong with him.
He is due his third (and hopefully final!) surgery before he starts school, and although we are dreading taking him back to hospital - he has had no admissions since he was five months old - we have every faith that he will do well and will be back home with us in no time.
Of course, there is no denying that life as the parent of a child with HLHS is fraught with worry. Even with a child as well as Charlie, there is always the fear that each cold or sniffle could be the start of something big.
There's also the ever-present fear that he may not see old age - but nothing in life is certain - HLHS or not.
He has been a blessing to us in so many ways - he and his brother have taught us so much. Living day-to-day with a life-threatening condition really makes you get your priorities straight, and teaches you to appreciate every minute of every day. You can never be sure of tomorrow, so you make the most of today. We never take Charlie for granted - even if he is being crabby (which doesn't happen very often!) we're just glad that he's actually here to BE crabby!
Every time he achieves something new, he makes us so proud. He is a delightful, sunny-natured, chatty, smiley little boy who is loved by all who know him. Our hearts sing as we watch him grow. We are proud and privileged to be able to call him our son.

PS If you're wondering how Charlie's hospital checkup went yesterday, the details are in the previous post!

Monday, August 20, 2007

August 20, 2007

Well we've just got back from the hospital, and I'm really pleased to report that Charlie is STILL doing brilliantly. His oxygen saturations were 83-84, which is the same as last time, even a little bit better. This is amazing, because he's growing bigger by the day, so the bigger his body gets, the harder it should be for his heart to pump the oxygenated blood around... but it doesn't seem to be affecting him at all at the moment, and he is maintaining really well. He's growing like a trouper too, and is rocketing up throught the centile charts.
His ECG was fine, and his echo (an ultrasound scan of his heart) showed that his heart function is still great, and his valves are working well too.
We were really pleased to speak to two cardiac consultants - the one who looks after Charlie now, and the one who cared for and monitored Charlie before he was born. Both said Charlie looks great. We were particularly touched to be told that one of the doctors often uses Charlie as an example for other parents who have been given the crushing diagnosis of HLHS, to show them how life can be, and how well these children can do.
Another brilliant bit of news is that Charlie is doing so well, there really is no rush for the next stage of surgery. In fact, we were told that Charlie probably wouldn't be called for a pre-surgery cardiac catheter until some time next year, with the fontan to follow even later on.
In all honesty, we'd thought Charlie's catheter would probably be in the next month or two, so it really feels like a reprieve. Although it does depend on the medics at Birmingham, because it's their call really.
We were also delighted to be given some more news about HLHS in general. When we were given Charlie's diagnosis, we were told that there was a 50% chance of children with this condition reaching the age of five. Today we heard that the figure being quoted now is 80+%. The consultant also told us that many of the previously-untreatable conditions/side-effects associated with HLHS, such as protein-losing enteropathy, are now treatable and even curable. According to the doctor, the prognosis for people with HLHS is much less grim now, and everybody is much more positive and optimistic about a favourable long-term outcome.
In the past, we've always been told that people relying on their right ventricle to do all the work - either because the left is missing, like Charlie's, or because their 'plumbing' is faulty, leaving their left ventricle redundant - would, in all probability, need a transplant in their late teens or early adulthood, because it wasn't thought that the right ventricle could sustain itself for any length of time.
However we were given another amazing piece of news today by the consultant, who told us that there are people out there relying on JUST their right ventricle, who are now in their forties and still going strong.
Although they do not have exactly the same condition as Charlie, the end result is the same. We were blown away by this news. I don't think the consultant realised quite what he had given us in those few words.
Until now, we had never allowed ourselves to think beyond mid-twenties for Charlie. Of course we have hazy hopes and daydreams about him getting married, having kids, even having grandchildren, but not in any concrete terms.
To be told that there are people out there, now in their forties, and living a good life has bought us another two decades-worth of hoping. This is enormous for us - as long as we have hope for the future, we can do anything.
Charlie behaved brilliantly throughout the whole appointment - he lay so still for his ECG that they got all the results they needed in just one go, plus he was really quiet and well-behaved for his echo, and did everything the doctor asked of him. We've been given another appointment in six months.
We're so proud of Charlie, and so grateful that he's doing so well.

Sunday, August 12, 2007

August 12, 2007

Well, the warm weather has held out for the weekend (although they're promising high winds and heavy rain next week!) and we spent a nice afternoon at the hot-air balloon festival in Bristol.
Charlie was very excited about the whole thing - one, because Grandpa was coming too, and two, because the event was backed by the Discovery Channel's 'Shark Week'! When we arrived, we found the shark stand, and he sat for ages, engrossed in a discussion all about the different types of shark. We were surprised how well he listened, because it wasn't really geared towards children his age, but he joined in, answered questions, helped the man giving the talk to pace out the size of a basking shark, and got his hands on a fossilised megalodon tooth (don't ask me - I'm just the typist!) and a whole jawbone from another shark complete with four or five rows of VERY sharp teeth!
There were also loads of rides and fairground stalls, which we obviously had to visit! The crocodile rollercoaster was a big favourite (see pic above) and we had to have several goes on a 'hook a duck' game, so Charlie could win a talking Puss in Boots from Shrek.
We found a prime seat in the shade, right by the main arena, and really enjoyed watching all the displays - although Charlie was most impressed by a man in a gorilla suit, riding a motorcycle. Well - I suppose he is only three! He'd decided to take his binoculars with him (or 'nocliers' as he calls them!) and entertained himself for quite a while by looking through the wrong end of them!
Although the weather was fab, the balloons were quite late taking off, so it was a late night for Charlie. In spite of this though, he really enjoyed watching them lift off, and spent the whole journey home spotting them out of the car window.
He's really had his appetite whetted by the rides at the balloon show, so now he's counting the days until we go to Legoland!
No more news really - I'm attaching a few more pics from Saturday.

Wednesday, August 08, 2007

August 8, 2007

Hello everyone. Well, we've finally had a bit more summer - only a couple of days of warm weather - but that was enough for Charlie to hit the paddling pool again. In fact, by the time we'd actually finished blowing the pool up, the sun had gone in a bit, but one wetsuit and several kettles of hot water later, the paddling pool was just right!
I'm attaching a couple of pics of our little water-baby. Mind you, there's not much of the baby left any more! Only today Charlie told me that I wasn't wiping his face with a baby wipe - 'It's a big-boy wipe, Mummy'. Every day he's getting bigger, and more grown up. His reading and writing are really coming on, and I think we're going to have to go up a couple of stages in jigsaws. We got a new 36-piecer the other day, which he did from scratch in the time it took me to make a cup of coffee. His Great-Grandma was a real whizz at jigsaws though, so maybe he's inherited it from her!
There isn't much more specific news really - we're just pottering along quite happily, enjoying the summer holidays. He thoroughly enjoyed himself at the parent/toddler group sports day. I'm attaching a pic of him in the running race. I watch him joining in with 'normal' things like sports day, and can't help but feel a bit emotional. What a marvel he is. I found a diary the other day, that I kept while I was expecting Charlie, and it is amazing to see by how much he has surpassed the small hopes and expectations that we had then. I know I've said it before, but having a son like Charlie has its advantages - we still take nothing for granted, and appreciate every minute of every day, and every new achievement. I'm sure he'll look at this when he's older and cringe, but we are so, so proud of him, and thankful for every day.
We're taking Charlie to Legoland in a couple of weeks, which should be fun. I had to go there once before to review the place for work, but this was before we had kids, so Pete and I felt a little out of place - but now we'll be able to go on all the rides without attracting suspicious looks! Charlie's itching to go on a rollercoaster - just hoping he's tall enough to get onto the 'kiddie-coaster'! Pictures to follow, no doubt!
Charlie's latest craze (in addition to sharks, which are still hot stuff as far as he is concerned) is Finley the Fire Engine - a new kids' TV show. Unfortunately, the only thing he's been able to find online is the theme tune, so he's playing it at least 20 times a day, and singing it in the times he's not playing it.... which means we can't stop singing it either!
On a sad note, it is Baby Isaac's funeral tomorrow. Saying goodbye to your child is a terribly difficult experience, so we're hoping that everything runs smoothly, and that his parents and grandparents will find peace as they remember their little boy. It is strange how strongly we feel the connection with other HLHS parents. We celebrate their triumphs and grieve for their losses almost as though they were our own. We will be thinking of Patience and Jordan tomorrow.

Monday, August 06, 2007

August 6, 2007

This is just a quick post to let you all know that Baby Isaac, who I mentioned a couple of posts ago, sadly died yesterday afternoon. He was at home with his family, and was in his parents' arms right to the very end. While his family are heart-broken that he has gone, they are glad that he doesn't have to go through any more pain. Please keep Isaac's parents Patience and Jordan, and the rest of his extended family in your thoughts and prayers over the inevitably difficult weeks ahead.