Congenital Heart Disease Awareness Week: 2020

Trigger warning: congenital heart defect, surgery, baby loss.

Going from a very mixed bag of general and community nursing into a specialist heart ward was definitely a shock to the system. On the other wards I’ve considered pulling the emergency for a child who’s oxygen saturation’s fell into the low 80’s if they didn’t correct it themselves imminently.

You and I, most likely walk around everyday with oxygen saturations sitting at 100%. Here I found myself thrust into a world of babies and children that lived everyday reading at 75%, a few less than that.

I was not prepared for the first handover either. A very full busy sheet full of anagrams and very long names to describe other things I had never heard of. I frantically wrote notes on the back of the sheets but ran out of room. I had forgotten my little note book (standard) and was just trying to remember completely foreign terms off of the top of my head. I had read a fair bit around congenital heart defects before starting placement, my heart anatomy is *alright*, but my god I’ve not even scratched the surface.

A lot of nursing is generic, obviously, the skills transfer from one ward to another. Counting a child’s breathing, reading the monitors, pulse checks, temperatures, so on, are universal. Heart babies are unusual in that they often look like very healthy, beautiful newborns, just looking at them you would never know that internally there is a struggle with every single heart beat.

1 in 100 babies are born with a congenital heart defect (CHD) and there is a broad spectrum of conditions. When I put out the question about CHD’s on my Instagram stories, the level of response I had was incredible. It’s hard to picture 100 babies lined up and one of those being born with heart disease, but the amount of responses that poured in showed that this was a reality for so many, and I never knew.

Some CHD’s are managed and monitored, others operated on – a few – are palliative. There are other CHD’s that are missed in childhood, and only become apparent in later life. Although ‘congenital’ means present from birth, there is not always a reason straight away to be concerned. Being diagnosed with a CHD as an adult can potentially be quite mind boggling because there is always the ‘what if?’ factor.

For the first time ever, there are now more adults living with congenital heart defects than babies being born with them.

Sarah

I was diagnosed with WPW (Wolff-Parkinson-White Syndrome) during my first year of uni at the age of 18.

Having had a relatively symptom free childhood, my diagnosis came completely out of the blue after a trip to A&E for an entirely different reason. A couple of ECG’s and a cardiology referral later, I was told that my fast heart rate and occasional palpitations were actually the result of a congenital heart defect, rather than being a lazy, unfit student…although I’m sure that didn’t help.

Post diagnosis, nothing really changed physically – my symptoms were mild so I was
monitored without medication. Although mentally, it definitely took it’s toll.
I’ve never been an overly anxious person, but I’ve struggled with a fear of dying since I was at school – and the knowledge that this little extra pathway in my heart had the potential to cause cardiac arrest (although rare in adults) – was enough to tip me into an anxious spiral.

Palpitations led to bouts of anxiety and sleepless nights (too much Googling definitely didn’t help…what if I died in my sleep?), hospital check-ups resulted in panic attacks, and the sound of a heart monitor or echo scan was enough to bring me to tears.

Eight years on though, I’m now WPW free following a successful EP Study and Catheter
Ablation in 2017. A family history of Atrial Fibrillation (AF) was enough to convince the
cardiologist that treatment was needed…because who wants more than one arrhythmia?
Now it’s gone, so too have the palpitations. My resting heart rate is now at an acceptable
number and I don’t feel like I’ve sunk a bottle of wine when I get out of the shower. Mentally, I’m a lot better too, but it does freak me out that without that hospital trip, I’d probably still be living with an undiagnosed heart condition.

‘Hole in the heart’

A bit of the most common feedback I received was that people felt like when speaking to friends and family about a child’s heart diagnosis was that it was often assumed it was a form of a ‘hole in the heart’ or were told ‘I know X and they had a hole in the heart that cleared up by itself’. Holes in the heart are the most common heart defect, yet another broad spectrum of severity. An atrial septal defect (ASD) is an opening inbetween the top heart chambers. A ventricular septal defect (VSD) is an opening inbetween lower heart chambers. It is entirely possible to have both, AVSD. A heart ‘murmur’ can sometimes be heard when there is a septal defect, a murmur is the sound of the blood passing through the hole into the wrong chamber. Some septal defects are very small, and able to close themselves, others might be keyhole (catheter surgery through the femoral vein/artery) as a younger/older child and some will require being closed in open heart surgery and can make a baby very unwell until fixed.

At some point in utero, sometimes still at birth, we all have a hole in our heart – ductus arteriosus. We rely on this ‘hole’ during our time in the womb. Most of these will close shortly after birth, in premature babies, medicine as simple as aspirin can stimulate closure. When they do not close, it becomes a patent ductal arteriosus (PDA) and requires intevention. PDA’s are so much more complicated when you encounter babies that still have one open on purpose. A few, rare conditions require the PDA to remain open as there is a defect causing insuffiencient blood flow, prostaglandin or surgery is used to keep the opening and allows the PDA to upkeep blood flow to lungs and aorta (which is ‘systemic’ and supplies blood to the rest of the body) this is a short term solution and being ‘duct dependent’ can result in its own issues, such as right sided heart failure.

Katie’s own daughter (who I also share a birthday with) was duct dependent for a while after a triple whammy of a diagnosis.

Katie & Martha-Rose @afarmerswifeandmommy

“After years of trying to conceive, multiply tests and procedures and some medical help along the way we found out we where expecting a baby in June 2015.

By the time we had our 20 week scan we had already found out we where expecting a daughter, announced it to friends and family and ordered her pram. However it was at our 20 week scan that we started to discover that things wasn’t as expected with our daughter.

It took another two weeks and three more scans at our regional specialist hospital to confirm that, Martha-Rose had, and was to be born with a complex congenital heart defect including Tetralogy of Fallot and Absent Pulmonary Valve. We where given a lot of information and a lot of different options surrounding how we could manage her diagnosis including the opportunity to terminate the pregnancy.

By the time her due date was approaching we had continued to meet with paediatric cardiologist, specialist fetal medicine obstetricians and clinical nurse specialist who explained what this diagnosis meant for us and our daughter.

Martha-Rose was born on Thursday 4th February 2016 at Birmingham Women’s Hospital via emergency Caesarean section with a NICU team around her. She was giving oxygen support straight away which soon turned to being completely ventilated and mildly sedated. She was transferred to Birmingham Childrens Hospital within 48hrs of birth which was when they discovered a third, a more rare, congenital heart defect. This is what we now know as a disconnected left pulmonary artery. Birmingham Childrens Hospital had never seen another child with this defect and is reported that she is only number 5 in the world to have found to have this.

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Martha-Rose lasted 12 days without needing surgery however it was felt that a stent being placed into the ductus arteriosus would help keep the prospect of open heart surgery at bay.This would come to last her until she was nearly 13 weeks old. Her first surgery, which lasted over 6 hours, involved giving Martha-Rose a replacement pulmonary valve aswell as repairing the hole in her heart, correcting the disconnected left pulmonary artery and bring all of her heart and arteries forward as these had begun to compress her airway.

Although the surgery was a success it only took 6 months for problems to begin. The repair which had been made to the disconnected left pulmonary artery (otherwise referred to as a conduit) had become stiff due to the large amount of scar tissue that had developed. A cardiac catheter was planned for a few months time with the hope of being able to fit another stent to keep it open however when they came to do the surgery they found that this was not possible.

Martha-Rose’s second open heart surgery took place in July 2017 in which the conduit was replaced, using pig tissue.

Thankfully since this surgery we have had no problems and remain on 6 monthly check ups.

However it is not to say that this journey has been easy, in fact it is one of the hardest things we have ever had to do as people and parents. We have been faced with decisions we never thought we would be, we have done things no parent should have to do and we have seen things we wish we hadn’t. I have been left with post traumatic stress disorder and anxiety. I wake up having panic attacks because of a nightmare I have had, nightmares that take me back to that time or I have unrealistic thoughts and anxiety about her health and future and my,our biggest fear is that we will lose her at any moment.

The child that was potentially described to us when we were given her diagnosis, to the reality are polar opposites. Martha-Rose is a lively, funny, dramatic 4 year old who now had 1 year old brother, Freddie, to join in on the action with.

The reality of congenital heart defects are not what people believe or understand. A regular thing you hear when you explain Martha’s diagnosis is ‘so is she ok now? Is she fixed?’ The short answer is no. She will never be ‘fixed’. She is faced with a lifetime of surgeries, check ups and scans. The surgeries that she has had so far has given her as close to an anatomically correct heart that they could. The valve will need replacing in time as this was a human donor valve. So although it is human tissue it will not grow with Martha-Rose. The conduit which is in situ at the moment will again need replacing as this is also donor tissue from a pig, so this also won’t grow alongside Martha-Rose. They are hoping that we will have another 10 years or so wear out of these before they would be replacing”

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Hypoplastic Left Heart Syndrome (HLHS)

I have included a separate section for HLHS because I have received a few submissions regarding this rare, serious condition. Hypoplastic essentially means ‘underdeveloped, incomplete’ so simply, HLHS means ‘underdeveloped left side of the heart’ and all of the things associated with this side of the heart are affected. It impacts the ventricle, the aorta, the valves on the left, so on. The left of your heart is responsible for delivering oxygen rich blood to the whole of your body (Hypoplastic Right Heart Syndrome does exist also, but is even rarer)

There are three treatment pathways for antenatally diagnosed HLHS, a medical termination, palliative care once the child is born and no surgery, or a three stage surgical plan. These surgeries are commonly known as  ‘The Norwood’, ‘The Glenn’, and ‘The Fontan’.

HLHS cannot be cured. However these ‘palliative’ surgeries can provide weeks, months and years of life. The oldest HLHS patient known was sixty nine years old. As medical science improves and the surgeries are adapted time and time again, it can be expected for this to be more commonplace in future years.

Siobhan & Alfie @siobhan_and_the_boys

Before you go for your 20 week scan people ask you questions like “are you going to find out the sex”.

It was 2012 and while having our 20 week scan I remember being told we were having a baby boy but also being told they couldn’t see his heart clearly. Another scan was booked for the following week but I just knew something wasn’t right. My partner couldn’t get time of work so my sister came with me and that’s when I was told that I would need to go and have a foetal heart scan done as they couldn’t see all of the chambers of the baby’s heart.

A couple weeks passed and that’s when we made the 2 hour trip to the Evelina children’s hospital in London. I remember feeling sick waiting to see the Doctor and during the scan I just looked at the screen silently pleading for nothing to be wrong with our baby. After the scan we were taken into a side room where the doctor started to draw something on a piece of paper. He drew a heart, a heart containing four chambers. He then continued to cross out two of them to explain to us that our unborn son had a condition called hypoplastic left heart syndrome (HLHS). He said put in simpler terms the left side of our baby’s heart had not formed and therefore he would only have half a working heart.

I burst into tears, how could this be happening?! Is this my fault?! What did I do wrong?! While asking questions trying to find out what this meant for the future of our baby we were told/advised that we might want to consider an termination as it “could be for the best”. At this point I was 24 weeks pregnant so the doctor explained I would need signatures from two doctors to have the termination but that would easily be done. Having carried my baby boy for months and finally feeling him move I knew, that that wasn’t something I was going to do.
We were told to go home and think about it but at that point I knew he deserved a chance to fight and I didn’t care if we only got 1 day with him because I already knew I’d give him the world.

Skip forward a horrible/worrying few more months and on the 11th December 2012 at 6.29am our beautiful boy Alfie was born weighing a very good 8lb 15oz. Alfie was rushed away as soon as he was born and all I got to see was the top of his head that was full of dark brown hair. 12 hours later I was finally taken to PICU to see my beautiful baby. He was hooked up to machines to help him breathe and while holding him for the very first time I was told how his surgery would be on the Friday, Alfie would be having open heart surgery (the Norwood procedure) at three just days old. The surgery went to plan and Alfie was in hospital for five weeks before finally being about to go home.

We were back and forth between our home in Norfolk to the hospital in London very frequently for tests and small procedures then at twelve weeks old Alfie had his next planned open heart surgery (hemi-fontan) this time we were home within a week.

Life was hard, I didn’t sleep through the stress and worry and spending time in and out of hospital made it harder being at home as I started to feel like I needed machines to watch that my baby was breathing probably or making sure that everything was ok. Alfie was on so many medicines and I was constantly checking what he needed worried that I would forget something and trying to make sure that everything was just perfect for him. I had to give up work after my maternity leave ended as it just wasn’t possible for me to work around all the hospital appointments but also because I couldn’t bring myself to leave him. When Alfie started nursery in 2015 he only did three hours for three mornings and people would joke that I would be like Forrest Gump waiting outside the whole time and there were days where I had to actually stop myself from doing it.

In August 2016 Alfie had his third open heart surgery (fontan). I don’t know if it was the fact he hadn’t had surgery for a few years or the fact that he was now three years old but it just felt like the worst time ever (and we’ve had some bad times). Alfie was taken down and put to sleep while I waited the seven gruelling hours for a phone call to stay everything had gone to plan. Alfie was out of hospital in ten days and the doctors couldn’t believe how quickly he had recovered after such a big surgery.

Alfie is now seven and even though he has daily struggles like having to be in a wheelchair as he can’t walk very far and not being able to everything his friends can that boy is amazing. I still don’t sleep through worry and stress and check on him more than a dozen time’s a night but he is so worth it. He is so clever and bright, funny, charming, kind, loving and the best big brother to two year old Frankie.

One of the hardest parts of having a child with HLHS is that people don’t understand. They don’t understand how severe it is or how it will never be cured. It’s not a condition they have heard but I knew nothing about CHD until we had Alfie and now it’s basically my whole life. They see a happy little boy who looks just like everyone else on the outside (apart from his scar which he LOVES to show people) but inside he is fighting a battle. We don’t know what the future holds for Alfie as his surgeries are palliative and therefore done to prolong his life and his heart will never be “fixed” but our boy is the bravest and strongest person I know and makes me proud every single day and will always be our amazing heart warrior!

Becky, Adam & Teddy @rebeccajcrosby

“Teddy was diagnosed with a congenital heart defect when I was 24 weeks pregnant. My previous pregnancy had ended at 9 weeks through miscarriage, so I thought I was past the ‘danger zone’ by the time I got to 24 weeks. As they scanned my big belly for an obscenely long time, I wasn’t worried. I thought that Teddy was just being an awkward little thing, as he had been for my first anomaly scan 2 weeks previously. It didn’t cross my mind that something could be wrong.

I have no history of heart problems, everyone in my family was born healthy, as in Adam’s – my partner – family. Hyperemersis Gravidarum (severe vomiting) was a concern. Exhaustion was definitely a problem. The logistics of how I was going to squeeze this baby out of me was starting to terrify me.

Something being wrong with the baby? Nope.

‘I’m sorry, sweetie,’ the sonographer said, not even looking at me as she clicked the keyboard taking screen shots of the scan – of the heart. ‘It’s not good news today.’ For some reason, I noticed that she was barely older than me. Suddenly, I was terrified.
My mum was with me. We asked panicked questions in a flurry, but the sonographer couldn’t answer them – except with a vague ‘There’s something wrong with the heart.’
It was a Friday afternoon, and no consultant was around to talk to us, so we had a long weekend of crying, panicking, and random googling.

Monday I saw a fetomaternal consultant who told me he believed Teddy had Hypoplastic Left Heart Syndrome, a life limiting condition, which did not have a cure. Tuesday, I saw a paediatric cardiology consultant who confirmed it. We were given the statistics. 3 open heart surgeries. A 60% chance of survival. I was offered a termination. We chose to continue the pregnancy. I think we were deluded, thinking that the worst wouldn’t happen to us. That we would be the lucky ones of an unlucky bunch.

I also think I couldn’t conceive of a world where babies died.

My care was transferred from Sheffield to Leeds as they were the nearest heart unit. Every week, my divorced parents, Adam and I would pack ourselves into the car and travel over there, wondering what terrible news we’d receive that appointment. Every appointment was like being punched in the face. My placenta was failing; Teddy’s oesophagus might not be connected to his stomach; I had too much fluid in the womb; I might have Gestational Diabetes; I could go into premature labour.

Every week we went begging for a sliver of hope. It didn’t come.

Teddy was born at 37 weeks, on the 14th October 2018. He was 4.4lbs, and avoided being born on Margaret Thatcher’s birthday by 2 hours and 53 minutes (good lad). He was pink and crying, and Adam and I got to hold him and hug him for a few moments before he was taken to NICU.

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Teddy died on the 25th March, being put under General Anaesthetic for his second open heart surgery.

Obviously a lot of things happened in between those two events. An entire life worth of things. Teddy dying isn’t the most important thing in the story, but it is important.
When he was alive people would tell me how ‘doctors could do amazing things these days’. People would tell me how their hairdresser’s second cousin had a hole in the heart that had healed by itself and they were healthy and perfect now. They’d tell me that Teddy was strong, and a trooper, and would be fine. Everyone wanted to believe that babies couldn’t die, as if by even acknowledging it would jinx it.

Platitudes don’t save babies’ lives, unfortunately.

Congenital heart defects are the most common birth defect. And they kill more children than all childhood cancers combined. Raising awareness is obviously important – research needs funding to find a cure. People need to know the signs so that they can seek help and early intervention. Sonographers need to know what they’re looking for.

But it’s also important because no matter what, some babies will die. And when babies die it can leave people feeling so alone because no one dare talk about it”

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Thank you to everybody who has contributed to this post for Congenital Heart Disease/Defect Awareness Week.

Lots of love, Erin.

Resources:

Great Ormand Street Hospital: https://www.gosh.org/what-we-do/research/our-research-strategy/heart-conditions

NHS: https://www.nhs.uk/conditions/congenital-heart-disease/

Little hearts matter: https://www.lhm.org.uk

 

2 thoughts on “Congenital Heart Disease Awareness Week: 2020

  1. Such an important subject. Thank you for tackling it so sensitively. My sister was born with tetrology of fallot and has had 3 open heart surgeries and countless catheter operations. She’s 40 this year and healthier than me! She has always been one of the best case scenarios for her condition. She had a little boy last year who had the same condition plus a few more complications. He was much healthier at birth than expected and was able to go home to wait for surgery. Unfortunately a routine catheter didn’t go to plan and after emergency open heart surgery and a month in hospital he died a few days before he was 4 months old. The nurses that looked after him deserve a medal, they were so kind and caring as well as knowledgeable. They helped make lovely memories during the worst time of our lives xxx

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