Lucy’s heart journey – a Christmas story

Written by her parents Ruth and Adam 

Lucy aged 4

We found out that Lucy had Down syndrome when we were 13-14 weeks pregnant. We were shocked and in emotional turmoil. Down syndrome hadn’t been in the ‘plan’. So when I got a call from an unknown nurse telling me our unborn child had an extra chromosome, our world fell apart. We were terrified that our child would never walk, never communicate with us. How little did we know and how wrong were we?!

But while we were seeking the counsel of close family and friends, events overtook us that shook us to our core. Over 50% of babies with Down syndrome have a congenital heart defect,  and so, at 15 weeks gestation we found ourselves being ushered in for our daughter’s first ante-natal heart scan, at a fetal cardiology clinic run onsite at St George’s by the Royal Brompton Hospital, a world-class centre of excellence for paediatric cardiology. 

Lucy at 3 months old

It was at this point that our perspective started to change. We learnt that our precious girl had a congenital heart defect, a suspected large Ventricular Septal Defect, and that while in some cases this could resolve itself during pregnancy, we were warned that open heart surgery was highly likely for our baby in her first 6 months of life. For the second time in as many weeks, our world fell apart. 

Already struggling to come to terms with what our life would be like with a child with Down syndrome, we now faced the prospect of open heart surgery on an infant, that while often successful, still carried significant risk to life. That news, although devastating, was like a lightbulb moment for us. Instead of worrying about how our life would be with her in it, our focus turned to how desperately we wanted the chance to find out. We wanted her to live.

Lucy’s first few months were difficult. The energy required for her to feed took more and more out of her and so she was booked in for her surgery at just 4 months old, and weighing a tiny 4.3kgs. Full of apprehension about how it would all go, we had taken her for her first Christmas photo shoot a couple of weeks earlier, knowing there likely wouldn’t be time after her surgery before Christmas.

Our beautiful girl was admitted to the Royal Brompton for open heart surgery on 29th November 2017. It’s a terrifying experience, even when it all goes smoothly. Handing Lucy over –  who by this point had learnt to roll on to her tummy, had a killer grin and was starting to show us what a strong and independent character she was – to the surgical team, was one of the hardest things we’ve ever done.  “It will be fine…it’s bread and butter to these amazing surgeons…she will bounce back” the many professionals we met during our pregnancy had said. But for Lucy, it didn’t quite go to plan. 

We paced the floor for 6 hours and finally, the surgeon came to see us. He seemed exhausted and it was not the positive ‘she’s all fixed’ message we had hoped for. It turned out that Lucy’s barely the size of a walnut heart had required four separate procedures. We were told the next 72 hours were critical. She was so small in that big hospital cot, and she had been returned from theatre to PICU with her chest open – in case rapid access was required. And so we sat with her, watching her tiny heart beat through the transparent dressing and waiting…

A few hours later the nurses told us to go to bed, so we did, but were woken at 4am to be told she was very unwell. By the time we had run from the parent accommodation to PICU her surgeon had already attended, and had drained a build up of fluid around her heart. Again we sat and waited. 

At 7am we left to get showers and breakfast, and were again called to come quickly. This time, we were met at the door of PICU and ushered into an office. They told us our baby girl had had a cardiac arrest. The stress of the surgery had been too great and her little heart had stopped.  ‘Is she going to die?’ I asked. ‘We don’t know, they are doing everything they can,’ was the terrifying answer. 

For 40 minutes the PICU crash team – a mix of consultants, registrars and nurses, took turns to perform CPR directly on her little heart. We had been told about the existence of an ECMO circuit, a complex heart and lung bypass system but we hadn’t really understood its complexity, or the gravity of the situation that you needed to be in to require its support. By the time we were able to see Lucy several hours later, she was plumbed up to an ECMO circuit, was on kidney dialysis and had 17 drug pumps going into her. 

Lucy on CPAP

And so the longest vigil began. For 6 long days, we sat and waited. We were told to expect a long stay in hospital and hopes for our first Christmas at home dwindled. The greatest risk on ECMO is that a blood clot will form and pass to the brain, before it can be filtered out by the circuit. So every hour, night and day, a specialist ECMO nurse studied the tubes with a little torch, looking for evidence of clotting. 

We learnt it was all about balance. Fluid in, fluid out, every ml was counted. Countless ECGs, ultrasounds; a cardiac catheterisation that required a team of 12 staff to move her and her ECMO circuit down a floor to the cath lab to see if the specific cause of the cardiac arrest could be determined; a femoral artery blood clot that caused one foot to be 2 degrees colder than the other, while blood seeped from her groin for 6 long days as anticoagulants were essential to reduce clot risk. 

We sat, we waited, we prayed. After 6 days, on the 6th December, the consultants decided it was time to attempt to wean her off the ECMO circuit. We were given a 40% chance that Lucy would survive. The chaplain came and prayed over her. The psychologist asked us some very hard questions, in case she did not make it. Would we want to carry her to the morgue? Would we want to dress her? In hospitals these decisions have to be made quickly so they wanted to give us a chance to think through some of the awful decisions we had a high chance of needing to make. 

And then after several nervous hours, our first Christmas miracle occurred. Lucy’s heart started beating! She was weaned off the ECMO circuit and over the next few days, the drug pumps were reduced, and after 11 days her chest was finally closed. 

Finally home!

Next she was extubated, and we thought we were on our way home. We got to hold her, and she started to move and open her eyes as the drugs were reduced. But then, when her final chest drain was removed, the pain caused her to gasp in air, causing a pneumothorax. She was placed on CPAP and we anxiously waited to find out if further surgical intervention was required. 

But to the amazement of everyone, 3 days later she literally bounced back and after 23 days in intensive care we finally got stepped down to Rose ward and began being assessed for discharge. 

And so on the 21st December, 2017, our miracle girl was discharged. We can never thank the incredible team at the Royal Brompton Hospital enough. We didn’t have a Christmas tree, or a turkey, or any presents. And we didn’t care. She was the best present ever and we count our lucky stars we got to take her home. 

Lucy has continued to fill our lives with joy ever since. Over the last four years she has become who she was meant to be. A strong minded, independent girl with an adventurous spirit and a wicked sense of humour. She loves nothing more than dancing along to Strictly with her daddy, jumping off the stairs, running as fast as she can, singing along now almost word perfect to virtually every Singing Hands song ever recorded, and pretending to be a cat at every opportunity. 

It’s become a Christmas tradition now to have a photo shoot every year, to commemorate her difficult time in her first year and to celebrate everything that she has become. We are so proud of our Lucy the Lionheart ❤️ and we wouldn’t change a thing.

Lucy aged 2

Merry Christmas from Adam, Ruth and Lucy xxx