Hypoplastic Left Heart Syndrome: Huxley’s Story

Huxley’s condition, Hypoplastic Left Heart Syndrome (HLHS), was diagnosed at his 20 week-scan. His mum, Shannon, tells us their story so far:

At my 20 week scan, Huxley was diagnosed with HLHS. Our world fell apart with the news. We were told 50% of children don’t make it through all three surgeries needed for his condition and if they do make it through the surgeries, this isn’t a cure and would only prolong his childhood. He would possibly need a heart transplant in the future. The second half of my pregnancy was filled with fear and anxiety, but we stayed positive.

At 39 weeks Huxley was born via planned C-section weighing 8lb 2oz! He was a great size but wasn’t in a good way when he was born. Huxley’s dad and I didn’t get to see or hold him, he was taken straight to intensive care and intubated.

At under 24 hours old, he had septostomy procedure, before I had even met him. A septostomy is a surgical procedure in which a small hole is made in the wall between the left and right atria of the heart. When I finally got to meet Huxley, he was 24 hours old. He was sedated, on a breathing tube & had too many wires, cannulas etc. to count.

We were told to take each hour as it comes, but as the hours and days passed Huxley started to thrive! Within five days he was awake and breathing alone with no oxygen support! We finally got to have cuddles with our baby and see his beautiful eyes. He was also able to meet his big sisters, Scarlett (5 at the time) and Aria (1.5 years at the time).

When Huxley was just eight days old, it was time for the first of three big open heart surgeries – The Norwood procedure. We took Hux down to theatre and kissed him goodnight. In that moment I remember feeling angry. Why my baby?

It tore me apart leaving him, knowing his chest was about to be cut open, to perform a massive surgery on his tiny heart. 11 hours passed and we finally got to see Huxley. Nothing could prepare us for what we saw. He was grey, swollen & his chest was left open.

The days after surgery were critical. Three days after The Norwood, Huxley was rushed back into theatre for a blood clot on his heart. The doctors said, “We need to get to theatre now or he will die.” I felt like I was going to collapse with those words.

Hux pulled through thanks to the amazing surgeons, doctors and nurses. Two days later his chest was closed and a week later he left PICU and moved to the children’s cardiac ward. Here we trained to take our baby home. Training included tube feeding, weighing him, giving meds, baby CPR and to look out for signs he needed medical attention.

When Huxley was five weeks old he came home for the first time, but unfortunately he couldn’t keep any feeds down so we were back in hospital within two days. We then decided, with the doctors, that Huxley would stay as an inpatient until his second open heart surgery (OHS).

At four months old, the second OHS was preformed – The Glenn. This one was quicker at four hours and when he came out his chest had already been closed and he was doing well. Within a few days we realised Huxley had developed Chylothorax. Chylothorax is a rare condition in which lymphatic fluid leaks into the space between the lung and chest wall. This set his recovery right back and we spent the next eight weeks on the cardiac ward, fighting a tough recovery, including sepsis.

When Huxley was about to turn six months old, he was doing amazing and it was time to go home!  FINALLY! But smack in the middle of the coronavirus pandemic. Scary!

He’s now nine months old and thriving. We’ve made so many memories and absolutely love having our family all back together at home. Huxley requires another open heart surgery – The Fontan. We have no idea when this will be yet – it all depends on when he starts needing it. Hopefully not until around 3-5 years of age.

The surgeons, doctors and nurses at Southampton hospital are our heroes. They saved our boys life countless times and we owe them ours.

We are so proud of Huxley!! He’s fought so hard for his life and is the happiest and most beautiful little boy in the world.

Find out more about Hypoplastic Left Heart Syndrome (HLHS) here.