A brave story straight from the heart
Tremendous advances have been made in medicine that allows the diagnosis of some medical conditions even before babies are born. However, sometimes even the forewarning and preparedness do not cushion the experience itself. For seasoned mother of four, Nikki Murphy, the experience of giving birth to her fifth child James was a whirlwind when he had to be rushed to CHI at Crumlin for life-saving open heart surgery.
“James came into our family in April 2018. He had a very uncertain future ahead of him and we didn't know if he would get to come home to join his three brothers and sister. The months leading up to his birth were traumatic and heart-breaking,” says Nikki James’s Mum, “James was diagnosed prenatally with a rare and complex Congenital Heart Defect (CHD) called Hypoplastic Left Heart Syndrome (HLHS). This effectively means James was born with half a heart. His left ventricle, which is the body's main pumping chamber that pumps oxygenated blood around the body, did not develop. He has other associated issues with his valves and aorta. Without surgical intervention the condition would have been fatal. Unfortunately, not all babies born with this condition are candidates for surgery and this was a very real prospect for us leading up to his birth. The news was not always good.
“As soon as James was born, he was quickly baptised and whisked away to the Neonatal Intensive Care Unit (NICU) and then blue-lighted to the Children's Heart Centre in Crumlin whilst I remained in the Rotunda. In Crumlin, we met the most amazing team of cardiologists, surgeons and a whole team of nurses and doctors whose life's work is to care for these kids and give them the best chance possible. We will be eternally grateful to them all but particularly to Professor Colin McMahon, Professor John Mark Redmond, Professor Damien Kenny, Helene, Jackie, Kathleen and the dozens of other nurses and doctors who cared for James and continue to do so. Greg and Aimee will always hold a special place in our hearts.
“At barely 2-days-old, James underwent his first open heart surgery. Nothing can prepare you for handing over your defenceless new born baby to a team of doctors for open heart surgery not knowing if he would come back. After 13 long hours we were finally reunited with James thanks to the skill and talent of his surgeon Professor Mark Redmond. Surgery was a success, a few hiccups along the way leading to a delay coming off bypass but a success, nonetheless. He returned to PICU with his chest open and connected to tubes, wires, machines and a ventilator. It didn't matter, he had survived and crossed the first hurdle.
“Over the next few days he gradually started to come around and smiled when we spoke or read to him and squeezed our fingers. Life in PICU was difficult with limited visiting hours. During this time, we had an army of supporters wishing him well and praying for him. It wasn't long before he was moved up to the Children's Heart Centre ward and he could finally start to meet his family. After a relatively short stay, he finally came home at 21 days old where he continued to thrive. We had weekly visits to Crumlin and daily injections and at 3 1/2 months old he let us know he was ready for his second open heart surgery. We had expected this between 6-8 months old, but he was thriving and gaining weight at an impressive rate defying the expectations of those around him. Again, he made an amazing recovery and was home within 10 days. Having met dozens of other heart parents I know how lucky we were with his recovery. The reality for some is that not every baby has such a good recovery and some do not get to come home so quick or at all.
“James has remained healthy and well since his last surgery and is now a very active, energetic, funny and curious 2-year-old boy. He is an absolute joy to be around (even at 3 o'clock in the morning) and is almost always happy. Temper tantrums are starting to appear though. His siblings adore him and get great craic out of him. We take every day as a gift and enjoy every minute of him. He is now waiting for the last of the 3 planned surgeries to have the right side of his heart take over the job of the missing left side. He will undergo a series of tests in the next year to prepare for this. After that he will require lifelong follow-up care. Surgery is not a cure.
“Until James was born I knew nothing about Congenital Heart Defects (CHD) other than perhaps hearing about a child with a hole in the heart. I never realised how prevalent it was. I was shocked to discover that CHD is the most common form of birth defect. A significant number of new-borns (approximately 1% of all live births) are affected by congenital heart disease. In Ireland, about 650 children are born with structural abnormalities of the heart every year (translating to about 1.5 million children born annually with such defects worldwide).
“There is no cure for CHD, children who undergo surgery are not fixed, they are not cured which is one of the biggest misconceptions out there. Some children with CHD can go through life with little impact on their health and energy levels, with some even playing sports. To look at James you would not know how he had any issues at all. His scar is not visible and to the untrained eye he does not show any outward signs of being sick. Yet he will always have less energy than his peers and will always be affected by his condition. He may never be able to run his own marathon or take part in a triathlon like his uncle Frankie. That said our hope for James is that he will not be held back in life because of his condition and that he will grow up with the same opportunities, hopes and dreams as his siblings and peers. This will only be made possible by continued research and advancement in the treatment of CHD.
“We have not shared James's story up until now but are extremely grateful to Frankie for choosing the Children’s Heart Centre as his charity in doing his Ironman challenge that we felt the time was right to share his story. Whilst neither I nor James will be running, swimming or cycling with you Frankie we will be with you in spirit supporting you every step along the way and will be waiting at the finish line to celebrate your achievement. Thank you to all who have donated and supported this worthy cause so far.”
Nikki, James’s Mum
With your support we will continue to do #WhateverItTakes
To find out more about James’s Uncle Frankie’s epic Ironman challenge and to donate click the following link: https://ie.gofundme.com/f/becoming-an-ironman-for-our-children-in-need