When I was pregnant with Evie, we found out that she would likely be born with a set of heart defects too severe to be corrected with surgery. Our local hospital does not provide fetal medicine speciality services so after our 12 week scan we were referred to Galway which was over 2 hours away from where we were living at the time. This was where the majority of our scans took place until we decided to that we would deliver in Dublin – 3 hours from home. The main factor for this decision was that Galway does not provide fetal or paediatric cardiology. There would be no paediatric cardiologist to review Evie right after birth, so it made sense for us to deliver in Dublin instead. Ross’ parents also live nearby so we knew we’d have their support, whereas in Galway we would have had no nearby family support.
We spent the entire pregnancy attending scans every 2 weeks in Galway and later on in Dublin also. Most of my energy was used up just on travelling and the thoughts of such frequent scans was completely overwhelming; looking back I wish we had opted for a lot less. Mostly they were just checking to see if Evie still had a heartbeat, with no new or valuable information for us. One scan, however, did show a smaller right ventricle in her heart and this was when we were referred to Dublin.
At 20 weeks we had our first fetal echocardiogram (this is a scan of the baby’s heart while still in utero). This scan took place in Dublin and it was our first time attending The Coombe hospital. It was an emotionally draining day and I remember being overwhelmed with another new hospital and again, without Ross due to covid restrictions. It was so hot and there were so many appointments ahead of mine, I ended up waiting for 5 hours to be seen. Ross wasn’t allowed to join me until the scan was complete and it felt like an eternity.
I can only describe Evie’s cardiologist Orla Franklin as being an absolute lady, she has a powerful presence and although I felt safe with her guidance during the echo, it was made more worrying for me without Ross’ support. During the scan, Orla Franklin and Dr. Lynch (my obstetrician) had told me a few details from the echo but nothing in depth and nothing about the potential that they wouldn’t be able to offer Evie heart surgery.
With Ross in the room, and scan complete, Orla Franklin while writing her notes looked at us with clear concern in her eyes. She first explained the make up of Evie’s one of a kind, special little heart (which the nurse had said was about the size of your thumbnail at this stage), and then described its function with a diagram. When she was sure we understood, she went on to explain her concerns. Firstly, she said that while Evie continued to grow, her heart might not continue to grow and develop. In this case, she would likely not make it to full term and even if she did, she wouldn’t survive delivery or the immediate period after. The next scenario was that Evie’s heart would continue to grow with her but again, it would not develop properly and we would be facing the same outcome. The more hopeful option was that nothing would change structurally, Evie and her little heart would continue to grow and we would reach full term and hope for a successful delivery.
Evie’s cardiac diagnoses at that time was severe hypoplasia of the right ventricle, complete unbalanced atrioventricular septal defect (AVSD), common atrioventricular (AV) valve with regurgitation or “leaky valve”. This set of defects did not paint a very good outcome for our baby girl, but with a tonne of luck and positivity – she made it. Her heart grew with her, did not change structurally and allowed her to arrive pink and crying into this bright, strange world. This was the first real indication that Evie is made of much stronger stuff than any health care professional could ever envisage.
However, the stance regarding options for surgery remained unchanged from that first detailed look at Evie’s heart. Without wanting to overload you as the reader with too much detail and medical jargon, I will try and explain in simpler terms. We had requested this from Orla Franklin, as although I have a nursing background, I am not au fait with cardiology – especially paediatric cardiology. Receiving knowledge in lay man’s terms, I’ve found to be hugely beneficial when you have been overloaded with information about your own child, also for speaking to other people about your journey. And of course, there’s Ross with his limited knowledge of the medical world; sometimes I mildly envy him for that.
Evie’s complete unbalanced AVSD (holes in the septum of the heart) are huge, there is very little tissue there that a surgeon could put a patch on. Her next problem is her severely underdeveloped (hypoplastic) right ventricle. The right side of the heart pumps blood to the lungs so Evie’s symptoms include rapid breathing, poor oxygenation, feeding difficulties, sweating and tiring quickly; and that’s all before she developed acute respiratory distress from contracting rhinovirus (the common cold). Where there should be two valves between the atriums and ventricles, Evie has one known as a common AV valve. This is inefficient and allows oxygenated blood to mix with deoxygenated blood – known as regurgitation. Regurgitation can vary from mild to severe – you guessed it, Evie’s is severe. Evie also has a patent ductus arteriosus (PDA) which is a persistent opening of the two major blood vessels that lead from the heart. This is a normal part of the circulatory system of a baby still in the womb and usually closes shortly after birth. If it remains open, like in Evie’s case, it causes bidirectional blood flow. This again, causes poorly oxygenated blood to flow the wrong way which leads to weakening of the heart muscle and heart failure.
Evie went into heart failure at around 6/7 weeks old. She was commenced on diuretic medication to offload extra fluid and take pressure of her heart. Her NG (naso-gastric feeding) tube was inserted at this time as well. Her weight gain had been slow as she had such difficulty feeding but after the NG tube we noticed a dramatic improvement. She did loose the ability to breastfeed and drink from a bottle but the immense pressure had been take off us which was a huge boost for two tired, stressed out parents. There had been mention of a palliative surgery called PA banding where a band is placed on the pulmonary artery to slow down blood flow of deoxygenated blood to the lungs. The team in Crumlin however, decided that this would likely put even more pressure on Evie’s leaky valve and by not doing this we would avoid Evie risking the complications of open heart surgery.
On paper, Evie shouldn’t have survived this far without surgical intervention. She has and continues to prove her worth and desire to be here with us. My job is to guide our family in the right direction and collect all of the necessary information needed to make those difficult decisions. Second, third, fourth opinions are required and I will pursue them. This won’t be a quick decision nor a quick fix but I’m determined to achieve the best outcome for our Little Bird ❤️
The following is taken from the Instagram post I shared after that initial fetal echo in March 2021.
Our appointment in The Coombe yesterday was draining. We met with two consultants – paediatrician cardiologist and fetal medicine consultant who were both wonderful. Ross was allowed to come in after the scan to speak to the consultants. We learned that the cystic hygroma is still there, we thought it had gone/significantly reduced in size. We also learned about our baby’s heart – underdeveloped on the right side (hypoplastic right heart syndrome), a common valve instead of 2 separate valves from the atriums into the ventricles which is causing regurgitation of oxygenated blood allowing it to mix with deoxygenated blood, quite a small aorta, and 2 large holes between the atriums and between the ventricles (AVSD). They told us that AVSD is quite common and very manageable, they told us that the other conditions aren’t as manageable and if things don’t improve in the coming weeks they they won’t be able to offer her surgery after she’s born. It’s impossible to feel optimistic after hearing something like that. I don’t feel like trying to be positive, I just want to feel down and let myself be sad. I know I’ll feel okay again soon. I know that I don’t want to give up hope for our little girl and she’s fought this far. It’s a painful waiting game and I don’t know if we’re going to win. Taking pleasure in the small things like feeling her tiny kicks and knowing how much we’ve loved her from the very beginning will get us through this ❤️
