By Sonia Milbradt
Ada’s story begins before she was born—her heart defect was detected at the 20-week anatomy scan. The scan happened on a Friday afternoon and I received a call on Monday from the Burnaby Maternity Clinic, where the pregnancy was being followed, to come in to discuss the results. The doctor must have been very busy, because I was given the news very quickly and frankly that the scan showed that half of her heart hadn’t formed. He pointed out how all four chambers were supposed to be the same size and highlighted how the right ventricle was not formed. He said he would refer me to BC Children’s Hospital (BCCH) and we would find more out after a fetal echocardiogram.
What followed was an agonizing week while my husband and I waited for our appointment at BCCH. The day arrived, and it was a whirlwind. We had the fetal echocardiogram, then met with the amazing cardiology nurse and cardiologist who talked us through the diagnosis, then met with the genetics team, then met with a doctor from Maternal Fetal Medicine.
We were told that Ada had tricuspid stenosis (diagnosed as functionally tricuspid atresia after birth), which means the valve between the right atrium and ventricle was too small and there wasn’t enough blood flow on that side of the heart during development. This resulted in a hypoplastic right ventricle, meaning an underdeveloped right ventricle. She also has a large ventricular septal defect. This is good in her case, because it means oxygenated blood and deoxygenated blood can mix and get to the stronger left ventricle, as her heart acts like only a single pump.
Knowing the diagnosis before birth was both a blessing and a burden. Ada was our first child, and we didn’t know how eager to be and how much to prepare, despite the assurances of the cardiology nurses and cardiologists we saw in the next months. The phrase “knowing but not knowing” kept coming up, because we knew about Ada’s congenital heart defect (CHD), but we didn’t know exactly what she would need or how the interventions would go until she was born. It was very hard to be excited, because we didn’t want to get too attached for fear of the worst-case scenario. Luckily, medical advances in the field of cardiac surgery in the past 40 years have meant that children born with this defect can live into adulthood.
Ada made her appearance into the world via an emergency C-section on September 8, 2019, at 4:00 am, when progress stalled after many hours of active labour. She was whisked away to the cardiac intensive care unit (CICU) for tests as planned quickly after birth with my husband right by her side while I went to recovery. I did get a cheeky detour to the CICU to see her before they brought me to my own room on the maternity side. I was given one of the smaller rooms since Ada was in her own big room in the gorgeous Teck Acute Care Centre. I am so thankful that we have such a lovely facility in Vancouver for such a challenging time. It was very hard listening to all the moms bonding with their children and hearing the cries of the babies while I was alone in my room on the maternity side. I was able to arrange it so that I spent most of the time over with Ada and my husband in the CICU, only being wheeled back every four hours for the required checkup and to take my pain medication. That way I was able to begin my breastfeeding journey with Ada as well, which she did well at despite the ballet of tubes and wires we had to do with the CICU nurse every time.
Ada was stable and didn’t need any interventions for the first day of her birth. On day two, she had a catheter procedure, called an atrial septostomy. This involved running a tube through her veins with a little balloon on the end that was then blown up in her heart to widen the small hole she had between her atria. This allowed better mixing of the deoxygenated and oxygenated blood in the upper chambers of her heart. She was sent back to her room in the CICU with an adorable diaper hat to keep her warm.
We were very lucky to be discharged on day four, with plans for many more doctor appointments and needing her weight checked by our GP every two days. She struggled staying awake with feedings, so we supplemented with formula or pumped milk after every nursing session. We were then in the waiting game to see when her first intervention would be, but they suspected it would be a pulmonary artery (PA) band at a few weeks old due to her high oxygen saturations levels, which meant that there was most likely too much pressure to her lungs.
At two and a half weeks old, Ada had another catheter procedure for diagnostic purposes and to open the hole between her atria a bit more, as it was “floppy.” She was inconsolable before the procedure as she wasn’t allowed to eat anything, which was really hard to deny a newborn baby (especially when we kept getting pushed to later in the day due to emergent patients). Finally, she was brought in to the cath lab. We were told afterwards that there was a brief arrythmia during the procedure but that they got it under control without incident. She stayed overnight in the CICU for observation, but we were discharged the next day.
A few days later, we had another round of scans and a pre-surgery consultation. This is when we were able to speak to her surgeon, Dr. Gandhi. We were so impressed with his professionalism, knowledge, and confidence, and we knew Ada was in good hands.
On Wednesday, October 2, 2019, at three weeks old, Ada had her first open-heart surgery for a PA band to reduce the pressure on her lungs. When Ada was taken away from us in the surgical waiting area by a nurse, she said, “Let’s make a warrior out of you,” and it really hit me how amazing the entire specialty of pediatric cardiology is and also how incredibly resilient and strong Ada is. Placing a PA band is both a scientific procedure and an art form to get the tightness just right, and Dr. Gandhi did an amazing job and completed her surgery in about two hours.
Ada’s little body had a hard time adjusting to the new pressures her first night in the CICU after her surgery, and I remember a team of people swooping in and lights turning on at 2:00 am while I was in the middle of pumping. Ada’s breathing rate was dropping to very low levels, and they couldn’t figure out if she was “snowed under” because of all the pain meds or if it was her heart. They ended up deciding to intervene, and she was put back on some oxygen. After a couple scans, they determined that her heart was struggling to adjust to the new physiology, so to help her manage this, they started giving her some drugs (dopamine and milrinone) that helped her heart beat stronger. She was also placed on a mini hot air bed that helped with her body temperature. She started to feel a lot better the next day, and I remember Dr. Gandhi calling in for rounds even though it was his day off, which is when we knew how much he cared.
The next few days were a waiting game in the CICU while they tried to wean her off her medicines and kept chasing a seemingly elusive blood pressure. Five days after her surgery, we were moved up to the ward. Two days after that, after scans, tests, and wound care/CHD teaching from the nurse practitioner, we were able to go home!
Everything was able to be “normal” for a few months as we settled into a routine and waited out the months until we knew she would need her next open-heart surgery. Ada grew into a happy and goofy little baby who loved independent play and being silly. We even got to go the 2019 CHN Christmas party when she was three months old. It was so great seeing so many families and children who were in similar situations to our own. We were also able to travel to Calgary to visit family for Christmas, where she got to meet her cousin for the first time.
March 2020 rolled around with Ada’s half birthday (six months old). We had a celebration at a local community centre where we were able to introduce her to a lot of friends who hadn’t met her yet. The following weekend, the world shut down, and COVID lockdown began.
One Friday in early April, Ada had a breath-holding/cyanotic spell, and we called an ambulance to take her to BCCH. Since it was so early in the pandemic, it was a very strange experience, because a lot of policies were changing by the day and/or were not well established. My husband had to wait in the car in the parking lot while I was in the ER triage room since only one parent was allowed in. They decided to keep Ada overnight for observation and so they could do a series of tests the next day. She was also swabbed for the major respiratory viruses (including COVID-19) and thankfully came back negative for all of those. We saw Dr. Gandhi and Ada’s cardiologist, Dr. Sanatani, and they decided based on her scans and her lower blood oxygen levels they would move up her surgery by a month to the following Monday.
Just shy of her seven-month birthday, Ada went in for her second open-heart surgery—the Glenn—at 7:45 am on Monday, April 6, 2020. Even though this was our second time doing it in less than a year, and knowing how skilled Dr. Gandhi is, it was still very hard to hand her over. We ended up watching a movie on my husband’s phone in the room we were waiting in (no coffee shops or family spaces to hang out in due to COVID), because we couldn’t focus on anything else. At 10:30 am we got the word that she was through surgery. We were able to see her in the CICU an hour later. It was so hard seeing her with all of the wires and tubes again. We were moved to the ward the next day and then able to go home a couple days later. It was hard being in the CICU and ward so early in the pandemic, because everything was really shut down and we were basically stuck in the room with nowhere else to go, but the staff were really lovely and we were so lucky to have such a short stay as well.
Since her Glenn surgery, Ada has grown into a creative, smart, chatty, silly three-year-old who loves playing and attends daycare full-time. She has done swim lessons and soccer lessons and loved them both. She’s a feisty toddler who loves books, colouring, and everything Mickey and Minnie related. We have been able to enjoy life with her without worrying for a while, other than when she gets sick. She became an amazing big sister in December 2021 and is the sweetest little helper. We know that the Fontan surgery is looming over us for when she is around four years old, but until then we enjoy every day with her, and it’s easy to forget she has a “special heart.” ♥
“Ada’s Story” is from our winter 2023 newsletter, Heart Matters. See our Newsletters page for more stories and to subscribe.