Our heart journey started with a simple routine ultrasound. In November 2013 at 19 weeks pregnant, we had an appointment for a regular detailed ultrasound, but all I could think about the fact that we were about to find out if we were having a boy or a girl. I had made plans for a gender revel party for later that evening, and was giddy with excitement to find out what we were having. I was surprised at how long the appointment took, and looking back, I definitely felt like the ultrasound technician took forever, especially on the baby’s heart. We were brought into a private room, and told to wait for the radiologist that would be with us shortly. I assumed that he would just come in and tell us that everything a-okay, and send us on our merry way with gender results in hand. The radiologist walked in and stated that there was a problem with the babies heart, and that we needed to come back the very next week for fetal echocardiogram done by a pediatric cardiologist. My husband and I were in shock. We were two healthy individuals with no medical issues, and had no trouble conceiving.
We left the office without saying anything to each other as we each digested the news. We cancelled the gender party, and once we were ready, we opened the envelope to revel that we were having a baby GIRL. The following week, it was confirmed at the fetal echocardiogram, that our baby would be born with Pulmonary Atresia with a VSD.
My pregnancy continued without any further complications, and weekly ultrasounds and obstetrician appointments became my new normal. As the pregnancy progressed, they started to suspects few other things with the heart, but we would have to wait until she was born before we would have a concrete diagnosis.
At 36 weeks pregnant I relocated to Edmonton, Alberta and moved into the Ronald McDonald House. Though we were from Calgary, home to it’s own children’s hospital – only two hospitals in Canada could deal with the complexity of our baby’s heart, and pediatric cardiology was not something that our local children’s hospital could deal with.
After being induced at 39 weeks, Evanna Verena Irvine was born via emergency c-section on April 29, 2014. She was taken by ambulance to the Stollery Children’s Hospital with my husband following, while I stayed behind, healing from my own surgery. The few days following Evanna’s birth were a bit a of a blur. They did a CT and ECHO on Evanna, and confirmed their suspensions that Evanna’s heart was much more complicated then initially thought. She was officially diagnosed with Tetralogy of Fallot, with Pulmonary Atresia, VSD, ASD and MAPCAs. Due to the MAPCAs, we were told surgery would have to be put on hold, and if they didn’t grow, Evanna would be considered terminal. Her heart is so complex, that it’s actually intertwined with her lungs, making it impossible for even a transplant to be an option.
Originally, I had prepared myself for a surgery to be done within the first few weeks of Evanna’s life, followed by a couple of months in the hospital before we got to bring her home. Now we were being flown back to Calgary, were we spent just one week in the NICU and discharged home.
We spent 5 amazing months at home where Evanna grew, thrived and did extremely well. We also confirmed that a mircodeletion on her 22 chromosome called 22q11 (also known as DiGeorge Syndrome) is what caused her heart defect, among a few other minor issues. Considering her very complex heart and DiGeorge Syndrome (it’s the second most common chromosomal defect next to Down Syndrome), our medical team was constantly amazed at Evanna’s progress and growth.
On October 6, 2014 we handed Evanna over into the surgical teams arms, as she underwent her first Open Heart Surgery. The surgery went well, but we experienced many bumps and major detours on our journey home. Our 3-4 weeks in hospital had turned into almost 11 months in the PCICU, 1 code blue, 6 additional surgeries – including 2 more open heart surgeries, 7 ICU transfers, multiple blood transfusions, drug addiction, and 6 failed extubations – as our biggest issue was taking the breathing tube out. Evanna has developed Tracheomalacia (weakened trachea), and so she requires extra pressurized air to keep her airway and lungs open, and had to be supported by a ventilator for over 4 months would eventually be trached.
Around this time last year I finally grasped that we were going to be in this for the long haul (we had already been in the PCICU for over 4 months) – and once we realized that, we settled into a routine, and ICU life almost became our new normal. I remained at Evanna’s bedside day in and day out, while my husband continued to commute between two different cities on a weekly basis. After a few investigations, a second open heart surgery presented to my husband and I to insert a tiny mechanical valve, and we again signed our names on the dotted line. The procedure went very well, and in fact they decided to transfer us back to our home children’s hospital just a few weeks later to continue the recovery. Unfortunately disaster struck, and not even 24 hours after the transfer Evanna began to take a turn for the worst, and not a single antibiotic seemed to help. They finally discovered that the mechanical valve that they had just inserted had clotted in an almost closed position and they had to act fast. Regular ICU transfer was deemed too slow, so they airlifted Evanna by STARS Air Ambulance (a medical helicopter) back to the Stollery Children’s Hospital, and started Evanna on a TPA infusion (TPA is essentially a “clot buster”).
However, due to the mechanical valve clotting multiple times between February – April, the medical team decided to replace that mechanical valve with a bovine one, and a third open heart surgery was booked. During this time, we also came to the conscious that Evanna was not going to extubate successfully, and we finally agreed to proceed with a tracheostomy. It was one of the hardest decisions that we’ve had to make thus far, but after being intubated for over 5 months, we knew there was no other option and that it was time to face our new reality. Following these 2 surgeries, the team again decided to transfer us back home in time for Evanna’s 1st birthday.
A week after her 1st birthday – on Mother’s Day no less, Evanna unfortunately had to be airlifted back up to Stollery Children’s Hospital in Edmonton yet again after coming down with an infection that was causing havoc on her little body. Thankfully with the help of antibiotics (and being chemically paralyzed), Evanna began to recover a few days later on her own. The intensivists (ICU physicians) decided that instead of transferring her back to our home children’s hospital, that maybe they should keep her a little longer at the Stollery up in Edmonton. So that’s exactly what we did. While we couldn’t stay there until final discharge due to logistical issues and getting homecare setup now that she had a trach, we decided to catch our breaths and not transfer back to Calgary right away. Finally at the end of June, it was decided that a transfer out of ICU was around the corner, so it was safe enough to leave the Stollery and go “home”.
We arrived safely back “home” on June 29, but something was off with Evanna. We originally thought it was because of the transfer (these can be very stressful and strenuous on patients), but things continued to spiral out of control (we began to joke that she hated Calgary and this was her way of trying to convince mommy & daddy that we needed to move to Edmonton).
July 1st is a day I’ll never forget for the rest of my life. We were just getting ready to leave for the hospital, when we received a phone call from our intensivist telling us they needed us come immediately. While I knew things were bad and was anxious the whole drive there, nothing could have prepared me for how that day would unravel. We walked onto the unit and saw emergency carts piled up in front of Evanna’s room (this is never a good sign in the ICU). We were met by the intensivist and the cardiologist who was on call, and they both asked if we could talk somewhere private, and were escorted us into a private room off to the side. They told us that Evanna was in Septic Shock – which can fatal for even healthy individuals, and that things were not looking good. They brought up ECMO (it’s life support that essentially can act as the body’s heart and lung to allow the body to rest), but due to Evanna’s anatomy, it wasn’t an option for her here in Calgary, and they seriously doubted she would survive the transfer back up to Edmonton to put her on it, and whether even ECMO would be a benefit to her in this particular situation. We were basically told that we needed to make a decision on whether we wanted to transfer her back up to Edmonton with her most likely dying in our intensivist’s arms along the way, or here in the comfort of ours. We were both in shock and devastated. After everything we had previously gone through – we couldn’t believe it had gotten to this point and we were going to lose our baby girl. We ultimately decided to not go through with the transfer; however, we asked that they continue to do everything they could for her here in Calgary. So they set to work, got more lines/access into Evanna, and pumped every “antibiotic under the sun” and blood product they could into her little body, and supported her the best way they could to help her overcome this. We called our family (it also happened to be Canada Day – so almost everyone was off work anyways), and asked them to make their way to the hospital, and even my grandparents came drove in from out of town in order to say their goodbyes. We turned to social media and pleaded for prayers, and we were absolutely flooded with support, love and prayers. Hours past, and with each passing hour, Evanna was starting to improve and by the afternoon, she seemed to finally turn a corner. Before leaving that day, our intensivist walked in and stated that she was thrilled that the day wasn’t going to end like she thought was going too, and hoped that Evanna would continue to improve – and with a bit of patience and time, she did just that. Eight weeks later, we finally were transferred to the floor and discharge suddenly seemed obtainable.
After 400 days – 323 of which were consecutively spent in ICU, we finally got to bring our little girl home on November 9, 2015. Other than a quick admission in mid-January for a planned GTube surgery, we’ve been able to stay home, where Evanna is growing and thriving beautifully. Our road to recovery is far from over, and it will take years of intense OT and PT to overcome the intense trauma she has endured. Much of our life had been put on hold, but we’re finding our feet and even talking about adding to our family in the near future (like in most cases, my husband and I were tested, and it was determined that Evann’s heart defect was not genetic). While we remain optimistic that her respiratory issues will subside, we still face so many unknowns in regards to future complications and even a life expectancy. Surgery is not considered a cure for congenital heart defects, and it’s a lifelong aliment that requires constant monitoring by a cardiologist.
Isn’t Evanna just the cutest?! I know many of you have been praying for this sweet baby girl through their heart journey. Kierra, you amaze me. You guys have been through so much and I love your cute family! You inspire so many, many people. Thank you for sharing your story with us today!
To follow Evanna’s story on Instagram click HERE.
And the Irvine Family Blog HERE.