My husband and I were not trying for our third child when we found out we were pregnant. Our first children, twins, were born 6 weeks early and required a NICU stay. With Greyson, I was picturing an easier pregnancy, delivery, and breastfeeding experience. I was picturing a normal hospital stay without the NICU, feeding tubes, and scheduled feeds. These thoughts were exciting and something I couldn’t even imagine because of my previous experience. However, after our 20 week anatomy ultrasound on March 15, 2018, I knew the experience I pictured would be extremely different from anything I had imagined.
“Something is wrong with the right side of your baby’s heart. It may not be survivable,” my OBGYN said. “Survivable,” a word that still haunts me to this day. How could this happen? I did everything right.
We were immediately scheduled for genetic testing, and would see the pediatric cardiologist 5 days later. Needless to say, the next 5 days were some of the longest in my life. My mind was racing with possibilities, and I was fearing the viability of my baby. During our first pre-natal cardiology appointment we received Greyson’s diagnosis–Pulmonary atresia with intact ventricular septum (PA/IVS) and Hypoplastic Right Heart Syndrome (HRHS). Essentially, our son would be born with only half of a functioning heart. We were told this diagnosis was considered a critical CHD and his specific case was severe. We were encouraged to expect multiple surgeries or even a heart transplant. All plans changed, and the dream of having a “normal” birth experience was gone. Now, I would be induced and there would be Cardiac Intensive Care Unit (CICU) stays, surgeries, procedures, and medications. All the things I had pictured about my pregnancy and delivery were now altered beyond recognition.
During this period, I was intensely grieving so many things: my son’s health, his future, and missing out on valuable time with my other boys to live away from home and within the walls of Seattle Children’s Hospital. It was so hard to know the most difficult challenge of my life was just around the corner, and I had no control over the situation. Needless to say–for me, the remaining portion of my pregnancy was emotionally taxing, and I felt very alone. I began having panic attacks weekly if not more often. I cried every moment I was alone–on the drive to and from work, while showering, and in our bedroom during the darkness of night.
Our heart warrior and one of the greatest loves of our lives, Greyson was born in July 2018. My last shred of hope this was all a mistake was quickly dismantled. Unfortunately, he was not miraculously cured. His pre-natal diagnosis was confirmed at birth and his “treatment” plan was decided. He would undergo multiple open heart surgeries to allow his heart to palliatively function on the left side. “Palliative”- this means not curative; only life lengthening. These procedures may allow him to live into his 30’s before requiring a heart and/or liver transplant. My son will be lucky to outlive his parents. As much as I try not to dwell on this ugly truth, the deep emotional pain associated with this knowledge knows no end. At times, it takes over my mind and I can physically feel my heart shattering. I want something to change. I need something to change.
Greyson underwent his first heart catheterization on his second day of life. During this procedure, Greyson coded and required CPR. I heard the “code Blue” alert over the intercom. I knew it was my son, and I have never felt so helpless or terrified. Thankfully, he was successfully revived and suffered very little from this event. In fact, this procedure allowed him to skip his first planned procedure, the BT shunt. It allowed him to come home 6 days later.
Greyson’s interstage period was tenuous. At six weeks of age, he was emergently admitted for SVT. During time of admission, Greyson’s heart rate was over 250 bpm. He required adenosine and an esmolol drip. Unfortunately, he continued to go into SVT for 2-3 days. Finally, we were able to control his rate with propranolol, and we were sent home. Two weeks later at eight weeks of age, he began having severe bleeding in his stools and desaturations. He was anemic and had developed a dairy protein allergy.
At three months of age, Greyson’s oxygen saturations began rapidly declining. His first open heart surgery, the “Glenn” was moved up two weeks earlier than scheduled. Greyson had his first open heart surgery, the Glenn, in November 2018. Unfortunately, he had multiple complications including: stenosis of the left pulmonary artery, narrowing the innominate vein, and partial paralysis of the right diaphragm. Two heart catheterizations and one stent later, we were finally stable enough to discharge home.
From November 2018 to June 2020, we were able to stay out of the hospital. In June 2020, it was discovered Greyson had a right ventricular thrombus. We were immediately admitted, and started on lovenox shots. Lovenox involves injections twice daily along with regular blood work. Needless to say, administering injections twice daily grew very tiring for Greyson as well as for us. Being the one causing pain to your son is incredibly dehumanizing–I was supposed to be his safe haven, and he began fearing me. Thankfully, our prayers were answered when after two weeks of doing injections at home, Greyson was accepted into a study utilizing an oral anticoagulant. He remains on this drug, but will ultimately transition to warfarin.
Greyson is expected to undergo his second open heart surgery, the Fontan around age 5. For now, Greyson is a “normal” 2 year old. He tries his best to keep up with his brothers, and he’s hitting all his important milestones. And for this, we are considered lucky. He’s funny, smart, and extremely feisty. You would never know of the battles he’s fought and won without seeing his bare chest. He amazes us everyday, and truly is our hero.
Being a heart parent has rocked me to my core. It is single-handedly the most influential and difficult transition of my life. The emotional trauma I have endured during this journey has strengthened my level of compassion, empathy, and determination. My strength and grit has surprised me, along with my new found ability to prioritize the most important aspects of life. I often think of my life as split into two parts: Pre-CHD and Post-CHD. And while I hate CHD with my whole being, I love my son with my whole heart. This incredibly rocky roller coaster is worth every moment I have him.
– Sarah Ouellette- mother of Greyson (2) w/ PAIVS, HRHS
The Heart of MFB 