“Like mother like daughter. Since Libbey was born, we struggled to get her weight up. Small but mighty, she finally cracked the growth chart at 2 years of age, but it remained a daily struggle. It never occurred to us that her growth issues could be related to the same congenital heart defect I had as a child. We discovered it by accident, as many people do, when a chest x-ray during a stubborn cold revealed a significantly enlarged heart. Our pediatrician immediately referred us to a cardiologist. Having been in and out of doctors’ offices for 2 ½ years, no alarms bells when off. I walked into that afternoon appointment with Libbey feeling like it was just another doctor’s visit to check off. “Just to be safe.” One EKG and echocardiogram later, the cardiologist looked at me and said “well, like mother like daughter.” Libbey was diagnosed with three CHDs: a large atrial septal defect, mitral valve prolapse, and pulmonary valve stenosis. I blinked.
And that was the beginning of what became a sprint to find the best surgeon… figure out how serious it was… second guess our pediatrician… conduct follow-ups with the nephrologist to make sure her kidney was functioning… get calendars in order… wake up every morning at 5am in a pile of nerves… make a LOT of decisions about something we knew nothing about during a period of enormous stress.
Hard decisions. One decision we had to make early on was whether to try a device closure via catheter, or move straight to open heart surgery. This threw me for a loop. Why was such a pivotal decision up to us? Shouldn’t the medical professionals know the best course of treatment? I dug through research journals to familiarize myself with the factors associated with either option, and the short- and long-term risks associated with each. Libbey seemed too small, her hole seemed too big, and the long-term risks of erosion associated with device closures—while relatively small—were scary to us. In the end, with confidence in her surgeon and support from our cardiologist, we chose to move directly to open heart surgery. It felt surreal that our 2 year old daughter was about to undergo the same open heart surgery I had experienced 38 years ago…
Surgery day. We left our house at the crack of dawn. The summer sun peeking up over the hills was as effervescent as Libbey’s smile – a beautiful, amazing smile she would retain through a grueling week. After saying goodbye to the bunnies outside our house, Libbey sang all the way to Boston Children’s Hospital. She then spent all of pre-op driving around in a kiddie car, scooting around doctors and nurses and filling the tank with gas every 5 minutes. I made silly mental notes, like the OR nurse seems way cooler than us, and the anesthesiologist most definitely doubles as the Headspace narrator. By the time they wheeled Libbey back to the OR, she was drifting off to sleep in front of Peppa Pig, and Dave and I settled in for the 4-6 hour wait. Weeks of anxiety and sleepless nights seemed to have prepared me for this moment – I was oddly calm, relieved to have it underway… Dave, on the other hand, broke down in tears.
Libbey was in surgery for 5 hours. She was young enough to undergo a mini-sternotomy – a 3-4 inch incision rather than the more common 8-10” incision — which required Dr. Emani, her surgeon, to use specialized instruments to project the dexterity of his hands in performing the operation (just one of several medical interventions available to us at this top-ranked pediatric heart center). Through this small incision, Dr. Emani patched a 20mm oval-shaped hole between the two upper chambers of her heart using a piece of her own pericardial tissue. Since the remodeled heart and corrected blood flow would hopefully mitigate both the MV regurgitation and pulmonary valve stenosis, he left those two valves alone.
After surgery, Libbey remained asleep for six long hours. The CICU staff dubbed her “sleeping beauty” – they wouldn’t extubate her until she was conscious enough to maintain independent breaths. When she finally woke up that evening, her first raspy words were “I want my brothers!” Dr. Emani came by and noted that although the hole was much larger than anticipated, everything had gone smoothly.
Libbey spent four days in the hospital. Within 48 hours of surgery, she was running through the grass in the rooftop garden, which was mind-blowing. Words cannot describe our gratitude and appreciation for each and every individual who cared for her during this time. Their faces and names are seared in my memory—the CICU nurses (Kat, Jenna, Jennie, and Ben) who took incredible care of Libbey in those first 24 hours; the step-down floor team (Marcia, Anne, Micayla, Steph, and Meena) who laughed and cajoled Libbey into taking her medicines; and the doctor team (Avinash Shukla – anesthesiologist/Headspace narrator, Abbas Zaidi – cardiologist, and Ram Emani – surgeon/BFF) who treated her as if she was the most important person in the world, even though she was probably their easiest case of the week. They held her heart in their hands and transformed her life forever. To me, they became family. We went home filled with gratitude.
“I’ve seen this get better, but in this case…” Libbey’s ASD was fully repaired and the pulmonary valve stenosis disappeared along with it. The mitral valve wasn’t so cooperative. The valve worsened… quickly. Within 9 days of surgery, the regurgitation went from trivial to moderate to severe, and her cardiologist launched an aggressive treatment to stop its progression. “I’ve seen this get better, but in this case I don’t know…” were Dr. Emani’s exact words. We held our breath for 3 more months, counting the days between echos that would tell us if the drugs were working, or if she had another, more risky surgery in her future. During this time, our oldest son was diagnosed with a bicuspid aortic valve – another congenital heart defect that will be monitored for the rest of his life. In the moment, this felt manageable in light of Libbey’s journey.
On track to good as new. Six months after surgery, Libbey’s MV regurgitation is back to trivial and she is off all medications. Equally exciting, she had gained almost five pounds and grown two shoe sizes! She is “on track to good as new,” according to her cardiologist. She continues to get regular echo’s (CHDs can be repaired but are never cured), and Gideon will have annual echocardiograms to monitor that bicuspid aortic valve. And before bedtime each night, Libbey and I compare scars – “heart twins” forever, and forever grateful.
Making a difference. We are a CHD family, with 3 of 5 of us born with a congenital heart defect. And yet I am continually reminded of our luck. Libbey and Gideon’s CHDs are treatable. We live near one of the best heart centers in the world with cardiologists and surgeons who are leading cutting edge surgical innovations and treatments around CHD, which has no cure. From advanced imaging technology that allowed Dr. Emani and her cardiologist to zero in on and track the gravity of her defects, to decisions about treatment and care like the mini-sternotomy and chest-tube removal within 24 hours (remember that run through the grass?!), we benefited from access to the best care. This is not always true for everyone, and Libbey’s case was simpler than most. I’m committed to ensuring that other families have immediate, equitable access to the comprehensive information they need to make critical decisions about their own heart warriors, so that they too can live #heartstrong.” – written by her mom, Claire. January 2020