There isn’t a day that passes where I don’t wonder what things may be like if I had any idea on the existence of and the severity of NEC. Would my almost-four-year-old be crawling on my lap? Would I be shopping for her on the holidays? Or would NEC have claimed her life even had I known?
Our daughter Leyden Saige Cournoyer was born on February 20th, 2014, in a planned delivery at 39 weeks. I delivered at one of the best children’s hospitals in the world. She was born to two nervous parents, in front of nineteen sets of eyes, with two known holes in her heart and a lot of plans and support in place for her care. But, there was no mention or worry of NEC.
Leyden made pregnancy and delivery easy. She made a grand entrance after just a few minutes of active pushing. Nineteen sets of eyes stared at my six-pound, 2-ounce daughter. Her father held up his phone to play her previously chosen song, Bob Marley’s “Three Little Birds” as my cardiologists, her cardiologists, the delivery team, the NICU and CICU staff, and of course, students just there to observe, declared she was healthy. We really believed, despite all of our cardiac concerns, everything really was going to be alright. We knew before Leyden was born that she had a “relatively simple” form of congenital heart disease. We were told she had a 97% chance of survival and not to worry. After brief observations at the hospital, we were sent home.
Leyden was healthy, the goal was to get her bigger and stronger, and then she would have her full heart repair. Home wasn’t exactly normal – we had visiting nurses come in, different medications to administer and since growth was the goal, I was pumping my breast milk and then fortifying it to add caloric density and to measure exactly how much she consumed. We weighed her every morning and all of her data was tracked diligently and shared with her Team. After a couple of weeks at home, Leyden hadn’t gained weight. She was refusing her bottles. She became increasingly fussy. Her diapers started to fill with black stool. We called her Team and it was decided that she would come in for the full heart repair. They reasoned that her heart was working too hard to be able to gain weight and she was uncomfortable. A week before her surgery we went in for the intake. Her doctor’s asked if there was anything new of concern. I noted that her diapers were incredibly foul-smelling and that she was pooping black. They smiled and said fortification or oil (which was also added to increase the density of my breast milk) can do that.
Two days before her scheduled date of surgery I knew something was wrong. She was inconsolably fussy, vehemently shaking her head when I would try to feed her, and incredibly gassy. I called her doctor. They had us go to the ER (it was a Saturday) where we were then admitted to the hospital to remain until her surgery. We were told it was a safety precaution just to keep an eye on her but not to worry. Monday morning came, the date of her heart repair. I was confused when they told me there might be a complication or a problem. Her nurse had discovered blood in her diaper. We didn’t know what that meant. It was a whirlwind of people in and out of the room, consults, discussions and finally, her attending surgeon told us that she might have something called “NEC.” I had no idea what he was talking about. He continued that it would be too much for her body to do the full heart repair in the event she had NEC, so they were going to do a partial repair. Essentially, rather than sew up the two holes in her heart, they were going to place a PA band to constrict blood flow. This would suffice for anywhere between a year or three and then once older and stronger, they would do the full repair. We complied.
I kissed Leyden on her forehead while sobbing, as I handed her over to the anesthesiologists. The surgery went perfectly. They were pleased. She responded well. We didn’t hear of NEC again, and they told us that in just a couple of days, we were going home. The only requirement for discharge, other than her cardiac signs continuing to be positive, was weight gain. Two weeks later, Leyden hadn’t gained weight. Because Leyden was refusing to breastfeed and wasn’t successfully taking her full bottles, they put in an NG feeding tube. Leyden began vomiting her feeds. I stopped them midway through. I was scolded. I called a Team meeting with her doctors. Her attending cardiac surgeon was too busy. Through tears, I shared my concerns with her cardiologists. Since fortifying her formula again, and adding the NG tube she was once again fussy, gassy, and vomiting. I was scared and confused. Conversations centered around whether or not this was related to her heart. Shortly after Leyden had blood in her stool again. For the second time in her life, we heard the word “NEC.” She was put on “NEC- Watch” so she couldn’t eat while they assessed what was happening.
We were told it was a milk allergy or NEC, but most likely a milk allergy since she wasn’t premature. Plus, with her previous “potential” case of NEC, it was unlikely it would revisit. They brought in GI to visit and consult on her case. They left a list and told me that it was likely NEC or a milk allergy. After 48 hours on NEC watch, they decided to take her off and came to the room- with fortified breast milk. I was confused. I requested a meeting with her attending cardiac surgeon. He was too busy, as was the NP. After missing two feeds and hours of me bouncing Leyden on my chest as she screamed, they came with a dairy-free formula. I gave her the bottle and my hungry little girl took it like a champ. Her mood improved, gas lessened and she was as happy as can be over the next couple of days.
It was Easter weekend and she spent the holiday kicking her legs in joy, taking in the sun from the garden outside, and giggling with the nurses who took turns holding her. I had spent way too much money on an Easter dress she would outgrow but decided in that moment, I didn’t care. She was happy. This was a memory. Then… another bloody diaper. GI returned. I sobbed. Her father Mike and I revisited our notes. I wrote a long letter outlining my concerns and raised the “flags” I felt like Leyden’s body was telling us. She didn’t need words to let us know, something was off. Her attending cardiac surgeon said we were not going to test the diapers anymore. I protested. It was a “distraction” he said. And that was that. He scheduled a discharge date for us stating that we could feed her and get her to grow at home. He had already performed the partial repair. It was time for us to let her get acclimated to her home environment where she might grow more easily and feel better.
I was dumbfounded on how we could go home, when at almost 3 months old, my daughter weighed only one more ounce than she did at birth. I didn’t have a medical degree, and intuition didn’t seem like enough to discredit the top hospital in the world. But when a feeding pump was sent home and staff came to our hospital room to train us on how to use it at home – medical degree or not, I refused. I apologized but let them know my daughter wasn’t healthy enough to leave. Mike and I requested a second opinion. We debated going to a different hospital, but feared leaving the best in the country. Keeping our fingers crossed for the second opinion, we wrote to the head of Cardiology. He agreed to review her case. Focused on her cardiac care, they were stumped. The reviewer of the case was actually the same surgeon who completed my open-heart surgery at BCH 16 years earlier.
My cardiologist, in semi-retirement, came to review as well. They adjusted a blood pressure medication. She responded well. But after a couple of days that wore off and she was back to sleepy, gassy, bloated, fussy, and refusing to eat. They decided she needed the full heart repair. Leyden went into that surgery on May 14, 2014. There was no mention of NEC. Surgery went well – in fact, “better than expected.” They told us we would be going home in just a couple of days. We were elated. Leyden quickly came off the ventilator and the day after her surgery we gave her her bottle. She took it like a champ! After she finished and was asleep we were relieved and elated. We could see the light.
What we couldn’t yet see was the expansion in her stomach. Leyden’s belly grew larger and larger. We didn’t know why. Still no mention of NEC. And truthfully, I still didn’t really know what NEC was. It took over 48 hours before she was admitted for an explorative surgery. They removed intestine that had died during the heart repair. Her abdomen was closed and we were told this was a “two-week delay.” Still no mention of NEC. She didn’t respond well. We didn’t know why. I could see Leyden slipping from my fingers. A second abdominal surgery ensued. No more dead intestine. But they kept her abdomen open to allow it to be “less angry.” Still no talk of NEC. She still didn’t respond well. Suddenly she was placed on ECMO. She had more machinery in her room than I could have imagined. Two full time 24-7 staff were assigned to her. She fought like hell.
Despite max dosages of morphine and sedatives, my baby girl would open her eyes, wiggle her toes and fingers, and do her best to kick every time she heard our voices. We knew she was fighting, so we did the same. We covered her room with her pictures. At this point she was unrecognizable. We needed every person treating her to see her – to see healthy Leyden. Not the sick baby who couldn’t eat, move, or be held.
We recorded hours of our voices reading stories, singing songs. I wiped breastmilk on her lips each day and placed my pillowcase next to her head. “Keep fighting,” I whispered, “we are here with you.” And she did. After 2 weeks, the maximum amount of time they allowed her to be on ECMO (for risk of brain bleed or further complications) we were told Leyden had to come off the machine. And that she wouldn’t live. June 3rd, 2014 they took her off ECMO. Our friends and family had all come to say their good-byes. Our church performed a dedication ceremony. I was in shock. It was surreal. At the conclusion of the removal, they came back to the waiting room and said “she did it. She lived.”
I ran back to her room tears of joy streaming down my face. My little fireball wasn’t going to stop. And neither was I. She transitioned successfully to dialysis. Her brain was functioning well- there were no signs of brain bleeds and she still engaged with us every chance possible. Four nurses helped lift her so I could rub her back and feel her skin. Her heart was beating beautifully. We accepted that this would be a long road to recovery, but she was going to recover. A couple of weeks later, on Father’s Day, I witnessed Leyden crying for the first time in all of these surgeries and interventions. I pressed my face against hers and cried with her.
Two days later, they discovered more dead intestine. They called a Team meeting. She had zero chance of survival. They believed she was beginning to suffer. We didn’t call everyone back in. We spent the next few hours reading to her, cuddling her, and giving every ounce of love that was in each of us. For the first time in about a month, Leyden was removed from the bedspace. They placed her in our laps and removed the machines. In our arms, we held her as she took her last breath. Her death certificate read that she died from NEC. Secondary causes: organ failure and renal failure. Her heart, spirit, and brain were fully functioning. Her cardiologist termed her case a “catastrophe.”
Every day, I can’t help but wonder, what if we had talked about NEC. What if I knew what it was and the danger of it? What if it wasn’t until it was too late that we were educated as parents, about the danger of NEC? What if GI hadn’t been a consult but had been a partner? What if the directions GI left were followed and cardio didn’t “trump” gastro? I can’t get my daughter Leyden back. I cry for her most days, and nearly four years later I still sleep with her stuffed animal most nights. It is SO painful to share and to talk about this but if it can help one other family, if it can ignite one extra conversation, it is my responsibility, as Leyden’s mom, to let her story spread awareness. And to let her fight, her spirit, and her legacy spread love, strength, and joy. Because she was every one of those qualities.
