Our story with a bilateral congenital diaphragmatic hernia (CDH)

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When I became pregnant with our daughter last year, it was after one healthy pregnancy (our 2-year-old son) and three miscarriages. A few weeks into this pregnancy, some blood work showed a possible cause of the miscarriages and I started taking medicine for it. We made it past the usual time I’d miscarried previously, and we thought the fear for this pregnancy was behind us.

However, after our 20 week anatomy scan, we learned our daughter had a bilateral congenital diaphragmatic hernia, a birth defect in which the diaphragm (the muscle separating the chest from the abdomen) does not form properly. As a result, abdominal organs move into the chest and inhibit the growth and development of the lungs. We learned the majority of diaphragmatic hernias are left-sided, some are right-sided and very few are bilateral. An MRI showed her stomach and liver were both high up in the chest and her lungs were two tiny slivers.

We met with a pediatric surgeon who promised to be honest with us and then as gently, but matter-of-factly as possible, told us what steps would be taken after her birth to try to help her, each step prefaced with if she makes it that far. Later, we met with a neonatologist who reiterated what the surgeon had said, adding that she might not even make it out of the delivery room.

My husband and I drove home in stunned silence. As time passed, we thought a lot about our wishes for our daughter. We planned to repeat the MRI at 32 weeks, and then, if the lungs were still so underdeveloped, we wanted just to hold her after her birth, tell her we loved her and let her go as peacefully and comfortably as possible. We read books on neonatal loss and we asked our family not to send any baby gifts. I found a wonderful organization that turned my wedding dress into an “angel gown” for her to wear if/when she passed away.

Then, at 25 weeks, we got a surprising call from Jayme, our nurse coordinator at the Fetal Center. She said there was an experimental in-utero procedure being done at Texas Children’s for congenital diaphragmatic hernias. The procedure was called fetal endoscopic tracheal occlusion (FETO). In this procedure, a balloon is inserted into the baby’s trachea, preventing fluid normally produced by the lungs from escaping. The fluid then accumulates in the lungs, hopefully stimulating their growth. As an experimental procedure, at the time it was only available for severe left-sided hernias. However, the team of physicians involved had reviewed our chart and decided to try it for us off-protocol as “compassionate treatment.”

We were ecstatic to have something that might improve her chances after delivery. Three weeks later, the balloon was placed in our baby’s trachea in an operating room full of doctors. My OB/GYN, Dr. Longerot, was there to hold my hand as they prepared. The next day, my husband and I were sitting in our hospital room, thinking about what happened and still very much afraid of what might be. I’ll never forget Dr. Espinoza, one of our maternal-fetal medicine (MFM) doctors, stopping by and saying, “We believe in miracles here.”

After the procedure, I moved into an apartment near the hospital and returned twice a week for ultrasounds and monitoring. The MFM doctors were hopeful each week, telling me the lungs did appear to be growing, but I was afraid to believe them. Six weeks later, an MRI showed the lungs had grown. We returned to the OR a few days later, and the balloon was removed. They told me that while we knew the lungs had grown, we did not know if they would work until after she was born. And so, we waited.

On Jan. 8, 2017, Lydia was born. The neonatal response team was there to intubate her immediately. Then, they gently picked her up, shuffled to my bedside, and handed her to me for a moment before taking her to the neonatal intensive care unit (NICU). One of the doctors took my phone and snapped dozens of photos in quick succession. I cannot put into words what those photos, and that moment, mean to us.

Lydia spent the next 80 days in the Level 4 NICU. The Fetal Center had given Lydia a chance, and the NICU was running with it. Dr. Olutoye repaired her diaphragm when she was 3 days old, putting her abdominal organs back where they were supposed to be and giving her lungs room to grow. She remained intubated for two-and-a-half weeks, then was on continuous positive airway pressure (CPAP) for the next six weeks, then finally discharged home with a nasal cannula and a nasojejunal (NJ) tube. We paraded through the NICU and said our goodbyes while holding back tears of joy and disbelief.

IMG_2015We think about the staff in the Fetal Center and the NICU often. I wonder if they realize just how much they mean to us. There were times during our pregnancy that if someone had told me we were the only family being seen at the Fetal Center, I would have believed them. I cried in my OB/GYN’s office once during a routine prenatal appointment. I told her I was worried I’d go into labor in the middle of the night or on the weekend and she wouldn’t be on-call. She promised me she’d be there anyway. Months later, when my water broke in the middle of the night on a Sunday, and she wasn’t on-call, she was there. When I texted Dr. Belfort and Dr. Shamshirsaz on our way to the hospital at 3 a.m., they both texted back within a minute. The neonatologists, pulmonologists and cardiologists welcomed us into the NICU, including us in rounds, answering our questions and asking our thoughts. Then there were the NICU nurses who spent hours caring for our daughter, and caring for me, while I sat at her bedside day after long, hard, lonely day.

After too many ups-and-downs to count, we have a beautiful 3-month-old baby at home, and our son gets to be a big brother. We are so grateful for all the people at Texas Children’s Pavilion for Women who not only believe in miracles, but find ways to make them happen.

The Bad Pants Open supports the needs of premature and sick newborn babies and their families at Texas Children’s Hospital – join us for this special event and celebrate 20 years of The Bad Pants Open!

Posted in Fetal Center, Guest Post, Neonatology, NICU

One Response to Our story with a bilateral congenital diaphragmatic hernia (CDH)

  1. BOBBIE CARPENTER says:

    What a beautiful story. What beautiful babies you have. Yes I too believe in miracles . I am Joe & Laura’s neighbor’s & friends. You really know how to make them smile. THERE GRAND BABIES. congratulation.

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