Congenital Diaphragmatic Hernia: My Granddaughter's Experience After Birth

Congenital Diaphragmatic Hernia occurs, according to the statistics, once in every 2500 births. Until we found out during a routine ultrasound during my daughter's pregnancy that this condition was present, we had never heard of CDH. Many parents every day learn that they have to face this with their own babies, and like us, feel uninformed, unprepared, and terrified. I have written a previous article on AC about CDH, and what we have learned of this birth defect. This is the second half of the story, what happened during and after our granddaughter's delivery.

My daughter and son-in-law went for their routine nineteen week ultrasound with one goal in mind, to hopefully find out the sex of their expected baby. Their excitement over finding out they were expecting a daughter quickly turned to terror when the technician said she thought there was a problem. After a second ultrasound at a high risk obstetrician's office, they found out their baby had CDH, or Congenital Diaphragmatic Hernia, which means when the baby's diaphragm was forming, it didn't close completely. We later found out, after delivery, that this had allowed most of her intestines, her stomach, spleen, and a small corner of her liver to move up into the chest cavity and impede her lung development.

We had heard that if the liver "is up," the outcome is worse than with "liver down" because the liver is such a large organ, but later found out this is not a reliable indicator. Nor was head to chest ratio a very reliable indicator. As Elizabeth, founder of the Breath of Hope support group for CDH mothers has repeatedly said, "It's all about lung function." Each baby has to breathe after birth, and how well those little lungs are able to function is really the determining factor in how well the baby will survive. Our granddaughter's liver appeared to be "down" on the subsequent ultrasounds, but we were always told it could move up, or it could be up already because it was just very difficult to say for sure.

My daughter had a mild problem with excess amniotic fluid, as some CDH moms do, and had a lot of swelling of her feet, ankles, legs, and hands. At eight months of pregnancy, she was told she had pre-eclampsia, and was admitted immediately to the hospital to be induced. A cervical balloon was inserted at around 2:00 PM, and a few hours later her water broke on it's own and she was started on Pitocin. By 9:45 PM, she was fully dilated and ready to push. After twenty-five minutes, our baby Claire was born into the world, with a room full of people waiting, the doctor, four nurses, a neonatologist, three nurses from the NICU, daddy, and me -- the grandmother. (And of course, mom!) Claire weighed five pounds and eight ounces.

We had been told that many of these babies do not cry at birth, but Claire immediately cried loudly, so loudly in fact that my husband who was waiting in the hallway said he could hear her and so could the nurses halfway down the hallway at the nurse's station. A tube was put into her stomach immediately to keep her from swallowing air and distending her tummy. The doctors all stood ready to intubate her, but with sheer delight on all of our faces, she began to turn a healthy pink color on her own. She seemed to be breathing heavily, but on her own, and the doctor's decided not to intubate her at that time. They even wrapped her and let Mom hold her for a brief time, before whisking her away to the NICU.

She was born at UAB in Birmingham, Alabama, and we were told before the birth that she would probably be transferred immediately from UAB's NICU to Children's Hospital, which was a few blocks down the road. They took her to the NICU to get her stablized, and then let mom, dad, Grandpa, and I go to see her. When we went into the room to see her, an hour had passed since the time of her birth, and she was hyperventilating. Her little chest was heaving rapidly and she seemed to be struggling. They said they needed to intubate her and quickly transport her to Children's. We were all upset to see her struggling for air, and quickly commanded, "Then DO IT!" She was intubated and transported to Children's and put into the NICU there.

My daughter was taken back to her room at UAB, and because of her blood pressure problems before the delivery from the pre-eclampsia, she was not released for 36 hours to see her baby, when she was then given a pass to leave the hospital for a few hours. Dad, Grandpa and I went to see her after they had time to get her over there and stabilized. She was put into what we later learned was "ECMO" row, the back row they put babies who are expected to need ECMO, an artificial heart/lung bypass machine that allows the lungs to rest until they can develop more. This area of the NICU is quieter and there is less to distract the babies or affect their blood gas readings.

Claire was a trooper and did not need ECMO. By the next day, she was moved out into the main part of the NICU, and we could get a better look at her. She had a head full of dark hair, and was lying in a diaper in a heated bed. She had an oximeter to measure the oxygen levels in her blood, a gold heart-shaped metal patch taped to her chest to measure her temperature, and monitors to measure her heartbeat. She had the intubation tube in her throat and taped to her mouth and also the tube going into her stomach to keep it emptied. She also had a "PICC line" inserted into her scalp for nutrition. She had an IV in her hand to give medicines. They kept her hooked to monitors that constantly beeped and if her oxygen level dropped below a certain level a red light over her crib would flash and an alarm would sound. There were about twenty-five babies in the NICU at the time, so there were constant alarms going off, enough that my son-in-law said it sounded like a casino.

She progressed so well that they decided to do her hernia repair surgery on the sixth day. It was found she had a very thin membrane of tissue to make up her diaphragm, but there was enough muscle tissue that they could connect that she didn't need a gortex patch, as many babies do. X-rays showed her heart had been pushed slightly to the right because of the lack of room in the chest cavity, so her right lung was slightly compressed, and her left lung was about a third the size it should have been and collapsed. Everything was successfully moved back down from her chest cavity into her abdomen and it was all done laporoscopically.

She continued to stay in the NICU for two more weeks, and a "step-down" NICU (for babies who need less intensive intervention but still need round the clock care) for another week after that. She was then moved to a regular room where immediate family could visit without time constraints. At one point while she was in the first NICU, her blood gases suddenly started going bad, and it was discovered her intubation tube was totally clogged with mucus at the bottom, had to be removed, and replaced with a new one. She immediately did better, but we all had a scare there for a while. She was taken off the respirator after ten days and had a nasal cannula after that. After many days of nutrition through her IV, she was given breast milk in a bottle, which she did well with. My daughter pumped and stored milk in the freezers at the hospital. My daughter tried to breastfeed her but the baby had a hard time latching on and would become frustrated, so when she was finally released to go home my daughter was still pumping and giving her bottles of breast milk. The baby was also sent home with the nasal cannula and oxygen tanks, and an oximeter strapped to her foot to measure her oxygen levels.

She is now three months old. She finally caught onto breastfeeding a few weeks ago so my daughter can now just nurse without the pumping and bottles. She has steadily gained weight. She is off all oxygen and does not need any special equipment or monitoring. The only medicine she is taking is Zantac for reflux. Other than three tiny pink inch-long scars on her tummy from the surgery, you can't tell her from any other normal baby. She plays, she sucks her thumb, she coos, she smiles, and she's gorgeous! She sees her pediatric surgeon regularly to check to make sure the hernia repair is healing fine, sees a pulmonary specialist to check on her lung development, and sees her regular pediatrician. Subsequent x-rays show her heart is shifting back into normal position, her right lung looks normal, and her left lung is growing and filling up the left side of her chest cavity. They say she is "moving air well." Our biggest concern right now is the cold and flu season. Babies and children are very susceptible to RSV, a virus that can be very hard on their lungs, and our granddaughter, because of having this higher risk of complications should she get that, will receive monthly immunizations to prevent that. She will have to be kept somewhat isolated from other children or people who may have colds, etc. Only a limited number of people are allowed to hold her at this point.

I want anyone who is facing CDH with their baby to know that not all babies die, not all babies have serious complications and not all babies need long-term feeding tubes, etc. Some babies, depending on their lung function, do very well. There is hope. Through a lot of tears, worry, and prayer, our granddaughter is thriving.