Two days after our appointment at GBMC and 19 weeks along, we were headed on a two-hour journey to one of the best hospitals in the country for birth defects, the Children’s Hospital of Philadelphia (CHOP). We were referred to their Center for Fetal Diagnosis and Treatment (CFDT) and they had scheduled a comprehensive evaluation. The fetal consultation consisted of the following:
* A high resolution ultrasound, which lasted two and a half hours, was performed to confirm the diagnosis and the side of the hernia, identify any additional malformations, determine lung size, and calculate a lung-to-head ratio (LHR) and an observed over expected ratio (O/E). The ultrasound also determined the position of the liver.
* A fetal echocardiogram lasted one hour and determined if there were any structural defects of our baby's heart.
* A one hour fetal MRI that provided further anatomy detail regarding the liver position.
* A meeting with their genetic counselor who reviewed our family history and prenatal genetic testing.
Once the diagnostic exams were complete, we met with a high-risk maternal-fetal medicine specialist (MFM), a pediatric surgeon and our nurse coordinator who reviewed the results of all exams and confirmed our baby boy’s diagnosis. We discussed all treatment options and prenatal and postnatal care, as well as their delivery recommendations. They answered our questions and provided us with additional educational information about CDH to take home.
While at CHOP, they determined the severalty of our baby’s CDH, by evaluating major factors such as LHR, observed over expected ratio, and liver position. Not only were his ratios lower than we had hoped, but the tips of his stomach, bowel, and liver had also herniated and moved up in his chest, indicating a more severe form of CDH. Two things that proved favorable for him were his size since he was measuring six days bigger and his amniotic fluid being within normal limits. In order to ensure there were no chromosomal anomalies in additional to the CDH, an amniocentesis was recommended, but unfortunately that carries its own risks and we had to decide if would pursue that farther. They stressed that although they could give a prognosis on the severity of the CDH, until he is born, there isn’t a test to perform during pregnancy that could predict his lung function. Our baby boy’s lung’s function would only be determined after he was born. Overall, in their opinion, our baby was not the worst or best case they had ever seen, but he fell in the middle range and they predicted a 70% chance of survival.
The ride from Philadelphia seemed like forever and yet our day didn’t seem so bad when we arrived home and were again immediately greeted with the biggest smile and hug from Brody. Our heads were spinning with all of the information we had received and we were now faced with one additional decision and that was if we decided to continue the pregnancy, would it be practical for our family to deliver at CHOP. Delivering at CHOP meant I would be relocated to Philadelphia at 35 weeks and we would remain at the hospital for weeks even months depending on his recovery. His progress would be tracked in the days following his birth and they would perform a surgical repair of his CDH, as early as three days of life. This would entail guiding the organs in his chest back down into the abdomen and repairing the hole in his diaphragm. In addition to the surgical repair, he could require specialized equipment such as an oscillator ventilator, heart lung machine (ECMO) or nitric oxide. His length of stay in the NICU would widely depend on his prognosis. Although CHOP is one of the best hospitals in the world, we knew that being away from our family and friends, when we needed the support most, would be incredibly difficult for everyone, especially Brody.
We discussed our options and believed that meeting with the team at Johns Hopkins Hospital, another amazing hospital, would be our next step.
* A high resolution ultrasound, which lasted two and a half hours, was performed to confirm the diagnosis and the side of the hernia, identify any additional malformations, determine lung size, and calculate a lung-to-head ratio (LHR) and an observed over expected ratio (O/E). The ultrasound also determined the position of the liver.
* A fetal echocardiogram lasted one hour and determined if there were any structural defects of our baby's heart.
* A one hour fetal MRI that provided further anatomy detail regarding the liver position.
* A meeting with their genetic counselor who reviewed our family history and prenatal genetic testing.
Once the diagnostic exams were complete, we met with a high-risk maternal-fetal medicine specialist (MFM), a pediatric surgeon and our nurse coordinator who reviewed the results of all exams and confirmed our baby boy’s diagnosis. We discussed all treatment options and prenatal and postnatal care, as well as their delivery recommendations. They answered our questions and provided us with additional educational information about CDH to take home.
While at CHOP, they determined the severalty of our baby’s CDH, by evaluating major factors such as LHR, observed over expected ratio, and liver position. Not only were his ratios lower than we had hoped, but the tips of his stomach, bowel, and liver had also herniated and moved up in his chest, indicating a more severe form of CDH. Two things that proved favorable for him were his size since he was measuring six days bigger and his amniotic fluid being within normal limits. In order to ensure there were no chromosomal anomalies in additional to the CDH, an amniocentesis was recommended, but unfortunately that carries its own risks and we had to decide if would pursue that farther. They stressed that although they could give a prognosis on the severity of the CDH, until he is born, there isn’t a test to perform during pregnancy that could predict his lung function. Our baby boy’s lung’s function would only be determined after he was born. Overall, in their opinion, our baby was not the worst or best case they had ever seen, but he fell in the middle range and they predicted a 70% chance of survival.
The ride from Philadelphia seemed like forever and yet our day didn’t seem so bad when we arrived home and were again immediately greeted with the biggest smile and hug from Brody. Our heads were spinning with all of the information we had received and we were now faced with one additional decision and that was if we decided to continue the pregnancy, would it be practical for our family to deliver at CHOP. Delivering at CHOP meant I would be relocated to Philadelphia at 35 weeks and we would remain at the hospital for weeks even months depending on his recovery. His progress would be tracked in the days following his birth and they would perform a surgical repair of his CDH, as early as three days of life. This would entail guiding the organs in his chest back down into the abdomen and repairing the hole in his diaphragm. In addition to the surgical repair, he could require specialized equipment such as an oscillator ventilator, heart lung machine (ECMO) or nitric oxide. His length of stay in the NICU would widely depend on his prognosis. Although CHOP is one of the best hospitals in the world, we knew that being away from our family and friends, when we needed the support most, would be incredibly difficult for everyone, especially Brody.
We discussed our options and believed that meeting with the team at Johns Hopkins Hospital, another amazing hospital, would be our next step.
