Each year, 1 of of every 4,500 babies is both with EA/TEF. Even after their surgical repair, many of these children battle a long list of chronic issues like acid reflux, weak immune system, compromised trachea, barky cough, aspiration, and others.
January is our official awareness month. It is a chance to spread the word about this unknown condition. It is also a time to celebrate modern medicine gifting our children a life they could not have lived in another time or place.
What is EA/TEF?
EA/TEF is common terminology in the US. In the majority of the rest of the world, the terms OA or TOF are used.
Esophageal Atresia is a congenital anomaly, where babies are born with their esophagus ending in a blind pouch, not connected to their stomach. Tracheoesophageal Fistula is a congenital anomaly where babies are born with a fistula, or an extra tube, extending from their trachea to their stomach.
There are several different types of EA/TEF.
- Type A (7.7% of cases): Esophageal atresia in which both segments of the esophagus end in blind pouches. Neither segment is attached to the trachea.
- Type B (0.8%): Esophageal atresia with tracheoesophageal fistula in which the upper segment of the esophagus forms a fistula to the trachea. The lower segment of the esophagus ends in a blind pouch. This condition is very rare.
- Type C (86.5%): Esophageal atresia with tracheoesophageal fistula, in which the upper segment of the esophagus ends in a blind pouch (EA) and the lower segment of the esophagus is attached to the trachea (TEF).
- Type D (0.7%): Esophageal atresia with tracheoesophageal fistula, in which both segments of the esophagus are attached to the trachea. This is the rarest form of EA/TEF.
- Type E (also called Type H) (4.2%): Tracheoesophageal fistula in which there is no esophageal atresia because the esophagus is continuous to the stomach. Fistula is present between the esophagus and the trachea.
Approximately 50% of babies born with EA/TEF are also born with additional congenital anomalies, usually part of the cluster called VACTERL. Source
Our son James was born at 39 weeks in February, 2016. At first he seemed perfectly healthy. After a few hours we noticed that he was ‘purring.’ We asked for a doctor to examine him. She attributed the purring to his fast delivery, and said he just needed a good cry. About 8 hours after his birth, the nurses prepared for shift change. Our new nurse was uncomfortable with the ‘purring’ baby, and brought in the head respiratory NICU nurse. The NICU nurse was in our room for approximately 2 minutes before she correctly guessed that James had esophageal atresia. An x-ray quickly confirmed the diagnosis, and a new pediatrician came in to explain that our new baby would need to be life-flighted to a bigger hospital that night to undergo major surgery.
We are so thankful that James’ EA/TEF was discovered quickly. The treating physicians told us it can often take up to 3 days to discover EA, during which time a baby is not receiving any nutrients, and they often aspirate fluids and develop pneumonia.
You can read our full story here.
We are now ten months, one major surgery, and seven dilation operations later. James sounds like a barking seal when he laughs or coughs. Respiratory infections are still frightening due to his underdeveloped trachea, but he has handled them well. Feeding an EA/TEF baby is tricky. But with the help of a speech pathologist, he has even started to eat solid foods. Overall, he is doing wonderfully. We feel immensely blessed and grateful for modern medicine.
January, official EA/TEF awareness month, has been amazing. Each day, parents post photos of their EA/TEF children, along with a brief update of how they are doing. It is incredible to see so many thriving! It is also heartbreaking to see those who have lost precious children to this condition.
Current Research and Advances
Since EA/TEF is so rare, there is a dearth of research on this condition.
This is why it is so exciting that the first worldwide EA/TEF study is being conducted right now. Dr. Evan Dellon of University of North Carolina School of Medicine, Division of Gastroenterology and Hepatology is conducting a survey examining the characteristics and long-term outcomes of patients with esophageal atresia, with a goal of improving health outcomes for EA patients. This study is IRB (ethics committee) approved. As a clinical research professional, and an EA parent, I highly encourage those affected by EA to consider participation in this important study:
There is also a national registry available to citizens of France:
In the spirit of research and medical advances, Baby James says STOP esophageal atresia 😉