Stephanie's Story - Congenital Diaphragmatic Hernia (CDH)

Two weeks before my due date, I had an ultrasound. I was 40 years old with two children ages 9 and 13. The technician couldn't find the baby's stomach. Long story made a little shorter, I traveled to a specialist in St. Petersburg, Florida (from Anna Maria Island, FL) where I had an amniocentesis to make sure her lungs were strong enough to deliver her. I had to wait 24 hours to find out the results. It was a go, and I went to my local hospital (I worked there, so it made life a little easier). They weren't a hospital equipped to my situation but we really had no idea that it was going to turn out this way. I did have a natural child birth with a Pitocin drip. When I delivered Stephanie, she was a little over 5lbs. She made one sound, and then they took her away.

I waited about 2 hours to find anything out. They hooked her up to all kinds of machines and didn't tell me much except she may have to go on ECMO. I had no idea what that was at the time. 22 years ago, there were only 4 ECMO machines in the country: California (my sister lived outside LA), Houston, Texas (brother lived there), Miami, FL (my mom was in Ft. Lauderdale), and the closest to us was Shands Hospital, in Gainesville, FL. Stephanie and I were transferred to Shands Hospital via flight, and my husband met us there just 20 minutes after we got there. Then Dr. Kays came in the room and explained what was going on to us. I really didn't understand except Stephanie had no diaphragm and all her parts floated up into her left chest cavity except her liver. He wanted to wait 5 days until she was stable. Every book I looked at said chance of survival was minimal. She was Dr. Kays’ 16th baby, and he was sure she would be okay. So we trusted him. On September 5th 1994, she had surgery with Dr. Kays. To this day, I have no idea how long the surgery took (I think it was around 4 hours). When Dr. Kays came out of surgery, he explained that when he took her little parts out, her left lung expanded and he only had a window of time to put it all back. After making her diaphragm fit (weaving muscle tissue and a piece of cortex), her heart ended up in the middle of her chest, her intestines in the opposite direction, but he got all her parts in place. It all worked. Then the real fun started. I stayed at the Ronald McDonald House (one of the greatest experiences for both my husband and me). I pumped my milk every day, hoping she would take it. But it took what seemed like forever. She would not take the bottle or my breast, but after about a week, she latched on. It seemed like a miracle. However, she kept vomiting. She couldn't hold anything down. Her esophagus was the largest Dr. Kays had ever seen in his career and wanted to wait a year before another surgery. So he asked if we would try a combination of Reglan and Cisapride to help the vomiting. We said yes, of course. It did help, but instead of vomiting 15 times a day it was about 8. I felt so bad for her; anytime she ingested anything, she would vomit. Other than that, she was doing okay. I can't recall why but something about her legs was off. They put something on her hips to align her better, but that was a horrible mess.

It only lasted a few days, and we demanded it be taken off of her. We wanted to go home. I was over all this; I had 2 other children at home, and it didn't seem like there was anything left to do. But she wasn't gaining weight, and we couldn't leave until she did. I kept begging the nurse practitioners and Dr. Kays to please let us go home. After 33 days, we got to go home. From what I heard, that was incredible. It seemed like forever, but I guess we are very lucky. After a year and all the horrible vomiting and not thriving, Steph had her second surgery, Nissen fundoplication, which went perfectly. During the surgery, Dr. Kays put a G-tube in place as a precaution. We used the G-tube to burp her. Her G-tube was removed after 6 months. Steph is now 22, beautiful, with no apparent side effects at all. We were told she would never be able to burp. Oh boy were they wrong!!