Joseph's Story - Congenital Diaphragmatic Hernia (CDH)

My husband and I got married in 2008, we wanted children right away. After a year, we tried fertility treatment and to our amazement it worked the first time. I conceived in September of 2009 and the baby was due June 7, 2010. We debated about wanting a boy or a girl and at 20 weeks we had the anatomy sonogram. I laid on the sonogram table the tech went over and over my belly with the wand. She never said a word and left to get the doctor. The doctor spent well over 45 minutes scanning. Then I finally asked, “Is it a boy or a girl?” “It's a boy.” My husband beamed. However, the doctor said, “But there is a problem. You baby has CDH, a congenital diaphragmatic hernia. It’s often fatal. I am so sorry. The nurse will give you name of a specialist.” I stammered through my thoughts, “Wait! Don’t you have medicine or a diet I can follow or exercise? I will do anything.” The doctor responded with, “I am sorry you need to ask the specialist.”

The nurse scheduled an appointment with Maternal-Fetal Medicine for the following day. We were devastated. I can remember sobbing all the way home and my husband holding it together until he talked to his dad. When he said, “Dad, it’s not fair” and his voice broke, I could have ripped my heart out. When I got home I just started to pray, it was all I could do for my son now. The next day both our parents drove in from out of town to go to the doctor with us. I wanted them to hear it first-hand and ask any questions to the doctor directly. She did yet another sonogram and told us that his defect was severe and to “give up.” We should “consider our options." We were young we could “start over.” Those words cut like a knife. Never! No never! That was my answer. The next suggestion was to find a center that specialized in CDH, but of course she didn’t know of any such center.

At this point, we were on our own to find help. We started researching online. My mother-in-law came across the old Kay’s Kids site. She read some stories and told us to look into it. After we did, we called the Firestones, a family that had a story on the website. Mr. Firestone told us that there were other centers to treat CDH but we should consider meeting Dr. Kays. I called the next day and got an appointment for a consult. In the meantime, we made an appointment to fly to Pennsylvania for a consult at CHOP.

We met with Dr. Kays first. I had sonograms done there and he met with us to discuss treatment. Dr. Kays talked with us for hours even missing dinner with his wife and daughter to talk with us. He first asked about our home, pets, family, religion and more. Then he explained that he would spend a lot of time with us during treatment so getting to know us personally was important. He asked the baby's name and from then on called him by name, Joseph. It was refreshing to be present with a doctor who wanted to know us personally.

 Joseph had a left-sided hernia with liver up. He was in the moderate to severe range. After talking about Joseph, we asked him about the wall of photos behind him. They were patients and he knew them all. Those kids mattered to him; he loved them. Joseph would matter to him. We left Gainesville feeling better. Dr. Kays had said 80% chance of survival. That was the highest number we had recieved yet.

Two weeks later we went to CHOP, and toured the facility with a nurse. They did sonogram and fetal MRI. Then we met with 4 surgeons in a tiny room. They put kleenex on the table and began to tell us how severe or baby’s defect was and that they only gave him a 40% chance of survival if he survived birth. They didn’t want to know us or our story. After the explanation of diagnosis, they left. We were again pulling back into despair. I emailed Dr. Kays and told him of all CHOP had said. His response “When I say his name, 80% chance is still what I believe.” That did it. We would go to Gainesville.

I continued to see Maternal-Fetal Medicine while still in Louisiana and planned to move to Gainesville in May, just weeks before delivery. Joseph however created lots of extra fluid and by 7 months there was 40 cm of fluid. My local doctor considered Joseph already dead. I had horrible care. I had to move to Gainesville as soon as possible. So my husband and I took a few suitcases and moved. My in-laws packed up our apartment after we left. My husband still had to work so he would be with me on the weekends and then drive 900+ miles home to work Monday through Friday. During the week, my mom and mother-in-law took turns staying with me. Once in Gainesville, I was put on bed rest and twice fluid was drawn out, more than 2 liters each time. Joseph would just make more though. The last month was the hardest but my new doctor, Dr. Richards, decided to schedule a C-section.

June 1, 2010 at 9:05am, Joseph was born by C-section. He was 7 pounds 14 ounces, 20 ½ inches long. He took 15 breaths on his own before being intubated. I only saw him for a moment before he was taken to NICU and my husband followed him. Once in the NICU, Joseph was on a vent, with oxygen monitors on his head, back, foot and finger. A blood pressure cuff on his ankle. Tubes down his nose and IVs in his head and hand. The IVs were soon replaced with a PICC line in his left arm. It was quite a sight. I didn’t get to see him until 5:00 in the afternoon. It was torture to wait all day. You would think you would be overwhelmed by the equipment but actually all I saw was my son. Looking back at pictures I see the machines now.

Joseph did great. We waited 48 hours to see how he would do. We were told that he would have surgery the next day. On June 4th at 3:00pm, they took him off to surgery. I got a little kiss on his hand before they took him but that still was not enough. It was a very long, stressful four hours. I kinda shut down until it was over. I didn’t talk or eat, just waited. We met with Dr. Kays afterwards and he explained that Joseph had a 60% breach. So only 40% was his diaphragm and 60% was the patch. He explained that the diaphragm would grow around the patch. Joseph also had a full lung on the right and a fourth of a lung on the left. His heart had been compressed on the right side of his chest but no damage was done. They explained that they would let his heart drift back into a natural position rather than move it back themselves. After explaining everything, Dr. Kays hugged me. But it was not just a hug, it was like he was holding me together and like he needed the hug too. He had poured himself into helping my baby and he needed that.

Joseph stayed on the vent for the next 8 days then he was put on CPAP for 24 hours, then a nasal cannula. One day I walked in and Joseph’s isolette bed was gone! My baby looked like a baby, wrapped in a blanket, laying in a crib. I would finally get to hold him. After 14 days, the nurse brought me a chair and handed me my baby. I held him and the next day he got to drink the breast milk I had been pumping since he was born.

Joseph was soon moved to NICU II and he ate and gained more weight. We had a small scare with a staph infection but that was only in his PICC line, which was promptly removed. Joseph was doing great. Then on July 2, 2010, 31 days after birth, Joseph was sent home. We packed up and went home back to Louisiana. Today Joseph is a happy healthy first-grader. He does whatever he likes and has no medical restrictions. We have been lucky enough to have no complications from this. Thank you to Dr. Kays and Jesus who walked with us every step.

Basic information: Max and Deborah Hidalgo. From Walker, LA Our son, Joseph Hidalgo, left sided, liver up, no ECMO, LHR less than 1.0. Feel free to contact us at hidalgoteacher@gmail.com or 225-236-6387. We are more than willing to talk to parents-to-be and answer questions.