Arabella's Story - Congenital Diaphragmatic Hernia (CDH)

We were shocked to find out we were having another baby. Our older two boys were 2 1/2 and 9 months. We were actively trying not to have a baby so soon, but God had a plan. We excitedly announced to our families and friends about the newest member of our family.

We paid an outside source for an early gender reveal. We delayed going home be we could not contain our excitement to finally have a girl. We were over the moon. We celebrated and danced around singing "we're having a girl." Little did we know how irrelevant gender was during the anatomy scan.

We went to the official anatomy scan a couple weeks later. It was confirmed she was still a girl. We were so happy. Then the sonographer said "there are a few things I cannot see. Let me go get the boss and see if he can get a better look." I tried staying calm. I wanted to believe her and that she just couldn't see what she needed. The doctor came in and took a look. He told us it was a diaphragmatic hernia. I heard hernia, and like many, I thought "oh no big deal. It could be so much worse." Then he continued to talk. It was so much worse. The doctor was honest and told me he did not know much about it and would have to figure out where to refer me.

He set up my appointment with the children's hospital who confirmed her LHR was .7, but liver at that time appeared to be down, although it was teetering depending on her movement. We needed to go back in a couple of weeks to see if liver was up. If it was she would be a candidate for experimental FETO treatment. I told the doctor we would do whatever it took to save our baby. He responded "I do not think you understand the seriousness, I cannot even give you an estimate of survival, it will just depend on what she does once she gets here." At the follow up her liver was up and LHR was .6 . After many talks with God and my husband and a lot of research, we decided the experimental treatment was not what we thought was best.

I was distraught. I was done reading about the horrible thing called CDH. I went from not knowing it existed one day to allowing it to control me, my every thought, and every emotion. I turned away from the screens and turned it over to God. I thank God for my mother who kept looking. She sent me the link to Tiny Hero. Tiny Hero was my first glimpse of hope. The first sign God was hearing my prayers. I read countless stories of Dr. Kays and his dedication and love for these babies. I researched his success and how he derived his numbers. Tiny Hero gave me Joy Perkins contact information and helped me get to the consultation.

It was in the middle of a weekend when I sent an email to Joy. I was still feeling like there had to be a catch somewhere. I wasn't expecting a response. Joy contacted me first thing Monday morning. She was so kind and patient with me, even though I was frazzled and it showed. She called and talked to me about all of my concerns and explained Dr. Kays's method. She took my information and started the process of getting me to Florida.

I met with Dr. Kays and he gave Arabella a 90% chance of survival with an 80% chance of ECMO. He was able to answer my all questions without making me feel more confused and scared. He spoke to me as though my daughter mattered and wasn't a lost cause that he was already expecting to die.

A huge concern for us in particular was where would we stay with 2 other children for the amount of time we would need to be there. Joy helped with that as well. She contacted and sent a referral to the Ronald McDonald House. We were able to stay there 4 weeks prior to delivery and the entire time Arabella was in the hospital, until she had her follow up and was released to go home.

August 27th, 2018 Arabella was born via scheduled c-section. She let out a whimper. She wasn't strong enough to give a full cry, but I heard her voice. Something I prayed God would allow me to have. She was intubated and I got to see her as she was rolled past my head. She remained stable for a while. We were looking like she might not need ECMO. At 36 hours she started declining rapidly. Her oxygen saturation dropped to 22 and she was placed on ECMO. The next morning she was less than 48 hours when she had her repair surgery. She had 5% diaphragm, 40% liver up, and a measly 15% lung. She really started improving pretty quickly after surgery. She was cooled to preserve brain cells and she was retaining a lot of fluid. She was a little slow to start weaning, but once she did everything seemed to go pretty smooth.

She had one pretty scary set back when it was time to remove her chest tube. She developed a bleed. Since she was still on ECMO recieving blood thinners, she had to be rushed off ECMO. She was weaned every 4 hours. We were just hoping and praying that she could handle the rapid weaning. She was weaned 4 times in a 16 hour period. She handled it like a champ. She impressed everyone around her. Even more so than when she was such a trooper leading up to ECMO.

She just continued to steadily progress. She handled each wean when it came. She did struggle a little at first once she was extubated, but she was able to stabilize. Then came time to feed. She did have some reflux and does require medication, but she was lucky enough to not need any further procedures.

At 3 months old she was able to come home on oxygen at night. The oxygen is mainly used to give her a break so that she can burn less calories and gain more weight. She has not had any further complications. 

I give credit first to God. He was in control of the entire thing. Then Dr. Kays and his team for their love, dedication, knowledge and skill. I also thank Tiny Hero for giving us hope.