Myla's Story - Congenital Diaphragmatic Hernia (CDH)
In November 2016, we found out we were pregnant! Stella our first born, was a year and a half old, and we were excited to give her a sibling. In December, we had an ultrasound to confirm the pregnancy. I was about 12 weeks along and everything looked great; we had one healthy baby growing inside. We scheduled our 20 week anatomy scan in February 2017….it couldn’t come soon enough. Little did we know, our world was about to be rocked.
Casey, my husband, and I went into the clinic in our small town of Wenatchee, WA for our 20-week anatomy scan. I could not contain my excitement! I was so anxious to see our baby on that big screen. We had decided to keep the gender a surprise. The ultrasound tech was friendly and telling us what exactly we were looking at on the screen. She was just about finished but wanted to get a few more images of the baby’s heart. She became very quiet as she was looking at the screen. Then she told us she needed the radiologist to come in and see if he could capture the images they needed. As soon as she left the room, I looked at Casey and said, “Something is wrong”. The positive and upbeat guy Casey is, he reassured me everything would be fine and that baby was most likely just lying in a bad position. The radiologist came in and tried to get some images of the heart but still couldn’t get what they needed. He was talking to the tech quietly under his breath, making me feel uneasy, so I asked if something was wrong. He told me the baby’s heart was displaced and not in the correct spot. I asked why, and he couldn’t tell me. He said he was going to review all the images and get back to us in a few days. We were devastated. We tried to remain positive and prayed everything was going to be ok, but that was hard to do.
3 days later, I got a call from my OB. He said the radiologist was concerned about the baby’s heart being in the wrong place. They wanted to send us to the University of Washington in Seattle for a level 2 ultrasound (3-hour drive from where we live). We couldn’t get in for an appointment in Seattle for almost a month; talk about torture!
Finally, the day had come, we were in Seattle having another ultrasound. It was nice to see baby on the big screen again, but we were anxious to get some answers. The ultrasound lasted about an hour, then we were taken into a consult room and told a high-risk OB would be in to talk with us shortly. She gave us some news we never expected to hear. A parent’s worst nightmare is hearing something is wrong with their child. Our sweet unborn baby had been diagnosed with a Congenital Diaphragmatic Hernia (CDH). Casey and I had never heard of this before. The doctor said our baby had a hole in the right side of their diaphragm, and the baby's liver was coming up through the hole and into the chest cavity which is causing the heart and lungs to be pushed out of place. Baby's heart is up against the left side of the rib cage and rotated slightly. Baby's lungs are not able to grow properly because they don't have enough space to do so. She said they could not see the baby’s right lung on the ultrasound. Baby will need surgery to repair the hernia after birth. They will put the liver back where it is supposed to be and patch the hole in the diaphragm. After the surgery, the heart and lungs will go back to their correct place. The doctor said the surgery is fairly minor and not complicated. The major concern is baby's lungs. We don't know how well the lungs will develop since they have limited room to grow. She told us most times CDH occurs the hole is on the left side. But our baby's hole is on the right. We were told survival rate with left side CDH is 80% and survival rate with right side CDH is 50%. She told us our baby had a LHR (lung to head ratio) of .8, and for optimal survival the LHR should be greater than 1. So basically, our doctor was telling us this was the worst-case scenario for CDH.
Then we were asked if we wanted to consider terminating the pregnancy. I was 25 weeks pregnant and right at the cut off for an abortion in Washington State. I could not believe she was asking us this! We immediately said no, we did not want to terminate. I think after the doctor asked us that, I realized how severe this was. The doctor told us not to google CDH. She said there are a lot of sad stories out there, and we didn’t need to read them. We left Seattle feeling like we had been punched in the stomach. We felt defeated. Sad. Scared. Lost. Hopeless. How could this happen to us? How were we going to tell our family and friends about our baby’s condition when we didn’t even understand it ourselves?
After receiving the CDH diagnosis, Casey and I decided we now wanted to know the gender of our baby. Being pregnant with a sick baby is not easy. It was hard for me to remain positive and happy when I knew my baby was sick, very sick. If we didn’t get to bring our baby home from the hospital, we wanted to know who was growing inside. I felt like I could bond better and decide on a name if we knew the gender. We found out we were having a GIRL! Stella was so excited to be getting a sister. We decided to name her Myla Faith. Myla means “soldier or merciful”. We knew Myla would be fighting for her life, so we thought it was very fitting.
As soon as we got home from that second ultrasound in Seattle, Casey and I began doing research on Congenital Diaphragmatic Hernia. We read countless articles, medical journals, and looked at numerous websites of the top children’s hospitals in the country. We wanted to learn anything and everything we could about CDH. One thing a lot of these articles had in common was that the same doctor was saving all these babies lives. The name Dr. David Kays kept coming up. Naturally, I googled him and found he was located at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida. Dr. Kays was the director of the Congenital Diaphragmatic Hernia program at that hospital and also the director of the ECMO program. Casey and I were intrigued. No other hospital we had found had a program like this for CDH. But Florida? All the way across the country? There was no way we could make that work. Also, while googling Dr. Kays, a YouTube video came up of Steve and Annie Zolman (founders of Tiny Hero) and their little boy Adam who also had CDH. They had relocated from Utah to Florida to see Dr. Kays. Seeing the video of Adam, and how well he was doing gave us so much hope that our baby could someday be doing as well as him. And maybe, just maybe there was a way we could get to Florida to see Dr. Kays too?
Long story short, I got in touch with Annie Zolman and a few other families who also had CDH babies. These families had relocated from all over the country to see Dr. Kays to get the best treatment possible for their babies. We had our records from Seattle sent to Joy, the coordinator of the CDH program at Johns Hopkins All Children’s. We did a phone consult with Dr. Kays; after we got off the phone with him, Casey and I knew then we HAD to get to Dr. Kays if we wanted our baby to survive.
After that, everything just fell into place. It was totally a God thing. We left Washington State on May 10, 2017 and arrived in St. Petersburg, Florida to await the arrival of our baby girl. We got all moved in to the Ronald McDonald House across the street from the hospital. My due date was July 5, (I was at risk for pre- term labor because our first daughter was born early) so we wanted to be in Florida way before the due date, in case Myla decided to come early too. I was scheduled to be induced on June 28. My water broke in the early morning of June 18 (a week before my scheduled induction), so I went to the hospital to get checked out. They admitted me, and Myla was born the next day on June 19. She did not cry when she came out; she was immediately taken away and intubated; and she was very blue. After Myla was born, I vividly remember Dr. Kays walking over to me and telling me that Myla was much bluer than they typically like to see, and he wanted to get her up to the ICU right away. They wheeled her by me, I got to touch her hand, and then she was gone. Casey went with her and was texting me updates until I was able to get up there and see her a few hours later.
The first 12 hours after Myla was born, she remained stable. But at 18 hours old, Dr. Kays decided it was time to place her on ECMO. He had done everything to keep her off ECMO, but she was declining. He told us Myla was much sicker than we expected. She had been on nitric oxide and the high frequency oscillator ventilator, but her numbers kept dropping, and she was not doing well. She was put on ECMO and 10 hours later she had her repair surgery. The surgery lasted about 4 hours. After surgery, Dr. Kays told us Myla was missing about 80% of her diaphragm, and he used a gore-tex patch to sew her diaphragm together. She had 60% of her liver, part of her intestines and part of her kidneys in her chest. Now all we could do was wait. We waited for her lungs to inflate and for her little body to rest, heal and get strong enough to come off of ECMO. But that wasn’t happening.
After 9 days on ECMO with very little improvement, Dr. Kays decided Myla needed a patch revision surgery. The first patch that was put in was slightly too big, which was limiting the movement of her diaphragm and her lungs were not expanding like they should be. So back into surgery Myla went. This time it lasted about 3 hours. Dr. Kays said he trimmed about 25% of the patch away, and he was much happier with how things looked. A few days later we were finally seeing an improvement in Myla’s numbers. On day 20, she was taken off ECMO and was stable. We thought the hardest part was behind us, but we were wrong.
On day 21, a routine echocardiogram was done on Myla’s heart. During this echo, they discovered a massive blood clot in her SVC (superior vena cava). Myla had a MRI done to see how bad it was. Her SVC was completely occluded with no blood flowing through it. Amazingly, her body had formed collateral veins around this clot so her body was still getting adequate blood flow. We met with a cardiologist and Dr. Kays and they went over the risks and benefits of trying to go in and remove the clot. I remember Dr. Kays saying, “If this clot breaks loose and goes to her lung she will die. Game over. I cannot save her.” But putting her on a high dose of blood thinners to try to dissolve it was also dangerous. Dr. Kays said he thought Myla should go to the Cath lab to see if they could get the clot out. We trusted Dr. Kays 100%, so we agreed with him. Off to the Cath lab Myla went.
Waiting for Myla to be out of the Cath lab was harder than waiting for her to come out of her repair surgery. We knew Dr. Kays was going to fix the hernia, so we weren’t as anxious when Myla had her repair surgery. But not knowing what was going to happen with the blood clot was terrifying. Four long hours later, we got a call saying they were able to get the majority of the clot out but had to place a stent to help keep blood flowing due to the SVC being occluded for a while. We were so relieved to hear this news. Two days later, Myla left the CVICU and moved up to the NICU.
On day 31, after a few days in the NICU, Myla had her breathing tube removed, and we finally got to hold our baby girl and hear her cry. We had been anxiously waiting for this day to come, and it was finally here! She was immediately placed on CPAP, a nasal cannula with constant air pressure going into her nose. A few days later, Myla was taken off CPAP and put on a smaller cannula and on wall oxygen. Then she got to start feedings. Myla did pretty well with feedings, so her intake was increased by a small amount every day. A few weeks later she had an upper GI study done to determine the severity of her reflux. Luckily, being right side CDH her stomach was not affected much, so her reflux was not severe.
Several weeks later, when Myla was taking full feeds and steadily gaining weight, they started all of her discharge tests. Everything looked great and discharge day was here! 64 days later, we got to leave the hospital with our sweet baby girl. Myla got discharged on quite a few medications and on a small amount of oxygen. She was alive and had beaten the odds stacked against her.
Myla is now 10 months old and thriving! She is currently only on reflux medication and aspirin. She is off of oxygen during the day but continues to be on a small amount at night. She is learning new things every day. She loves her big sister Stella and is the happiest baby. We are forever grateful to Dr. Kays and his amazing team at Johns Hopkins All Children’s Hospital for saving our baby girl and giving her the opportunity to live a normal life.
Quick Look Information:
Liver up
ECMO needed
Right side CDH
LHR of .8