Charly's CDH Story - RCDH
In 2016, November 2nd was the final game of the World Series, and my home team finally had a winning shot. I really don’t care for baseball but when a team like the Cubs might win, and you’re from Chicago, you can’t help but be excited! Our older daughter, Willow, was still a half day student in preschool and came home very pumped about the game.
We had an ultrasound appointment scheduled for the end of the day, around 4pm. We already knew Charly was a girl, but we were looking forward to the visual confirmation. In our mind, an anatomy scan was simply a sex scan. It was a tool to find out the baby’s sex while the tech looked at a bunch of other boring stuff.
Just five or six months prior, I had suffered from a horrible, long, drawn out miscarriage. Even though baby stopped growing around week 9, I didn’t miscarry until week 12-13. The process took a month. In my mind, I’d made it past that point and nothing else could go wrong! Now, we were finally going to have the baby we’d been trying to have for over a year.
The tech had finished and sent the doctor in to “check everything”. Willow was getting so bored and needed to use the restroom. Josh told her to wait but the doctor said it’ll be a while longer, go ahead and take her. After they left the room, the doctor asked if we’d had the blood test done to check for abnormalities. I told him we did, and it was normal. He said nothing. I asked him why he wanted to know, and he said, “Well, we have a problem. A BIG problem.” He continued to explain that she had a Congenital Diaphragmatic Hernia. I didn’t understand why a hernia was a big deal; people get hernias all the time. Then he started to explain what it meant, how he couldn’t find her right lung and the left was too small to support life, how organs had migrated from her abdomen into her chest and because they were compressing her heart, she may have heart defects as well. At this point, Josh and Willow walked back into the room. Josh noticed I was crying and came to my side, asking the doctor in a very matter of fact tone what was wrong.
He explained it all over again. The weight of the situation fell heavy on my shoulders, and I burst into tears, “I’ve had wine! I’ve had wine during this pregnancy did I do this to my baby?!” It was my fault, I couldn’t think of anything else I’d done wrong but it was my fault. Why couldn’t I make a baby anymore? I got pregnant with Willow out of wedlock because I missed 5 days of birth control. Where did all my baby making talent disappear to?!
Dr. G assured me wine was not the culprit. There’s no known cause for CDH beyond rare genetic issues arising when two parents with certain carriers have a baby, or other genetic abnormalities including a few trisomy defects which would be fatal anyway. He recommended we get an amniocentesis to rule all of those things out and started to talk about odds. Here are a few statements he made:
· She has less than 5% chance of survival.
· Right sided CDH is more severe than left sided and harder to treat.
· If I don’t have a late term miscarriage and I do give birth, she won’t last more than a few hours, maybe a few days.
· He offered termination and spoke extensively on the subject, explaining cut off times and having to travel to New Mexico if we surpassed the time allotted in Arizona for an abortion. Overall, if my memory serves me correctly, we had about two weeks to decide whether we kept our very, very much wanted pregnancy.
Carrying a child you are unsure will be able to live after birth is a painful, devastating road to walk. People ask questions, they expect you to get excited, talk about nursery colors and baby clothes. I wasn’t excited; I was petrified. Her first kicks brought me to tears, her hiccups filled me with worry for her future, every comment about my precious bundle of joy made me feel a dark, oppressive doom that I can’t imagine any woman would want to feel.
We eventually left the doctor’s office. We had separate cars as we’d both come from different places to get to the appointment; I drove home alone. I toyed with termination for about five minutes, and then made up my mind I wouldn’t do it. I didn’t care how long she lived. God gave me Charly, and I was going to meet her come hell or high water. He had a story in play for her; He was knitting her in my womb, and whether I liked it or not, he assigned me to bring her into this world.
On the way home, I called my mom and my sister via 3 way. I asked my mom to put my dad on speaker and proceeded to explain what was happening as well as I could. To be honest, I think I called it CHD... and I couldn’t remember what any of the letters stood for. My parents and sister insisted they meet us at home. We put the World Series on and pushed the ottoman up to the couch. I’m pretty sure at one point, I laid in my mom’s lap as she stroked my hair back away from my face, something she’s done to comfort me since I was a small child. I needed my mommy so badly that night. We talked for what felt like forever. Josh quickly researched some things, but we mostly talked. We talked about God’s plan and how I was determined to make Charly’s life worth something, even if she didn’t survive. I wanted God to be able to use her story to further His Kingdom. That’s why He blesses us with babies, after all.
When everyone left and we got Willow to bed, we started our full-fledged research. We couldn’t get enough. In one night, we taught ourselves about what CDH is, various treatment options including FETO, what EXIT to ECMO was, which major children’s hospitals provided these treatment options. We also found a YouTube video made by Annie and Steve Zolman about their Tiny Hero, Adam. They had traveled all the way from Utah to some doctor in Florida for treatment.
While that was very inspiring, moving that far for treatment sounded crazy to both of us. I’m pretty sure we gave each other the “they’re crazy (raised eyebrows)” look. Eventually we decided that our first choice would be a leading children’s hospital in California. Within a week and a half, we had worked with sending our records to their team. We flew up there to have 2 hours’ worth of interviews and discussions along with a full day of tests, tours, and meetings with doctors and staff. After all the tests were done, the team prepared our case to be reviewed by their peers to determine if they’d offer us the surgery. I don’t remember how long the wait was but it felt like forever... and then we got the approval!
The next hurdles seemed too high, but we trusted in God to provide. We needed approval from both our insurance company and the FDA. Within days of the cutoff for no longer being able to have FETO surgery, we were approved by both insurance and the FDA. I packed up my entire life (because after the surgery in mid-December I wouldn’t return home until Charlotte was released from the hospital), we left for northern California. I grieved so hard. I spent every night leading up to our flight sobbing myself to sleep, huge, heaving sobs. How was I going to leave my life and my baby behind? We planned for Willow to stay in Arizona because I’d be on some form of bed rest the remainder of my pregnancy and wouldn’t be able to care for her. All of it was awful, but I knew we had to give everything we had to save Charlotte.
Long story short, we got to the hospital and the day surgery was scheduled to occur. I had a Fetal echo, MRI and ultrasound done to see a current picture of Charlotte’s hernia. They wheeled me into the pre-op room, but there was a hold up, the radiologist needed to come down and look at something one more time. I was panicking. Did I mention I was 6 months pregnant and by 3 pm hadn’t been allowed to eat all day? Yeah, that too!
She scanned my stomach, looking at the same few angles over and over. By the end of it, several doctors plus another radiologist specializing in CDH diagnosis had looked at the ultrasound and determined Charlotte very likely had a CPAM on her lung. Essentially making FETO surgery useless. So there I sat, in a sterile pre op room with my husband and daughter, hearing that what we thought was our one hope to save Charlotte all of a sudden was no longer an option anymore. This was the second time ever that I’d witnessed my husband cry.
Why would God line all of this up just to tear down every hope we had to save our baby? For the first time in our journey, I was so angry at God. I lashed out at my family, I cried for about 24 hours consistently. We did some sightseeing and changed our flights to come home on Christmas Eve. We were in my favorite city and as much as I love it, I’m still not sure I’m ready to go back any time soon. Afterwards, we spoke with the coordinator at the California hospital and explained that we’d still like to deliver there. She explained what the steps would be, and I began making those arrangements.
After the holidays had passed, I’d found that I was having a difficult time getting a hold of the staff via phone or email, but before (when we were fetal therapy patients and candidates for FETO) I got almost immediate responses. It began to feel like, because they could no longer experiment on Charlotte, they lost interest in helping us save her life. I posted something to that effect in a comment on someone’s post in a CDH social media group. Afterwards, I had comments in response and several private messages from women telling me about Dr. Kays. You know, that doctor I’d mentioned earlier that was way out in Florida? That one. He was in St. Petersburg. And all I could think was, isn’t that in Russia?
I spent days crying tormented by the thought that at almost 30 weeks pregnant, I still had no solid plans for Charlotte anymore. I didn’t know what to do but pray. Then, finally, someone gave me Annie Zolman’s number from Tiny Hero. Nothing anyone else said made me feel as strong and valid and intelligent as she did. She praised me for everything we were doing to get our baby help. She gave me questions to ask teams at various hospitals, and then she subtly suggested I contact Joy Perkins to discuss our options for going to Johns Hopkins All Children’s Hospital in St. Pete.
I had been talking to Annie on my way to work at our church. I went into the building and explained to my coworkers what was unfolding. They all provided incessant love, encouragement, and prayers for direction. At the end of the day, I called Josh and said “you’re going to think I’m crazy, but if we don’t at least call this Dr. Kays guy and talk to him, and Charly dies at the hospital in California, I’ll never forgive myself.” Within the next 24 hours, I spoke with Joy. Mind you, at this point I was about 30-32 weeks pregnant. My time was basically up. I had polyhydramnios and was at risk for preterm labor. Wherever I was going, I needed to be there by week 34 to ensure I didn’t have Charly in the wrong city.
Joy was so kind and gentle on the phone. I don’t remember much about our conversation except for that she asked me what Charly’s name was. She asked if we had other kids, how old, what her name was. She asked how long we’d been married. We were people to her. Not another opportunity to experiment. We scheduled a call with Dr. Kays within a few days and spoke with him for over an hour.
Here are a few of the statements he made (feel free to compare to what the diagnosing doc said and also note, the hospital in California quoted 60-70% chance of survival):
· Right sided CDH is NOT more severe than left, it’s just different.
· Career survival rate is around 95%.
· Based on the info he had, she would likely need ECMO.
This is a lot of info, but for me this was what was important. What I was told would NOT be an option everywhere else, here I could have a vaginal, pain med free delivery; I could encapsulate my placenta (I actually think he had to check on that - he laughed and said he’d never heard that one before); and importantly, to the best of my ability, I could have the birth I wanted - it’d just be in an operating room instead of a birth center.
But the most important piece of information he gave us was his prediction that Charlotte would have a 95% chance of survival. Let me repeat that. NINETY FIVE PERCENT.
Done. No questions asked; we were moving our care to Florida. We made all the arrangements necessary and planned for Willow and I to move to Florida on February 4th 2017 with the help of my friends and family rotating turns staying with me to watch Willow if I went into early labor until Josh could come out in late February.
I could go on forever detailing the rest of this story, but for now I’ll leave you with this, God throws us obstacles every single day. Some are huge and some are tiny. But by following His guidance, you can navigate those trials with more grace and determination than you ever thought possible. This journey showed nothing about my strength, or Josh’s. It showed God’s glory, His faithfulness, and His mercy. God cradled us every step of the way and led us all the way to St. Petersburg, FL where we found the best treatment for our sweet Charlotte Francesca. After spending 8 days on ECMO with her repair surgery occurring at about 36 hours old, a nissen fundoplication and gtube surgery, and countless other procedures and care tactics, Charlotte came home from the NICU at 59 days old. We are so blessed to have had the opportunity to seek care at the best possible hospital for our family.
If you are currently seeking out CDH care, we think these are some of the most important factors to consider based on our experience with Charlotte:
1. FETO is on the cutting edge of research and many leading hospitals are offering it. Any reputable organization will tell you that while it is promising, there is no guarantee it will help your child grow in utero. Because of this – please don’t get hung up on FETO like we were, and ignore what matters most, protecting and caring for babies with underdeveloped lungs after they are born, and repairing the hernia as soon as possible.
2. Learn about the continuity of care your baby will receive. At John’s Hopkins All Children’s Hospital, Dr. Kays and his team were in the labor and delivery room when Charlotte was born. He led her care for 59 days, seeing her every single day, until we were discharged. Caring for CDH appropriately takes an incredible level of experience that isn’t found in many places. In addition, if even the most incredible hernia repair surgeon hands your child’s care off to a doctor that doesn’t know CDH, the outcome may be less than favorable. Find a hospital that will give you one team from delivery until discharge.
3. Learn about the hospital’s experience with ECMO for CDH babies. Ensure two things:
a. There is no arbitrary time limit for ECMO use. Some facilities have a number in mind, and if your baby cannot support him or herself after that day hits, the assumption is the child is not strong enough to recover.
b. Your surgeon will perform repair surgery while on ECMO if needed. Some facilities and surgeons will not, as the risk for “bleeding out” due to blood thinners is higher than surgery off ECMO. We truly believe Charly would not be alive today if our surgeon wasn’t willing to operate on ECMO.