CDH International’s Featured Patient of The Week is CDH survivor RJ. RJ is the son of Jessica and Bobby Nylen and they shared their journey with us in their own words. You can also read their journey on their blog, RJ’s Story: The boy who didn’t know he had CDH, https://hopeforcdhbabes.wordpress.com/2020/05/19/rjs-story-the-boy-who-didnt-know-he-had-cdh/
Making a Plan
I always planned to have a natural labor and delivery, I’m one of those crazy women- you know the ones who say they want to feel EVERYTHING so they can experience that bond with their baby during the most magical time. Months leading up to the big day, my husband and I took breastfeeding classes, we even hired a doula to teach us a father centered coaching for the big day to be sure we could have this natural labor I always dreamed of having. After 9 months of an extremely healthy and picture perfect pregnancy, I was sure I would be able to push my healthy baby out intervention and med-free!
Fast forward to November 11, 2019; Our final ultrasound. Everything looked great, however I had gone over a week past my due date so my doctor was concerned that baby RJ was getting a little too big inside me, so he scheduled an induction date for later in the week. Yes, induction- like NOT a natural way to give birth.
It’s go time!
I was induced at 8pm on November 13, 2019 with cervidil and it brought my labor on rather quickly. Bobby was up all night with me coaching me through the contractions, watching them on the monitor with me, and applying counter pressure to alleviate some of my pain. The doctor, my Aunt Kathy (who happened to be our labor and delivery nurse) and another nurse named Faith came in to check how dilated I was at 7am on November 14, 2019. In my doctors words, “it’s go time, start calling family and friends the baby should be here in 2-3 hours!.” He was able to break my water and my body naturally dilated to 8cm. It was a dream come true, I was going to have a natural-ish pregnancy (minus the cervidil to soften my cervix), but I was still going to FEEL everything with no epidural or Pitocin! 4-6 hours later, the Doctor came back in to check me again…. I was still only 8cm.
My doctor knew how desperately I wanted to have a natural birth, so he gently recommended I try some Pitocin. I broke down into tears knowing my “birth plan” was not going to go the way I intended. I agreed to the lowest dose of Pitocin. Fast forward again past the most intense pain I’ve ever felt in my entire life, I was fatigued and losing steam to keep pushing. Feeling defeated and knowing my body was not progressing, I did something I never in a million years thought I would do, I requested an epidural so I could receive higher doses of Pitocin to get our baby out. I was so eager to meet our little man, and I knew the stress my body was under was not good for him or myself.
It was not “go time”
Fast forward again to 6pm, sitting at 9.9cm dilated for hours- it was time to push, so I thought. I spent nearly 3 hours pushing, and baby was not coming down. Falling asleep between contractions, going on 24hrs of labor, I read the expression on my doctors face saying “c-section.” It got to a point that I knew things were becoming unsafe for my baby, as his heart rate would drop occasionally through my contractions. My body was shaking, I was nodding out between contractions, and the ice chips and sugar IV’s were becoming completely ineffective for keeping my stamina up. I did another thing I thought I would never do and elected for a c-section.
Everyone gowned up, I was prepped and medicated, and scared to be cut open on an operating table but I knew it wouldn’t be much longer until I had my baby boy in my arms! The time was here, my baby boy was born! The doctor passed him along to the nurses who brought him over to the nearby table in the operating room. I heard the team announcing his entrance into the world “Robert Joseph Nylen Jr., weight 8lbs and height 21 inches, entering the world at 8:22pm.” With my husbands hand in mine I anxiously waited for the nurses to bring him over to me so I could experience the little bit of skin to skin I was promised and could see my husband cut his cord. They weren’t bringing him over…I began to panic, why weren’t they bringing me my baby? I heard him cry and they told me everything was okay, but they weren’t bringing him to me. My heart was racing and I knew that something was wrong.
Where is my baby?
The first time I saw my baby was when one of the sweet nurses showed me photos and a video she took of him for me. Not knowing why my baby was taken into the nursery without my husband or I seeing him, we were forced to wait in the recovery area for two hours before going into a room. One of the nurses came to visit us in recovery to let us know that our baby was just finishing up a chest x-ray and than the Neonatologist would be coming to speak to us shortly. This confirmed our fears that something WAS wrong. After two hours that felt like an eternity, I was wheeled to our hospital room, and the Neonatologist entered to speak to us.
Your baby has CDH
She explained to us that when RJ was born the nurses noticed that his heart beat was not heard where it should be, and it was heard towards the center of his chest. After completing chest x-rays they were able to identify that he has a rare condition called Congenital Diaphragmatic Hernia (CDH) and even more rare was the fact that it was undetected throughout my entire pregnancy. Typically, CDH is diagnosed early on via ultrasound. She continued to say she was not sure the severity of his condition, whether he had two functioning lungs or how many organs pushed through the hold into his chest cavity and he would need to be transferred immediately to a specialty level 1 NICU hospital for further testing and most likely surgical repair. She gave us three options of nearby hospitals and told us to decide which one we would like him transferred to. We chose the furthest of the three but the one that we knew had the best level 1 NICU, the Children’s Hospital of Philadelphia (CHOP). This hospital was 66 miles from home but a 20 minute helicopter ride away.
I will never forget when my Aunt entered our room with tears in her eyes and asked me if we wanted RJ to be baptized before his departure to CHOP. I knew that they asked that only when patients were at risk of not making it. My heart broke into a million pieces when the thought of that set in.
The nurses asked if I wanted to see our baby before they flew him out. Of course I did!!! They wheeled him into our room at around 3:00am and it was not at all what I expected when they asked if I wanted to see my baby. I thought I would get to hold him in my arms and kiss him, instead the respiratory team from CHOP wheeled in a stretcher with what looked like an little insulated cooler with little windows that he would be transferred in- I peeked through and saw my sweet boy for the first time, with tubes out of his mouth and hooked up to all sorts of monitors. I was able to tell him I loved him and prayed that my voice would bring him some hope and strength.
Since I was recovering from the c-section, we were separated and my husband left to drive out to Philadelphia to stay with our baby until I could be discharged. To say the least the next few days were full of fear, tears, prayer, and FaceTime calls to my husband who was bedside with our sweet boy.
{The first night at CHOP: Dad’s story}
That night, with my son’s condition still unknown, I wept as I watched the helicopter leave the hospital. I went back to the room to say goodbye to my wife, and couldn’t help but to be upset to leave her in the condition she was in, but I knew I had to be with our son in this moment. Jess’s Aunt drove me to the hospital as I tried to rest on the drive there after being up 48hrs.
When I got to the hospital I couldn’t wait to see my son. As I entered The room I saw the nurse checking over him, I had finally got to see my son; not in the condition I would have liked but I could finally hold his hand. I couldn’t help but to miss my wife and think of how much it was killing her to be away from our son.
The hospital arranged for me to stay in a sleep room just across the wing from RJs NICU bed. That night, It took me a lot of prayer to get some comfort from God and get some rest.
The next morning I woke up with hope. I spent the whole day with my son, looking over him. The doctors made their rounds and assured me that his case was looking to be a good diagnosis as far as CDH went. Throughout the day, genetics teams, specialists, and surgeons came by to talk to me and helped me to understand all that was to come.
I found out later that day that my wife would be able to come join us in Philadelphia. That evening I traveled back to the birth hospital where I got to see my wife again. I picked her up and we were on our way back to our son, to be together as a family.
Meeting my sweet boy
On November 16th I was able to convince my doctors to discharge me early (despite my hunched over limped walking from my still fresh incision), I just had to be with my husband and RJ. My husband left our son’s bedside for the first time and came to pick me up. The anticipation was so real, I was finally going to meet my baby boy. The hour and a half car ride felt like days. Late in the evening we arrived.
I broke out in tears the second I walked up to his bed. He looked like such a fighter, but the sight of him with a tube down his throat, strapped down in place, hooked up to wires and beeping monitors was extremely unsettling. My husband raved about how amazing the nurses and teams of specialists had been the past few days, and now I got to see for myself.
Our days were filled with me pumping milk for our little guy, speaking to the doctors and nurses during their morning rounds, listening to the monitors (and panicking if they appeared abnormal), praying over our son at his bedside, lots of fearful tears, and eating the same cafeteria food daily.
God-sent surgeon
On day three we met with the surgeon who would be performing RJ’s hernia repair. Her name was Dr. Emily Partridge, and if you asked my husband or I to describe her, we would say a real life angel. She lit up the room with her smile and positivity and put both of our minds at ease the best she could. Dr. Partridge explained to us that it was hard to tell how severe RJs case was until she was able to take a look in the operating room, however she felt confident that his case was on the mild side and that she would be able to preform the procedure minimally invasively with laparoscopic techniques. In her words “RJ is acting like a little boy who doesn’t even know he has CDH, he’s such a fighter.” Her confidence helped, but I was still terrified that my baby boy would undergo surgery at just four day’s old.
Surgery day
November 18, 2019… surgery day. Bobby and I had always had a strong faith, and both my delivery and the unknown of RJs condition led us closer than ever to god. We knew this entire situation was out of our control and all we could do was trust. We prayed every single day, multiple times a day.
Watching the Anesthesiologists and surgical team prep RJ for surgery was a scary sight to see. The thought that my tiny baby would be put under, the fear that he would only have one functioning lung, and not knowing if he would come out needing a blood transfusion (another possibility we were prepped for ahead of time) brought on all types of emotions and again all we could do was pray and have faith.
I still remember pacing the surgery waiting room from the moment he went in. Every time a messenger would come through the doors my heart sank a little thinking of if they would be bringing us good or bad news. Finally Dr. Partridge came through the doors- her first words were “everything went great, it was one of our fastest repairs and done minimally invasively, he behaved like a perfect gentleman!” The entire procedure was done minimally invasively and both of RJs lungs were fully developed. The only organs that made their way through his diaphragm was his small intestines. Dr. Partridge was able to pull them down robotically and sew up the hole. Thank god, our baby boy was now on the road to recovery!
Long road to recovery
I thought that surgery would be our biggest obstacle, but little did I know there were a lot more hurdles to overcome. Our next steps were to get off oxygen support, remove his hydration IV lines and introduce breast feeding, and ween his morphine and medication drips to zero.
Skin to skin
The nurses and doctors knew how deprived I felt from not getting to endure any skin to skin with my baby, so they ensured that was the first thing we did when RJ was recovered enough from his surgery. It was the most amazing feeling in the world and although it didn’t happen right when he was delivered, it felt just the same to me. Feeling my babies little hands and warm body against mine was forever life changing. There was in fact the bond that I feared we would be missing.
First breaths
November 20, 2019- The day after RJs surgery, the respiratory team came over to his bedside for their morning rounds. The doctor was quite impressed with RJs progress thus far, and felt that he would do great on room air. He put an order in for the breathing tube to be removed and replaced with a small nasal canula for minimal support. In my mind, I was thinking “this is amazing, we’ll be home with our baby boy in no time.”
Unfortunately, this process wasn’t as smooth of a transition as the doctor anticipated, and RJ was still working a little too hard to breathe on his own. The order had to be re-modified to include support from a CPAP mask.
The pressure was re-evaluated daily and two days later on November 22, 2019 the CPAP mask was swapped over to a nasal cannula. Two more days later on November 24, 2019 all breathing assistance was removed and RJ was breathing 100% on his own!
One step forward, one step back
Although we were slowly progressing, each and every day I experienced many highs and lows. It felt like we were taking one step forward and one step back when a treatment needed to be re-evaluated or if we didn’t make any progress that day. It was hard to stay hopeful with so much unknown but I knew we had lots of prayers coming in from family, friends, and our church. I needed to stay strong for my baby boy, but as the days went by it was harder and harder to stay hopeful. I had to truly trust and let go of my attempts to control every situation.
He was addicted!
Each day, the nurses would attempt to ween RJs morphine drip to zero. This was the most heartbreaking task of all. I will never get the image out of my head of my baby boy having full body tremors and sneezing uncontrollably. He was addicted to the pain medication. I was angry that the health professionals had him on such an intense narcotic.
Bobby had to remind me daily that it was something they needed to do so RJ was not in pain and so that they could care for him without him ripping his tubes out, but as a mama bear, I was still angry.
Every time we thought he’d be free of it, he’d go through another withdraw and the nurses would have to give him another push of the medication. This caused me to have MANY breakdowns and it was hard to bring a positive vibe into the room seeing my son go through something so intense.
CDH was not so rare at CHOP
Since we were living in the sleep rooms of CHOP, we came to realize there were many families going through similar experiences as us. We had the opportunity to meet many parents who’s babies also had CDH. It was very enlightening to hear the stories, some parents had been there 7 months, some were going through the discharge process after only 2 weeks.
Taking a step back and looking at the big picture really helped me to stay hopeful. Our baby had TWO functioning lungs, some babies only had one. Our baby will not be on long term oxygen support, some babies needed a lifelong trach put in. Our baby had surgery 4 days after birth, some parents were patiently waiting for their baby to be stable enough to endure a surgery. And lastly, the doctors predict that he will not need lifelong follow ups, some babies will forever struggle with the effects of CDH.
RJ was a fighter, and his diagnosis was mild compared to how devastating it could have been. I needed to stay focused on that and be grateful, as hard as it was to do in those moments.
Please latch, baby
Our next obstacle to face would be our biggest yet, feedings. On November 23, 2019 I got to experience the moment I was anticipating even months before delivery, the breastfeeding latch. It was not the experience we learned so much about through the classes Bobby and I took. I didn’t get the initial skin to skin triggering my babies latch response hours after birth. Here we were 9 days into life expecting RJ to know exactly how to feed after he had IV fluids providing his body with the nutrients he needed.
It was very challenging to get RJ to learn how to suck and swallow. Daily we had visits from the lactation team, the speech therapists, and nurses to help position him correctly. RJ did not yet have the stamina needed to get the amount of milk he needed to maintain his weight, and he was not grasping the concept of sucking and swallowing. Each and every feeding was a struggle. The nurses would weigh RJ before he fed, let him breastfeed for as long as he would latch for, and weigh him after the feed to see how much he was taking in. The goal was 50ml and he was taking in 5ml on a good day. The rest of the feed was to be done through a bottle of my breastmilk.
The doctors and nurses suggested that bottles would help to strengthen his jaw muscles. When he would only take an additional 5-10ml with the bottle, the doctors resorted to inserting a NG Feeding tube through RJ’s nose. For every feed he didn’t finish orally, the remainder of the milk would go through his tube; than we would wait and observe if he would spit it up.
I remember thinking, our baby is never going to learn how to feed without this tube. As someone who yearned so much for a close bond with my son through exclusive breast feeding, this was my worst nightmare.
After another week of little progress with oral feedings, CHOP arranged for Bobby and I to attend a training course on how to insert the NG feeding tube. This brought us to the realization that this feeding struggle actually may be a long term thing. I remember Bobby and I would ask each and every nurse how long CDH babies typically take to learn to feed on their own, each time hoping for them to respond with “he should grasp it by tomorrow,” however that was never the response…the nurses would gently say “each baby works at their own pace, some grasp it quicker than others and some take a little more time.”
Getting the best of us
Bobby was my rock since day one. I would ask him If everything was okay and he would always respond “of course, we’ll be home before we know it, RJ is killing it and he’s making progress everyday!” I knew he was trying to stay strong for me through my postpartum emotions and my own personal fears of the situation we were in, but deep down inside the situation was a struggle for him too. I remember looking at him as we ate the same cafeteria dinner we ate each night for weeks and seeing a little depressive expression in his eyes. Truthfully it was a struggle to stay positive each day. On days where there was no progress and we would wait for a change, it was hard to see the light at the end of the tunnel.
Happy Birthday to me!
After weeks of minimal changes in RJs eating, the team decided it was time to discharge us with RJs feeding tube. It was ironically my birthday, December 6th. It was the best birthday gift I could ever have.
However, it brought on a whirlwind of emotion; overwhelming joy and excitement that we were finally bringing our baby boy home, but extreme fear and anxiety of taking on the responsibility to be his medical caregiver for a feeding tube! We couldn’t wait to be home, but I was terrified at the thought that we would no longer have a call button to have the nurses check his vitals, or to help us insert a new feeding tube if he pulled it out, or to reassure me that his incisions were healing properly. I guess being a new parent and bringing your newborn home for the first time comes with new fears and emotions, ours were just a little different. As a new mom, this was my time to step up and be the strong one.
Getting into our own routine
The first few days were nerve wracking. I didn’t like that we no longer had monitors displaying RJs heart rate and oxygen levels. There were so many times I would lie awake and watch his chest move up and down as he slept at night. We continued to work with RJ on bottle feeding my breastmilk to him. Five days into being home, a miracle happened, RJ decided he was over his NG tube and he began taking 75ml of milk on his own consistently! We were able to pull his feeding tube out and we could finally see his entire handsome face, free of tubes or tape for the first time ever!
What this CDH diagnosis has taught us
This experience has taught us many things. I have learned that I can not control everything in my life. I’ll always remember the day I was discharged from the hospital; one of my labor and delivery nurses (Faith, ironically) came to visit me and she said “sometimes we say things during labor and don’t remember them after, but do you remember that you said, “my labor not going to way I planned might just be a sign that I have to let go and trust God and stop trying to control everything” she said that really stuck with her, and it stuck with me too. This experience has sharpened my faith like no other and has shown me the true power of prayer.
Another thing I learned is that CDH babies are like no other; they are strong and resilient and can overcome anything thrown at them. My baby boy is stronger than I’ve ever been, especially with everything he’s been through and everything he has overcame.. as Dr. Partridge said “he acts like a little boy who doesn’t know he’s sick.” My CDH baby will forever be my little hero and I will be the strong mama I’ve grown to be because of his bravery and strength.
A final note of HOPE to those who are facing a CDH diagnosis
As I sit here writing this on May 19, 2020, six months out from all we have experienced, I am reflecting on all that we have faced and OVERCAME through this journey. Our baby boy is healed and you would never know he is a CDH baby (except for his resilient and strong personality).
Our baby boy is growing and thriving! He is now six months old, weighing 18.5 lbs and measuring 25 inches tall. He is smiley and full of giggles, and even began CRAWLING this week. He loves to explore his surroundings by crawling around or running (yes running) in his walker. His incision marks are practically invisible at this point. We can tell he is not going to be one for relaxing and staying still for too long. He loves his mamas milk, right from THE SOURCE, exclusively breastfed on demand, and actually has developed a dislike for bottles.
I tell you all of this not to brag or gloat about how amazing our baby is, but to bring you hope that you too will be experiencing all of this in no time. I hope that as you have read our story, you are filled with the hope that we yearned for while we were in your shoes. The NICU is full of highs and lows, but once you ride those waves it will be smooth sailing and all of the hard times will be left on the shore.
