Guest Blogger Letter:
Dear Mr. Sparks,
Could you take a moment and read my daughter’s story? I know your books are a fantasy, but this is reality for all of us affected by CDH.
The reality is there even when people don’t want to face it. The reality is my daughter didn’t get the perfect beginning. She didn’t get to cry and be placed in her mom’s arms (or dad’s arms) for comfort. When she cried, she was whisked away to a different operating room to be intubated. People don’t want to face the reality of it all. I have had several family members tell me they didn’t want pictures of her with all those tubes. Those tubes were our reality. Why shouldn’t we have pictures up with her tubes? Why should we pretend it never happened? The reality is my daughter was born with Congenital Diaphragmatic Hernia. The reality is my daughter was very sick when she was born. The reality is my daughter survived.
Ryan and I found out that we were pregnant on Thanksgiving Day 2012. I didn’t take the pregnancy test until the very end of the day because I didn’t want to be heartbroken when the test came back negative. We were overjoyed to be expecting our first child. I knew immediately we were having a little girl. Call it mother’s intuition.
I felt sick all the time. I wasn’t able to eat anything besides pasta and cheese pizza. Around 17 weeks I ate a cheeseburger without feeling sick for the first time and was so excited to feel normal.
At 19 weeks we had our gender and anatomy scan. My mom and Ryan’s mom came with us. When the tech told us the baby was a girl I yelled, “I knew it!” We really didn’t care either way, I just hate being wrong. After the doctor looked at the ultrasound, we were asked to meet with him for a minute. Our moms waited in the waiting room for us and we met with the doctor. He told us our daughter had fluid around her lungs, this was called pleural effusion. This could mean a number of things anything from a chromosomal disorder like Down’s syndrome or it could be nothing. He wanted to see us again in a week to evaluate the amount of fluid and to see if there were any changes. Ryan and I were scared. We didn’t tell any of our family and actually lied saying the reason we were having more ultrasounds was due to me just being diagnosed with hypothyroidism a month prior to becoming pregnant. We didn’t want to worry anyone until we knew what we were dealing with.
At 20 weeks the ultrasound looked about the same and we were told to come back in two weeks to see if there was any change. After our 22 week ultrasound we were being referred to the St. Louis Fetal Care Institute (FCI) at Cardinal Glennon. Our doctor didn’t have the technology to assist us anymore.
The first day at Fetal Care Institute was long and overwhelming. We had an entire day scheduled out for us that included meeting everyone from our social worker to our doctor. We had our first 3D/4D ultrasound of our daughter, Lucie. We could see Lucie’s features and could tell she looked like me. As I lay in the chair for our ultrasound, we met our doctor, Dr. Vlastos. Dr. Vlastos had a way about him that we fell in love with immediately. After introducing himself, he told us, “Let’s take a look at the babe.” He pointed out all Lucie’s anatomy and pointed out that there was “something in Lucie’s chest.” He went onto explain that he believed her intestines were in her chest. Lucie had congenital diaphragmatic hernia.
Up until this point, Ryan and I didn’t have any idea what was wrong. It could be any number of things. To hear a diagnosis was actually a comfort. Even if it was the beginning of more fear, we were comforted to know what was wrong. Then the fear kicked in.
I went home and did a little research to understand the diagnosis better. I found a website for an organization called CHERUBS. The only other place I was finding anything helpful was from the DRHEAMS research study. I learned that these babies only have 50% survival rates.
At 25 weeks I went back to FCI for an MRI to confirm Dr. Vlastos diagnosis. Lucie had left-sided CDH (the hole in her diaphragm was on the left side) and her intestines were in her chest pressing against her left lung. Her heart was slightly pushed to the right side. Lucie had a predicted lung volume of 40% so they felt that she had a fighting chance. One thing I learned very quickly was that even if your CDHer had a good prognosis, it didn’t mean anything. Some babies who had the worst prognosis survived with others with great chances did not. Dr. Vlastos told me that Lucie had a good chance, but she was safest in the womb.
The following week we went for another check up with Dr. Vlastos. We met with a neonatologist and her surgeon, Dr. Chatoorgoon. I was able to hear what the surgeon had to say with very little fear. He told us that Lucie would have her repair as soon as she was stable enough to survive surgery. They would push her intestines back into the abdomen and repair the hole. After that, she would remain in the hospital as long as it was necessary for her lungs to develop and survive.
It was talking with the neonatologist and hearing what would happen when she was born that sent me over the edge. I could barely hear the words without tears streaming down my face. Ryan held my hand as I cried. Lucie would be born vaginally if possible. As soon as she was born she would be taken to be intubated. After being intubated, Ryan and I would have the opportunity to see her. If we choose to, the rest of our family could see her briefly. She then would be transferred to Cardinal Glennon. We would not have the opportunity to hold her.
I dealt with lots of emotions through the next few months. I considered changing Lucie’s name in case she didn’t make it. I had picked out the name Lucie when I was 10 and didn’t want my Lucie not to survive. I contemplated not having any baby showers, but ended up having 6 different ones. I told everyone that I had no doubt that she would survive. Every day I feared that she wouldn’t live. I feared that I would never hold my child alive.
We toured the high risk hospital that we would deliver at. I am pretty sure we made the others touring with us uncomfortable and probably fearful. We asked questions about babies being intubated, babies being transferred to the Cardinal Glennon, would we be able to see the baby before they are transferred, and other questions expecting parents don’t want to think about. Maybe I should have asked these questions privately, but I was afraid I wouldn’t have the opportunity to ask later.
I was finishing up my school year and going to see FCI every 2 weeks. Lucie was thriving. The fluid around her lungs was still there, and there was still no explanation for it being there. Lucie was also developing hydrops (swelling under the skin), and I had developed polyhydramnios (excess amniotic fluid). At 34 weeks I had 41 cm of amniotic fluid when someone at 34 weeks should roughly have only 21 cm of fluid. It was becoming more difficult to breath and much more uncomfortable.
At 32 weeks, Lucie’s pleural effusion and hydrops was looking worse and I was admitted to St. Mary’s Medical Center until further notice. Dr. Vlastos was out of town and the other doctor told us to go home, pack a bag and then head to the hospital. They would call ahead to the hospital so they would be prepared for us. Traffic was horrible so we decided to go straight to the hospital and have my mother-in-law take care of my bag. We decided to have one last meal together before I was admitted to the hospital. There was a sandwich and cheesecake shop across the street from the hospital. The place was called Daddy’s and it felt fitting that we should eat there.
When we got to the hospital we were told they had no idea we were coming. No one was sure what they were supposed to do with us. Eventually, the hospital staff figured out who we were and settled me into a room. I was put on continuous monitoring. Being on continuous monitoring was so uncomfortable. I couldn’t move much or the monitor wouldn’t pick up Lucie. My sciatic nerve would shoot pain through my back and legs because Lucie was pushing on it and I couldn’t move out of that position. I was extremely tired, but sleep didn’t come easy. I could have my child at any time, but ideally we had to keep her put for another couple weeks. We didn’t want her to come prior to 38 weeks if possible. After 5 days in the hospital, I was told that the monitoring Lucie had been very reactive during all the monitoring and I could go home and do the same thing I was doing in the hospital.
I eagerly went home, had my last 2 baby showers, and relaxed as I much as I could. Twice a week I went to FCI to have a non stress test and ultrasound. I was getting pretty good at reading the ultrasounds and enjoyed guessing what things were with the ultrasound tech, Cindy. Lucie was staying stable.
At 35 weeks, she was even more swollen and to keep her in me for as long as possible I was readmitted to the hospital indefinitely. We had an EXIT procedure (a special type of c-section that gives the baby a better chance of survival outside of the womb) scheduled for 37 weeks. I was very nervous and sad to have an EXIT procedure because I would be under anesthesia and Ryan would not be able to be in the operating room with me. I had a great fear that Lucie wouldn’t survive long enough for me to wake up from the anesthesia.
For 4 days, I was on continuous monitoring. Once an hour I was able to get up to use the restroom and that was it. I was even bigger because the fluid kept increasing and Lucie was getting bigger.
I finally was given the okay to be moved to a different room and be on intermittent monitoring. I was able to move around my room and once a day Ryan was able to take me on a wheelchair ride. We visited the chapel and prayed or we would go outside and sit next to a waterfall in front of the hospital.
A week before our planned EXIT procedure, I was given the best news. Lucie seemed to be doing so well and things weren’t getting worse that we could have a traditional c-section. We were so excited that Ryan could be with me in the operating room. We now had a new plan.
June 30th I couldn’t sleep. I was uncomfortable, I had to pee every half hour, and I was nervous. The transport nurse Mary came to pick me up at 4:30 a.m. She told me that she was the only person there only for me. Everything I needed, she would take care. I was wheeled to the ambulance and Ryan drove to Cardinal Glennon separately. I will never forget the feeling of my belly swaying back and forth with every turn in the ambulance.
I was ushered in through the ER and wheeled to FCI. I was ushered into the ultrasound room to be prepared for surgery and for one last peek at Lucie. My family joined me for hugs before the big moment. And then it was time.
Ryan walked with me and waited outside of the OR while I received my epidural. The nurses from FCI and Mary held me while I received my epidural. I was laid down and Ryan was finally able to join me in the OR. Ryan held my hand the entire time.
Before Lucie was delivered, the fluid from around her lungs need to be removed. Dr. Vlastos used a needle to remove some of the fluid, but not all of the fluid was removed. It’s funny looking back at what I remember from the c-section because I really don’t remember most of it. One thing I do remember was when they broke my water I heard it splash and saw it come over the curtain. I was later told that I had 2 gallons of amniotic fluid.
The moment Lucie let out a cry, I couldn’t have been happier in my entire life. The moment she let out her second cry, I lost it. Ryan was standing next to me holding my hand and watching Lucie be born. Immediately, Lucie was rushed out of my OR room to her OR room to be intubated. It felt like ages before Ryan was given the okay to go see her. In reality, it was only about 15 minutes.
Dr. Chatoorgoon took Ryan over to see her. He was able to hold her hand and take pictures to show me. The first time I saw what Lucie looked like was in a picture. She was big. She weighed seven pounds fourteen ounces. After a little bit the surgery team brought Lucie over for me to see. I held her hand and cried. I told her I loved her. And as quickly as she came in, she was taken away again.
After I recovered, I was brought up to the NICU on a stretcher. I don’t remember much of the half hour I spent with her. She was doing really well and her NICU room was crowded with Ryan and me and our family taking turns coming in to visit with me and Lucie. Many pictures were taken. Looking back on it, it was probably way too over stimulating for her. I didn’t want to leave her, but I knew the EMTs actually had other places to be and couldn’t stay all day in the NICU with me.
Going back to the high risk hospital was one of the toughest things I ever did. The other toughest thing I did was go home without my baby.
The first day I pumped every 3 hours and slept. Ryan spent the entire day with Lucie and came to the hospital to be with me that night. He helped me sit up to pump, clean my pumping tubing, and do anything I needed that I couldn’t do stuck in bed.
That night I was awakened by my phone ringing. I tried to wake Ryan, but he was exhausted from the day and I don’t think I really tried very hard. It was the NICU calling to give me an update. Now a call from the NICU in the middle of the night is not a good update- ever. What makes it worse is when you are on morphine and really have no idea what they are talking about. I had no idea what a good O2 level was and what a bad CO2 level was. I hadn’t spent the day in the NICU. I had spent the day in my hospital bed, away from my child, recovering from major surgery, and praying that I would be able to spend time at the NICU the next day.
When Lucie was born, she was put onto a traditional ventilator. At her 10:00 blood gas, he CO2 levels were high meaning her body wasn’t pushing the CO2 out of the blood stream, so they switched her to an oscillator which gave her lungs an opportunity to expand. I was also told things weren’t looking good. Chances were she would be on ECMO by morning. ECMO is a heart and lung bypass machine which would give her heart and lungs the time to heal without being used to sustain her life. To me ECMO was what I wanted to avoid at all costs.
I called in the morning to check on her, and she was doing much better. Her CO2 levels had come down and she was stable. I wasn’t able to go visit her until the afternoon. When I was wheeled into her room it was a completely different feel than the day before. The room lights were dimmed as low as they could be and still be on. The oscillator was extremely loud. Her entire body moved with every breatht he machine took for her and since it had her breathing 600 times per minute, she was shaking constantly. Lucie had her bonding baby covering her eyes and ear muffs covering her ears. I was completely overwhelmed. I was scared to touch her at all, but I longed to hold her hand more than anything. I sang to her, I read to her, and I stared at her while she lay there sedated.
On July 3 they switched Lucie back to a traditional ventilator to see how she would do. When we left for the evening she wasn’t releasing the CO2 as well as they wanted and we were warned that she may be put back on the oscillator in the middle of the night. After her 10:00 blood gas she was put back on the oscillator.
After four more days on the oscillator she was put back on the traditional ventilator and did well on it. Her oxygen levels were in the 90s, which was excellent. At 9 days old I was able to hold Lucie. It was quite a production because of all of the tubes. I never wanted to let go of her, but I barely was able to hold her.
She had her repair at 10 days old. Remember how I said that leaving her at the hospital and going home were 2 of the worst days of my life, well her surgery day tops that list as well. We went into the hospital early so we can have some time with her before the surgery team came to prep her for surgery. We talked with her and prayed with her. Judy from pastoral care came to pray over Lucie with us. Judy will forever be special to us.
The surgery team came in to prepare her for surgery around 7:00 a.m. They had to disconnect her from the ventilator and manually bag her to keep her breathing while the transferred her to the operating room. Everything that was sustaining her life had to be brought with her to the operating room.
When Lucie was wheeled out of the room Ryan just held me as I cried. The room that had been filled with machines and our daughter was now empty. I had to wait and trust in a team of people I barely knew to save my daughter’s life. We waited 4 and half hours for Lucie’s surgery to be finished.
Lucie’s hole was larger than the surgeon had anticipated. He tried to repair it without a gortex patch, but the repair started to rip before he had finished sewing it together. He used the gortex patch to repair the hole. Our surgeon felt very confident that everything went excellently.
The night of her surgery I stayed at the hospital with her. She was on fentynal for the pain, but she was experiencing more pain than normal. It broke my heart because you could see the pain in her eyes. I’m so glad that I stayed that night because her nurse wasn’t as familiar with Lucie and didn’t know Lucie’s normal heart rate and blood pressure. I was able to tell her that Lucie’s heart rate was high and she was in pain.
It only took 9 more days for Lucie to begin breathing completely on her own. During that time she continued to heal and began to become more awake and active. She was extubated at 19 days old. She tried over and over to extubate herself before the doctor came in to do it. Once she was taken off the ventilator, she was put on a high flow nose cannula. It blew forced air into her nose to ensure that she was getting enough oxygen.
Her weak little cry was the most beautiful thing I ever had heard. Ryan came by the hospital on his lunch break just to see her without a tube down her throat. We were overjoyed. Now all we had to do was get her off the nose cannula and get her eating. Lucie was stronger than ever at this point and was off the nose cannula in a few short days.
Lucie went home July 29 without any respiratory support or feeding support. I was thrilled. She has been fortunate enough to not have any issues that have landed her back in the hospital since we brought her home.
Our reality is very different than other CDH families. Our reality may not be the perfect beginning. Our reality may have been very different than what I envisioned when I dreamed about becoming a mom, but our reality is a dream come true. Our reality is that our daughter survived.
Mr. Sparks, thank you for reading my daughter’s story. I hope she inspires you write about this reality that we have all faced. Some of our realities end with a child coming home healthy and happy. Others have their reality end bringing their child home on much needed medical support and happy. Others have their reality end by not bringing their child home. This is a reality I pray that can be stopped. The only way to stop it is for more funding for research. The only way for more funding for research is for more awareness. Please, Mr. Sparks, consider writing about a CDH family in one of your upcoming novels. These are love stories like none other.
Mom to left sided CDHer Lucie born 7/2013