Christian's Story - Congenital Diaphragmatic Hernia (CDH)
Our journey with congenital diaphragmatic hernia began at our 20-week ultrasound. The ultrasound tech was steadily talking to us and describing our baby’s body parts. It was a boy! Then she became silent as she moved the wand over my belly and kept checking the chest area of our little boy. Our joy over the news of having a little boy was very brief. Quickly, we became panicked by watching her face. She concluded the ultrasound, said that she needed to speak with the doctor and to wait just a moment before leaving the room, because she wanted to get his opinion on something. After she left the room, my husband and I looked at one another, puzzled. I am not an ultrasound tech, but I am somewhat familiar with the inside anatomy of a person and couldn’t help but notice how she took extra notice of the area around his heart. After the doctor returned and took a look, he confirmed her suspicions of an abnormality in the chest.
We were then referred to a high risk doctor and had another ultrasound scheduled within the week. After meeting with the high risk doctor and receiving our second ultrasound, we were told that our baby boy had one of two things going on. He either had a cyst growing on his lungs or a rare condition called congenital diaphragmatic hernia. If it was just a cyst, then it would be better to deliver in Birmingham, AL. But if it was CDH, then Gainesville, FL would be the way to go. Either way, our child would require surgery. Due to his size at 20 weeks, they could not tell for sure. But as he grew, we would be able to confirm which diagnosis and go from there. We were then asked if we wanted an amniocentesis to check for other abnormalities. I opted to have the amnio and the results came back fine.
My son was our first child. I am sad to admit that I never got excited about my new baby’s arrival. I cried a lot during my pregnancy. I didn’t decorate a nursery for him. I didn’t even want to buy him a crib for fear that I would have to come home to a baby crib, yet no baby with me to put to sleep. As with most CDH pregnancies, I had polyhydramnios (extra amniotic fluid) and had to have an amniotic fluid reduction done just to be comfortable enough to breathe during my 8th month of pregnancy.
Two weeks before my due date, my husband and I relocated to Gainesville, FL. My son was delivered via C-section on the evening of January 31, 2006. We heard 2 small cries before he was whisked away with a team of doctors and nurses to be intubated. It all happened so fast. I was then wheeled into a recovery room as my husband went out to talk to family members. Alone in recovery, I tried to recall my son’s face. “Would I know him if I saw him in the NICU with all the other babies?” I thought to myself. About 30 minutes after delivery, my husband came in to check on me. He had decided on a name for him as well: Christian Peter, which means strong rock. He then handed me a picture of our son that was taken by a very nice volunteer with the March of Dimes. I clung on to this picture for the next 24 hours as I lay in my bed, unable to visit my sick baby boy.
The next few weeks were a busy blur. I remember finally getting permission to walk to the NICU III to see my baby boy after 24 hours away from him. Nothing prepares you for seeing your newborn with tubes and wires everywhere. I wanted so badly to hold him. All I could do was place my hand on his head or hand, but had to be careful not to stimulate him. We had to talk in a whisper and camera use was limited, due to the stimulation from the flash.
We were told Christian had a mild left congenital diaphragmatic hernia, with only intestines and spleen located in the chest. He had some respiratory failure, which they tried to help with a high frequency oscillator and nitric oxide, but that wasn’t enough. At just 2 days old, due to worsening respiratory failure, he was placed on venoarterial ECMO. He stayed on ECMO for 10 days. He also had PPHN, which stands for persistent pulmonary hypertension in a newborn.
Let me just say that witnessing a baby on ECMO is not a pleasant thing. His whole body was really puffy and lumpy. Whenever he did open his eyes, they just rolled around in his head as if he was really dizzy. We were afraid he might have brain damage, since he didn’t seem to be able to focus his eyes on anything. But in hindsight, I know that was only because of all the medications they were using to keep him sedated to prevent him from pulling at his ECMO tubes (which he did try to do a few times!).
Christian's surgery took place when he was 16 days old. Dr. Kays and his team did his repair with a Gore-tex patch. When they wheeled Christian to the NICU III, we noticed he looked sort of gray. After a few hours of close observation by the doctor, he noticed our son’s abdomen began to swell. They confirmed there was internal bleeding and they rushed him back to surgery to find and control the bleed. Our whole family was a wreck waiting for news from the doctor. We paced by the elevator doors, waiting for his return. Thankfully, Dr. Kays found the bleed and was able to stop it.
In the days following surgery, Christian had to have chest tubes placed on both sides of his chest due to pleural effusions. He had a barrage of tests and scans during his stay. He was weaned off the ventilator at 26 days old and then placed briefly on CPAP for about a week. After CPAP, he was placed on a nasal cannula. Up until this time, I had not gotten to see my baby’s face free of tubes and tape. It also wasn’t until he was on the nasal cannula that I was able to hold him for the first time. He was 6 weeks old when I first held him. It brought me such joy!
Time seemed to slow down once we moved from NICU III to NICU II. Our biggest goals were to get him to tolerate his feeds, gain weight, and keep his oxygen saturation levels up. In NICU II, I was able to be more hands on. I could feed him, change him, and dress him (unlike in NICU III). Finally, after a very long 57 day stay in the hospital, we were discharged with supplemental oxygen and medications for fluid retention and reflux.
Things went rather well once we got home, though I was constantly checking to make sure he was still breathing and wasn’t strangling himself with the nasal cannula. He only used the supplemental oxygen for the first month when we got home. We did have issues with finding an understanding pediatrician who would listen to the request of our pediatric surgeon, Dr. Kays (to keep our son on supplemental oxygen to help his lungs).
I was hypervigilant about keeping germs away from him. I refused to take him out in public until he was almost 6 months old. I remember having to fight with the doctor and insurance about getting him an RSV immunization shot when winter rolled around. Nobody seemed to understand how sick he was and could get with just a cold. Due to his medical issues, I was afraid to put him in daycare for fear of him getting sick and having to go back in the hospital. I ended up quitting my job to stay home and take care of him.
We continued traveling to meet with our pediatric surgeon yearly for the first 5 years of Christian’s life. That was how we were able to spot the reherniation when he was only 3 years old. We went back to have Dr. Kays and his team repair the reherniation. It turned out that the Gore-tex patch that they had used, had come away from the back wall of his chest and it was an easy fix. Dr. Kays noted that the muscle tissue of the existing diaphragm had fused together with the patch rather well on the other 3 sides of the repair.
We are finally down to visits every 3 years, with our next visit scheduled when he turns 12. Christian has not had any big issues other than the reherniation. He doesn’t have to take any medications anymore. His only issues are getting winded easily, eating smaller amounts of food at a time (he grazes when it comes to eating in order to prevent puking), immediately needing the restroom after eating, issues with weight gain, and seasonal allergies. We will always be worried about possible reherniation as he grows. We also worry about long term side effects that he may have from ECMO, but it all is an unknown path.
Christian will be in the 5th grade this year. He is 10 years old. He is an active child. We joke about his energy level because he can’t seem to walk anywhere; it's always a jog. Over the years he has taken tumbling and karate, played baseball, and has been active in Cub Scouts and 4-H. He excels in school, making A’s and B’s. He was recently inducted into the honor society, math team and gifted program at school. He is a very curious child and has a love for life. I can’t wait to see what his future has in store for him. I hope that those reading this can find hope for their child.
