By Monica Cruz, mother of CHOC patient Tavik
When my fiancé Ryan and I found out we were having another baby, we were excited but also a little overwhelmed. Our firstborn son Raiden was barely a year old and very strong willed. We weren’t quite ready for another, but we knew we could manage. We had no idea the journey that lay ahead of us and our new baby, but today we are so grateful that he’s healthy and happy.
During an ultrasound when I was 26 weeks, my OB/GYN found a mass on the baby’s lung. They said it was likely congenital pulmonary airway malformation (CPAM). CPAM is a mass or lesions of abnormal lung tissue that forms during pregnancy. The mass or lesions can vary in size. It is usually only in one lung and does not function as normal tissue. We did two more ultrasounds to confirm the diagnosis, and then we were referred to a maternal-fetal medicine specialist (MFM).
Our MFM confirmed that our baby, who we named Tavik, had CPAM. The cause of this condition is unknown and only 1 out of 25,000 pregnancies are affected. Research shows that this abnormality is not related to anything the mother did or didn’t do during pregnancy. Some think it may be caused by genetics, but there isn’t enough research to confirm this. CPAM was previously referred to as congenital cystic adenomatoid malformation (CCAM).
There was still a lot of time left in my pregnancy, so Tavik’s lungs had more time to grow—but so did the mass. On the ultrasounds, we could see that his heart had been pushed off to the right side. This was a lot to take in and made the next few months extremely stressful.
Babies in the womb who have CPAM are also at risk for hydrops fetalis, or hydrops. When a fetal lung mass pushes on the heart and blood vessels, the heart has trouble pumping blood. If the heart can’t keep up with the baby’s needs, fluid builds up around the lungs and in the belly, a condition called hydrops fetalis. After the baby is born, the fetal lung mass can cause problems because it can get infected (pneumonia) or take up room so that the healthy lung lobes can’t fill with air. I had ultrasounds every two weeks until Tavik’s due date.
We knew Tavik would need care after he was born, so our MFM referred us to Dr. Peter Yu, a pediatric general and thoracic surgeon at CHOC who has special training and expertise in fetal conditions.
My fiancé and I went to CHOC and met Dr. Yu, who explained everything that was going on with the baby in utero, and what would happen after he was born.
The good news was that most babies with CPAM are born with no symptoms and can go home after a few days in the hospital. Surgery to remove the mass on the lung usually comes a few months later, when the babies are bigger, and better able to handle anesthesia. If CPAMs are not removed, they can cause breathing problems or serious lung infections, and sometimes they can even become cancerous later in life.
Dr. Yu was honest with us and educated us that there are some babies who do have complications at birth due to the CPAM and can need immediate surgery after delivery. Not knowing what that outcome would be was one of the hardest things I’ve ever had to process.
Dr. Yu was so knowledgeable and made us feel more at ease about our baby’s diagnosis. We knew that Tavik was in great hands.
Tavik was born on Nov. 15, 2018 weighing 7 lbs., 10 oz. and 21 inches long. We were so lucky and beyond happy that he came out without any complications, and that he didn’t need immediate surgery. Unfortunately, later that night when the doctors came back to check in on us, they noticed he was working extra hard to breathe. They weren’t sure if this was related to his CPAM. He was admitted to the neonatal intensive care unit (NICU) at the delivery hospital and was given oxygen and put on CPAP (continuous positive airway pressure). CPAP delivers constant air pressure into a baby’s nose to help the air sacs in the lung stay open and prevent sleep apnea.
During our NICU stay, doctors performed a chest X-ray and ultrasound to get a better picture of the mass in his lung. They noticed he also had an abnormal blood vessel carrying blood to the lung mass.
Tavik spent eight days in the NICU before he was healthy enough to go home. The doctors determined that his breathing troubles at birth were not caused by the CPAM. He just had some residual fluid from the C-cection.
Two weeks later, we had an appointment with Dr. Yu so he could meet Tavik and we could make surgery plans. A few weeks after that, Tavik had a CT-scan so Dr. Yu could see exactly what was going on inside Tavik’s lung. That gave Dr. Yu a more precise roadmap for surgery.
When Tavik was three months old, he underwent surgery at CHOC Hospital. It was scary to hand over my baby for surgery, but looking back now, everything seemed to go so smoothly and quickly.
During the three-hour surgery, Dr. Yu removed the affected part of Tavik’s lung and repaired the blood vessel. While surgery was in progress, a member of Dr. Yu’s team came out to the waiting room every 30 minutes to provide an update to Ryan and me.
When surgery was over, Dr. Yu came out to let us know that he had removed half of Tavik’s left lung, sealed off the artery, and everything had gone smoothly. He showed us photos of what he had been looking at through his scope during surgery. He did this to also give us a better understanding of what had been going on inside of our son. It was really neat to see.
Dr. Yu performed the surgery thoracoscopically, using minimally invasive instruments. Only three very small incisions had to be made in Tavik’s abdomen to remove the entire mass. Tavik only has three tiny incisions on his left side. It’s mind-blowing to think that you can safely remove half a baby’s lung through three tiny incisions. Since Tavik had this surgery as a baby, the remaining portion of his lung will be able to grow and compensate for what was removed as he grows.
Tavik spent only three nights in the hospital. The first two days after surgery were hard for Tavik; he was groggy from the anesthesia, and he made some sad sounds, which was a little heartbreaking for us to hear as parents. Thanks to the awesome rooms at CHOC I was able to stay with Tavik the entire time, both day and night. During this time, I stayed with Tavik, while Ryan and Raiden stayed close to CHOC at the Ronald McDonald House. We had stayed there during our unexpected NICU stay, and they invited us back during Tavik’s surgical stay. Without them, our family wouldn’t have been able to stay together and so close to Tavik.
By the third day after surgery, Tavik was alert, smiling and getting right back to his happy self. It’s seriously amazing how fast babies heal! I was a little nervous getting ready to leave on the fourth day. The doctors constantly reassured me by telling me that he was healing wonderfully and that he was in great shape to head home. They were right. By the next day, Tavik was doing even better—he had no pain or discomfort and his incisions were already fading. You would have never known that he just had a major surgery.
A few weeks later, we had a follow-up appointment with Dr. Yu, who confirmed everything still looked great and Tavik was now CPAM-free.
Although Tavik underwent a major surgery as a baby, looking at him now, you’d never know what he had been through. You can barely see his scars, and he’s growing like any little boy should—he’s in the 80th percentile for height and weight. His prognosis is great, and we look forward to a normal life with him.
My son is such a sweet, loving, happy boy with a little bit of spice to his personality. He absolutely loves music and always wants to play in the water. He has so much fun going on walks with his older siblings Leila and Raiden.
Tavik is our little miracle baby! He has kept us on our toes since before he was born and continues to do so today. He’s always making us laugh and smile.
We are so blessed and are so grateful that he is healthy.