This was written for a journalist, so it's not very emotionally written - mostly just the timeline of our story:
On March 9, 2011 , I was 20 weeks pregnant. At the Anatomy ultrasound, the doctor told me only that the baby’s lung looked echogenic or bright and she wanted me to have a second doctor look at it at the hospital the next day. She called me later in the evening with an appt scheduled for the next evening. I did some research on echogenic or bright lungs on ultrasounds and came up with a few possibilities as to what it might be – I was scared.
The next day - March 10, 2011 – I went to a private hospital, with my husband, Y. We had quite a long ultrasound and the doctor explained that the entire right lung looked to be covered in a tumor/cyst. It was a microcystic lesion called CCAM type III. The baby was at risk of developing hydrops – heart failure – and dying in utero. If the baby survived pregnancy, it would have to have surgery to remove most of or the entire right lung. The doctor brought in several other doctors to look at the ultrasound – one of them visibly gasped at the sight of it. They gave us no further information, no prognosis, but told us that we had less than 4 weeks to decide if we wanted to terminate the pregnancy. I scheduled a fetal echocardiogram for the next day, a follow up appt with my OB for the following Wednesday and another ultrasound at the private hospital two weeks later.
The echocardiogram came back completely normal. Although the cyst was pushing the heart further left in the chest than normal, the baby’s heart was anatomically fine.
For the next 5 days, I did nothing but research. After reading practically everything published online regarding CCAM, I was scared, nervous, but also hopeful. I found a facebook group called Mums of CCAM babies that completely changed my outlook. There were mothers all over the world in the same situation as me and mothers who had been through it all. Some of their babies were born with no problems breathing and after surgery were just fine. Finally, I had some hope. Still, our case was pretty severe because at the diagnosis, the cyst covered the entire right chest cavity. The risk of hydrops is measured by a measurement called CVR (cyst volume ratio – calculated by the volume of the cyst/head circumference). At a CVR of 1.6, the baby had an 80% chance of developing hydrops. We were at 1.4 and the cysts typically grow until around 30 weeks gestation.
I went back to my OBGYN on Wednesday, March 16th. She asked me if we had decided what to do with the pregnancy. I said that we were going to continue the pregnancy. She pressed by asking if my husband agreed with me. I said yes, we both agreed that we were not going to terminate the pregnancy. I asked several questions about CCAM and could understand that in the week that passed, she had done no further research on CCAM and that I knew more than she did. She had still not given me any sort of prognosis other than it may or may not be fatal for the baby. She then told me that we should do another ultrasound in her office to check if the CCAM had spread to the left lung. I told her that in all of my research, I had never read of the cyst spreading. She became quite defensive when I challenged her knowledge. So she began an ultrasound, and pointed to the lung area, and showed me that yes, it had spread to the left lung. I didn’t exactly believe her, but I was still worried that I had missed something in my research and that It could spread. I had to wait one week for our next ultrasound at IASO to find out if it had spread. Before I left, she told me that if we did continue the pregnancy, we should probably have the baby at Elena Hospital (where she does not work) because they have the best intensive care unit for babies in Athens. My overall feeling when I left her office was that she didn’t want to have me as a patient if I chose to continue the pregnancy. I knew I wouldn’t see her again.
On Wednesday, March 25th, I went back to IASO for a follow up ultrasound. The CCAM hadn’t grown much, but the baby had, so the CVR was a bit lower. It was still pushing her heart left, but other than that she was coping well. Again the doctor asked us if we had decided whether we were going to continue the pregnancy. We said that yes, we were. Again, we were give no information about CCAM from the doctors. All information that we knew was from my research alone. Our report from that ultrasound said that they had advised us to terminate the pregnancy, but that we were choosing not to terminate.
At this point, I started researching hospitals around the world with experience with CCAM. I was checking into Kings College in London and Children’s Hospital of Philadelphia (CHOP) in the US. At the same time, we started searching for another doctor in Athens that had some knowledge and experience with CCAM. We found a doctor to help us. When Yianni told him our story so far, he said it was criminal to suggest termination of the pregnancy. We went to see him for an ultrasound. Again the CCAM hadn’t grown, so things were looking a little bit more positive. He said that if we had the chance to leave the country for the remainder of the pregnancy and birth, we should do so.
I scanned and emailed all of my ultrasounds to CHOP for evaluation. They agreed that my baby was at great risk of developing hydrops and that if I chose to go there, I needed to do so in the next week or so. If the baby developed hydrops, I needed to be there for fetal surgery. And 24 – 28 weeks was the period of greatest risk. I booked my tickets for Philadelphia. I was going to have to leave one daughter the day before her 5th birthday and leave my other daughter a month before her 3rd birthday. Meanwhile, my mom, in Denver, Colorado where I am from was doing her own research. Through the facebook group, she found another Greek family who had used a doctor in Denver to perform surgery on their daughter for CCAM in 2009. My mom called and left a message with Dr. Rothenberg, a pediatric surgeon in Denver. Three days before I was set to leave for Philadelphia, Dr. Rothenberg called my mom back and discussed my case . He told her to have me contact Dr. Porreco, a high-risk OBGYN and Fetal Medicine Specialist. I called and left a message at Dr. Porreco’s office and within 15 minutes, he had emailed me back requesting my ultrasounds. I emailed them to him and he responded immediately, saying that they would take me as a patient and not to worry because CCAM Type III’s often shrink in utero. I changed my plans, and flew on to Denver on April 2nd.
From the time I arrived in Denver, I had ultrasounds every two weeks. The CCAM grew hugely. I requested an experimental steroid treatment that could possibly shrink the CCAM, and my doctor agreed. At 28 weeks, the CVR was 2.6. The baby’s heart was pushed completely to the left rib cage and the left lung didn’t have much room to grow. But the baby was still tolerating everything well. There were no signs of hydrops. At 30 weeks, the CCAM started to shrink. Two weeks later, it shrank some more. At 34 weeks pregnant, they could only find a 1.8cm x 1.2 cm lesion.
At 39 weeks pregnant, my water broke. My husband was scheduled to arrive from Athens 3 days later. I had a vaginal delivery and my husband watched via Skype on the computer. We had about 7 doctors and nurses from the NICU in the room waiting for the baby. At 6 am on July 17th, 2011, Thalia was born. She was 3210 grams and healthy. They put her on my chest for a few moments before examining her. She did have some rapid breathing at birth – transient tachypnea, so they took her to the NICU. She required CPAP forced oxygen for the first 24 hours of her life, but after that, she was fine. The transient tachypnea was unrelated to the CCAM, but most likely because my water broke more than 24 hours before she was born. She had a chest x-ray after birth and there was a small spot of CCAM visible. At 2 days old, she had a CT scan. The CCAM was visible on the lower right lobe only. I took her home 3 days after her birth and scheduled her surgery for September 15th at 8 weeks old.
She had a thoroscopic right lower lobectomy. Two days later, she was released to go home. One week later, she had a complication from the surgery – she had developed an air leak. We were back in the hospital for another week waiting to see if the leak would heal itself. It didn’t heal, so she went back in for surgery exactly two weeks after her first surgery. After a 45 minute surgery, the leak was closed. Two days later we were released from the hospital. Before leaving Denver, we had a chest x-ray to check her progress. In about a month’s time, her remaining right lung had already expanded to fill the entire lung space. The pathology report came back as not CCAM at all, but another type of pulmonary malformation called a Bronchopulmonary Sequestration (BPS).
Thalia is now a perfectly healthy 9 month old baby. She requires no further follow up. She will have completely normal lung capacity and will not be limited in her life in any way. She can play sports, she can play instruments, she can do anything she wants to do.
My overall feeling from this experience is anger toward the doctors here in Athens. Apart from one doctor here, we were pushed in every way possible to terminate the pregnancy. They didn’t offer us any prognosis or hope at all. And the OBGYN that I had the first 20 weeks lied to me, saying that it had spread to the left lung. I feel like the doctors played to our fears as parents and painted a very bleak picture. The nature of CCAM BPS is that they grow until about 30 weeks gestation and then shrink. Not one doctor here in Athens told me this fact. I was told it looked like her entire right lung was cystic and that she would probably lose an entire lung. It is extremely rare (4% or so) that more than one lobe would be affected and I’ve come across one case where three lobes were affected in all of my research. My pregnancy and my baby girl’s journey are really typical of a CCAM pregnancy, birth and surgery – we weren’t lucky, we were typical. I get so angry when I think of how many pregnancies were ended in cases like ours. So many lost babies because the doctors push for termination of the pregnancies – and don’t offer parents any information, prognosis and hope. It’s estimated that CCAM affects 1 in 25,000 pregnancies. That’s roughly 10 cases in Greece per year. How many of those pregnancies were terminated?
I know that we want our babies to be perfect – and no one wants to have their baby go through surgery. It’s a heartbreaking, terrifying ordeal. But aren’t our children worth it? Isn’t it our job as parents to do anything for our children? It meant that I had to be separated from my two oldest daughters and my husband for a few months. But we now have a healthy baby girl who brings so many smiles to our lives. We are lucky to have her.
