11/ Aaliyah’s Story

Aaliyah was diagnosed with a large high-risk CPAM at the 20 week scan. After steroids didn’t slow down the CPAM’s growth, she developed hydrops and a shunt procedure was performed to drain the mass. She was born symptomatic, had surgery at 2 months old and is now a happy and healthy 20 month old! Her mom shares the family’s story.

During my first pregnancy, we learned at the 20-week ultrasound (at OHSU in Oregon) that my baby had a large, high-risk CPAM. We had gone into our appointment with so much excitement to find out if we were having a boy or girl. The appointment had instead gone in a very different direction, and we were urgently sent to get a fetal echocardiogram done with little-to-no information as to why or what was happening. 

Returning to our ultrasound appointment for another set of scans, we were finally told that our daughter had a high risk CPAM. The radiologist had given us a small description of what the diagnosis was, and we left with so many questions, and several Google tabs open as we tried to do our own research. Quickly learning that there wasn’t much information online, we turned to a Facebook support group.

Roughly 5 days after that ultrasound we returned to being seen at the MFM clinic. Unsure of how exactly it was going to go, we were feeling optimistic and hopeful. The ultrasound was done and we were brought into another room to speak with the OBs. They were incredibly sweet and gentle with us. The appointment ended with the OB suggesting that we have surgery asap for the thoracoamniotic shunt placement, even as soon as the next day. 

The nurses set up a virtual visit for me and my husband to talk with the fetal surgeon about our options and what his opinion was. I could feel us dissociating during the whole conversation. There was just so much information and we were in shock. 

The fetal surgeon gave us his recommendation of attempting the course of prenatal steroids before rushing to do the shunt placement. That day we left our appointment with 1 shot done and so many tears running down our faces.

This room became our meeting room for our weekly appointments; it began to give me a large amount of anxiety upon entering just from the appointments we were having. 

After one round of prenatal steroids, our baby’s CVR was 2.5 and she had developed hydrops. 

The surgeon made the recommendation to have fetal surgery the next day as we could now see that her heart was under severe stress. A thoracoamniotic shunt was placed with no complications. We were admitted for an extra day to keep an eye on the membrane wall and confirm that it stays intact prior to being discharged home. 

There were weekly appointments to closely monitor the CPAM. About a week later they had found that the shunt had been dislodged and the CPAM began to grow again. It got as high as 2.1 when the fetal surgeon recommended we place another shunt as they found hydrops a second time. 

Feeling overwhelmed with anger, sadness, guilt, and anxiety, we had decided to attempt another course of prenatal steroids before rushing into another surgery. Little did I know that a week later we had made the correct choice as the CPAM began to stabilize and then finally shrank to a CVR of 1.2 

Fast forward to being 36 weeks and at our weekly appointment they discovered that I had preeclampsia. I was sent to the emergency room. 

I had several blood pressures taken that were in the critical range. The doctors decided that I would be induced and placed on a magnesium drip. After nearly 24 hours of labor and 45 minutes of pushing, Aaliyah was born. During the pushing, her heart rate continued to drop significantly. The NICU team was there and immediately took her away when she was born. 

She was born symptomatic, needing CPAP breathing support for the first few hours of life. 

After a few days of being in the NICU she was discharged with no complications. Fast forward to two months later, we noticed that her breathing was hard and fast with tracheal pulling. A trip to the pediatrician sent us to the emergency room due to her oxygen being in the low 80s and her respirations ranging from 80-90. 

Upon arriving in the ER, a chest X-ray was done and an IV was placed. Several doctors came in to chat with us and let us know that her CPAM was trapping air and had grown to a massive size. We were quickly admitted into the hospital while we waited for her lobectomy. 

There was a mixture of emotions while we waited for the doctors to come and grab Aaliyah for her surgery. Her surgery went great but she was in a significant amount of pain afterwards. The nurses continued to give her pain medication, but we eventually realized that what she really wanted was to be soothed by being rocked in the crib. We were unfortunately unable to hold her because the chest tube was making it incredibly uncomfortable for her to move around. 

The following day they had removed the chest tube and everybody felt a huge breath of fresh air. She continued to thrive and did so well that she was discharged 2 days after surgery. She has continued to do amazing things and grow so strong. You would never know that she had so much happen so early in her life. She is now 20 months old, full of life and character, such a brilliant baby girl.