Our Journey Begins

On November 27th, my husband and I attended our 20 week ultrasound appointment. I was so excited when I finally had the opportunity to schedule that appointment that I kept referring to it as my "gender ultrasound" which my doctor was quick to correct me on. It was the "anatomy ultrasound" first and foremost. In my mind, that was just a formality. No one expects to go into this ultrasound and hear anything other than positive feedback about their baby's health. For fun, I had even started a poll on Facebook asking friends and family to put in their guesses as to whether the baby was a boy or girl. I remember people posting that they hoped that it was a boy (since we already have a little girl) but that they would be happy with a healthy baby. Of course it was a healthy baby, right? Despite the trials we went through during my first pregnancy (preeclampsia, kidney stones, gall bladder attacks), we already had one perfectly healthy baby. Plus, this pregnancy seemed to follow my last very closely. I felt comfortable going in to this ultrasound.

I should also mention that while I was excited about the "gender ultrasound", we had not planned to not find out the gender of the baby that day but instead asked the ultrasound tech. to provide the sex in an envelope which we would pass on to a baker so that we could do a gender reveal photoshoot the following day. . .my husband's birthday. As a result, she appeased us by turning off the monitor while she was performing the ultrasound and taking her measurements. She did not speak the entire time during the ultrasound which was a little unnerving. However, when she finished her exam, she asked us if we would like her to turn on the monitor and only concentrate on the face and hands and such since we had brought our 2.5 year old daughter with us. Of course, we were thrilled to see our newest peanut up on the monitor. Even my daughter kept referring to her as "sister" and we had not even received the gender results yet. While we didn't end up finding out the gender at the "anatomy ultrasound", that IS the day that we learned that there was a complication with our sweet baby. The ultrasound tech. told us that she needed to run the exam by the doctor so that he could take a look at all the measurements and confirm that everything looked okay prior to sending us home.

I just remember the doctor walking in and pulling up a stool. He said, "I want to show you something" and my heart immediately dropped. Seeing my reaction, he said, "No, no. As far as things that I have to talk to people, this is one of the least worrisome." While that was a little bit of relief, I was still hanging on his first words. He opened up one of the pictures from the ultrasound and said, "This is the chest cavity." Immediately, my mind turned to heart problems. My mom and grandma both had suffered from heart disease and had endured bipass surgeries so I was sure we were dealing with a heart issue. He continued on. "This is the lung. See that dark white over there. That is not supposed to be there. I think that we are looking at something called 'pulmonary sequestration' which just means that this is a benign mass that is growing with the lung. It's actually lung tissue that is not functioning. There is also the possibility that it could be 'congenital lobar emphysema' or 'congenital pulmonary airway malformation' however I really think that the 'pulmonary sequestration' is the most likely scenario. I'm going to call your OB and she'll put in a referral for you to see a Perinatologist who will be able to provide you with a better idea of what you're dealing with." Upon finishing, he just stared at us blankly, waiting to see if we had any questions. I was in such shock that the words coming out of his mouth didn't seem to make any sense to me. It was all a blur. How could I possibly ask any questions when I didn't even understand the terminology? I had him write down the medical terminology for us and began to plan my research attack for the ride home. My husband and I both walked out of the ultrasound very quiet. I remember thinking to myself, "Did I do something wrong? Was there something that I didn't do that I should've done?" These are obviously very painful questions to ask yourself when you already know that there is nothing you can do NOW to change the outcome.

My husband had to return to work that day so I drove myself home, all of the while fighting back the tears. I did a lot of research the remainder of that day but still couldn't make sense of all of the information that was presented. My first response was to contact some of my nurse friends (one of which was a pediatric nurse) and ask them what they knew about "pulmonary sequestration" and if they could explain it in lamens terms. That proved to very helpful to me. While they may not have remembered exactly what it entailed, they were able to reference their old text books and ensure me that they believed this to be something that could be treated with surgery and that most babies go on to lead a totally healthy and normal life. These two friends were my angels that day. At a time when I was scared and felt helpless, they were able to provide me with a tiny bit of strength and reassurance and for that I will be eternally grateful to them. Based upon my research, I began forming questions that I would ask the Perinatologist once I met him. Will he/she have to have surgery immediately after birth or is this something that could occur later on in childhood? How dangerous is that surgery? What is the likelihood that this child will go on to lead a normal life? What are other associated side effects? What is our plan of attack? How will my delivery go? Will any extra precautions need to be taken?   By the end of the day, after explaining the circumstances to my mother and close friends, I was totally drained. I had cried so many tears and had so many unanswered questions. My mind was unable to shut off.

The doctor referred us to a Perinatologist at the naval hospital who we met with two days later (November 29th). When he walked into the room, the first words out of his mouth were, "So how freaked out are you?" That was putting it lightly. He started out by saying that he suspected it was a CCAM (a term that had not even been mentioned two days prior which really threw me for a loop) and that there were two different types that he would be looking for: the first would be mostly liquid which would be the most preferable out of the two. These type could potentially be drained and had a 90% survival rate. The second type, which is mostly solid, was a little bit more difficult, yielding an 80% survival rate. He also went on to say that CCAMs aren't "that rare". I would be the sixth case that he has seen in his entire career. Therefore, "it wasn't one of those things that a physician can go his/her entire life only encountering one case." Somehow, the fact that I was only the sixth case he had encountered was not very much of a relief to hear.

After starting my ultrasound, he confirmed that the mass looked to be solid based upon the color and he guessed that it would be classified as a Class III CCAM. He measured the mass and said that it was 2 cm by 1.5 cm. When I asked if that was large, he said that it is big but he has definitely seen bigger. This CCAM encompassed 25% of the baby's chest cavity. The only thing that did provide comfort was the fact that he kept telling me how good everything else looked. "This is a beautiful baby", he repeated over and over again. The heart looked great and strong and appeared to be in the right place. Apparently, with the growth of a CCAM, the heart can sometimes become crowded which in turn affects it's growth. The spine and diaphragm all looked good as well. The left lung looked great and the right lung even showed some good lung tissue. Nothing appeared to be impeded by the CCAM. He said that he wanted to order an MRI for me which would provide us with more definitive answers. Additionally, he said that I might be a good candidate to consider undergoing surgery in utero. It was a little premature to determine that at this point in time. He would have to consult with the pediatric surgeon (whom I have since scheduled an appointment with for next week) and see his thoughts on the risks versus rewards. Apparently, only six hospitals in the country preform this surgery in utero. Most of them are on the east coast, I believe. The closest to us is UCSF which is where he would most likely refer us to in the case that we decided to go this route. The thing that terrified me most about this surgery is that it HAS to be done before 26 weeks. I'm currently 20 weeks and 2 days so the timeline was very unsettling. On top of that, the Perinatologist told us that while they are able to do amazing things with these surgeries now and days, there is still a 37% chance that they can break your water during this surgery. We'd be having a baby right then and not knowing how underdeveloped it would be. On the other hand, if we wait, they would continue to monitor the CCAM's growth and make sure I don't develop something called "hydrops" which is ultimately signs of heart failure in the baby. I asked the doctor if anything could be done if the baby develops hydrops to prevent heart failure and he said that they can give the baby steroids. However, after further reading, it looks like hydrops is pretty untreatable.

 

During our appointment, the Perinatologist did reference another CCAM case that he had recently dealt with. This was different in that it consisted of twins, one of which had a CCAM that was large and encompassed 50% of the chest cavity and the other that was completely normal. They did not want to operate in utero because then they would also be putting the healthy baby at risk so they waited until the babies were born. Luckily, while monitoring the baby's CCAM through frequent ultrasounds, they were able to tell that the CCAM had shrunk significantly as the baby grew. They were born without complications and apparently the twins are currently nine months old and the one has not had surgery yet to treat it's CCAM.

 

Since our meeting with the Perinatologist on Thursday of this week, I started having sharp pains in my bladder that felt like the baby had lodged it's foot in it. . .only it lasted for hours. I started reading online and decided that it could possibly be a UTI so I made an appointment with my OB to have a urine test taken. It came back negative for a UTI but the doctor asked me if I was familiar with the signs of preeclampsia. I did suffer from preeclampsia with my first daughter, and had to be induced 3.5 weeks early, so I was very familiar with the symptoms. He asked me if I had experienced any headaches, which I have, so he ordered me to do a 24 hour urine test. Apparently, to my surprise, women with CCAMs have a higher risk of developing preeclampsia. Great! I thought I was in the clear with this one. Guess we will find out more when I turn that in on Monday.

 

Other than that, just waiting to talk to the pediatric surgeon next Thursday and have my MRI done on the 10th. In the meantime, the doctor suggested that I talk to a social worker in the NICU to have someone to discuss my feelings and stresses with. I'm not opposed to it because I'm guessing that we'll be getting to know the NICU pretty well over the next year. It's just a little odd for me because I've never talked with a therapist before EVER and now I'm going to be talking to her about something that I'm still processing. The only thing easing my troubled mind is that this woman may have worked with other CCAM mothers and have some insight into my feelings already. She would be impartial and not judge my thoughts as someone who has never experienced a health scare with their child may.