The Bump in the Road

This entry is a bit of an aberration because, well, things felt like they fell off track. I haven't posted in a while because I had a lot to emotionally process and go through. With the amazing support of my somehow perfect husband, our families, and our closest loved ones, we got through the story you're about to read. I say "got through" as if it's done. Really, it's not. But where we are now is so far from December 10. Hm, December 1o. I waited for that day for so long, I wrote about it the day before, and then it brought this. Here are excerpts from emails I wrote sharing what was going on with our families and a very small group of loved ones.

"Hi there everyone,

Well, a lot of news to share, some great and wonderful and a bit of difficult news to share.

Nico and I learned at our ultrasound this morning that we are having a GIRL! It was so wonderful to see her during the ultrasound. She was moving so much (no surprise since I've been feeling her move a lot over the past few days) and she even waved to us! She looks beautiful and it was so great to see her on the ultrasound. She is growing fine and she's just the right size for her age--12 ounces. Nico was predicting girl but I was so sure she was a boy! Smile.

During the ultrasound, the doctor noticed the development of two cysts in the baby's chest. This is the first time they were detected. Based on what he saw, there are two possible conditions that could exist: congenital cystic adenomatoid malformation (CCAM) or congenital diaphragmatic hernia (CDH). The doctor says treatment options are available for both conditions, and that both conditions are not very uncommon.

Based on what I've learned so far from the doctor and through a short internet search, the first condition CCAM refers to development of the cysts in the chest which are interfering with development of the lung. It seems that in most cases, the condition is monitored through pregnancy and then surgery is likely soon after the baby is born to remove the cysts. If the cysts grow too much during pregnancy, the doctor can drain them or do surgery during pregnancy. In a small percentage of babies with CCAM, there is one condition called hydrops (blood flow is interrupted and blood moves throughout the baby's body) that can develop that can cause great danger for the baby and can cause serious health problems for me so I know that they will have to do close monitoring through the rest of my pregnancy. The baby may have to be put on a breathing tube soon after she's born until surgery to help her breathe. About 90% of babies who have CCAM have no problems after the surgery. It is monitored while the baby is young, but the prognosis is very, very good.

Here is some more info about CCAM.

Type III congenital cystic adenomatoid malformation (CCAM)

http://fetus.ucsfmedicalcenter.org/ccam/learn_more.asp#a2

http://www.childrenshospital.org/az/Site2192/mainpageS2192P0.html


The more serious condition is called congenital diaphragmatic hernia. In this condition, the diaphragm does not develop properly and there's a hole. Then when the intestine is developing it moves into the chest and it interferes with development of the chest organs. There are surgical treatment options for this condition but the prognosis is not as good as about half of babies with this condition don't make it past a few months old. I feel very sad writing this but I'm going to work hard to be honest with myself about the possibilities. The doctor told us that this condition was more concerning, but that there are treatment options so no matter what we learn is true, we have options.

Here is more info about CDH:

Congenital Diaphragmatic Hernia

http://fetus.ucsfmedicalcenter.org/cdh/

http://emedicine.medscape.com/article/978118-overview

With all of that said, the doctor said he's not sure which condition the baby has because the baby was moving so much. :) One image from the ultrasound appears to suggest one condition while another image suggests the other condition. Other than the cysts though, the doctor said that they baby looks great. Her heart looks strong, her brain is fine, her limbs look great, everything else is in place. The doctor even said everything else looks "perfect."

At this point, I will have to go through a one day of fetal testing at the Children's Hospital of Philadelphia in the next 2 weeks. The doctor said it's a long and difficult day where they will do another ultrasound, MRI (which is fine for the baby), a fetal echo, and then Nico and I will have a team meeting with a surgeon, maternal fetal medicine expert, and case manager. After these tests, they will be able to learn which condition our little girl has and we can discuss options. I also will come in for another ultrasound in 4 weeks with my current prenatal medicine doctor.

The great news for us is that CHOP is one of the top hospitals in the country for fetal surgery and medicine. The doctor told us that he has great confidence in their skills, and the top surgeon in the world for fetal lung surgery works there so we already have a wonderful team right in town. It appears that my dreams of having a natural childbirth in a normal way may not come true, but I'm willing to deliver anywhere that's best for the baby. I know that the place I was already planning to deliver (Pennsylvania Hospital, which is affiliated with CHOP, both under the University of Pennsylvania) has a NICU unit so that place might still be an option. I will do whatever they tell us is best.

Regardless of what happens, Nico and I will make the best decisions for us and for the baby. Nico reminded me that anytime in life, you deal with hard things and often times you deal with it when you are older but sometimes you deal with it when you are young. I feel like our baby is mighty and I'm gonna stay super positive. She kicked me twice while I was writing this email so I think she's sending us a message. :)

We appreciate your love and support. This will be hard but we'll be okay. We are still so excited for our little baby girl!

Love you all so much, xoxo, amanda"

Regardless of my best attempt to think positively after this appointment, I have to admit, I fell into a bit of serious despair. It was shocking to have such utter joy at the pronouncement of the sex of our baby (a little girl! what a surprise!), and then just minutes later the deep descent into a sad, fearful unknown. I remember when the doctor said he saw something cystic--I hardly reacted because I thought he meant on me, an ovarian cyst or something of the like--and honestly, I didn't flinch. That didn't matter. It was when he replied "Oh no, not you, on the baby" in a don't worry kind of tone that I felt my chest heave, my heart crumble. I cried the rest of the day, stuck at work, and I cried every day the following week. I cried at least every hour. I felt so unlucky, so angry, so helpless, and so confused. At times, I felt like I gave up. I thought I failed, I created a poor home for our baby. Why us? So deeply sad, my body showed the effects: I keeled over in stomach aches at least once daily, and had to alter daily plans to accommodate how much of normal life I could tolerate.

I wished I could make my belly look small, so no one would ask about my pregnancy. I dreaded enduring faces of constant pity hanging on others' heads. I didn't want this pregnancy to have an asterisk. I abhorred the idea of adding more entries to this site, I wanted to just shut it down and let it go away. And I tried so hard to not be so overwhelmed with sadness. More and more is being learned about the detrimental impacts of the stress hormone cortisol on fetuses in the womb, and some of it points to lasting effects. I didn't want to hurt our baby, or make things worse, by feeling so upset.

I couldn't help but imagine another reality: I thought our baby might be born and then die, if she was ever born, and I wondered what scary choices awaited us. Our doctor told us that some parents, even at this unknown place, elected to terminate. He discouraged us from doing anything like that without more information, but even his words, every one of them, felt like single punches to my gut.

At moments, I wanted a do-over, a new start for our baby, to rewind conception day and still somehow have our same wonderful baby just start one day later, because somehow, that would make all the misfortune that may await disappear. My husband hugged me constantly, he reminded me that we still had hope, my parents told me that life brought challenges for people at all phases of their lives and infancy was no exception. I sobbed at the sight of girl baby clothes, I felt longing when I saw babies, I felt jealousy when I saw other pregnant women.

Besides the love and support of the wonderful people in our lives, I was comforted by the feeling that my baby felt mighty--I felt like she was strong, she moved so much, she surprised me, she was a special little enigma. I read stories and posts by women who elected to keep their pregnancies going even with very poor prognoses for survival. I was curious about how they felt and how they got through it--I mentioned it to my husband and let him know this was what I was thinking. If we got bad news about her ability to survive, and if she would be comfortable in the womb, I'd want to carry her to term to at least see her face for a second. Just to blow her a kiss and tell her: thank you, you are a wonderful child, you never let me down, I love you so much already.

I confessed to my husband over several days that I strongly wanted us to give her a name. In case something happened to her, I needed for her to have a name. On the way to our appointment of tests at CHOP, we chose a beautiful name for her.

"Hi all, here's a detailed update on the baby.

Nico and I are so thankful for all of your kind words of support as we dealt with a rough week of wondering what was wrong with our baby. It really meant so much to us to hear from you all.

As you know, a cystic mass was found in the baby’s chest at her 20 week ultrasound. At that time, the cyst was estimated to be either a case of CCAM or CDH, both of which are fairly serious conditions in which the cyst can interfere severely with lung and heart development and/or proper placement of lower abdomen organs like the stomach and intestines.

We were very, very excited to learn yesterday after a long day of tests at Children’s Hospital of Philadelphia (CHOP) that the baby has NEITHER of these conditions. Instead, her current diagnosis is congenital esophageal duplication cyst. Here’s all the info I’ve learned in the last 24 hours.

It’s a cyst that forms along the wall of the esophagus, and it’s called a duplication cyst because it follows the shape and build of the esophagus, almost like a copy of the esophagus. This condition is very rare—it only shows up in about 1 in 8,200 births. There is not a lot of research about it (most studies I have been able to find regarding detection and treatment are dated in the 2000s). It likely developed sometime during the 7^th week of pregnancy, and to date it is most common in white mothers who are older and having their first children.

Nico and I knew that even up until the end of the day yesterday that the doctors were having a difficult time figuring out what was going on. Before the doctor would meet with us, he said that he wanted the feedback of a newborn surgeon before talking to us. We were the last patients waiting, so we knew it was not a cut and dry case. In our meeting with the docs, they explained to us that it was difficult to diagnose the cyst because it is large. Usually, they appear smaller, and according to what I’ve read so far, they often are not even diagnosed until after the baby is born.

The prognosis for the baby is very, very good. Essentially, the baby will need surgery soon after she is born to remove the cyst. If the cyst is not removed it can cause respiratory problems and feeding problems, but cases show that once the cyst is removed, the symptoms are gone and there are really no long-term problems. The surgeon told us that she’ll likely have acid reflux, but that was the only long-term issue they mentioned.

It’s good for us that it was discovered so early. At this point, the plan is for me to report to ultrasound appointments every two weeks so they can monitor the size of the cyst. Half of these ultrasounds will be at CHOP and the other half will be at my normal maternal-fetal medicine doctor. Once I’m 34 weeks along, all of my prenatal care will be done at CHOP as they prepare for delivery.

Right now, the cyst is near the baby’s back so it is not interfering with development of her lungs and heart—which is GREAT news. They did a fetal echocardiogram yesterday and they said that her heart looks great. The monitoring will keep an eye out to make sure it’s size does not grow so large that it interferes with lungs and heart and to make sure that it doesn’t break into the wall of the esophagus. If it grows too large, they will have to drain the cyst before the baby’s born.

Another complication that they will be looking out for is called polyhydramnios, which happens when the baby can’t swallow enough amniotic fluid and there’s a buildup of fluid in the placenta. If the cyst interferes with the baby’s ability to swallow fluid, this condition can occur. I can go into preterm labor if this happens. They will be monitoring the amount of fluid in the placenta, and they can relieve some fluid if needed.

Because sometimes the cyst can cause early problems with breathing, etc, they want her to be delivered at CHOP. They built a brand new maternity ward there 2 years ago so that moms and dads could stay near their newborns who need advanced medical care instead of recovering at another hospital across town. This way, I’ll be able to see her while I am recovering in the hospital after she is delivered. AND they have a lactation unit so they will help me be able to feed her breastmilk very early on. Being at separate hospitals would make that almost impossible.

We don’t know anything yet about what precautions they’ll need to take during delivery, but they know that my earlier hopes were to have a natural childbirth. I’m sure they ‘ll have to do a fair amount of medical monitoring but CHOP has its own midwives and they made it sound like I may still be able to keep some elements of what I had hoped for as part of my delivery experience. Of course, if they tell me they want to do an emergency c-section, then I’m all in. I want them to do WHATEVER they need to do to keep the baby safe and healthy.

The delivery unit at CHOP looks really nice. And Nico will be able to stay overnight with me while I’m there, which is great. Each delivering mom has her own family room. Nico and I had a really great experience with the doctors and staff there. They were very attentive and helpful and emotionally present during a difficult day for us. They remembered to say things to keep our spirits up while we were in the tests and they understood when I just needed to cry. I think overall this will be a really great birth experience and we’re relieved to have such skilled doctors so close to home.

It’ll be hard to see the baby go into surgery so young, but there are only a few places in the country where folks could feel really confident about newborn surgery and CHOP is at the top of the list so we are in good hands. I already know from yesterday that the baby does NOT like MRIs (she was kicking up a crazy storm when I was in the machine) but the MRI area yesterday had at least two babies there so I know CHOP folks are skilled at helping newborns through it.

One of the gifts I took from yesterday, besides our great, relieving news, is that we got to spend a lot of time with the baby. We watched her on ultrasound for hours, and we got to see her on 3D ultrasound and bring home cute 3D pictures of her face and hands and little body. She is already a beauty, and we cannot wait to hold her.

We’ll be sure to keep you posted as things progress. We will probably have some tough moments ahead, but it’s what we can handle. Thanks so much Debby for reminding me of that—we are not given more than we can handle. Even though there are some concerns for her, this is so much different than the scary things that we imagined. We can handle what comes next.

Hearing from you all made both of us feel better along the way and your emails and well wishes were really helpful. You kept positive thoughts in our minds, I really needed that!

Mom and Dad, after we talked on vp on Sunday morning I felt like I could get through the day so much better. That’s what made me feel okay about posting on FB that we knew she was a girl. Before that, I was dreading anyone asking me about the pregnancy.

Much love to all and thanks so much. Hugs!

Xoxo, amanda"

The day was full of both laughs and deep cries. I cried through the fetal echocardiogram. Something about the dark room and the start of the long day just overwhelmed me. During the second test, the long ultrasound, we joked. We shared our prospective baby name, we talked about her like things were going to be okay. And during the MRI (oh boy, Little did NOT like that one, she kicked up a storm at those scary sounds), I felt like I was in a surreal place. It was hard to see so many sick children, but the way the staff treated them and us was reassuring. And we saw two infants while we were there, in hospital beds, being doted on by their parents and nurses, and I felt like a piece of our future became clear. That those next steps were going to happen because she was going to make it.

At the end of the day, we were the last patients in the waiting room. She really was a little special enigma, and it took a whole team of folks to figure out what her little body created. The docs honestly looked baffled all day. It wasn't until we walked into the final meeting of the day that they spoke with greater confidence.

I hugged a big box of tissues, but I didn't need a single one. Everything they were telling us was infinitely better than what I had imagined. Yes, she would need special monitoring and extra care and surgery in her first days, but she was going to be with us. We'd get to hold her. And I wouldn't steal all of her lifetime in my womb. The world would be open to her.

We even cracked jokes in the early evening meeting. When the doc told us he was impressed by the size of her cyst, I said "I'm not surprised that she's already impressing you." I couldn't believe I was wearing a coy smile, our David Bowie looking doctor sneakily smiled back. It was a moment of transformation.

And so, now, we have a new road. There was a bump, well, let's be honest, a detour. We're on a whole new road, with a new hospital, new prenatal care providers (I am switching all of my records this month to commence all of my care at CHOP), a new birth story, but lucky, lucky, lucky us, our same little baby girl. Little, we're with you. I hope that when you read this, you won't feel bad or sad, but just loved. And special.