Layne’s Story – Part Three

The day after getting the news that Layne was sick we were able to get an appointment with the Maternal Fetal Medicine group my OB referred us too, which was amazing!  Usually getting an appointment would of taken weeks, but we were so grateful to get in so quick as no one was sure how much longer Layne could survive in her current condition.  Jeff and I decided to bring both our moms as we figured we would be hit with information overload and we wanted the extra set of ears.  

It was going to be a long and emotionally exhausting day. I was scheduled to have an ultrasound and then an echocardiogram to check the structure and function of her heart. After waiting for my name to be called, all four of us packed into a tiny room and waited for the ultrasound technician to begin.  After she started I intently stared at the screen hoping and praying whatever our OB saw the day before was no longer there.  I prayed so hard for our baby to be healthy, but you could feel the energy in the room.  Something wasn’t right, and you could see the concern in the technician’s eyes. She even called in for backup.  A second technician came in and took over.  After a long hour of little to no talking we wrapped up the ultrasound and I was transferred into another room to begin my echocardiogram.  During the echo the cardiologist hardly said anything.  I had no idea what she was seeing and I had no idea what I was looking at.  She was a hard read.  I was having a hard time judging her demeanor and had no idea if things were good or bad. 

After what seemed like hours of being under an ultrasound probe it was time for our last appointment of the day. We were scheduled to meet with a genetic counselor and the doctor to discuss the findings of the ultrasound and the echocardiogram.  All six of us gathered around a table and the doctor dove right into telling us her diagnosis.  She told us Layne had CCAM, Congenital Cystic Adenomatoid Malformation.  She told us that these cysts that were in her chest originated from her lung and they were so large that they had completely displaced her heart to the right side of her chest.  As if that was not enough devastating news, she also went on to tell us that Layne was in heart failure.  The cysts were so large and consuming so much of her chest that her little heart was severely compressed and working in overdrive to keep her alive.  They even made mention that they were not sure how it was still beating.  My heart broke all over again.  All of the emotions from the day before were resurfacing and we were a mess.  I could not believe our little girl was so sick.  I remember feeling half relieved that we finally had a diagnosis, but still so scared about how our journey was going to play out.  

After having a few minutes to process her diagnosis we had a million questions.  I wanted to know if it was something I did?  How did it happen?  What is the prognosis?   The genetic counselor went on to let us know that I did absolutely nothing wrong and I could not have prevented it.  It was a total fluke and only 1 in 40,000 babies are diagnosed with this condition.  She told us that it was not genetic and not hereditary, and the chances of it happening again were slim to none. Then the answer to my last question will stick with me forever.  Her response to what is the prognosis was “the outcome is grim, but there is still hope”.  I will never forget those words.  In that one sentence she gave Jeff and I all the hope we needed to fight for our little girl.  We were going to do whatever we needed to do to give her the best chance.  Terminating our pregnancy never crossed our minds, and when it was brought up we quickly shot it down.  Layne’s little heart was working so hard to keep her alive and we were going to do the same.  No questions asked.  

At this point in our conversation, we had been given the diagnosis and learned how severe Layne’s case was and all we wanted to know was what we needed to do to care for her.  I wanted to know what our options were and how soon we could start.  The doctor basically told us that since our case was so severe that there was nothing they could medically do for us.  She said that there was not a doctor here that would feel comfortable operating on me or the baby and without fetal intervention I would end up miscarrying.  I was so discouraged.  Here we are just being diagnosed with a serious fetal anomaly and there was nothing we could do about it.  That did not sit right with me. After lots of questions and tears, the genetic counselor said she knew of one hospital that would possibly take us as a patient, but that it was a long shot.  She said The Children’s Hospital of Philadelphia has an amazing Maternal Fetal Medicine group that works miracles and that would be our last hope.  Jeff and I agreed that we were up for traveling and our doctor said she would put in a referral and we should hear something by Monday or Tuesday of the next week.   

Just before leaving our appointment the doctor suggested I get a round of steroids.  The same round of steroids a mother would be given if she was suspected to be in pre-term labor to help strengthen the lungs prior to the baby being born.  She said Betamethasone has been linked to shrinking these cysts and it was a good preventative measure we could take prior to getting in with CHOP.  Without any hesitation, I was back in an exam room preparing to get shot one of a two round series.  Round two would need to be given exactly 24 hours later. Now at this point in my life I never cared for needles.  I mean who does? They hurt and there was nothing fun about them.  Little did I know that was the beginning of a lot of needle pricks.  I made Jeff come in the exam room with me and within minutes the nurse was there to administer the shot.  And there began the first of 12 shots in my bum.  And let me tell you, THEY HURT.  

That night I spent hours and hours scouring the internet trying to find anything and everything I could find about CCAM (also sometimes referred to as CPAM).  I came across CHOP’s webpage specifically for CCAM and I was amazed.  The dialog in the article was written in a way that made it seem so manageable.  I remember thinking that this condition seemed so severe, but they spoke about it like it was a papercut.  They have dealt with CCAM so much and had so many successful outcomes.  I knew CHOP was where we needed to be, and for the first time since her diagnosis I had hope.  I felt so confident that we were going to beat this and I could not wait to get to Philadelphia; so much so, that I called CHOP myself that weekend.  I was able to get in touch with a triage nurse and told her our situation.  She took my name and number and assured me I would be hearing from someone first thing Monday morning.    

Tallies

Ultrasound – 1

Echocardiogram – 1

Steroid Shots – 2