Bringing You up to Speed…Sort of

So, my first post ‘The Beginning’ left off somewhere back in May 2012 when we still knew very little about Atticus’ condition and what the course of treatment was going to be.  It is now over a year later, and from now on I will be writing regularly, but I wanted to fill you in on what has transpired between him leaving St. Louis Children’s Hospital and now (August 2013).

So, to throw another wrench into the situation, Brian had a job offer in Charlotte, NC, but without knowing what Atticus’ condition was, and how long he would be in the hospital, that whole thing was up in the air until Atticus got discharged from the NICU.  Once we realized that Atticus could eat and breathe on his own, we felt much more comfortable making a move to a whole new state, in a whole new area that we were not familiar with, and an area where we had no family or friends there.  “Comfortable??” you ask?  Well, this would not be the first time that we picked up and moved to a whole new part of the country without knowing anyone.  We had moved from Binghamton, NY to Champaign, IL about two years before Atticus was born.  We knew no one in the Midwest, but we met some great people there and had access to amazing doctors via Barnes-Jewish Hospital in St. Louis, MO (about 3 hours from Champaign, IL).

Actually, instead of telling you what happened after Atticus was born, I am going to back up a bit to December of 2011.  I was 4 months pregnant and had no idea that there were any issues with Atticus.  Everything in my 12 week and 18 week ultrasounds looked perfectly normal.  I remember still having this nagging feeling though that something wasn’t right.  I remember saying to people, “I just can’t wait until he’s born so I know that everything is OK.”  I just chalked it up to first-time pregnancy jitters.  I felt so much pressure to do everything right.  I stressed over everything that I ate and became obsessed with washing everything (even bananas) before I ate them.  I just wanted to do everything right for this little guy.  After all, I had dreamed of being a mother for so long, and it was finally happening!

I was working at University of Illinois in Urbana, IL and, although towards the end of my time there I became close to some people and made long-lasting friends, I really struggled at first to find people that I clicked with.  I felt alone a lot.  I felt depressed at times.  When I finally became pregnant though, I was over the moon, and now I was mainly just focused on the baby’s health.

Anyway, one day in December 2011, I went to use the restroom at my work place.  I started talking to Amy, who also worked in the School of Social Work but in a different office.  She had just had a baby not too long ago and I felt that we would have a lot in common.  I forget who asked who, but we ended up having lunch together and we clicked right away.  She told me about this placed called ‘Little Wonders’ about an hour away from Champaign that does 3-D ultrasound images of your baby (just for fun, not a medical facility).  I didn’t even know places like that existed so I was really excited to get this done.  I called soon after and scheduled my appointment for March 17^th, 2012 (you got the best pictures if you waited until around 30-35 weeks pregnant).

When March 17^th came, something just didn’t feel right to me.  I was nervous going to get the 3-D ultrasound.  I already had 2 ultrasounds by my doctor and both looked normal, so I didn’t know why I was nervous.  I thought that maybe it was just nervous excitement about seeing the baby for the first time.

Brian and I got to Little Wonders that afternoon and we were taken to the ultrasound room.  The technician put the cold stuff on my stomach and started to do her thing.  The whole thing was projected onto a large screen so you could see the images very big.  The first moment that his face came on the screen I knew something was wrong.  Because this was just sort of a ‘for fun’ thing to do, these technicians were not medical professionals – there was no doctor on site to answer questions.  I remember saying to the technician, “Why does his chin look so large?”  (Because ultrasounds are not in color, I couldn’t tell that the large protrusion was a tongue – it sort of looked like a really large chin).  The technician tried to skirt around my questions and just said it’s probably just from him moving around a lot and the picture is blurred.  She could not make any guesses or comment on something like this – she was not a medical professional.  We had paid for a one hour session.  So we had to sit there and wonder if there was something really wrong or was it just movement like the technician said?   I knew there was something wrong but I tried to tell myself that maybe this is just what ultrasounds look like.

We left with pictures and a video of the hour long session.  I spent the next few days Googling whether other people’s ultrasound pictures made it look like their babies had a massive chin or some other distortion.  I couldn’t bring myself to watch the video (I still never have) or look at all of the pictures.  There were a few pictures where Atticus’ arms were covering the lower portion of his face.  I sent those out to our families.

A few days later I received a call from my obgyn’s office asking if I had the phone number to Little Wonders.  Apparently the receptionist called me by accident.  I couldn’t understand why they were calling me for Little Wonder’s phone number.  “Yes, I was there, but why are you calling me for their phone number?”  The receptionist stumbled and said “Never mind, I called you by accident” and wanted to get off the phone.  I said “No, there must be a reason why you are calling me, is something wrong?” (I knew there was).  I went into panic mode and can’t remember much after that besides leaving work and going home to Brian.  We decided to just go to the obgyn’s office and demand some answers.  We got there and we were told that Little Wonders had sent over some ultrasound pictures from our session and they were reviewing them now.  My fears were confirmed.  There was something wrong.

We went in to see the doctor and she said there was a mass in the jaw area.  My heart sank.  I was right.  But, what exactly did this mean?  Was it a tumor?  I was so confused.  After that, we went to Maternal Fetal Medicine in Champaign and they did another ultrasound.  They said the mass was his tongue and the most likely scenario was that Atticus has Beckwith-Wiedemann Syndrome (BWS).  Much discussion ensued and I remember the doctor shaking Brian’s hand as we left the ultrasound room and saying “I’m sorry.”

We met with a geneticist.  She gave us a list of about 15 additional genetic or chromosomal disorders that Atticus might have.  I had never heard of most of them.  I refused to look anything up on the internet.  Brian did some research on BWS but I was too scared.  I felt that if I looked up everything it would be too overwhelming and I was afraid of what I would find out.

At that time, we didn’t know what the quality of Atticus’ life would be, but we coped with those 2 months of not knowing by telling each other that no matter what is wrong, we will love Atticus and do whatever we can to make his life good.  We would have to be strong and deal with whatever lied ahead.

In the weeks following, we met with specialists at Barnes-Jewish Hospital in St. Louis.  I would have to have a planned EXIT procedure.  Not many hospitals do these procedures and our local hospital did not, so we would have to travel to St. Louis to have Atticus.

One morning in April, I had a fetal MRI in St. Louis which took really detailed images of Atticus. That afternoon, we were brought to a large conference room at Barnes where an Ear, Nose, and Throat (ENT) surgeon had projected the images of Atticus on a large screen and was reviewing them.  The good news was that all of Atticus’ other organs looked fine.  There was no evidence of anything else wrong with our baby.  The ENT confirmed that the mass was his tongue (there was a time when some doctors thought it was a tumor).  The ENT had a good feeling that it was a lymphatic malformation and not a genetic disorder based on the fact that nothing else was wrong.  I had never heard of this.  I remember the ENT saying the words “lifelong condition” and “no cure”.  That meeting started off rocky, but got better.  The doctor said it is “manageable”, that Atticus would need surgeries and possibly a tracheostomy throughout childhood, but that if this is what he had, he would not have any mental or cognitive disabilities.  That was a huge relief.

I remember asking the ENT if we would be at an increased risk of future babies also having lymphatic malformation.  He said, “No, but even if you were, no reasonable parents would let that stop them from having more children.”  I took comfort in those words.  It made me feel that whatever this lymphatic malformation would bring, it would not be that bad, especially compared to what some parents go through.

When I looked back at everything that had happened, I remembered that time that I bumped into Amy in the restroom at work.  We went out to lunch and she told me about Little Wonders.  Had I not used the restroom at that exact time, I might never have had that conversation with Amy and might never have gotten the 3-D images that showed the enlarge tongue and airway obstruction.  I would have gone on thinking that everything was normal and proceeded with a normal delivery.  Who knows what would have happened then…

We got lucky.  There are families out there who are not as fortunate as we have been.  Some babies don’t make it or are not as fortunate as Atticus.  We are grateful that, although Atticus has had to deal with a lot in his little life, things could have been so much worse.  LM is manageable and will only make him stronger.  Please look around you and recognize everything there is to be grateful for.  Not a day goes by where I am not grateful for how our situation has turned out.  I will never forget the course of events that led us to this point, and I will never stop being grateful for each and every one of those events.

*Since I got sidetracked on this post, stay tuned for another post that really will bring you more into the future instead of back in time 🙂

Learn More about Lymphatic Malformations Here

Visit the National Organization for Rare Disorder’s information page on lymphatic malformations:

http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/646/viewAbstract

The Beginning

This blog has been a long time coming.  Not only because I feel like I have a lot to say, but because I feel that I owe it to other parents out there who have a child with some type of rare medical condition.  Our son, Atticus, now 15 months, was born with a big tongue.  Not just big, but pretty huge actually.  It protruded out of his mouth and was so big that his mouth was permanently wide open.  We knew before he was born that this was the case, but I don’t think that family members or friends realized exactly how large we were talking.  Up until he was born, we didn’t know what was causing this large tongue – was it indicative of a more troubling genetic or chromosomal disorder?  Was it an isolated event?  Were there other not-so-obvious things wrong?  What would the quality of his life be?  What would his future be like?  Is it fixable?  There were so many things going on in our minds at that time and the last couple months of my pregnancy were some of the darkest days I ever experienced.

We drove to St. Louis on Sunday, May 6, 2012.  I was going to have a planned EXIT procedure, which is an extension of a C-Section, the following day.  May 7^th was dark and rainy.  We checked into the hospital early and I was put under general anesthesia.  I did not witness my child being born.  My husband, Brian, was not allowed in the operating room.  It was me and 28 doctors in the sterile hospital room.  I was so nervous that my body was literally jumping off of the operating table because I was shaking so bad.  Each team of doctors had their last names (color-coded by team) on their backs.  Every doctor was extremely focused and had a mission.  Their concern was delivering my baby, establishing his airway around this massive tongue, and closing me up before I lost dangerous amounts of blood.   They finally put me to sleep and I remember looking up at the light and wondering if I would ever see Brian again.  I know that sounds possibly exaggerated but I couldn’t help it.  I couldn’t help but think “What if they can only safely deliver the baby if I have to stay open and lose fatal amounts of blood”.  It was a concern.  All the while, I still didn’t know what was wrong with my baby.

Atticus Blake Kurilla was delivered on May 7, 2012 at 1:29pm and weighted 7lbs 7oz.  We had no family or friends in St. Louis (we didn’t even live there), so Brian was all alone waiting for updates on what was happening with his wife and baby.  He was talking to his mother on his cell throughout the delivery, but they were both probably too nervous to even know what they were saying.  I am glad that she was a comfort to him at that time though.  Once Atticus’ airway was established via breathing tube, he was put in an incubator and a team of doctors and security guards rushed him to the NICU in the St. Louis Children’s Hospital.  Brian was able to see him for the first time and I was still asleep.

I woke up some time that afternoon.  The first face that I saw was Brian’s.  I remember the first words that I said were “I love you”.  I was confused.  My stomach was smaller.  I had had a baby.  But, I still hadn’t seen him, hadn’t heard him cry, hadn’t heard those words “It’s a boy”!, had no idea what he looked like.  Brian told me Atticus was beautiful – that it looked like he had red hair.  I was still out of it and could barely comprehend what Brian was saying.  I received two blood transfusions because I had lost so much blood.

One nurse was assigned to taking pictures of the delivery, since neither me nor Brian were able to experience it.  They blew up one photo and put it near my pillow.  I saw him for the first time in that photograph – this mystery baby that I had carried for nearly 38 weeks.  He was beautiful.  In the photo, a doctor had placed two fingers over Atticus’ large tongue, so I still could not see what it looked like.  I remained out of it for the rest of the day and into Tuesday.  Still, I had not seen my baby.  I was not well enough to go to the Children’s Hospital.

Sometime on Tuesday afternoon, I began to feel lightheaded.  My heart rate shot up to 180 beats per minute and I was losing consciousness.  I felt my awareness slipping away.  Doctors rushed in.  Someone was asking me if I knew what year it was – if I knew who the president was.  I knew the answers at that moment, but felt that soon, I would not know the answers.  They asked me if I had a family history of heart conditions.  I thought of my grandfather who died when he was very young of a heart attack.  Did I have some sort of condition that I didn’t know about?  What if I die before I meet my child?  Doctors were puzzled and nurses were scrambling.  I didn’t know what was happening and was scared.  I looked over at Brian with his head in his hands.  This was a lot for him to go through.  Sometimes this happens when you lose a lot of blood, but they had already given me two transfusions.  They did an EKG and it was normal.  They thought maybe I had a blood clot.  They started giving me more blood and I remember starting to break out in hives.  Am I allergic to the blood?  They contemplated stopping the transfusion but soon the hives dissipated and I was getting more conscious.  They sent me for some sort of test to rule out blood clots.  I was in so much pain and was getting stuck with endless amounts of needles.  It was over 24 hours since I had given birth and I still had not met Atticus.  I didn’t know what he looked like and I still didn’t know what was wrong with him.

On Tuesday evening, they put me on a heart monitor and determined that I was well enough to go over to the NICU at the Children’s Hospital to meet Atticus for the first time.  I have to admit that I was scared.  What would he look like?  His facial malformation that I had only seen on ultrasounds would be real now.

Brian wheeled me over in a wheelchair and I was nervous going into the NICU.  When I first saw Atticus I thought he was beautiful and loved him instantly.  There were still so many unknowns though, and I remember being upset that this beautiful baby had this massive protruding tongue.  One nurse came in and said “He’s beautiful, but, hopefully they can do something about that”.  Well, what if they can’t?  I thought, “He is beautiful either way” and was offended at her words.

I remember Brian sending out texts to our family showing a just-born baby Atticus from about the nose up.  Were our family members ready to see the rest of his face?  They were all back in Pennsylvania and were dealing with all of the uncertainty from afar.  I remember Brian sending out a text asking close family members if they wanted to see a picture of his whole face.  That affected me.  I understand why Brian asked, and I would have done the same thing, but it made me sad that we had to ask this question.  Of course, they said yes, and for the first time, I think they realized how big his tongue really was.  They were confused, had never heard of this, and didn’t understand why this happened.  They wanted to know what caused this, and what would be done about it.  We didn’t have answers and neither did the doctors.

It was pretty clear to doctors that his enlarged tongue was an isolated event and was not related to a genetic or chromosomal disorder.  MRIs taken right after he was born proved that everything else was perfectly normal.  All of his organs were fine.  This was a HUGE relief!

We thought that Atticus would be getting a tracheostomy to help him breath and a feeding tube soon after birth, but two days after his birth, they removed his breathing tube and he breathed just fine on his own!  Most of the tongue enlargement is in the forward part of the tongue and not the part that goes down your throat.  Next, came feeding, how was he going to eat with a tongue this size?  Well, super baby did it on the first try.  His doctors were so pleased and he was released from the hospital after only 11 days.

I remember not feeling ready though.  I felt that we were getting discharged and still didn’t have any answers.  We still didn’t know what was going to be done about his tongue.  I had never seen anyone with a tongue this large and expected them to just surgically correct it right after birth.  Things are not that easy though.  I was worried about his future and what it would mean for him to go through life with a facial malformation this noticeable.

I quickly learned, however, to let go of needing answers.  He was healthy and beautiful and OURS!  This baby that I had wanted for so long was now here and I was not going to get hung up on the “What ifs”.  I focused on all of the positives of the situation – number one being his health and the fact that cognitively and mentally – he is perfect.  I was grateful that Atticus has what is called a lymphatic or vascular malformation, and not something that will affect his development.  I focused on the fact that he will lead a long and normal life and all of the struggles that this big tongue might cause will only make him a stronger and more compassionate person.

The doctors at St. Louis planned a wait-and-see approach.  Sort of follow the course of his tongue and see how it grows as he gets older.  They planned that he would have a surgical tongue reduction later on, probably sometime around his first birthday.  Brian and I accepted this approach.  It was not a quick fix approach, but we realized that there would not be a quick fix.  This would be a long process and one where Atticus’ tongue might never be a ‘normal’ size.  Only time will tell that.

For now, I firmly believe that we were given Atticus for a reason and he was given us for a reason.  We love him so unconditionally and believe that he will embrace himself, his strengths, his weaknesses, his imperfections, and that he will grow into a strong and amazing individual.  His life will not be ordinary, but that is not a bad thing.  Brian and I often joke that Atticus is already so much more interesting and cooler than we are and he is only a baby.  I also truly believe that Atticus has saved me and changed me in deeper ways that I ever could have experienced, had we had a normal delivery and a baby with no abnormalities.  He has made me a better, more carefree person.  He has taught me that things will work out, things will fall into place, and to accept things, and see the positives.  My life is so much brighter since I met him.  I always say that he cured me of all of my anxieties and worries.  I can honestly say that I enjoy every day of my life.  I don’t worry about anything unnecessarily and I am truly grateful for everything that I have.