This “Heart Story” gives you a raw and emotional account of one family’s early CHD diagnosis during pregnancy. The time in your life that is supposed to be filled with nothing but happiness and hope is all of a sudden exchanged with “what ifs” and more questions than answers. This mom wrote straight from the heart – and her message is sure to hit home with our readers.
I discovered I was unexpectedly pregnant one week after sending out the “save the dates” for our upcoming wedding. Awesome, right? Yeah we thought so, too. Lol. There was some disappointment at first to learn that our plan was to be delayed, but we got over it and started to get excited about the idea of a child. We knew we wanted to start a family immediately after we were married, and this was really only a slight derivation from the original plan we had constructed for ourselves. No big deal. We were fine with it. We started thinking of how amazing it would be to have a little one around. I have always wanted to be a mom and looked forward to welcoming our new addition.
Our little friends condition was discovered at our 21 week appointment. We found ourselves at the mercy of an ultrasound tech who was kind enough to offer up the sex of the baby before asking if we even wanted to know. A sure sign that this would not be the visit we had hoped for. “Okay, so it’s a little girl and she looks like she is growing well”. Thanks a pant load, Chet. What a jerk. Before I had a chance to give her a piece of my mind for opening her big, fat mouth she began to twist it into a strange shape and squint her eyes at the monitor, slowly but obviously turning it away from us so we could no longer see the fuzzy image of what was apparently going to be our daughter. We knew from looking at her that there was something off, but were told everything was fine and to go home. As we made our way back to the car we joked about the events that just transpired. “Did you see her making those faces? What the fuck was up with her?” “I know, right? It looked like she was trying to hold in a fart or something. Maybe she was constipated?” We giggled and made more snarky comments as we got ready to leave. Looking back, I think it was our subconscious way of dealing with the fact both of us were thinking something could be up. The tech hadn’t said anything, but there was something unsettling about the first picture session of our baby. The whole experience just felt…weird. We were five minutes into our drive home when we received a call. Before I even looked at the phone to see who it was I knew it was the doctors office. I looked at my soon to be husband who had already pulled over, his eyes trained on me. I swallowed, telling myself, ” knock it off you moron. Nothing is wrong or they would have told you about it then. You probably left your wallet there or forgot to sign something”. Any kind of benign thought to quell my sudden rise of anxiety. In the 5 seconds it took me to answer the phone, I swear I thought of about 3700 reasons why they could be calling. ” May I speak with Corinne, please?… How far away were we? Could we come right back? Yes, the doctor needs to speak with us as soon as possible”…. I relayed the info to Joe and sighed. It was probably nothing too serious. Maybe they forgot to talk to us about labor or vitamins or something ridiculous like that. We giggled again but we drove back in silence. We both stared blankly in front of us, never once exchanging a glance. Truth is, I think neither of us wanted to show the other the fear behind our eyes.
We were ushered into a conference room immediately and told the doctor would be right with us. In 20 more minutes. Super. So we sat for what seemed like an eternity, making innocuous conversation, commenting on the posters of baby growth cycles in the room. ” Look at that one, honey. Can you believe in a few months, there is going to be something the size of a meatloaf inside my body?” Insert your own version of my husbands obvious and horribly timed phallic joke. Again, another attempt to divert our attention away from the anxiety we were suddenly feeling (hey, I laughed). Knock on the door, doctor breezes into the room. Not my doctor. My doctor was a man. This was a woman and one I had never met. Introducing herself, she sat down and began to explain the sudden need to reroute our departure. “There was something on the ultrasound we need to discuss”(Deep breath. In. Out. Ready. Not ready. okay, ready…) “It appears there is a slight concern for the babys heart”(Ummmmm.. Okee Dokee. Still breathing, but palms are getting a little shweaty. Breathe.)”It looks as if the heart is not developing properly and you are going to need to see a specialist. The ultrasound is showing us what could be a heart defect.” Stop breathing. Swallow. Breathe in. There is suddenly a faint ringing sound in my ears. I tilt my face a little to the right, trying to let out whatever was beginning to block the information from entering. “You see, sometimes while the heart is developing it can experience a “hiccup” and…. “..That was kind of all I heard. After that it gets a little blurry. My ears were conscious of the sound coming from her mouth but they were no longer actual words. Picture the sound the grown ups from the Peanuts strip make. It was funny. Funny strange though, not funny ha-ha. I could hear my own heartbeat. My breath sounds. And that damn slight ringing, but nothing from her was registering. Not that it mattered what she was currently saying, I only heard what had already been said. “Babys heart is not developing properly” just ran on a repeating cycle in my head. I saw lips moving, but only caught bits and pieces. ” Congenital Heart disease…Can be life threatening..multiple surgeries..babys survival…….” A tidal wave of words and phrases were starting to flood my brain and it was all I could do to not drown in them. None of it made sense. What do you mean? My ears wouldn’t stop ringing. My hands were tingling…….I flexed my fingers out and back as I tried to take in what I was hearing. What did all of this mean? How was it possible? I am healthy. I don’t do drugs. I exercise, eat well enough. Sure I drink and smoke, but not to the point of this? Oh my God, did I do this? I didn’t know I was pregnant until nine weeks and did plenty of late night drinking sessions with the ol’ hubster-in-training, that bad influence. I was assured this was almost definitely congenital and therefore was not something I was directly or indirectly responsible for. Okay, so now what? “We will send you to a specialist who can answer all your questions for you”…Phew. Someone to answer our questions. What a load off, right? Ummmm..yeah….
Fast forward. We were referred to a Perinatologist. A doctor that specializes in high risk births.We were referred to a pediatric cardiologist for further testing and to hopefully gain more information about what she was going through in there to help us understand better out here. An ultrasound and echocardiogram and another conference room later and BAM. We were hit with it like a Mack truck at full speed, no breaks, and the driver passed out from too much oxy and booze. High Risk. High Risk. High Risk. Over and over and over again on a loop in my thoughts. High risk indeed. She was diagnosed with Hypo Plastic Left Heart Syndrome, a congenital heart defect that affects 4 out of every 10,000 births. Not to shabby with the stats there, right? I mean, 8% of all pediatric cardiology cases contain these symptoms. A sad, albeit strangely uplifting fact. Her condition wasn’t unheard of so we didn’t need to be overly fearful…right?. Yes, a fairly frequent diagnosis but the additional complications that occurred made her individual diagnosis slightly more rare than most with her condition. Whoops. Spoke too soon. Our little overachiever wouldn’t stand for being one of the general pop. A veritable smorgasbord of defect related complications were thrown in the air. Malposed great arteries. Pulmonic Stenosis. Double outlet right ventricle. Malrotation of the duodenum and intestines. Asplynia. Is your head spinning yet? It was like some freakish late night infomercial. “You get the congenital heart defect – but wait! There’s more!” Holy shitballs. What the hell where these people talking about? Want to know exactly? Here is the verbatim description from her medical records. Staple this shit to your forehead and memorize it:
“Heterotaxy syndrome with unbalanced atrioventricular septal defect with right ventricular dominance, double outlet right ventricle, and d-malposed great arteries and severe subvalvar and valvar pulmonary stenosis with dysplastic pulmonary valve and hypoplastic pulmonary valve annulus. Bilateral superior venae cavae, pulmonary venous drainage to the confluence posterior to the left atrium, single coronary artery arising from the right facing sinus”.
Basically her heart is all jacked up. It doesn’t look, function or sound like a normal heart.. Her left ventricle(normally the dominant chamber) is so small it is hardly noticible on an echo. It is now too weak to do much of anything and its diminished size and capacity leaves it no choice but to put the majority its workload on the shoulders of its more lazy brother, the right ventricle. Hypoplastic hearts cannot pump oxygenated blood out to the body the way they are supposed to. The now weaker, almost non existent left side puts all the work on the stronger right side causing the heart chambers and blood vessels to become larger and stretch more than normal. There are only two kinds of surgery to help with this issue. The first, a series of three seperate operations, can help alleviate the stress the heart is under. There is no way to “fix” this kind of heart. You can only help it out a little. Unless of course you opt for available surgery number two- a heart transplant.
What.The. Fuck. This was no joke. It was the real deal Holyfield of heart defects and it was the happy resident of my unborn childs chest cavity. It was there and it was not going to get better. There was no magic pill to swallow, no mystical potion to drink to change what was going on. Her internal development had hit one hell of a pothole. What a mess. Even her freaking appendix was in the wrong damn spot. A stupid, practically useless organ couldn’t even do its job and get over to her right side.(Side note: Did anyone else think that it was supposed to be on the left? My mind immediately sifted through all the tv shows I had ever seen where a character gets appendicitis and what side they would hold while doubled over in imaginary pain. A totally moronic and completely random thought at the time. But there it was).
A certifiable, Type A control freak, I had no choice but to become “that know-it-all mom” who had almost as much knowledge as the paid professionals. I couldn’t control what was happening inside my body, but I’d be damned if I can’t control what goes on out here. There is so much information out there and I devoured each and every bit I came across -the good, the bad, the really, really bad and the horrifying just so I could get my hands around the neck of this whole situation. I felt like my head was on a swivel going backwards and forwards between technical and medical jargon and the desperate need to figure it out in layman’s terms. I wanted and needed to know every ounce. Every thing from treatment to what life would be like to how long a child with this condition could live. The terminology that was spewed in our general direction from 21 weeks prenatal until even this very day has been enough to drive a person to drink ….or want to attend medical school…but mostly, drink.
We were told that while she was in utero I was doing all the work for her, so her growth until birth would be watched like a hawk but overall we could expect no complications. While she continued to bake in my ever expanding midsection she would be fine and frigging dandy. Great. So why can’t she just stay in there forever? Some peoples kids never leave the house. Mine will just never leave my uterus.
We were advised once she is born and has to begin processing oxygen on her own, things will decline. She will need to be cared for around the clock. Meds will be a part of her life for its duration however short or long it may be. She will have to undergo multiple surgeries, including the possiblity of a heart transplant. She could have developmental problems. These heart defects are usually associated with Downs Syndrome or other chromosomal disorders. Now stop for a minute and try to picture yourself 23 weeks pregnant with your first child, sitting in a room with a doctor you don’t know in a place you don’t want to be, discussing the life of your child in ways you would have never possibly imagined. Developmental delays? Downs Syndrome? Surgery and life long aftercare? What the hell happened to sex, pregnant, labor, birth, go home?
To read more about baby Sophia, please visit her mom’s blog at: Life in the Oncoming Lane.
Her mom told me that she is almost 5 and a half now! She managed to bypass the Norwood (the first in the usual 3 part series of operations), had her first OHS at 6 months, a 2nd surgery for a malrotation at 9 months and her 3rd surgery (2nd OHS) just before her 3rd birthday. She has had some pretty big “downs”, but some even bigger “ups”, (They went on her Make A Wish trip for her 5th birthday this past August!) and she is in kindergarten. There have been a few rather large bumps in the road and yet she continues to grow and thrive despite. She is the toughest kid and always greets the day at 6 am with puffy eyes and a smile on her face. She is all kinds of amazing!