Saturday, January 31, 2015

Broken Hearted

No parent wants their child to have so much as the sniffles, so imagine the frisson of alarm that went through me on Monday when I was told my perfect, pink, newborn daughter had a "significant heart murmur." Let's start from the beginning....

As you know, Eden was born at home on purpose. There were a few issues with her breathing at the very beginning, but we all assumed it was due to the suddenness of her birth. After we got her breathing well, she was pink and perfect and showed no signs of distress or illness of any kind. Which we expected. She was a perfectly normal baby.

Since she was born at home, we had to take her to the pediatrician to get her newborn vaccinations and order the blood type test (I'm RH negative but that's a whole other story), which we did Monday afternoon. She got her PKU and her shots and while the doctor was doing her routine check, she noticed the murmur. She immediately decided that we needed an echocardiogram, or ECG. Very basically, that's an ultrasound of the chest. She didn't think it was any kind of emergency, so we simply called Abilene Regional and scheduled it for Thursday morning.

We went home and started researching online what exactly an ECG is, what heart murmurs are likely in newborns, and what the outcome could be. I was actually fairly comforted by what I found, especially after my mom said it was probably a PDA, patent ductus arteriosus. It's essentially a little bridge between the pulmonary artery and the aorta that is present in the womb and closes very soon after birth. Asher had a tiny murmur at 2 weeks that was gone at 2 months that very likely was something like that that self corrected. So we figured that was probably it.

Thursday we dropped the boys off a little early (Kathy McFall is the best) and headed to Regional. We checked in and began the first of what was to be many waits. Around 9.30 we were called back to fill out paperwork, then went to wait again for the ECG.

The two techs who did the test actually knew Dad, so that was pretty cool. Mom had told us it would probably take about a half hour, so when we hit the 60 minute mark of them rubbing a little wand around on my newborn's chest, I felt that tingle of "something's not right." Plus I heard the phrase "pulmonary artery bifurcation" which means the pulmonary artery is split. Not good. Occasionally they'd do a color scan and you could see red and blue stuff pulsing along with her heart beat and they were mixing. Also probably not good. At about the 30 minute mark they got a doctor in to look at the scans and he spent another 30 minutes looking. Of course, no one tells you anything, but one tech did leave in order to send some scans to another doctor somewhere to be evaluated.

After the scan, around 11am, we were escorted to a small exam room where they took her blood pressure on all her limbs and told us to wait. Which we did. I nursed, Eden pooped, we both napped. Around 12.30, I realized I hadn't eaten since 8am and was starving, so I went to Subway across the street to get some food for us. The doctor looking at scans elsewhere was getting a third opinion and we had no idea how long it would take for anyone to decide to say anything.

While I was gone, Austin talked to the second doctor, Dr. Dyer at Children's Medical Hospital in Dallas. Basically, his understanding was that there was something wrong and we needed to go to Dallas for observation. Her heart was twisted. What that meant, we had no idea. Mom was confused by our fumbling words since they didn't make sense.

We headed towards home with no clue what was going on. We got a call from Children's telling us to come that night and to pack for a few days. Now we were really freaked out.

We met Mom and Bob at the house around 1 and proceeded to pack blindly, throwing clothing and miscellaneous stuff into bags and bags into cars. For the boys, rather than attempt to pack a suitcase, we just sent the laundry hamper full of clean clothes with Mom, who offered to take them for us. We stuffed clothes into a bag for Eden, even knowing she wouldn't wear them while in the hospital, and headed out. At one point, I just stopped and said out loud "I can buy anything I need there, let's just go."

Bob went and got the boys from FKO early so we could say good bye and we were on the road shortly after 2. I posted on facebook a little about what was going on and was immediately inundated by texts and comments offering support and prayers and offers of help in any way we could name.

We got to the hospital around 5.30 and after a (seemingly) lengthy check in process, we were escorted to the cardiac floor and shown to our room. Eden had last nursed at 11am, then proceeded to sleep until 5.45 and insisted on nursing as soon as she woke up, so I walked through the hospital nursing my baby with no shame.

On the way, Austin's parents agreed to take the boys so Mom and Bob could come be with us in the hospital and Mom could get a better understanding and help us understand what was going on. They met us at the hospital around 7 and we met with a doctor who finally told us what was going on.

Eden has four things going on in her tiny little heart (tiny in size, not necessarily in emotion): a patent ductus arteriosus (PDA), an Levo Transposition of the Greater Arteries (L-TGA), a Venticular Sepal Defect (VSD) and a Pulmonary Stenosis (PS). The doctor drew this diagram to help explain things:

Yeah, it's confusing. Basically, the PDA is the little bridge between the pulmonary artery and the aorta, The L-TGA is when the pulmonary artery and the aorta are switched and the left and right ventricles (lower chambers of the heart) are switched. It's a congenitally corrected thing meaning that during development the cells attempted to fix the problems. A VSD is a hole in the wall between the ventricles. This allows the red and blue blood to mix (with and without oxygen). The PS in Eden is thickened pulmonary valves leading out of the left ventricle. 

I know. A lot to take in and it's hard to really understand. The diagram actually helps, I promise. Maybe this one will help a little more. It's still confusing.

Several of these issues will occur together when they occur and typically can be fatal if not taken care of. All together, though, they're what's keeping Eden alive. 

The left ventricle is stronger than the right and so it'll pump the blood harder and faster. That could wear out her heart quickly and cause some issues with the PDA. HOWEVER...the PS slows the blood down so that everything pumps a little better. The PDA being open is helping circulate the blood around the correct way, too. The L-TGA means the blood is going from the correct ventricle to the correct location. The VSD is mixing the blood so that helps keep things working too (sorry, I can't remember all the details, it's been a bit of an info dump the last two days).  

It's insane, but her little wackadoodle heart is so messed up that it's working ok for now. 

They hooked our tiny baby up to a bunch of machines (luckily no iv's or anything inserted into her body, just wires taped down) and we settled in for the night. Because of an child's death during co-sleep, it's no longer allowed at the hospital, so we were in for a long night. Eden sleeps best when on someone, or at least very close. So she (and we) didn't really sleep until she just wore herself out around 3.30. 

Friday morning, we went for another ECG around 11. It was another hour long procedure with the tech getting the doctor in for the last 10 minutes or so. Then we went back to our room to wait some more. Mom and Bob came and kept us company, more doctors, nurses, residents, medical students, fellows, nurse practitioners and physician's assistants came in, all interested in hearing our daughter's heart. Apparently, it's a pretty cool murmur, at least according to the medical students. They were two guys who looked almost giddy to be assigned to Eden's team. Later, I thought "I should have told them that it's probably not best in the future to tell a sick child's parents 'We heard your kid has a really cool heart murmur, can we listen?'" We weren't offended and were fine with them listening, but others might not be so open minded. 

We got no new information from the second ECG except that they know where some arteries or veins or something are and that will be very important for later. 

At some point, we were talking to the doctors again and they confirmed that yes, she will have surgery and yes, it will be open heart. They hope that everything can be fixed in one surgery, but there's a chance they won't be able to. They're also certain that she'll have to have multiple surgeries in her life and for sure she'll always need to see a cardiologist. After the laundry list of acronyms going on, that didn't surprise me. 

Interesting note: open heart is not the same as a bypass surgery. They will literally open her heart to operate on it. Bypass is open chest for sure, but the heart isn't opened up. 

We were told they wanted to go ahead and do a hearing test, an EKG and an ultrasound of her head and kidneys and take an x-ray of her chest just to make sure everything was good there. 

She passed her hearing test, her x-rays were good, and the ultrasound of her kidneys was perfect. She has a very slight bleed in her brain, a Grade 1 Inter-ventricular Hemorrhage. It's probably from her birth. A couple of people said that if they did the head ultrasound on all newborns, they'd probably find it a lot, so there's absolutely no concern on that front. I forgot to ask for the results of the EKG. 

The ultrasound and EKG were done late Friday night, after 9pm, so we could get out of the hospital at a decent hour on Saturday. We still got to do the sit around and wait thing Saturday morning, which is pretty much what you do in hospitals it seems. We were scheduled with a Children's cardiologist who comes to Abilene twice a month for some follow up visits. And then, finally, we were told we could go home. 

We left the hospital around 2.30, went to Arlington to get the boys, then headed home. Of course as we hit Baird, Elijah threw up on himself so we pulled into a gas station and cleaned him up. He's not really sick, just tired and overly excited after a weekend with his grandparents. As I type, he and his brother are passed out in their own beds, grumpy enough, but happy to be home. It's a confusing bunch of emotions for them. 

The surgery to fix Eden's heart is going to be a major one. No idea on how long it will take, but there's a lot to do. 

They're going to close the PDA and the VSD and switch the L-TGA and probably fix the PS at the same time (it's not mentioned in the above diagram but we're assuming that). A lot to do on a tiny heart. They're hoping to let her grow as much as possible with the soonest time to do the surgery being when she's 6 months old. Obviously, if she starts having issues, they'll do it sooner. We have a whole list of things to keep an eye out for, so we'll be a tad hyper vigilant. (Yes. That's an awkward phrase. I'm sticking with it.) 

She is especially susceptible to respiratory illnesses so we'll have to practically quarantine ourselves during cold and flu season at least until she has the surgery. This really means staying out of unnecessary crowds and making sure people who hold her aren't sick with any kind of cough or sinus anything (including allergies because who knows what you're coughing or sneezing out that doesn't bother other people but might affect her). We'll likely insist on hand washing and even use of hand sanitizer before people hold her just for precaution's sake. We don't think you're gross, we just think you might have germs that can possibly hurt our baby. I'm sorry if that offends you, but my child's health is more important to me than your feelings being hurt. And I'm sorry for how callous that sounds. 

To help her with that, she's going to be getting shots of Synergis every month, an antibody against RSV. It's not a vaccine, but that's the easiest way to explain it. It's new and super expensive, but Eden is basically exactly what it was developed for. Fingers crossed insurance pays for it. They should. I've got a whole bunch of literature if anyone cares to read it. 

It's all so overwhelming. I have this tiny baby that I'm still getting to know, I'm still recovering from her birth and adjusting to breast-feeding and sleeplessness. I'm trying to figure out the juggle of three kids instead of two...and now I have this thrown at me. 

I'll admit, on the way to Dallas, I had no idea what they were going to tell us, but a small part of me tried to brace myself for being told there was nothing they could do and my baby was dying. Were they going to say the murmur was something small and easily repaired? Were they going to tell me this baby I wanted so badly and adore so much had a life expectancy of just a few months or years? I refused to even allow myself to go down the path of the worst case scenario and just blocked it all out. Maybe it was all some kind of mistake and everything was actually ok and this was just going to be an expensive anecdote to tell in the future. I didn't want to even think about all the plans we had for her, all our hopes and dreams because what if....?

Luckily, no one said anything like that. Everyone is very confident that this will be something that can be repaired. Her quality of life will be normal, though she may not be able to be a superstar athlete. We asked about her activity levels as she gets older and the doctor said Eden should be able to play sports and run and play, though she may not play Varsity level sports. We laughed and said a child of ours wasn't terribly likely to be athletic anyway but it's good to know she'll be able to keep up with her brothers. Some of the things are things people can live decades with before it starts to affect them. So she'll be fine. She'll be normal. She'll be a little girl who's heart was broken and then fixed. 

The staff at Children's was amazing. Everyone was so friendly and oohed and aahed over our perfect girl. They were impressed with our handling of the situation and her easy going nature. We assured them we do cry about it, but we try to keep that private, just because it doesn't do any good or change the situation. 

Our family and friends have been so supportive over the last 72 hours, calling, texting, messaging on facebook, visiting and bringing us things (thanks James and Nhu and Cari!) and that is overwhelming in a good way. There's so much more to learn about and more to go through and I'm sure I've got some of the details wrong above, but this is my current understanding of the situation. 

My daughter has an extremely rare heart. It's put together in such a way that it's working against the odds. She's perfect in every other way and the only way her heart is negatively affecting her is that she has poor blood circulation in her feet (they're a bit cyanotic, or purple). She eats and sleeps well (when against people) and her kidneys and bowels work great. She's wonderful and we fall more in love with her every day. And we'll get to do so for a long time. 

Our sweet little ladybug. I promise that's a newborn outfit, she's just tiny.

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