Happy Congenital Heart Defect Awareness Week!

Miles about a month post-op, Nov 2012

I'm hijacking the kids' blog in honor of Congenital Heart Defect (CHD) Awareness Week! Each February is Heart month, so we try to raise awareness of heart disease and defects all month long. But Valentine's Day week is set aside to focus on CHD's.

I'm always happy to answer questions about Miles' heart condition, but I haven't really figured out how and when to fit it into conversations. "Hi, I'm Lisa and my son has a heart defect." is a bit blunt. Never bringing it up seems odd too. The balance is obviously in the middle, but how to go about it remains a bit of a mystery.

So, in honor of CHD week, I'm sharing a bit of my side of the story here.

Pregnancy and Birth
We had a great pregnancy. I received great prenatal care. Our tests and ultrasounds all came back with glowing results, and I had a comfortable, healthy pregnancy. When I was 10 days overdue, we had a routine NST, which looks at things like fluid levels and the baby's movements, to make sure all is well. After seeing the results, my Doctor wanted to induce labor. It was Friday around noon when we left the office, and I was already having contractions and was dragging my feet about getting induced, so we took our time to call people, feed the cats, grab food, etc. I was checked into the hospital and hooked up to pitocin around 5. Miles arrived at 11:54pm and was everything we hoped he'd be. Screaming, nursing, 10 toes, and so on. Of course, there was a heart murmur. Since I had a 'benign childhood heart murmur' into my 20s, I wasn't too concerned. They called the on-call pediatrician and since the vitals all looked fine, they said they'd just monitor him.

Diagnosis
About 6 hours later, we were all finally dozing when the pediatrician knocked on the door to do his rounds. He did an exam, didn't like the murmur, and asked the nurses to do a pulse-ox, which measures the blood oxygen level (also known as your "sats"). They just wrap a thing around a toe or finger, so it's non-invasive and all hospitals have the kit so it's pretty dang cheap as well. It will hopefully be a standard test on all newborns soon! Call your legislators... Anyway, the handheld machine said he was in the 60s. You should be at or very near 100. Unless you're in an airplane or live in Denver (I've heard both can drop you to the low 90s). They rushed in a bigger machine in case that one wasn't working, and it confirmed the results.

They told me they needed rush Miles to NICU and that there was something wrong with his heart. I stared at them with big eyes and a clenched jaw. They told me to tell my husband to stay with the baby and that I should stay in the room for a minute. So I did as told.

After they left, my nurse stayed behind and basically told me that I needed to be strong for my baby and to call my family. After that things are a bit of a blur. Dr King, a fabulous pediatric cardiologist who Miles still sees, was working and available to come right over. He diagnosed Miles with Tetralogy of Fallot with Pulmonary Atresia. TOF is the most common complex CHD, and the severity of it can vary greatly depending on factors like how much blood gets through their pulmonary artery. The pulmonary atresia makes Miles' condition more severe.

First Surgery
Miles was transferred to another hospital, and his first open heart surgery was a few days later. It was a palliative surgery to patch things up so he could grow big enough for the "complete repair." Since baby hearts are the size of a walnut, I can understand why they'd want it to be a bit bigger before doing the more complex repairs. He did pretty well and had a little trouble getting off the ventilator, but as far as complications go, he was lucky.

Second Surgery
When he was 6 months old, he had his second surgery where his heart was fully repaired and a donor pulmonary conduit was placed. His chest was left open for a few days, which was scary, especially since no one had warned us that was an option. One of the most memorable moments for me was seeing him wheeled down the hall from the operating room to PICU. From birth to the second surgery he was 'dusky' in color, but I didn't really know what it meant until I saw him post-op. He was pink! The color change was shocking. We were home in less than two weeks and Miles' post-op complications were relatively minor. We went home with a healthy baby, and his sats were 100!

Third Surgery
Unfortunately, his body broke down the new conduit pretty quickly. There was talk of maybe needing to go right back in, but instead we were in a wait-and-see mode for the next 3+ years. This past October, shortly after his 4th birthday, Miles had his third surgery and received a new donor conduit. We still don't know how his body will react to this one, or how long it will last, but things looked good as of his first follow-up appointment.

The Future
Miles will continue to have surgeries throughout his life. Once he gets big enough (teen years) he can probably get a new valve inserted in the cardiac cath lab. He's had two caths in the past, and they're no fun, but beat open heart surgery. The name brand for those valves is the Melody Value, and they are pretty new. Right now those valves last a few years (3ish). They think they can put two valves in before the entire conduit needs replacing. So it can delay open heart surgery for a bit but doesn't eliminate the need for future ones.

CHD Facts
So that's a bit about our story, and here are a few facts about CHD's.

• CHD's occur in almost 1 in 100 births, though the severity varies greatly. Some never need surgery, others are fatal.
• Less than half are diagnosed in utero, and some aren't diagnosed until much later in life (get your teen athletes screened!). 
• More and more CHD kids are surviving to adulthood, which is creating a demand for cardiologists who specialize in treating adults with CHD's.
• CHD's are the leading cause of infant deaths in the US.
• Almost half of those with complex CHD's also have neurological and developmental disabilities.
• In the United States, twice as many children die from congenital heart defects each year than from all forms of childhood cancer combined, yet funding for pediatric cancer research is five times higher than funding for CHD.

Thanks for reading and helping us raise awareness of CHD's. I'll let the kids blog about some cute kid stuff soon :)