A congenital heart defect, for those that don’t know, is one that has been present since birth. In these modern times, with so much screening, tests, and other procedures doctors are capable of, most are found at birth and can be dealt with in the most appropriate way. Early detection. You hear it everywhere, whether talking about cancer or with credit fraud and your personal identity. I never associated “early detection” with congenital heart defects because I never knew anyone with any, especially in my family, and these things are usually dealt with in children. When you are born in the 80’s in a small town where the nearest major hospital is roughly fifty miles away, like my sister, things are a little different. My sister is five years and two weeks older than I am, and has always had fairly good health. A little asthma and allergies (I got the rest of those not so little genes), but other than that she was a normal healthy girl growing up. She gave birth to my niece when I was fifteen years old, and she will be turning sixteen this year (and will be driving on the same roads as me……I’m getting old). My niece’s dad was never really around, and I along with the rest of the family, would help make that as easy on her as possible until, the right man came alongto fill those shoes.
Last year my sister met a man that she fell in love with, and they have been inseparable since. He even got a job with the family business. They soon got married, and decided that since my sister had always wanted another child, that they should try while everything was still relatively achievable without complications. Soon they were pregnant, and happy as could be. A little boy. One that the family desperately needed considering I have been surrounded by girls my entire life, save for my father.
On Wednesday March 22nd 2017, my sister had an appointment with a cardiologist setup by her family doctor after going into his office with complaints of malaise, fatigue, dizziness, and chest pain all while being 21 weeks pregnant. At that cardiologist appointment, the doctor heard a murmur. He did an echocardiogram and immediately admitted her to the hospital his practice was through. I found out late that evening while beginning to install a garage door opener. Specific, I know, but I’m willing to bet that when something life changing occured to you and your family, that you knew exactly what you were doing when you heard. Time kind of slows down, your mind races, you can’t find the words to articulate what you are feeling, and become lost with the most menial things as if they matter. At least, that’s how my brain handled it. My wife had come to the garage to say “Did you know your sister is in the hospital?!?”, to which I replied with a dumbfounded “What?”. I immediately looked at the social media post she saw and remembered that I was going to text to check how her visit to the cardiologist went after I was through with the opener. We both immediately started texting, making plans for my in-laws to watch our daughter so we could rush to the hospital to find out what was happening.
When we arrived, she already had a room and had multiple small tests. They had also decided that she needed to be transferred to the major hospital I mentioned earlier, which happens to be one of the top medical schools and hospitals in the south, and they happen to have one of the best cardiac units in the nation. That’s when I felt like I was the one who needed to be on a hospital bed. After some talking with the cardiologist when the transfer was completed (and extensive research that I did because it is a OCD compulsion with perfectionism), we found out what was wrong with her heart.
Since birth, going unnoticed because murmurs from this are relatively hard to hear, she has had two heart defects. A bicuspid aortic valve and coarctation of the aorta. The reason she started showing so many symptoms was because she had developed endocarditis and had calcification on her aortic valve. A bicuspid aortic valve is a deformity in the development of the aortic valve in utero. It should have three flaps that open and close with the beating of the heart to allow proper blood flow. Her valve only had two flaps, which had calcification buildup that happens as you get older, but it tends to buildup more on defects like this. This is what caused the endocarditis, which is an infection in the lining of your heart. The coarctation of the aorta is a narrowing of the aorta after it leaves the heart and begins to turn back behind the heart. Theses two defects are commonly seen together, but usually only found in babies or in adults after they have died. She is extremely lucky to have found it, but unlucky in the fact that she was 21 weeks pregnant when it was found. The pregnancy causing more stress on her heart was probably what brought on the symptoms that allowed her to make an appointment in the first place. It was just an all around rare circumstance.
Obviously the first thing on everyone’s mind was having to lose the baby, which they had affectionately named “Bean” for his appearance when first being known about. The first cardiac team was very blunt at first in explaining things. They hadn’t yet got the head of cardiology down to talk to her and everyone wasn’t aware of the rarity of the case yet. As soon as they were though, boy did they all want something to do with it and help in every way possible. She had the top OB team, the top NICU team, and the head of cardiology’s team. She was set. They went through many different conferences together and came up with a lot of different plans. All had to do with fixing her heart first and hoping for the best for the baby during the surgery even though it was very risky. They also had to clear the infection up first. Eventually they found that she as also in congestive heart failure too. So they began to formulate the final plan that would start to come into play on April 5th 2017. They decided that was the day they would do a balloon valvuloplasty to widen her aortic valve enough to allow her to give Bean 10 more weeks of gestation which would put him just shy of 33 weeks, easily a survivable and a high chance of not having any problems with a premature baby at that age. They said she had a good chance of delivering well, and after some time to heal, then they would plan the open heart surgery to do a more permanent fix for her heart. They did the procedure and she was home within a few days.
This whole time she wasn’t able to work, and her husband was the sole provider for the family which put a strain on their finances. She has insurance, but between regular bills, having a teenage daughter in a lot of extra curricular activities, and having to hurry to get ready for a baby that was coming sooner than they thought. I took it upon myself as her brother, and knowing that she would never ask for anything herself, to set up a GoFundMe page for them. To date it has generated $2,000 in help for them, which has been a tremendous relief, but still doesn’t scratch the surface of what they need.
On June 9th 2017 at 4:22pm, she gave birth to a healthy 18 inch long, 5lb 3oz baby boy they named Kamari. He was exactly 32 weeks and 1 day. He had a little trouble with regulating his temperature, and keeping his oxygen levels normal. But it didn’t take long before he was doing great on his own and taking feedings like he should. He came home not long after having his feedings down. He has been growing great since, and is the best baby. He never cries, only grunts and is getting all kinds of love and attention from everyone.
They soon scheduled my sister’s open heart surgery for August 8th 2017. She has a couple of consultations and appointments the day before to set everything up. She will have about 12 weeks of recovery after the surgery in which she will be not doing very much at all, much less taking care of a child. My mother has moved a lot of things to her house so that she can keep Kamari while she recovers. My wife and I have volunteered to keep him every so often to give my mother a break also.
So here we are. A few days away from the scariest surgery my sister has ever had. I’m not good with vocalizing my emotions in times of stress and anxiety. I am pretty good at writing them out however. I don’t know why this is, but it is therapeutic in a way. I’m worried to death, on top of being over worked and having health problems of my own in which I have been dealing. I also have my own echocardiogram, stress test, and calcium PET scan to go to at the end of August to check for any congenital heart defects that I may have also. When a woman with Lupus (like my mother) has children, she is 3 times more likely to give birth to a child with a heart defect. That is added to the normal chances, and add to it the statistic that men are twice as likely to have the heart defects than women. That is why I mentioned the saying “Early detection” above. My sister’s ordeal has so far turned out the best it possibly could have, and we are all hoping that things continue to trend that way. It also very well could have helped me learn more about myself, and get some early detection of my own. We will find out shortly. Until then, we will be getting ready for the major things about to occur in our family very soon.