Jimmy Kimmel’s recent monologue about his son Billy’s heart condition and the whirlwind that was his birth, diagnosis, and surgery has struck a chord with many of us. I know that it pulled at my own congenitally defected heartstrings.
Let me explain: I was born with a bicuspid aortic valve (BAV). Basically, the aortic valve of my heart looks like a fish mouth (two leaflets) instead of a Mercedes symbol (three leaflets), which is, incidentally, a pretty good metaphor for my life. I am not all that special. An estimated one percent of the population has BAV, making it the most common congenital heart defect. Though some are diagnosed at birth, many, like me, do not find out until they experience symptoms or a catastrophic event later in adulthood.
I found out when I took way too many decongestants my senior year in college because sometimes nurses make mistakes on prescription bottles. Sometimes mistakes result in a trip to the hospital to understand your tachycardia. Sometimes that isn’t so bad (really) because you get to leave in the middle of a final exam and by that point you had run out of things to say about Beethoven anyway. Luckily for me, a full cardiac work-up sometimes results in life-saving incidental findings.
My diagnosis is cataloged in a file in my brain where I store memories that make me want to take a time machine back to kick myself. The cardiologist who told me the news could not have been more blasé. I was twenty-one years old and alone at the appointment with no expectation of bad news, watching as he looked mindlessly at my echocardiogram report and said big powerful words in the distracted monotone of a man who could use a vacation, “You have a blah-biddy blah…in ten years get a cardiologist…it just might kill you when you’re in your 60s or 70s…oh and take antibiotics before the dentist…blah-bitty blah symptoms mean go right to the hospital…any questions?”
Naturally, in my recently college-educated empowered voice I chirped a devil-may-care, “Nope, sounds good!”
The whole interaction took only a few minutes, and all I said was sounds good?! Time machine. Kick. Kick. Kick.
I never gave my condition too much thought, particularly after the demands of motherhood took over. Weird palpitations and periodic anxieties that my valve would someday get worse were assuaged at my yearly cardiology appointments. That is, until 2014. My first echo with a new doctor revealed an aneurysm in my ascending aorta. Like stenosis (thickening of the valve), and regurgitation (leaking), aneurysms (aortic enlargement) are among the complications of BAV along with scarier things like heart failure, endocarditis (infection of the heart), and aortic dissection (tearing of the aortic wall) or rupture.
My aneurysm became quite the a-hole in my life. It made it dangerous to pick up my snuggly four-year-old daughter or get pregnant again as we'd hoped. It had to go, and thus began our preparation for open heart surgery. Not the least of my concerns was protecting my preschooler from being traumatized by the whole thing. Having a sister and friends who are Certified Child Life Specialists® made all the difference. Together we came up with developmentally appropriate words, activities, and distractions that helped immensely.
These Child Life angels among us use evidence-based practices to help kids of all ages understand, cope, and play during difficult medical encounters. Their services are really needed, too. According to the Maternal and Child Health Bureau of the federal government, nearly twenty percent of all children in the U.S. under the age of 18 have special healthcare needs. Chances are you already know at least one family whose child needs clinical supports, in some capacity or another, and most of those families are carrying much heavier loads than they let on.
Tough times have a way of highlighting all kinds of strengths. My husband is a man of many talents, not the least of which is his carnival-act ability to guess weights and measures. He can eyeball the poundage of a pumpkin from across the patch, that kind of thing. I’m not bragging, but it’s like a superpower. So after several rounds of imaging gave us the exact size of my aorta, we began playing a game I called, “Would this fit in my aneurysm?” Sure it’s morbid, but it’s a lot more fun than you would think.
For those playing at home here are some items that would have fit:
My BAV and pending open heart surgery now had my full attention. It turns out doctors and researchers had been hard at work in the 15 years since my diagnosis learning all sorts of new things.
For example, it appears to be a family affair. About 30 percent of immediate family members are likely to also have BAV or other cardiac malformations (with names like aortic coarctation, ventricular or atrial septal defect, abnormal mitral valve, aortic root dilation, or hypoplastic left heart syndrome). A study published in 2009 reinforced that idea and added that immediate relatives might also develop an aneurysm in their aortic root (where the aorta connects to the heart) even if their valve has three leaflets. There is still so much to be learned.
My six siblings (yeah, six, it’s a lot, I know) were encouraged to be screened, and so far all have been negative. Then, there was my daughter. There is a famous quote attributed to Elizabeth Stone, “Making the decision to have a child – it is momentous. It is to decide forever to have your heart go walking around outside your body.”
Here she was twirling, skipping, and dancing outside of my body. This idea of her being my heart held new weight for me now. As a mom, I do not like anything messing with my children, especially when that anything happens to be my own genetics. Even so, I work in the field of public health where we deal with the idea of increased risk with measured caution. My little girl seemed healthy, and if she wasn't, she would be in excellent hands.
So I didn't panic about taking her to Boston Children’s Hospital to be checked. Thanks to the awesome staff and her spritely spirit, my daughter enjoyed getting EKG leads stuck to her little body and giggled during blood pressure hugs. She even lay perfectly still and quiet during the part the echocardiogram when they shoved the wand into her neck presumably to get a peek inside her soul. The girl was a champ! And her heart looked perfect.
Of course, it did. I knew it would. That is why the absolute relief that washed over me nearly knocked me off my feet. To say that our little girl is a better version of her parents is an understatement, and I could not be more grateful. (According to the National Survey of Children with Special Health Care Needs there are roughly 335,000 kids in the U.S. with cardiac conditions, and nearly half of those children experience daily limitations in their lives.)
Soon her baby brother will have to be checked too. He bounced into our family, a ball full of energy and vivaciousness, a little over a year after the open heart surgery that spared my diseased valve but replaced my ascending aorta. I hope that he too will avoid the diagnosis I may have passed along to him. If he doesn’t – if he takes after his mother – I just don’t know how my little fish-mouthed heart would bear it.
You come and mess with me, BAV! I can take the multiple open heart surgeries, the complications, the insurance battles, the silent fears, and the changed life. But you do not mess with my kids.
To all of you parents already in the fray, with your strengthened mom-armor and your sharpened swords: I am over here on the sideline with the others who are rooting for you. We are advocating for insurance coverage and research funding, hoping it will help. And if I join your ranks, I hope that dozens more spill in from every direction to take my place of support on the side, because none of us can do this alone.