Difficult But Never Impossible

Kayley was born a year before Loeys-Dietz syndrome was named and was diagnosed at age 9 after emergency heart surgery.

“My legs looked like they had been put on backward and my fingers were crooked. Of course, everyone knew something was wrong, but no one in the world knew what that meant for me, what had happened, or how to fix it.”

Kayley with a few of her Loeys-Dietz friends at Disneyland during the 2022 Annual Conference

I was born in 2004, exactly one year before Loeys Dietz syndrome found its name, so from the minute I took my first breath, I was a mystery. In comparison to my twin brother's healthy, squishy newborn frame, I was tiny and frail. My legs looked like they had been put on backward, and my fingers were crooked. Of course, everyone knew something was wrong, but no one in the world knew what that meant for me, what had happened, or how to fix it.

People hate not knowing things (especially doctors, I’ve found). So when they don’t, the easiest thing to do is fill the unknown with grim possibilities. I spent just a few minutes in the world before my mom was told by a delivery doctor that her daughter would never walk. While many would be rightfully shocked or devastated, my mom simply disagreed... while her best friend calmly told the doctor, “you bet your *** she will walk.” I love this story because it’s exactly how I was taught to tackle LDS growing up. I owe so much to my parents in this regard. They believed from the moment I was born that this mystery illness would not define me, and I believe that my determination and bravery can all be attributed to this fact.

In the first years of life, my brother crawled around freely, touching the world around him (Including an instance of super glue, unfortunately), while I lay in our crib with pink full-length leg casts. I’ll never forget the leg braces or the dread of tightening them after days at pre-school. Dr. Campion, my first pediatric orthopedic surgeon, was a man who wore funny ties and sometimes told not-as-funny jokes. If I knew he was behind all the braces and casts, I probably would have hated him. But I would have also known in my heart he was a good doctor, not because of the funny ties (though that added a lot), but instead because he believed in me. Every time we’d have a visit, he’d ask me to walk to each side of the doctor's office, and one particular day I finally did without falling. As I confidently walked back and forth for the very first time, I watched teardrops form in his eyes. I had no idea how good of a day that was.

And then, when I turned three, I began ballet. I cannot imagine how much self-control it took for my mom not to invite my delivery doctor to my first ballet performance. My twin brother began playing soccer, and I (not very well) danced through every genre under the sun. Things seemed normal, like my health journey was finally over. I dreamed that story would be told in the past tense on national television when I became a famous dancer. This was the narrative I entertained anyways, until around five when I found myself back in Dr. Campion’s office for a pain in my back.

A pretty serious case of scoliosis was presented to my parents in the form of an x-ray, and that was the day my second health journey began. There was always hope that nothing was related, that I was kicked by my brother in the womb or something (I actually think I told people that in kindergarten, sorry, Isaac). But every new health journey made that possibility feel less and less real. It could have been incredibly discouraging, but it was also continuously stressed to me that this would never mean the end of things I loved. A story in particular I love is when I needed emergency surgery on my back and scheduled it on the same date as my kindergarten talent show. I cried to my mom about it, and instead of telling me to grow up because my health was more important, she held my hand as we went back into the doctor's office and changed the date of my surgery. “We plan Loeys Dietz around our lives, not our lives around Loeys Dietz.” is how my mom now says it. By second grade, I had undergone 24 surgeries, but never truly felt like I had a difficult or strange childhood. I didn’t even really consider that I was disabled until my junior year of high school. The life I wished for was difficult but never impossible; that was our view.

When I turned nine, I went to an allergy doctor to get diagnosed with, well, allergies. Instead, I was diagnosed with heart failure, a life-threatening, rare connective tissue disorder that 98% of the world had never heard of, and, of course, dog, cat, and tree nut allergies. It didn’t all happen that quickly, but it felt like it. One minute I was getting routine vitals done by my allergy doctor, and the next, incredibly concerned, she told my parents to visit a cardiologist right away. My cardiologist, the next extremely concerned doctor, took one scan of my heart and found a piece of my aorta was twice as big as it should be as an adult. The technician who took my scan later told us that he assumed I’d need a heart transplant. I was possibly weeks away from an aortic dissection and had no idea.

To a nine-year-old, hearing you need emergency surgery in which they stop your heart with a machine is extremely cool. I even remember bragging about it to my friends. Never as a child did I understand the gravity of the situations I was under, and because it was so normal for me, my perception was skewed. This is not necessarily bad, but I am reminded in the stories of my parents waiting 12 hours in a cramped room to hear if their child going through open heart surgery was alive or not that sometimes my normal is devastating. And sometimes, it is ok to grieve. Recently doctors found an aneurysm at the base of my neck, and it hit me like a ton of bricks. I craved my childhood ignorance and the confidence in her future that that little girl had. I also realized that after almost ten years of diagnosis, that was the first time in my entire life that I grieved my syndrome. And that is an incredibly strange feeling.

With the love of every human being my family possibly knew, I was able to recover from heart surgery in a matter of days. My wonky heart also happened to be the missing puzzle piece needed to connect my quirks to an actual name, and with some genetic testing, I was finally diagnosed with Loeys Dietz syndrome. The story after diagnosis only begins, but it's easier and more beautiful. I met my best friend in middle school through the Loeys Dietz Facebook page, who, in hindsight, probably helped me out of depression when my head was collapsing into my chest. The Baltimore LDSF conferences gave me some of my mutants (we call ourselves because of our mutated gene… anyways). And, of course, my other mutants who I’ve met in all different ways, having long talks about how to have a meaningful life while also grieving the fact we have a life-threatening condition, how to take up space while looking different, and playing silly little games like “Is this Loeys Dietz or am I just weird?”

I struggled with how to end this because my journey with LDS doesn’t just stop when my writing does. Loeys Dietz is as complex as any human experience, there are good and bad days, and there are lessons to be learned in all of them. My life will never get more predictable, and that is a difficult pill to swallow (and I’ve swallowed a lot of pills). But I am thankful for the incredible people and lessons I’ve learned and the person I get to be, and the life I get to live because of that.

-Kayley Tigges