I’m Not Me Without You: Living (and Writing) with VHL

I didn’t realize I had Von Hippel Lindau Disease (VHL) until I was thirteen years old. I was in P.E. class, asbestos lurking underneath the tiles of the ancient gym that only us middle schoolers used, and we were on the floor. The asbestos floor, doing sit-ups. Hating sit-ups as I did, I barely lifted myself off the ground when it happened—the lightning bolt of pain in the back of my head. Wincing, I settled back down. That was weird, I thought. And then I went about my business. I moaned and groaned about how much I hated P.E.. I counted down the seconds until we were done. I probably wondered when I’d be getting my hands on a copy of Evanescence’s newest album (The Open Door, by the way). I did not wonder about the pain.

I should have, because that singular moment in time marked the beginning of the end of my life as I knew it.

This is me after my second brain surgery, which happened in 2019 right before COVID came into the world. It was a very long surgery with a lot of scary stuff attached to it, but I made it through okay! Even got my hair braided.

As I thought I knew it, anyway. I’ve always had medical problems. I was born three months premature (!) and as a result, developed mild cerebral palsy. Though the CP didn’t affect me as much as it does others, I still spent much of my time as a child in and out of the hospital, getting my legs “fixed.” I had casts on both legs for a while, then a wheelchair, then a walker. I had to learn to walk all over again. It was an excruciating experience. I was also diagnosed with dyscalculia and a processing disorder when I was twelve, so having things “wrong” with me wasn’t necessarily shocking.

I was a weird, wrong, strange kid. I thought I was a wolf up until the age of eleven. I begged my friends to read Edgar Allan Poe while I was reading The Hot Zone by Richard Preston. So of course I’d have a weird, wrong, strange disease to go with me.

I’m sure you can guess where this is going. Long story short, the pain in my head didn’t go away. It got significantly worse, even. Imagine that. Over the course of three months, I started getting dizzy. I developed debilitating headaches that made it hard for me to perform even the most basic of functions. I lost my sense of balance, I kept running into walls. Eventually, I couldn’t keep food down. I threw up everything I ate, losing so much weight that a feather could have knocked me over. My vision was failing, and finally, the neuropathy came. I had no language for this symptom, no way to describe what I was feeling. I only knew that I had to be dying, because what else would feel like this?

This is from a recent Gamma Knife session I’d had about six months ago.

Gamma Knife is highly concentrated radiation that my doctors point at my brain tumors so they’ll stop growing so dang much. I’ve had it done five times so far.

I wasn’t dying, dear reader. I had a large brain tumor shoved into the right side of my cerebellum. Exactly where that mysterious pain had been three months before. This is when my doctors told me: I had Von Hippel Lindau Disease. Same as my father, who I inherited it from. Thanks to some genetic testing I’d had done when I was a baby, we know I had a predisposition, if you will, for the disease, but doctors weren’t totally sure if I had it. If I did, symptoms would likely manifest when I was around 13 or so. Puberty. Turns out, they were right. Brain tumors are very punctual.

That first tumor opened the floodgates to a bunch of other Stuff. So much Stuff started happening in my young life. So much more than I’d already been dealing with. I had specialists now at a hospital two hours away. I had MRIs every six months. I had a tumor in my right ear that had to be removed a year after my first brain tumor. I’m completely deaf in that ear as a result. I’ve had radiation treatments, eye surgery, another brain surgery, and kidney surgery since then.

I am now 30 years old. Nearly the same age my dad was when he discovered he had VHL.

When I’m not at the hospital or having MRIs, I’m writing books. It’s the one thing I’ve always known I wanted to do. While I majored in creative writing in college and knew I wanted to pursue traditional publishing by then, I wasn’t sure how to get there. I queried on and off for probably eight years until I finally landed my agent, the fabulous and endlessly patient Ernie Chiara. He’s truly the best agent I could have asked for and very much worth the wait.

This is me right after the eye surgery I had in September of this year (2023). Complete with the glasses I now have!

Anyway, when I first got my agent, I had the same thought many newly agented writers do when they get agented. I thought my life was golden. I thought, yes, this is it! I’m finally going to take the literary world by storm! As I carry on with my publishing journey, I also carry on with my VHL journey. I’ve had more, shall we say, issues with my disease pop up as of late. I’ve changed hospitals, started new meds, and developed a super exciting tumor in my eye that has the possibility to make me completely blind. Yay!

As fun as that sounds, I decided to write a book about VHL for the very first time. My upper YA contemporary novel, The Only Hope for Me is You, is the YA novel of my dreams. It’s about a girl with VHL who falls in love with her next door neighbor…who also happens to be a rock star. There’s fake dating! Mutual pining! Normal person/celebrity dynamics! So many tropes.

Most importantly, it’s a book about VHL. My disease, that has shaped every part of my life since that fateful day in the gym when I was thirteen. It’s a rare disease and it isn’t talked about much. It barely exists in fiction. When it does, the person with VHL usually dies by the end (thanks a lot, Grey’s Anatomy). Well, I set out to write a book wherein the sick person doesn’t die at the end. They get a happy ending, even, and a passionate romance to boot! I hope you get to read it one day. I hope you get to love it as much as I do. It’s a wonderful book, the book of my heart, even.

And it’s got some pretty great song lyrics in there, too.

Until next time,

Lex