In April 2020, NF patients and families received the news of a lifetime — the United States Food and Drug Administration (FDA) announced the approval of Koselugo (selumetinib). Finally, for the first time ever, there is an FDA-approved drug treatment for patients living with NF. Early-stage discoveries from Children’s Tumor Foundation-funded researchers proved that MEK inhibitor drugs have the potential to affect the size of NF tumors, bringing us to this moment. More than 70% of NF patients taking selumetinib in a clinical trial had shrinkage of 20 to 60% in the size of their tumors.
While Koselugo has helped so many patients living with inoperable plexiform neurofibromas, it does not help all patients living with NF. Patients like Ethan Brown, who has neurofibromatosis type 2, are still waiting for breakthroughs in drug development and treatment options. At age 16, Ethan received his NF2 diagnosis. Tumors formed inside and outside his body, on his brain stem, his spine, and hands. The tumors that compressed his brain stem required three brain surgeries. The operations left him with the symptoms that stroke victims endure. He now needs a wheelchair, has slurred speech, and has difficulty swallowing.
Keep reading for an exclusive excerpt from Ethan Brown’s memoir, The Fight of My Life.
The night before I was born, the smell of freshly popped popcorn filled the cool, crisp May air as my dad, Rick Brown, stepped up to the plate, confidently locked eyes with the pitcher, and with the tip of his bat pointed to the fences. The pitcher lobbed a softball to the plate, and the ping of ball to bat pierced the air. I guess it’s thanks to him that I have a love of sports and the kind of tenacity one needs to win—both in the game and in life.
When I was a kid, I was terrified of many things, especially bees and the dark. Every night, I left my closet light on with the doors open. Sometimes, I’d even beg my mom to lie down with me. Her head would be at the foot of my bed, so I’d grab and hold her legs. Often when I would wake up and she wasn’t there, I would run across the house to my parents’ room to be with them.
My favorite song back then was a VeggieTales song called “God Is Bigger.” He was bigger than the dark, which seemed so big and scary back then. I guess a lot of kids are scared of the dark, probably because they’re scared of the unknown. I’m not scared of the dark anymore, maybe because I’ve had to deal with the unknown for a long time now— that’s life with neurofibromatosis.
If you think that’s hard to pronounce, try living with it! Actually, we just call it by its abbreviation, NF. NF has three types: NF1, NF2, and schwannomatosis. You can inherit NF from a parent (there’s a 50 percent chance of passing it to your offspring if you have it), or you can get it from a spontaneous mutation like I did.
NF1 is the most common, occurring in 1 in 3,000 births worldwide regardless of race or gender. It can cause a loss of eyesight—some have to have their eyes removed because of tumors behind them— loss of limbs, and cancer.
NF2, which I have, is the next most common form of NF. It occurs in 1 in 25,000 births worldwide regardless of race or gender. It also can cause loss of eyesight, hearing, and limbs, and it can cause cancer and loss of balance.
The recently discovered form schwannomatosis is rare and affects 1 in 40,000 births worldwide. Not much is known about this form of NF.
Since I have NF2, I will focus on that type in this book.
Since 2008, I have had five major brain surgeries, lost my hearing, and developed facial weakness. I have limited vision, and my eyes do not produce moisture like they should. My left eye has an ulcer and is also sewn three-fourths of the way shut. My vocal cords are paralyzed, which makes it difficult for me to talk and for others to understand me.
For some reason, I drool, especially when I’m warm or hot. My mouth gets really dry, despite the drool. I can’t swallow well, so all my liquids must be thickened to keep me from choking. The drooling is worse when I eat, so I wear a bib to avoid a messy shirt. I used to hate to go out to eat because I feared what others might think of me, but I got over that because this is who I am.
NF has taken a toll on my physical appearance. I used to go to the gym five days a week, but now I’m lucky to go twice a week. I prefer to use my left hand for almost everything because my right hand is uncoordinated. I can still move it, but I can’t control it well. I use American Sign Language a lot to communicate, but it’s very difficult, and I’m still learning. If you had asked my dad before 2013, he would have told you that my handwriting looked like chicken scratch. Now, I can’t even manage that. It takes all I have to make a legible EB.
I have tumors in my left hand. We went to see a hand specialist, but he said he would not try to remove them because if the surgeon cut one of the many nerves in my hand, I could lose hand function.
I have a tumor on my spine and tumors on my feet and ankles, requiring me to wear foot drop braces to keep my feet straight. I don’t have my balance, so I need a wheelchair to get around.
None of this stops me from trying to improve myself and to inspire those around me. It’ll be very interesting to see how NF affects me in the years to come. Isn’t it comforting to know that God is bigger than anything we face (John 16:33)? Jesus tells us he has overcome the world. If we have accepted Jesus as Lord, the Holy Spirit lives inside us. We are told in 1 John 4:4, “You, dear children, are from God and have overcome them, because the one who is in you is greater than the one who is in the world.”
Fear not, friends! Don’t fear for me, and if you know Christ as your Savior, don’t fear for yourself either.
My journey with NF started with a series of seemingly unconnected medical episodes that gave no hint of the challenges that lay ahead. I was in third grade when I had a fibroma, a benign tumor, removed from the back of my head. The doctor told us I could have it taken off if it bothered me. So that’s what we did. Little did we know how much that one bump would mark a change in our lives. The surgery left me with a bald spot. My doctor told me to tell anyone who asked that I had my “memory expansion chip” removed. (I think I’ll be needing that back!)
Later that year, I had my wisdom teeth removed, and I also had a growth on my tongue removed. The pathology report said that I needed to be evaluated for something called neurofibromatosis, but I wasn’t. So, we went on with our lives like normal. Nobody in my family has NF, so we really didn’t give it much thought.
The FIght of My Life, with a foreward by CTF friend and Colorado Rockies baseball player Ian Desmond, published by Lucid Books, is available on pre-sale now. Visit ethanbrownnf.com to pre-order your copy. (25% of book earnings will be donated back to the Children’s Tumor Foundation. If you complete your order on smile.amazon.com and select the Children’s Tumor Foundation as your charity, an additional .5% of your purchase will be donated back to support our mission to fund critical research to help NF Heroes like Ethan.)
Click here to learn more about Ethan’s friendship with Ian Desmond, and the Colorado Rockies baseball player’s role as Honorary Chair of the NF2 Accelerator Initiative.
