Meet Cortney, EPP Warrior
By Cortney Johnson
"My hands and feet swelled up so bad the skin cracked so where you could almost see bone"
-Cortney, EPP patient.
Life with EPP
I started showing symptoms at the age of 2 during an end of summer beach day with my family. My hands and feet swelled up so bad the skin cracked so where you could almost see bone. The doctor said I was probably stung by a jellyfish and we went on with life until spring rolled around and I had another reaction. I was lucky to not have a lengthy process for diagnosis, however, the only answer we received was, “I read this in a textbook while I was in college, but don’t know much about it. Here’s your daughter, just keep her out of the sun.” at this point is was 1991, so we couldn’t google anything and were left to figure it all out on our own.
My tolerance is about 15 minutes of sun before the warning signs of my skin tingling and stinging sets in. Knowing I only have a few minutes to completely escape the sun before a full blown reaction starts, at that point causing me to burn from the inside out, like I have a chemical burn underneath my skin. Nothing is ever visible on my skin, except for swelling occasionally, but I get a major burning/stinging sensation that I constantly itch to try and get to. I become extremely sensitive to both cold, heat, and water and just want to sit in a cool, dark room. I usually roll a cool water bottle over my skin to keep from ripping it apart, but nothing truly takes the pain away that lasts for 3 days.
It’s more than missing out on outside activities, it affects everyday life. Driving to work with the sun coming through the windshield, running errands during the day, house chores, having to plan for the entire day ahead of time to figure out how much clothing to wear that day. It’s an invisible disease that most don’t understand so it gets downplayed and brushed off by others. During a recent interview I was asked, “If I could go back and change one thing about growing up with EPP what would it be?” My response, “I wouldn’t change how I dealt with it, we figured it out as we went and did the best with what we knew. BUT I would change how others treated me, judged me, and picked on me.” I had to school my doctors on it because they never heard of it and then they downplayed it. I was judged for being different. I was picked on with rude comments when I covered up from head to toe. It wasn’t easy.
“I didn’t want to miss out on things. I covered up in UV protective clothing and did the things I love”
-Cortney, EPP patient.
Living Fearless
I always thought it was ironic that I’m allergic to the sun, but was also given a huge love for being outside. My parents and I found out ways so I could participate in most things, even if I had to look different or do it in the dark. While in school I had my gym class changed to first thing in the morning before the UV rays would rise and hung out inside during recess. I would swim in our pool as the sun would set and stay out a little later to play in the dark. Going out during the day required me to completely cover up head to toe, keeping my skin from being exposed to the sun.
It’s not easy covering up, I had to dress with a purpose, instead of look cute like other girls growing up. Wearing long sleeves, pants, gloves, hat, and a full face cover brings on a lot of stares and judgement, especially during hot summer days. It’s mentally and emotionally taxing. But I chose to do it as I got older, to live life fully, live fearless, and do things despite my limitations. I didn’t want to miss out on things. I covered up in UV protective clothing and did the things I love like kayaking, bike riding, hiking, and exploring outside on vacation.
Cortney enjoying the sun after clinical trial.
Life in the sun
In April 2023 a clinical trial completely changed my life. I still get teary eyed 2 years later that I get to live in the sun pain free thanks to bitopertin. I’ve always been an advocate for research and trials, participating for a brighter future for everyone with EPP. I now spend my days in cute shorts and sandals out in full sun. I’m able to say yes to anything without a worry. I look forward to sunny days and can’t wait to get off work so I can get outside. I went from 15 minutes of sun with pain to 8 hours pain free. Bitopertin didn’t just give me freedom from pain. It gave me freedom to live fully, to live a life I’ve wished for on every birthday candle.
I never knew how much I was truly missing out on prior, because I never knew a different way of life. I’m a health and science nerd so I’ve always believed in the power of research and clinical trials, not only for myself but for everyone living with EPP. I’m always up for a challenge, a limit pusher that’s willing to push it to the max. I joined this trial with hope for a brighter future, and now I advocate even harder—because I’m living proof that change is possible.
Huge shoutout to the UPA for bringing our community together. It makes a difference to walk the journey with others that fully understand it. I was 25 when I first met others with EPP and wish I had met everyone sooner! Now at 36 I have such a huge porphamily!
Want to share your story? Contact us at katri@porphyria.org
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