How old were you when you were diagnosed?
5 years old.
Do you remember your first flare/reaction?
Yes, I was two years old at the Beach in Florida
What did it feel like to you?
It was hard for me to remember, I remember the pain & bath I had made it worse
*The liver failure/transplant caused a rare porphyria side effect of nephropathy that left me paralyzed and on mechanical ventilation. It has taken nearly a year to get to where I am now. Doctors were only able to find a few documented cases of this occurring in porphyria patients.
What things help you feel better? (Cool water, ice, shade, bath, clothes)
How long does it take before you start to feel better?
2-5 Days before I start to get relief. It really varies for me.
What kind of clothing/trends do you wear when you go outside or in bad lighting?
Lots of long sleeved shirts, face shields, ball cap, sunglasses, and gloves
What is your most favorite sport to play or watch? Do you play it inside or outside? During the day or at night?
Started with indoor activities when younger (bowling, jiu-jitsu) but hard to keep up with just because of travel. Easy to get burned out on certain activities when they are so limited. Will start going to Nashville Predator hockey games when I’m recovered. SEC football is my favorite (GO VOLS)
What ways are you able to adapt to do certain activities outside?
Trying to go outside around sunset when the sun is low, while keeping covered up.
What fun things are you able to do inside your home while the sun is out?
Video Games, Cooking, having friends over
What has been the best vacation ever?
Camping with my best friend
Have you met any other kids with EPP before?
I did have someone come see me in the hospital, but I had just had my liver transplant and was not responsive. I was on a ventilator and very sick. I don’t remember, but it was very nice for my mom to meet someone.
Do you go to School/Homeschool?
Before complications with my liver and needing a transplant, I went to regular school.
Do you have to go by car, bus or walk?
I drove myself
What things do you have to do to protect your skin?
Cover with long sleeves, gloves, hat, face shield, and sunglasses. Prior to last April the indoor lights didn't bother me. I now have to be covered under fluorescent lights but have not been back to school or left the hospital since then. They have changed lights in my room to LED.
Is it hard for you to tell your friends, teachers or family what EPP is?
As I’m older it is easier to explain. When I was young it was difficult to get the schools to believe me even with doctor notes. My mom had to be very firm and direct with school administration when they didn't believe me. She brought them pictures of what I looked like during a reaction.
What do you tell them?
Basically, that the sun, even when its overcast, sets off a photo toxic reaction in my blood. I let them know that it starts to hurt before you see any symptoms.
What things do you do if you feel sad or left out?
Never really felt left out. Didn't let it bother me.
What do you want to be/do when you grow up?
What advice can you share to help other kids that have EPP
Speak up and reach out to others. The technology we have today makes this possible.
Parents- How do your children deal with the pain/reaction/flare?
He goes into a dark room and try’s to distract himself. We give Medications sometimes for flares, but it may act as a placebo more than anything. Additional Medications taken daily has helped the flairs not be as bad.
Would you benefit from a new treatment?
Had this been available previously it would have been life changing! Even at this time when a bone marrow transplant will cure him, it would have been easier for the hospital to care for him without having to worry about lights. Even trips to the ICU were delayed while the lights were changed out.
Skyler, Thank you for your words of wisdom! Keep living each day to the fullest. Know you’re surrounded by a loving family and friends and now that you’re in another medical facility I hope that each day you gain the strength and determination to follow your dreams.