Kids With Rare Disorder Live in a World Without SunlightJune 12, 201502:26
If you saw Brady Wheeden on the playground you might not notice anything odd. He’s often in a baseball cap and always wearing a long-sleeved hoodie with the hood up. So are a lot of ten year olds.
But watch for a while and you’ll notice Brady never steps out into the sun. Instead, he hops from shadow to shadow. If it looks like a game — it’s not.
Brady has an extremely rare condition called Erythropoietic Protoporphyria, known by the shorthand EPP. Doctors aren’t sure exactly how many people have the condition in the US, but probably only a few hundred.
Living Life in the ShadowsJune 12, 201501:12
People with EPP lack an enzyme that helps the rest of us process toxins from sunlight. Because people with EPP have decreased levels of a certain enzyme, there’s a buildup of a compound in their blood vessels beneath the skin. This compound is very sensitive to light, so if they go in the sun—even for a few minutes-- they have an incredibly painful reaction.
Watch more about Brady's story on Dateline NBC on Sunday.
“Some patients said it was like… dipping their hand in boiling water, or being stabbed by 100 knives,” says Dr. Manisha Balwani, Associate Professor of Genetics and Genomic Sciences at Mount Sinai Hospital in NY, who has treated many patients with EPP.
Brady says sometimes it’s annoying that he has to sit inside the cafeteria during recess on a sunny day when everyone else is running around outside.
“Sometimes… I wish I could just be like them, my friends.”
There is no cure for EPP. But Brady and his mom Kristen Wheeden are hopeful about a new treatment that might offer some relief. So far, however, it is only available in Europe and only for adults.
For now, Brady has learned to live with his condition. This summer he’ll be back at a summer camp in upstate New York called Camp Sundown, where kids with many conditions that make them avoid the sun gather. Most of the activities take place at night.
When he’s there, Brady says, he feels like he’s not alone in the dark.
“It feels like I'm a normal person.”
To learn more about EPP, you can visit the America Porphyria Foundation's website at www.porphyriafoundation.com.