This blog is dedicated to all the Porphyria patients worldwide.
The American Porphyria Foundation will provide updates and information here, as well as on the main site - http://porphyriafoundation.org
I’m 18 years old and have experienced pain after staying in the sun since I was about 2 years old. When I was 2, I was outside and all of the sudden I started crying. Nobody knew why and my parents asked themselves whether or not I was pretending. Slowly they figured it had to do with the sun. We tried so many things and we went from doctor to doctor, but nobody had a clue what exactly my problem was. Several times my eyes were so swollen I couldn’t see anything. In the summer it was horrible. At night I couldn’t sleep, I itched myself until I was bloody – awake or sleeping. The only solution for me was to protect myself and to stay out of the sun. But I didn’t want to be different from my friends and I didn’t want to be a burden for my family. So I often crossed the border, to make it possible for my family to take me with them.
When I was about six or seven, I met a doctor by chance, who guessed what my problem was. She sent me to a university hospital, where my family and I got a diagnosis: Erythropoietic Protoporphyria. Just this fact helped us a lot.
For a long time I thought I was the only one having this stupid problem. But then my mother found a support group for German EPP patients. We went to a meeting and it was so strange hearing people talk about things I experienced as well. My family supports me a lot but they can’t understand me like someone with EPP is able to. Just simply talking with someone so similar was great. I got to know a girl about the same age as I am and we became very good friends. Last year we spent two weeks in Greece. During day we stayed inside or in the shade and in the late afternoon we lay on the beach.
Growing older I got to know my limit. Now being more responsible for myself, I have to measure the fun and the pain I likely will have when I do any outdoor activity. By now I’m so self-comfortable that I don’t feel strange anymore wearing my huge hat and long sleeve. I try to avoid taking pain killers, but sometimes it’s inevitable even though I protect myself.
EPP of course limits me in certain ways, but sometimes I don’t care. Having an awesome day downhill skiing, a class trip to Rome (all day sunny, 42°C the time I was there) or having fun at a bike-tour with friends is worth having any pain.
Q & A WITH PORPHYRIA EXPERT, DR. BRUCE
WANG, UCSF The APF asked our Facebook friends for their top questions they
would ask a porphyria expert.
The following questions were submitted
to Dr. Wang for his responses ...
Q. Does EPP give us bad teeth? Also, do people with EPP
get stomach pains or is that with the other porphyias?
A. The porphyrin that accumulates in EPP patients is protoporphyrin
IX, which does not cause discoloration to teeth or abdominal pain.
The type of porphyria that leads to discolored teeth is Congenital
Erythropoietic Protoporphyria. The porphyrias that lead to episodic
abdominal pain attacks are the acute hepatic porphyrias.
Q. I have EPP and I have a severe reaction on my hands
and lips. Do I seek urgent care? Also, what can you even do
when you burn your lips?
A. The acute reactions to sunlight in EPP can be very severe and,
unfortunately, there are not many effective options to treat the
symptoms. Nonsteroidal anti-inflammatory drugs (NSAIDS such as