I know I said I would use this blog to talk about not only writing, but my life. It is time for me to relate a very important piece of this to those who read these words. I have a disease that dictates many aspects of my life. It is called erythropoietic protoporphyria, or EPP for short. With that giant question mark that just popped above your head, I now will attempt to explain what that is.
According to the American Porphyria Foundation's website:
Erythropoietic Protoporphyria is characterized by abnormally elevated levels of protoporphyrin IX in erythrocytes (red blood cells) and plasma (the fluid portion of circulating blood), and by sensitivity to visible light that is usually noticed in early childhood and occurs throughout life.If you just made a face to go with that question mark, let me clarify. The protoporphyrin that has accumulated in every portion of my body that blood flows through is irradiated by long wave UV rays resulting in photosensitivity. This photosensitivity does not feel like a normal sunburn when you have been out swimming too long. It's more like holding your hand directly over the flame of a campfire, but not being able to pull it away. Or like getting hit with hot grease when cooking bacon. Or like a steam burn. Repeatedly stabbed by tiny needles works too. I've even heard the pain being compared to putting your hand on a hot stove and cuts filled with salt. To sum it up, the sunlight hurts.
Now, before any of you feel bad for me, I will come right out and say that I've been lucky. My case remained relatively mild until my sophomore year of college. I was able to wear tank tops and go swimming in my childhood and teen years unlike some children who have more severe cases of EPP. I've always known that I had it because I was diagnosed by my father, another with EPP, but I was formally diagnosed in June of last year. I was able to have a "normal" life, but perhaps that is why it is so hard for me now.
With the onset of spring this year in a new region of the US called central Cali, it has been especially difficult. Where before all I had to do was cover my arms and hands when outside, now I must cover my head and face. If I am "burned", light from my computer screen and those nifty florescent light bars they use in commercial buildings irritate the damage. Now I must wear gloves and sleeves when just getting online, and I can't take off my cardigan that I wear over my work shirt when it gets too warm at work because I am surrounded by brand, spanking new lights.
The ordeal has been frustrating with such gorgeous days. I am relegated to closed windows to limit the amount of exposure, something that is surprisingly hard on me. I never thought I would ever get this bad or feel this amount of pain. I never dreamed that I would have to plan my day around how much exposure I would get. I heard it said by a man with EPP in a video posted by the FDA that it was more like, "how much pain do you want to deal with that day." I think that is about as accurate as a person could get. When burned, I am tired and in pain. I feel the strong desire to tear off my skin, if it's especially bad, and often suffer from bouts of irritability. I feel like a wounded animal who just wants to limp away and find a cool, dark place to recover. The one thing that doesn't happen to me is the outward signs of scabbing or discoloration. I swell a bit, but stay pale.
So, how do I deal? I suck it up. I use my weekends to stay inside and recover from any exposure that I've gotten. To limit my exposure to strong light, I have lowered the brightness on my computer. My typewriter is also getting a pretty good work out lately since I can't spend too long in front of the screen. I also keep my house cool to lessen the pain, much to the chagrin of my husband, but he deals with his favorite snuggie. (He wears it like a robe.)
As you can see, I'm managing but the mental implications have been more difficult to overcome. I know I'm trying to relay my experience, but there are some things that are still too personal for the internet. Besides, I'll feel like I'm whining if I haven't been already. My father has probably had it a lot harder than me, so I feel like I'm putting my foot in my mouth, but I do have one more thing to say:
Being in public is a strange experience when you're all covered up. People look at you with concern and skepticism. It's almost like they think I'm going to rob them, even if I'm driving in my own car sitting at a stop light. Once a lady gave me the most terrified look I have ever seen before she took a right. This is where I voice my confusion on societies fascination with vampires and the romanticism associated with them, and yet very little know about people with photosensitive disorders. When I tell people what I have, they look like they have found Bigfoot. If they have already heard of or known someone with a porphyria, they often ask very polite questions about how I'm doing and are generally pleasant to talk to. Yet, I still have a conflict with what I like to call Vamp-fans. The popularity vampires have in our culture makes it easier to explain what I might be going through, but at the same time certain aspects of it baffle me to no end. To sum up this conflict, I shall relay a conversation I once had with my mom in a chinese restaurant. I believe it was triggered by a girl wearing a Team Edward shirt. (The following conversation may be paraphrased.)
Mom: I can understand why younger girls might like vampires, but I don't get the women who are in their thirties, or even my age, who are into it. I mean, I watch those things, Buffy and Angel, but I don't understand how obsessed they are with it.
Me: Mom, you married a vampire.
Mom: You're right, I did.
A fun revelation it was, but the best thing ever said was my husband: "Who ever thought being married to a vampire was romantic must have been on something."
There you have it, my short, half-assed explanation of what it's like for me these days with EPP. I could get more in depth, but I think I'll let the pros do it for me. Visit The American Porphyria Foundation for more on EPP, the innovations to treat it, and other forms of porphyria. For the best stories on the experience of living with it, the FDA interviews with two well spoken gentlemen and a doctor are worth viewing. They won't take much of your time and will help you fill your quota for Porphyria Awareness Week (April 22-28). I see that question mark again, so yes, that is a thing.
I'm going to go work on my book now.