Porphyria

     A lot of people ask me what it is I "have".  I don't want this to turn into a pity party, so I'm just going to talk about Porphyria.  I have a couple of things going on with me, but this is one of the major ones.

     Most people don't know a lot, or anything about porphyria; that includes the doctors.  The spell checker on most writing programs even say it's not a word.  That's because there is not a lot of research done for the disease because it's exceptionally rare.  

     The second problem is that there are several forms of porphyria.  Acute Intermittent Porphyria (AIP), Variegate Porphyria (VP), Hereditary Coproporphyria (HCP), ALAD Porphyria (ADP), Porphyria Cutanea Tarda (PCT), Erythropoietic Protoporphyria (EPP), Congenital Erythropoietic Porphyria (CEP), and Hepatoerythropoietic Porphyria (HEP).  And there are subgroups for several of these types of porphyrias. And... testing for any type of porphyria is difficult.

     Okay, so now that you've seen all the big words, and have no idea what they mean, I'll tell you how they affect me.  (You can click any of the types of porphyrias and read more about them from the APF)  I have AIP and either PCT or VP.  Like I said, testing is very difficult, and sketchy at best, so they haven't narrowed it down yet.  

     AIP is not fun.  Supposedly there are triggers for the attacks.  I have not been able to identify mine yet.  I stay away from all of the usual triggers (drugs, alcohol, sulfa-based meds, and certain foods), but attacks can also be triggered by stress, and, sometimes, because the mutated porphyrins have simply been building up.  When I have an attack I get severe stomach cramps (women who have AIP compare them to labor pains), nausea, I usually throw up several times, my whole body aches, and I have either diarrhea or constipation (I know, TMI, right?).  Most people have attacks, which are severe enough for hospitalization, but then move on with their lives.  A very small percentage of us AIP'ers have chronic symptoms in between attacks.  I always have a dull, but moderate, pain in my stomach.  It feels like the day after a surgery without pain-killers.  I know that sounds awful, but when you have that pain for 14 years, you learn to tune it out most of the time and it feels like a normal part of life.  Years ago, I always walk around hunched over because of the pain, but now I only have a hard time in the morning, before my brain remembers to forget about it.  I'm always nauseated, but it's the worse first thing in the morning.  I often throw up in the mornings, and after meals.

     The PCT or VP is not so bad, for me.  I only suffer symptoms when I'm having an AIP attack.  Basically, if I go out in the sun during an attack, I feel like Dracula. (The legends of werewolves and vampires actually originated from people suffering from different types of porphyria.)  My exposed skin gets a really bad rash which sometimes blisters and bleeds.  Most days I can go in the sun and be fine, but during attacks I stay inside with the blinds shut.

Here's a picture of one time I went in the sun during an attack.

     What I have is not contagious.  I'm not a real vampire, though that is my kids' favorite thing to tell their friends about me.  One thing, important above all others, is that I am not my disease!  I'm sure everyone with a disease or disability feels the same way.  People tend to only see your defect.  The first question everyone asks me is how I'm feeling.  I know they are trying to be nice/sympathetic, and I do appreciate the concern and love, but I am more than my disease.  It consumes a lot of my life.  I'm not able to do a lot of things.  I stay inside a lot.  I don't feel well most of the time.  But, I'm still just a normal guy.  I'm very social, at least I use to be, and I love to talk to people.  I like to go out and have fun.  Some days I can't do it, but most days I can live a pretty normal life.  Come say "Hi" to me, I don't bite. :)

     At any rate, there is little the medical community can do for those who suffer from porphyria.  It is a life-long disease.  I, like so many others, have learned to live with it.  I have a strong, supportive, loving wife who has picked up all the pieces I couldn't carry.  She even lets me complain to her when I'm not well.  I have three wonderful kids, who have learned to be independent because they have to be sometimes.  And I have supportive friends and family around me.  I've got a pretty good life.  I see some of the problems other people have to deal with and wonder how they manage.  We all have our little trials, this is just one of mine.