The first thing I see when I google “Porphyria” is VERY RARE. It says fewer than 20,000 US cases per year. So, I assume it IS a very rare disorder. If you scroll down, you can see “Porphyria vampire” as related search, but no, I am not a vampire.
When James and I started living together in 2017, A LOT of health crisis happened. One time, we were both on antibiotics at the same time. I like to think of it as… a dark period. I can’t really describe with words how bad I felt about myself from everything that was happening to me. Well, here’s what happened:
First, I have had 5-6 Urinary Tract Infection (UTI) in one year. I had UTI for the first time in my early 20s, which warmly reminds me how shocked I was that time and thought I was going to die. From then, I had a couple UTIs until mid 20s before James and I started going out. 5-6 UTIs was mind-blowing #s for doctors as well because they suggested I should go see a urologist, and mostly importantly it hurt like shit. AND, I was in grad school that time. I felt miserable getting over UTIs — one after one. I have wasted a lot of time googling how to prevent it, but nothing worked. I have tried cranberry juice, which I realized later it doesn’t do anything. I tried drinking a lot of water, taking supplements, exercising, and followed every basic prevention tips I could find online. NOTHING worked for me. And I went to the ER on Christmas Eve and naturally, had the worst Christmas ever. If you had UTI before, you know how shitty it is. You just feel DISGUSTED and there are no words to describe, it is just disgusting.
Second, my skin started to act out. I have had eczema since I was little. Since my eczema was getting better as I grew older, it’d appear seasonally on my fingers. This was not a big deal for me because it used to be much worse when I was younger that it was to the point I was ashamed to show my hands. However, this was different. I had such fragile skin, it gradually became worse and worse. At first, I thought it was eczema because I would get blisters on my hands and it’d be itchy. I could notice that my skin — on the top of my hands — was getting thinner and thinner that if I hit my hand somewhere, it would scratch my hand really hard. Just little bit of bump, my skin would be peeled off.
I have gone to see a dermatologist before it got this bad. He didn’t run any tests on me, he thought this was just eczema. Compared to skin cancer, my problem wasn’t as big and I think that’s what he thought. And because it was getting so much worse after seeing him, I made another dermatologist appointment with a different doctor. Luckily, this guy ran some tests on me. When he first saw my hand and after I explained what has been happening, he told me he thought it was Porphyria. That time, my urine color started to get pinkish. Plus, hair on my face and my hands were getting much more than before although people didn’t seem to think so. It was definitely NOTICEABLE for ME.
My screening labs suggested a condition called PCT (Porphyria Cutanea Tarda). He messaged me, “This metabolic disorder causes sun sensitivity, skin fragility, hirsutism and can be associated with iron overload. Your iron levels are also elevated. It usually occurs sporadically though there is a familial subtype.” He ordered another blood test and a confirmatory 24 hour urine collection. Meanwhile he suggested to use sunscreen with zinc oxide/titanium dioxide, no alcohol consumption, and stop birth control pill. He also told me treatment can include phlebotomy (if not anemic) to lower iron stores and make heme biosynthesis improved and also take plaquenil twice a week which may encourage urine secretion of porphyrins which are photosensitizing and accumulate in this condition. He also said, “I think we have caught your condition early” that treatment should work well and blisters will stop appearing and also likely some of the increased facial hair will disappear.
I started phlebotomy on April 2019 and did total of 4 phlebotomies so far along with continuing taking plaquenil 100ml twice a week. It has been a real painful journey because I can’t express enough how much I hate doing phlebotomy. I have never even donated blood because 1) I am underweight forever and 2) I knew it would hurt. And I had to do it every month for 4 months until I finally told my derm I wanted to take a break. The very first day I did phlebotomy, I fainted at the restaurant. I mean, I literally fainted at a PUBLIC restaurant. My friend came to provide me some mental support doing the blood work and we planned to go grab brunch afterwards. I didn’t really have much to eat in the morning, and I guess that was a big reason why. Ever since that time, I ate a big meal before going in to the lab. After 4 phlebotomies, my ferritin level was down. It started with 361 ng/ml and now it is at 102 ng/ml. Although I am within the normal range, I still do get minor blisters once in a while. And because it’s gotten much better, I sometimes get too relaxed and get lazy on putting sunblock in the morning and after a while, I will get a blister on my hand.
And as for UTI, I haven’t had UTI since Christmas Eve of 2018, the last time I went to the ER for UTI. I’ve researched a lot about PCT and although there’s not ample studies done for PCT (being a very rare disorder), I found a couple articles that led me to understand why I have been getting UTIs. I think there IS a connection between UTI and PCT. I don’t know if it’s the iron level or my liver condition, but with my ferritin level going down, I haven’t had one UTI.
My dermatologist wanted below 80 ng/ml for my ferritin level. I thought it’d still get lower by taking plaquenil alone, but it doesn’t seem like it is getting lower after over a month of watch. My derm thought it’d be a faster process to do plaquenil and phlebotomy together, and I guess he was right. So, I have decided to go for more phlebotomy until it is below 80 like my derm wanted. I would probably cry the whole time, but I know I have to do it. I would probably keep saying, “I can’t do it! I can’t do it!”, but I know I would do it. The crying part is very embarrassing, I know. Every time I am sitting in the chair and they wipe my arm and put that filthy thing in me to get my blood out, I can’t help pitying myself.
Sometimes I wonder if the pain is worth living. Whenever I faint (although I have only fainted a couple times), it feels like it is easier and much more comfortable until you wake up. It’s very quiet, peaceful, and blank. However, I know that cuddles and kisses are also comfortable and happy. It’s just that having certain health problems make people blue and dark — can’t help it.
Note: I am not suicidal.