28 03 2014

Here I go, blogging on a stupid iPhone. But I’ll do it, this once. I’m feeling a strong pull to get some things out of my heart and into the world. (Maybe one of you blessed readers will send me a pretty computer in the mail to replace the one that a toddler decided to explore and destroy, thus allowing me to share with you in a manner that doesn’t drive me crazy from typing on a phone screen. A phone, people!)

I’m at a pinnacle of sorts. On an edge. A razor’s sharp, shiny edge. One wrong move and I fall hard. Or slice myself right down the middle, from crotch to head? Maybe that.

I came home from the hospital again seven days ago from yet another week-long stay. I’ve stopped writing about my attacks because I doubt they’re interesting to anyone anymore, yeah? But for those of you keeping track, it was a grand total of something like 7 visits (totaling about 48 days or so?) in 2013, and 4 so far for 2014. Each incident brings a new complication, becomes a little harder to treat, requires more intervention. I lose energy, momentum, will, dignity, modesty each time. My body doesn’t feel like mine anymore. There are no more private parts. After as many catheters, monitors, enemas, scopes, and cameras as I’ve had stuck in and on my body, there is just no room for propriety any longer.

I’ve gone through being unresponsive with only 4-6 breaths per minute, prompting the rapid response team to race to my bedside. I’ve laid perfectly still and remained calm while they told me they were about to administer a drug to stop my heart, in the hopes of resetting it and breaking it from the pattern of supra-ventricular tachycardia it was stubbornly racing through. (Feeling your heart straight-up stop is terrifying and exhilarating and I’m almost inclined to recommend it.) I’ve experienced moments of searing nerve pain so excruciating that all I can do is beg the nurse to rip all the clothes off my body, so I squat on the bed naked, minimizing anything coming into contact with my skin, and cry, because no medications stop nerve pain. I’ve watched blood splatter out of my mouth during moments of violent hurling, from the gashes burned into my esophageal track after such constant illness. I’ve had two ports yanked out, and three put in, because after several months of hemetin and dextrose they gunk up and stop working, essentially threatening my dripped-in promise of life.

And these attacks have, for the most, fallen into a neat little pattern. About four days before my period is to start, I usually feel the first twinges of pain. Usually less than 48 hours from that point, I’m hooked up to my machines and pumps and I’m struggling to post squinty smiley pictures on social media to assure everyone I’m fine.

Seeing as the attacks are following such a tidy calendar, the obvious (to many) conclusion is to eliminate that calendar. Take away my cycle. Treat me with hormones. I started talks with one of my doctors, who happens to be a smart, quiet, funny researcher from NIH for whom I have a great deal of respect and fondness, about just this a couple of months ago. In my mind, we were talking about birth control pills (or a shot or IUD or something,) to stop Lady Flo in her tracks. But In his mind, we were not discussing birth control at all.

We were discussing menopause.

Dr. NIH wants to give me a hormonal therapy that will send me into post-menopausal hormone levels. He dropped a few printed pages of a study done on the treatment for AIP, and said “This is what we’ll do. Find a gynecologist to work with and we’ll get stsrted.”

Just like that.

As though facing menopause at 33 is something I shouldn’t take issue with. Sure! Let’s shut the ovaries down! Dry that silly uterus up! Gain weight! Get hot flashes and mood swings! Kill what libido remains after 5 years of chronic illness! Make sex painful and difficult! Get wrinkles and saggy parts! Turn old, now!

Of course it may not be quite that extreme. And I don’t mean to be offensive to women who are menopausal! (I’m being hyperbolic here because I’m sort of panicking, obvi.) I’m not one to say that I’m dreading menopause, WHENEVER THAT MAY NATURALLY HAPPEN.

But y’all. I’m 33. I’m not 100% sure I’m done with my baby making! I’m feeling (other than sick and exhausted and beat-down,) pretty bangin and confident, actually. I’d like to sit in this third-life space for a while, experience my thirties as a 30-something year old. Not having to worry about significantly increased risks of lady-cancers (I already have a greatly increased risk of liver cancer to work with,) with crazy-times mood swings and even less energy that I have now.

Maybe I’m making too big a deal of it .

Maybe I should do it and shut up .

Maybe I can’t.

This has literally stopped me in my tracks. Reading through the study has not encouraged me, with only about a 25% rate of good results, out of only FOURTEEN WOMEN , one of whom died… But if I don’t, then I am selfishly robbing my family of the opportunity to not lose their mother/wife/teacher/housekeeper/meal-maker/laundry-doer monthly to serious illness and recovery.

I don’t know what to do. Obviously I have more research to sift through, but it’s hard to come by, and no clear answers are going to glare at me from the pages of study summaries . I have a strong feeling that my decision will ultimately be made from my gut. The one that bleeds when it’s upset with me. My heart. My silly heart that beats too fast and too hard and needs round- the- clock monitoring at times.

This is the beginning of the end of living in denial for me.

You see, I truly have been in denial since I was diagnosed. You may not think I have, but I have. Each time I experience an attack , I think it’s the last (you’d really think I’d have caught on after the 12th or so hospitalization…) And I have a deeply buried belief that I am responsible for being ill, that my symptoms are not some disease that I innocently inherited through no fault of my own, but that are manifestations of my own poor decisions and sins. And that porphyria wouldn’t rule my life if only I didn’t let it.

It’s time to face my life head-on now. Either find a way to live peacefully with the disease , or find the fortitude (and energy and money) to fight the disease. I think there are merits in both. Validity in wanting rest and surrender and passivity, and validity in not wanting to roll over and settle for this as a way of life.

Which way will I go?

It’s time to make some choices.



A girl at every port…

17 05 2013

So I’m home and re-ported now! The surgery went well. I was at least not awake this time. (I’ll have to share the story of getting the infected port out with no sedation next post…)
I know the staff of IR (interventional radiology) well now, thanks to a cranky old port that required quite a bit of… intervention. So I felt pretty comfortable going into it this time. After some back-and-forth with the anesthesiologist about which drugs we could and couldn’t use, we landed on a pretty simple plan.
In the freezing cold theater of IR (I came prepared this time with wool socks,) he started me with a hefty dose of fentanyl to take the edge off. It made me relaxed and friendly. We were all besties there for a few minutes, me and my anesthesiologist and nurses. I shared my hopes and dreams, funny stories, embarrassing stories, dark secrets, and promised to name my next child after them all. Then he gave me the actual anesthetic and that was that.
I woke up asking for more of it.
I remember them telling me it’s what killed Michael Jackson, see why he liked it so much, hahaha… Geez. Glad they didn’t mention that little nugget of trivia before sending it into my bloodstream.
I felt good and woozy for a whole, then the pain came, so they generously dosed me up with some toredol and morphine. After an hour or so of laying around and nibbling gluten free pretzels, I decided it was time to go. I stood up and promptly decided it was in fact not time to go.
There it was… The sea legs and double vision and nausea and floppy muscles.
I’m home now and still a bit woozy, but I’ve managed to eat something decent, but the morphine has worn off and my chest and neck hurt like a bitch. I forgot how bad this hurt the first time! It absolutely sucks folks! Good thing I have a cabinet full of narcotics, which I’ll be dipping into momentarily.
Before I go, I leave you with a treat. They removed the picc line after surgery was over (they used it during surgery instead of starting an IV.) And pulling a picc seems so closely related to extracting a giant blackhead, so Eric couldn’t resist taping it for your viewing pleasure 🙂 Click here to watch it.


Well, then.

13 05 2013

It seems I’ve committed the worst of blogging sins. I’ve started and stopped and started and teased and stopped.

Here’s the thing, folks. Two kids, a crappy computer, lots of weird moves and shuffles in life, and the blog hit the back burner. I’ve thought of it often, and I’ve been asked about it and I get comments every now and then with questions from readers, and I’m always like “one day, I’ll get back to that!”

And a year later…

My last post was a year ago.

My next-to-last post was a year before that.


If you’ll have me, I’d like to tip-toe back into this space. But maybe in a different way?

I like to write. A lot. But your time is precious, and so is mine. And you don’t have time to read epic-length blog posts, and frankly, I don’t have time to write ’em.

But I’ve had so many, many relevant experiences and thoughts and ideas in the last year, and I’d really like to be sharing them again. It’s cathartic to me at the very least. And it’s potentially helpful to some of you.

So let’s try again, K?

Here’s the quick rundown since my last post: That illness I spoke of last? Yeah. Resulted in 4 nights in the hospital, with an attack, step throat, and a staph infection in my bladder. It was SWELL. But the hospital let little 5 month old Esther Pearl stay in the hospital with me, sleeping in my bed, with no interruption to her nursing schedule, Eric in the pull-out chair thingy next to us, so it all worked out ok.

Then life went all roller-coaster on us, and we ended up moving to Alexandria. For Eric’s job. That told him he HAD to move or he’d lose his job. So we lost our house to not lose his job. Then he lost his job anyway. And we were stuck in Alexandria, in what felt like The Most Expensive City In The World in The Most Expensive Apartment Ever.

So that was fun.

(He found a new job after a few months, and he’s doing great now, and we moved out of that dumb expensive apartment and to a more rural area of western Northern Virginia in a great big old stone house with swings and a big yard and a marsh and a creek. It’s all good.)

So, I was ok-ish until spring of last year. Specifically Mother’s Day 2012. The day porphyria came to visit. It apparently decided I needed a Mother’s Day vacay, so we checked into the hospital, porphyria and me. We stayed for 4 days or so. In the end, it was all worth it: The hospital gave me a lovely little Mother’s Day gift of some decorative ceramic measuring spoons from Pier One. Win!

A few months later, in August, another attack slammed me out of nowhere. (OK… Not exactly out of nowhere, but more on that later.) This time, it was my birthday! Weeee! Four nights that time. But again, as with the Mother’s Day gift, the hospital pulled through with gluten free doughnuts stacked up and topped with whipped cream as a special “cake” for me. Even though I was too busy puking to eat it, I was pleased as punch, as it was the first time in YEARS I didn’t have to make my own birthday cake.


My special birthday cake concoction on the left. A regular, glutenous chocolate cake on the right, that they threw in for my visitors. I was told it was the worst cake ever in the history of cakes.

So then on New Year’s Eve, which was also our 5th wedding anniversary, porphyria was all “Oh? Another special day we can spend together? Let’s hold hands!” I woke up New Year’s Eve morning thinking I was going out on the town with my groom-of-five-years. I ended New Year’s Eve in the ER with yet another FALL RISK bracelet on my wrist, pumped full of fun mind-altering drugs, aiming for the little bean-shaped vomit caddy the hospital so generously provides, coming up for air to tell my date (Eric, of course,) stories about the orange children’s chairs floating in the corner of the room. Yep.


Eric took this in between vomits. It was a very fleeting, drug-induced, shit-eating grin. In my head, in that moment, I was probably telling off the imps who kept shaking their little butts in my face. It was a weird night. Pain+Delirium+Narcotics+Fever+Dick Clark’s NYE Special on TV= Weird Freaking Night.

That one was almost a week in l’Hotel l’Hopital, thanks to a very special strain of the Flu From Hell. Not only was a pain pump not keeping up with the ninjas punching me in my stomach, but I couldn’t breathe without some O2 assistance to boot. It was, by far, the scariest, most horrible hospital stay yet, thanks to some serious incompetence on the part of the hospital staff (to be discussed later) and some serious asthmatic behavior on the part of my lungs.

And last, but not least, to round out this last year of getting to know porphyria all the more intimately, I was yet again invited to be a guest of the hospital for 5 days, thanks to an infected port. That was a couple of weeks ago. The port was yanked (without sedation and with very little pain medication, by the way; A really fun story for another post!) IV antibiotics were administered in the hospital for five long, boring days, a picc line was put in, IV antibiotics were continued at home (mostly by my six year old daughter) for another 2 weeks, and now that I have absolutely no sign of flora or fauna or bacteria or fungus or elves in my system whatsoever (thanks to 2+ weeks of shooting powerful antibiotics straight into my veins,) I’m allowed to have a new port put in, which happens this coming Friday.

That’s where I am with this. Two years in a nutshell.

I’d love to share more with you about each of these ordeals, and what I’ve learned (and not learned,) but not here, not today. I’ll feed you little bites, yes? We can do this snippet by snippet.

And when the bullshit explodes like it does, I’ll give you play-by-plays in realtime! No more playing catch up. How does that sound? (Friday should be good, y’all. Watch out! Should I live-Tweet the surgery???)

Don’t be mad at me for disappearing. Let’s just pick up where we left off. Pretend that year didn’t go by. And get ready for some more me!

So, that’s where I’ve been….

15 05 2010

So my worst nightmare became a reality.

I had an attack.

While pregnant.

It started a couple of weeks ago I guess. Really, the second I found out I was pregnant, I started feeling shitty. But that’s pregnancy, right? I figured fatigue and nausea were par for the course, and even though I was getting hit harder than I did with Adelaide, I figured it was because I also have porphyria, and maybe it would just be a little more intense.

But then there was the pain.

I remember feeling a little crampy with Adelaide, and being freaked out by it. But this time, when the cramps turned into exercises in deep breathing, I scoured the internets to assure myself that cramping was perfectly normal in early pregnancy. Some is, of course. But not lots. So I thought, well, maybe it’s a UTI. I’m no stranger to those. And it’s pretty common to get bladder infections in pregnancy. So that must be what it is. Yes. UTI.

I went to a Patient First, and told the doctor that I had AIP, was about 7 weeks pregnant, and was having pelvic pain, but no bleeding, and I needed a urine culture. He promptly replied, “What’s porphyria? How do you spell that?” Great.

Culture came back clear as could be. But he said that the symptoms matched with a UTI, so why not try an antibiotic anyway? The first one he suggested was a big two-red-triangle no-no on the porphie unsafe drug list. So he panicked, and rather than rationally and calmly decide on the next best thing, he threw a bottle of amoxicillin at me.

I never took it.

I downed cranberry pills for the next several days, and convinced myself that the pain was easing up. It wasn’t really, though.

By this past Thursday, queasiness had turned into full-fledged nausea, and even a little vomiting, which I still tried to attribute to being knocked up. I took Adelaide to her homeschool co-op, and couldn’t eat because I was so nauseated, and I gritted my teeth through the morning, still in denial that the pain was bad bad bad. By the time we left school, I was so miserable that I cried the whole drive home.

I emailed my hematologist a couple of times that day. First to say that I was freaking out a little about having such strong pregnancy symptoms that were so perfectly mimicking porph symptoms, to which she replied that she was sure everything was fine, and we’d kick up the glucose infusions if we needed and if worse came to worse she’d suggest some pain meds that were safe during pregnancy.

Then I emailed to tell her that taking any pain meds made me really uncomfortable, and I’d rather get hemetin, since it’s “just” a blood product, and not something with neurological effects. She went along with that.

Then I emailed to tell her I was going to the ER because I give up.

So Thursday night, Eric drove me to the Bon Secours ER facility in Harbour View. The plan was to waltz in, announce that I have porphyria, am pregnant, and in increasingly severe discomfort, and required an immediate glucose drip and another urine culture for a UTI, and that I would NOT be accepting any pain meds.

I don’t know what I was thinking.

I did waltz in and do all of that, and explain to several clueless nurses what AIP is. And then the PA on duty swaggered into the room and said “So, when you were in the ER last fall, they were considering celiac sprue. Have they abandoned that idea?”


“Uh, I have porphyria. I need glucose.”

“Yeah, well there’s really no test for porphyria, so you can’t really know if you have it.”


“Uh, yes, there is. And I have it.”

“No, those tests come back with false negatives and positives all the time, so you can’t pay much attention to them.”


“I’ve tested positive twice, and I have Acute Intermittent Porphyria Type II. I NEED GLUCOSE.”

By the way. As for those tests. I looked up my hospital records from my attack in the fall. The preliminary urine tests they ran look for two things that would point to porph: ALA and PBG levels. A normal ALA range is 0-35, and my level was 157. A normal PBG range is 0-8.8. Mine was 130.  hmmph.

So, this was the first time I’d ER’d it since my diagnosis, and I always figured that in the event I needed emergency care, the docs would be glad that I was so educated and absolute about what I needed. I never guessed I’d have to prove to them all over again that I have the freaking disease.

It was obvious to me that this guy saw “porphyria” on my chart and googled it real quick. He ran his own battery of invasive, offensive tests, but as I continued to bombard him with AIP information and medical lingo, he sort of lost his resolve and finally gave in and admitted that I needed a push of glucose. Three hours after I arrived.

So then shift change happened, and doctor number two came in, and I had to do the same thing all over again (although, he was a little quicker to accept my story.) But by that time, I was in really severe pain, with a kickin headache, and I was really, really trying everything I could to get through it without meds. I had an ice pack strapped to my head and was rocking back and forth, trying to get in a trance. And crying. I cried a lot. I finally asked for some tylenol, which doc told me was perfectly safe during pregnancy (I don’t think I ever took any with Adelaide,) and it helped take the edge off the headache. Just enough to let the excruciating abdominal pain really shine through.

So doc starts trying to convince me to take something stronger. He told me that he used to work as an OBGYN, and that opiates are considered perfectly safe during pregnancy. He told me that, in fact, heroin is an opiate, and that moms who use heroin throughout their pregnancies give birth to perfectly healthy babies.

I had a feeling that his idea of healthy and my idea of healthy are two different things.

But after agonizing over the pros and cons of being in severe pain and distress, versus taking an opiate that medical science claims has no effect on a fetus, (and after thrashing and screaming and sweating and not being able to meditate through the pain anymore,) I gave in and accepted a 10 mg morphine drip.

He also recommended an antibiotic drip, since there was a tiny bit of bacteria in my urine, and he told me that UTIs are really dangerous in pregnancy, so treating them is essential.

After these two drips, the pain calmed down, and the puking began.

I puked all the way home, and all through the night. The urinary retention kicked in, too, so every 10 minutes I was on the toilet, pushing little squirts of pee out while Eric did pee-pee dances for me.

The next morning, I woke up and puked. Eric took Adelaide strawberry picking, which had been planned for a week and she was totally excited about, so I didn’t have the heart to tell her she couldn’t go. They came back with big smiles on their faces, and a huge bucket full of bright red, big, juicy strawberries. And red lips.

Eric tried to get a little water/food into me, but I promptly rejected even the most trivial amounts of anything that went in. I entered the puking-up-bile stage, until I was all empty of that, too, and just dry-heaved the rest of the afternoon.

I went to the hospital for my infusion of hemetin and glucose. Again, deciding to get the hemetin was heart-wrenching. There have been no studies on the effects of it on a pregnancy, and it’s recommended that it be avoided by pregnant women, save for only the most dire of situations.

This was dire alright.

Normally, in an attack, I’d get four doses in four days of the hemetin, but my hematologist was not comfortable with that. She only let me have one, in the hopes it’d give me the boost I needed, and we’d follow-up with lots of glucose.

So I knew it’d take a day or two for the hemetin to make any difference, and I went home to suffer. I told Eric I was absolutely NOT going to take any more pain meds, and that he’d have to just help me through it.

But he and my mom couldn’t deal with watching me writhe. My mom decided to get a hold of Jennifer, who was my palliative care nurse when I was hospitalized last fall. On the phone, she first tried to convince me to head to the ER, where I could receive pain meds under supervision. But I told her about my less-than-impressive experience the night before, and adamantly refused to go back. She understood. So she talked me through my options. She explained that receiving the 10mg drip in the ER of morphine was equivalent to about 30mg orally, so if I took 10mg orally at home, it wouldn’t knock me for such a loop. And we sort of hashed out the risks to my unborn that come along with me being in severe agony and distress and anxiety. That’s almost as bad (if not worse) than a chemical. (In fact, another pregnant mom friend of mine had recently told me of an article she read about a study showing that high levels of anxiety are more detrimental to a fetus than alcohol consumption. So we decided we should start a new trend of drinking up. For the sake of the baby.)

I gave in and took a 10mg of my liquid morphine, and it was the perfect amount to take the edge off the pain, let me rest, and not send me into complete lala land.

Rinse and repeat.

I think I took three or four doses over the next couple of days, and each time, I had to have my arm just about twisted off. Eric had to keep reconvincing me that I was making the right decision.

I’m still not sure about that. But what’s done is done.

I got three infusions of glucose this past week, and I’ll get three more next week. It’s not fun. It’s 500 grams of sugar being pumped into me over the course of 30 minutes. I leave the hospital feeling like I could lift cars, and then crash and burn an hour later. Only to then crave massive quantities of cheesecake and ice cream. Sugar is addicting, did you know?

But the pain is 100% gone. The nausea is mild, and what I’d associate with typical first-trimester gunk. I’m tired as hell, but there are ways to deal with that (like plopping Adelaide in front of the TV so I can nap. Several times a day.) Oh, and in the ER, they did an ultrasound of Babo, who appears perfect with a healthy heartbeat. And single. Thank god.

So I’ll raise my glass of sugar water to getting through that, and may it be the only time during this pregnancy I have to go through it. No more drugs for Babo!

Calling all vampires!

12 04 2010

I forgot to tell you all about the phlebotomy thing.

So, for whatever reason, getting the hemetin infusions, while good for controlling porph attacks, can also increase hemin levels (duh, right?) which means too much iron. High levels of iron can lead to an attack, and I was even told by my hematologist that she’d like me to stay on the “slightly anemic side.”

So, only a couple of weeks into the treatments, my iron levels were too high (of course, because it’s me, and why wouldn’t they be?) (And also, phtthtthtth to all you people who think that my being vegetarian means I’m a walking medical journal entry for anemia.)

So, when the iron is too high, the way to remedy that is to bleed me. Yep. Take the blood right outta my veins. Stick the leeches on and let ’em go.

Not really leeches.

The nurse used my port, rather than an arm vein, and was supposed to take 20 viles, but could only get to 17 because the port got all backed up with clotting blood.

And here’s what all my blood looks like.

mmmm. bloooood.

Yep. There it is. Just chillin on the table. Ready for the ol’ garbage can. Which I felt totally guilty about, by the way, since there was a patient RIGHT NEXT TO ME getting a blood transfusion. I felt so dirty, like I was burning dollar bills in front of a homeless person or something. (My nurse told me that years ago a patient who got regular phlebs would take the blood home in a plastic freezer bag and use it to fertilize his roses. But hospital regs have since changed and they won’t let me take mine home to fertilize my roses. I asked.)

Yeah, I almost fainted when it was done. Not because of the blood loss. But because of the freaking blood loss. Seeing it all just sitting there in tubes on a table…. It was so strange. Blood is life, you know? I watched it pour out of me and fill up tube after tube and I wondered if they were going to leave me with any, and I sort of symbolically felt my body collapse a little, like it was sinking in on itself. Like a beach ball with an air leak.

It’s a very surreal experience, a phlebotomy. Lucky me, I get to look forward to more!

I have to admit, it was made all the more interesting by the fact that I was, at the time, reading through the stupid Twilight series, and I couldn’t help but note all sorts of dumb irony: the timing of my decision to read my first ever vampire tale (and a lame one, at that,) having my blood sucked, having it sucked because of the disease I have, which is responsible for the myth of vampires in the first place… It’s all just stupidly too much. Seriously, I think I’m growing fangs. Right now.


26 03 2010

I know, I know. I’m really sorry, I am. But here’s the thing. After two attempts at writing this post, wordpress erased it all, which made me want to poke my eyeballs out. Writing it once was tough, then having to write the whole thing again was exhausting, so after that got dumped, I didn’t have it in me to do it again for a while. Plus, I have to admit, I got a little sick and tired of being sick and tired. I needed a break from thinking about porphyria. I took the “SICK” label off my sleeve and left it in the drawer for a while.

But a few of you have emailed me. prodding me for the rest of the story, and I owe it to you. I really do. So, (as I slap the label back on my sleeve,) here it is.

I left off with the hemetin infusions. Good for my attack, bad for my arms. And since the plan was for me to start getting the infusions once a week for the rest of my life (or the rest of porphyria’s life, whichever comes first,) there needed to be a better way to get the hemetin into me than forcing it into my unwilling, hardened, bruised veins. My biceps had already puffed up into warm, firm masses that would make Popeye jealous. So the nurses told me how to fix the problem. A port.

What’s a port, you say? That’s what I said too.

So, a port, or a mediport, or a portacath, or a Totally Implantable Venous Access System, or TIVAS, is like one’s very own permanant little IV that gets to go everywhere said person goes. The port part is surgically implanted under the skin, with an attached catheter that is surgically inserted into an artery in the neck. When it’s time to infuse, a nurse pokes a little needle through the skin into the port and the IV runs directly into it, and the contents of the IV are then run through the catheter directly into the artery. The advantage is that this way, the nurse doesn’t have to find a usable vein to insert an IV into each time, AND, since it’s being dumped into a big ol’ artery, rather than a tiny litte arm vein, the thick hemetin flows right on in, pain-free. Its like one of those beer-holding helmets with tubes: no need to waste time picking up the can to take sips. Just let gravity do the work and deliver it directly to your stomach.

The kind suggested for me was the Bard Power Port. The nurses brought one out of the back room that Bard had kindly provided for educational purposes. It’s a purple little thing. A triangle box about the diameter of a silver dollar and the thickness of, I don’t know, about a seven dollar stack of those silver dollars. Maybe more like five. How thick are silver dollars? Anyway, on the top of the triangle, there’s a little silicone soft top, with three little bumps on it, that work kind of like brail, so that when a nurse is trying to access it, she or he can feel for the little bumps, and aim for the middle.

I held it, and it made me want to throw up.

But the nurses needed only to turn my attention to the searing pain in my arm, and I was convinced.

They also arranged for me to meet Heather. Heather is another AIP porphie. Here’s that story:

When I went to my PCP, Dr. Lea, to tell her that an attack was coming on, she told me that she’d just recently spoken with a collegue, a hematologist, Dr. Bremer, who said she was currently treating an AIP patient. Her patient was getting weekly maintenance infusions, so Dr. Bremer had gotten the whole set-up down pat. She told Dr. Lea to send me to her, especially if I had an attack, because she already had access to the hemetin (which can be difficult to get a hold of sometimes.) So the timing was perfect, and I was absolutely THRILLED to find a hematologist who’d be proactive in my treatment. And, of course, I was super interested in this other porphie! Imagine that! Another one, right here in town! The odds are not in favor of that AT ALL.

So when I showed up at the infusion center at Norfolk Gen., you can imagine that the nurses sort of looked at me like I had just pulled a rabbit out of a hat. “ANOTHER porphyria patient?” they’d said. “Unebelievable.” They quickly got to work scheming. They were like little matchmakers. Like moms who’d stumbled across a real nice, wholesome single lady that they just HAD to make sure their 35 year old son-who-lives-at-home-with-no-real-promise-for-a-bright-future met. They were just so excited for us to get together. I’d learn a lot from her, they’d told me. She’d been doing this for a while. And she had a port, so I could see hers. (Yippee.)

So my blind date was all set up. When I went to the infusion center for my third day of treatment, I glanced around at the chairs. I was really hoping the elderly lady coughing up all sorts of phlegmy spittle wasn’t Heather. Or the lady laying unconcious in one of the beds, who I’m pretty sure had peed herself.

But then I saw her: A young, “healthy” looking gal, looking kind of bored. Like she’d been doing this for a while. She saw me at the same time, and I sat in the chair next to her. We exchanged hellos, and I was tickled pink. I determined just from the tone of her “Hi” that I liked her.

I learned that she is 25, and moved here in October from some northern snow hell of New York State. (No offense to NY, but the thought of being buried in snow 8 months of the year truly is my personal hell.) She’d been diagnosed with AIP when she was 16 or something, and had been having a really, really rough time with the disease since. She told me that about 7 years ago she had her first port implanted. It lasted a little while, but then got clogged, so she’d had to have it replaced. The second one was put in too loosely and would flip around (gag.) so she had that one replaced as well. So she’s now on her third. She showed me the scar, which looked kinda substantial to me, but I had to take into account that it’d been cut on a few times. She told me that the skin over it just turned into numb scar tissue, so she didn’t even feel the stick anymore. She’s been having weekly infusions for years. If she misses one, or even goes a day or two over 7 between infusions, she starts having an attack.

Phew. Seeing the scar and watching the thing get poked and hearing about the mishaps made me think twice about getting the port. And then they spent 20 minutes trying to get a good IV in. And then my arm was on fire for hours. And I told the nurses to schedule my surgery.

The very next day, I received a call from a vascular specialists’ office to schedule my surgery time. She asked if I could come in the NEXT day, Friday, and have it done. I panicked. That just seemed a little fast for me. I felt like I needed to, I don’t know, at least tell my friends and family that I was going under the knife, and maybe arrange for someone to watch my kid. And I asked if she or the doctor was aware that I had porphyria and therefor could not be given just any drugs, and that many anesthetics are unsafe for porphies and that all meds needed to be checked before they were administered. She paused. She said it was probably best to not schedule it for tomorrow, and that she’d talk to the doctor and get back to me.

After several phone calls and reschedules, I was finally given an appointment for Tuesday morning at VA Beach Gen. Usually the procedure would be done in the specialist’s own outpatient center, but because of an issue with my insurance, they’d had to schedule me in an actual hospital. Which turned out to be a good thing.

I was told that my surgery was scheduled for 8 AM, so I needed to arrive by 6. Holy hell. So Eric and I set our alarms for 4 AM, and then, just to make things fun, I started having withdrawal from the morphine. I’d gone on it for about 8 days during my attack, but I wanted to come off it quickly so I wouldn’t have withdrawal symptoms again. So much for that. I was awake all. night. long. I really don’t think I ever slept at all. I had the heebie-jeebies so bad that my entire body thrashed and jerked for hours. I was sweating and itching, and my mind was racing. And just when I felt like I was calming down a little, both of our phone alarms screamed at us.

So in my zombie state, I walked myself into the hospital at 5:45 that Tuesday morning. I was actually looking forward to the surgery, because it meant I could sleep. Really deeply. As I waited to be called back to the pre-op room, a nurse informed me that I needed to have some preliminary blood work done. I froze. You see, I’d already had a little pep talk with my arms, telling them that they only needed to give me one more good stick. For the IV they’d have to use to put me under, of course. And then my lovely arms were free to heal and relax, and once again let blood circulate freely through them. I was not, however, counting on TWO sticks that morning. And with my lack of sleep, I couldn’t take it. I cried the whole way down the hall, and I sat in the chair in the lab and sobbed. The lab tech taking my blood looked really, really sorry, and really, really perplexed. She either thought I was a drama queen, or just plain crazy. She found a little vein to get the blood out on my left hand, in between my index finger and thumb. It hurt. And I cried more.

So by the time I was called back to pre-op, I was beyond exhausted. I was done. I wanted to run. But I didn’t have the energy. So instead, I tearfully changed into my really aesthetically pleasing bluish greenish gown, my shapeless, thin socks with grippies on the bottom (which are actually required in all hospitals, to reduce one’s fall risk, because, yeah, THAT’S what’s gonna keep me upright when the drugs get to me and my blood pressure plummets.) I asked the nurse for all the blankets she could give me, and I “snuggled” up in the bed and waited for Eric to join me.

Several minutes later, he walked in and made a poop joke or something, and I cried some more. I really was starting to feel doomed, like I was willingly laying myself on a bed to be wheeled off to my death at the end of the hall. A nurse came in to start my IV, which made me cry more. I explained that there was nothing left, that all my veins were shredded. She didn’t believe me, until she tried to find one. After a few obnoxious sticks, she found one, again on my left hand, up high between my pinky and ring finger knuckles. A teeny tiny little thing. And I’d feel every drip-drop of everything they’d put in me that morning. It was awful. So I cried even more. I was certainly not establishing myself as the strong soldier warrior patient ready to beat my disease and destroy the enzyme enemy.

When the nurse said she was going to start my antibiotic drip, I asked what it was. I decided I was going to check all the drugs against my two databased: the 80 page Swedish database I’d printed out and carry with me at all times in a hot pink binder, and the NAPOS online database. The vascular specialist’s office had assured me that they’d checked on the safety of all the drugs they’d be using, but I figured it could hurt to double- and triple-check for myself. And man, am I glad I’m an anal, obnoxiously researchy kind of patient.

The antibiotic was a no-no according to one list, and not classified (which means a no-no,) according to the other. She said she’d wait on it until I’d talked to the doctor. The one cutting me open. Whose name I hadn’t known until that morning. And whose face I wouldn’t see until minutes before my sugery was scheduled. So I asked the nurse to tell me all of the other meds that were supposed to be used during the procedure. Wouldn’t ya know it, most of them were at least questionable, and a couple were outright red. Dangers. Do-not-uses.

So when Dr. Vascular Specialist came in for his obligatory greeting, we kind of laid into him, frantically. He humored us enough to start rambling off, in a really know-it-all, lazy, stoner sort of way, alternative drugs he could use for sedation, and Eric, with the printed list, and I with my my iPhone searching the online database, would look each one up. The procedure was a simple one, typically performed in the radiology dept, with just the surgeon and a nurse present, with some sedation and a local anesthetic. But after Eric and I vetoed many of his drug choices, and he figured he couldn’t even so much as use lidocaine to numb the incision site, he finally got serious about it. With each “Nope, that’s red in this list,” and “Nope, that one’s unclassified,” I saw his body tense up more, and his suaveness dissolve. He ultimately decided to move it into the OR, with actual anesthesiologists.

Of course, this was a scheduling nightmare for the OR staff, and so my 8 AM surgery, for which I’d arrived at the hospital at 5:45 AM, didn’t happen until 11:30. In the meantime, I had three anesthesiologists, one vascular surgeon, and two nurses crammed into my little room, naming drugs while Eric and I looked them up. We finally all agreed on a plan of action. Glad I could be there to do their job for them.

Around 11, the anesthesiologist came in to start my drugs. I cried again. Each time she’d push something new into the IV, I was sure my vein was going to explode right out of my hand. I actually wailed a little. Screamed “fuck!” alot. She looked at me like I was crazy. And she warned me that the stuff she’d use to put me under was known to sting normal patients, who didn’t cry like little babies, so it’d probably just go ahead and light my hand on fire, for real. Then she pushed a little sedative to calm me down. But it didn’t really, and I could tell she was annoyed. (When dealing with someone like me, who’s been on heavy narcotics for some time, sedatives and things don’t work the way they would on someone, well, normal.)

I was completely alert when they rolled me down the hall into the OR. I was kind of surprised by what I saw. I always imagined ORs to look like this, but it felt more like this. OK, not at all that bad, at ALL. But it was really messy and cluttered with, I dunno, operaty stuff. I wondered if they’d managed to squeeze me into the OR schedule by actually using a storage room instead. I supposed they don’t really count on patients being aware of their surroundings when they’re in this room, or else they’d tidy it up a little. At least make me think they cared what I thought. Kind of like, when I’m having someone over for drinks, I at least shove the dirty dishes into the oven, so it looks like I don’t actually let them stack next to the sink until the smell of mildew forces me to break out the scouring pad and pick which ones will just get thrown away. I’d like for my guests to at least think I care about their opinion of my worthiness as a housewife, even though it’s all just deceitful packaging.

So before I could take in too much of the clutter, the nurses hoisted me onto the table, and strapped me down. A plastic mask was placed on my face with forced oxygen, the smell of which always makes me think of almost-cookies. Or the smell of rubber dog toys when they’re brand new. The mask wasn’t placed well, though, and instead of giving me nice big gulps of air, it had the opposite effect and made me feel like I was trying to breath through a pillow. But just as I went to adjust it, I realized I’d been swaddled. My right arm was firmly wrapped to my side, and my left was firmly strapped down to the arm extension thing on the table, so I couldn’t move. Which, while laying belly-up on a table with the brightest lights ever blinding my vision and faceless blue bodies whose eyes were obscured through goggles, made me feel, well, a bit, um, vulnerable. Before I could freak out out loud, though, the anesthesiologist (golly I’m tired of typing that word!) told me that she was about to start the really sting-y stuff. And holy freaking shit. It was amazing. I’ve never felt that kind of pain in my life! And I never want to again! I had to sort of marvel at the grandiosity of the sensation… Like my hand and arm were dissolving in acid from the inside out. I got out a half-scream, I think. And that was it.

Surgery over. Ported.

Forever to be metal-detected. Fun times in airport security lines to follow.

Then I woke up, again, way more alert than anyone expected, as they were moving me back onto the gurney. They wheeled me down the hall, back to the recovery room, where I greeted my nurses coherently and asked for some damn food. Eric joined me, and agrees now that I was not at all acting like someone who’d just been put under. It’s that tolerance to narcotics I’ve got now. The pain meds, did, however, leave me a little brazen, because I thought it’d be a really good idea to talk to one of my nurses about my lacey underwear (and show her, ) and to another about constipation and my experiences with it.

Anyway, everything went fine, I was ported, and I went home to recover. It hurt. A bunch. For a while. And it took me a long time to get the nerve up to look at it in the mirror. In fact, the day after the surgery, I went to have an infusion, and when the nurse changed the bandages on it, I almost fainted. Not for any particular reason. Just because I’m still a wimp somehow. And then a couple of days after that, I was supposed to change the dressing again, and rather than do it myself, I went to Adelaide’s preschool class, sat down in a little chair (not in the same room as the kids, of course,) and made one of the other moms, who happens to be an RN, do it for me.

About a week post-op. That line going from the lump (the port) to my neck is the catheter that goes into my artery.

But it’s all healed up now, and it only hurts a tiny bit now and then. Having it accessed hurts, because it’s kind of like getting a nose pierced or something. They really have to shove the needle with some oomph to get it through all layers of skin and the silicone top. But imagine having  your nose re-pierced every single week. And I’m, of course, allergic to the tape. Even the hypoallergenic tape, and it itches and leaves a rash. Every week. Just when the rash clears up, I get more tape.

But the hemetin seems to be holding the attacks off thus far. It’s making me really tired, though, so maybe I’ll adjust my dosage or frequency of the infusion. I’m going to my hemetologist today to discuss these things.

I’ll also try to get a better technical explanation of what hemetin is and why it works, and why it sends my iron levels sky-high and requires periodic phlebotomies. Oh yes. Phlebotomies. That’s another story for another post. I have really graphic pictures! Anyway, hopefully I’ll be able to understand it all more myself, and then pass that knowledge along to you. Since I know it keeps you up at night.

I’m back, like an attack. Or with one anyway.

16 02 2010

OK. Here I am. I’m back.

Truth be told, I didn’t write for a while because there just wasn’t too much to write about, porphyria-wise. That, and I stayed really, genuinely busy during the holiday months. I know, I know. No excuse for leaving my loyal readers in the dark. I do apologize for that, but I’ll try to make up for it now.  (And, I’d also like to throw in that our internet has not been working properly for the last few weeks, making it very difficult to do anything online, but after countless hours of phone tech support and two new routers, I fixed the problem. Just for you.)

So, like I said, I stayed really busy over the holidays. Too busy, in fact. I found myself constantly teetering on the edge of attack-mode. I played a game of pushing myself really hard, then crashing and trying to rest up and do absolutely nothing for a couple of days to ward off an attack. Not the greatest strategy, I’d find out.

By late December, I’d weaned myself off morphine. That was a HUGE accomplishment. I was in no way addicted psychologically, but holymoly, was my body hooked. I created a very calculated and slow weaning schedule, and stuck to it, but not without feeling like a heroin addict in detox. (I even smeared black eyeliner under my eyes and didn’t wash my hair for two weeks, just to look the part.) I had headaches, night sweats, muscle spasms, nausea, and worse of all, the all-over heebie-jeebies. My body was in constant motion. My muscles felt like they were itching, and the only thing that lessened it would be to shake or kick or punch the air. It made for some fucking awesome bedtimes for Eric. I begged him to give up and sleep on the couch until I was through it, so he could actually get some sleep, but being the good supportive husband he is, he stuck by me. Somehow. I think he just has a secret fetish for junkies.

So I coasted along, staying somewhat healthy, getting back to the gym and working out pretty hard… And then a couple of weeks ago, BAM.

I felt the ab pain coming on for a couple of days, but I tried to ignore it. I decided that mind-over-matter was the way to go, just because I didn’t want to deal with another attack in the midst of both of us being unemployed and trying to file for disability and Cobra and unemployment checks… The paperwork for government assistance has gotten so intense that I’m now using it as toilet paper when we run out. And, frankly, I knew that being sick would mean more paperwork to drown in, and it was just too much to deal with. But by the time I was leaning over the egde of the bed barfing into a trashcan (that Eric had insisted on placing there after I kept complaining of having “that queasy feeling that doesn’t actually lead to throwing up”,) I gave in and admitted defeat and headed to my doc’s office.

So, it just so happened that my primary care physician, the AWESOME Dr. Lea LaPlace, had recently mentioned to one of her hematologist friends, Dr. Bremer, that she had a patient with porphyria. And Dr. Bremer said, “Yeah? Me too.” Turns out she’s been treating someone for a while, ordering weekly maintenance hemetin infusions for her, so they schemed and decided that Dr. Bremer should take me on as well. When I walked into Dr. Lea’s office to tell her I felt an attack coming on, she told me that lucky for me, my timing was good, because she’d found me a new hematologist with hemetin. This was really, really good news.

She called up Dr. Bremer to tell her it was showtime. This was on a Friday afternoon, and Dr. Bremer told her she couldn’t see me until Monday, so I had to go home and maintain the attack until then. I was OK with this, since I still had a cabinet full of morphine, and Dr. Lea told me she’d alert the ER in case I got to the point that I couldn’t hold any food down at all, so that I could just pop in and get a glucose IV. Luckily, I didn’t get to that point. Good thing, since this all happened the same weekend that Portsmouth had 12 inches of snow, which hasn’t happened, um, ever.

This is your brain on drugs.

Getting my first infusion of the week. Just glucose and dilaudid.

I stayed in bed for the weekend eating nothing but potatoes, rice, and glucose tablets. And morphine. Monday didn’t come too quickly at all. I stumbled into Dr. Bremer’s office, seriously on the brink of about-to-lose-it. She sent me straight back to the infusion room, and hooked me up to a glucose bag and some dilaudid. The narcotic helped my pain situation a great deal, but I barfed my way through the treatment. Cute. She then called Norfolk General Hospital to set up my hemetin infusions. The plan was to get me in for four days in a row of infusions to halt the attack, and then start on weekly maintenance infusions, to continue for as long as I need it. In other words, potentially for the rest of my life.

The next day, a Tuesday, I went in for my first hemetin experience. And what and experience it was. Eric drove me to the hospital and we pulled right up to the front door and took advantage of the free valet parking. He stayed with me the whole time, since I was sick and pukey and drugged. We walked the maze to the back of the hospital to the infusion center: basically a big room outfitted with reclining lounge chairs, each with its own TV and TV Dinner Table. And complimentary IV stick.

They started with the glucose, which was set to drip for an hour, and they told me they’d call and order my hemetin from the pharmacy. They have to wait and order it after I get there, because once the pharmacy mixes it up, it has to be administered immediately. And the plan was to let me have about half the glucose, then stop and give me the hemetin when it was ready, and then give me the rest of the glucose. Well, before they ordered the stuff, they had to take my medical history. And when they posed the question “Are you, or might you be, pregnant?” I couldn’t say definitively that I was not. I told them I was probably not, but that’s not good enough, apparently. So there was a pregnancy test ordered (which happens to be the running joke between Eric and me, that everytime I see a doctor, the first thing that happens is that I get a pregnancy test,) and they had to call Dr. Bremer to find out what to do in case it was positive, which it wasn’t, but the point is, they lost precious time, and by the time the hemetin got to me, there was no glucose left. I didn’t know it at the time, but that would pose a little problem.

You see, the hemetin arrived, and what I saw absolutely floored me. It was a big glass jar full of black, thick, frothy liquid. It left a greenish tint on the glass. It made me throw up in my mouth. They had a decent IV in, in a pretty good vein. But. Once they started the sludge, my arm was on freaking fire. It was horribly painful. My very kind infusion nurse wrapped my arm in warm blankets to try to help, and Eric dumped dilaudid pills down my throat, but geez laweeze. Crazy pain. And the problem with the glucose being gone was that there was nothing to flush the hemetin through my veins after the infusion was done, so it just pooled in my arm, and I went to bed that night feeling like I’d lifted cars over my head.

Second day of infusions, pre-hemetin.

Hemetin nasty

That vile you see on top of the IV poll full of black guck is the hemetin being pumped into my vien. Mmmm.

Hemetin tube

...and there it is. That tube is not a black tube. It's a clear tube, with black foamy pudding in it.

The next day, the timing was better, and I got a good 30 minutes of glucose after the hemetin, and of course, they used the other arm, so it wasn’t AS bad, but it was still bad. It became very clear that this set-up just wasn’t going to work. For anyone. The nurses had a harder time each day finding a vein to use, and I had a harder time not punching them in the face while they looked for the vein, and my arms were turning into swollen masses of lumpy, veiny, hard, blue, hot pain.

My infusion nurses had a solution. One that my new other-girl-with-porphyria-friend utilizes, but is not without great risk. One which I’ll tell you all about in tomorrow’s post. And I’ll explain how I met said friend. Which I’ll write as I go get my weekly infusion.

I’m tired and going to bed now. But I promse, it gets interesting and kind of gross, with awesome graphic pictures.

Good night for now, folks.