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.