Part Two on happy hippie rose’s health: Current Update
Did you read Part One?
It’s about my medical history and explains where I’ve been and how I’ve gotten to now. It’s the lead up. Go check it out if you’re interested!
People have been asking me what’s going on with my health, so I keep promising to write an update. And then I keep finding reasons to put it off. I have so much sunshine to spew out that I hate to post about the crappier side of my life. The down side, my illness. But life isn’t all rainbows and giggles, and I can appreciate being real and down to earth, even if that means being Debbie Downer for a hot minute.
My Health Update
What’s going on with me now? I have porphyria.
Acute Intermittent Porphyria, to be more precise. I’ve known (for sure) that I have Porphyria since 2007, but I’m just now really dealing with it. I kinda dealt with it back in Leesburg in 2009 when I was in nursing school. I had a really cool hematologist who educated me some. But I’m just now REALLY dealing with it. It’s def been the focus of my 2011 and if I’m painfully honest: I’ve been pretty ill this year.
I’ve been way more sick (or is it sicker? I’m an English major, hello, I should know this kinda junk… I’ll go with sicker) I’ve been much sicker this year than you probably know, more worse off than you can glean from my facebook profile, not doing nearly as well as I let on. I promise I’m not lying to everyone I know, I just don’t focus on the bad stuff.
My illness is insidious. It sneaks up on me. I can hide it sometimes. I don’t have an obvious tell: no bald head like a cancer patient. No cast like someone with a broken bone. I look and seem normal. Sometimes, some days I am normal. But a lot of times I am sick. I wanted to write a post titled “I wish I had cancer,” and vent about how it is to have this untellable disease. Back in the cancer days everyone knew I was sick and I was given this sorta breadth of understanding. People just knew what was up, as soon as you see the bald chemo head, you just know. And there’s this quiet respect that’s offered.
But when people see me at church or in the grocery store now, when I post pics of snorkeling and hiking, when I leave my health out of conversations, it seems impossible that I’m anything less than spot-on healthy. So it’s hard to get the understanding that I used to get back when my illness was apparent. It seems like I’m not even sick sometimes. And strangers have NO idea, they can’t tell at all.
When you have cancer you still do tons of normal things. In between chemo treatments you function and live your life. So that’s how I am now. In between I have a life.
Of course I don’t really wish I had cancer. But I wish that people just knew that I don’t feel well, but I’m still alive, and kept that in perspective. I don’t want or need special treatment from my loved ones, friends, and acquaintances, just understanding. Not kid gloves, by compassion maybe? Everyone knows what cancer is; explaining porphyria is tedious. A lot of people readily realize that having cancer is a like a full-time job: so many appointments, doctor’s visits, diagnosis testing, treatments, taking medications around the clock, etc. I wish people realized how time-consuming my porphryia is for me. It is my full time job right now.
And then when I’m not at the doctor, on the days I do have energy: I can be normal-ish. I can hike for a few hours. I can go boating. Some days I can run. It feels good to be active, it’s good for me. Anything that keeps me moving and reduces stress is excellent for me. But just because I’m well enough to sit on a boat or walk to the dog park with my husband doesn’t mean I’m totally well.
That’s the hard part. If and when I am able to do things, people who see me don’t believe or grasp that I’m ill. At worst they think I’m faking, and at best they just don’t get it. Or sometimes when I feel miserable, people have no idea. So when I’m slow to get my wallet out of my purse at the store, people get annoyed. There’s no tag on my shirt that explains my hands hurt from my medicine. Every time I answer the phone people keep asking if they just woke me up, so I know something must be wrong with my voice. I just don’t feel good, that’s all.
Porphyria ebbs and flows. I have good days and bad days, good days with bad nights. That’s another thing: I sundown. I sundown badly (I’m crying as I admit this part, it makes me feel elderly, or weak, or just something not myself). I sundown; meaning I get worse at night, sometimes a lot worse. My pain gets worse. Sometimes I get confused. Last night I curled up in a ball shivering so intensely. Duggs put a blanket around me and I cried that I don’t think I’m ever going to be better. But yet…. just hours before I went on a three mile run. It’s hard for me to accept and understand, it’s hard for everyone to understand.
Once, back while Duggs was still deployed, I tried to rest. People were insisting that my activity level was hurting me. So I spent several days in bed. Literally. I was propped up like a princess. Back when I had my laptop, I stayed in bed all day long chatting online and watching shows on Hulu and Netflix. I ate my meals in bed. I didn’t do a thing. And it made me feel worse.
My doctors actually encourage me to be active. Yoga, walking, running, getting sun. Sun is good: the natural Vitamin D I produce in the sun is better absorbed than the supplements I take. The activity reduces stress. So I try to stay active, even if that’s confusing to people. The daylight is good for me, I love daytime.
A few hours of action isn’t wellness though. It’s a break. It’s a gift, honestly. I’m not working at all other than the tie dye business. Thankfully people haven’t questioned that too much or given me a hard time about staying home everyday. The sad truth is that I’m just not up to it. I’m not well enough for full-time work, yet.
Let’s back up… This genetic blood disease runs rampant on my Mom’s side of the family, having been passed down from generation to generation just like our recipe for red sauce. The disease works in an ebb and flow kinda way – sometimes popping in to make you feel crummy and sometimes going away for weeks, months, even years. During the ebb times the disease can be almost untraceable, not showing up in simple blood or urine tests. But when it flows, or rather when the patient experiences “attacks” or “decompensations,” the disease can announce itself in many colorful ways.
Literally colorful, one of the hallmarks is making your peepee dark. Other symptoms are relentless tummy aches, headaches, neurological problems from tremors to seizures, and on and on. GI problems, neurological problems, anxiety, insomnia, low blood pressure.
Like I said, it’s a colorful disease. And the disease has different forms, and people with different kinds have different symptoms. It’s complicated to say the least. Most of my doctors hardly know more than a textbook paragraph or two about porphyria.
But what about ME? What’s this all got to do with me now?
The miscarriage I had stressed my body out and caused me to “decompensate,” or in other words: have a porphyria attack. The bleeding, the physical stress on my body, the emotional stress, the crazy amounts of Motrin they treated me with: it all contributed to a porphyria attack. As part of that attack I suffered some nerve damage/inflammation in my neck/face, on just my right side. Porphyria can cause nerve cell death/destruction all over the body. So I emerged from the miscarriage feelings worse and worse, and my face and ear started hurting.
(Side note: my docs actually think that i don’t have crohn’s disease, that its really been porphyria all along, causing nerve cell death in my GI tract. crazy, huh?)
Okay, anyways… this porphyria attack (that probably started) in January caused the nerves on the right side of my face to get wonky. I have problems with cranial nerve five and six. The location of my pain mimics “trigeminal neuralgia” and for a while they thought I had that. I actually went into the ER thinking I had an ear infection, that’s how this whole thing started and how they got to the trigeminal neuralgia diagnosis. I wrote a blog about it a while ago: Blog on Trigeminal Neuralgia.
I saw a neurosurgeon back in the spring, who treats trigeminal neuralgia all the time, and she definitively said that’s not what I have. My situation is different. So, now i’m diagnosed as “peripheral neuritis, caused by porphyria.” They also call it “atypical facial pain.” So laymen’s terms: the blood disease I have flared up and in doing so messed with the nerves that concern the right side of my noggin; I suffer loss of sensation, facial pain, twitching, increased sensitivity to sounds, touch. It’s odd and hard to pin down. It changes daily. But basically, the blood disease I have creates neurological problems, and for me, during this attack, it seems to be making my face hurt.
I get terrible ear aches, but anatomically, my ear is perfect. I’ve had ultra sounds, MRIs, CT scans, X-rays, cameras stuck up my nose, you name it – they’ve done it. I’ve had lab work drawn, tests run, everything. And nothing is “wrong” with my ear! Yet it HURTS. The pain is crippling. When it flares up real bad, i’m rendered nonfunctional. The ear pain starts deep down inside, it feels like an inner ear infection, and it’s only ever on my right side.
The pain starts in my ear and radiates out. So my teeth, my jaw, my cheekbone and my eye socket all hurt. I get headaches behind my right eye. When I open my mouth and air touches my teeth, sometimes it hurts. And it’s only on the right side. It’s so weird!
So anyways, they have me on all kinds of meds.
Nerve meds, like lyrica. (i was on gabapentin, but that didn’t work out so well). I’m on pain meds and more pain meds. The Lyrica has all kinds of side effects: I retain water terribly. So I’m on diuretics (lasix) to help with that. But when you take diuretics, you have to take potassium, so i’m on that too. It’s a crazy cycle!
When I wake up in the morning, the water retention is the worst. My hands and feet get so puffy and swollen, I can’t even make a fist and it hurts to walk. The skin gets stretched so tight on my fingers that I get these little cracks in my skin. It hurts to type when that happens. So I take the lasix in the morning, and I pee all day long. If I take the meds on an empty stomach, I get sick. So I have to time my meals around my meds.
Since I had a small bowel resection in 2004 and I’m missing a couple feet of small intestine (I have no terminal ileum) I can’t take certain time release meds, or anything that absorbs enterically. I have to take stuff that gets absorbed in your stomach. Then, with porphyria itself, there’s all kinds of meds that are dangerous to take. Meds, meds, meds. It’s insane.
In May, before Matthew’s homecoming, I went to my docs and said I needed to get off all these meds. The pain was finally under control and I was doing better. So they started me on a taper. I was tapering off the meds, bit by bit every week. I had some rough side effects, like dreadful night sweats for one. By the end of June, I was pretty much done with most of the meds. I had like a week of tapering left to go.
It was amazing!
I felt like things were under control. I felt like the end was in sight and that I’d be back to normal. The big deal for me is that while I’m on all these meds I can’t get pregnant. They’re all really bad for in vitro babies. Sadface. So, tapering off felt great. I was proud of myself!
Then, I cracked that tooth. And I was in severe pain. Dental drama is a big deal for me. The meds they use at the dentist is bad for porphyria. Having dental work done stresses my body out. And since I’m terrified of the dentist, my anxiety and stress levels were going up and up.
So the doctors decided I should go back up on the meds. Stressful events kept piling on… and they decided to up and up my dosages to “hold me.” I mean, as soon as I cracked that tooth and my mouth started hurting, my ear started throbbing again. I know I needed the meds, but it was a big bummer to come SO CLOSE to being done, and then go back on again.
So, all of my docs: my neuro, the neuro surgeon, my geneticist, the metabolic team, my case manager RN, the pain clinic, and my hematologist all are in agreement that i need to see a porphyria specialist. That’s a pretty intense team, huh? I love my case manager. She calls me all the time and she sorta keeps everything organized for me. Cathy. She’s great. My neuro is the chief of his dept, and he’s the one who has really taken the reigns of this whole porphyria ship. He manages my meds and treats me for the ear pain. He’s amazing.
My geneticist has been eductaing me about porphyria and she’s been building a family case for me. You see, the disease is SUPER genetic and about half my family (on my mom’s side) seems to be affected. I won’t call any of them out specifically… but yeah. Anyways. The geneticist is amazing. She made me an emergency protocol. If I were ever hurt or let’s say – in a car accident – and brought in to the ER, I’d need special treatment because of my porphyria. So I have these papers I keep with me, and Matthew keeps a copy with him, the glove compartment, etc. – explaining what to do.
I have to wear a medical ID now. They made me a red dog tag, but since that’s ugly I have a Road I.D. bracelet. It’s awesome.
There are only five porphyria specialists in the US, and they’re all on the mainland. So my case worker has been organizing my trip to see one of these doctors. I still have no idea where I’m going or when – but it’s in progress. I’m having some genetic testing done now for one of the specialists. I’m probably going to go to Mt. Sinai in NY.
So yeah. My face hurts all the time. I wear my medicine patches on it. Sometimes I ice it. I get nerve blocks and shots of steroids in my neck and shoulder to help. I have a HUGE muscular knot in my back on the right side which might be playing a role in this. The steroid shots are “trigger point” injections. It’s a nerve path thing. They shoot you in one area to affect pain in another area. A shot in my back can aid the pain in my face, because that’s how the damaged nerve path works. In theory. I’ll try anything, so I get the shots often: sometimes in my head (that didn’t work well), sometimes in my back, sometime sin my neck. We’re trying to figure out where the damaged nerve path is. The shots have some numbing medicine (one of the -caines, like lidocaine) in there and some steroids.
I’m learning more and more about porphyria as all of this goes on. It’s been crazy. 2011 has been a wacky, roller coaster of a year!
From my porphyria notebook the genetics dept prepared for me:
Rose has the clinical features and a family history for Acute Intermittent Porphyria (AIP). The human porphyrias are clinical disorders reflecting defects in heme biosynthesis. Acute porphyrias cause acute attacks of neurological symptoms that can be life-threatening. Acute attacks are triggered by environmental stressors such as certain drugs, sex steroid hormones, reduced intake of calories and carbohydrate, alcohol, and unknown factors. Many of these factors stimulate heme synthesis in the liver, which in the face of a metabolic enzyme defect, leads to increased production of heme precursors that may be neurotoxic.
Does this link work? It’s for a video i posted on FB of what happened to my face after some trigger point shots my doc gave me. Oy. Half of my face went numb! I tried to upload it to this post, but I can’t figure it out. My apologies if the link doesn’t work!
So that’s what’s been going on. This weird balance of okay and not-okay, of heathly times and illness. I seem to be getting better, in many ways. In some ways, I’m stalling out. I need to get to a specialist, one of the five porphyria clinics on the mainland. Hopefully, that’s something that’s able to happen and I’m able to get better so once Duggs is out of the USMC I can work. I want and need to be able to work, so hopefully something will give soon.
Any prayers for healing, good vibes, good thoughts, anything happy is VERY appreciated.
And, I will update. I’ll update sooner next time, I promise. As always, thanks for reading. It means a lot. Love ya, happie hippie rose
PS – and let me just add… and i’m such a heel for not mentioning this earlier: my husband has been so amazing throughout all of this. He comes to as many appointments as he can, he takes care of me dutifully and with love. He knows my whole medicine routine and regimen better than I do! He is the best. He cares for me with so much love and tenderness, it’s beyond words how blessed I am to have my Duggs caring for me day in and day out. Thank you my love, for taking such good care of me. I’d be a goner without that guy for sure, he’s the BEST.