Girl 1, Pemphigoid 0

Hello everyone! I write to you all with some exciting updates:

First off, I have been officially declared in remission! <Insert celebratory dance> This means that my disease is no longer actively rampaging through my system, though my treatments will continue as usual for the time being. (Recall that with pemphigoid, treatments are continued even after the disease is quiet, and then very slowly withdrawn, so as not to exacerbate anything.)

I must admit that the declaration - which took place at Dr. Foster’s office in Boston - felt rather anticlimactic. I had gone into that appointment hoping to discuss strategies for how to cope with my eyes, because the current predicament I was beginning to depress me. I was happy that they were less red, to be sure, but every day seemed to revolve around putting in eye drops. Wake up, put drops in, wash face, drops, brush teeth, drops, figure out what to wear, drops, drops, drops... even my sleep was interrupted by needing to wake up to put more gel or ointment in my eyes. It was common for me to go through an entire 70-count box of eye drops (.6 ml each) in about three days and at the time of my doctor’s appointment, my right hand was actually sore from squeezing stuff into my eyes every few minutes.

You know it’s not a good sign when you start developing an overuse injury as a result of your eye drop habits...

These were strange times to be sure, I’d tell myself, but once I was better, everything could be normal again. I could get back to working, driving, studying. I could be a doctor and hopefully turn this freak occurrence into something positive, using it as motivation to help others during their struggles with some aspect of their health. The problem was, by Dr. Foster’s account, this was better. Honestly, I was more frightened hearing that, than I was when I was diagnosed.

Anyway, after freaking out and then finally processing this news, I got back down to the business doing battle with my eyes. I’m pretty sure Dr. Foster thinks I’m exhausting, poor man, because I’m constantly asking questions, pestering him about treatments, and generally pushing to make my eyes the best they can be. Part of the problem may lie in that I’m literally, his youngest pemphigoid patient. According to another physician Dr. Foster works with, all the others are significantly older and of those, most are retired. Obviously, getting sick at any age is a horrible thing, but to have this happen to you during your retirement years is, in my mind, much less disruptive than having it happen to you in your early 20s, when you’re just trying to get started out in life.

So perhaps this is why Dr. Foster thinks it’s odd that I can’t accept things as easily as his others patients. “You were a very severe case,” he says, expecting me to content with the large improvement I’ve experienced. But I’m not. Because what I’m fixated on is the quality of life I’ll experience while in remission. Unlike his other patients, I’m not retired, and I’m facing 60 or so more years with this disease. If I want those years to be productive, it’s imperative that my eyes be as close to normal as they can be.

The result of this line of thought was that I went back up to Boston and basically insisted that that my upper puncta be occluded. For those who are unfamiliar with ocular anatomy, the lacrimal puncta are essentially little holes at the inner corner of your upper and lower eyelids that allow your tears to drain from your eyes. You have four, in total. Two on each eye. For people with reduced tear production, stopping up these little canals traps your tears so they can’t just uselessly drain away down your nose. This can be done with tiny little silicone plugs, or with a surgical procedure that shuts little hole completely. There is some discussion about whether or not trapping all your tears on the surface of your eye is entirely good for you, as this is thought to perhaps increase the amount of inflammatory junk your eye is exposed to since now nothing can drain from your eye. I assume that this is why Dr. Foster didn’t suggest this on his own. But I decided that felt that complete occlusion (having all four puncta closed up) was something that I wanted to try, since it had the potential to significantly improve my symptoms. Prior to this, I’d had only the bottom puncta (one on each eye) plugged.

So this leads me to my next piece of news: thanks to the procedure, I can now last a whole hour without eye drops! Granted, sometimes it’s less than that. Later in the day, or if the air is particularly dry, then I can maybe only go fifteen minutes. But on the whole, I’ve experienced a vast improvement from the endless eye drop cycle of before and so I have no regrets about pushing Dr. Foster to do the procedure. To put things in perspective, here are a few things that I can do now, without having to stop to “top my eyes off."

• take a shower
• bake muffins
• wash a load of dishes
• sit through a job interview

I know, I know. What a glamorous life I lead.

Annoyingly, the plugs sometimes fall out (I’ve had to replace the new ones twice already) and terrifyingly, it’s possible for them to get “lost” and cause infection. The latter really freaks me out, so I’m considering asking that my puncta just be surgically (and permanently) closed. But for now, I’m just happy to feel like my life has some greater meaning beyond incessantly pouring stuff into my eyes.

In other news, over the last few months I’ve been studying for the MCAT and generally just trying to get things lined up for applications to medical school. I’ll admit that sometimes I get really nervous about it - mostly because I worry about what admissions committees will think when they see that I haven’t done much over the past year besides some volunteering... But I’m lucky enough to have lots of people around me to basically tell me to shut up and calm down when I start to get really anxious.

And finally, if things go according to plan, I’m going to return to work soon! I’m in talks to begin working with a very nice woman who does research in autoimmunity and I think the word “excited” just doesn’t cover how I feel about all this. I don’t want to say any more, so as not to jinx anything, but fingers crossed that I’ll have lots of new and exciting developments to tell you about in my next installment!

Please address any complaints regarding this post to the 50 mg of Benadryl currently in my system. I'm writing this in the midst of a treatment and it's pretty much a miracle that I'm still conscious and writing in complete sentences.

The No Makeup Day

Have you ever read Psychology Today? I can remember the very first time I picked up a copy of the magazine.... It was in a Barnes and Noble and I thought it looked scientific and therefore possibly interesting. Instead, I found a multitude of articles that seemed designed to make you feel bad about yourself, including one on attractiveness - expounding on the idea that the more symmetrical you are, the more attractive you are.

I used to think about that study constantly because a few years ago, I had an eye surgery that was done poorly. (So poorly, in fact, that it didn't even solve the original problem.) It left me with a mild incomplete blink - meaning I can't shut my eye quite all the way anymore, and essentially no eyelashes on the upper lid. Neither were risks that I'd been warned about.

I waited for months for my lashes to grow back and for my eyelid to "drop down" completely, like Dr. Anna Rothstein, the doctor who did my procedure, promised. But neither happened. Suffice it to say that it was quite upsetting and that I learned the hard way to choose my surgeons more carefully.

And so began my life as a girl with an asymmetric face.

Perhaps if I'd hadn't read that article, or never seen a cosmetics ad featuring models with perfect lashes framing perfectly symmetric eyes, I wouldn't have felt so badly. But whatever the cause, I never felt the same after that and so I went from being the girl that wore makeup for fun, to the girl who wore makeup because she "had to." Every morning I would put fake lashes on one eye, and then eyeliner on both. Then I would stand back and hope that my eyes looked even enough for no one else to notice the differences.

I got sick of this routine quickly though. I missed going out without makeup on, but without it I felt so self-conscious that I literally wasn't able to look people in the eye. I found myself trying to turn slightly to the side, so that people couldn't see my eyes straight on. I decided it was worth it to spend the fifteen to twenty minutes each morning putting eye makeup on, so that I didn't have to feel so awkward. It's pretty terrible, feeling ugly all the time, and unfortunately, I think that this is something that many people with chronic illnesses have to deal with.

Anyway, last week I had a mini-breakthrough: I skipped my makeup for an entire day. All in all, it wasn't so bad. If anyone noticed my uneven eyes (which they probably did) at least they didn't act differently or oddly, which was pretty much my greatest fear. I think it's difficult to admit that you have a disfigurement, and even more difficult to show it. So I'm a bit proud of myself for being able to do that; I often don't feel very brave.

I think what precipitated this change was mainly that I was:

1. Exhausted - now that my inflammation is more under control, I've resumed my medical school plans and I'd been studying the night before. 
2. Not looking forward to a day spent pouring eye drops into my eyes and worrying about keeping fake lashes glued onto one lid at the same time. Trust me, this is quite an exhausting balancing act. Especially when you're putting in drops every few minutes, as I do. 

Ok, so maybe it wasn't really bravery that got me to do this... But hey! Progress nonetheless!

I still miss my "pre-surgery" eye, and hopefully one day I'll be able to fix the damage done, but until then I guess it's not the worst thing in the world to be a little asymmetrical. We all get a little down on how we look sometimes, so I just wanted to remind you all to focus on what you have, rather than what you don't. I certainly wish I had appreciated my eyes a little more before all of this.

For the imperfect. 

Above all shadows rides the sun. Let's just stare at the sun.

I've been delaying my blog postings out of a perfectionist desire to deliver nothing but good news regarding my health. Today was the day the realization that this was an absolutely stupid idea dawned on me. I went out of town this past weekend and was feeling quite good the day I left. Over the past few months I've been riding a bit of a roller coaster of minor health issues: colds and infections, some severely unpleasant treatment side effects, etc. So on Friday I thought, "I should write a post when I get home! Before something happens again and I end up with only mediocre news to deliver and feeling like that little cloud that follows people around in antidepressant commercials."

I swear I'm not trying to inspire this emotion and drive you all into the arms of Zoloft.

And then I woke up this morning with two very sore and moderately inflamed eyes. 

I have no explanation for why this happened. Things are definitely much much better for me now, but still a little unpredictable. Suffice it to say that I'm a bit tired of constantly reporting my symptoms. It's just, well, depressing! I don't like depressing. As proof, I will offer up the tidbit that my favorite Shakespearean play is As You Like it. You know, the one that the starts with a banishment and after a few disguises and some hanging of poetry on trees, ends with everyone getting married? Sometimes realism is overrated and you just want a reason to smile.

In a similar vein, my most beloved part of Lord of the Rings (the movie - this line was a rewording of the book) is when Sam says:

It's like in the great stories Mr. Frodo, the ones that really mattered. Full of darkness and danger they were, and sometimes you didn't want to know the end because how could the end be happy? How could the world go back to the way it was when so much bad had happened? But in the end it's only a passing thing this shadow. Even darkness must pass.

Well, I like the part where they destroy the ring too... But you get the point. I like heartwarming things! It's time to start skipping the darkness and danger in my story. It's getting tedious and while Tolkien may have been able to lead readers through three really long books before the happy stuff, I am not he. I would rather focus on the funny, the ironic, and the happy parts of my tale - as was my original intention, though I got a bit lost along the way. It's guaranteed to be more fun for everyone and it also ensures that I won't feel like a constant bearer of gloom who then needs to hide from her blog in shame. I will, of course, provide updates if anything very awful happens - which it won't! I am much improved from where I was this time last year. Or, if anything very amazing happens - which it probably will! Because remission seems very very close!

So in honor of this restated theme, the description under my blog title is being edited from the old, "a fight against dry eye, cicatricial pemphigoid, and autoimmunity," to what it now reads.

As Frodo says, "that's what I like. It makes me laugh."

Me too Frodo. Me too. 



This post was inspired by a Ukrainian Orthodox Easter weekend full of far too much food and apparently, so much fun that my immune system got a little excited too.

Veinless Wonder

Due to recent events, I feel the need to make those of you out there with "good veins" aware of your excellent fortune. First, I will define the term "good veins" to encompass those lovely visible (or at least palpable) bluish-greenish vessels that sometimes protrude, rope-like, along the arms of you lucky ducks. I never thought I'd envy anyone who looks like this:

 Courtesy of Wikipedia 

But look at that vasculature! I am jealous. Jealous enough to have wondered if starting a serious weightlifting regimen would help push my seemingly non-existent veins closer to the surface. You see, my veins are the shy, retiring type. The nurses look, they feel, they tourniquet, they hot-pack, they cut off my circulation with a blood pressure cuff until my arm turns blue, but still, the introverted buggers can't be easily found. For a girl who requires IVs/ blood draws at least 6 times a month, this is highly ironic. Case in point: one day I ended up being poked 7 times before the IV team (I like to think of them as a SWAT team for IV placements) was called. And after all that the IV was finally inserted in my most used vein, which I'd been trying to avoid all along as it was starting to develop scar tissue from the repeated punctures. Now, at the Wilmot Cancer Center, where I go for my treatments, you are allowed to leave IVs in for 72 hours maximum, assuming you prove yourself trustworthy enough to not inject IV drugs in to your accessed vein... I passed this test with flying colors by apparently being so naive about this possible use that I was looking around in desperate confusion asking, "what do you mean I need permission to leave it in? I promise to wrap it up and not get it wet or dirty or pulled out just please let's not do the great vein hunt all over again tomorrow!" The nurses thought this was cute and I could hear them retelling the story of how the thought of shooting anything into my IV "didn't even cross my mind" to my doctor - which made me seriously wonder how many people actually did think of this nefarious usage on their own.

And so the IV was left in along the inside edge of my right wrist for the three remaining days of my infusion. Over this period of time it proceeded to ache like crazy, presumably because it was in such a sensitive spot, and I was forced to mount feeble attempts at washing my hair left-handed since any movement of my thumb intensified the pain. As I have a fairly large amount of hair, this did not go well, and ultimately I was left with an unconditioned frizzy mess and a healthy regard for opposable digits.

Anyway, I do tell this rambling story for a reason and it's because I hope that maybe I can offer some tips for those of you who have also experienced the joys of hard-to-find veins. This is what seems to work for me:

1) Hydrate - drink lots of water starting the day before your anticipated needle stick. When your dehydrated your veins can shrink down on you a bit and become more difficult to locate. This is fairly common advice.

2) Cut down on salt - Have you ever felt puffy after eating too much chinese food? Yes? Well part of that awesome feeling is due to water retention in your tissues from the excess sodium in that tasty lo mein. I find that making sure I stay away from anything salty helps my veins to be more visible.

3) Exercise - if you can, squeeze in a workout the day of your blood draw or IV placement. Exercise causes your blood vessels to dilate and helps to bring once hidden veins to pop up. I find that I stay rosy-cheeked for at least a few hours after exercise and my veins seem to stay up too.

4) Use heat to your advantage - ask for a hot pack to help further dilate veins once you get to the location of your stick. Doctors offices and labs usually have these on hand. I find that running my arm/ hand under very warm water works even better than using a hot pack.

5) Speak up - if you have tricky veins, say so! My aforementioned experience was primarily a result of me being stubborn. I've drawn blood before and I really don't find needle sticks too alarming, so I'm a pretty good sport about being poked and prodded. I know it can be nerve-wracking to draw blood. You don't want to hurt the patient, you don't want to look bad, etc... so I joke and laugh about my silly, reticent veins, try after try. That is, until I see a needle wiggling around so aggressively that it pokes back up through my skin. I didn't mind the pain so much as I minded the unnatural sight, which proceeded to immediately break me out into a cold sweat. However, this experience did result in the Santa Bruise - which made me feel pretty special:

 

 Can you spot Old St. Nick? Complete with white beard and hair? 

So moral of the story: unless you want a festive contusion, tell the nurse/ phlebotomist that you have difficult veins. This generally results in them calling over one of their eerily skilled colleagues who seem to have a sixth sense for finding blood, which means less bruising and pain for you. As long as you're not mean about it. As in don't yell about "only an attending being allowed to put your IV in" like a woman near me did. In that case, I can't guarantee anything...

This post is dedicated to my boyfriend. Who kindly put up with me as I gleefully plotted out locations where I could theoretically draw blood from his nice bouncy veins and simply accepted this as normal behavior.

Return of the... blogger

Hello again all. As you may have noticed, I have been a bit neglectful of my blog recently. I apologize for my absence and will now proceed to place the blame squarely on my eyes. Suffice it to say that they took a turn for the worse for a long while. Doctors were visited, medicines were changed, and my eyes began a month or so of completely inconsistent behavior before finally beginning to calm down to their current state (which is actually pretty darn good). What did I do during my good spells you ask? Well, Christmas cookies, Star Wars I-VI, and Lord of the Rings may also have had something to do with that... I'm going to claim that this was all both necessary and good for the healing process because 1) thumbprint cookies make me happy, 2) Han Solo and Gollum make me happy, and we all know that being happy releases stress, which reduces inflammation, which is good for people who have too much of it in their eyes.

IVIG treatments, which I started just last month, also seem to be good for the eyes. It became quite clear that my previous combination of treatments was not working after a complete regression occurred after my prednisone was stopped, and so I decided to try “intravenous immunoglobulin G,” which is not a drug in the usual sense really, but actually a type of antibody. The antibody is collected from healthy donors (blood donors), and, well, no one is really sure exactly why it works for some autoimmune diseases. I'll discuss the possible mechanisms in one of my next posts. I've only had one cycle of treatments so far but almost immediately after, my eyes started to improve. I'm hopeful that this means that this is the medicine that will finally tip the balance in my favor. The only real side effect I've noticed so far are some pretty nasty, nausea-inducing headaches – unpleasant yes, but in my opinion, much more favorable than uncontrollable ocular inflammation. So we'll see how things go! I'm writing to you from the infusion room as I receive my second round of IVIG so hopefully next week I'll have some progress to report. 

Until then, I'm mighty thankful for the magic of Advil.