The Bright Places, Where Boom Bands Are Playing

Dear readers, I come to you with some fairly exciting news. The Girl Who Couldn't Cry is going to be a doctor. Yes, really! With an MD after her name and everything!

Over the past few months I've: 

1. Taken the MCAT
   
2. Cried all my eye drops out for joy when I got my score back
   
3. Been applying to, and subsequently, interviewing at, various medical schools

Apologies for my time away from you all; medical school applications ended up eating a tremendous amount of my free time. To put this in perspective, consider the fact that I've written about 40 pages in essays and short answers for said applications. It was utter insanity for awhile there and boy, am I glad it's over... for the most part at least. I still have a few more interviews to attend and a few more essays to write, but at least it feels nice to know that I've gotten in somewhere and now it's just a matter of where I go to medical school, not if I go.

I have a variety of hilarious stories to tell you all, pertaining to worries about eyelashes falling off during interviews and how I managed to apply eye drops during day-long school visits and walking tours without looking like a crazy person... But I'm going to have to wait to tell you all about that until after my interviews are completed. I don't want to give all my secrets away and then have some member of an admissions committee read about them on here!

But enough about medical school. Let's get back to the endlessly entertaining world of cicatricial pemphigoid. I'm proud to report that my eyes have been fantastic lately. Really fantastic. For instance, I'm now working as a science/English/MCAT tutor, and I can actually last through an entire 2 hour session without needing to use a single eye drop. It's so wonderful. And I know I sound a bit crazy for being excited to go 2 hours without eye drops but hey, that's like a 2400% improvement for me. I'll take what I can get people. Additionally, my eyes look fairly white most of the time and with all 4 punctal plugs in, they feel great. Unfortunately, I've been having trouble with one plug in particular, which first started rubbing my eye, and then fell out - multiple times. The first time it fell out was right before my first medical school interview and I was in a complete panic. As soon as it was gone, I was back to using drops in that eye on order of minutes. My poor boyfriend had to deal with the brunt of the fallout. Every few hours I would turn to him and wail, "I'm going to have to put drops in during my interviews! And then my interviewers will think I'm weird/ rude/ unprofessional!"  

This marked the beginning of The Great Punctal Plug Fiasco of 2012. First, I tried to have the plug replaced by my local eye doctor, but all the plugs she had were too small and so the one she placed fell out only days later. Undaunted, I went all the way to Boston, where they had the ability to place an intracanalicular plug, which goes all the way inside the tear duct and therefore, can't rub your eye or fall out. Supposedly. Unfortunately, this plug was misplaced from the get-go. As I was leaving Boston, I actually called the office to ask if I should return before leaving town because my eye still didn't feel right. I was told to give it a few days because what I was experiencing was "normal."  

I gave it a few days. It was not normal.

The plug didn't seem to be doing much of anything at all and I could still feel all my eye drops just falling right down into my nose. So essentially that whole trip was just a giant waste. Cue more panicking. 

At this point, I was able to get an appointment with a new doctor in DC. He was able to fit the correct size plug, but scared me a little when he said he was going to "push it down" to make sure it didn't scratch my eye. I knew that the plug he was using wasn't meant to be shoved too far down into the duct though, and I was now extremely paranoid about it falling out again. Luckily, it did not. At least not until a few weeks ago. So this Friday I have yet another appointment to replace the accursed thing.  

And so the Great Punctal Plug Fiasco of 2013 has now begun. 

In happier news, I recently began using something known as “autologous plasma eye drops.” These are preservative-free drops made from the plasma (or serum) component of one's own blood. This treatment is believed to be beneficial for people with severe dry eye and/or ocular surface damage due to trauma, or diseases such as Stevens-Johnson syndrome, cicatricial pemphigoid, etc. The idea behind the treatment is that those with severe dry eye are lacking essential tear components, since their bodies no longer produce tears normally. Generally, these patients are advised to use artificial tears, which are great for providing wetness, but do absolutely nothing to help replace the missing growth factors, proteins, and vitamins (such as vitamin A), that are normally delivered to the eye in healthy tears. This is where the serum/plasma drops come in. The drops are made by spinning blood down to remove the cells. What's left behind is called either serum or plasma. (It's called plasma if the clotting factors are still present, and serum if the clotting factors have been removed.) This portion of your blood is then separated into small vials or tubes and given to you, the patient, to take home and stick in your eye. Since these drops have been made from you, and not some eye drop factory somewhere, they contain some of the good stuff that your eye may have been missing. Presumably, returning these naturally present tear components to your eye is beneficial, and may help to improve existing ocular surface damage. Pretty awesome right?

I've actually only been using these drops for about a month, so I plan to wait a bit longer to write up a real review of my experience with them for you all, but I did want to make a quick mention of this treatment since it's rather unusual in approach. So far, I don't think I've noticed a tremendous difference but I have noticed that my eyes are tending to be less pink if I stay up late. Whether this is just a fluke, or is actually due to the plasma drops... well, I guess time will tell.

So until next time my friends. May you all have white eyes, copious tear production, and unobstructed meibomian glands! And all your punctal plugs in the right place...

This post is dedicated to the wise words of one, Dr. Seuss, who took the time to remind me, in catchy verse, to never ever ever give up on my dreams. Even when life throws horrifyingly named Prickle-ly Perches, Slumps and Cicatricial Pemphigoids at you: 

Congratulations!
Today is your day.
You're off to Great Places!
You're off and away!

You have brains in your head.
You have feet in your shoes
You can steer yourself
any direction you choose.
You're on your own.  And you know what you know.
And YOU are the guy who'll decide where to go.

You'll look up and down streets.  Look 'em over with care.
About some you will say, "I don't choose to go there."
With your head full of brains and your shoes full of feet,
you're too smart to go down any not-so-good street.

And you may not find any
you'll want to go down.
In that case, of course,
you'll head straight out of town.

It's opener there
in the wide open air.

Out there things can happen
and frequently do
to people as brainy
and footsy as you.

And when things start to happen,
don't worry.  Don't stew.
Just go right along.
You'll start happening too.

OH!
THE PLACES YOU'LL GO!

You'll be on your way up!
You'll be seeing great sights!
You'll join the high fliers
who soar to high heights.

You won't lag behind, because you'll have the speed.
You'll pass the whole gang and you'll soon take the lead.
Wherever you fly, you'll be the best of the best.
Wherever you go, you will top all the rest.

Except when you don't
Because, sometimes, you won't.

I'm sorry to say so
but, sadly, it's true
and Hang-ups
can happen to you.

You can get all hung up
in a prickle-ly perch.
And your gang will fly on.
You'll be left in a Lurch.

You'll come down from the Lurch
with an unpleasant bump.
And the chances are, then,
that you'll be in a Slump.

And when you're in a Slump,
you're not in for much fun.
Un-slumping yourself
is not easily done.

You will come to a place where the streets are not marked.
Some windows are lighted.  But mostly they're darked.
A place you could sprain both your elbow and chin!
Do you dare to stay out?  Do you dare to go in?
How much can you lose? How much can you win?

And IF you go in, should you turn left or right...
or right-and-three-quarters? Or, maybe, not quite?
Or go around back and sneak in from behind?
Simple it's not, I'm afraid you will find,
for a mind-maker-upper to make up his mind.

You can get so confused
that you'll start in to race
down long wiggled roads at a break-necking pace
and grind on for miles across weirdish wild space,
headed, I fear, toward a most useless place.
The Waiting Place...

...for people just waiting.
Waiting for a train to go
or a bus to come, or a plane to go
or the mail to come, or the rain to go
or the phone to ring, or the snow to snow
or waiting around for a Yes or a No
or waiting for their hair to grow.
Everyone is just waiting.

Waiting for the fish to bite
or waiting for wind to fly a kite
or waiting around for Friday night
or waiting, perhaps, for their Uncle Jake
or a pot to boil, or a Better Break
or a string of pearls, or a pair of pants
or a wig with curls, or Another Chance.
Everyone is just waiting.

NO!
That's not for you!

Somehow you'll escape
all that waiting and staying.
You'll find the bright places
where Boom Bands are playing.

With banner flip-flapping,
once more you'll ride high!
Ready for anything under the sky.
Ready because you're that kind of a guy!

Oh, the places you'll go! There is fun to be done!
There are points to be scored.  there are games to be won.
And the magical things you can do with that ball
will make you the winning-est winner of all.
Fame!  You'll be famous as famous can be,
with the whole wide world watching you win on TV.

Except when they don't.
Because, sometimes, they won't.

I'm afraid that some times
you'll play lonely games too.
Games you can't win
'cause you'll play against you.

All Alone!
Whether you like it or not,
Alone will be something
you'll be quite a lot.

And when you're alone, there's a very good chance
you'll meet things that scare you right out of your pants.
There are some, down the road between hither and yon,
that can scare you so much you won't want to go on.

But on you will go
though the weather be foul
On you will go
though your enemies prowl
On you will go
though the Hakken-Kraks howl
Onward up many
a frightening creek,
though your arms may get sore
and your sneakers may leak.

On and on you will hike
and I know you'll hike far
and face up to your problems
whatever they are.

You'll get mixed up, of course,
as you already know.
You'll get mixed up
with many strange birds as you go.
So be sure when you step.
Step with care and great tact
and remember that Life's
a Great Balancing Act.
Just never forget to be dexterous and deft.
And never mix up your right foot with your left.

And will you succeed?
Yes! You will, indeed!
(98 and 3/4 percent guaranteed.)

KID, YOU'LL MOVE MOUNTAINS!

So...
be your name Buxbaum or Bixby or Bray
or Mordecai Ali Van Allen O'Shea,
you're off to Great Places!
Today is your day!
Your mountain is waiting.
So...get on your way!  
 

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.

How much is that cow in the window?

My eyes have been worse lately. So today I'm going to follow the time honored tradition of sticking my head in the sand... and focus on something else. As I am best distracted when outraged, I've decided to tell you all about this article I read the other day, regarding the impending expiration of Lipitor's patent: http://today.msnbc.msn.com/id/45182184/ns/business-us_business/#.TrrGKXLZeuI. I felt a little bit like vomiting after reading it, so I suggest you sample the article for yourself with a mental barf-bag prepared. I didn't really know much about Lipitor prior to this, besides the fact that it's a cholesterol drug, but apparently Lipitor was a huge money-maker for it's developer (Pfizer). What's disturbing is the fact that this entire article reads kind of like a eulogy for some sort of mythological creature - which I guess in this case is a cash cow.

Rather than commenting on how “unleashing a wave of generics,” as they put it, might actually be a really good thing for all the people out there who have to pay for this medicine, the article only laments about the end of Lipitor's “fabled run,” and eagerly posits which new drugs could make as much money. Could it be Humira or Remicade? Drugs that are commonly used to treat rheumatoid arthritis? Or maybe it could be... an Alzheimer's drug! So many people are going to get Alzheimer's as the baby boomers age!

Does anyone else see anything wrong with this picture?

It disturbs me enough that pharmaceutical companies view patient populations as just money-making potential, waiting to be tapped. But to see journalists, who make no money off of drug sales, doing it as well is just downright frightening. Has viewing the medical/pharmaceutical industry as a profit machine become so pervasive in our society that no one will blink twice at considering a diabetes drug for how much money it will make rather than for how many lives it will improve or save? I guess the answer is yes. Obviously the authors of this article would be considered “business journalists” but it's a little bit disturbing if you can just throw the word “business” into your work title and it becomes immediately justifiable for you not to care about other human beings.

As far as Pfizer is concerned, now that their patent is up, they have apparently cut a deal with a generics manufacturer to withhold the sales of the generic version of Lipitor in the US. Just so they can squeeze every last penny out of US patients. You can read about this here: http://www.bloomberg.com/news/2011-11-08/pfizer-ranbaxy-sued-by-pharmacies-alleging-lipitor-price-fixing.html. I feel like the reason why pharmaceuticals feel like they can get away with tricks like this is precisely because the attitude that they deserve to be making billions of dollars off of sick people is just accepted as the way things are. But it is not the way things should be.

So today's blog post is dedicated to your health. May you never need the next "blockbuster" drug that these pharmaceuticals are developing, for they will certainly ask you to pay dearly for it. And then you'll have the pleasure of reading articles, such as the first one, celebrating the huge profits made off of your suffering and hard-earned money.

Ninth Circle, Antenora

I'm a little bit in love with Dr. Foster right now. I just had an excellent visit with him in Boston yesterday and I am so excited to be making progress! I hadn't seen Dr. Foster since starting my infusions and steroids so I really was anxious to hear what he thought about how I was doing.

Last time I was in for a visit, Dr. Foster was the picture of frustration. I was not responding to treatment and in fact, my eyes had gotten drastically worse. We tried giving me an emergency IV of steroids (to quickly calm down the inflammation) but it ended up not doing anything. It's pretty scary seeing your doctor bury his face in his hands after looking at you. You can't help but think that you must be the worst case he's ever seen or something. It didn't help that giant pictures of my tomato-red and generally horrible looking eyeballs were floating around on the huge computer monitor over Dr. Foster's shoulder as he did this. A reminder of how completely abnormal and well, diseased, my eyes looked. I promptly burst into tears.

The funny thing is, I knew exactly what Dr. Foster was going to say. I knew I was worse. I knew I would have to start taking prednisone daily, along with probably either methotrexate or cyclophosphamide (different immunosuppressants, since my current one wasn't working). I knew my eyes were very very inflamed. I guess it was just kind of unsettling to see my doctor look so upset about my particular case. I already had a rare disease, I didn't want to be further sorted into the “tough case” subset and unfortunately, that's exactly where I was.

But yesterday, Dr. Foster could not have looked more different. He was positively gleeful after looking me over. He declared that I was doing much better and responding so well after only a few months of treatment that even he was excited. I wanted to hug him, jump around the room, and possibly climb up on the exam chair and do a little dance in celebration. Luckily, I remembered social norms and settled for just smiling 'till my face hurt. Tough case to success story? I liked the sound of that. I flew out of Boston in a state of joy-induced delirium.

Science aside: Ever since this appointment, I have been having little fantasy-visions of my B cells getting chased down and destroyed while I stand by watching happily. One of the immunosuppressant medications I take is called rituximab. Rituximab goes after a protein called CD-20 and it is basically supposed to attack my B cells. B cells are cells of the immune system that are involved in making antibodies, among other things. These cells are supposed to be destroyed, fixed, or otherwise incapacitated if they act abnormal towards your own body. Since I am starting to get better with the addition of rituximab, this seems to suggest that something went wrong with this process and I have abnormal B cells running around, wreaking havoc. Of course, the immune system is really complicated and I am not a doctor and there is more to the story of my disease than just renegade B cells (you can read more about the “double hit hypothesis” here: http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v5/v5c027.html). But for now, I'm going to imagine that hunting the little traitors down is the key to making me better and continue rooting for their destruction. Had they had souls, I'm sure Dante would have placed them in the second ring of the ninth circle of the Inferno - reserved for traitors to country. (My body = homeland for these guys therefore, to Antenora they go.)

But back to reality. Other good news from my Boston visit includes being given the green light to continue with my steroid taper. I've been dropping 5 mg each week and so I will officially be prednisone-free by the first week of December! 

Oh pemphigoid, I told you I'd kick your butt. Just you wait.

This post is dedicated to Dante Aligheri. Without whom I would not have known where exactly in the Inferno to place my misbehaving B cells so that divine justice could be properly enacted. I like to call this method of cheering myself up "Dante-vengeance."

Comic Relief

So the other day I realized that one of my medications was kind of humorously named. This is a picture of the prescription in question.

Note the name in bold: FML. For those of you unaware of the meaning of the abbreviation “FML,” it is commonly used to stand for “F--- my life.” In fact, there is an entire website dedicated to this usage (www.fmylife.com). Anyway, I just think it's kind of hilarious that I've been instructed to apply FML to both eyes, three times daily... It makes me smile whenever I use it.

Despite my use of pessimistically-named medications, life has actually been pretty good as of late. I just finished my last weekly chemotherapy treatment and according to my current protocol, I will only be receiving treatments once a month from now on! This is especially good news for logistical reasons; I am literally running out of veins to be used for infusions. We've used veins in my hands, my forearms, the crook of my elbow, my wrists... I need some time to heal up! Especially since the steroids I take diminish my wound-healing capabilities and cause me to bruise easily. As a result, all my needle sticks have left me with a patchwork of bruises on my arms. I look like someone let a five-year old tattoo me with splotches of blue, purple, and red. I personally think this is kind of funny, and I proudly show off my bruises as badges of honor. My boyfriend, however, thinks this is less funny, as I suppose it could also look like he beats me with a stick on the arms or something. In any case, it will be nice to give my arms some time to recover. It was getting difficult for the nurses to see behind all the black and blue.

Besides making me extra-fragile, I believe prednisone is also giving me food-related nightmares. Not the “oh my God a giant cheeseburger is chasing me” kind, but the “I just ate a carton of ice cream and now I have steroid-induced diabetes” kind. Maybe I should dress up as an overworked insulin molecule for Halloween! Get it? The insulin is “stressed” because it can't keep up with skyrocketing blood glucose levels? Well I certainly think it's frightening.

This post is dedicated to nerds. Viva la science!

You come here often?

Yesterday I had my fifth infusion. This means I only have one more weekly infusion to go before I can switch to a monthly schedule. Yay! This may sound odd, but I had a really good time during my treatment yesterday... which I suppose is saying something because during treatments you're basically just sitting still for six hours while they pump a poison into you.

I do have to admit that I've never really had a bad time during my infusions, and I credit the awesome nurses in the infusion room for this. They are hilarious, always upbeat, and always ready to help you with anything you need (water, a blanket, gossip). I don't know if any of you have ever had the chance to see a nurse in action, but it's kind of amazing. Sometimes I feel like I'm taking part in a wildlife study as I watch them zip around, like frantic birds in their natural habitat. My nurses can multitask like pros – prepping you for a blood draw while taking your vitals and somehow managing to carry on a conversation with you about politics through the whole thing. To me, a girl who can't even talk and type at the same time, this is beyond impressive. It also makes me feel quite lazy to be lounging around in a comfy chair while they do all this running around... but as I'm connected to an IV pole, I suppose it's not like I could keep up even if I tried.

But I've gotten sidetracked, because the point was that as fun as the nurses make the experience, sometimes your fellow patients are even more entertaining. Case in point, a sixty-something southern-born-and-bred retiree who plopped herself down in the infusion chair next to mine. I am going to call this patient Mrs. Bree. Yesterday, Mrs. Bree and I had quite the conversation. She likes to talk and I especially liked to listen because I was so in love with her Georgia drawl. Here is what I learned over the course of the day. Mrs. Bree loves clogs, her twenty year-old cat, her soon-to-be-retired navy officer husband, and Hugh Jackman. I think she loves Hugh Jackman most of all. I honestly have never seen anyone go so crazy over a Hollywood star before. Mr. Jackman happened to be a guest on Regis and Kelly that day, which as usual, was playing in the infusion room. As soon as they announced the guest, Mrs. Bree clapped both hands over her mouth, started kicking her feet up and down like an excited five year-old, and I think began to hyperventilate a little bit. She then proceeded to ask me and the man seated on my other side if we didn't think Jackman was just the kindest, sexiest, most talented man alive and then launched into a detailed account of why she thought he was so amazing. The poor guy next to me (and incidentally the only male in the room) looked bored at first, and then looked increasingly annoyed as the nurses were drawn into a discussion on the finer points of Hugh Jackman's facial hair.

Now I will admit, Hugh is a hot guy. But most people in Hollywood are attractive so I'm really not that impressed by you just being good looking if you're an actor. I do like him better than most I suppose, because he seems well-spoken, kind, and he has a really good voice (if you can sing you are instantly more attractive according to my logic). Still, I was unprepared for Mrs. Bree's reaction during his interview. She shushed us all urgently whenever Jackman was on screen. She wildly flailed every single one of her limbs in an attempt to shoo nurses from blocking her view of the TV. And she thought nothing of basically shoving aside the poor nurse who was, at that precise moment, attempting to take her IV out.

As crazy as I found Mrs. Bree's behavior, it was also kind of adorable. I'm not sure exactly what illness she has, but I do know it's a chronic and life-threatening autoimmune disease for which she will receive treatments for the rest of her life. It's nice to see that despite all her years of treatments and despite all the years ahead, she's still more gregarious and energetic than a teenage girl at a Robert Pattinson movie.

I wanted to mention this little experience because I feel like you get used to hearing only about how horrible chemotherapy is. But really, it's nice to sit and talk with the other patients in the room during treatments. As one other patient said later on in the day, “It's like we're all at a bar! We've each got our different cocktails – hey can I buy you a drink?” I found this to be a stunningly good comparison actually. Just like at a bar, you never know who you might run into or where the conversation might take you. In my case, I now know everything I could ever possibly want to know about clogs.

This post is dedicated to Hugh Jackman.

The Waiting Place

Today I have decided to rant about my eyes. They have been rebelling since last week and what I mean by this is that I kind of look like someone who's just had grapefruit juice thrown at their face. Ouch right? Well this is what it feels like too. Almost ALL THE TIME. I'm not sure if this is a sign that my treatment is not working, or maybe if it's just not working yet. I will have to ask my doctor about this at my next appointment and I guess switch to a different medicine if I need to.

Normally, I try to be positive about this and think, “well, at least I'm not dead, at least I'm not blind, at least I have all my limbs, etc.” But today I don't feel like being positive because I'm just downright annoyed. I miss having normal eyes that can see properly. I miss being able to read and type without having to strain and squint and look super awkward as I shove my face right on top of whatever I am trying to work on. But most of all, I miss being able to keep my eyes open. Honestly, no one ever thinks about how amazing it is that your eyes can self-lubricate so that you can walk around with the really delicate membrane exposed to all the world... but trust me, it's a miracle. My eyes are exceptionally dry at the moment, and though I try to solve this by using eye drops about every ten minutes, my eyes are also so inflamed that anything I add to them in an attempt to alleviate the dryness also irritates the crap out of them. It's pretty much a lose-lose situation.

As unpleasant as it is to be in pain, I think the real reason behind my foul mood is that I sometimes get tired of making plans and having them ruined because I am sick. For instance, my health problems have kept me delaying my plans for med school since, well 2007 really. It's 2011 now. That's three years I've lost. I guess it's not good to think about how behind on your life plans you're getting but sometimes I just can't help it. Normally, I try to cheer myself up by thinking “well, it's not your fault that you got sick,” but today, that doesn't really make me feel any better. I hate feeling like a bum and even more, I hate thinking that other people might look at me and think I'm just a lazy person that might just not have what it takes to be a doctor. 

I don't know if any of you have ever read “Oh the Places You'll Go!” by Dr. Seuss, but in the book, he describes a place called “The Waiting Place.” You can read the text of the book here (it's short, and I recommend it): http://www.teamhope.com/seuss.htm, but here is an excerpt:

The Waiting Place... for people just waiting.

Waiting for a train to go or a bus to come, or a plane to go or the mail to come, or the rain to go or the phone to ring, or the snow to snow or waiting around for a Yes or No or waiting for their hair to grow. Everyone is just waiting.

Waiting for the fish to bite or waiting for wind to fly a kite or waiting around for Friday night or waiting, perhaps, for their Uncle Jake or a pot to boil, or a Better Break or a string of pearls, or a pair of pants or a wig with curls, or Another Chance. Everyone is just waiting. (Seuss, 1990).

I feel like I'm in the waiting place. Dr. Seuss forgot to include a line about the people who are waiting for their immune systems to stop attacking them so they can go back to driving and working and school... but it's ok Dr. Seuss. I still like you.

I hope that like Dr. Seuss says, “somehow [I'll] escape all that waiting and staying.” Here's to tomorrow and not giving up!

This post is dedicated to the magic of hair salons. I decided to get a long over-due haircut (2 years since my last one!) after writing this post and felt instantly better about everything. Do they infuse happiness into their fancy shampoos? My eyes might be hurting, but at least my hair looks pretty!

Germ Warfare

This week I got a cold. This is bad news for an immunosuppressed person since it's not only easier for you to get sick, but also harder for you to fight off. So on Monday, as the first signs of a sore throat were kicking in, I attempted to smother any growing cold bugs with copious amounts of hot tea, zinc gluconate (Cold- Eeze), and sleep. Unfortunately, none of my little home remedies worked and I woke up the next morning with a full blown sore throat. There was a slight fracas as I had to call and tell the infusion center I was sick, and they then had to verify that I could continue my treatments with my doctor. But in the end, it was decided that it was fine to continue on with my regularly scheduled protocol. Phew! I really didn't want to have to stop anything just because of a stupid sore throat.

As a result of all this, getting sick felt like a personal failure. At my first chemo treatment I was handed a packet on precautions to take in order to prevent getting sick - which I thought I followed to the letter. I got a flu shot, I washed my hands constantly, I avoided getting too close to other people, I sanitized things with a little packet of disinfecting wipes... I resolved to do better at keeping up my germ fighting techniques since apparently some had broken through my defenses.

With this in mind, I strolled into Whole Foods that afternoon and as usual, grabbed a disinfectant wipe from next to the cart area and began to wipe off the handle. I'm not sure if I looked extra intense about my cleaning or something but for whatever reason, a germ terrorist decided to target me that day. Just as I was turning to throw away my wipe and walk on to do my shopping some random dude slapped his hand down on the freshly wiped handle and smirked. I stood there confused for a moment. Surely that guy didn't just do that on purpose? Maybe he thought my cart was his cart? I eventually regained my composure and asked "Did you just touch my cart after I cleaned it?". He nodded and continued to smirk as he walked away.

I should remind you all that I am on daily prednisone at the moment, one of the side effects of which is mood swings. I've never had any sort of steroid-induced rage experience thus far but I think what followed may have been my first one. I'm normally not an aggressive person but I wanted to punch this guy in the face very badly. Instead I decided to try for public embarrassment by walking after him and loudly explaining that he was a total jerk for doing something like that to an immunosuppressed patient on chemotherapy who has to be mindful of germs for serious health reasons. At least a few people in our vicinity turned to stare at him and I felt vindicated even though the dirt-bag didn't look the least bit sorry. Meanwhile my non-confrontational little brother had quietly slipped away with our cart and was trying to pretend he didn't know me. I guess he thought I was going to do something worse than just yell at the guy.

Some people are so rude. Keep your germs to yourself people!

This post is dedicated to my little brother. Sorry for being an embarrassing sister!

Can't I have a disease with a pretty name?

Vainly, I have wished for a more "elegant" sounding disorder but alas, I am stuck with the clunky syllables of cicatricial pemphigoid. Read: sick-a-trish-ul pempf-eh-goid. I suppose that the complex name does lend me an air of mystery though. Often people look at me with a mixture of confusion and awe (as if I've suddenly switched to speaking in Chinese) when I tell them what I have. Now that you know how to pronounce it, you can experience this too!

As I mentioned in my first post, what I have is pretty rare. It affects all races equally but like many autoimmune disorders, it is more common in women. The age of onset is generally older (about 50-60) but I seem to have gotten lucky because we think my first symptoms began to appear at about 15, although it wasn't until college that the symptoms worsened enough to become a real problem. This disorder is also known as mucous membrane pemphigoid, which means it affects... you guessed it! Mucous membranes. Essentially, your immune system turns against you and can attack these membranes in your eyes, mouth, larynx, pharynx, esophagus, etc. I consider myself fortunate in a way because I could be so much worse off if I had more areas of involvement. Currently, I seem to have only or mostly ocular involvement, and so for the purposes of this blog, that is what I am going to focus on.

The ocular manifestations of cicatricial pemphigoid can lead to blindness if not treated. This begins with chronic conjunctivitis, scarring of the conjunctiva, eyelid positioning issues, dryness, and vision loss. In case you don't know what the conjunctiva is, it's the membrane that covers your eyeball and inner eyelids. You need it, and your lacrimal glands (which make the watery part of your tears) to be healthy in order to properly lubricate and protect your eye, and allow you to see. I have all of the ocular symptoms but I personally feel that the dryness/vision loss is by far the worst. Dry eyes can be extremely painful, and not being able to see well is not only annoying, but also really debilitating. My dry eye/vision loss is pretty severe right now because I currently have a great deal of inflammation. But I hope that with treatment, I can get the inflammation under control, get my sight back to normal, and get my tear production going again! At the moment, my eyes are so dry that I can't cry anymore. It's actually kind of amusing to see what happens when I try. My eyes just turn bright red (or at least a brighter red then their currently constant medium red state) and get really sore with the apparently tremendous effort of attempting to squeeze out wetness from their parched depths. As a result, I have added crying to the list of things that I never expected to miss.

Treatment for cicatricial pemphigoid involves suppressing your immune system with chemotherapy, possibly in combination with steroids (used to control inflammation quickly). This is why the sooner it is caught, the better. The faster you get your immune system under control, the less overall damage is done and the fewer symptoms/problems you will have. Unfortunately, the disease is so rare and getting a positive biopsy is so tricky, that it is often quite difficult to diagnose. I have had firsthand experience with this. I had the ocular symptoms of the disorder for years but had five negative biopsies, and so I was given the diagnosis of chronic cicatrizing conjuctivitis (which is a fancy way to say your eyes are messed up and we don't know why). I sat like that for a long while - being monitored, but getting worse. I suspected that I had cicatricial pemphigoid based on my symptoms. It is well known that the biopsies have a high false negative rate and so at every appointment I tried to push to get started on some treatment plan, fearing that the longer I waited the worse off I would be. I was of the opinion that my symptoms were so indicative of pemphigoid that it would be far worse to let the disease progress than to start treatment, especially since I didn't trust my negative biopsies one bit. Unfortunately, my doctor was reluctant to proceed at that point and so eventually, my symptoms did worsen. As a patient, sometimes the way you wish to attack your illness will not match up with the way your doctor treats and thinks. These things happen and when they do, I suggest going for a second opinion.

This is exactly what I did when my eyes suddenly got worse and my doctor was still unable to give me a diagnosis or new treatment plan (other than eye drops). I went immediately for a second opinion with another ocular immunologist, Dr. Stephen Foster. Dr. Foster did a sixth biopsy, using a different, more sophisticated analytical technique and lo and behold, I was diagnosed! I tell you all this story because if you are reading this out there and you have started to have symptoms of dry eye and conjuctival scarring of unknown origin, GO SEE DR. FOSTER. Go see him right now. He is the guy to go to for the ocular manifestations of disorder and many other ocular diseases and I appreciate that he will start treating patients even without a positive biopsy of pemphigoid if he feels their symptoms match up.

If I could be granted one wish, it would be to have been diagnosed earlier. I wish I had been referred to Dr. Foster, or known to ask for a repeat biopsy analyzed using an immmunoperoxidase technique sooner. It's slightly upsetting to me to acknowledge this because for about five years, it was the one regret I knew I didn't want to have. Often I have to tell myself that I really did everything I could at the time. I thought I was seeing basically the only expert for the disease and so when I doubted the handling of my case, I didn't know better than to think "well, I'm not the specialist." I know for a fact that if I hadn't worked as hard as I did to get diagnosed and treated, I would be far worse off and never would have found Dr. Foster on my own. I do take some small comfort in that. I shudder to think of what would have happened to me had I not already had an interest in medicine (I want to be a doctor) because my fascination made it second nature for me to read all the papers and studies I could about my symptoms. As a result, I was a fairly well-informed patient and this helped me to be really proactive about searching for a diagnosis and having the surgeries that I needed to along the way. Knowing how seriously debilitating my symptoms could become was also the motivation for me to immediately seek out the best care that I could. Despite my deterioration under the care of the first doctor I saw, both ocular immunologists who looked at my case are regarded as the foremost experts in this disease, both connected with very well-known and reputable research institutions.

Though my efforts weren't able to save me from getting to stage 3 of the ocular disease (out of 4 - untreatable), I hope that writing about all of this might help someone else to get diagnosed earlier. Please, if you think you may have the symptoms of this disease, feel free to contact me with questions. I am not a medical expert, but I can help point you in the right direction for care. In the meantime, Ophthalmalogic Manifiestations of Cicatricial Pemphigoid is a great first online resource.

As horrible as it is to be sick, I like to imagine that maybe the reason for all of this was to make me into a better or more motivated physician one day. Who knows? Maybe I'll go into ophthalmology. Maybe I'll help cure dry eye! Wouldn't that be a cute story.

This post is dedicated to medicine and to my good fortune. If I'd been born without access to good medical care and research, I have no doubt I would be blind. And then where would this blog be!? I can only blow up the font size on my computer so much...