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...

Girl With One Eye

So here we go with my very first blog post! I'm not really sure where to start this off so I guess I'll just describe the motivation behind this and all future ramblings. I am 24 and was recently diagnosed with an autoimmune disease - cicatricial pemphigoid (also called mucous membrane pemphigoid). Being sick is scary, but sometimes it's just hilarious. I hope to document my experiences with this disorder so that other people who are suffering from the same disease have a chance of stumbling across my writings and will maybe not feel quite so alone, quite so confused, and hopefully end up a little cheerier. Additionally, the sooner this condition is diagnosed and treated, the better off you are. It's pretty rare, but I know others out there will get this in the future, or maybe already are in the beginning stages. I hope that my little blog will play some small part in helping these people to be diagnosed as soon as possible.

Of course, if you end up reading for other reasons, I welcome you as well! I hope that I provide some amusing insights into what life with an illness is like and remind you all not to sweat the small stuff.

That's all for now. Soon to come, a short description of cicatrical pemphigoid and a recap of how I got to where I am today.

Today's blog was brought to you by Jo's right eyeball! Picking up the slack of the currently useless left one, which is being squeezed shut as I type. Thank you evolution, for giving me two eyes.