Tuesday, June 28, 2011

Something To Believe In

     One of the main annoyances with Marfan's Syndrome is that it's such a reactive disorder, rather than a proactive one. For someone like me, who wants to do something about it when her husband is in pain, it's beyond frustrating. I can't even get angry with the doctors who treat him, because truthfully? There's not that much they can do anyway. It's such a wait-and-see-how-bad -it-gets kind of thing, so unique to each individual. Nobody, not even the experts, know exactly what's going to happen to my husband's body, nor at what rate. All anyone can do it wait for things to break and deal with it when they do.

     Yesterday was my husband's first trip to the pain management doctor in about three years, so I wasn't quite sure what, if any, new knowledge or treatment options I was expecting him to come home with. I'm happy to report that he was given not one, not two, but three things to try to more effectively manage his daily pain. Well, to be more accurate, I should say that he was given two methods of preventing/managing his back pain and one method of curbing it when it gets really bad. Semantics aside, I'm just really happy that we're maybe getting somewhere with his treatment.

    He went into the appointment intending to tell the doctor that he wanted to try something other than drugs, because they just weren't all that effective. That's exactly what he did, though he had to add the disclaimer that he's going to be embarking on a long car trip in October (yes, we settled the great debate and decided to drive to North Carolina, barring a medical incident that necessitates flying) and will likely need some sort of painkiller to make the plan a feasible one. From what my husband told me of his visit, it seemed like the doctor was very receptive to his request for alternate methods of pain control.

      The first thing he pulls out of his bag to show me is a prescription for a steroid, something in the same family as prednisone, which belongs to the corticosteroid group. I was kind of surprised when I saw it, because it stuck me as odd that he'd be given a steroid to use as a painkiller. Granted, it's only to be used once or twice a year, not at all like his prescription for Flexoril, which he uses semi-regularly to keep his back muscles from completely locking up.

     I guess it makes sense, given that the doctor wrote the prescription as a super-dose, to only be used for the road trip. It's also true that the issue isn't necessarily his back muscles in this case - it's the spine itself. He's afraid the dural ectasia will start acting up and from what I understand, the steroids will help alleviate this. (Further research into this one will ensue, don't you worry.)

     Okay, fine, more drugs, no real surprises there. But then....he pulled out a prescription for a chiropractor. Um, excuse the hell out of me, but since I've been with my husband, "chiropractor" has become sort of a dirty word in my vocabulary. I know, that's probably unfair to the profession as a whole and I really don't intend it to be that way. It's just that, when you're dealing with someone who has Marfan's and the tissues that keep their bony bits connected and in place are weakened, the very last thing you want is someone popping and aligning and pulling on things. So it's never really been a treatment option that's been under consideration by us.

     The doctor seemed to understand my husband's apprehension, though, and assured him that she was sending him to a chiropractor that she worked with quite often and who was aware of Marfan's Syndrome and its effects on the body. In fact, the prescription actually makes note of the fact that my husband's spine is not to be messed with. I'm thinking it's going to be a treatment based more on loosening and maintaining the muscles in the back, rather than alignment of vertebrae and the like.

     If that's indeed what's going to happen, then I'm all for it. My husband pointed out that it's not always muscle pain that keeps him in bed; sometimes it's skeletal. My argument was that while I understand that, a good sixty percent of the pain is muscular and if we can alleviate even a fraction of that, then it's a small victory won. When it comes to my husband's health, I'll take any victory, small or large; I'm not picky.

     Finally, he pulled one last thing out of his bag - a brand-new back brace. As soon as I saw it, I had to start laughing. I tried to stop, but it only got worse when my husband then proceeded to put the damn thing on over his Black Rebel Motorcycle Club t-shirt and stare at me balefully. His clear displeasure ("I'm not that old yet!") at having been told to wear it whenever he was doing any sort of potentially strenuous physical activity was, in an admittedly twisted way, amusing. I'm pleased about it because it's a step in the preventative direction and you know how I love to be proactive. Plus, I assured him that to me, a back brace = sexy sexy, so he doesn't have to be so bummed about it.

     Oh, alright. Truthfully? The back brace itself does not = sexy sexy, but I find my husband to be awfully damn cute regardless of his attire, so that still counts, right? Pretty sure it does.

Monday, June 27, 2011

We Are Family

     I love my family, a lot. In so many ways, I really feel like I was blessed with the ideal parents and sister. It is solely because of the unwavering love, support and understanding that I received with regards to everything I did and did not do that I was able to grow into the person I am now. I also believe that my family is one more piece of evidence to my fatalistic argument that my husband is the one person that I was fated to be with. What's my family got to do with my marriage, you ask? Well, when dealing with something like Marfan's Syndrome, you need a pretty damn good support system in place to help with everything. My family is a cornerstone of my support system and it's hard to imagine a better one. So you can imagine that when a piece of that system fails me, it's difficult to stomach.

     Last weekend, my sister and I were at a family gathering to celebrate the recent graduation from college of two of my cousins. We were sitting around, talking with my mother and an aunt whom we hadn't seen in awhile. I don't even know how the topic came up, but all of a sudden, we'd moved from discussing the awesomeness of Game of Thrones to her boyfriend's medical issues. Brendon has ulcerative colitis, which is a form of inflammatory bowel disease.(If you don't know what that is, clicky-clicky on the link and it'll explain. Fair warning, though, there are some photos included on the page that are NOT cute.) It affects quite a few corners of his life and, as they've been joined at the hip (mostly) since they were in high school, more than one corner of my sister's life as well.

     Usually, our partners' respective medical ailments are something we can joke about with each other - "Damn, we sure know how to pick 'em, don't we?". It's our way of coping with the often-frustrating world of medicine, emergency rooms and hospital stays. Now, I have to admit that we don't usually commiserate about our fears for the future and I wasn't really sure why not. We're pretty close, my sister and I, and nothing has ever been off-limits in our discussions over the years. In fact, I've often told people that the next best thing to being in my head is being in Liz's, as they work in such a similar manner. Most of the time, anyway.

     On the day in question, Liz made some remark about her "boyfriend's broken colon" and how it was making their impending cross-country move a little more difficult than it would otherwise be. I remarked that yeah, I understood that sentiment, since I deal with my husband's Marfan's making things a little more difficult every day. At this, she rolled her eyes and remarked, "Yeah, Brendon's is a little bit more than just, 'Oh my back hurts sometimes.'" Not gonna lie, I was momentarily speechless at her flippant callousness. (It takes a LOT to render me speechless, so you can imagine how many thoughts were flying through my head at that moment.) Even when I did regain use of my speaking abilities, I wasn't quite sure what to say. I mean, what do you say to that?

      I have to make it clear, before I go any further, that my sister isn't callous by nature. She's as much of a bleeding heart as the rest of my family and she loves her brother-in-law as much as I love Brendon. (She's  been with Brendon for so long that I consider him my brother-in-law, for all intents and purposes.) At first, I couldn't figure out what possessed her to say such a thing or really, to cop such an overall attitude about my husband's disorder. She knew better than that, I thought. Upon further reflection, though, I began to wonder if maybe she actually didn't know better.

     I know I've mentioned before my tendency to research the hell out of things when I don't feel I know enough about them. That's exactly what I did when I found out that Brendon had acquired, somehow or other, the ulcerative colitis. I wanted to know everything I could about it - what it did to the body, what it could potentially do to the body that it hadn't done yet, how it would affect day-to-day life, all of it. Mostly, I wanted to know what, if anything, I could do to help him and my sister. That's what family does, in my world.

     Liz also researched the hell out of this disease, especially after the third or fourth hospital stay Brendan had to endure on account of the colitis. She's knows a fair amount about it know, enough that she can help him make informed decisions with regards to his treatment and care. She's also been a front-row witness to the devastating effects an illness like this can have on someone's life, like the multiple jobs Brendon has lost because he's been sick. However, she did not do any research on Marfan's Syndrome and therefore has very little idea of what it actually is and just how much of an impact it has on just about everything. Can you blame me for feeling frustrated and hurt by her oversight?

     Please don't think I'm suggesting that everyone in my and my husband's life should go out and read about his disorder until they're cross-eyed and their brain feels like it's going to ooze from their ears; it's not that at all. Almost everyone I know has so much going on their life that they can hardly keep it all straight and end up at the grocery store when they should be at a doctor's appointment. I expect no one to put their time and attention into something like this, which does not directly concern them. I do, however, expect them not to run off at the mouth when they do not understand what in the hell they're talking about. I see that as a basic courtesy; my sister apparently does not.

     Some people will probably read this post and say that I'm making far too big a deal out of nothing. In all likelihood, I will probably look back at this and think that it really wasn't much of anything after all. Right now though, it's a big damn deal to me. My family and friends are such a big part of why I'm able to handle my husband's disorder and everything that comes with it as well as I do, because I know that I've got that (usually) unwavering support behind me. When you consider that some key members of that family-and-friends network are on the other side of the country and not easily accessible, it really clarifies why I cherish those that are close to me so much and why it's so troubling for me when someone like my sister puts a tiny crack in the foundation.

Saturday, June 25, 2011

Aeroplane Blues

     My husband and I are going on a trip to North Carolina this October and it would appear that we've hit a snag in the planning process. We live in St. Louis, MO, you see, and we need to get to Charlotte, NC, which is approximately seven hundred and twenty miles away. The most obvious question that needs to be answered here is how are we going to get to the other side of the country? Most people would be looking at two choices in this scenario - flying or driving. Since we are, in many ways, "most people", we're currently debating the pros and cons of each method of travel. It's just that, like so many other things in our life, my husband's disorder is making what should be a fairly simple decision into the traveler's version of Sophie's Choice.

     I'm the one who takes care of the finances in our household, so I'm the one who's already trying to plan how much money we need to put away for our little sojourn and checking Travelocity every week to see if the price on our plane tickets has gone down since the last time I looked. Finances are a big factor in just about everything we do, so of course I'm  trying to do this ten-day trip as economically as possible. I priced two round-trip plane tickets at a couple of different airlines and the priced a rental car. Nothing fancy, just a something with four wheels and a steering wheel that will get us where we want to go while we're there.

     The prices really aren't bad and since we've been saving up for this trip, it's feasible that we could just fly out there, stop at the Enterprise counter and then be on our way. That's option number one and it's a good one, for a couple of reasons. One, it would be the quickest way to North Carolina, as the flight only takes four and a half hours, including one stop in Memphis to switch planes. It wouldn't take much actual vacay time to get to our destination, which is always great, because we want to spend as much time as possible visiting with friends and family.

     The second (more important) argument for flying out there is that it would be much easier on my husband's body, as he'd only be in the seat for a couple of hours at most. (The problem is that the muscles in his back seize up if he's in one place for too long. Twelve hours in a Mazda6 probably qualifies as too long.) I don't want him to be in pain while we're traveling and I really don't want to have to go straight to the hospital for the big-kid drugs as soon as we roll into town. It also usually takes a day or two of recovery time once he gets home from the ER to get back to his usual self, which would further cut into the time we could spend doing what we want to do.

     On the other hand, the case for driving out there isn't exactly weak. The most practical argument for it is, of course, the financial difference between a road trip and buying plane tickets and renting a car once we're there. It's a difference of about five hundred dollars and even though we've got the money for it (like I said, we've been saving for this for awhile), does that mean we have to spend it on travel accommodations? Couldn't we also use it to do more stuff while we're out there? My husband seemed to think so, when I was discussing it with him.

     There's also the small problem of me being absolutely terrified to fly. Granted, I've never flown before, (unless you count that time after a car crash in high school that I was flown via emergency helicopter to the hospital, but I was in and out of consciousness the whole flight and don't remember anything from that night anyway) but I have no desire whatsoever to do it. Seriously. I was watching an episode of Friends one time in which Rachel was flying to Paris and when the guy shut the door to the terminal behind her, I about lost it. I don't know why, but I couldn't help but think, "Oh my God, what if I don't want to do it and they won't let me off the plane?! THE MAN ALREADY SHUT THE TERMINAL DOOR!!!!!!!" Pretty sure I'm borderline-insane sometimes.

     Also? I just love driving. It's a stress-release for me, to drive around and listen to music loud enough to shake the mirrors of my car. I think it's also a nostalgic thing for me, as we always took a vacation every summer when I was growing up. We always drove to our destination, never flew, which meant Daddy was rarely included on the trips. (He drives too slow and he wants to make too many stops along the way. My mother trained my sister and I from a young age to get in the car and go.) I made most of the StL-to-Charlotte drive a few years ago, on my honeymoon with my first husband. It's an easy drive and really pretty, so I would love to travel that route with this husband.

     My husband also mentioned to me that when he lived in North Carolina, he and his friends would sometimes take road trips and drive the Blue Ridge Parkway while blasting Yes (he's got a thing for prog rock, I don't know why) and that's a really good memory for him. He actually loves road trips just as much as I do, but that's something that's been essentially taken away from his as the Marfan's has progressed. When I think of that, how such a simple pleasure has become so difficult for him, I can't help but feel angry and frustrated. It's just one more thing that I can do nothing about and instead watch impotently from the sidelines as he struggles to accept the latest disappointment.

     Gotta bring it back, though, and not dwell on the hurdles in our path. We've been talking about it and there may actually be a way to drive it. My husband will go to see his pain-management doctor on Monday, whom he hasn't seen in almost two years. Perhaps she will have some good news for us, some way that she's figured out to better manage his chronic, sometimes debilitating pain. At the very least, my husband's planning to talk to her about our trip and see if she has any suggestions that would make driving a viable option. He seems to think that with the right combination of painkillers and stops along the way so he can move around a bit, it can be done. I'm not entirely convinced of that plan and I won't allow him to be in pain just so I can avoid the flying death-trap.

      It looks like at this point, it's going to depend on what the doctor tells us on Monday. If she and my husband think it's actually going to be okay, then we'll drive it. We'll get to spend some quality us-time together, see the pretty sights along the way and save a few dollars. If the driving turns out to be an absolute "no", then I'll just have to get over my fear of the completely unnatural aluminum alloy contraption that has no business being miles above the lovely safe ground. Wish me luck with that one.

Thursday, June 23, 2011

Soul Meets Body

     On Saturday night, my husband came home from work and sat down in his computer chair, which is right next to mine in our rec room. I was quite literally in the middle of a raid with my World of Warcraft guild (If you really want to know, I can explain it to you. Otherwise, suffice it to say I was playing a video game on my computer with lots of other people and we were at a pretty important part in the game.) and not really able to pay much attention to what he was saying as he started talking to me. I actually started to shush him, because the raid leader was busy explaining what we were about to do, when my husband got my full attention by looking me straight in the eye and telling me, "Jean called - my grandpa died yesterday."

     As soon as it registered with my brain what he'd said, I turned back to my computer and apologized to the rest of the people I was playing with, telling them I had to leave immediately. I told them why I was leaving them as I was logging out and of course, they told me to go, that I needed to be with my family. My husband now had my full, undivided attention. He told me what had happened, that his mother had been trying to get ahold of him all day, but had been calling his work cell instead of his personal cell, which is the one he keeps on him when he's not on call. When he got off work that night and was able to return a phone call from his sister, he found out what had happened.

     My husband loved his grandfather and even though they weren't extraordinarily close, I could see that he was shaken by the news. Granted, his grandfather was ninety and his health had recently been less than great, but it's never an easy thing to accept. As I mentioned before, I immediately quit what I was doing and gave my husband my full attention so that we could discuss what had just happened and what, if anything, we needed to do for the family. I wanted to make sure, first and foremost, that my husband was really okay with his grandfather's passing. (As okay as anyone can be with the death of a loved one, I mean.) He can be all kinds of "yeah, I'm fine" when it comes to major things like this when the reality is that he is actually no such thing.

     I'm writing about this now because I can't help but wonder if the stress of the events of this past weekend didn't exacerbate the Marfan's to some degree. It probably wouldn't even have occurred to me that that could happen, except for the fact that Sunday was very painful (physically) for my husband and Monday was still bad enough that he had to call into work. While it's been known to happen that way on occasion, it's not the norm for him. He's worked longer, harder doubles at the pub and still been able to go into his regular job come Monday morning. I think the emotional weight of the his grandfather's passing had a physical effect on my husband's body.

     As usual, when I looked for information about a possible link between emotional stress and worsened Marfan's symptoms, I came up empty-handed. It's still such a source of frustration to me that in this day and age, when information is practically vomited at you from all corners of life, that I can find so little information on this disorder. So all I have to go on are the various studies that say yes, emotional stress can have a physical effect on a person and can heighten the symptoms of a preexisting condition. None of these mention Marfan's syndrome by name, so I'm taking liberties here and making a semi-educated guess that if it can happen to someone with fibromyalgia, it can happen to someone with Marfan's. (And yes, I know the two are not related - I'm simply using fibromyalgia as an example.)

     While it didn't take any sort of big-brain reasoning for me to figure out that there's a potential issue with the stress doing bad things to his body, I'm having a significantly more difficult time deciding what, if anything, we could/should do about it. My husband, while not the best at dealing with his emotions in a healthy way, has at least never been one to shy away from bad things happening. By that I mean that he doesn't hide his head in the sand or pretend the badness isn't happening; he'll face it head-on. (Whether he fully processes his emotions resulting from those happenings is something else altogether...) So trying to keep him in the dark when potentially stressful things go on is not an option. It's not in his nature and truthfully, it's not in mine to be able to do that to him.

     We're partners, on every level that you can be partners with another human being. As much as I may want to prevent the ill effects stress has on his disorder, the fact of the matter is that I need him to be with me during the bad parts of the movie, you know? I can do a lot of things on my own, but I don't necessarily want to. I want us to be in it together, for all of it, and I know he feels the same. Actually, if I were to be completely accurate, I would have to say that my husband would prefer to shoulder the bulk of the emotional load for both of us when it's necessary. I'm big on equal opportunity, though, so that doesn't really fly around our house; we share.

     My husband can't live in a bubble, no matter how potentially beneficial it may be to his physical health. He wouldn't stand for it if I tried, and I'm not going to live that way in any case. You can call me selfish (you'd be wrong, but you can do it if you feel you must), but dammit, he's my husband, my partner in crime, my perfect match and by God we're going to suffer together if there's to be suffering. I fully believe that the heart rules the body and though that occasionally results in negative consequences, it can't be any other way. I feel like if we start to do that, to move into avoidance of life's unhappy moments in an effort to curb the effects the resulting stress has on his Marfan's, then we're letting the disorder gain a foothold in an area it has no business being in. Which means...I've just talked myself in a circle and still don't have a clear idea of how to proceed with this stuff from here on out. I love it when that happens.
    

Tuesday, June 21, 2011

Sick Again

     Well, it was nice while it lasted. My husband had to call into work yesterday because he was just in too much pain to do it and we both knew that if he would've gone in anyway, it would have resulted in nothing but badness. He'd worked his second job on Saturday and had pulled a double, so it wasn't entirely unexpected that he woke up on Monday in a decent amount of pain. (His second job is waiting tables at a local pub and is fairly labor-intensive. This, despite the fact that he has several little workarounds to make it actually doable for someone in his condition, like the fact that he never carries full trays; he just makes several trips to the tables to serve their food.)

     It's a little more complicated when he needs to call into work that it would be for someone who's healthy. I know he feels guilty when he has to do it, because he's got a limited amount of sick days and has gone through them pretty quickly this year. Luckily, before yesterday, he hadn't called in for a little more than two months, so yesterday wasn't unpaid. That's always key in our household. He always asks me now before he actually makes the call, to make sure I'm okay with it that he's missing a day. I'm aware of how very "Wow, we know who wears the pants in that family" that sounds, but I promise you, it's not really like that.

     It's just that I'm the one who's a little more removed from the situation and so can better see when he really does need to take a day off to give his body a break and when he just feels kind of "meh". Truthfully, most people would call into work for a "meh" day or two and not think anything of it. Hell, I'm sure I've done it myself once or twice. My husband doesn't have that luxury. What with all the days that he can't move from the pain in his back and the days that he has to spend at the hospital dealing with things like a squished eyeball, he really has to pick and choose what's worth it to take time off for. Then when you factor in all the half days and days he goes in late to accommodate the 8,265 doctor's visits he has to have to make sure everything is still (more or less) functioning as it should, it gets even more convoluted.

     I think, though, that it's better for him and for us in the long run if he takes the time he needs as he needs it. The Marfan's is degenerative, which basically just means that it's going to break him down as the years go on. No one, not even his specialists, know how fast it's going to happen or to what extent. They can make well-educated guesses, but at the end of the day, that's all they are - guesses. My husband and I are managing this disorder as well as we can, but it's totally an as-we-go sort of thing. He knows his body well enough at this point to know when he needs to stay put to avoid the more dire consequence of pushing it too far and then landing in the emergency room. And all that? That's just the physical and on-the-surface part of the issue. The guilt that he feels for having to have so many more accommodations than the average person is another story altogether...

Thursday, June 16, 2011

What You Never Know

     The other day, when I was bopping around the internet looking for information to use in my last post, I began to see just how much I don't know about Marfan's Syndrome. I mean, he's always been honest with me whenever I've asked him a question about it and I know how to use Google as well as the next girl, but there's so much that I somehow skipped over in my searches. There are some things that I'm actually embarrassed to admit that I didn't know, like what an aorta actually is. (I knew it was attached to/part of the heart and that it was very, very bad if it got damaged, but I didn't know what it really was.) Wouldn't you think that little details like that wold be important for someone in my position to know?

     That's not to say that I just run off at the mouth without having the first clue what I'm talking about; I don't. I know that my husband was born with a degenerative genetic disorder, I know it could've been a random gene mutation that caused it or it could've been hereditary, I even know the name of the specific gene which mutated and is causing all the issues. I promise you, I'm far from uninformed on this matter. I have to admit, though, that I skipped over a lot of the details.To be fair, there are A LOT of details to skip over, but still.

     I'm a curious person by nature, so whenever I'm looking up information on something, I tend to dive deeply into the subject until I can absorb no more information. I think this is a little different, though, and what kills me is that I didn't even realize what I was doing until just now. On one hand, I want to know as much as I possibly can about Marfan's and what it does to someone and what possible complications could arise as a result. Obviously, I also want to know the best way of handling any potential medical issues that pop up so that I can take care of my husband the best that I can.

     I want to read everything I can find (which is a frustratingly small amount, let me tell you) so that I know what's going on with my husband, health-wise. I never thought I'd been in this position when I envisioned what my married life would look like and the fact is, I'm looking for guidance. As I've mentioned before, I feel sometimes that I'm flying blind and I think my husband feels the same, to some degree. He's never known anyone who has anything similar to his disorder, so really, he's just as much deal-with-things-as-they-happen as I am. If that's how we're going to live, then I want to be as prepared as possible when it comes time to make decisions that I'm not really qualified to make, but have to anyway.

     The flip side of all this is the fear factor. I read some really, really scary stuff the other day, a lot of which I now wish I didn't know. I figured out exactly what an aorta is and why we should be so damned afraid of it rupturing. I also read about all the other bad things that I knew were possibilities, but never allowed myself to consider overly much. If you don't get too in-depth with your research, it's easy to say, "Well, sure, that happened to that Marfan's kid, but that was a freak thing, not a common one." Except for when you start reading story after story of people it actually happened to. Read enough stories, you start to realize that it can happen to your Marf, too.

     As much as it scares me, I can't hide my head in the proverbial sand here, which I was apparently doing without even realizing it. It shames me, quite a bit, to know what was going on. I'm someone who takes pride in my desire to learn and educate myself about all manner of things. So now, when it really matters, what did I do? Allowed myself to skip over so much of what's out there for me because I didn't want to deal with it. That's not me, not my way of dealing with things. In this case, the old adage, "What you never know won't hurt you," is dead wrong, because what we as a couple don't know about Marfan's could very well kill my husband.

     I think, "What you never know won't scare you," or some variation thereof is what was floating around at the back of my head. It's still there, actually, if I'm being completely honest. (You don't magically get rid of that stuff just because you acknowledged its existence.) I don't want to think about my husband dying, don't want to think about the millions of extra things that could go wrong because of a stupid genetic mutation that no one had anything to do with. That's ridiculous and selfish, though, because lots of people have to think about things like that everyday. I do believe I've even mentioned before that avoidance of such issues is a pet peeve of mine. So maybe I need to start taking my own advice and learning how to deal with my fear instead of pretending it's not there, because it so clearly is.

Tuesday, June 14, 2011

Feed My Frankenstein

     As I was driving to work yesterday, I heard the most interesting thing on NPR - a man who had amyloidosis  was told that he had about twelve hours left to live because his heart was so badly damaged. The man and his wife, knowing they had nothing to lose, opted for a procedure in which his rapidly-failing heart was removed completely and replaced by two centrifugal pumps. No, really. The man's heart was taken out and replaced by these little pumps made of titanium, Dacron, silicone and fiberglass. I've never heard of such a thing before, but the possibilities this procedure opens up are fascinating and of particular interest to me.

     While it's true that Marfan's Syndrome affects just about every bit of connective tissue in my husband's body, it's his heart that concerns me the most. That's not to say that I don't care about the effect this disorder has on his eyes, his spine, his joints, his muscles and his every-damn-thing else, because I do care, very much. I feel, though, that those symptoms, while not necessarily more easily managed than his heart business, are at least less life-threatening in nature. True, the dural ectasia is less than fun and often causes my husband to have what he refers to as a "spinny head" (translation - he's dizzy and often seeing spots swimming before his eyes) and numbed legs. It's not going to kill him, though. I'm afraid his defective heart will.

     I know he's had an aortic valve replacement and an aortic root replacement, (basically, the part of the aorta that the valve connects to has been replaced by a piece of garden hose, which is much more durable. It's not actual garden hose, but you get the idea.) but he's still susceptible to an aortic aneurysm rupturing out of the blue. He sees his cardiologist once a year and the most recent visit yielded good news, but a lot can happen between annual visits. What if he develops an aneurysm between now and then? What if a weak spot pops up on his heart somewhere and it becomes an aortic tear?

     These thoughts are usually always in my head, so of course I was intrigued by the idea of replacing someone's heart with two mechanical pumps. According to the two surgeons who developed the technology and pioneered the surgery, the pumps don't wear out like a traditional artificial heart, probably because they only have one moving part - the rotor itself. I found out that lots of people have one of these devices implanted to assist a weak heart, but never before has someone hooked two of them together, effectively creating both a left and right ventricle, and replaced a heart altogether.

     I mean, my husband's already got hundreds of thousands of dollars' worth of man-made parts in him anyway; a Franken-husband, if you will. Would something like this be beneficial? Apparently, due to the design of the pumps, there is no pulsing to move the blood. Instead, it's a continuous flow. Obviously, I'm not anything close to a doctor and this is, as I understand it, very new technology, but I would think that the very design of the pumps would potentially be beneficial to someone with Marfan's. The connective tissue throughout the body is weak, right? One of the most worrisome areas is the aorta, due to the possibility of a ruptured aneurysm. So wouldn't it make sense that the continuous flow as opposed to the pressure of a pulsing flow would be easier on the tissues, thus reducing the chances of a rupture?

     It seems that way to me, but again, I'm theorizing based on common sense and whatever I can remember of my senior-year science classes. (It's not much, I assure you.) Then again, I would be the first to point out that these pumps would not address the problem of the aorta itself. That would still be weakened by the Marfan's and thus, a potentially life-threatening issue. Also, if you'll allow me to get all existential on you for a moment, what happens if these pumps really do work as well as the doctors say they do and they never die? I mean, the rest of my husband's body will eventually stop, Marfan's or no, but what happens when the pumps keep going? Would I, as his spouse, be faced with the awful eventuality of telling the doctors to turn them off because the rest of his body is done?

     He and I make light of the fact that he's kind of like a Frankenstein, what with all the artificial parts he's got going on, but that's because A) you have to have a sense of humor about this stuff and B) none of his acquired pieces are literally responsible for keeping him alive. (The titanium valve could be debated, I guess, but it's not the thing that keeps him moving.) To actually be alive because of a machine that's replaced one of the most vital organs in the body is a whole different ballgame. My imagination is fully developed, I assure you, and I can get my head around some pretty out-there concepts, but this one...

     I'm struggling with it, I can tell you that much. The possibilities are so intriguing, especially for someone with a heart that was never healthy to begin with. I'm all for medical research and progress, but I just don't know about this one. Is it a little too how-to-make-a-monster to anyone else, or is it just me? I can't help but think of what it would be like to witness my husband's body dying, as everyone's must, but being denied a natural, peaceful exit because of the unnatural nature of his "heart". This isn't like a Pacemaker, a supplementation to what's already there. This is something entirely different, something that exists in uncharted territory. I really don't know if I'm ready to go there.


    

Saturday, June 11, 2011

Hang On To Your Ego

     You know what one of my biggest pet peeves is? (I have a lot of them, so if you don't guess correctly the first time, I won't hold it against you.) People who play the you-can't-possibly-understand-what-it's-like-to-be-me card. Flat-out drives me up the wall and makes me want to smack them upside the head and say, "You egotistical moron! Stop being so elitist and smug about whatever it is that you think makes you a special snowflake. YOU'RE NOT!" Or something to that effect. I just cannot for the life of me understand why people seem to feel the need to flaunt it when faced with something like a chronic illness. It seems as though they'd rather use it as a flag to wave, declaring how different they are and how much no one else could ever begin to relate to their life, instead of just accepting it as one more aspect of their life, of no more import than eye color.

     If you've read any of the other entries in this blog, you know by now that I'm the wife of a man who was born with Marfan's Syndrome, a degenerative genetic disorder. And if you haven't read any other entries, now you know. As I was telling my mother-in-law just the other day, I don't necessarily view my life as all that much different than anyone else's. Now, I should qualify that statement by admitting that yes, I know there are a few differences between me and the next married girl. I'm planning for a different future than most, I'm twenty-six years old and already worried about how long we have until my husband becomes fully disabled. I'm not stupid; I'm well aware that there are some unique challenges with my marriage. Hell, that's the whole point of this blog, isn't it?

     There's a fine line, though, between acknowledging and accepting the challenges unique to my situation and wallowing in them. It's a line that's not always easy to walk, because I'm capable of throwing a pity party just as well as anyone else. If I really wanted to, I could rock a pity party. That, however, is not my modus operandi and never has been. In my mind, when you're faced with a problem of such magnitude as a potentially life-altering disorder, you figure out a plan of action, you keep your head up and you damn well keep it moving. Militaristic? I don't think so.

     The summer I was twelve and my little sister was ten, I learned my father had cancer. There was no crying, no wailing, no ohmygodwhatarewegoingtodo?!?! My parents were very straight with us about what my dad had been diagnosed with, what his treatment options were and what we, as a family, were going to do to get through this. As my father is so very fond of saying, we made a game plan. He endured chemotherapy and radiation treatments all summer and into the fall and continued to work 40+ hours a week at his job as yard boss at a local lumberyard while he did it. Come the fall, he went in for surgery to remove the tumor and he's been cancer-free for almost fourteen years. All the while, my family kept living as we always had - my mother went to work, my sister and I went to summer camp, we watched TV and played in the pool in the evenings. In short, we kept living our lives.

     That whole time, Daddy never brought up his illness to anyone, though he would answer questions when asked. Mama did the same and my sister and I followed their example. It was not because we were ashamed, or too scared, or felt it didn't warrant discussion. It was because the cancer was simply another part of our lives, no less, no more. It did not make my father any more unique than the next guy down the line, as there were millions who were in the same boat. It did not make my family any more unique, for the very same reason. It is that mentality that I use when I think of my husband's disorder and our life now.

     I think that people who have disorders like Marfan's Syndrome can potentially become egomaniacal about it, given the way our society is currently set up. To read the newspapers or a magazine, you'd think that every person who is diagnosed with any damn thing is the first and only; every case is unique and special. While that may be true from a medical standpoint, as a practical matter, that just doesn't hold water for me. Why, if you're faced with a diagnosis which could potentially change the course of your life, would you want to wrap yourself in the No One Knows What It's Like Cloak instead of seeking out individuals in similar situations?

     Wouldn't you want to do what I'm trying to do here, look for people in the same position so that you're not alone and you do have someone to commiserate with? Wouldn't that be a comfort to you, to know that there's someone out there in the great wide world who can relate to you on this one? (Not saying that this is the only/best way to deal, it's just the one that makes the most sense to me. To each their own, though.) I admit, most people can't fully understand the tangles I sometimes encounter with my husband's disorder and its effects on our relationship, but they can empathize and they can listen and offer what they've got available to them. Meanwhile, make it a point to search out others like you, so that you do have someone who gets it completely. I promise, there's nothing in this world so unique that you're the only one affected by it.

    


Friday, June 10, 2011

Out of the Blue

     I wonder what it's like to know someone for years and not know anything about something that's a huge part of their life. And what must it feel like when you do finally find out about it? Is it a huge revelation, something that you never even knew existed? Or is it more like a light bulb going off when all the pieces of something that lived blurrily in the corners of your mind finally form a cohesive picture? It's so hard for me to imagine what it's like to be in that position because I was raised in a family in which anything and everything was open to discussion. Especially with medical issues, nothing was hidden from anyone. Quite the contrary, as soon as there was any sort of diagnosis, telephones across the country started ringing like crazy from all my relatives racing to make sure that everyone else had as much information as possible.

     The same kind of mentality was also pervasive among my core group of close friends. If something significant (or insignificant, for that matter) happened to one of us, the rest of us knew about it in short order. We discussed it, dissected it, figured out the whys and the hows and then decided what, if anything, was to be done about it. As I got older, I began to realize that this was not the norm, that many times, people just kept to themselves about what they considered sensitive subject matters, only opening up to a few people close to them. My husband most definitely falls into the latter group, those who keep things to themselves and only talk about it when absolutely necessary.

     I've known that he has Marfan's Syndrome since we first became friends, though I understand from him that I'm unusual in that respect. Apparently, his disorder is not something he ever really disclosed to his friends, though I'm sure they knew something was up with his health when he was scheduled to undergo open-heart surgery at the ripe old age of twenty-eight. And that was after he'd been cut open and had his spine reconstructed with titanium rods and his own pulverized rib.(True story, in order to help reinforce my husband's deteriorating spine, the doctors removed one of his ribs, ground it up into powder and sprinkled it on his spine. I'm oversimplifying, of course, but you get the idea.)

     Despite being witness to these major events, there are still people in my husband's life who don't know the root cause of the medical adventures. I think that's mostly due to my husband's closed-mouth policy when it comes to his disorder and, to a much lesser extent, due to friends not wanting to ask questions for fear they're treading on boggy ground. Lucky for him, I have no such fear, nor have I ever. I dove right in with the questions as soon as I figured out that he ticked. (His heart, I mean.) So for me, there's never really been a not-knowing and consequently, no huge revelation somewhere down the line of a years-long friendship. I know that's not the case for everyone, though.

     I don't know how I'd feel about it if I found out after all that time had passed that my friend had something as serious as Marfan's Syndrome. Would I feel betrayed at the lack of disclosure? Perhaps begin to doubt just how strong our bond was, that he didn't deem it necessary to share such an important part of himself? Anger at being kept in the dark, no matter that it wasn't by design, but rather the result of a lack of desire to talk about something that he wasn't ready to deal with? Sympathy is probably the obvious reaction, though if it were me, it would be more tangled than just that. I think I'd also feel some sadness (possibly guilt, unwarranted though it may be) when I thought of all the time that I could've done my damndest to help him when he needed it, if only I had known. Which might then lead to resentment and other sticky, sticky emotions.

     I have to discuss this in bits and pieces, because there's so much to this rather delicate topic. It's one that I believe is a key piece to understanding the prism through which my husband views himself and his disorder. Everyone makes their choices in life based on what they feel is the right path for them. In general, I don't believe anyone has the right to criticize the choices made by another individual, especially when it comes to situations like the one my husband was born into. It's the old walk-a-mile-in-my-shoes school of thinking. Some might disagree, especially considering that my husband's habit of keeping his disorder as private a matter as possible can and does affect those close to him. I have to respect his decisions in this arena, though, as ultimately, he's the one who has to deal with any repercussions due to said non-disclosure, not me.

Wednesday, June 8, 2011

Carry That Weight

     One of my biggest goals in life is to live up to my own expectations as a friend. I want to be a good friend and in fact, pride myself somewhat on the fact that most of my closest friends have been around since grade school and our bonds are as strong as ever. I would even venture to say that we're closer now than we've ever been, because we've all experienced more than a decade of living with one another. Deaths, births, divorces, marriages, moves to other states, unemployment, career changes - you name it, we've done it together. I can't even begin to convey how much I've been given over the years by these people, who are just as much my family as my blood is.

     It follows then, that I want to be able to provide the comfort, the advice, the anything else that's been given to me whenever I've needed it, whether I asked for it or not. My husband has a small group of friends about whom he feels the same, though many of them live half a country away from him now. He wants to be there for them just as much, whether they're having an issue large or small. Right, you get it, we like our friends and want to be good friends to them. What's the issue? The issue is that I've noticed, more and more, that people seem to think they're burdening us with their problems and so shy away from saying anything to either of us. That could not be further from the truth.

      I need to explain what I mean a little better, I think. My husband has Marfan's Syndrome, a degenerative genetic disorder, right? Right. I, as the wife of said Marfan's person, have to deal with all the fun things that accompany such a disorder, like the daily meds, the hospital trips, the overly-complicated surgeries (hello, two-week recovery time for a fricking hernia repair!). I promise you, it's really not that big a deal to us when it comes to the day-to-day of everything. We live our lives just like everyone else, albeit with a few modifications here and there to allow for what my husband can and cannot do. In short, we make it work.

     I'm beginning to notice that it's as if some people don't fully believe me when I tell them that. Actually, to be more accurate, it's as if they think I'm just saying, "No, it's okay, tell me what's going on," to cover up what I really mean - "Of course I don't want to hear about your petty problems! I've got much bigger and more legit fish to fry over here with my sick husband!" People, that's not at all what I'm saying. I'm someone you can take at face value and if I don't have time to talk about whatever's on your mind, I will tell you so and ask if we can dissect it later. That said, I don't really think I'd ever be in a position where that would be necessary, 'cause I do have the ability to, you know, think about two things at once. Even better than that, I'm able to not think about me all the damn time and focus my attention on someone else's problems.

     I don't want my friends (or my family, for that matter) to think of my husband's disorder first and try to decide whether or not they should "bother me" with whatever it is that's going on in their lives. I don't want them to preface something with, "I know you've got a lot going on, but..." because it shouldn't matter. I don't want them to ever hesitate to tell me or my husband about an injury they're dealing with because it surely isn't as significant as my husband's physical ailments. It actually IS as significant. Things like that have just as much of an impact on their lives as anything with my husband's health has on ours. You're having a hard time dealing with bad choices your little sister is making? Tell me about it, I can try to help. Pulled a muscle in your back while you were at work? Hey, commiserate about that stuff with my husband. I know he knows of some pain-relief remedies that your doctor would never suggest.

     I hate feeling like less of a friend just because my life is what it is. Yeah, my life may be a little harder than some, but how many millions of people can claim the same thing and be right? A lot of them are far worse off than my husband and I, but that in no way invalidates what we have to deal with. By the same token, our life being affected by my husband's Marfan's in no way invalidates anything that our friends/family are dealing with. Their problems, concerns, injuries - they're just as important as anything I have to deal with. I'm a great friend, so is my husband, but only when people let us be. I want to share the burdens of those I love as much as I possibly can, to make the weight easier to carry. It works out so much better that way, I promise.




Friday, June 3, 2011

You Say It's Your Birthday

     My awesome husband turns thirty-eight today and as a birthday surprise, he got a burst blood vessel in his eye! Happy birthday! Ugh. It's the same damn eye that he squished, too, so that's slightly worrisome. Also, he just had yet another appointment with the eye doctor to (supposedly) wrap up the squishing drama, but it seems that that's not completely over. The hole in his eye has sealed itself up and it's no longer leaking juice, but the pressure is still really low. The doctor wasn't entirely sure why and said that my husband needs to go back to be seen in six weeks, sooner if he starts feeling that pain in his eye again. If the pain comes back, that means the hole is back and that means a laser to close it up. For reals?

     Can I be completely honest here and confess that neither I nor my husband really care all that much right now about the latest burst thing on his body? Granted, it's just a burst blood vessel in his eye, which for us is so beyond old hat it's not even funny, but still. I feel like one or both of us should give a little bit of a damn. And yet...we don't. Most of that indifference is due to the fact that we know it's nothing serious and will go away in a couple of days. The rest of that indifference is probably because we're just tired of being worried about something of his that's once again broken.

     It's kind of like what I was saying before about knowing when to go to the hospital and when to ride it out. Eyeball is causing him large amounts of pain and he's seeing spots? Most definitely time to see the doctor. Yet another red blotch due to a burst blood vessel? That gets greeted with a resounding "Meh". Yeah, we'll watch it, but really, the fact that his eye still has low pressure in it is far more worrisome. That's what we'll keep an eye on (no pun intended), just in case it gets worse. Truly, though, something like this is just par for the course now and I wonder if that sentiment is going to get progressively worse.

      I think it likely will and I'm totally okay with that. I know I'll never become completely indifferent to the Marfan's Syndrome and everything that goes with it, but I know that as time goes on, I'll worry less about my husband carrying too much weight up a flight of stairs or trying to move furniture that I deem too heavy. I hope that I'll eventually stop freaking out if I can't hear his heart ticking while he sleeps because he's laying at an odd angle. (Sounds strange, I know, but it happens. Sometimes, if the comforter is on top of him just right, it muffles the sound of his titanium valve and I panic, thinking that his heart has stopped.)

     You know, as I sit here thinking about it, I have to admit that I'm actually looking forward to the day when I can learn to relax about more things pertaining to his health. My husband is already in that frame of mind, having lived with this disorder far longer than I have and he's actually really good about dealing with my freak-outs. He's quietly patient and I know that every time I don't make a big damn deal out of something, he silently cheers. I'm going to get better with time, I know and that'll make it a little easier on both of us when something big actually does come up. Now, though, I think it best if I just enjoy his birthday with him and anticipate the many more to come.

Thursday, June 2, 2011

Best of You

     There's a feature on this blog that allows you to see the various statistics related to the audience. I can see how many people have looked at my words today, which posts they've read, from which countries they're doing their reading and so on and so forth. It's vaguely creepy, knowing that much about the seemingly anonymous internet crowd, but I kind of like it. I like seeing that other people in random parts of the world, like Russia, can relate to what I'm talking about, that they find it interesting. There's also a feature that shows me what phrases people put into Google and end up here. I always like to check that stat in particular, because sometimes people google the damndest things. Two days ago, though, I checked it and found that someone out there in the great wide world had used the phrase, "Marfan's I hate my life". Oh, God...

     As I've said so often before, my husband and I are on different sides of the same issue. He is the one who was born with Marfan's Syndrome, I'm the one who married into it. It follows, then, that our perspectives and our experiences don't always line up exactly. When I saw this phrase, I immediately felt pity (which is the thing my husband hates most) and sadness for whoever it was that typed it in. How much does that break your heart, to know that there's someone living among the billions of people on the planet and they still felt so alone that the best they could do to find comfort/answers was to turn to a fricking search engine and hope something popped up?

     Even having seen my husband at his low points, when the magnitude of what he's dealing with and what's coming just presses him into the ground, I still can't understand what it must feel like. The real hell of it is, as low as my husband has gotten since I've been around, I know it's not even the worst of what he's felt since he found out about the disorder. I can only imagine what those times were like for him and wonder if that's what this nameless, faceless character is also feeling at the moment. I wonder if it genuinely gets better for them, or if the bad thoughts just abate for periods of time and then creep out from the dark corners when the guard is down.

     I remind my husband with some degree of frequency that he is much more than the Marfan's and most days, he knows that. He still has days, though, when he feels worthless and broken, a burden to society at large and mostly to me. Those days? Doesn't matter what I say or how often I say it - he feels what he feels. I sincerely hope it's not the same for my mystery reader, but I have a suspicion that it is. I have a sinking feeling that whoever typed that it is younger than my husband, perhaps someone who just found out and hasn't yet learned to cope with everything they can now not do. Maybe it's a teenager who's relentlessly and unmercifully taunted for their physical appearance, for the skin-and-bones look that they're involuntarily sporting.

     Or maybe not. Maybe it's someone who's a bit older and perhaps has never had a relationship with someone who understood the delicacies of a degenerative disorder and thus they have no one to talk to about the strange and sometimes frightening thoughts that surely must be running (screaming?) through their head. Maybe it's someone who's older still and is now wheelchair-bound because their spine can't hold them up anymore and the loss of freedom is something they've not yet be able to cope with. Or maybe all of these dire assumptions are wrong and that phrase was put in by a bratty, well-to-do teenager who just doesn't like the limitations now imposed on an otherwise carefree life.

     I'll never know the person who typed that, never know if they found any comfort or sense of hope in my publicized thoughts, never know if they ever come back here. Strictly speaking, I don't really write for people who have Marfan's Syndrome - I write for the ones who are in love with them. However, I do believe that my blog could perhaps inspire some hope, seeing as how this labor of love can be seen as proof positive that life can and does get better. My husband has told me on many occasions that while he may physically be getting worse, in a lot of ways that matter very much, his life has improved over time. He's learned to deal with his disorder, to not fight it or push his body to do things he knows it won't do. He's nowhere near full acceptance of what is just yet, but he's definitely made progress down that path.

     I hope the person who typed, "Marfan's I hate my life" does come back to my blog. I want desperately for them to know that I don't think they've even begun to hit the good stuff in life. Yeah, it's going to be a little different for them and for the ones around them (as I can attest), but it won't be as bad as you think. And yes, I'm not the one who has Marfan's, I can't know what it's like, so on and so on. You're right; I don't know what it's like to be betrayed by your body and never be able to walk away from the sickness. I would counter that with the argument that I can't walk away from the sickness any more than you can. We're equally bound to it, albeit in different ways - you can't physically remove yourself from your body, I can't imagine how I'd survive without my love, my husband. Bottom line, as I see it? This disorder doesn't have to get the best of you and unless you allow it, it cannot get the best of you.

Wednesday, June 1, 2011

I See You, Baby

     My husband and I were sitting on the couch last night, watching season 4 of Buffy the Vampire Slayer, and something happened to me which I thought was a phenomenon exclusive to my husband - I looked at one of the characters on the show and thought, "Marfan's?" Wait, what? I said it out loud, too, "That guy's hand make him look like he could have Marfan's." My husband took this in stride and just commented, "Yeah, it definitely seems possible," but I've been thinking about it ever since. It's the first time I've ever even had that thought about someone else based solely on their physical appearance and I don't quite know what it means, as far as the workings in my head go.

     The episode we were watching, "Hush", is one I've seen a dozen times before. I own all seven seasons of Buffy (I know you're jealous) and this isn't the first time I've watched them, obviously. That particular episode is one I remember watching when the show was originally on the air, because it's such a good one. (Really, if you haven't seen it, you should. There's a reason it won an Emmy.) I only mention my familiarity with the episode because I want it to be very clear how much differently I view certain things now without even realizing it.

     Before my husband, having never heard of Marfan's Syndrome, those actors portraying the villains of the piece, the "Gentlemen", would've registered in my head as just being tall and skinny, IF that characteristic even registered at all. Now, though, because it's such a presence in our lives, I guess it's wormed its way into my subconscious and pops out when I least expect it. Is that a good thing, that I'm apparently more aware of the disorder and those like it? Maybe. Perhaps that means I can see things, just a little, as my husband sometimes does. I know he can spot potential Marfs in the wild more easily than I can, probably because he's so much more aware of the physical characteristics than me. So maybe this means that I'm becoming a little more sensitive to the physicality of his disorder, the obvious presentations.

     Or maybe this is an indicator that I'm becoming overly sensitive to the issue, which is SO not who I want to be. I like to take the most practical approach I can to "sensitive" matters. I don't agree with the mindset of brushing things off and under the emotional carpet, never to be dealt with, but neither do I hold with making every.little.thing about the issue. I can't stand it when people let themselves become defined by what is truly one aspect of their lives, however large that aspect may be. In my case, yes, my husband has Marfan's Syndrome, but no, I am not going to dedicate my life to educating everyone and their brother about the intricacies of the disorder. There is much more to me, much more to him, and much more to us to allow it that large a role.

     That being said, I'm no closer to figuring out where I stand on my newfound awareness than I was when I started. I think, for me, it's more of a curiosity and an, "A-ha! There are more! I found someone else who's like my husband!" than anything else. It's a feeling not so much of solidarity as being not as alone and that's always kind of nice. It is for me, anyway; my husband is still very uncomfortable when a stranger recognizes him for what he is and the one time he saw another Marfan's patient in the wild, he was a little wigged out. I suspect it's because it's not fun looking in a mirror when the reflection is jacked up.