Tuesday, December 29, 2015

Oh, Darling

     It feels like it's been forever since I sat down to do this. Nothing has felt settled for months now, not since leaving North Carolina in July. A huge part of that unsettled feeling was because my husband was still there, until about three weeks ago, while I've been here in St. Louis, trying to get us re-established in the Midwest. He's here now, in the place that he considers home, even though he was born and raised half a county away. Home, to paraphrase that annoyingly catchy hipster song, is wherever we're with one another, and we've both been away from it for too long.

     We're currently in the process of setting up our cozy little apartment, getting re-acquainted with semi-communal living after half a decade in rental houses. As I struggle to get the right words out and in the proper order, I'm realizing that I need to get re-acquainted with this as well, because it's, as always, a more important part of my well-being than I realized. It keeps the ugliness, the worries, the parts of my life that are less than good from overtaking the wonderful parts. It's so, so easy to lose focus on everything else and get lost in the fog of, "Well, now what are we going to do?" I hate feeling lost, and though I haven't felt that way in awhile, I think I did lose sight of the ones who do.

     The last blog entry I posted was in the middle of October, right before I did the Marfan Walk for Victory. It's the first event like that I've ever attended since I started dating my husband, and while I think it was a good thing, and one that I/we need to do more often, I didn't quite feel like I belonged there, not like I do here. I'm working on changing that, but the fact remains - I've neglected my tiny corner of the internet, which, despite appearances to the contrary, matters to more people than just me.

     I found two comments on a couple of older posts a few of weeks ago, both from the same person, and they wrenched my heart a bit, because I know the feelings behind them. I am that girl, the one who has a partner who means the world to her, a soulmate who's slowly (and, occasionally, faster than I can keep up with) breaking down thanks to bad genetic luck. I'm the one reading anything, everything, until her eyes are dry and bleeding, in hopes of finding some good news, but coming up empty-handed. And that right there is the whole fucking point of airing my dirty laundry - I'm trying to keep others from those futile searches.

     You (yes, ma'am, I mean you who posted the comments) said that something's gotta give in your life - it will. I can't say that it will be a good something, because in my experience, it gets worse before it gets better. Example? My husband and I are currently in a "worse", waiting for it to get better. He was supposed to start his new job two weeks ago, but thanks to administrative forces beyond our control, it's been postponed twice, and by the time he does finally get to start, we'll have been on only my paycheck for a little over a month. Not the best position to be in under normal circumstances, but when you factor in that we now have to pay for a month of COBRA along with all of his regular monthly medical expenses, it starts to get ugly.

     I know that if we just wait it out, though, the pendulum will swing back the other way. I also know that sounds like I'm being blindly optimistic, but I swear I'm not. (Mostly because no one who knows me in real life would ever use the word "optimistic" to describe me. Ever. Even on my best day.) The thing is, if I've learned anything since my husband and I have been together, it's that the universe seems to pull us back from the edge every time we think we're about to go flying off of it. I don't know how and I don't know why, but it does; that much I'm sure of.

     You also mentioned that reading about this is easier than talking about it. That, I absolutely understand. I mean, not only do I write this blog, but I carry around a journal thick enough to knock somebody out with, because there are times when I just can't concentrate on what I'm supposed to be doing, and I have to quiet the roar to get anything accomplished. Even now, I'm sometimes self-conscious about speaking to anyone about my husband's disorder, because what if they don't care? What if they're just being polite and didn't really want to know the nitty-gritty? So, I write, because that's a much less obtrusive way for me to handle it. This way, if someone gets TMI, they have no one to blame but themselves.

    More than anything, I wish I could make it better for you. I wish I could tell you that it gets easier, but I don't believe it does - I think you just constantly adapt to your environment. You're probably going to hit a point that you think has gotta be the wall, the break-point, your absolute limit. It's not, though, not in my experience. My take on it? You don't have an absolute limit. (Which is a thought both awesome and terrifying.) Every time I've been where you are, that feeling of "I can't do this anymore, I canNOT get my teeth kicked in again," I've somehow come out the other side of the tunnel. And since we're being honest here, I'll just throw it out there - no, it's not my "faith" that's sustained me. I'm agnostic bordering on atheist, as is my husband. We are what got us through, with a kick-ass support system comprised of our families, friends, and co-workers.

   Also? Sometimes strangers that I've connected with through this blog. They find my ramblings and reach out to me, and if we're lucky, we're able to carry each other through. I can provide that for you, a place to come and read and hopefully feel the comfort of knowing it's not you against the world - it's us. It's a tiny, tiny, tiny light in the dark, like, a dying-lighter-flame-sized light, but sometimes that's enough. 

Thursday, October 15, 2015

Walk

     This Sunday, I'm going to do something that feels foreign and uncomfortable - I'm going to actively seek out other people with whom to talk about Marfan Syndrome and its effect on my and my husband's lives.

     That doesn't mean I'm going to go out looking for people on the street who look friendly and say, "Excuse me, do you have a moment to talk about Marfan Syndrome?" No. That's how you get branded the neighborhood creeper, and then people walk on the other side of the street when they see you coming. What I'm actually going to do is walk in the Marfan Walk for Victory, along with my best friend, because this is important, in more ways than one.

     I've been with my husband for six years, and writing this blog for four of those years, and I have yet to speak, in person, with anyone else who has Marfan Syndrome. Honestly, the vast majority of people I do speak with about it have no idea what it is, because so few people have ever heard of it. Isaiah Austin has certainly raised visibility of this disorder to some degree, but still, it's one of the ones that tends to lurk in the shadows, without much funding for research, or resources for those who live with it. It's frustrating for me, because it's not something that's easy to talk about, but the longer I, we, everybody keeps our collective mouth shut, the longer it stays in the shadows.

     It's easy for me to hide, in a sense, behind this blog, because one of the things the internet offers is a glorious sense of anonymity. It's not completely anonymous, of course, since I do post these things on my Facebook wall, and most people who know me in real life know I have this, but it does allow me a lot of freedom to say what I want or need to say without having to say the words aloud, immediately. That's huge for someone like me, who has a lot going on in her head at all times, which, when combined with a somewhat-impulsive nature, can result in saying things faster that I can consider the potential consequences of giving voice to those thoughts.

     I realized, though, that if there's any hope of my husband and I getting to a better place than we are/have been with regards to his health issues and waves they create in our world, I can't keep to the same path I've been walking for more than half a decade. We have to get out, have to at least try to find others like us, because I know damn good and well they're out there. Jesus Christ, we (or at least I, we're working on the "we") live in St. Louis, MO, home to one of the best Marfan clinics in the country. If we're going to find like-minded individuals, this is a pretty good place to start the search.

     So this weekend, I'm going to gather up my friend, and we're going to journey out to Queeny Park to see what we can do about meeting more Marfan people. By all appearances, it's a good community to be a part of, and I think I may even be able to add to it with my perspective. Or maybe that's just wishful thinking. Either way, here we go.

Tuesday, October 13, 2015

Spookshow Baby

     Apparently, we're living in a horror movie now. I was sitting at work this morning, minding my own business and cursing the annoyance that is insurance adjusters, when my husband texted me that he'd just left the eye doctor's and he had some news for me. The good doctor had informed him that if his cornea does not make "significant improvement" in the next six days, he's going to have to move on to the next option for treatment - SEWING PART OF MY HUSBAND'S EYE CLOSED. I wish I were making this up.

     Ever since my husband underwent emergency surgery to repair his detached retina, that eye has been abnormally red and inflamed. Finally, after his final follow up with the surgeon, who knew that the cornea should have looked a lot better than it did at that point, he was referred to a cornea specialist. (Not gonna lie, I didn't know such a thing existed.) The new doctor did an exam and concluded that the cornea was abraded and inflamed because of all the poking and prodding that it had recently undergone, and frankly, it was just fed up.

     Wikipedia tells me that the cornea is made up of different layers of connective tissue, though I didn't see that red-flag word - fibrillin; I only read that there's collagen involved. So, how much of this connective tissue is affected by the Marfan's, I don't know, since collagen production isn't a problem with Marfan's. This most recent circus is, however, a secondary result of the underlying syndrome, inasmuch as the previous monkeying-about with my husband's eyeball was a direct result of Marfan-related issues. His lens dislocated over a decade ago and a new one had to be implanted, the suture from that surgery gave out in 2011 and had to be repaired in an emergency surgery, and his retina detached a few months ago, which resulted in yet another emergency surgery.

     Combined with years of contact-wearing and the subsequent daily eye-poking, all those procedures really did a number on his eyeball, which wasn't in the best shape to begin with. Honestly, I think it would have been difficult for a healthy person's eye to withstand so much, but in his case? I suppose it was really just a matter of time before hit a wall yet again.

     He's been wearing a protective contact for the past few weeks, and that, along with the special preservative-free eye drops he's  been using daily, have improved the situation to some degree. He confirmed last night that it no longer hurts, and he no longer looks like a sleep-deprived maniac. It's just not enough, though, and when he went to the specialist yesterday, he was given the potential next step of removing the contact and sewing the outside third of his eye closed for the next six to eight weeks to allow the corneal surface a chance to really heal itself.

     Seriously? Are we seriously moving to Frankenstein-esque treatment methods now?

     Sorry. I know I should give this a chance, especially since we're not yet 100% certain it's going to happen. The truth, though, is that my husband and I both know that his body is not going to miraculously speed up the healing process that has so far moved at a snail's pace. Why would it? How could it?

     I'm not even sure how sewing the eye closed would be better for him than the protective contact, and when my husband posed that very same question to the doctor, he was told, "I can't explain how it works, but I know it does." Now, what the doctor was likely trying to convey was, he can't really explain in layman's terms how or why this is a thing, but he, the one who's had years of experience and medical school training, knows what's going on. And honestly, we're both fine with that, because despite everything, we both still have more faith in medical science than anything else.

 

Monday, October 5, 2015

Cause for Alarm

     It wasn't hyperbole yesterday when I said that my husband had to undergo emergency surgery. By the time we conceded the fact that there was something seriously un-good going on in his eye, we were almost too late.

     I feel like there are times when people, sometimes even those within the Marfan community, forget that Marfan's is a connective tissue disorder - and that includes all of it! Every single piece of those fibers within my husband's body are flawed; his very foundation is cracked, hopelessly. The most dangerous condition to result from is an enlargement of the aorta, which can and often does lead to aortic dissection, though that, thankfully, is not something we have to worry about, since he had his aortic valve replaced with aftermarket parts over a decade ago. However, there are a plethora of other things that can go wrong, and the eyes, a delicate part of the body on the healthiest person, are especially susceptible in my husband's case.

     He hadn't done anything particularly strenuous even by his standards, when he started to notice that he was losing vision in one eye. It was the eye that had been operated on years ago, when the lens dislocated (another common Marfan symptom) and a new one was installed. He's had issues since, like that time when the incision opened up and he squished the eye juice out in his sleep, but nothing that hasn't eventually gone away. We're even used to him bleeding into his eyeball(s) on occasion when his INR is off and his blood's too thin; in fact, we've actually come to view that phenomenon as a handy little indicator of whether or not his warfarin is off.

     We let it go for a few days, hoping that it was simply blood in the eye like it's been so many times before. This time, though, his vision became less and less every day, until all that was left was a tiny fingernail-sliver of sight at the top of his eye. No bueno.

     He called his eye doctor, and when he told the front desk what was happening, an appointment was found for that same afternoon, probably because the medical professionals knew what we were trying to deny - the Marfan kid had a detached retina. It's always been a back-of-the-mind fear of his, at least since I've been around, and until now, we've been lucky in avoiding it. He knew what it would mean if the retina did detach - namely, surgery. And surgery for him is bad news any way you look at it.

     The problem with my husband and surgery is that he can't be put under general anesthesia without weeks of preparation. When you're on warfarin for a metal valve, it's different than being on warfarin to control blood clots. Generally speaking, people who take it to control clotting are on a lower dose than he is, and they keep their blood thicker (literally) than he has to. Why? The human body is a clever, clever devil sometimes, and in his case, it knows that that titanium valve is not supposed to be there. In an effort to defend itself from the supposed foreign invader, his blood will clot up around the valve and try to force it from the body. Obviously, that's not possible, so what happens instead is that the clot that forms then breaks up into tiny little pieces, which can lead to a life-threatening stroke. Neat. (That was sarcasm, if you couldn't tell. It's already happened once and it sucked. Hard.)

     There's a way around it, which I'm not going to bore you with right now, but it takes a couple of weeks to accomplish, and that was time we didn't have. So just like last time something went wrong with his eyes, the doctor, once he determined that he was indeed dealing with a detached retina, told my husband that he would have to have surgery, like, yesterday if there was any chance of saving his sight, and he was just going to have to hold as still as possible while they deflated his eye, cut into it, and sewed that pesky little retina back on.

     I know more than my fair share of people who have issues touching their eye to put a contact in, so can you imagine what it's like to be AWAKE when the surgeon is cutting your eyeball open? It has to be like you're trapped in a horrifying Devil's Rejects/Clockwork Orange mashup. I mean, I've had eye surgery myself, and not the laser kind, but I was fully asleep while the good doctor did what he had to do. I don't know, nor do I care, how he accomplished the task he set out to do, and I'm okay with that. My husband, though. He experienced everything in glaring detail.

     I went into it suspecting it was going to take the full 2 hours the doctor advised it might, because a 42-year-old Marfan patient is so far from a good surgery candidate it's not even funny. Needless to say, I was not one bit surprised when the receptionist at the front desk of the surgery center turned off the lights and went home for the day, while I was still in the waiting room, watching the double doors that led to the recovery bays.

     Eventually, his surgeon came out to get me and let me know that it had gone well, and though we wouldn't know for sure for weeks, possibly months, whether or not his vision was completely intact, he was hopeful. I went back to see him, to gather up all the things that come with a day of surgical adventures, not the least of which was my husband's new face-doughnut. What's that, you ask? It's like a butt doughnut, the one you get if you break your tailbone, but for your face. Why, you ask? Allow me to elaborate.

     The fix for a detached retina is not quite as simple as sewing it back in place, as it turns out. First, the eyeball is drained of some of its juice, in order to allow the surgeon to go in and reattach the retina. Once it's secured, they then add a gas bubble to take the place of the juice and help keep the retina in place while the stitches heal. For the first 72 hours or so? You have to keep your head in a certain position, mostly face-down, so the gas bubble stays in a certain position, so that everything has a chance to heal properly and this whole thing wasn't just an exercise in futility. So yeah. Face-doughnut.

     The worst part of it, though? The fact that I had to leave him five days later.

Sunday, October 4, 2015

Unfaithful Servant

     This is the longest break I've ever taken from posting since I started this blog, and so.damn.much has happened during that time.

     I'm back in Missouri, and my husband's still in North Carolina. This is a less-than-ideal situation, especially when you consider that the main reason we moved back was so that he could resume treatment for his Marfan's with his specialists here. He can't come back until he secures a job in St. Louis, and so far has had no luck. This is, obviously, beyond frustrating for us, as it would be for anyone in the same situation; it's made worse by the fact that he's still dealing with the repercussions from an emergency surgery that went down right before I had to leave to come back to the Midwest.

     Yeah. Emergency retinal detachment surgery. Why? Because it decided to detach the week before I was scheduled to leave for good. I was able to push my departure date back by a few days, but I couldn't stick around as long as I would have liked, because I was lucky enough to have a job waiting for me, and I already had a scheduled start date. So that happened.

     There are about 936 other things that have happened in the four months since I've posted, and I'm finally in a place where I can start doing this again with regularity. I know nobody's hanging on the edge of their seat waiting for these updates, but I've had enough conversations with enough people from locales near and far to believe that reading about my and my husband's adventures with Marfan Syndrome help them somehow, in some small way.

     And so the saga continues...

Saturday, May 30, 2015

Torn

     Walking out of work the other night, one of the tendons in my husband's ankle tore halfway through. He didn't step wrong, he didn't roll his ankle, he wasn't coming down a flight of stairs - he was just walking across the flat surface of the parking lot at the end of the night. By the time he got home, it was already starting to swell. Dammit.

     He's been getting steroid injections in his ankles for a few months in order to alleviate the burning pain that was making mobility difficult for him, and he'd had no problems up until this point. That's not to say that the injections have anything to do with the tearing of his tendon, because they likely don't. Marfan's is a connective-tissue disorder, and that's exactly what tendons are made of. The dengenerative nature of the disorder meant that it was really just a matter of time before something like this happened, and frankly, we got off kind of lucky this time.

     It wasn't any better the next morning, so I suggested he go to the urgent care, even though that's one of those proceed-with-caution things due to past experiences there. At the very least, I wanted him to call the orthopedist to see what they could suggest. He did, and there had apparently been a  cancellation for that morning, so he was told to come in to be examined.

     His ortho did the exam and figure out that he'd torn one of the big tendons in his ankle, I think the one that runs from just above the ankle bone down to the arch of his foot, about halfway through. We have to take our breaks where we can get them, so I was just grateful that he hadn't torn it all the way through, which likely would have required surgery. Not only would that mean time away from work coupled with extra medical bills, but my husband's a lousy surgery candidate, mostly due to the blood thinners he's on. His susceptibility to spontaneous pnuemothorax doesn't help the situation, either, as that can and has happened during a previous surgery.

     The doctor wanted to put him on prednisone, which a steroid that everyone ever has probably been on at some point or another, and was going to be most helpful in reducing the swelling and helping the tendon to heal. This, in addition to a heavy-duty ankle brace/walking boot hybrid. Problem? The lovely, stroke-preventing blood thinners that my husband has been taking daily since getting his after-market heart valve installed a little over a decade ago.

     Steroids typically don't play nicely with blood thinners, a fact we learned the hard way about three years ago, when he was issued a prescription to clear up an infection in his elbow. Unfortunately, not only did they not clear up the infection, they reacted with the warfarin and shot my husband's INR up so high that he was hospitalized for several days because they were afraid he was going to start springing bloody leaks at any moment. What doesn't kill you, eh?

     Now that he's aware of the potential danger posed by the steroids, my husband made sure to ask the ortho whether or not it was a good idea to take them alongside his blood thinners. If it wasn't, the plan was just to wear the brace and suck it up, because not taking his warfarin is not an option. Teamwork to the rescue, though, because the ortho said that he'd talk to my husband's primary care physcian about it before sending the order to the pharmacy. If she said no, then the only option was to suck it up, buttercup.

     A compromise was reached, though, one which everyone felt comfortable with. My husband could start the steroids, but he would have to go get his INR tested twenty-four hours later so that they could be sure he wasn't about to bleed into his eyes, or suffer the alternative, which was a possible mini-stroke (again) due to his blood being too thick. I say that because that's the other possible interation between blood thinners and steroids - they make the blood thinners much less effective, thus thickening a person's blood to potentially dangerous levels. This has happened once before, and the result was my husband suffering a mini-stroke one day while I was at work. It's not an experience I'm eager to repeat.

     We got lucky again, and when he went in the next day, his INR was at 2.4, just a tiny bit outside the lines of acceptability, but certainly no reason to get alarmed. His pcp gave him the thumbs-up to continue the course of meds, and his ankle is doing much better, though he's still wearing the brace.

     What seems like a minor incident, though, is actually more likely a portent of things to come. Like I've said before, Marfan's is a connective-tissue disorder, and it's not selective in which tissues it affects. His aortic valve has been replaced, and that's the biggest danger zone, but what about the rest of him? It's becoming more and more of an issue, the overall deterioration, and no one seems to know how to stop it, or at least slow it down to normal levels.

      

Sunday, May 3, 2015

The Weight Is More

     I'm just gonna go ahead and get this off my chest, since I kind of opened the door yesterday when I was talking about the mobility issues my husband's currently experiencing. There are a lot of times we come out on top when we're pushing back against the Marfan's, and there are a lot of times when we don't. In fact, there are a lot of times when we don't even know how to arm ourselves for the particular battle at hand, and so we're on the losing side before we ever show up.

     I think there are other people out there who probably feel the same way, and I think that helplessness could be alleviated, in some small part, by the powers that be making it a point to remember that this disorder doesn't just affect the heart, the aortic valve - it affects every single piece of connective tissue. I get so frustrated sometimes when I'm looking for something, anything, that could help explain why my husband's symptoms are doing what they're doing. More than that, I get frustrated when we know why they're causing chaos within his body, but there's nothing out there about the treatment of the same. Hell, we both struggle just to find anecdotal stories most times, nevermind actual medial articles.

     The thing is, I know it's the aortic valve and the possibility of dissection that's one of the main, if not THE main issue when it comes to Marfan's; I know it's potentially the most deadly. An aortic dissection has so much potential to be the fatal flaw, and if it happens, there is every possibility that the person to whom it happens isn't coming back. I'm very, very aware of this, just like I'm very, very grateful for the titanium aortic valve my husband now has. That clicking in his chest provides me no small measure of comfort, because it's an indicator of one less nightmare scenario.

     However, it doesn't do anything to address the concerns of the rest of his mind and body. And yes, I say mind and body, because one of most painful symptoms my husband suffers with is major depression. We're struggling to find help for so many of his problems, and the mental component of living with a chronic disorder seems to be the one that's given the least attention of all. I mean, I even went so far as to email The Marfan Foundation to see if they could point us towards a therapist in our area who worked with disabled individuals, and the response I got back was that they had no one to suggest. It's not that I expect them or anyone else to have all the answers, because that's not possible or even realistic to expect. It's disheartening, though, when one of your go-to's, if not your main go-to, doesn't have any more answers than anyone else.

     I don't want you to think that I'm ripping on The Marfan Foundation or any other organization for not having every answer ever; I'm not. I think they do great things for the Marfan community, and I think they provide a great centralized point of contact for the thousands of people struggling with this disorder. They, along with so many other organizations for so many diseases and disorders, are working with what they've got, to the best of their ability; what they do is substantial. What I'm saying is, I've found a definite lack of resources anywhere for the Marfan patients who are like my husband - ones who have had the aortic-valve replacement surgery and are out of immediate danger, but still struggle with the toll the disorder exacts in other ways.

     What about chronic pain? Every website I've ever looked at acknowledges that chronic pain is almost a certainty in Marfan patients, yet I can't find much of anything new on it. What about new surgeries, new pain management techniques? What about looking into ways to increase mobility for the 40+ crowd, those who've exceeded the pre-surgery expected lifespan? (And what I mean by that is that if my husband wouldn't have been able to have his aortic valve replaced, his life expectancy was somewhere around 31, 32; he'll be 42 next month, thanks to the after-market parts.) With the valve replacement surgery becoming more and more common, and Marfan's patients living near-normal lifespans, what happens to them when the rest of the connective tissue starts to give out?

     I've mentioned a few of these issues before and gotten replies, emails and such, from other people with Marfan Syndrome who find themselves in a similar situation, experiencing some of the same issues. They're always quick to offer their stories and suggestions, things that have worked for them. These people give us hope, and they give us things to keep trying, suggestions to keep making to his medical providers. It's about all we can do at this point. Chiropractic care? Sure. Physical therapy? Yeah, why not. Cold-laser therapy? Let's give that a shot, too, even if we don't know what the hell it is. (Seriously, he's going for his first treatment later this week. It's either cutting edge medical technology, or something out of a Marvel comic. Either way.)

    The thing is, I know it's out there, the research, the procedures; I know it's not as though the powers-that-be are ignoring this component. I mean, the Losartan trials are proof of that. I'm not sure how much good that will do my husband, though, because we're past that point - he's already got the hardware. It's fantastic news for the younger generations, though, and that's a really, really good thing. I guess i just feel like we're stuck, because he's of the generation that there's not really much data on. Dr. John Lynn talks about it in an article for the Summer 2014 issue of Connective Issues and says, essentially, that there's just not enough data on this to be able to draw any real conclusions. I suppose, then, that's my real frustration - the lack of information, rather than a lack of willingness to go after it.

     And what about the mental health help? This is, in my opinion, definitely one of the most dangerous aspects of my husband's disorder, and I refuse to believe he's alone in that. He's been through a slew of therapists, and finally seems to have found one that he's able to work with. It's an extremely delicate relationship, because he's entrusting a total, albeit thoroughly trained, stranger with the most complex organ in his body - his brain. I can't find any real statistics on it, but I would be willing to bet every dollar in my bank account that depression is extremely common among individuals living with chronic illnesses. Why, in 2015, does it seem as though no one wants to talk about it, still?

     I think it's always hard being on the front edge of something, as my husband kind of is, just by virtue of his age. He was already twenty three and about to undergo his first major surgery before the diagnostic criteria of Marfan Syndrome was agreed on, so it stands to reason that in this case, being first in line isn't necessarily a good thing. He's the one who's watched, rather than the one doing the watching.



     

Saturday, May 2, 2015

The Ghost in the Machine

     It finally happened, and I have to say, I'm kind of surprised it took as long as it did. I mean, my husband looks able-bodied, and I am able-bodied, so when someone sees us getting out of our car, I doubt very seriously they would ever think we have a need to be in the accessible parking spot we just pulled into. Even if they did see the baseball-sized tattoo on his left forearm, I would put money on the fact that more often than not, someone's going to assume we took my grandma's accessible parking permit to use. The truth, though, is more complicated than that, and I wish like hell the woman who was doing her best to kill us with a look when we were walking into the grocery store the other day would have at least made the attempt to contemplate that possibility.

     The thing is, even though my husband was born with Marfan's Syndrome and lives with that debilitating disorder all day, every day, the vast majority of people have no idea he's disabled. (And before you launch an "able-bodied" vs. "disabled" war of words, just stop. It's the term my husband feels best describes him, so that's what I'm going to use.) He works a full time job, and between the small army of specialists, primary caregivers, and the fentanyl patches, he's still pretty mobile. Well, I suppose I would be more accurate in saying that he was still pretty mobile, until this past winter.

     We were at the mall sometime around Christmas, and he discovered, much to his dismay, that he couldn't do more that 75 yards or so at a time without stopping to rest because his ankles simply wouldn't carry him. This was disconcerting, to say the least, because ankles are fairly important in the grand scheme of things. We didn't know what the hell was going on, and frankly,  neither did his pcp, because at this point, his medical care is as a much a guessing game as anything else. So, she referred him over to an orthopaedist for further inspection, where it was discovered that the tendons in his ankles were essentially useless.

     Marfan Syndrome is a disorder of the connective tissue, and that means every single piece of it, anywhere in the body. I know there's so much focus on the aorta and the heart, but that's only ONE of the many systems in the body that's affected. Know what another one is? Musculoskeletal, which includes tendons! Basically, since his body doesn't produce the necessary amounts of fibrillin, the ligaments and joints in his body are falling apart. We knew it would happen eventually, and it seems that that day has come.

     So what do we do now? Well, the ortho said the tendons in his ankles are just hanging there, like old elastic, which is why his feet are so absolutely flat - there's nothing pulling his foot up to create the arch. This also means that the tendons don't do their job in supporting his ankles, and they're kind of just swinging free, taking on all of weight of his body and doing the work to propel him forward with no supporting structures. Neat.

     He's had two rounds of steroid injections directly into the tendon, and that seems to provide some relief, though we're not sure how long that's going to last. Like almost everything else, we're in a holding pattern of let's-wait-and-see-how-this-works. Hence, the reason for the accessible parking tag that we've only recently starting making use of. Makes a little more sense now, yeah?

     My husband has had it for almost three years, technically, because his doctor told him point-blank that he'd better start thinking about doing anything he could to minimize the everyday wear and tear on his body. If that meant twenty less steps to walk to get into the store, then do it. Even after such a straightforward discussion, he and I were both reluctant to use it, each for our own reasons. His, though, was primarily because he was not looking forward to the day when some sanctimonious jackass decided to offer their completely unwanted opinion on his using an accessible parking tag.

     I'm not even going to say "to her credit" when speaking about the woman who clearly thought we were abusing a privilege the other day, because she deserves no credit. None. I will fully admit to having a (possibly unreasonably) long list of pet peeves, but high up on that list? Sanctimonious jackasses. I can't stand people who thoughtlessly make their opinion on a subject known, without knowing the first thing about said subject or the specific situation. It's just rude.

     I know he doesn't look disabled, he knows he doesn't look disabled. Not to the casual observer, anyway. To those who know him? It's obvious that his legs are slightly, unnaturally perma-bent at the knee - because his hamstrings are too short to allow him to fully extend them. It's clear that he's not walking at the same pace as everyone else, because the nightmare that is his spine won't allow him to move very quickly. Why do I never push the cart in the store? Because he needs to lean on it to remain upright for periods of time longer than 20 minutes. If he doesn't have that, or his cane, or something else to help him stand, he has to sit down and take breaks, then continue.

    And so, lady who clearly thought we had no business parking in that accessible spot, you would do well to keep your death-stares to yourself. I wouldn't have called you out on it, and I didn't, because a) I detest those who think it's acceptable cause confrontation over anything, everything, and b) it would have embarrassed my husband terribly, and he's already painfully self-conscious enough most days. Instead, I'll settle for hoping that someday, you can open your pea-brain just enough to allow for the thought that, "Hey, maybe just because I can't see something that screams DISABLED PERSON!!!! about that gentleman, it doesn't mean there's not something going on behind the scenes." 

Thursday, April 9, 2015

Electric Worry

     You know how when things have been good for a long time, you're sometimes waiting for the monster at the end of the book? I kind of feel like that right now. I'm having trouble deciding if the foreboding is just me being paranoid, or if it's the result of experience with this scenario and knowing what likely comes next. I'm really hoping it's the former.

     It's been more than a year since my husband had to go the emergency room because of the crippling pain in his spine, and that's a minor miracle that we seldom take for granted. He's currently rocking a 50 microgram fentanyl patch for his pain management needs, with oxycodone as back up for the breakthrough pain, and that's been the magic potion for the past fourteen months or so. In the time I've been with him, which is almost six years, this is the longest he's ever gone without an emergency room visit. Hell, if we had to go tomorrow, I wouldn't remember how to navigate the various parking lots at our nearby hospital. That's a really good feeling, by the way.
 
     It was a solution that his doctor stumbled upon almost by accident, when my husband mentioned in passing one day that he knew of someone who used it; it was a lightbulb moment. Everything's got two sides, though, and one of the problems with being on that heavy a dose of opioids (it's about 80-100 times more potent than morphine, 15-20 more than heroin) is that it can mask what's actually going on underneath it all until the situation is pretty far gone. There is every possibility in the world that I'm getting ahead of myself in my fear, but I'm having the worst feeling that this could be what's going on at the moment.

    His back pain is never gone, not all the way, but the intensity comes and goes, and the majority of the time, it's a low-frequency hum. By that I mean it's kept at a level that his body can adjust to in order to go on about its business, like when you get a tattoo. Technically, you're being pierced with a needle or needles thousands of times per minute, and that should equal OHMYGOD PAINFUL, but in reality, your body typically adjusts the pain level after a little bit and it becomes bearable, sometimes amounting to little more than an annoyance. Most days are like that for him.

     Lately, though, it's started to change, and that's why I'm getting nervous. His back has been at flat-out painful levels for over a week, to the point that he can't comfortably sleep in our bed every night, as it's too soft. Instead, he falls asleep on the firmer couch, which I suppose offers him more support. I don't think I would be as worried under other circumstances, but for the pain to persist for this long, at this level, while he's wearing his patch and using the oxy like he's supposed to? What the hell is going on back there?

     

Monday, March 30, 2015

Real Solution #9

     One of the hardest things for my husband is talking about himself or his disorder, which are, too much of the time, the same thing in his head. Truthfully, when you live with it day in day out, it becomes more and more difficult to extricate the two, and they become one hopeless,warring tangle, a construct of blood and oil. I do it, too, though not, I think, to the same degree that he does. It's not a conscious decision on anyone's part, I'm fairly certain, but it's one that becomes harder and harder to change the longer we exist with it. Someday, both of us will learn how to make peace with his jacked-up genetics, but we're nowhere near that finish line yet. Still, I think he moved one tiny step closer this weekend when he agreed to talk to our friends' daughter for a school project.

     Our friends Adam and Carrie have a twelve-year-old daughter, and she was apparently assigned a genetics project in school. Rather than go for the standard "make a model DNA strand", which anyone in possession of toothpicks and glue has been doing since time began, she chose to interview my husband about his genetic disorder and create an information pamphlet about it. I think her choice shows initiative and an aptitude for thinking independently, but I'm biased. Long story short, she asked if she could conduct an in-depth interview with him, and he agreed.

     He was nervous in the couple of days leading up to it, and honestly, I think it was better that the question and answer session happened sooner rather than later, before he had even more time to think about it and worry over it. As long as I've known him, he's always been someone who has internalized whatever's going on in his head, good or bad, and the prospect of talking about not just himself, but the often-troublesome part of himself, was stressful. He never talked about not wanting to do it, though, and whether that was because he's fond of Riley or he knew that this was a baby step towards something bigger, I don't know. Hell, I doubt he knows. I doubt he even thought about it, for that matter.

     I sat in on the interview, as did Carrie, and I think it went really well. Riley came fully prepared with a list of questions, plenty of paper to take notes, and a recording app so she could revisit the conversation as needed to create her brochure. My husband explained how he was first diagnosed, that it was actually a gradual process, as a collection of symptoms was realized, rather than a positive blood test or something of that nature. He showed her the various physical manifestations, like his oddly-angled thumbs, and his wingspan that's wider than he is tall. (And he's 6'5", so that's saying something.)

     He explained the different surgeries he's had, beginning with a spinal fusion when he was twenty-four, and continuing until just a few years ago, when he had to undergo emergency eye surgery to repair a hole in his eyeball that he'd accidentally opened one night while sleeping. It's kind of gruesome if you look at it from a purely medical standpoint, but he handled it with no small amount of humor, so she wasn't overwhelmed by it. Plus, Riley digs the creepy, so it's not like she's easily icked-out.

     I don't know if it was the fact that he was speaking to a child, someone who never thought to pity him or judge the choices he's made in regards to his medical care, but he was pretty relaxed throughout the conversation. Some of that could have just been the particular child/pre-teen he was talking to, as well. Riley's a smart girl, well aware that my husband isn't healthy, and she just accepts it as part of him. I've never known her to feel sorry for him, or anything close to that. Though we've never talked about it, I get the sense that she views him as just another of her step-dad's friends; he's not her step-dad's friend who's sick, which is how more than one adult acquaintance has classified him. Sometimes, I really believe kids are far superior to their adult counterparts.

     She took a picture of his medical tattoo for the cover of the brochure, and I was able to find his spinal x-rays so  she could see the severe scoliosis that made his spine resemble a snake, plus the ones that showed his titanium hardware that's now a permanent part of his frame. I've seen them a dozen times or more, and every time, I'm shocked all over again that he was still walking around with that catastrophe.

     It was actually a really pleasant couple of hours, one that he became more comfortable with as the conversation went on. (I could see him start to relax and become more animated when answering questions, so I know he was feeling better about it as we went along.) I can't say I was surprised that it wasn't that big a deal, mostly because I never think it's a big deal to talk about myself. And I take such liberties here to talk about my husband that I don't give it a second thought to answer questions when asked. My feelings on the subject don't really matter, though, because I'm not the one who struggles with it.

     He does, and for good reason. There's so much there, things that he's working on, things I'm not ready to discuss, but I think Sunday was a good day. Maybe it was because Riley was a friend, maybe it was because she approached him with a kid's innocence, no judgment, just curiosity and a desire to know and understand. Whatever it was, I believe she helped my husband as much as he helped her, by cracking open, just the tiniest bit, a door that's been closed his whole life.
   
     

Thursday, February 19, 2015

Since I've Been Loving You

     I will be the first to admit that I am really, really bad at being sick. And that encompasses the whole spectrum of sickness, from your basic cold all the way to surgery. It's not my finest hour, and God bless anyone who's not my parents for sticking around for any length of time when I'm under the weather. As you'd imagine, the closer my illness gets to the "surgery"end of that scale, the more determination it takes for the person taking care of me to stick around and get the job done. So when I found out I was going to have to have surgery on my sinuses, I knew my husband would not be looking forward to the special, special times to come.

     I actually had the surgery done last Monday, and though it was outpatient, that procedure marks the first time I've been the one going under the anesthetic since we first got together in August of 2009. So that's what, five and a half years? In that half-decade, I've been by his side for 2 surgeries, a mini-stroke, hundreds of ER trips, and countless in-patient hospitalizations. I can rattle off his medications, including how much of each he takes and when, like most people rattle off their Social Security numbers. At this point in our life together, it really feels normal. What feels weird is being the one in the hospital gown with the IV in her arm, because that's a complete role-reversal.

      I believe there's a reverse side to everything, and maybe times like these are where the silver linings make themselves known. Like I said, though he's usually the one in the hospital bed, our positions were reversed here and I have to say, it worked out for the best.

     While my surgery was outpatient, meaning I didn't have to spend the night in the hospital, it still sucked. Hard, it sucked. I've been under general anesthetic before for previous surgeries, so I knew the roughest part for me (and my husband, consequently) would be in the recovery room, when I was coming up from under the drugs. Sure enough, the first thing I was aware of was not being able to open my eyes and having the distinct impression that there was a razor blade anchored at the back of my throat that was trying to force its way down. Ugh.

     He was there, my husband, Gatorade and ice chips at the ready. He's had so many procedures, spent so much time in a hospital bed, that he knew before the nurses told him what he needed to do, what to have ready for me when I came to. There's a huge comfort there that simply cannot be overstated, especially when you're disoriented and the simple act of speaking is intensely painful. It was a combination of hand gestures, telepathy, and his first-hand knowledge that got us through the first night after surgery as easily as could be hoped.

     For example, thanks to his long history with every painkiller imaginable, (thanks, Marfan's! He wasn't using that spine anyway...) he knew exactly what the demerol prescription would do to my sensitive stomach, especially after it had already been subjected to the trauma of the general anesthetic. Hence, the pills stayed in the bottle, and I rode out the night alternating bags of frozen peas and corn on my face to alleviate the pressure and discomfort. It sounds like such a small thing, that he would have that forethought, but I assure you, it wasn't. It was huge in a moment when I wished desperately that I could just take my face off and set it aside until it felt better. (Though that imagery is kind of creepy when you think about it, because Face/Off.)

     There were a million other things over the past two weeks that I'm sure I don't even remember. I think it's more of an overall knowing exactly what I needed, sometimes before I did, that made this so much better than it could have been. It feels strange to say that I'm grateful for my husband's experiences and subsequent knowledge of how to care for me in this instance, because that knowledge comes from his living with Marfan's Syndrome.

     Not a day goes by that we don't wish he was healthy, that his disorder wasn't a part of our lives, but (and I really, truly hate this phrase, however accurate) it is what it is; it's part of him, literally a piece of his DNA. So, rather than get frustrated this time over something that will never change, look at it from a different angle - for once, his disorder has given us something, instead of taken it away.  It's a tiny silver lining, I know, but sometimes that's all that's needed.