Saturday, May 30, 2015


     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."