Thursday, October 15, 2015


     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...