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valve replacement and an aortic root replacement, (basically, the part of the aorta that the valve connects to has been replaced by a piece of garden hose, which is much more durable.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.



Anonymous said...

I can't seem to forget the day I was reminded how being different is like an abomination to normal people...

There was a program in each school around where I lived, an outreach program to disabled students or 'special' as most say. Slow-learners,Down-syndrome and of course few marfans. Of course nothing wrong with that, People act nicely,mingled without any apparent disgust, but that was during the day.

That night, I went to extra class and as I wait for the teacher to come in, I overheard a group behind me was talking about the outreach program during the day. It goes something like this ;

" Eew.. You saw that down-syndrome people? So weird "

" Tell me about it. Especially those what was it again? Marfan? Look at their arms so long and narrow, ugh disgusting. "

They laughed. I gritted my teeth so hard that I think I might have chipped few. My fist clenched so hard I broke my pen. Good thing of them stopped those idiots and point at me.

I cried that night.

Kristin Lee said...

I want to thank you for commenting, for sharing with me what happened to you. I can sit here and tell you how sorry I am that happened, but to what end? I can't ever know what that felt like, to be laughed at because of something like a genetic abnormality, so I feel like my "I'm sorry" refrain, however sincere, doesn't really mean a whole lot.

I have a lot of hatred for those people, even though I know I shouldn't. Those people are the ones that have done more to break down my husband than the Marfan's ever could and I don't think that's something I'm able to move past at this point.

He read your comment, by the way. My husband just nodded his head and said, "Yeah, I've been there. It sucks," and walked away. I think, even after thirty-eight years of living with it, incidents like the one you described are still too painful for him to think about/discuss with me.

I hope you see this comment, I hope you come back here. I know I'm not the one with Marfan's and I don't understand what it's like for you; how could I? But maybe you'll find something in my writing that you can relate to, or maybe just be entertained by my ramblings. Either way, hope to see you (such as is it) soon.

Anonymous said...

I'm still here.

Yes, I'm happy that you replied and happier that you're not one of those people who said "I understand" how others feel and that you're husband is not like that as well.

I never really tell anyone that incident. I just seem to repress it and make it as a memento, for what? i don't even know. Maybe as a reminder not to really trust or hope the best out of people. How hypocrite a person can get.

Sure enough, the same thing, in that sense happened again. This time from a magazine. There's a article on " The ugliest celebrity in the world " and one of them was the lead singer of Ramones, Joey Ramones. I read he had marfan syndrome. I sighed while reading the article, didn't have the will to shed my tears this time. The article commenting how he's lanky,skinny and looks like a bone demon.

People are really cruel don't you think? if they knew they might not publish such thing, it would be out of sympathy but deep down, they are disgusted and would make fun of us..

Kristin Lee said...

No, I'm not one of those people and never have been. As I said before, it would be wrong of me to say, "I understand what you're going through," when I clearly don't and never will. That doesn't mean I can't empathize on a purely human level.

I get why you wouldn't want to bring that memory up to anyone and maybe you were able to here because I'm completely anonymous. Hell, for all you know, I don't really exist. (I do, actually. Everything I've written about is the truth. I'm a 26-year-old girl who lives in StL with her Marfan's hubby.)

I don't know what magazine that was, but the editor must be a desensitized jackass for allowing such a thing to be printed. If he looked like a bone demon, then so does my husband, because they look very alike. (My husband has long hair, too.) Guess I have a different idea of what's attractive in a guy...

I think people have the capacity to be cruel and the most insecure of them are the ones that lash out the hardest. I don't, however, believe people are inherently cruel; I think it's a learned behavior. Not an excuse for it, just my opinion.

My husband was lucky to find a group of friends who have never made him feel like a freak and luckier still to find me. I'm not ringing my own bell with that last, I'm just saying that I know it was more difficult for him on the dating scene than it was for most people, for a multitude of reasons, all of them Marfan's-related.

Thank you for coming back, thank you for telling me what you did. I know we're on different sides of the issue here, but thank you for letting me know that there's another one out there somewhere who gets it.